Case report
Psammomacarcinoma of the peritoneum

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Abstract

Psammomacarcinoma is a rare histologic subtype of serous carcinoma originating in the ovary or peritoneum, characterized by massive psammoma body formation, invasiveness, and low-grade differentiation. Its clinical behavior appears similar to that of serous borderline tumors rather than that of typical invasive serous carcinomas. Peritoneal psammomacarcinoma is even rarer than its ovarian counterpart with only 10 cases have previously been documented in the literature. A case of F.I.G.O. stage IIIC peritoneal psammomacarcinoma is described. Optimal debulking surgery, including omentectomy, total abdominal hysterectomy, bilateral adnexectomy, and resection of segment of jejunum, was performed. The patient received adjuvant chemotherapy with taxol and cisplatin, and to date, 15 months after surgery, she is alive and with no evidence of disease. It is concluded that peritoneal psammomacarcinoma is a very rare tumor that behaves less aggressively than typical serous carcinoma. The mainstay of treatment is surgical debulking. The role of adjuvant chemotherapy has as yet not been established.

Introduction

Psammomacarcinoma is a rare histologic subtype of ovarian and peritoneal papillary serous carcinoma morphologically characterized by extensive psammomatous calcifications (“psammoma bodies”), invasiveness, and low-grade cytological features [1], [2], [3], [4], [5], [6], [7], [8], [9]. Based on a small number of reported cases, it seems that the clinical behavior of psammomacarcinoma more closely resembles that of serous borderline tumors rather than that of typical invasive papillary serous carcinomas, with a prolonged clinical course and relatively favorable prognosis [2], [4], [6], [7], [8], [9]. Peritoneal psammomacarcinoma is even rarer than ovarian psammomacarcinoma with only 10 cases previously documented in the English literature [1], [2], [3], [4], [5], [6]. We describe an additional case of peritoneal psammomacarcinoma and review pertinent literature.

Section snippets

Case report

A 67-year-old, nulligravid, married postmenopausal Sephardic Jewish woman was admitted in April 1999 because of a 7-month history of increasing abdominal pain and girth and progressive weakness. Menarche had occurred at age 13. Menses, until menopause at age 50, had been regular with a 28-day cycle and flow lasting 5 days. She had not received hormone replacement therapy and had not experienced postmenopausal bleeding. Her past medical history included obesity, diabetes mellitus and

Discussion

Psammomacarcinoma is a rare form of invasive papillary serous carcinoma associated with extensive psammoma body formation [1], [2], [3], [4], [5], [6], [7], [8], [9]. Psammoma bodies are calcified spherules (calcospherules), measuring 5–100 μm in diameter, with concentric laminations, thought to be secondary to accumulations of hydroxyapatite [Ca10(PO4)6(OH)2] in cells that undergo dystrophic calcification related to cellular degeneration [2], [7], [9]. A single layer of epithelial cells with

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