Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
Clinical history: 50 y/o Female with a breast mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
Clinical history: 50 y/o Female with a breast mass
Correct
Diagnosis: A. Granular cell tumor
Histology: The resection specimen reveals an infiltrative process comprised of epithelioid cells with round nuclei, centrally located and prominent nucleoli, and abundant eosinophilic granular cytoplasm. The cells are infiltrating around and through benign ducts and lobules. There is no evidence of an atypical intraductal proliferation. There is no appreciable mitotic activity within the cells. Immunostains show the cells to be diffusely immunoreactive for S100 and inhibin, and to be negative for AE1/AE3 and Cam5.2.
Discussion: The morphologic features are classic for a granular cell tumor, which a type of peripheral nerve sheath tumor that can rarely involve the breast. Granular cell tumors characteristically involve the head and neck, where they can incite a prominent pseudoepitheliomatous hyperplasia in overlying squamous epithelium. In the breast, granular cell tumors clinical, radiographically and grossly mimic infiltrating carcinoma because they can appear as a fixed, stellate and infiltrative solid mass. Histologically, granular cell tumors display abundant eosinophilic, granular cytoplasm and can be either epithelioid or spindled, and occur either as nests of cells or as single file rows of cells. The differential diagnosis includes histiocytic proliferations such as xanthogranulomatous inflammation and fat necrosis, as well as other pink cell tumors—melanoma, apocrine carcinomas, and histiocytoid lobular carcinomas. Immunohistochemistry can resolve any diagnostic dilemma, as granular cell tumors are immunoreactive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin and more specific melanocytic markers (HMB45, Melan A). The majority of granular cell tumors are benign and are adequately treated with complete excision.
References:
1. Adeniran A, Al-Ahmadie H, Mahoney MC, Robinson-Smith TM. Granular cell tumor of the breast: a series of 17 cases and review of the literature. The breast journal. 2004;10(6):528-31Brown AC, Audisio RA, Regitnig P. Granular cell tumour of the breast. Surgical oncology. 2011;20(2):97-105.
2. De Simone N, Aggon A, Christy C. Granular cell tumor of the breast: clinical and pathologic characteristics of a rare case in a 14-year-old girl. J Clin Oncol. 2011;29(22):e656-7.
3. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779-94.
4. Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110 patients. Journal of surgical oncology. 1980;13(4):301-16.
5. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Archives Pathol Lab Med. 2004;128(7):771-5.
Incorrect
Diagnosis: A. Granular cell tumor
Histology: The resection specimen reveals an infiltrative process comprised of epithelioid cells with round nuclei, centrally located and prominent nucleoli, and abundant eosinophilic granular cytoplasm. The cells are infiltrating around and through benign ducts and lobules. There is no evidence of an atypical intraductal proliferation. There is no appreciable mitotic activity within the cells. Immunostains show the cells to be diffusely immunoreactive for S100 and inhibin, and to be negative for AE1/AE3 and Cam5.2.
Discussion: The morphologic features are classic for a granular cell tumor, which a type of peripheral nerve sheath tumor that can rarely involve the breast. Granular cell tumors characteristically involve the head and neck, where they can incite a prominent pseudoepitheliomatous hyperplasia in overlying squamous epithelium. In the breast, granular cell tumors clinical, radiographically and grossly mimic infiltrating carcinoma because they can appear as a fixed, stellate and infiltrative solid mass. Histologically, granular cell tumors display abundant eosinophilic, granular cytoplasm and can be either epithelioid or spindled, and occur either as nests of cells or as single file rows of cells. The differential diagnosis includes histiocytic proliferations such as xanthogranulomatous inflammation and fat necrosis, as well as other pink cell tumors—melanoma, apocrine carcinomas, and histiocytoid lobular carcinomas. Immunohistochemistry can resolve any diagnostic dilemma, as granular cell tumors are immunoreactive for inhibin, CD68, S100 protein, and NSE, and are negative for cytokeratin and more specific melanocytic markers (HMB45, Melan A). The majority of granular cell tumors are benign and are adequately treated with complete excision.
References:
1. Adeniran A, Al-Ahmadie H, Mahoney MC, Robinson-Smith TM. Granular cell tumor of the breast: a series of 17 cases and review of the literature. The breast journal. 2004;10(6):528-31Brown AC, Audisio RA, Regitnig P. Granular cell tumour of the breast. Surgical oncology. 2011;20(2):97-105.
2. De Simone N, Aggon A, Christy C. Granular cell tumor of the breast: clinical and pathologic characteristics of a rare case in a 14-year-old girl. J Clin Oncol. 2011;29(22):e656-7.
3. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol. 1998;22(7):779-94.
4. Lack EE, Worsham GF, Callihan MD, Crawford BE, Klappenbach S, Rowden G, et al. Granular cell tumor: a clinicopathologic study of 110 patients. Journal of surgical oncology. 1980;13(4):301-16.
5. Le BH, Boyer PJ, Lewis JE, Kapadia SB. Granular cell tumor: immunohistochemical assessment of inhibin-alpha, protein gene product 9.5, S100 protein, CD68, and Ki-67 proliferative index with clinical correlation. Archives Pathol Lab Med. 2004;128(7):771-5.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
Clinical history: 75 y/o Male with a breast mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
Clinical history: 75 y/o Male with a breast mass
Correct
Diagnosis: D. Malignant phyllodes tumor
Histology: The resection specimen contains a fibroepithelial lesion that displays prominent leaf-like
architecture, marked stromal cell cytologic atypia, and a brisk mitotic rate (>10 mitoses per 10 high power fields) including numerous atypical mitotic figures. There is no stromal overgrowth in this section, nor elsewhere in the specimen. The tumor displays predominantly circumscribed borders in this section, but in other areas has a nodular infiltrative growth. The degree of stromal cellularity is relatively uniform on this particular section, but in other sections displays variation in the stromal cellularity with regions of marked hypercellularity. Despite the lack of stromal overgrowth, the constellation of features is in keeping with a malignant phyllodes tumor.
