Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis
A 35 year old man underwent an orchiectomy for a testicular 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
Testis0%
1
Answered
Review
Question 1 of 1
1. Question
A 35 year old man underwent an orchiectomy for a testicular mass.
Choose the correct diagnosis.
Correct
Answer: B
Histology. The testis showed a teratoma with glands, cartilage, and skeletal muscle differentiation, along with cystic spaces. The cartilage had cytological atypia and the muscle component resembled rhabdomyosarcoma. The cystic spaces were lined cells with abundant glassy dense eosinophilic cytoplasm and contained frequent cytoplasmic vacuoles.
Discussion: With the exception of rare pre-pubertal type teratomas, which are benign both histologically and in terms of behavior, all teratomas in post-pubertal males are malignant. Features important in the recognition of benign pre-pubertal type teratomas are absence of all of the following: cytologic atypia, intratubular germ cell neoplasia, significant tubular atrophy/tubular sclerosis, scarred zones, impaired spermatogenesis, microlithiasis, and evidence of chromosome 12p amplification. Other features include frequent organoid morphology and prominent components of ciliated epithelium and smooth muscle. Once there is a malignant teratoma, one does comment on immaturity or cytological atypia. The only time one would diagnose a sarcoma, carcinoma or other somatic-type malignancy is when the these elements sheet out greater than a 4x low power field. For example, focal rhabdomyosarcomatous elements, as seen in this case, is just part of a malignant teratoma. If the rhabdomyosarcomatous component sheeted out >4x field, then the diagnosis would be a somatic malignancy consisting of rhabdomyosarcoma arising in a teratoma. The other issue in this case is the cystic component. The lining cells are typical of trophoblastic cells with abundant glassy squamoid cytoplasm. Choriocarcinoma, in contrast to the current case, is composed of solid tumor typically with hemorrhagic necrosis and a biphasic component of Syncytiotrophoblastic and cytotrophoblastic cells. The cystic trophoblastic cells were positive for GATA3 and HCG. Cystic trophoblastic tumor may arise from regressing choriocarcinoma where the more aggressive cells are
eliminated either by chemotherapy or spontaneous regression. Also cystic trophoblastic tumor possible may represent an intermediate stage in the “maturation” of choriocarcinoma to teratoma. In the current case and in others published, cysts were partly lined by both trophoblasts and glands, in support of this concept. The importance of cystic trophoblastic tumor, it is totally Prognostically, cystic trophoblastic tumor lesion is similar to teratoma and no further treatment is required after surgery in the absence of other components.
Incorrect
Answer: B
Histology. The testis showed a teratoma with glands, cartilage, and skeletal muscle differentiation, along with cystic spaces. The cartilage had cytological atypia and the muscle component resembled rhabdomyosarcoma. The cystic spaces were lined cells with abundant glassy dense eosinophilic cytoplasm and contained frequent cytoplasmic vacuoles.
Discussion: With the exception of rare pre-pubertal type teratomas, which are benign both histologically and in terms of behavior, all teratomas in post-pubertal males are malignant. Features important in the recognition of benign pre-pubertal type teratomas are absence of all of the following: cytologic atypia, intratubular germ cell neoplasia, significant tubular atrophy/tubular sclerosis, scarred zones, impaired spermatogenesis, microlithiasis, and evidence of chromosome 12p amplification. Other features include frequent organoid morphology and prominent components of ciliated epithelium and smooth muscle. Once there is a malignant teratoma, one does comment on immaturity or cytological atypia. The only time one would diagnose a sarcoma, carcinoma or other somatic-type malignancy is when the these elements sheet out greater than a 4x low power field. For example, focal rhabdomyosarcomatous elements, as seen in this case, is just part of a malignant teratoma. If the rhabdomyosarcomatous component sheeted out >4x field, then the diagnosis would be a somatic malignancy consisting of rhabdomyosarcoma arising in a teratoma. The other issue in this case is the cystic component. The lining cells are typical of trophoblastic cells with abundant glassy squamoid cytoplasm. Choriocarcinoma, in contrast to the current case, is composed of solid tumor typically with hemorrhagic necrosis and a biphasic component of Syncytiotrophoblastic and cytotrophoblastic cells. The cystic trophoblastic cells were positive for GATA3 and HCG. Cystic trophoblastic tumor may arise from regressing choriocarcinoma where the more aggressive cells are
eliminated either by chemotherapy or spontaneous regression. Also cystic trophoblastic tumor possible may represent an intermediate stage in the “maturation” of choriocarcinoma to teratoma. In the current case and in others published, cysts were partly lined by both trophoblasts and glands, in support of this concept. The importance of cystic trophoblastic tumor, it is totally Prognostically, cystic trophoblastic tumor lesion is similar to teratoma and no further treatment is required after surgery in the absence of other components.
Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis
A 56 year old man underwent a transurethral resection of a bladder 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
Bladder0%
1
Answered
Review
Question 1 of 1
1. Question
A 56 year old man underwent a transurethral resection of a bladder lesion.
Choose the correct diagnosis.
Correct
Answer: A
Histology: Focally, Multiple large irregular nests are irregularly present amongst large bundles of muscularis propria without prominent desmoplasia. Cytologically, the nests are totally bland, with the cells identical to normal urothelium without appreciable mitotic activity. Focally, there is a detached fragment of non-invasive low grade papillary urothelial carcinoma.
