Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical History: 20 y/o F with a pancreatic mass.
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Clinical History: 20 y/o F with a pancreatic mass
Correct
Answer: C. Autoimmune pancreatitis, type 2
Histology:
This section of pancreas shows regions with normal appearing pancreatic acini and islets, and regions with fibrotic replacement of the parenchyma with residual nodules of ducts and acini. There is marked duct-centric acute inflammation with granulocytic epithelial lesions and abscess formation. There is no evidence of malignancy. There is no evidence of venulitis or prominence of plasma cells. The constellation of features is in keeping with type 2 autoimmune pancreatitis.
Discussion:
The constellation of features is in keeping with type 2 autoimmune pancreatitis. Two types of autoimmune pancreatitis have been described. Type 1 (or “lymphoplasmacytic sclerosing pancreatitis”) typically occurs in older male patients and is typically a systemic process related to IgG4 disease, with characteristic findings of obliterative phlebitis and increased numbers of IgG4+ plasma cells. In contrast, type 2 has equal gender distribution and occurs in younger adults than type 1. Type 2 (or “idiopathic duct centric pancreatitis”) is characterized by ductulocentric acute inflammation with granulocyte epithelial lesions and does not have an association with IgG4 disease. The clinical presentations of autoimmune pancreatitis can mimic those of an infiltrating carcinoma. Patients with autoimmune pancreatitis can be treated with steroids.
References:
1. Deshpande V, Gupta R, Sainani N, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011 Jan;35(1):26-35
2. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006 Dec 21;355(25):2670-6.
3. Kamisawa T, Egawa N, Nakajima H . Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811.
4. Shinagare S, Shinagare AB, Deshpande V. Autoimmune pancreatitis: a guide for the histopathologist. Semin Diagn Pathol. 2012 Nov;29(4):197-204.
5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539-51.
Incorrect
Answer: C. Autoimmune pancreatitis, type 2
Histology:
This section of pancreas shows regions with normal appearing pancreatic acini and islets, and regions with fibrotic replacement of the parenchyma with residual nodules of ducts and acini. There is marked duct-centric acute inflammation with granulocytic epithelial lesions and abscess formation. There is no evidence of malignancy. There is no evidence of venulitis or prominence of plasma cells. The constellation of features is in keeping with type 2 autoimmune pancreatitis.
Discussion:
The constellation of features is in keeping with type 2 autoimmune pancreatitis. Two types of autoimmune pancreatitis have been described. Type 1 (or “lymphoplasmacytic sclerosing pancreatitis”) typically occurs in older male patients and is typically a systemic process related to IgG4 disease, with characteristic findings of obliterative phlebitis and increased numbers of IgG4+ plasma cells. In contrast, type 2 has equal gender distribution and occurs in younger adults than type 1. Type 2 (or “idiopathic duct centric pancreatitis”) is characterized by ductulocentric acute inflammation with granulocyte epithelial lesions and does not have an association with IgG4 disease. The clinical presentations of autoimmune pancreatitis can mimic those of an infiltrating carcinoma. Patients with autoimmune pancreatitis can be treated with steroids.
References:
1. Deshpande V, Gupta R, Sainani N, et al. Subclassification of autoimmune pancreatitis: a histologic classification with clinical significance. Am J Surg Pathol. 2011 Jan;35(1):26-35
2. Finkelberg DL, Sahani D, Deshpande V, Brugge WR. Autoimmune pancreatitis. N Engl J Med. 2006 Dec 21;355(25):2670-6.
3. Kamisawa T, Egawa N, Nakajima H . Autoimmune pancreatitis is a systemic autoimmune disease. Am J Gastroenterol. 2003;98(12):2811.
4. Shinagare S, Shinagare AB, Deshpande V. Autoimmune pancreatitis: a guide for the histopathologist. Semin Diagn Pathol. 2012 Nov;29(4):197-204.
5. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012 Feb;366(6):539-51.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical History: 1 y/o M with an undescended left testis
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Clinical History: 1 y/o M with an undescended left testis
Correct
Answer: E. Vanishing testes syndrome
Histology:
The resection consists primarily of fibrovascular and adipose tissue with a focal area reminiscent of epididymis, but there are no seminiferous tubules or leydig cells. There are no germ cells. There is a region of hemosiderin laden macrophages, fibrosis, and dystrophic calcifications. These features in a cryptorchid testis are consistent with “vanishing testes syndrome” (also called “testicular regression syndrome”).
Discussion:
The three key features of vanishing testes syndrome (also termed “testicular regression syndrome”) are the presence of fibrosis, hemosiderin-laden macrophages and dystrophic calcifications. These changes occur in cryptorchid, undescended testes that undergo atrophy usually due to torsion (i.e., cut-off of the blood supply leading to infarction and atrophy). Undescended testes are resected to decrease the risk of developing subsequent germ cell neoplasia. Theoretically, the risk of developing germ cell neoplasia would be low in truly “vanished” testes, as there are no residual seminiferous tubules; in contrast, the risk of germ cell neoplasia is much higher in undescended, intra-abdominal testes.
In general, the differential diagnosis to keep in mind while evaluating a gonadal specimen from a child include: the presence of mixed germ cell types (ovotestis), the presence or absence of any seminiferous tubules, the presence of germ cell neoplasia, and the possibility of a paratesticular rhabdomyosarcoma.
References:
1. Pirgon Ö, Dündar BN. Vanishing testes: a literature review. J Clin Res Pediatr Endocrinol. 2012 Sep;4(3):116-20.
Incorrect
Answer: E. Vanishing testes syndrome
Histology:
The resection consists primarily of fibrovascular and adipose tissue with a focal area reminiscent of epididymis, but there are no seminiferous tubules or leydig cells. There are no germ cells. There is a region of hemosiderin laden macrophages, fibrosis, and dystrophic calcifications. These features in a cryptorchid testis are consistent with “vanishing testes syndrome” (also called “testicular regression syndrome”).
Discussion:
The three key features of vanishing testes syndrome (also termed “testicular regression syndrome”) are the presence of fibrosis, hemosiderin-laden macrophages and dystrophic calcifications. These changes occur in cryptorchid, undescended testes that undergo atrophy usually due to torsion (i.e., cut-off of the blood supply leading to infarction and atrophy). Undescended testes are resected to decrease the risk of developing subsequent germ cell neoplasia. Theoretically, the risk of developing germ cell neoplasia would be low in truly “vanished” testes, as there are no residual seminiferous tubules; in contrast, the risk of germ cell neoplasia is much higher in undescended, intra-abdominal testes.
In general, the differential diagnosis to keep in mind while evaluating a gonadal specimen from a child include: the presence of mixed germ cell types (ovotestis), the presence or absence of any seminiferous tubules, the presence of germ cell neoplasia, and the possibility of a paratesticular rhabdomyosarcoma.