Discussion: Fibroepithelial lesions of the breast consist of fibroadenomas and phyllodes tumors. Fibroadenomas are vastly more common and are entirely benign. Phyllodes tumors are fibroepithelial lesions of the breast that display stromal hypercellularity and a prominent leaf-like architecture, often with cystic areas. In fact, their original name was “cystosarcoma phyllodes,” referring to the gross appearance: cystic (“cysto-“) and fleshy (“-sarcoma”), with projections resembling leaf fronds (“phyllodes” comes from the Greek “phyllon”, or leaf). Phyllodes tumors are subclassified into benign, borderline and malignant on the basis of a constellation of histologic features. When evaluating a fibroepithelial lesion, it’s helpful to first scan all slides of the lesions at low power to get an overall sense of the lesion, and then to evaluate these histologic features from low power to high power. In general, the severity of the atypia increases across the diagnostic categories of benign, borderline and malignant. The histologic features include: the degree of circumscription (circumscribed vs. infiltrative), the degree of stromal cellularity (mild to marked hypercellularity), any variation in stromal cellularity and the presence of stromal overgrowth (defined as one 4x lower power field entirely comprised of stroma), the degree of stromal atypia (mild to marked), the mitotic rate, and the presence of malignant heterologous elements (limited to malignant phyllodes). The constellation of features here is most in keeping with a malignant phyllodes tumor.
References:
1. Tan BY, Acs G, Apple SK, et al. Phyllodes tumours of the breast: a consensus review. Histopathology. 2016 Jan;68(1):5-21.
Incorrect
Diagnosis: D. Malignant phyllodes tumor
Histology: The resection specimen contains a fibroepithelial lesion that displays prominent leaf-like
architecture, marked stromal cell cytologic atypia, and a brisk mitotic rate (>10 mitoses per 10 high power fields) including numerous atypical mitotic figures. There is no stromal overgrowth in this section, nor elsewhere in the specimen. The tumor displays predominantly circumscribed borders in this section, but in other areas has a nodular infiltrative growth. The degree of stromal cellularity is relatively uniform on this particular section, but in other sections displays variation in the stromal cellularity with regions of marked hypercellularity. Despite the lack of stromal overgrowth, the constellation of features is in keeping with a malignant phyllodes tumor.
Discussion: Fibroepithelial lesions of the breast consist of fibroadenomas and phyllodes tumors. Fibroadenomas are vastly more common and are entirely benign. Phyllodes tumors are fibroepithelial lesions of the breast that display stromal hypercellularity and a prominent leaf-like architecture, often with cystic areas. In fact, their original name was “cystosarcoma phyllodes,” referring to the gross appearance: cystic (“cysto-“) and fleshy (“-sarcoma”), with projections resembling leaf fronds (“phyllodes” comes from the Greek “phyllon”, or leaf). Phyllodes tumors are subclassified into benign, borderline and malignant on the basis of a constellation of histologic features. When evaluating a fibroepithelial lesion, it’s helpful to first scan all slides of the lesions at low power to get an overall sense of the lesion, and then to evaluate these histologic features from low power to high power. In general, the severity of the atypia increases across the diagnostic categories of benign, borderline and malignant. The histologic features include: the degree of circumscription (circumscribed vs. infiltrative), the degree of stromal cellularity (mild to marked hypercellularity), any variation in stromal cellularity and the presence of stromal overgrowth (defined as one 4x lower power field entirely comprised of stroma), the degree of stromal atypia (mild to marked), the mitotic rate, and the presence of malignant heterologous elements (limited to malignant phyllodes). The constellation of features here is most in keeping with a malignant phyllodes tumor.
References:
1. Tan BY, Acs G, Apple SK, et al. Phyllodes tumours of the breast: a consensus review. Histopathology. 2016 Jan;68(1):5-21.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
Clinical history: 50 y/o Female with a breast mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
Clinical history: 50 y/o Female with a breast mass
Correct
Diagnosis: E. Paget’s disease of the nipple
Histology: This slide contains a section of the nipple, characterized by overlying squamous epithelium (epidermis), smooth muscle bundles in the dermis/subcutaneous tissue, and large lactiferous ducts. Located within the epidermis, and primarily above the basal cell layer, are large atypical cells with pagetoid spread through the full thickness of the epidermis. The cells are characterized by abundant pale cytoplasm and large nuclei with prominent nucleoli. There is no obvious intracellular melanin pigment. An underlying lactiferous duct is involved by a solid proliferation of atypical cells characteristic of high grade ductal carcinoma in situ.
Discussion: This is an example of Paget’s disease of the nipple, with carcinoma cells involving the epidermis in a pagetoid spread (i.e., spread throughout in a scattered appearance). Paget’s disease of the nipple typically presents with clinical ulceration or erosion of the nipple skin and is most often associated with an underlying ductal carcinoma in situ, although occasionally no in situ or invasion lesion can be identified in the underlying breast parenchyma. Like extramammary Paget’s disease that occurs in the perineum or abdomen, a section of Paget’s disease of the nipple must be evaluated for any evidence of dermal invasion by the Paget’s cells (i.e., “invasive Paget’s disease”). The differential diagnosis for atypical pagetoid cells in the epidermis includes melanoma in situ, Bowen’s disease (squamous cell carcinoma in situ), and mammary or extramammary Paget’s disease. The differential diagnosis in the nipple also includes Toker cell hyperplasia, which are benign cells within the nipple epidermis. Immunohistochemistry can be useful in this differential. Melanoma in situ labels for melanocytic markers such as S100, SOX10, HMB45, MelanA, and MITF and is negative for cytokeratin. Bowen’s disease (squamous cell carcinoma in situ) labels for high molecular weight cytokeratin. Paget’s disease of the nipple labels for CK7, HER2 and AR (androgen receptor) and is typically negative for ER. Extramammary Paget’s disease also shows HER2 and AR labeling, although to a lesser degree. Another histologic clue in the differential diagnosis is that the Paget’s disease cells are typically located above the basal cell layer, whereas the melanoma in situ nests are often below the basal cell layer. Resections for Paget’s disease must be marginated, that is, the skin margins must be evaluated to ensure complete excision of the Paget’s disease.
References:
1. Reed W, Oppedal BR, Eeg Larsen T. Immunohistology is valuable in distinguishing between Paget’s disease, Bowen’s disease and superficial spreading malignant melanoma. Histopathology. 1990 Jun;16(6):583-8.
2. Liegl B, Horn LC, Moinfar F. Androgen receptors are frequently expressed in mammary and extramammary Paget’s disease. Mod Pathol. 2005 Oct;18(10):1283-8.
