27 May 2005 – Case of the Week #8
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This case was contributed by Dr. Hind Nassar, Wayne State University Department of Pathology, Detroit, Michigan (USA). We invite you to contribute a Case of the Week by emailing NPernick@PathologyOutlines.com with microscopic images (any size, we will shrink if necessary) in JPG or GIF format, a short clinical history and any other images (gross, immunostains, EM, etc.) that you have and that may be helpful or interesting. We particularly need cases of GI, GU and Gynecologic pathology. Please only send cases with a definitive diagnosis.
Case of the Week #8
A 52 year old woman had a breast mass, which was excised. She also had two positive axillary lymph nodes.
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Diagnosis: Secretory carcinoma of the breast
Secretory carcinoma of the breast is also known as juvenile carcinoma. It is a rare subtype of breast carcinoma in adults, but is the most common form of breast carcinoma in children. The tumors are usually small and well circumscribed. Tumors are composed of tubuloalveolar or focal papillary formations of relatively bland cells with abundant pale to eosinophilic cytoplasm, often vacuolated. Nucleoli are frequently prominent. The most striking feature is the luminal eosinophilic secretions, which are PAS+ and diastase resistant (image). Mitotic figures are rare. It is often difficult to determine how much of the tumor represents in situ versus invasive disease. The tumors have pushing margins, and often central hyalinization.
Tumor cells are immunoreactive for S100 and alpha-lactalbumin with variable staining for GCDFP-15 and CEA. They are often negative for estrogen and progesterone receptors. Electron microscopy shows numerous membrane bound, intracytoplasmic secretory vacuoles.
Secretory carcinoma is important to recognize as a subtype because of its excellent prognosis, with a 5 year survival approaching 100% in children. In women age 30 years or older, the prognosis is less favorable, due to late local recurrence and nodal metastases, but distant metastases and death due to disease are still rare. These tumors also differ from the usual invasive ductal carcinoma due to expression of a chimeric tyrosine kinase, encoded by a ETV6-NTRK3 fusion gene (Hum Path 2003;34:1299, Semin Cancer Biol. 2005;15:215, Genes Chromosomes Cancer 2004;40:152.)
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