Extragenital adenosarcoma: a case report, review of the literature, and management discussion

Abstract

Background: Müllerian adenosarcoma is a rare mixed epithelial-mesenchymal tumor. An extragenital site of origin and sarcomatous overgrowth are associated with aggressive clinical behavior.

Case: We present a rare case of extragenital adenosarcoma with sarcomatous overgrowth and coexistent endometriosis. She was treated with initial cytoreductive surgery and chemotherapy. She underwent a second surgery for management of a high-grade bowel obstruction, due to pathologically confirmed recurrent intraperitoneal adenosarcoma. A complete clinical response was achieved with liposomal doxorubicin, and the patient remains disease-free eighteen months after completion of chemotherapy.

Conclusion: Liposomal doxorubicin appears to be an active agent for the treatment of adenosarcoma with sarcomatous overgrowth. In addition, we conclude from our review of all reported cases of extragenital adenosarcoma that concurrent endometriosis may represent a favorable prognostic factor.

Figure 1

A: Benign endometriosis B: Endometriotic glands are cuffed by malignant stroma, a characteristic finding of adenosarcoma C: Higher power view of the malignant stroma, demonstrating nuclear pleomorphism, atypia, and hyperchromasia. In addition, the mitoses are in excess of 2 per 10 high power fields. D: In the areas of sarcomatous overgrowth, the malignant cells contain enlarged, hyperchromatic nuclei with marked nuclear pleomorphism and numerous atypical mitotic figures

Figure 2

A: Estrogen receptor expression is seen in the endometriosis component B: The glandular component demonstrates strong progesterone receptor expression C: Tumor cells are diffusely immunopositive for desmin D: Focal immunopositivity is seen for CD 10

Figure 3

The ileal mesenteric mass consists of a high-grade pure sarcoma with increased cytological atypia compared with the previously resected tumor. Background necrosis is also present.