Incorrect
Diagnosis: E. Paget’s disease of the nipple
Histology: This slide contains a section of the nipple, characterized by overlying squamous epithelium (epidermis), smooth muscle bundles in the dermis/subcutaneous tissue, and large lactiferous ducts. Located within the epidermis, and primarily above the basal cell layer, are large atypical cells with pagetoid spread through the full thickness of the epidermis. The cells are characterized by abundant pale cytoplasm and large nuclei with prominent nucleoli. There is no obvious intracellular melanin pigment. An underlying lactiferous duct is involved by a solid proliferation of atypical cells characteristic of high grade ductal carcinoma in situ.
Discussion: This is an example of Paget’s disease of the nipple, with carcinoma cells involving the epidermis in a pagetoid spread (i.e., spread throughout in a scattered appearance). Paget’s disease of the nipple typically presents with clinical ulceration or erosion of the nipple skin and is most often associated with an underlying ductal carcinoma in situ, although occasionally no in situ or invasion lesion can be identified in the underlying breast parenchyma. Like extramammary Paget’s disease that occurs in the perineum or abdomen, a section of Paget’s disease of the nipple must be evaluated for any evidence of dermal invasion by the Paget’s cells (i.e., “invasive Paget’s disease”). The differential diagnosis for atypical pagetoid cells in the epidermis includes melanoma in situ, Bowen’s disease (squamous cell carcinoma in situ), and mammary or extramammary Paget’s disease. The differential diagnosis in the nipple also includes Toker cell hyperplasia, which are benign cells within the nipple epidermis. Immunohistochemistry can be useful in this differential. Melanoma in situ labels for melanocytic markers such as S100, SOX10, HMB45, MelanA, and MITF and is negative for cytokeratin. Bowen’s disease (squamous cell carcinoma in situ) labels for high molecular weight cytokeratin. Paget’s disease of the nipple labels for CK7, HER2 and AR (androgen receptor) and is typically negative for ER. Extramammary Paget’s disease also shows HER2 and AR labeling, although to a lesser degree. Another histologic clue in the differential diagnosis is that the Paget’s disease cells are typically located above the basal cell layer, whereas the melanoma in situ nests are often below the basal cell layer. Resections for Paget’s disease must be marginated, that is, the skin margins must be evaluated to ensure complete excision of the Paget’s disease.
References:
1. Reed W, Oppedal BR, Eeg Larsen T. Immunohistology is valuable in distinguishing between Paget’s disease, Bowen’s disease and superficial spreading malignant melanoma. Histopathology. 1990 Jun;16(6):583-8.
2. Liegl B, Horn LC, Moinfar F. Androgen receptors are frequently expressed in mammary and extramammary Paget’s disease. Mod Pathol. 2005 Oct;18(10):1283-8.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is 5 month old male with a renal mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
This is 5 month old male with a renal mass
Correct
Answer: C. Clear cell sarcoma of the kidney
Histology: This is a fairly uniform neoplasm composed of spindle to epithelioid cells with inconspicuous cytoplasm and prominent clear extracellular matrix material, simulating the appearance of clear cytoplasm. Cells are separated by regular branching capillary vasculature. At the edge of the lesion, the neoplasm entraps single nephrons. The cytology of the nuclei is bland, with open chromatin associated nuclear grooves. These are the typical features of clear cell sarcoma of the kidney. The patient age in this case is more typical of congenital mesoblastic nephroma, but CCSK can occur in this age group.
Discussion: Congenital mesoblastic nephroma is divided into classic (fibromatous like) or cellular (fibrosarcoma like) types. Aside from differences in cytology, classic CMN has a widely infiltrative broader, whereas cellular CMN is typically associated with high mitotic activity and necrosis. Rhabdoid tumor of the kidney typically features a characteristic cytologic triad; vesicular chromatin, prominent nucleus, and hyaline cytoplasmic inclusions. Rhabdoid tumor of the kidney demonstrates loss if INI1 protein.
Clear cell sarcoma of the kidney is characterized by internal tandem duplication of the BCOR gene in the majority of cases. Approximately 5-10% of CCSKs harbor the recurring chromosome translocation t(10;17) (q22;p13), resulting YWHAE-FAM22 gene fusion. The same gene fusion is also found in a subset of high grade endometrial stromal sarcomas.
Incorrect
Answer: C. Clear cell sarcoma of the kidney
Histology: This is a fairly uniform neoplasm composed of spindle to epithelioid cells with inconspicuous cytoplasm and prominent clear extracellular matrix material, simulating the appearance of clear cytoplasm. Cells are separated by regular branching capillary vasculature. At the edge of the lesion, the neoplasm entraps single nephrons. The cytology of the nuclei is bland, with open chromatin associated nuclear grooves. These are the typical features of clear cell sarcoma of the kidney. The patient age in this case is more typical of congenital mesoblastic nephroma, but CCSK can occur in this age group.
Discussion: Congenital mesoblastic nephroma is divided into classic (fibromatous like) or cellular (fibrosarcoma like) types. Aside from differences in cytology, classic CMN has a widely infiltrative broader, whereas cellular CMN is typically associated with high mitotic activity and necrosis. Rhabdoid tumor of the kidney typically features a characteristic cytologic triad; vesicular chromatin, prominent nucleus, and hyaline cytoplasmic inclusions. Rhabdoid tumor of the kidney demonstrates loss if INI1 protein.
Clear cell sarcoma of the kidney is characterized by internal tandem duplication of the BCOR gene in the majority of cases. Approximately 5-10% of CCSKs harbor the recurring chromosome translocation t(10;17) (q22;p13), resulting YWHAE-FAM22 gene fusion. The same gene fusion is also found in a subset of high grade endometrial stromal sarcomas.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is a 55 year old female with a gastric mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 55 year old female with a gastric mass
Correct
Answer: A. Schwannoma
Histology: This is a bland but cellular spindle cell neoplasm centered in the gastric wall. There is an associated lymphoid cuff at the edge of the lesion. The neoplastic cells demonstrate angulated, tapering nuclei with palisading which can be seen in either GIST or schwannoma. In this case, the neoplasm was negative for CD117 and diffusely immunoreactive for S100 protein, supporting the diagnosis of schwannoma. The prominent lymphoid cuff surrounding gastric schwannomas is a clue to the diagnosis and helps distinguish them from GIST
Discussion: GIST would label for CD117 and not diffusely for S100 protein. Leiomyosarcomas and leiomyomas would demonstrate for immunoreactivity for desmin, and would feature more plump spindle cells with abundant eosinophilic cytoplasm and cigar shaped nuclei.