References:
1. Pirgon Ö, Dündar BN. Vanishing testes: a literature review. J Clin Res Pediatr Endocrinol. 2012 Sep;4(3):116-20.
Presented by Dr. Ashley Cimino-Mathews and prepared by Dr. J. Judd Fite.
Clinical History: 15 y/o M with symptoms of small bowel obstruction.
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Clinical History: 15 y/o M with symptoms of small bowel obstruction.
Correct
Answer: B. Cryptosporidiosis
Histology:
From low power, the section of small intestine looks essentially normal. There is no significant villous blunting, no ulceration, no neoplasm, and no granulomas. There are scattered lymphoid aggregates in the mucosa. Higher power examination shows eosinophils in the lamina propria, however the numbers are not markedly increased. Close examination reveals small round basophilic structures within the microvilli of the enterocyotes, distributed in a patchy distribution through the specimen. A diff quick special stain highlights the organisms, consistent with Cryptosporidia.
Discussion:
Cryptosporidia is an intracellular Protozoa that infects cells of the digestive tract and respiratory tract. Infection causes a mild, self-limited diarrhea in immunocompetent individuals, but it can be severe in immunocompromised patients. It’s unclear if the infection in this patient is responsible for the patient’s symptoms. Cryptosporidia are intracellular and can be observed on H&E stains as small basophilic, round structures attached to/located within the microvilli; there may be increased numbers of eosinophils in the lamina propria, but there are no diagnostic histologic findings. Cryptosporidia and Giardia are the most common parasitic enteric pathogen in humans. In contrast to Cryptosporidia, the classic histologic features of giardia are the presence of teardrop/pear – shaped organism located in the lumen between villi. The clinical differential for this patient is Crohn’s disease, but there is no evidence of that here.
Reference:
1. Chen XM, Keithly JS, Paya CV, LaRusso NF. Cryptosporidiosis. N Engl J Med. 2002 May 30;346(22):1723-31.
Incorrect
Answer: B. Cryptosporidiosis
Histology:
From low power, the section of small intestine looks essentially normal. There is no significant villous blunting, no ulceration, no neoplasm, and no granulomas. There are scattered lymphoid aggregates in the mucosa. Higher power examination shows eosinophils in the lamina propria, however the numbers are not markedly increased. Close examination reveals small round basophilic structures within the microvilli of the enterocyotes, distributed in a patchy distribution through the specimen. A diff quick special stain highlights the organisms, consistent with Cryptosporidia.
Discussion:
Cryptosporidia is an intracellular Protozoa that infects cells of the digestive tract and respiratory tract. Infection causes a mild, self-limited diarrhea in immunocompetent individuals, but it can be severe in immunocompromised patients. It’s unclear if the infection in this patient is responsible for the patient’s symptoms. Cryptosporidia are intracellular and can be observed on H&E stains as small basophilic, round structures attached to/located within the microvilli; there may be increased numbers of eosinophils in the lamina propria, but there are no diagnostic histologic findings. Cryptosporidia and Giardia are the most common parasitic enteric pathogen in humans. In contrast to Cryptosporidia, the classic histologic features of giardia are the presence of teardrop/pear – shaped organism located in the lumen between villi. The clinical differential for this patient is Crohn’s disease, but there is no evidence of that here.
Reference:
1. Chen XM, Keithly JS, Paya CV, LaRusso NF. Cryptosporidiosis. N Engl J Med. 2002 May 30;346(22):1723-31.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite.
Case 3. A 65 year old man underwent a nephrectomy for a renal mass. Atypical mitotic figures were noted upon histological examination.
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A 65 year old man underwent a nephrectomy for a renal mass. Atypical mitotic figures were noted upon histological examination.
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Answer: C. Malignant epithelioid angiomyolipoma
Histological Description: The tumor is composed of epithelioid cells with an overall high N/C with variable amount of pale clear to eosinophilic cytoplasm. Some are pleomorphic cells with multinucleation around the perimeter of the cell, and abundant cytoplasm termed “amoeboid cells”. Areas of necrosis are present. Mitotic figures are not frequent. The lesion lacks areas of usual angiomyolipoma.
Discussion: Angiomyolipomas (AML) is more frequently sporadic but can also be seen associated with tuberous sclerosis (TS). In patients with TS, presentation is at a younger age and lesions are multifocal and bilateral. AMLs can be epithelioid with and without atypia. In cases where the cells have a more epithelial appearance as opposed to spindled, yet there is no atypia, then these lesions are entirely benign. In cases with both epithelioid morphology and atypia there is a risk of malignant behavior. In our prior study we found the following criteria to be helpful to predict malignancy in these lesions, which in one third of cases, there is recurrence and metastases. Malignant behavior correlated with presence of 3 of the 4 following features: 1) >70% atypical epithelioid areas; 2) >2 mitoses per 10 HPF; 3) atypical mitotic figures; or 4) necrosis. In the current case all of the tumor has atypical epithelioid features, along with necrosis and atypical mitotic figures so the diagnosis was malignant epithelioid AML. In order to distinguish from a high grade renal cell carcinoma immunohistochemistry can be utilized. Typically positive these AMLs are positive for HMB45 and Melan A. In some negative cases, Cathepsin K is positive. Cytokeratins and PAX8 are negative, in contrast to what is seen in renal cell carcinoma.
Incorrect
Answer: C. Malignant epithelioid angiomyolipoma
Histological Description: The tumor is composed of epithelioid cells with an overall high N/C with variable amount of pale clear to eosinophilic cytoplasm. Some are pleomorphic cells with multinucleation around the perimeter of the cell, and abundant cytoplasm termed “amoeboid cells”. Areas of necrosis are present. Mitotic figures are not frequent. The lesion lacks areas of usual angiomyolipoma.
Discussion: Angiomyolipomas (AML) is more frequently sporadic but can also be seen associated with tuberous sclerosis (TS). In patients with TS, presentation is at a younger age and lesions are multifocal and bilateral. AMLs can be epithelioid with and without atypia. In cases where the cells have a more epithelial appearance as opposed to spindled, yet there is no atypia, then these lesions are entirely benign. In cases with both epithelioid morphology and atypia there is a risk of malignant behavior. In our prior study we found the following criteria to be helpful to predict malignancy in these lesions, which in one third of cases, there is recurrence and metastases. Malignant behavior correlated with presence of 3 of the 4 following features: 1) >70% atypical epithelioid areas; 2) >2 mitoses per 10 HPF; 3) atypical mitotic figures; or 4) necrosis. In the current case all of the tumor has atypical epithelioid features, along with necrosis and atypical mitotic figures so the diagnosis was malignant epithelioid AML. In order to distinguish from a high grade renal cell carcinoma immunohistochemistry can be utilized. Typically positive these AMLs are positive for HMB45 and Melan A. In some negative cases, Cathepsin K is positive. Cytokeratins and PAX8 are negative, in contrast to what is seen in renal cell carcinoma.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite
Case 2. A 33 year old man was noted to have a renal mass with lung metastases at the time of presentation.
