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Presented by Ralph Hruban, M.D. and prepared by Amy Duffield, M.D., Ph.D.
Case 6: This female patient complained of vague upper abdominal symptoms.
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1. Question
Week 365: Case 6
This female patient complained of vague upper abdominal symptoms. She was found to have a very large pancreatic mass. The mass was resected.images/09_15_08 6A.jpg
images/09_15_08 6B.jpg
images/09_15_08 6C.jpg
images/09_15_08 6D.jpg
images/09_15_08 6E.jpgCorrect
Answer: Invasive sarcoma arising in a mucinous cystic neoplasm with high-grade dysplasia
Histology: This tumor has the features of a mucinous cystic neoplasm- ovarian stroma, tall columnar mucin-producing cells. The neoplastic cells have significant cytologic and architectural atypia, making it a mucinous cystic neoplasm with high-grade dysplasia. In addition, a small invasive component is seen. This invasive component is focally undifferentiated and spindles out.
Discussion: Invasive carcinomas can arise in association with mucinous cystic neoplasms. As discussed in this conference previously, these invasive components can be focal. As seen in this case, the invasive component rarely is composed of undifferentiated spindle shaped cells. Since mucinous cystic neoplasms contain spindle shaped cells (the ovarian stroma) it had been wondered if the invasive spindle cells are derived from the ovarian stroma or the epithelium. Molecular analyses of each of the components of these tumors has shown that the undifferentiated spindle shaped cells have the same genetic alterations as are seen in the neoplastic epithelium. The ovarian-type stromal cells lack mutations. The invasive component is therefore best classified as an undifferentiated carcinoma.
Reference(s):
– Sarcomatoid MCN. Mod Pathol. 2000 Jan;13(1):86-91.Incorrect
Answer: Invasive sarcoma arising in a mucinous cystic neoplasm with high-grade dysplasia
Histology: This tumor has the features of a mucinous cystic neoplasm- ovarian stroma, tall columnar mucin-producing cells. The neoplastic cells have significant cytologic and architectural atypia, making it a mucinous cystic neoplasm with high-grade dysplasia. In addition, a small invasive component is seen. This invasive component is focally undifferentiated and spindles out.
Discussion: Invasive carcinomas can arise in association with mucinous cystic neoplasms. As discussed in this conference previously, these invasive components can be focal. As seen in this case, the invasive component rarely is composed of undifferentiated spindle shaped cells. Since mucinous cystic neoplasms contain spindle shaped cells (the ovarian stroma) it had been wondered if the invasive spindle cells are derived from the ovarian stroma or the epithelium. Molecular analyses of each of the components of these tumors has shown that the undifferentiated spindle shaped cells have the same genetic alterations as are seen in the neoplastic epithelium. The ovarian-type stromal cells lack mutations. The invasive component is therefore best classified as an undifferentiated carcinoma.
Reference(s):
– Sarcomatoid MCN. Mod Pathol. 2000 Jan;13(1):86-91.