Incorrect
Answer: A. Schwannoma
Histology: This is a bland but cellular spindle cell neoplasm centered in the gastric wall. There is an associated lymphoid cuff at the edge of the lesion. The neoplastic cells demonstrate angulated, tapering nuclei with palisading which can be seen in either GIST or schwannoma. In this case, the neoplasm was negative for CD117 and diffusely immunoreactive for S100 protein, supporting the diagnosis of schwannoma. The prominent lymphoid cuff surrounding gastric schwannomas is a clue to the diagnosis and helps distinguish them from GIST
Discussion: GIST would label for CD117 and not diffusely for S100 protein. Leiomyosarcomas and leiomyomas would demonstrate for immunoreactivity for desmin, and would feature more plump spindle cells with abundant eosinophilic cytoplasm and cigar shaped nuclei.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
Case 1: This is a 59 year old female with a large renal mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
Case 1: This is a 59 year old female with a large renal mass.
Correct
Answer: D. Clear cell carcinoma
Histology: The majority of this tumor has the appearance of a high grade renal cell carcinoma with prominent eosinophilic cytoplasm. This is a highly non-specific appearance, and may be seen in the high grade portions of a wide variety of primary renal tumors. The key in such cases is not so much a broad immunohistochemical panel (which is expensive) but rather thorough sampling of the neoplasm. Additional sections from this case revealed a lower grade area which is typical of clear cell renal cell carcinoma, in that it has a thin capillary vasculature and epithelioid cells with water clear cytoplasm. This area demonstrated diffuse immunoreactivity for carbonic anhydrase 9 (CA-IX), supporting the diagnosis of clear cell carcinoma. Hence, this case is a high grade clear cell RCC.
Discussion: As mentioned above, a large number of renal tumors can have a high grade eosinophilic appearance, and when such nested tumors degenerate the pseudopapillae mimic the true papillae of papillary renal cell carcinoma. The diagnosis of “type II papillary renal cell carcinoma” is always somewhat suspect, as many tumors can acquire this appearance in their high grade areas. Collecting duct carcinoma should be centered on the renal medulla, and demonstrate a high grade infiltrating adenocarcinoma appearance with prominent desmoplastic, inflamed stroma. Renal medullary carcinomas have a more extensive rhabdoid appearance, are associated with INI1-loss, and typically occur in young patients with sickle cell trait.
Incorrect
Answer: D. Clear cell carcinoma
Histology: The majority of this tumor has the appearance of a high grade renal cell carcinoma with prominent eosinophilic cytoplasm. This is a highly non-specific appearance, and may be seen in the high grade portions of a wide variety of primary renal tumors. The key in such cases is not so much a broad immunohistochemical panel (which is expensive) but rather thorough sampling of the neoplasm. Additional sections from this case revealed a lower grade area which is typical of clear cell renal cell carcinoma, in that it has a thin capillary vasculature and epithelioid cells with water clear cytoplasm. This area demonstrated diffuse immunoreactivity for carbonic anhydrase 9 (CA-IX), supporting the diagnosis of clear cell carcinoma. Hence, this case is a high grade clear cell RCC.
Discussion: As mentioned above, a large number of renal tumors can have a high grade eosinophilic appearance, and when such nested tumors degenerate the pseudopapillae mimic the true papillae of papillary renal cell carcinoma. The diagnosis of “type II papillary renal cell carcinoma” is always somewhat suspect, as many tumors can acquire this appearance in their high grade areas. Collecting duct carcinoma should be centered on the renal medulla, and demonstrate a high grade infiltrating adenocarcinoma appearance with prominent desmoplastic, inflamed stroma. Renal medullary carcinomas have a more extensive rhabdoid appearance, are associated with INI1-loss, and typically occur in young patients with sickle cell trait.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
80 year-old male presents with a spine bone mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
80 year-old male presents with a spine bone mass
Correct
Diagnosis: B. Metastatic hepatocellular carcinoma
Histology: The biopsy reveals a lesion comprised of polygonal eosinophilic cells with round nuclei and prominent, cherry-red central nucleoli. Some cells contain distinct cytoplasmic inclusions reminiscent of intracellular hyaline bodies. In areas, the cells are arranged in “plates” of 3-4 cells thick. There are scattered mitotic figures. There is no appreciable cytoplasmic pigment suggestive of melanoma or bile. Immunostains show the lesional cells to be positive for HepAR1 and arginase, with canalicular pattern labeling for CD10; the cells are negative for PAX8, inhibin and S100.
Discussion:
The histology and immunophenotype of this lesion are characteristic of metastatic hepatocellular carcinoma (HCC). At the time of presentation with back pain and bony lesions, the patient did not have a known history of HCC. Although the diagnosis for this lesion may be suspected on the H&E alone, but immunostains are necessary to confirm the diagnosis in the absence of a known clinical history. Upon further clinical investigation, the patient was found to have large hepatic masses. The differential diagnosis of the bony lesions on the basis of the morphology alone is of a “pink cell tumor.” The differential diagnosis of malignant “pink cell tumors” must always include melanoma, melanoma, melanoma (which can have variable morphology and cytology), followed by adrenocortical carcinoma, hepatocellular carcinoma, and renal cell carcinoma, as well as rarer tumors such as alveolar soft parts sarcoma, clear cell sarcoma, oncocytic neoplasms of the thyroid (Hurthle cell) and parathyroid, and granular cell tumors. HCCs may also display a variety of architectural patterns such as trabecular/plate-like (as in this case) or pseudoglandular/acinar, as well a variety of cytologic patterns such as the presence of pleomorphic cells, clear cells, spindled cells, cytoplasmic bile pigment, cytoplasmic hyaline bodies, or ground glass inclusions. By immunohistochemistry, HCCs typically label positively for HepAR1 (~90%), arginase, glypican 3, and canalicular pattern polylonal CEA and CD10.
References:
1. Thiese ND, et al. “Hepatocellular carcinoma.” In: WHO Classification of Tumors of the Digestive System. Ed. Bosman FT et al. 2010. pg 205-216.
Incorrect
Diagnosis: B. Metastatic hepatocellular carcinoma
Histology: The biopsy reveals a lesion comprised of polygonal eosinophilic cells with round nuclei and prominent, cherry-red central nucleoli. Some cells contain distinct cytoplasmic inclusions reminiscent of intracellular hyaline bodies. In areas, the cells are arranged in “plates” of 3-4 cells thick. There are scattered mitotic figures. There is no appreciable cytoplasmic pigment suggestive of melanoma or bile. Immunostains show the lesional cells to be positive for HepAR1 and arginase, with canalicular pattern labeling for CD10; the cells are negative for PAX8, inhibin and S100.
