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Presented by Ralph Hruban, M.D. and prepared by Maryam Farinola M.D.
Case 2: This patient in their early 40’s developed severe dyspepsia that did not respond to medical management.
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Question 1 of 1
1. Question
Week 198: Case 2
This patient in their early 40’s developed severe dyspepsia that did not respond to medical management. A gastrectomy was performed following biopsy. Of note, the patient had a family history of “abdominal” cancer.images/signet ring cell 1.jpg
images/signet ring cell 2.jpg
images/signet ring cell 3.jpgCorrect
Answer: Signet ring cell carcinoma
Histology: This neoplasm is composed of non-cohesive cells with a signet-ring appearance. A stain for mucin was positive, while immunolabeling for e-cadherin revealed a loss of e-cadherin expression. Immunolabeling for estrogen and progesterone receptors were both negative.
Discussion: Most signet ring cell neoplasms in the stomach are primary sporadic signet ring cell adenocarcinomas. As in this case, these neoplasms express cytokeratin, they do not express estrogen or progesterone receptors, and they show a loss of e-cadherin. E-cadherin is involved in the formation of cell-junctions and the maintenance of epithelial integrity. Germ line E-cadherin (CDH1) mutations cause hereditary diffuse gastric cancer (HDGC) (see reference). A review of patients diagnosed with diffuse gastric cancers before the age of 51 has shown that some of these young age of onset cancers are also caused by germ line CDH1 mutations. Thus, germ line CDH1 mutations should be thought of in patients with a strong family history of gastric cancer and in patients with early age at diagnosis.
Reference(s):
– Suriano et al, Identification of CDH1 germline missense mutations associated with functional inactivation of the E-cadherin protein in young gastric cancer probands.
Hum Mol Genet. 2003 Mar 1;12(5):575-82.Incorrect
Answer: Signet ring cell carcinoma
Histology: This neoplasm is composed of non-cohesive cells with a signet-ring appearance. A stain for mucin was positive, while immunolabeling for e-cadherin revealed a loss of e-cadherin expression. Immunolabeling for estrogen and progesterone receptors were both negative.
Discussion: Most signet ring cell neoplasms in the stomach are primary sporadic signet ring cell adenocarcinomas. As in this case, these neoplasms express cytokeratin, they do not express estrogen or progesterone receptors, and they show a loss of e-cadherin. E-cadherin is involved in the formation of cell-junctions and the maintenance of epithelial integrity. Germ line E-cadherin (CDH1) mutations cause hereditary diffuse gastric cancer (HDGC) (see reference). A review of patients diagnosed with diffuse gastric cancers before the age of 51 has shown that some of these young age of onset cancers are also caused by germ line CDH1 mutations. Thus, germ line CDH1 mutations should be thought of in patients with a strong family history of gastric cancer and in patients with early age at diagnosis.
Reference(s):
– Suriano et al, Identification of CDH1 germline missense mutations associated with functional inactivation of the E-cadherin protein in young gastric cancer probands.
Hum Mol Genet. 2003 Mar 1;12(5):575-82.