Discussion
In contrast to invasive low grade papillary urothelial carcinoma and why nested carcinoma can be difficult to recognize as cancer is that the nested variant of urothelial carcinoma typically lacks an inflammatory or desmoplastic stromal reaction, usually does not have an overlying papillary component, lacks more irregular shaped nests, and does not consist of small clusters of cells with retraction artifact. Desmoplasia is also unusual may be present in large nested urothelial carcinomas, and is of further value in confirming invasion when present. In contrast to an inverted growth pattern of noninvasive urothelial carcinoma, the presence of urothelial nests, regardless of how bland tumor between muscularis propria is virtually diagnostic of muscle invasive carcinoma. Similarly, florid proliferation of von Brunn nests would cluster under the urothelium as regular evenly-spaced nests with an even non-infiltrative base and would never be present in muscularis propria. Except for urachal rests and the intramural segment of the ureter, benign urothelial proliferations or lesions do not involve the muscularis propria. Nested urothelial carcinomas are frequently muscle invasive and, despite their innocuous histology, are paradoxically associated with aggressive clinical outcome including metastasis and death. Even with therapy, 70% of patients with adequate follow-up die within 4 to 40 months of diagnosis. However stage for stage their prognosis is similar to invasive high grade urothelial carcinoma, with their poor prognosis reflecting typical presentation at high stage.
Incorrect
Answer: A
Histology: Focally, Multiple large irregular nests are irregularly present amongst large bundles of muscularis propria without prominent desmoplasia. Cytologically, the nests are totally bland, with the cells identical to normal urothelium without appreciable mitotic activity. Focally, there is a detached fragment of non-invasive low grade papillary urothelial carcinoma.
Discussion
In contrast to invasive low grade papillary urothelial carcinoma and why nested carcinoma can be difficult to recognize as cancer is that the nested variant of urothelial carcinoma typically lacks an inflammatory or desmoplastic stromal reaction, usually does not have an overlying papillary component, lacks more irregular shaped nests, and does not consist of small clusters of cells with retraction artifact. Desmoplasia is also unusual may be present in large nested urothelial carcinomas, and is of further value in confirming invasion when present. In contrast to an inverted growth pattern of noninvasive urothelial carcinoma, the presence of urothelial nests, regardless of how bland tumor between muscularis propria is virtually diagnostic of muscle invasive carcinoma. Similarly, florid proliferation of von Brunn nests would cluster under the urothelium as regular evenly-spaced nests with an even non-infiltrative base and would never be present in muscularis propria. Except for urachal rests and the intramural segment of the ureter, benign urothelial proliferations or lesions do not involve the muscularis propria. Nested urothelial carcinomas are frequently muscle invasive and, despite their innocuous histology, are paradoxically associated with aggressive clinical outcome including metastasis and death. Even with therapy, 70% of patients with adequate follow-up die within 4 to 40 months of diagnosis. However stage for stage their prognosis is similar to invasive high grade urothelial carcinoma, with their poor prognosis reflecting typical presentation at high stage.
Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis
A 66 year old man with BPH underwent a TURP.
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
Prostate0%
1
Answered
Review
Question 1 of 1
1. Question
A 66 year old man with BPH underwent a TURP. Choose the correct diagnosis.
Correct
Answer: C. Prostatic infarct with reactive urothelium
Histology: Multiple prostatic chips have areas at low magnification with recent hemorrhage. In some of the chips there is infarcted tissue with ghosts of tubules. Surrounding these infarcts are nests with a urothelial appearance. Cytologically, some of the urothelial-appearing cells are moderately atypical with scattered mitotic figures identified. In other chips no infarcted tissue is noted but the same nests are seen amongst recent hemorrhage. Some of the nests contain focal necrosis in the center of nests with neutrophils and acellular debris.
Discussion: Prostatic infarcts are more commonly seen on TURPs done for BPH but can occasionally be seen on needle biopsy. It can markedly elevate serum PSA levels. On TURP, the lesion one can appear lobular with zonation. In the center of the infarct there is acute coagulative necrosis and recent hemorrhage with adjacent reactive epithelial nests. Progressing away from the infarct, there is more mature squamous metaplasia with fibrosis. The zonation is not as easily identified on biopsy, and likewise the needle biopsy may not sample the central infarct but only the edge of the infarct with the reactive epithelium. If one focuses on the epithelial nests, then one will likely misdiagnose the lesion as infiltrating carcinoma. However, if one first steps back to assess the overall lesion and notes that the nests are occurring associated with recent hemorrhage within the stroma, and that this is not the overall setting for urothelial carcinoma but is typical for a prostatic infarct, then the correct diagnosis can be made. Necrosis is also not common in nests of urothelial carcinoma and when present typically lacks neutrophils and central debris, as seen in prostatic infarcts.
Incorrect
Answer: C. Prostatic infarct with reactive urothelium
Histology: Multiple prostatic chips have areas at low magnification with recent hemorrhage. In some of the chips there is infarcted tissue with ghosts of tubules. Surrounding these infarcts are nests with a urothelial appearance. Cytologically, some of the urothelial-appearing cells are moderately atypical with scattered mitotic figures identified. In other chips no infarcted tissue is noted but the same nests are seen amongst recent hemorrhage. Some of the nests contain focal necrosis in the center of nests with neutrophils and acellular debris.