Discussion:
The histology and immunophenotype of this lesion are characteristic of metastatic hepatocellular carcinoma (HCC). At the time of presentation with back pain and bony lesions, the patient did not have a known history of HCC. Although the diagnosis for this lesion may be suspected on the H&E alone, but immunostains are necessary to confirm the diagnosis in the absence of a known clinical history. Upon further clinical investigation, the patient was found to have large hepatic masses. The differential diagnosis of the bony lesions on the basis of the morphology alone is of a “pink cell tumor.” The differential diagnosis of malignant “pink cell tumors” must always include melanoma, melanoma, melanoma (which can have variable morphology and cytology), followed by adrenocortical carcinoma, hepatocellular carcinoma, and renal cell carcinoma, as well as rarer tumors such as alveolar soft parts sarcoma, clear cell sarcoma, oncocytic neoplasms of the thyroid (Hurthle cell) and parathyroid, and granular cell tumors. HCCs may also display a variety of architectural patterns such as trabecular/plate-like (as in this case) or pseudoglandular/acinar, as well a variety of cytologic patterns such as the presence of pleomorphic cells, clear cells, spindled cells, cytoplasmic bile pigment, cytoplasmic hyaline bodies, or ground glass inclusions. By immunohistochemistry, HCCs typically label positively for HepAR1 (~90%), arginase, glypican 3, and canalicular pattern polylonal CEA and CD10.
References:
1. Thiese ND, et al. “Hepatocellular carcinoma.” In: WHO Classification of Tumors of the Digestive System. Ed. Bosman FT et al. 2010. pg 205-216.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
A 30 year-old male presents with a rib mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
A 30 year-old male presents with a rib mass
Correct
Diagnosis: C. Langerhans cell histiocytosis
Histology: Portions of the excisional biopsy show fragments of native bone. The lesion consists of sheets of loosely cohesive cells with hypochromatic nuclei with irregular contours, distinctive nuclear grooves, and occasional “kidney bean” clefted morphology. The cytoplasm is pale. Mitotic figures are readily apparent. The background is notable for a prominent mixed inflammatory infiltrate with an abundance of eosinophils. Immunostains show the lesional cells to be positive for CD1a and S100
Discussion: The histologic features are characteristic of Langerhans cell histiocytosis, which is a neoplastic proliferation of Langerhans cells with an associated prominent inflammatory background often containing abundant eosinophils. In fact, in the lung, Langerhans histiocytosis is sometimes called “eosinophilic granuloma” (technically a misnomer!). In addition to the lung, Langerhans cell histiocytosis can involve a variety of organs, including the bone as seen in this case, the skin, and lymph nodes. Most patients present with involvement of only one organ, and the bone is the most common site of involvement. The skull is the most common site of involvement in children. Langerhans cells are a type of dendritic cell normally located in the epidermis and mucosal surfaces. Histologically, Langerhans cells display a classic “kidney-bean shaped” nuclei with prominent central grooves, irregular nuclear contours and discohesion. The cytoplasm is typically pale or indistinct and contains the classic “tennis-racket shaped” Birbeck granules on electron microscopy. Unlike other dendritic cell subtypes, Langerhans cells label positively for CD1a, which is a useful diagnostic tool.
References:
1. Allen CE, Li L, Peters TL, Leung HC, Yu A, Man TK, Gurusiddappa S, Phillips MT, Hicks MJ, Gaikwad A, Merad M, McClain KL. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 2010 Apr 15;184(8):4557-67.
Incorrect
Diagnosis: C. Langerhans cell histiocytosis
Histology: Portions of the excisional biopsy show fragments of native bone. The lesion consists of sheets of loosely cohesive cells with hypochromatic nuclei with irregular contours, distinctive nuclear grooves, and occasional “kidney bean” clefted morphology. The cytoplasm is pale. Mitotic figures are readily apparent. The background is notable for a prominent mixed inflammatory infiltrate with an abundance of eosinophils. Immunostains show the lesional cells to be positive for CD1a and S100
Discussion: The histologic features are characteristic of Langerhans cell histiocytosis, which is a neoplastic proliferation of Langerhans cells with an associated prominent inflammatory background often containing abundant eosinophils. In fact, in the lung, Langerhans histiocytosis is sometimes called “eosinophilic granuloma” (technically a misnomer!). In addition to the lung, Langerhans cell histiocytosis can involve a variety of organs, including the bone as seen in this case, the skin, and lymph nodes. Most patients present with involvement of only one organ, and the bone is the most common site of involvement. The skull is the most common site of involvement in children. Langerhans cells are a type of dendritic cell normally located in the epidermis and mucosal surfaces. Histologically, Langerhans cells display a classic “kidney-bean shaped” nuclei with prominent central grooves, irregular nuclear contours and discohesion. The cytoplasm is typically pale or indistinct and contains the classic “tennis-racket shaped” Birbeck granules on electron microscopy. Unlike other dendritic cell subtypes, Langerhans cells label positively for CD1a, which is a useful diagnostic tool.
References:
1. Allen CE, Li L, Peters TL, Leung HC, Yu A, Man TK, Gurusiddappa S, Phillips MT, Hicks MJ, Gaikwad A, Merad M, McClain KL. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 2010 Apr 15;184(8):4557-67.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. Tricia Cottrell
Case 1: 60 year-old male with a brain mass
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
Clinical history: 60 year-old male with a brain mass
Correct
Diagnosis: A. Hemangioblastoma with partial cellular pattern
Histology: A portion of the tissue contains cerebellar parenchyma, suggesting that this lesion is located in the posterior fossa. Half of the lesion consists of packets of small cells with clear to eosinophilic and granular cytoplasm, separated by delicate capillary vasculature. The other half of the lesion shows larger nests of cells with fibrillary to granular cytoplasm. There is minimal atypia, no appreciable mitotic activity, and no necrosis. Immunostains show the entirety of this lesion to be positive for inhibin.