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Case 2. A 33 year old man was noted to have a renal mass with lung metastases at the time of presentation.
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Answer: A. Medullary renal cell carcinoma
Histology: The tumor consists of tubulopapillary structures, cords, glandular and solid sheets with extensive necrosis. It also has microcystic and reticular patterns somewhat similar testicular yolk sac tumors. Cell have a moderate amount of basophilic or eosinophilic cytoplasm. Rhabdoid morphology is focally present. Nuclei are vesicular and pleomorphic with prominent nucleoli associated with frequent mitotic activity. There is a diffuse infiltration by neutrophils within tumor epithelial sheets. Associated with the tumor is an inflamed abundant dense desmoplastic stroma. The tumor is infiltrative into adjacent renal tissue.
Discussion: The differential diagnosis of renal medullary cancer is predominantly collecting duct carcinoma as both can share similar morphology and involve the renal medulla. However, the microcystic and reticular patterns are more typical of medullary carcinoma. Most experts feel that a definitive diagnosis of medullary carcinoma can only be made in association with sickle cell trait/disease, and almost exclusively in black patients. Inactivation of tumor suppressor gene hSNF5/INI1 on long arm of chromosome 22 is frequently detected, and can be demonstrated immunohistochemically, although it can also be lost in collecting duct carcinoma. Occasionally sickled erythrocytes can be identified which if present would help to establish the diagnosis of medullary carcinoma. There are only rare reports of survival beyond 1st year post-diagnosis. The majority of patients have lymph node metastases at time of diagnosis often with metastases to liver, lung and bone.
Incorrect
Answer: A. Medullary renal cell carcinoma
Histology: The tumor consists of tubulopapillary structures, cords, glandular and solid sheets with extensive necrosis. It also has microcystic and reticular patterns somewhat similar testicular yolk sac tumors. Cell have a moderate amount of basophilic or eosinophilic cytoplasm. Rhabdoid morphology is focally present. Nuclei are vesicular and pleomorphic with prominent nucleoli associated with frequent mitotic activity. There is a diffuse infiltration by neutrophils within tumor epithelial sheets. Associated with the tumor is an inflamed abundant dense desmoplastic stroma. The tumor is infiltrative into adjacent renal tissue.
Discussion: The differential diagnosis of renal medullary cancer is predominantly collecting duct carcinoma as both can share similar morphology and involve the renal medulla. However, the microcystic and reticular patterns are more typical of medullary carcinoma. Most experts feel that a definitive diagnosis of medullary carcinoma can only be made in association with sickle cell trait/disease, and almost exclusively in black patients. Inactivation of tumor suppressor gene hSNF5/INI1 on long arm of chromosome 22 is frequently detected, and can be demonstrated immunohistochemically, although it can also be lost in collecting duct carcinoma. Occasionally sickled erythrocytes can be identified which if present would help to establish the diagnosis of medullary carcinoma. There are only rare reports of survival beyond 1st year post-diagnosis. The majority of patients have lymph node metastases at time of diagnosis often with metastases to liver, lung and bone.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite.
Case 1. A 55 year-old man presented with hematuria and underwent a TURB.
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Case 1. A 55 year-old man presented with hematuria and underwent a TURB.
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Answer: C. Lymphoepithelioma-like urothelial carcinoma
Histological description: The TUR is replaced by a dense infiltrate with a brisk benign lymphoplasmacytic infiltrate with occasional more prominent neutrophils. Interspersed within the inflammation are small nests and cords of cohesive clusters as well as individual cells containing large nuclei with multiple nucleoli, abundant eosinophilic to amphophilic cytoplasm and frequent mitoses.
Discussion: Lymphoepithelioma-like urothelial carcinoma (LELC) is a variant of infiltrating urothelial carcinoma. If the tumor is a pure LELC it may respond to chemotherapy such that cystectomy can be avoided. When mixed with conventional urothelial carcinoma, their outcome is similar to that for conventional urothelial carcinoma and depends on the stage of the associated carcinoma. If pure LELC, these tumors potentially have a better outcome when treated with chemotherapy. The major differential diagnosis is with large cell lymphoma. In some cases there is overlying CIS or papillary urothelial carcinoma or associated conventional infiltrating urothelial carcinoma which can aid in the diagnosis. In the current case, some of the LELC is composed of cohesive nests consistent with carcinoma. If these features are not present, immunohistochemistry for CK7 and pancytokeratin can help rule out lymphoma, with negative lymphoid markers in the large atypical cells. GATA3 positivity is only present in a minority of cases. In contrast to lymphoepitheliomas of the nasopharynx, LELC is negative for EBV.
Incorrect
Answer: C. Lymphoepithelioma-like urothelial carcinoma
Histological description: The TUR is replaced by a dense infiltrate with a brisk benign lymphoplasmacytic infiltrate with occasional more prominent neutrophils. Interspersed within the inflammation are small nests and cords of cohesive clusters as well as individual cells containing large nuclei with multiple nucleoli, abundant eosinophilic to amphophilic cytoplasm and frequent mitoses.
Discussion: Lymphoepithelioma-like urothelial carcinoma (LELC) is a variant of infiltrating urothelial carcinoma. If the tumor is a pure LELC it may respond to chemotherapy such that cystectomy can be avoided. When mixed with conventional urothelial carcinoma, their outcome is similar to that for conventional urothelial carcinoma and depends on the stage of the associated carcinoma. If pure LELC, these tumors potentially have a better outcome when treated with chemotherapy. The major differential diagnosis is with large cell lymphoma. In some cases there is overlying CIS or papillary urothelial carcinoma or associated conventional infiltrating urothelial carcinoma which can aid in the diagnosis. In the current case, some of the LELC is composed of cohesive nests consistent with carcinoma. If these features are not present, immunohistochemistry for CK7 and pancytokeratin can help rule out lymphoma, with negative lymphoid markers in the large atypical cells. GATA3 positivity is only present in a minority of cases. In contrast to lymphoepitheliomas of the nasopharynx, LELC is negative for EBV.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite.
Case 3. A 45 year old man presented with a scrotal mass.
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Case 3. A 45 year old man presented with a scrotal mass.