Discussion: Prostatic infarcts are more commonly seen on TURPs done for BPH but can occasionally be seen on needle biopsy. It can markedly elevate serum PSA levels. On TURP, the lesion one can appear lobular with zonation. In the center of the infarct there is acute coagulative necrosis and recent hemorrhage with adjacent reactive epithelial nests. Progressing away from the infarct, there is more mature squamous metaplasia with fibrosis. The zonation is not as easily identified on biopsy, and likewise the needle biopsy may not sample the central infarct but only the edge of the infarct with the reactive epithelium. If one focuses on the epithelial nests, then one will likely misdiagnose the lesion as infiltrating carcinoma. However, if one first steps back to assess the overall lesion and notes that the nests are occurring associated with recent hemorrhage within the stroma, and that this is not the overall setting for urothelial carcinoma but is typical for a prostatic infarct, then the correct diagnosis can be made. Necrosis is also not common in nests of urothelial carcinoma and when present typically lacks neutrophils and central debris, as seen in prostatic infarcts.
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 45 year old male with bilateral renal tumors.
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
Kidney0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 45 year old male with bilateral renal tumors.
Correct
Answer: C
Histologic Description: This renal neoplasm is difficult to classify. Some nests have round nuclei which suggest oncocytoma. However, these merge with other nests which are larger and have more irregular nuclei. These are gradual transitions throughout this neoplasm. Given the unusual morphologic features and the bilateral natures of the tumors, additional clinical history was obtained which revealed a history of Birt-Hogg Dube syndrome.
Differential Diagnosis: Oncocytoma would have nested pattern, adenomatous stroma, and oncocytic cells with perfectly round nuclei thoughout. Chromphobe renal cell carcinoma typically has solid architecture, hyalinized stroma, and irregular nuclei. Eosinophilic solid and cystic renal cell carcinoma is characterized by cells with volluminous eosinophilic cytoplasm and basophilic cytoplasmic stippling, and typically demonstrates focal immunoreactivity for cytokeratin 20.
Birt-Hogg-Dube syndrome is associated with mutations in folliculin gene, and patients typically have cutaneous fibrofolliculomas and may have a history of pulmonary pneumothorax. These hybrid oncocytic tumors are typically indolent neoplasms and do not metastasize.
Incorrect
Answer: C
Histologic Description: This renal neoplasm is difficult to classify. Some nests have round nuclei which suggest oncocytoma. However, these merge with other nests which are larger and have more irregular nuclei. These are gradual transitions throughout this neoplasm. Given the unusual morphologic features and the bilateral natures of the tumors, additional clinical history was obtained which revealed a history of Birt-Hogg Dube syndrome.
Differential Diagnosis: Oncocytoma would have nested pattern, adenomatous stroma, and oncocytic cells with perfectly round nuclei thoughout. Chromphobe renal cell carcinoma typically has solid architecture, hyalinized stroma, and irregular nuclei. Eosinophilic solid and cystic renal cell carcinoma is characterized by cells with volluminous eosinophilic cytoplasm and basophilic cytoplasmic stippling, and typically demonstrates focal immunoreactivity for cytokeratin 20.
Birt-Hogg-Dube syndrome is associated with mutations in folliculin gene, and patients typically have cutaneous fibrofolliculomas and may have a history of pulmonary pneumothorax. These hybrid oncocytic tumors are typically indolent neoplasms and do not metastasize.
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 90 year old 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
breast0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 90 year old female with a breast mass.
Correct
Answer: B
Histologic Description: This lesion is composed of solid nests of neoplastic epithelial cells. Cells are relatively low nuclear grade. Some of the solid nests have fibrovascular cores in their center, indicating a papillary neoplasm. At the edge of the lesion, the nests of epithelial cells merge with the fat in a non-lobular pattern, which suggesst invasive carcinoma arising in a papillary DCIS. This type of invasive carcinoma typically occurs in older patients, and often is not associated with desmoplastic stromal reaction. Immunostains for myoepithelial markers confirm that the lesion is diffusely positive for estrogen receptor, and that the invasive component lacks myoepithelial markers.
Differential Diagnosis: Papillary ductal carcinoma in situ would not be associated with the irregular nests that permeate the stroma in this case, and would have intact myoepithelium. Ductal carcinoma in situ or lobular carcinoma in situ involving adenosis typically shows rounded nests at its periphery, which is a clue that there is not invasion. In contrast, the nests at the periphery of this lesion are more infiltrative and irregular. Adenosis would demonstrate intact myoepithelium throughout, unlike the current case. Lobular carcinoma in situ would be more discohesive than the current lesion.
Incorrect
Answer: B
Histologic Description: This lesion is composed of solid nests of neoplastic epithelial cells. Cells are relatively low nuclear grade. Some of the solid nests have fibrovascular cores in their center, indicating a papillary neoplasm. At the edge of the lesion, the nests of epithelial cells merge with the fat in a non-lobular pattern, which suggesst invasive carcinoma arising in a papillary DCIS. This type of invasive carcinoma typically occurs in older patients, and often is not associated with desmoplastic stromal reaction. Immunostains for myoepithelial markers confirm that the lesion is diffusely positive for estrogen receptor, and that the invasive component lacks myoepithelial markers.