Discussion: This is a hemangioblastoma in the brain, which can be sporadic but is also part of the von Hippel-Lindau disease spectrum caused by an autosomal dominant mutation in the VHL gene on chromosome 3. The lesions seen in this syndrome include hemangioblastomas, retinal angiomas, pancreatic and hepatic cysts, pheochromocytomas, and clear cell renal cell carcinomas. This particular hemangioblastoma is unique because in addition to the common “reticular” pattern of growth, it also demonstrates a partial cellular pattern, which is the area of larger expanded cellular nests. If this cellular region is sampled on frozen section, the lesion may be mistakenly classified as a low grade glioma. In one published series of hemangioblastomas, the cellular variant of hemangioblastoma accounted for ~10% of cases and had a higher proliferative rate and greater rate of recurrence. The differential diagnosis of hemangioblastoma includes other clear cell tumors, notably metastatic clear cell renal cell carcinoma, primary brain clear cell meningiomas, and metastatic neuroendocrine tumors with clear cytoplasm (malignant paragangliomas, clear cell neuroendocrine tumors of the pancreas, parathyroid carcinomas, etc). The immunophenotype of hemangioblastomas is unique; in contrast to the other lesions in the differential diagnosis, the neoplastic cells of hemangioblastoma label positivity for inhibin.
References:
1. Hoang MP1, Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol. 2003 Aug;27(8):1152-6.
2. Hasselblatt M, Jeibmann A, Gerss J, Behrens C, Rama B, Wassmann H, Paulus W. Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. Neuropathol Appl Neurobiol. 2005 Dec;31(6):618-22.
3. Rickert CH, Hasselblatt M, Jeibmann A, Paulus W. Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Hum Pathol. 2006 Nov;37(11):1452-7.
Incorrect
Diagnosis: A. Hemangioblastoma with partial cellular pattern
Histology: A portion of the tissue contains cerebellar parenchyma, suggesting that this lesion is located in the posterior fossa. Half of the lesion consists of packets of small cells with clear to eosinophilic and granular cytoplasm, separated by delicate capillary vasculature. The other half of the lesion shows larger nests of cells with fibrillary to granular cytoplasm. There is minimal atypia, no appreciable mitotic activity, and no necrosis. Immunostains show the entirety of this lesion to be positive for inhibin.
Discussion: This is a hemangioblastoma in the brain, which can be sporadic but is also part of the von Hippel-Lindau disease spectrum caused by an autosomal dominant mutation in the VHL gene on chromosome 3. The lesions seen in this syndrome include hemangioblastomas, retinal angiomas, pancreatic and hepatic cysts, pheochromocytomas, and clear cell renal cell carcinomas. This particular hemangioblastoma is unique because in addition to the common “reticular” pattern of growth, it also demonstrates a partial cellular pattern, which is the area of larger expanded cellular nests. If this cellular region is sampled on frozen section, the lesion may be mistakenly classified as a low grade glioma. In one published series of hemangioblastomas, the cellular variant of hemangioblastoma accounted for ~10% of cases and had a higher proliferative rate and greater rate of recurrence. The differential diagnosis of hemangioblastoma includes other clear cell tumors, notably metastatic clear cell renal cell carcinoma, primary brain clear cell meningiomas, and metastatic neuroendocrine tumors with clear cytoplasm (malignant paragangliomas, clear cell neuroendocrine tumors of the pancreas, parathyroid carcinomas, etc). The immunophenotype of hemangioblastomas is unique; in contrast to the other lesions in the differential diagnosis, the neoplastic cells of hemangioblastoma label positivity for inhibin.
References:
1. Hoang MP1, Amirkhan RH. Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol. 2003 Aug;27(8):1152-6.
2. Hasselblatt M, Jeibmann A, Gerss J, Behrens C, Rama B, Wassmann H, Paulus W. Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. Neuropathol Appl Neurobiol. 2005 Dec;31(6):618-22.
3. Rickert CH, Hasselblatt M, Jeibmann A, Paulus W. Cellular and reticular variants of hemangioblastoma differ in their cytogenetic profiles. Hum Pathol. 2006 Nov;37(11):1452-7.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is a 64 year old male with a paraspinal mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 64 year old male with a paraspinal mass.
Correct
Answer: C
Histology: This is a highly cellular spindle cell neoplasm. These spindle cells form short fascicles with pink cytoplasm, and they have angulated nuclei. Focally there is mitotic activity as well as cellular necrosis. However, while these features suggest a malignancy, several features suggests that this represents cellular schwannoma. First, the vessels within the lesion have thick walls and are hyalinized. Second, there is admixed chronic inflammation, and focal foam cells. Third, while the lesion is highly cellular, there is minimal cytologic atypia, and mitoses while present are not brisk. Fourth, the tumor cell nuclei are angulated which suggests schwann cells. Fifth, the lesion appears encapsulated, unlike a typical sarcoma. The lesion is diffusely immunoreactive for S100 protein, which supports the diagnosis of cellular schwannoma.
Discussion: A malignant peripheral nerve sheath tumor would not demonstrate the characteristic vasculature of cellular schwannoma, and would not typically be diffusely immunoreactive for S100 protein. Leiomyosarcoma would have more rectangular appearing nuclei, and more abundant pink cytoplasm. Leiomyosarcoma would label for muscular markers such as desmin and actin, but not for S100 diffusely. Gastrointestinal stromal tumor may occur outside of the tubular GI tract, but the paraspinal area would be an unusual location. These lesions would not be diffusely immunoreactive for S100, but rather would typically label for CD 117.
Cellular schwannoma is a well-recognized mimic of sarcoma, and should be considered before rendering a diagnosis of sarcoma in the paraspinal region.
Incorrect
Answer: C
Histology: This is a highly cellular spindle cell neoplasm. These spindle cells form short fascicles with pink cytoplasm, and they have angulated nuclei. Focally there is mitotic activity as well as cellular necrosis. However, while these features suggest a malignancy, several features suggests that this represents cellular schwannoma. First, the vessels within the lesion have thick walls and are hyalinized. Second, there is admixed chronic inflammation, and focal foam cells. Third, while the lesion is highly cellular, there is minimal cytologic atypia, and mitoses while present are not brisk. Fourth, the tumor cell nuclei are angulated which suggests schwann cells. Fifth, the lesion appears encapsulated, unlike a typical sarcoma. The lesion is diffusely immunoreactive for S100 protein, which supports the diagnosis of cellular schwannoma.