Choose the correct diagnosis:
Correct
Answer: B. Leiomyoma with degenerative atypia
Histology: The lesions consists of a relatively circumscribed nodules on the dermis of the scrotum. It has the typical architectural pattern of a smooth muscle tumor consisting of both parallel and cross sections of long fascicles of spindle cells. The nuclei are oval with more blunt ended nuclei, in contrast to the more pointed ended cork screw nuclei with neural tumors. There are scattered larger nuclei which lack mitotic activity.
Discussion: Overall, the lesion has low cellularity more typical of a leiomyoma, rather than a leiomyosarcoma. Even a low grade leiomyosarcoma of the scrotum would be more cellular. The majority of cells in the current case have uniform nuclei with open light chromatin, whereas the background nuclei of leiomyosarcoma are more hyperchromatic. The more atypical nuclei that are scattered in this case have more degenerative atypia with indistinct smudgy chromatin often containing vacuoles in the nuclei. Mitotic figures are absent. In contrast, a leiomyosarcoma would have atypia with more irregular clumped chromatin accompanied by mitotic figures. Leiomyoma with degenerative atypia, also known as symplastic leiomyoma, has been recently described in the scrotal region. Am J Surg Pathol 38: 1410-7, 2014.
Incorrect
Answer: B. Leiomyoma with degenerative atypia
Histology: The lesions consists of a relatively circumscribed nodules on the dermis of the scrotum. It has the typical architectural pattern of a smooth muscle tumor consisting of both parallel and cross sections of long fascicles of spindle cells. The nuclei are oval with more blunt ended nuclei, in contrast to the more pointed ended cork screw nuclei with neural tumors. There are scattered larger nuclei which lack mitotic activity.
Discussion: Overall, the lesion has low cellularity more typical of a leiomyoma, rather than a leiomyosarcoma. Even a low grade leiomyosarcoma of the scrotum would be more cellular. The majority of cells in the current case have uniform nuclei with open light chromatin, whereas the background nuclei of leiomyosarcoma are more hyperchromatic. The more atypical nuclei that are scattered in this case have more degenerative atypia with indistinct smudgy chromatin often containing vacuoles in the nuclei. Mitotic figures are absent. In contrast, a leiomyosarcoma would have atypia with more irregular clumped chromatin accompanied by mitotic figures. Leiomyoma with degenerative atypia, also known as symplastic leiomyoma, has been recently described in the scrotal region. Am J Surg Pathol 38: 1410-7, 2014.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite.
Case 2. A 75 year old man was noted to have a papillary lesion at the verumontanum in the prostatic urethra which was removed by transurethral resection.
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Case 2. A 75 year old man was noted to have a papillary lesion at the verumontanum in the prostatic urethra which was removed by transurethral resection.
Correct
Answer: A. Prostatic ductal adenocarcinoma
Histology: The tumor consists of both papillary fronds and cribriform structures. The cells lining the papillary fronds are pseudostratified columnar. The cribriform glands have variably the same pseudostratified columnar epithelium or more cuboidal epithelium. There is not a lot of cytological atypia, with relatively few mitotic figures.
Discussion: The clinical history and morphology is typical of prostatic ductal adenocarcinoma. Prostatic ductal adenocarcinomas can present as a papillary urethral mass with hematuria, as seen in this case, mimicking a urothelial carcinoma. Alternatively, they can manifest as usual prostate adenocarcinoma with elevated serum PSA levels and be diagnosed on needle biopsy. The hallmark of ductal adenocarcinoma is based on its cytology of tall pseudostratified columnar epithelium as opposed to the simple cuboidal epithelium of usual (acinar) prostate adenocarcinoma. The two most common patterns of prostatic ductal adenocarcinoma are papillary and cribriform. It is not uncommon for there to be mixed ductal and acinar differentiation, as seen in this case. Ductal adenocarcinomas can have a range of cytological atypia from cases with very bland nuclei to those with more prominent nucleoli. Cases with bland cytology may be confused with benign entities such as prostatic urethral polyps. However, prostatic urethral polyps are polypoid not papillary and lined by either normal urothelium or benign cuboidal prostate cells with the underlying polyp filled with crowded but totally benign prostate glands, lacking the columnar epithelium of prostatic ductal adenocarcinoma. The term “intraductal carcinoma” refers to usual (acinar) prostate adenocarcinoma growing within prostatic ducts and acini surrounded by a basal cell layer. Prostatic ductal adenocarcinoma has a different cytology then “intraductal carcinoma” and usually prostatic ductal adenocarcinoma lacks basal cells, although when it arises in large periurethral prostatic ducts it may have an intraductal component. Papillary and cribriform prostatic ductal adenocarcinoma is graded as Gleason pattern 4.
Incorrect
Answer: A. Prostatic ductal adenocarcinoma
Histology: The tumor consists of both papillary fronds and cribriform structures. The cells lining the papillary fronds are pseudostratified columnar. The cribriform glands have variably the same pseudostratified columnar epithelium or more cuboidal epithelium. There is not a lot of cytological atypia, with relatively few mitotic figures.
Discussion: The clinical history and morphology is typical of prostatic ductal adenocarcinoma. Prostatic ductal adenocarcinomas can present as a papillary urethral mass with hematuria, as seen in this case, mimicking a urothelial carcinoma. Alternatively, they can manifest as usual prostate adenocarcinoma with elevated serum PSA levels and be diagnosed on needle biopsy. The hallmark of ductal adenocarcinoma is based on its cytology of tall pseudostratified columnar epithelium as opposed to the simple cuboidal epithelium of usual (acinar) prostate adenocarcinoma. The two most common patterns of prostatic ductal adenocarcinoma are papillary and cribriform. It is not uncommon for there to be mixed ductal and acinar differentiation, as seen in this case. Ductal adenocarcinomas can have a range of cytological atypia from cases with very bland nuclei to those with more prominent nucleoli. Cases with bland cytology may be confused with benign entities such as prostatic urethral polyps. However, prostatic urethral polyps are polypoid not papillary and lined by either normal urothelium or benign cuboidal prostate cells with the underlying polyp filled with crowded but totally benign prostate glands, lacking the columnar epithelium of prostatic ductal adenocarcinoma. The term “intraductal carcinoma” refers to usual (acinar) prostate adenocarcinoma growing within prostatic ducts and acini surrounded by a basal cell layer. Prostatic ductal adenocarcinoma has a different cytology then “intraductal carcinoma” and usually prostatic ductal adenocarcinoma lacks basal cells, although when it arises in large periurethral prostatic ducts it may have an intraductal component. Papillary and cribriform prostatic ductal adenocarcinoma is graded as Gleason pattern 4.
Presented by Dr. Jonathan Epstein and prepared by Dr. J. Judd Fite.
Case 1. History: A 65 year old man presented with a testicular mass.