Differential Diagnosis: Papillary ductal carcinoma in situ would not be associated with the irregular nests that permeate the stroma in this case, and would have intact myoepithelium. Ductal carcinoma in situ or lobular carcinoma in situ involving adenosis typically shows rounded nests at its periphery, which is a clue that there is not invasion. In contrast, the nests at the periphery of this lesion are more infiltrative and irregular. Adenosis would demonstrate intact myoepithelium throughout, unlike the current case. Lobular carcinoma in situ would be more discohesive than the current lesion.
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 4 year old boy with a renal tumor.
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
Kidney0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 4 year old boy with a renal tumor.
Correct
Answer: Wilms tumor with intralobar rest
Histologic Description: This is a blastemal rich Wilms tumor, composed of primitive cells with high nuclei cytoplasm ratio and abundant mitotic activity. Focally, tubular formations and stromal areas are present. At the edge of the tumor, where it interfaces with the native kidney, one can appreciate less cellular zones which have fat as a stromal component. These areas intermingle with native nephrons. This is characteristic of intralobar nephrogenic rest.
Differential Diagnosis: Perilobar nephrogenic rest are typically at the periphery of the lobe (most often subcapsular), and typically composed of primitive blastemal cells and tubules. Desmoplastic round cell tumor may label for WT1 and desmin like Wilms tumor; however, it would feature nests of primitive cells in desmoplastic stroma, and would not demonstrate tubule formations as seen in the current case.
Incorrect
Answer: Wilms tumor with intralobar rest
Histologic Description: This is a blastemal rich Wilms tumor, composed of primitive cells with high nuclei cytoplasm ratio and abundant mitotic activity. Focally, tubular formations and stromal areas are present. At the edge of the tumor, where it interfaces with the native kidney, one can appreciate less cellular zones which have fat as a stromal component. These areas intermingle with native nephrons. This is characteristic of intralobar nephrogenic rest.
Differential Diagnosis: Perilobar nephrogenic rest are typically at the periphery of the lobe (most often subcapsular), and typically composed of primitive blastemal cells and tubules. Desmoplastic round cell tumor may label for WT1 and desmin like Wilms tumor; however, it would feature nests of primitive cells in desmoplastic stroma, and would not demonstrate tubule formations as seen in the current case.
Histology: This tumor is composed of nest of tumor cells with abundant eosinophilic cytoplasm and round monomorphic nuclei.
Discussion:
Unclassified oncocytic renal cell carcinoma are tumors with predominantly solid growth that do fit diagnostic criteria for either oncocytoma or chromophobe renal cell carcinoma. They are most commonly localized to the kidney and have a relatively good prognosis. Some cases show nuclei with prominent nucleoli visible at 10x magnification but a nuclear grade should not be assigned. The diagnosis of unclassified RCC can be made once other eosinophilic tumors have been excluded, mainly eosinophilic solid and cystic RCC which is positive for CK20, and SDH or FH deficient RCC.
References:
– Trpkov K, Hes O, Bonert M, et al. Eosinophilic, Solid, and Cystic Renal Cell Carcinoma: Clinicopathologic Study of 16 Unique, Sporadic Neoplasms Occurring in Women. Am J Surg Pathol. 2016;40:60-71.
– Smith SC, Sirohi D, Ohe C, et al. A distinctive, low-grade oncocytic fumarate hydratase-deficient renal cell carcinoma, morphologically reminiscent of succinate dehydrogenase-deficient renal cell carcinoma. Histopathology. 2017;71:42-52.
– Li Y, Reuter VE, Matoso A, et al. Re-evaluation of 33 ‘unclassified’ eosinophilic renal cell carcinomas in young patients. Histopathology. 2018;72:588-600.
Histology: This tumor is composed of nest of tumor cells with abundant eosinophilic cytoplasm and round monomorphic nuclei.
Discussion:
Unclassified oncocytic renal cell carcinoma are tumors with predominantly solid growth that do fit diagnostic criteria for either oncocytoma or chromophobe renal cell carcinoma. They are most commonly localized to the kidney and have a relatively good prognosis. Some cases show nuclei with prominent nucleoli visible at 10x magnification but a nuclear grade should not be assigned. The diagnosis of unclassified RCC can be made once other eosinophilic tumors have been excluded, mainly eosinophilic solid and cystic RCC which is positive for CK20, and SDH or FH deficient RCC.
References:
– Trpkov K, Hes O, Bonert M, et al. Eosinophilic, Solid, and Cystic Renal Cell Carcinoma: Clinicopathologic Study of 16 Unique, Sporadic Neoplasms Occurring in Women. Am J Surg Pathol. 2016;40:60-71.
– Smith SC, Sirohi D, Ohe C, et al. A distinctive, low-grade oncocytic fumarate hydratase-deficient renal cell carcinoma, morphologically reminiscent of succinate dehydrogenase-deficient renal cell carcinoma. Histopathology. 2017;71:42-52.
