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Presented by Dr. Andres Matoso and prepared by Dr. Tatianna Larman.
An adult with a gastric mass.
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Question 1 of 2
1. Question
An adult with a gastric mass. Choose the correct diagnosis.
Correct
Answer: SDH-deficient gastrointestinal stromal tumor
Histology and discussion: SDH deficient GISTs occur almost exclusively in the stomach. Recently, a duodenal one was reported in a patient with SDHB germline mutation. They are characterized by epithelioid morphology, plexiform (multinodular) architecture and metastasis to lymph nodes. They may be part of the Carney-Stratakis syndrome (paraganglioma and GIST) or Carney’s triad (paraganglioma, GIST and pulmonary chondroma). These tumors lack kinase mutations but they can still be CD117 and DOG1 positive by immunohistochemistry. SDHB is lost by immunohistochemistry, which is diagnostic of SDH-deficient GIST. Negative immunostain for SDHB indicates either germline mutation in any of the SDH genes or SDHC hypermethylation. Rare somatic mutations have been reported. Conventional risk stratification does not accurately predict prognosis in this type of GISTs.
References:
1. Am J Surg Pathol. 2016 Dec;40(12):1616-1621.
2. J Clin Endocrinol Metab. 2017 May 1;102(5):1447-1450.
3. Endocrine. 2014 Nov;47(2):401-8.Incorrect
Answer: SDH-deficient gastrointestinal stromal tumor
Histology and discussion: SDH deficient GISTs occur almost exclusively in the stomach. Recently, a duodenal one was reported in a patient with SDHB germline mutation. They are characterized by epithelioid morphology, plexiform (multinodular) architecture and metastasis to lymph nodes. They may be part of the Carney-Stratakis syndrome (paraganglioma and GIST) or Carney’s triad (paraganglioma, GIST and pulmonary chondroma). These tumors lack kinase mutations but they can still be CD117 and DOG1 positive by immunohistochemistry. SDHB is lost by immunohistochemistry, which is diagnostic of SDH-deficient GIST. Negative immunostain for SDHB indicates either germline mutation in any of the SDH genes or SDHC hypermethylation. Rare somatic mutations have been reported. Conventional risk stratification does not accurately predict prognosis in this type of GISTs.
References:
1. Am J Surg Pathol. 2016 Dec;40(12):1616-1621.
2. J Clin Endocrinol Metab. 2017 May 1;102(5):1447-1450.
3. Endocrine. 2014 Nov;47(2):401-8. -
Question 2 of 2
2. Question
An adult with a renal mass. Choose the correct diagnosis.
Correct
Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Reference:
– Clin Genitourin Cancer. 2016 Dec;14(6):e553-e557.Incorrect
Answer: Multilocular cystic renal cell neoplasm of low malignant potential
Histology and discussion: Multilocular cystic renal cell neoplasm of low malignant potential is composed of a multiple cystic spaces lined by clear cells with low nuclear grade (ISUP nuclear grade 1 or 2). The stroma may have some clusters of clear cells but they are not expanding or forming solid nodules. The specimen has to be sample generously (at least one cassette per cm with 2 or 3 sections per cassette) with especial attention to sample well any potential solid areas. If there is any solid growth, then clear cell renal cell carcinoma with cystic change should be entertained as the diagnosis. In contrast, if the stroma shows no cluster of clear cells, then atypical renal cyst should be considered. The prognosis is excellent with a 5 year survival rate of 100% in the small series published.
Reference:
– Clin Genitourin Cancer. 2016 Dec;14(6):e553-e557.