Discussion: A malignant peripheral nerve sheath tumor would not demonstrate the characteristic vasculature of cellular schwannoma, and would not typically be diffusely immunoreactive for S100 protein. Leiomyosarcoma would have more rectangular appearing nuclei, and more abundant pink cytoplasm. Leiomyosarcoma would label for muscular markers such as desmin and actin, but not for S100 diffusely. Gastrointestinal stromal tumor may occur outside of the tubular GI tract, but the paraspinal area would be an unusual location. These lesions would not be diffusely immunoreactive for S100, but rather would typically label for CD 117.
Cellular schwannoma is a well-recognized mimic of sarcoma, and should be considered before rendering a diagnosis of sarcoma in the paraspinal region.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is a 3 week old female with two renal tumors, 5 and 3cm each.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 3 week old female with two renal tumors, 5 and 3cm each.
Correct
Answer: B
Histology: This is active proliferation of primitive nephroblastic elements, including abundant blastemal cells, primitive epithelial tubules and glomeruli. The lesion has an unencapsulated border and directly abuts the benign renal tubules. Thin fibrous septa separate lobules of the lesion. These are the typical features of hyperplastic perilobar nephrogenic rest.
Discussion: A favorable histology Wilms tumor is distinguished from hyperplastic nephrogenic rest by the presence of a fibrous capsule and its spherical shape (as opposed to the unencapsulated ovoid nature of hyperplastic rests). Anaplastic Wilms tumor would demonstrate hyperchromatic enlarged nuclei with atypical mitotic figures, and would be extremely unlikely at this young age. Neuroblastoma would not demonstrate the primitive tubules and glomeruli seen in the current lesion.
Incorrect
Answer: B
Histology: This is active proliferation of primitive nephroblastic elements, including abundant blastemal cells, primitive epithelial tubules and glomeruli. The lesion has an unencapsulated border and directly abuts the benign renal tubules. Thin fibrous septa separate lobules of the lesion. These are the typical features of hyperplastic perilobar nephrogenic rest.
Discussion: A favorable histology Wilms tumor is distinguished from hyperplastic nephrogenic rest by the presence of a fibrous capsule and its spherical shape (as opposed to the unencapsulated ovoid nature of hyperplastic rests). Anaplastic Wilms tumor would demonstrate hyperchromatic enlarged nuclei with atypical mitotic figures, and would be extremely unlikely at this young age. Neuroblastoma would not demonstrate the primitive tubules and glomeruli seen in the current lesion.
Presented by Dr. Pedram Argani and prepared by Dr. Tricia Cottrell
This is a 42 year old female with a pericardial lesion.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 42 year old female with a pericardial lesion.
Correct
Answer: B
Histology: This specimen demonstrates remarkable lobular fat atrophy. While the cells resemble neoplastic lipoblasts, their lesion is consistent with lipoatrophy in that the lipoblasts are set in a lobular configuration, and there is no significant atypia. Scattered within the lesion are multiple enlarged stromal cells, several which represent endothelial cells. These enlarged cells demonstrate nuclear inclusions consistent with cytomegalovirus, which is confirmed by immunohistochemistry. This patient suffered from HIV infection, and the lipoatrophy was likely secondary to cachexia or medication effect, and the CMV infection secondary to the underlying immunodeficiency.
Discussion: Lipoblastoma is typically a lesion of young patients less than 3 years of age, and forms a painless superficial mass. Lipoblastoma demonstrates more primitive lipoblastic cells at the periphery with maturation towards the center. Liposarcoma is suggested by the presence of lipoblasts in this case; however, the lobular nature of the proliferation and absence of atypia argues against liposarcoma.
Incorrect
Answer: B
Histology: This specimen demonstrates remarkable lobular fat atrophy. While the cells resemble neoplastic lipoblasts, their lesion is consistent with lipoatrophy in that the lipoblasts are set in a lobular configuration, and there is no significant atypia. Scattered within the lesion are multiple enlarged stromal cells, several which represent endothelial cells. These enlarged cells demonstrate nuclear inclusions consistent with cytomegalovirus, which is confirmed by immunohistochemistry. This patient suffered from HIV infection, and the lipoatrophy was likely secondary to cachexia or medication effect, and the CMV infection secondary to the underlying immunodeficiency.
Discussion: Lipoblastoma is typically a lesion of young patients less than 3 years of age, and forms a painless superficial mass. Lipoblastoma demonstrates more primitive lipoblastic cells at the periphery with maturation towards the center. Liposarcoma is suggested by the presence of lipoblasts in this case; however, the lobular nature of the proliferation and absence of atypia argues against liposarcoma.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tricia Cottrell
Clinical history: A 54 year old man underwent a partial nephrectomy for a solid renal mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
A 54 year old man underwent a partial nephrectomy for a solid renal mass.
Correct
Answer: C
Microsopic description: Areas of the tumor are composed of just thin capillaries as can be seen in clear cell renal cell carcinoma without identifiable intervening tumor cells. Other areas show large individual cells with abundant pink cytoplasm. Nuclei are ovoid and elongated with delicate chromatic and small nucleoli.
Discussion: There are less than 10 reported cases of hemangioblastoma involving the kidney. Typically, hemangioblastoma involves the cerebellum or spinal cord. More common than in the kidney but still rare, it may also be seen in the adrenal and soft tissue. Hemangioblastomas may be associated with von Hippel Lindau (VHL) syndrome, but is more commonly sporadic. Hemangioblastoma can mimic clear cell renal cell carcinoma as both share the same delicate vascular network. However, in contrast to nests of cells with either clear or eosinophilic cytoplasm, hemangioblastoma contains scattered individual cells with abundant eosinophilic or clear foamy cytoplasm with fine vacuoles that represent lipid. Most the nuclei in hemangioblastoma are benign appearing but there may be some with mild nuclear atypia. Recognition of hemangioblastoma in the kidney requires noting that it is identical to the more common lesions seen in the CNS. Verification of the diagnosis can be made showing the tumor cells are negative for keratin and PAX8, with positivity for inhibin. These lesions are benign with their only significance being their sometimes association with VHL syndrome.
Incorrect
Answer: C
Microsopic description: Areas of the tumor are composed of just thin capillaries as can be seen in clear cell renal cell carcinoma without identifiable intervening tumor cells. Other areas show large individual cells with abundant pink cytoplasm. Nuclei are ovoid and elongated with delicate chromatic and small nucleoli.