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Case 1. History: A 65 year old man presented with a testicular mass.
Correct
Answer: D. Metastatic Carcinoma
Histology: The tumor consists of nests of cell, individual glands, and focal cribriform glandular formation with dilatation of the rete testis. The nuclei are relatively uniform without marked pleomorphism and medium centrally located nucleoli.
Discussion: The tumor does not resemble any of the typical patterns or cytology seen in germ cell tumors or sex cord stromal tumors. It is not as pleomorphic as embryonal carcinoma which also does not make well-formed glands. Yolk sac tumor can have many architectural patterns, yet none consists of small nests of cells with glands and focal cribriform formation. The glands in yolk sac tumor resemble intestinal glands often with subnuclear vacuoles. Sertoli cell tumor typically has broad bands of dense pink collagen intersecting the tumor which is absent in the current case and cribriform formation is not seen in these tumors. The morphology of the tumor in the testis is consistent with metastatic prostate cancer. Metastatic prostate cancer is one of the most common tumors to spread to the testis. In the past before chemical castration when bilateral orchiectomy was the treatment for metastatic prostate cancer, it was not uncommon to see involvement of the testis. Typically, there will be a history of prostate cancer, as was present in this case. If the history is not present, immunohistochemistry for prostate specific markers can be performed to verify the diagnosis.
Incorrect
Answer: D. Metastatic Carcinoma
Histology: The tumor consists of nests of cell, individual glands, and focal cribriform glandular formation with dilatation of the rete testis. The nuclei are relatively uniform without marked pleomorphism and medium centrally located nucleoli.
Discussion: The tumor does not resemble any of the typical patterns or cytology seen in germ cell tumors or sex cord stromal tumors. It is not as pleomorphic as embryonal carcinoma which also does not make well-formed glands. Yolk sac tumor can have many architectural patterns, yet none consists of small nests of cells with glands and focal cribriform formation. The glands in yolk sac tumor resemble intestinal glands often with subnuclear vacuoles. Sertoli cell tumor typically has broad bands of dense pink collagen intersecting the tumor which is absent in the current case and cribriform formation is not seen in these tumors. The morphology of the tumor in the testis is consistent with metastatic prostate cancer. Metastatic prostate cancer is one of the most common tumors to spread to the testis. In the past before chemical castration when bilateral orchiectomy was the treatment for metastatic prostate cancer, it was not uncommon to see involvement of the testis. Typically, there will be a history of prostate cancer, as was present in this case. If the history is not present, immunohistochemistry for prostate specific markers can be performed to verify the diagnosis.
Presented by Dr. Pedram Argani and prepared by Dr. J. Judd Fite.
This is a 46 year old female with a cystic renal tumor.
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This is a 46 year old female with a cystic renal tumor.
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Answer: A. Epithelioid angiomyolipoma with epithelial cysts
Histologic Description: This is an extremely unusual case. The lesion is extensively cystic, with the cysts lined by entrapped native tubular epithelium that labels for PAX8. The cells in between the epithelial cysts demonstrate pleomorphism, but minimal mitotic activity. They are associated with dysplastic blood vessels. Focally, beneath the epithelium, one can appreciate a more primitive appearing “bluer” component. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC). The current case is unusual in that the angiomyolipoma component is predominantly epithelioid and demonstrates cytologic atypia. Therefore, this lesion has potential for aggressive behavior, and the patient should be followed closely.
Differential Diagnosis: Renal synovial sarcoma can be extensively cystic like the current case. However, synovial sarcoma features monomorphic bland spindle cells, and would not label for HMB45 like the current lesion. Mixed epithelial stromal tumor could also have extensively cystic architecture like the current lesion. However, the intervening cellular stroma is predominantly composed of bland smooth muscle cells or fibrous tissue, and would not demonstrate the features of angiomyolipoma such as dysplastic vessels and melanocytic marker immunoreactivity. Tubulocystic carcinoma is an extensively cystic low grade renal carcinoma with a characteristic “bubble-wrap” appearance. This lesion is extensively cystic, the cysts are lined by oncocytic cells demonstrating nuclear irregularities, and the stroma has a fibroelastotic appearance, unlike the cellular stroma with atypia seen in the current lesion.
Angiomyolipoma with epithelial cysts contains 3 components. First there is a peripheral muscle prominent angiomyolipoma that stains strongly for muscle markers like actin and focally for HMB45. Second there is an inner cellular stroma which demonstrate stronger immunoreactivity for HMB45 and ER/PR. Finally, there is entrapped renal tubular epithelium that labels for PAX8.
Reference Fine S et a.al Am J Surg Pathol 2006; 30:593-599.
Incorrect
Answer: A. Epithelioid angiomyolipoma with epithelial cysts
Histologic Description: This is an extremely unusual case. The lesion is extensively cystic, with the cysts lined by entrapped native tubular epithelium that labels for PAX8. The cells in between the epithelial cysts demonstrate pleomorphism, but minimal mitotic activity. They are associated with dysplastic blood vessels. Focally, beneath the epithelium, one can appreciate a more primitive appearing “bluer” component. These are the typical features of angiomyolipoma with epithelial cysts (AMLEC). The current case is unusual in that the angiomyolipoma component is predominantly epithelioid and demonstrates cytologic atypia. Therefore, this lesion has potential for aggressive behavior, and the patient should be followed closely.
Differential Diagnosis: Renal synovial sarcoma can be extensively cystic like the current case. However, synovial sarcoma features monomorphic bland spindle cells, and would not label for HMB45 like the current lesion. Mixed epithelial stromal tumor could also have extensively cystic architecture like the current lesion. However, the intervening cellular stroma is predominantly composed of bland smooth muscle cells or fibrous tissue, and would not demonstrate the features of angiomyolipoma such as dysplastic vessels and melanocytic marker immunoreactivity. Tubulocystic carcinoma is an extensively cystic low grade renal carcinoma with a characteristic “bubble-wrap” appearance. This lesion is extensively cystic, the cysts are lined by oncocytic cells demonstrating nuclear irregularities, and the stroma has a fibroelastotic appearance, unlike the cellular stroma with atypia seen in the current lesion.
Angiomyolipoma with epithelial cysts contains 3 components. First there is a peripheral muscle prominent angiomyolipoma that stains strongly for muscle markers like actin and focally for HMB45. Second there is an inner cellular stroma which demonstrate stronger immunoreactivity for HMB45 and ER/PR. Finally, there is entrapped renal tubular epithelium that labels for PAX8.
Reference Fine S et a.al Am J Surg Pathol 2006; 30:593-599.
Presented by Dr. Pedram Argani and prepared by Dr. J. Judd Fite.
This is a 44 year old female with a liver tumor.
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This is a 44 year old female with a liver tumor.