– Li Y, Reuter VE, Matoso A, et al. Re-evaluation of 33 ‘unclassified’ eosinophilic renal cell carcinomas in young patients. Histopathology. 2018;72:588-600.
Presented by Dr. Andres Matoso and prepared by Dr. Katelynn Davis
Adult female in her 40s with 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
Kidney0%
1
Answered
Review
Question 1 of 1
1. Question
Adult female in her 40s with renal mass
Correct
Correct: Angiomyolipoma
Histology: Section shows a neoplasm composed of spindle cells, adipose tissue, and vessels with thick wall.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epitheliod AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.
Incorrect
Correct: Angiomyolipoma
Histology: Section shows a neoplasm composed of spindle cells, adipose tissue, and vessels with thick wall.
Discussion: Renal angiomyolipomas (AMLs) are tumors formed of variable amounts of three components: adipose tissue, tortuous thick-walled vessels and cells with smooth muscle differentiation. There is evidence that the AMLs belong to the family of perivascular epithelioid cell tumors (PEComas) It can be associated with tuberous sclerosis but they are most frequently sporadic. Sporadic AMLs are 4 times more frequent in women. The clinical presentation is similar to other renal masses: incidental finding, abdominal pain and/or hematuria. A minority of patients may present with rupture and intrabdominal bleeding. Grossly, AMLs are typically well-circumscribed, sometimes with infiltrating edges. It may involve the perirenal soft tissue and even lymph nodes but these features are not associated with malignancy. Histologically, AMLs show vessels with thick wall and spindle cells radiating off the wall. The spindle cells have an appearance similar to smooth muscle cells with blunt end elongated nuclei and eosinophilic cytoplasm. Variants of AMLs include fat poor, epithelioid and epitheliod AML with atypia, and angiomyolipoma with epithelial cysts. Fat poor-AMLs are more common in needle biopsy specimens because they are difficult to distinguish from renal cell carcinoma on image studies and, therefore, undergo more frequent biopsy. The epithelioid variant is characterized by round cells with variable amount of eosinophilic or clear cytoplasm and sometimes bizarre nuclear atypia (atypical epithelioid AMLs). Rarely, epithelioid AMLs can have a malignant behavior. Malignancy in AMLs has been associated with the presence of at least 3 of the following findings: 1) >70% atypical epithelioid cells, 2) 2 or more mitotic figures per 10 HPFs, 3) atypical mitotic figures, 4) necrosis. By immunohistochemistry, AMLs are positive for melanocytic markers HMB45, Melan-A and tyrosinase. These are often focally positive and therefore a panel of markers is recommended. Other positive markers include cathepsin-K, and smooth muscle actin. PAX-8 and keratin stains are frequently negative and might be helpful in the distinction of epithelioid AMLs and renal cell carcinoma
References:
1. Eble JN. Angiomyolipoma of kidney. Semin Diagn Pathol. 1998;15(1):21-40.
2. Lin C, Jin L, Yang Y, Ding Y, Wu X, Ni L, et al. Tuberous sclerosis-associated renal angiomyolipoma: A report of two cases and review of the literature. Mol Clin Oncol. 2017;7(4):706-8.
3. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, Kim H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011;35(2):161-76.
4. Brimo F, Robinson B, Guo C, Zhou M, Latour M, Epstein JI. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol. 2010;34(5):715-22.
5. Fine SW, Reuter VE, Epstein JI, Argani P. Angiomyolipoma with epithelial cysts (AMLEC): a distinct cystic variant of angiomyolipoma. Am J Surg Pathol. 2006;30(5):593-9.
6. Park BK. Renal Angiomyolipoma: Radiologic Classification and Imaging Features According to the Amount of Fat. AJR Am J Roentgenol. 2017;209(4):826-35.
Presented by Dr. Andres Matoso and prepared by Dr. Katelynn Davis
Adult with an appendiceal 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
Appendix0%
1
Answered
Review
Question 1 of 1
1. Question
Adult with an appendiceal mass
Correct
Correct: Sessile serrated adenoma
Histology: The appendix is dilated with mucin and there is mucin extravasation in the peri appendiceal soft tissue. The mucosal lining shows a serrated polyp without dysplasia. Some of the glands show dilatation at the base.
Discussion: Not every mucinous distension of the appendix is low grade mucinous neoplasm. In order for it to be a low-grade mucinous neoplasm, the epithelium must show low grade dysplasia. Mucin extravasation without epithelium does not have much clinical significance although sampling is important to completely exclude a LAMN. Once there is disseminated peritoneal disease, the grade of the epithelium is important for the diagnosis: low grade epithelium and mucin is called “disseminated peritoneal adenomucinosis-DPAM; high grade epithelium and mucin is “peritoneal mucinous carcinomatosis-PMCA.”
References:
Ballentine, S.J., Carr, J., Bekhor, E.Y. et al. Updated staging and patient outcomes in low-grade appendiceal mucinous neoplasms. Mod Pathol (2020). https://doi.org/10.1038/s41379-020-0628-7
Figueroa-Rivera IM, Santiago-Rivera L, Magno P. Sessile Serrated Adenoma of the Appendix in an Asymptomatic Patient. Clin Gastroenterol Hepatol. 2018;16(4):A27. doi:10.1016/j.cgh.2017.07.014
Incorrect
Correct: Sessile serrated adenoma
Histology: The appendix is dilated with mucin and there is mucin extravasation in the peri appendiceal soft tissue. The mucosal lining shows a serrated polyp without dysplasia. Some of the glands show dilatation at the base.