Discussion: There are less than 10 reported cases of hemangioblastoma involving the kidney. Typically, hemangioblastoma involves the cerebellum or spinal cord. More common than in the kidney but still rare, it may also be seen in the adrenal and soft tissue. Hemangioblastomas may be associated with von Hippel Lindau (VHL) syndrome, but is more commonly sporadic. Hemangioblastoma can mimic clear cell renal cell carcinoma as both share the same delicate vascular network. However, in contrast to nests of cells with either clear or eosinophilic cytoplasm, hemangioblastoma contains scattered individual cells with abundant eosinophilic or clear foamy cytoplasm with fine vacuoles that represent lipid. Most the nuclei in hemangioblastoma are benign appearing but there may be some with mild nuclear atypia. Recognition of hemangioblastoma in the kidney requires noting that it is identical to the more common lesions seen in the CNS. Verification of the diagnosis can be made showing the tumor cells are negative for keratin and PAX8, with positivity for inhibin. These lesions are benign with their only significance being their sometimes association with VHL syndrome.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tricia Cottrell
Clinical history: A 65 year old man underwent a TUR of the bladder following an episode of hematuria.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Bladder0%
1
Answered
Review
Question 1 of 1
1. Question
A 65 year old man underwent a TUR of the bladder following an episode of hematuria.
Correct
Answer: D
Discussion: Infiltrating bladder tumors with this morphology have been variably described as all of the above choices. The unifying feature is the presence of loosely cohesive cells which lose ecadherin and infiltrate widely in the bladder in a linitis plastic fashion. Some of these tumors resemble infiltrating lobular carcinoma of the breast without plasmacytoid, rhabdoid, or signet ring cytoplasmic features. In a woman it can be hard to rule out spread from a breast carcinoma. GATA3 is not helpful as it is positive in both breast and urothelial carcinomas. GCDFP may help in this differential. In both genders, one also has to put a caveat in the report that a gastrointestinal primary should be excluded. In the current case, the presence of overlying CIS is diagnostic of a primary urothelial carcinoma. The presence of micropapillary features in CIS has no prognostic significance, as opposed to micropapillary features in infiltrating urothelial carcinoma where it adversely affects prognosis.
Incorrect
Answer: D
Discussion: Infiltrating bladder tumors with this morphology have been variably described as all of the above choices. The unifying feature is the presence of loosely cohesive cells which lose ecadherin and infiltrate widely in the bladder in a linitis plastic fashion. Some of these tumors resemble infiltrating lobular carcinoma of the breast without plasmacytoid, rhabdoid, or signet ring cytoplasmic features. In a woman it can be hard to rule out spread from a breast carcinoma. GATA3 is not helpful as it is positive in both breast and urothelial carcinomas. GCDFP may help in this differential. In both genders, one also has to put a caveat in the report that a gastrointestinal primary should be excluded. In the current case, the presence of overlying CIS is diagnostic of a primary urothelial carcinoma. The presence of micropapillary features in CIS has no prognostic significance, as opposed to micropapillary features in infiltrating urothelial carcinoma where it adversely affects prognosis.
Presented by Dr. Jonathan Epstein and prepared by Dr. Tricia Cottrell
History: A 55 year old male underwent a TURP from lower urinary tract symptoms.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
Not categorized0%
1
Answered
Review
Question 1 of 1
1. Question
A 55 year old male underwent a TURP from lower urinary tract symptoms.
Correct
Answer: B
Histological description: Focally within the TURP specimen there is a focus of crowded dilated glands. The glands are lined by flat and tufted pseudostratified columnar epithelium with nuclei showing visible nucleoli. The cytoplasm is amphophilic.
Discussion: Ductal adenocarcinoma is defined as a variant of adenocarcinoma of the prostate lined by pseudostratified columnar epithelium as opposed to the simple cuboidal epithelium of usual (acinar) prostatic adenocarcinoma. The classic most common patterns of ductal adenocarcinoma are cribriform and papillary and are associated with an adverse prognosis, comparable to Gleason pattern 4 acinar adenocarcinoma. More recently, a new variant of ductal adenocarcinoma has been described where the glands are simple non-cribriform glands resembling high grade PIN. Features that help to distinguish PIN-like ductal adenocarcinoma from high grade PIN is that in the former many of the glands are markedly dilated and often have a flat lining. In contrast, the glands comprising high grade PIN are usually the same size as normal prostate glands. While, there may be focal flat lining to high grade PIN, tufting patterns predominate. Ultimately, the distinction is based on demonstrating a lack of basal cells in PIN-like ductal adenocarcinoma. As a few glands of high grade PIN many lack basal cells in a given plane of section, one needs numerous glands with no basal cells in order to establish a definitive diagnosis of PIN-like ductal adenocarcinoma. PIN-like ductal adenocarcinoma has a relatively favorable prognosis and is graded as Gleason score 3+3=6.
Incorrect
Answer: B
Histological description: Focally within the TURP specimen there is a focus of crowded dilated glands. The glands are lined by flat and tufted pseudostratified columnar epithelium with nuclei showing visible nucleoli. The cytoplasm is amphophilic.
Discussion: Ductal adenocarcinoma is defined as a variant of adenocarcinoma of the prostate lined by pseudostratified columnar epithelium as opposed to the simple cuboidal epithelium of usual (acinar) prostatic adenocarcinoma. The classic most common patterns of ductal adenocarcinoma are cribriform and papillary and are associated with an adverse prognosis, comparable to Gleason pattern 4 acinar adenocarcinoma. More recently, a new variant of ductal adenocarcinoma has been described where the glands are simple non-cribriform glands resembling high grade PIN. Features that help to distinguish PIN-like ductal adenocarcinoma from high grade PIN is that in the former many of the glands are markedly dilated and often have a flat lining. In contrast, the glands comprising high grade PIN are usually the same size as normal prostate glands. While, there may be focal flat lining to high grade PIN, tufting patterns predominate. Ultimately, the distinction is based on demonstrating a lack of basal cells in PIN-like ductal adenocarcinoma. As a few glands of high grade PIN many lack basal cells in a given plane of section, one needs numerous glands with no basal cells in order to establish a definitive diagnosis of PIN-like ductal adenocarcinoma. PIN-like ductal adenocarcinoma has a relatively favorable prognosis and is graded as Gleason score 3+3=6.
Please enter your email address to continue to the Johns Hopkins Surgical Pathology Case Conference website.
Why do we ask for your email? We’d like to send you periodic updates regarding Pathology educational materials released by our department. You’ll hear about new websites, iPad apps, PathCasts, and other educational materials.