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Answer: D. Mixed hepatocellular carcinoma-cholangiocarcinoma
Histologic Description: This is a fully malignant lesion that is predominantly composed of neoplastic hepatocytes. Hepatocytes show significant nuclear atypia and are arrangement in trabeculae well over 3 cells wide. These are features of a hepatocellular carcinoma. In addition, this tumor contains a mucinous glandular component which is consistent with a cholangiocarcinoma. Therefore, this is a combined hepatocellular carcinoma-cholangiocarcinoma. The diagnosis is supported by differential staining for arginase (specific to the hepatocellular component) and cytokeratin 7 (specific to the cholangiocarcinoma component).
Differential Diagnosis: Hepatocellular adenoma would lack the significant atypia and malignant cytologic and architectural features of the current case. Hepatocellular carcinoma and cholangiocarcinoma are represented within this tumor; the current tumor represents a mixture of both components.
Incorrect
Answer: D. Mixed hepatocellular carcinoma-cholangiocarcinoma
Histologic Description: This is a fully malignant lesion that is predominantly composed of neoplastic hepatocytes. Hepatocytes show significant nuclear atypia and are arrangement in trabeculae well over 3 cells wide. These are features of a hepatocellular carcinoma. In addition, this tumor contains a mucinous glandular component which is consistent with a cholangiocarcinoma. Therefore, this is a combined hepatocellular carcinoma-cholangiocarcinoma. The diagnosis is supported by differential staining for arginase (specific to the hepatocellular component) and cytokeratin 7 (specific to the cholangiocarcinoma component).
Differential Diagnosis: Hepatocellular adenoma would lack the significant atypia and malignant cytologic and architectural features of the current case. Hepatocellular carcinoma and cholangiocarcinoma are represented within this tumor; the current tumor represents a mixture of both components.
Presented by Dr. Pedram Argani and prepared by Dr. J. Judd Fite
This is a 65 year old male who undergoes a right hemicolectomy following the diagnosis of colonic adenoma on colon biopsy.
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This is a 65 year old male who undergoes a right hemicolectomy following the diagnosis of colonic adenoma on colon biopsy.
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Answer: C. Composite villous adenoma-microcarcinoid
Histologic Description: This is large high risk villous adenoma which is associated with areas of dissecting mucin that lacks epithelium, and hence does not qualify as mucinous carcinoma. The striking features of the lesion are the infiltrative nests with mixed endocrine and squamoid features which involve the lamina propria. Unlike the intestinal adenoma, these nests lack intracellular mucin, mitoses and atypia, and hence do not represent adenocarcinoma. By immunohistochemistry, they label for both endocrine markers (synaptophysin) and squamous markers (cytokeratin 5/6, p63). These are the features of a composite intestinal adenoma-microcarcinoid. These are indolent lesions.
Differential Diagnosis: Adenocarcinoma would be associated with greater atypia and intracellular mucin in the infiltrating cells. Pseudoinvasion is associated with irregular nests of intact mucosa (including lamina propria) which herniate into the submucosa, simulating invasion. Mucinous adenocarcinoma is associated with neoplastic mucinous epithelium floating in pools of dissecting mucin.
References
Lin J et al. Am J Surg Pathol 2012; 36:292-295.
Sarlin J et al Am J Surg Pathol 1984; 8:281-285.
Salaria S et al J Clin Pathol 2013; 66:302-306.
Incorrect
Answer: C. Composite villous adenoma-microcarcinoid
Histologic Description: This is large high risk villous adenoma which is associated with areas of dissecting mucin that lacks epithelium, and hence does not qualify as mucinous carcinoma. The striking features of the lesion are the infiltrative nests with mixed endocrine and squamoid features which involve the lamina propria. Unlike the intestinal adenoma, these nests lack intracellular mucin, mitoses and atypia, and hence do not represent adenocarcinoma. By immunohistochemistry, they label for both endocrine markers (synaptophysin) and squamous markers (cytokeratin 5/6, p63). These are the features of a composite intestinal adenoma-microcarcinoid. These are indolent lesions.
Differential Diagnosis: Adenocarcinoma would be associated with greater atypia and intracellular mucin in the infiltrating cells. Pseudoinvasion is associated with irregular nests of intact mucosa (including lamina propria) which herniate into the submucosa, simulating invasion. Mucinous adenocarcinoma is associated with neoplastic mucinous epithelium floating in pools of dissecting mucin.
References
Lin J et al. Am J Surg Pathol 2012; 36:292-295.
Sarlin J et al Am J Surg Pathol 1984; 8:281-285.
Salaria S et al J Clin Pathol 2013; 66:302-306.
Presented by Dr. A. Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical history: A 50 year-old male with history of low grade papillary urothelial carcinoma.
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Week 639: Case 3
Clinical history: A 50 year-old male with history of low grade papillary urothelial carcinoma
Correct
Answer: C. Nephrogenic adenoma
Histology: The bladder biopsies show partially denuded benign urothelium that where intact displays small uniform nuclei with overlying umbrella cells. The lamina propria contains chronic inflammatory cells including eosinophils. One tissue fragments contains irregular glands/tubule-like structures located in the lamina propria immediately underneath benign surface urothelium. The glands are lined by hobnail cells with bland cytology. By immunohistochemistry, the glands are positive for Pax8 and negative for GATA3.
Discussion: The bladder biopsy contains a nephrogenic adenoma, a benign proliferation which is believed to be derived from renal tubule epithelium. Evidence supporting renal derivation of nephrogenic adenoma comes from both immunohistochemistry (positivity for PAX8 and PAX2), but also based on studies of nephrogenic adenomas in patients with renal transplants. In these studies, the X-chromosome status of the nephrogenic adenoma mirrored the gender of the kidney donor and not the host patient. Nephrogenic adenomas most commonly occur in the bladder but can also be seen elsewhere in the urinary tract such as the urethra, ureter or renal pelvis, and typically occur in patients with a history of prior genitourinary instrumentation (e.g., renal transplant, genitourinary surgery). Nephrogenic adenomas can have variable histologic appearances: the spaces may appear more tubular, glandular or vascular-like; the individual cells may be cuboidal, eosinophilic, mucinous or signet-ring like. Nephrogenic adenomas are typically bland with minimal mitotic activity, but they can show degenerative-type atypia. One primary diagnostic pitfall with nephrogenic adenoma is prostatic adenocarcinoma, but they can also mimic urothelial carcinoma with glandular differentiation, vascular lesions, and clear cell adenocarcinoma. By immunohistochemistry, nephrogenic adenomas are positive for PAX8, PAX2, and AMACR/Racemase; may be positive for PSA; and are negative for vascular markers (CD34, ERG, CD31) and urothelial markers (p63, GATA3). (Notice that nephrogenic adenomas may be PSA+, Racemase+ and p63-, thus mimicking the staining pattern of prostatic adenocarcinoma on a PIN4 stain!)