Discussion: Not every mucinous distension of the appendix is low grade mucinous neoplasm. In order for it to be a low-grade mucinous neoplasm, the epithelium must show low grade dysplasia. Mucin extravasation without epithelium does not have much clinical significance although sampling is important to completely exclude a LAMN. Once there is disseminated peritoneal disease, the grade of the epithelium is important for the diagnosis: low grade epithelium and mucin is called “disseminated peritoneal adenomucinosis-DPAM; high grade epithelium and mucin is “peritoneal mucinous carcinomatosis-PMCA.”
References:
Ballentine, S.J., Carr, J., Bekhor, E.Y. et al. Updated staging and patient outcomes in low-grade appendiceal mucinous neoplasms. Mod Pathol (2020). https://doi.org/10.1038/s41379-020-0628-7
Figueroa-Rivera IM, Santiago-Rivera L, Magno P. Sessile Serrated Adenoma of the Appendix in an Asymptomatic Patient. Clin Gastroenterol Hepatol. 2018;16(4):A27. doi:10.1016/j.cgh.2017.07.014
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 52 year old male with thigh 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
soft tissue0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 52 year old male with thigh mass.
Correct
Answer: Extraskeletal myxoid chondrosarcoma
Histologic Description: The neoplasm is composed of cords of plump cells with finely vacuolated cytoplasm floating in a myxochondroid matrix in a lace like pattern. The neoplasm was associated with the gene fusion of the EWSR1 and NR4A3 gene.
Differential Diagnosis: Metastatic mucinous adenocarcinoma would be diffusely immunoreactive for cytokeratin, and patients should have a history of such a neoplasm. Myxoid liposarcoma would feature branching papillary vasculature, vacuolated lipoblastic cells, and the characteristic FUS-DDIT3 gene fusion resulting from the t(12;16) translocation. Chordomas are typically midline neoplasms, associated with immunoreactivity for brachyury.
Incorrect
Answer: Extraskeletal myxoid chondrosarcoma
Histologic Description: The neoplasm is composed of cords of plump cells with finely vacuolated cytoplasm floating in a myxochondroid matrix in a lace like pattern. The neoplasm was associated with the gene fusion of the EWSR1 and NR4A3 gene.
Differential Diagnosis: Metastatic mucinous adenocarcinoma would be diffusely immunoreactive for cytokeratin, and patients should have a history of such a neoplasm. Myxoid liposarcoma would feature branching papillary vasculature, vacuolated lipoblastic cells, and the characteristic FUS-DDIT3 gene fusion resulting from the t(12;16) translocation. Chordomas are typically midline neoplasms, associated with immunoreactivity for brachyury.
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 2 year old male with a renal tumor.
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
Kidney0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 2 year old male with a renal tumor.
Correct
Answer: Clear cell sarcoma of the kidney
Histologic Description: This is a neoplasm composed of undifferentiated epithelioid cells separated by a regular capillary branching vasculature. The neoplastic cells have effaced chromatin, without prominent nucleoli. The cytoplasm is eosinophilic and scant, and the cells are separated by mucopolysaccharides given a “clear cell” appearance. The neoplastic cells do not label for cytokeratin, but instead demonstrate diffuse nuclear immunoreactivity for BCOR protein, supporting the diagnosis of clear cell sarcoma of the kidney.
Differential Diagnosis: Rhabdoid of the kidney would feature cells with vesicular chromatin, prominent nucleoli, and hyaline eosinophilic cytoplasmic inclusions, and would demonstrate loss of INI1 protein. Blastemal Wilms tumor typically has coarser, hyperchromatic chromatin, and is often associated with foci of true epithelial or stromal differentiation not seen in CCSK. Cellular congenital mesoblastic nephroma may have a round cell appearance; however, the chromatin is typically coarser, and the neoplastic cells are associated with ETV6-NTRK3 gene fusions and not BCOR immunoreactivity.
Clear cell sarcoma of the kidneys are typically characterized by internal tandem duplications of the BCOR gene, resulting in protein overexpression. A subset harbors the YWHAE-NUTM2B gene fusion also seen in a subset of cellular endometrial stromal sarcomas, and these also demonstrate BCOR immunoreactivity. Finally, a subset of renal neoplasms which overlap CCSK but tend to be spindled and occur in slightly older children are associated with the BCOR-CCNB3 gene fusion.
Incorrect
Answer: Clear cell sarcoma of the kidney
Histologic Description: This is a neoplasm composed of undifferentiated epithelioid cells separated by a regular capillary branching vasculature. The neoplastic cells have effaced chromatin, without prominent nucleoli. The cytoplasm is eosinophilic and scant, and the cells are separated by mucopolysaccharides given a “clear cell” appearance. The neoplastic cells do not label for cytokeratin, but instead demonstrate diffuse nuclear immunoreactivity for BCOR protein, supporting the diagnosis of clear cell sarcoma of the kidney.
