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Presented by Pedram Argani, M.D. and prepared by Maryam Farinola M.D.
Case 2: A 17-year-old female with a breast mass, and a history of a pancreatoduodenectomy.
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1. Question
Week 165: Case 2
A 17-year-old female with a breast mass, and a history of a pancreatoduodenectomy./images/metisletcelllow.jpg
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/metisletcellchr.jpgCorrect
Answer: Metastatic islet cell tumor
Histology: This is an unusual case. There is a solid and nested tumor, which intermingles with the normal breast epithelium. These tumor cells have uniform, “salt and pepper” nuclei, and show minimal mitotic activity. These are typical features of a well-differentiated endocrine neoplasm. However, this case is most unusual in that the neoplasm actually grows within native duct epithelium, within areas of sclerosis that mimic a compact sclerosing lesion. This intraepithelial growth simulates an in situ component. The endocrine tumor labels strongly for CD 56, chromogranin and synaptophysin. It is negative for gross cystic disease fluid protein. The presence of an intraepithelial growth pattern is supported by immunostains for myoepithelial markers such as p63 and smooth muscle myosin heavy chain (SMM-HC). This patient had a previous resection for an islet cell tumor of the pancreas, which was focally immunoreactive for insulin; a similar pattern of immunoreactivity was seen in the current case.
Discussion: In a different setting, this pattern of growth would be very difficult to distinguish from in situ and infiltrating ductal carcinoma of the breast, specifically since it is well known that breast cancers can show neuroendocrine differentiation. However, the key to this case is both the patient’s prior surgical history and the patient’s age; breast cancer is distinctly uncommon under the age of 20. One type of breast cancer that does occur in patients below the age of 20 is secretory carcinoma of the breast, which characteristically has the same ETV6-NTRK3 gene fusion as infantile fibrosarcoma and cellular congenital mesoblastic nephroma. However, secretory carcinoma is typically circumscribed, has vacuolated cytoplasm with PAS-positive intracellular and extracellular material. Alveolar rhabdomyosarcoma has a propensity to metastasize to the breast of children; however, the growth pattern usually mimics invasive lobular carcinoma rather than invasive ductal carcinoma.
This case is an excellent example of the fact that metastatic epithelial neoplasms can colonize the epithelial surfaces of the organs to which they metastasize, and therefore simulate an in situ component. This has been well described in metastatic colorectal carcinoma involving the liver, which can grow within the bile ducts to simulate an intrahepatic cholangiocarcinoma.
Incorrect
Answer: Metastatic islet cell tumor
Histology: This is an unusual case. There is a solid and nested tumor, which intermingles with the normal breast epithelium. These tumor cells have uniform, “salt and pepper” nuclei, and show minimal mitotic activity. These are typical features of a well-differentiated endocrine neoplasm. However, this case is most unusual in that the neoplasm actually grows within native duct epithelium, within areas of sclerosis that mimic a compact sclerosing lesion. This intraepithelial growth simulates an in situ component. The endocrine tumor labels strongly for CD 56, chromogranin and synaptophysin. It is negative for gross cystic disease fluid protein. The presence of an intraepithelial growth pattern is supported by immunostains for myoepithelial markers such as p63 and smooth muscle myosin heavy chain (SMM-HC). This patient had a previous resection for an islet cell tumor of the pancreas, which was focally immunoreactive for insulin; a similar pattern of immunoreactivity was seen in the current case.
Discussion: In a different setting, this pattern of growth would be very difficult to distinguish from in situ and infiltrating ductal carcinoma of the breast, specifically since it is well known that breast cancers can show neuroendocrine differentiation. However, the key to this case is both the patient’s prior surgical history and the patient’s age; breast cancer is distinctly uncommon under the age of 20. One type of breast cancer that does occur in patients below the age of 20 is secretory carcinoma of the breast, which characteristically has the same ETV6-NTRK3 gene fusion as infantile fibrosarcoma and cellular congenital mesoblastic nephroma. However, secretory carcinoma is typically circumscribed, has vacuolated cytoplasm with PAS-positive intracellular and extracellular material. Alveolar rhabdomyosarcoma has a propensity to metastasize to the breast of children; however, the growth pattern usually mimics invasive lobular carcinoma rather than invasive ductal carcinoma.
This case is an excellent example of the fact that metastatic epithelial neoplasms can colonize the epithelial surfaces of the organs to which they metastasize, and therefore simulate an in situ component. This has been well described in metastatic colorectal carcinoma involving the liver, which can grow within the bile ducts to simulate an intrahepatic cholangiocarcinoma.