References:
1. Oliva E, Young RH. Nephrogenic adenoma of the urinary tract: a review of the microscopic appearance of 80 cases with emphasis on unusual features. Mod Pathol. 1995 Sep;8(7):722-30.
2. Mazal PR, Schaufler R, Altenhuber-Müller R, et al. Derivation of nephrogenic adenomas from renal tubular cells in kidney-transplant recipients. N Engl J Med. 2002 Aug 29;347(9):653-9.
3. Netto GJ, Epstein JI. Benign Mimickers of Prostate Adenocarcinoma on Needle Biopsy and Transurethral Resection. Surg Pathol Clin. 2008 Dec;1(1):1-41.
Incorrect
Answer: C. Nephrogenic adenoma
Histology: The bladder biopsies show partially denuded benign urothelium that where intact displays small uniform nuclei with overlying umbrella cells. The lamina propria contains chronic inflammatory cells including eosinophils. One tissue fragments contains irregular glands/tubule-like structures located in the lamina propria immediately underneath benign surface urothelium. The glands are lined by hobnail cells with bland cytology. By immunohistochemistry, the glands are positive for Pax8 and negative for GATA3.
Discussion: The bladder biopsy contains a nephrogenic adenoma, a benign proliferation which is believed to be derived from renal tubule epithelium. Evidence supporting renal derivation of nephrogenic adenoma comes from both immunohistochemistry (positivity for PAX8 and PAX2), but also based on studies of nephrogenic adenomas in patients with renal transplants. In these studies, the X-chromosome status of the nephrogenic adenoma mirrored the gender of the kidney donor and not the host patient. Nephrogenic adenomas most commonly occur in the bladder but can also be seen elsewhere in the urinary tract such as the urethra, ureter or renal pelvis, and typically occur in patients with a history of prior genitourinary instrumentation (e.g., renal transplant, genitourinary surgery). Nephrogenic adenomas can have variable histologic appearances: the spaces may appear more tubular, glandular or vascular-like; the individual cells may be cuboidal, eosinophilic, mucinous or signet-ring like. Nephrogenic adenomas are typically bland with minimal mitotic activity, but they can show degenerative-type atypia. One primary diagnostic pitfall with nephrogenic adenoma is prostatic adenocarcinoma, but they can also mimic urothelial carcinoma with glandular differentiation, vascular lesions, and clear cell adenocarcinoma. By immunohistochemistry, nephrogenic adenomas are positive for PAX8, PAX2, and AMACR/Racemase; may be positive for PSA; and are negative for vascular markers (CD34, ERG, CD31) and urothelial markers (p63, GATA3). (Notice that nephrogenic adenomas may be PSA+, Racemase+ and p63-, thus mimicking the staining pattern of prostatic adenocarcinoma on a PIN4 stain!)
References:
1. Oliva E, Young RH. Nephrogenic adenoma of the urinary tract: a review of the microscopic appearance of 80 cases with emphasis on unusual features. Mod Pathol. 1995 Sep;8(7):722-30.
2. Mazal PR, Schaufler R, Altenhuber-Müller R, et al. Derivation of nephrogenic adenomas from renal tubular cells in kidney-transplant recipients. N Engl J Med. 2002 Aug 29;347(9):653-9.
3. Netto GJ, Epstein JI. Benign Mimickers of Prostate Adenocarcinoma on Needle Biopsy and Transurethral Resection. Surg Pathol Clin. 2008 Dec;1(1):1-41.
Presented by Dr. A. Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical history: A 70 year-old male with a duodenal mass
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Week 639: Case 2
Clinical history: A 70 year-old male with a duodenal mass
Correct
Answer: E. Pancreatic heterotopia with intraepithelial neoplasia
Histology: The mass is comprised of pancreatic parenchyma with all components of the normal pancreatic gland—acini, islets, and ducts. The pancreatic ducts are lined by columnar type cells with pseudostratified nuclei, hyperchromatic nuclei, and micropapillary architecture.
Discussion: This is an example of heterotopic pancreas with pancreatic intraepithelial neoplasia (PanIN). Heterotopic pancreas is a relatively uncommon lesion that can occur anywhere throughout the gastrointestinal tract; it can be detected as an incidental mass or can cause symptoms such as obstruction. Normal pancreatic ducts are lined by bland cuboidal or low-columnar cells. In contrast, ducts that contain PanIN are lined by columnar epithelial cells displaying a spectrum of nuclear atypia and micropapillary architecture. PanIN has traditionally be classified as PanIN-1A, -1B, -2 and -3 on the basis of increasing micropapillary architecture and cytologic atypia from grades 1 to 3. However, the recommended classification scheme for PanIN has recently shifted from a 3-tiered system to a 2-tiered system (low grade and high grade), wherein PanIN 1 and 2 are grouped into “low grade” and PanIN 3 is “high grade” (Basturk O, et al.). PanIN lesions are precursor lesions to pancreatic ductal adenocarcinoma, and interestingly, PanIN and even intraductal papillary mucinous neoplasms (IPMN) have been described in heterotopic pancreas, including in patients with no evidence of pancreatic adenocarcinoma in the main pancreatic gland.
References:
1. Armstrong CP, King PM, Dixon JM, and Macleod IB. The clinical significance of heterotopic pancreas in the gastrointestinal tract. Br J Surg 1981. 68:384–387
2. Zhang L, Sanderson SO, Lloyd RV, Smyrk TC. Pancreatic intraepithelial neoplasia in heterotopic pancreas: evidence for the progression model of pancreatic ductal adenocarcinoma. Am J Surg Pathol. 2007 Aug;31(8):1191-5.
3. Basturk O, Hong SM, Wood LD, et al. A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas. Am J Surg Pathol. 2015 Dec;39(12):1730-41.
4. Ma C, Gocke CD, Hruban RH, Belchis DA. Mutational spectrum of intraepithelial neoplasia in pancreatic heterotopia. Hum Pathol. 2016 Feb;48:117-21.
Incorrect
Answer: E. Pancreatic heterotopia with intraepithelial neoplasia
Histology: The mass is comprised of pancreatic parenchyma with all components of the normal pancreatic gland—acini, islets, and ducts. The pancreatic ducts are lined by columnar type cells with pseudostratified nuclei, hyperchromatic nuclei, and micropapillary architecture.