Differential Diagnosis: Rhabdoid of the kidney would feature cells with vesicular chromatin, prominent nucleoli, and hyaline eosinophilic cytoplasmic inclusions, and would demonstrate loss of INI1 protein. Blastemal Wilms tumor typically has coarser, hyperchromatic chromatin, and is often associated with foci of true epithelial or stromal differentiation not seen in CCSK. Cellular congenital mesoblastic nephroma may have a round cell appearance; however, the chromatin is typically coarser, and the neoplastic cells are associated with ETV6-NTRK3 gene fusions and not BCOR immunoreactivity.
Clear cell sarcoma of the kidneys are typically characterized by internal tandem duplications of the BCOR gene, resulting in protein overexpression. A subset harbors the YWHAE-NUTM2B gene fusion also seen in a subset of cellular endometrial stromal sarcomas, and these also demonstrate BCOR immunoreactivity. Finally, a subset of renal neoplasms which overlap CCSK but tend to be spindled and occur in slightly older children are associated with the BCOR-CCNB3 gene fusion.
Presented by Dr. Pedram Argani and prepared by Dr. Katelynn Davis
This is a 15 year old male with mediastinal 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
Mediastinum0%
1
Answered
Review
Question 1 of 1
1. Question
This is a 15 year old male with mediastinal mass
Correct
Answer: NUT midline carcinoma
Histologic Description: This is a small round cell tumor. The neoplastic cells have coarse chromatin, thick nuclear membranes, and are associated with necrosis. The neoplastic cells demonstrated focal labeling for cytokeratin and p63, and diffuse nuclear labeling for NUT protein, supporting the diagnosis.
Differential Diagnosis: Ewing sarcomas would have finer chromatin, and would label for CD99. Lymphoblastic lymphomas would have finely dispersed chromatin along with angulated nuclei, and would label for markers such as CD43 and TdT. HPV-related squamous cell carcinomas would typically arise in the oropharynx, and would demonstrate high risk HPV by in situ hybridization. NUT midline carcinomas are typically undifferentiated small round cell tumors harboring NUT gene fusion that may show up focal abrupt squamous differentiation. There prognosis is poor, with the mean survival less than one year.
Incorrect
Answer: NUT midline carcinoma
Histologic Description: This is a small round cell tumor. The neoplastic cells have coarse chromatin, thick nuclear membranes, and are associated with necrosis. The neoplastic cells demonstrated focal labeling for cytokeratin and p63, and diffuse nuclear labeling for NUT protein, supporting the diagnosis.
Differential Diagnosis: Ewing sarcomas would have finer chromatin, and would label for CD99. Lymphoblastic lymphomas would have finely dispersed chromatin along with angulated nuclei, and would label for markers such as CD43 and TdT. HPV-related squamous cell carcinomas would typically arise in the oropharynx, and would demonstrate high risk HPV by in situ hybridization. NUT midline carcinomas are typically undifferentiated small round cell tumors harboring NUT gene fusion that may show up focal abrupt squamous differentiation. There prognosis is poor, with the mean survival less than one year.
Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis.
A 40 year-old man with a partial excision of a scrotal mass. The lesion was positive for S100 and Melan A and negative for HMB45.
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 40 year-old man with a partial excision of a scrotal mass. The lesion was positive for S100 and Melan A and negative for HMB45.
Correct
Answer: Granular cell tumor
Histological Description: The dermis is replaced by sheets of bland cells with abundant eosinophilic granular cytoplasm and round/oval nuclei with small nucleoli. Focally at one edge there is a squamous proliferation with irregular nests of squamous epithelium extending superficially into the dermis. The overlying skin shows focal ulceration.
Discussion: The findings in this case are classic for a granular cell tumor with pseudoepitheliomatous hyperplasia, mimicking invasive squamous cell carcinoma. An unusual feature in this case was positivity for Melan A. Typically granular cell tumors are positive for S100 protein and negative for HMB45 and Melan A. However, approximately 5% of granular cell tumors are negative for Melan A.
Incorrect
Answer: Granular cell tumor
Histological Description: The dermis is replaced by sheets of bland cells with abundant eosinophilic granular cytoplasm and round/oval nuclei with small nucleoli. Focally at one edge there is a squamous proliferation with irregular nests of squamous epithelium extending superficially into the dermis. The overlying skin shows focal ulceration.
Discussion: The findings in this case are classic for a granular cell tumor with pseudoepitheliomatous hyperplasia, mimicking invasive squamous cell carcinoma. An unusual feature in this case was positivity for Melan A. Typically granular cell tumors are positive for S100 protein and negative for HMB45 and Melan A. However, approximately 5% of granular cell tumors are negative for Melan A.
Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis.
Case 2. A 35 year old man was noted to have a mass in testis and an orchiectomy was performed.
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
Testis0%
1
Answered
Review
Question 1 of 1
1. Question
A 35 year old man was noted to have a mass in testis and an orchiectomy was performed.
Correct
Answer: Scar resulting from regressed germ cell tumor
Histological Description: The testis had a fibrotic mass (2.9 cm) with ill-defined granulomatous reaction. Focally, there are seminiferous tubules surrounded by a lymphocytic infiltrate with isolated cells or a single row along a thickened basement membrane. The cells have clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli. These seminiferous tubules lack active spermatogenesis and contain mostly Sertoli cells.
The background testicle have predominantly sclerotic seminiferous tubules, with <5% of seminiferous tubules showing spermatogenesis.
Discussion: The findings in this case are classic for a regressed germ cell tumor. Whenever there is a dense scar in the testis, which has wiped out seminiferous tubules, one has to consider a regressed germ cell tumor. In contrast to trauma, there is typically not a lot of hemosiderin and the surrounding testis shows Sertoli only pattern in many tubules. If there is no germ cell neoplasia and no history of metastatic germ cell tumor, one can conclude that the findings are suspicious for a regressed germ cell tumor. However, finding germ cell neoplasia in situ in the setting of a testicular scar is diagnostic of a regressed germ cell tumor. One can see an associated granulomatous reaction to both invasive and germ cell neoplasia in situ. In this case, we verified the germ cell neoplasia in situ with positive staining for OCT3/4. Most frequently, patients present with distant metastases of germ cell tumor and are subsequently discovered to have sonographic signs of a regressed germ cell tumor. Regression does not affect prognosis with prognosis being stage dependent.
Incorrect
Answer: Scar resulting from regressed germ cell tumor
Histological Description: The testis had a fibrotic mass (2.9 cm) with ill-defined granulomatous reaction. Focally, there are seminiferous tubules surrounded by a lymphocytic infiltrate with isolated cells or a single row along a thickened basement membrane. The cells have clear cytoplasm, irregular nuclear contours, coarse chromatin, and enlarged single or multiple nucleoli. These seminiferous tubules lack active spermatogenesis and contain mostly Sertoli cells.
The background testicle have predominantly sclerotic seminiferous tubules, with <5% of seminiferous tubules showing spermatogenesis.
Discussion: The findings in this case are classic for a regressed germ cell tumor. Whenever there is a dense scar in the testis, which has wiped out seminiferous tubules, one has to consider a regressed germ cell tumor. In contrast to trauma, there is typically not a lot of hemosiderin and the surrounding testis shows Sertoli only pattern in many tubules. If there is no germ cell neoplasia and no history of metastatic germ cell tumor, one can conclude that the findings are suspicious for a regressed germ cell tumor. However, finding germ cell neoplasia in situ in the setting of a testicular scar is diagnostic of a regressed germ cell tumor. One can see an associated granulomatous reaction to both invasive and germ cell neoplasia in situ. In this case, we verified the germ cell neoplasia in situ with positive staining for OCT3/4. Most frequently, patients present with distant metastases of germ cell tumor and are subsequently discovered to have sonographic signs of a regressed germ cell tumor. Regression does not affect prognosis with prognosis being stage dependent.
Presented by Dr. Jonathan Epstein and prepared by Dr. Katelynn Davis
Case 1. A 65 year-old man underwent a left supraclavicular lymph node biopsy. There was not history or signs/symptoms suggesting a primary site.
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. A 65 year-old man underwent a left supraclavicular lymph node biopsy. There was not history or signs/symptoms suggesting a primary site.
Choose the correct diagnosis:
Correct
Answer: Metastatic Prostate Cancer
Histological Description: The tumor in the node consists of variably sized nests of tumor with central necrosis and focal cribriform formation. Cytologically, the tumors cells are relatively uniform with out marked variation in size and shape. Chromatin is delicate with prominent nucleoli. Mitotic figures are frequent. The cytoplasm is lightly eosinophilic.
Discussion: The morphology is typical of high grade prostate cancer. Prostate cancer is relatively unique, in that even for very high grade tumors, there is typically relatively little pleomorphism. Rarely, patients can present with metastatic disease in distant lymph nodes, such as the left supraclavicular neck nodes where the thoracic duct terminates. In cases presenting with left supraclavicular lymph node metastases, serum PSA values and digital rectal exam may be normal and there may not be bone metastases, such that ultimately the pathologist must establish the diagnosis. The most sensitive and specific marker for prostate is NKX3.1.
Cho KR, Epstein JI. Metastatic prostatic carcinoma to supradiaphragmatic lymph nodes. A clinicopathologic and immunohistochemical study. Am J Surg Pathol. 1987;11(6):457-63.
Incorrect
Answer: Metastatic Prostate Cancer
Histological Description: The tumor in the node consists of variably sized nests of tumor with central necrosis and focal cribriform formation. Cytologically, the tumors cells are relatively uniform with out marked variation in size and shape. Chromatin is delicate with prominent nucleoli. Mitotic figures are frequent. The cytoplasm is lightly eosinophilic.
Discussion: The morphology is typical of high grade prostate cancer. Prostate cancer is relatively unique, in that even for very high grade tumors, there is typically relatively little pleomorphism. Rarely, patients can present with metastatic disease in distant lymph nodes, such as the left supraclavicular neck nodes where the thoracic duct terminates. In cases presenting with left supraclavicular lymph node metastases, serum PSA values and digital rectal exam may be normal and there may not be bone metastases, such that ultimately the pathologist must establish the diagnosis. The most sensitive and specific marker for prostate is NKX3.1.
Cho KR, Epstein JI. Metastatic prostatic carcinoma to supradiaphragmatic lymph nodes. A clinicopathologic and immunohistochemical study. Am J Surg Pathol. 1987;11(6):457-63.
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.
Correct