Discussion: This is an example of heterotopic pancreas with pancreatic intraepithelial neoplasia (PanIN). Heterotopic pancreas is a relatively uncommon lesion that can occur anywhere throughout the gastrointestinal tract; it can be detected as an incidental mass or can cause symptoms such as obstruction. Normal pancreatic ducts are lined by bland cuboidal or low-columnar cells. In contrast, ducts that contain PanIN are lined by columnar epithelial cells displaying a spectrum of nuclear atypia and micropapillary architecture. PanIN has traditionally be classified as PanIN-1A, -1B, -2 and -3 on the basis of increasing micropapillary architecture and cytologic atypia from grades 1 to 3. However, the recommended classification scheme for PanIN has recently shifted from a 3-tiered system to a 2-tiered system (low grade and high grade), wherein PanIN 1 and 2 are grouped into “low grade” and PanIN 3 is “high grade” (Basturk O, et al.). PanIN lesions are precursor lesions to pancreatic ductal adenocarcinoma, and interestingly, PanIN and even intraductal papillary mucinous neoplasms (IPMN) have been described in heterotopic pancreas, including in patients with no evidence of pancreatic adenocarcinoma in the main pancreatic gland.
References:
1. Armstrong CP, King PM, Dixon JM, and Macleod IB. The clinical significance of heterotopic pancreas in the gastrointestinal tract. Br J Surg 1981. 68:384–387
2. Zhang L, Sanderson SO, Lloyd RV, Smyrk TC. Pancreatic intraepithelial neoplasia in heterotopic pancreas: evidence for the progression model of pancreatic ductal adenocarcinoma. Am J Surg Pathol. 2007 Aug;31(8):1191-5.
3. Basturk O, Hong SM, Wood LD, et al. A Revised Classification System and Recommendations From the Baltimore Consensus Meeting for Neoplastic Precursor Lesions in the Pancreas. Am J Surg Pathol. 2015 Dec;39(12):1730-41.
4. Ma C, Gocke CD, Hruban RH, Belchis DA. Mutational spectrum of intraepithelial neoplasia in pancreatic heterotopia. Hum Pathol. 2016 Feb;48:117-21.
Presented by Dr. A. Cimino-Mathews and prepared by Dr. J. Judd Fite
Clinical history: A hospitalized 65 year-old male develops septic shock
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Week 639: Case 1
Clinical history: A hospitalized 65 year-old male develops septic shock
Correct
Answer: D. Pseudomembranous colitis
Histology: The specimen received is a total colectomy. The entire mucosal surface is grossly remarkable grossly for a thin, yellow-green membranous film. Histologic sections show regions of the colonic mucosa to be entirely necrotic with alternative areas of residual viable epithelium. The luminal surface of the bowel has a layer of fibrin and acute inflammatory cells corresponding to the grossly apparent pseudomembrane. There is submucosal edema, but the underlying muscularis propria and subserosal soft tissue are fairly unremarkable with no evidence of vasculitis. There are no granulomata or viral cytopathic changes. The patient’s stool was tested by the molecular microbiology lab and was positive for Clostridium difficile toxin B gene by nucleic acid testing.
Discussion: The clinical presentation, laboratory results, and pathologic findings are diagnostic of pseudomembranous colitis due to Clostridium difficile infection in the setting of prior antibiotic use. C. difficile is an anaerobin, gram-positive, spore-forming, bacillus that produces pathogenic exotoxins A and B. C. difficile is not part of the normal gut flora, but rather will colonize the intestines after the normal flora is disrupted by prior antibiotic treatment. C. difficile colitis is a leading nosocomial infection and can result in fulminant colitis, toxic megacolon, bowel perforation, and sepsis with high mortality. The damage to the intestine is due to the effects of the toxin, not due to actual bacterial invasion or overgrowth within the mucosa. Gross and histologic examination of the tissue often reveals the classic “pseudomembranous colitis” with diffuse necrosis of the mucosa and an overlying “pseudomembrane” of necrotic tissue, mucus, fibrin and inflammatory cells. Diagnosis of C. difficile can be done in several ways including enzyme immunoassay and DNA based assays such as RT-PCR for the toxins; although the histologic presence of diffuse pseudomembranous colitis raises the possibility of C. difficile infection, the diagnosis should be made in conjunction with the laboratory results.
References:
1. Svensson AM, LaSala PR; Education Committee of the Academy of Clinical Laboratory Physicians and Scientists. Pathology consultation on detection of Clostridium difficile. Am J Clin Pathol. 2012 Jan;137(1):10-5.
2. Leffler DA, Lamont JT. Clostridium difficile Infection. N Engl J Med. 2015 Jul 16;373(3):287-8
Incorrect
Answer: D. Pseudomembranous colitis
Histology: The specimen received is a total colectomy. The entire mucosal surface is grossly remarkable grossly for a thin, yellow-green membranous film. Histologic sections show regions of the colonic mucosa to be entirely necrotic with alternative areas of residual viable epithelium. The luminal surface of the bowel has a layer of fibrin and acute inflammatory cells corresponding to the grossly apparent pseudomembrane. There is submucosal edema, but the underlying muscularis propria and subserosal soft tissue are fairly unremarkable with no evidence of vasculitis. There are no granulomata or viral cytopathic changes. The patient’s stool was tested by the molecular microbiology lab and was positive for Clostridium difficile toxin B gene by nucleic acid testing.
Discussion: The clinical presentation, laboratory results, and pathologic findings are diagnostic of pseudomembranous colitis due to Clostridium difficile infection in the setting of prior antibiotic use. C. difficile is an anaerobin, gram-positive, spore-forming, bacillus that produces pathogenic exotoxins A and B. C. difficile is not part of the normal gut flora, but rather will colonize the intestines after the normal flora is disrupted by prior antibiotic treatment. C. difficile colitis is a leading nosocomial infection and can result in fulminant colitis, toxic megacolon, bowel perforation, and sepsis with high mortality. The damage to the intestine is due to the effects of the toxin, not due to actual bacterial invasion or overgrowth within the mucosa. Gross and histologic examination of the tissue often reveals the classic “pseudomembranous colitis” with diffuse necrosis of the mucosa and an overlying “pseudomembrane” of necrotic tissue, mucus, fibrin and inflammatory cells. Diagnosis of C. difficile can be done in several ways including enzyme immunoassay and DNA based assays such as RT-PCR for the toxins; although the histologic presence of diffuse pseudomembranous colitis raises the possibility of C. difficile infection, the diagnosis should be made in conjunction with the laboratory results.
References:
1. Svensson AM, LaSala PR; Education Committee of the Academy of Clinical Laboratory Physicians and Scientists. Pathology consultation on detection of Clostridium difficile. Am J Clin Pathol. 2012 Jan;137(1):10-5.
2. Leffler DA, Lamont JT. Clostridium difficile Infection. N Engl J Med. 2015 Jul 16;373(3):287-8
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