Korean J Gynecol Oncol.  2008 Mar;19(1):87-92. 10.3802/kjgo.2008.19.1.87.

Endometrial stromal sarcoma associated with extrauterine endometriosis: a case report and literature review

• Park SK
• Lee SJ
• Kwon HS
• Sohn IS
• Lee JY
• Kim SN
• Cha YJ
• Lim SD

• KMID: 1979506
• DOI: http://doi.org/10.3802/kjgo.2008.19.1.87

Abstract

Endometrial stromal sarcoma (ESS) that arises from extrauterine endometriosis is a rare form of malignancy. We report the case of a 37-year-old ESS patient with extrauterine endometriosis who was treated with ifosfamide/cisplatin chemotherapy. A woman presented with epigastric pain and abdominal distension. Computed tomography imaging revealed a profuse amount of ascites, including a 12.4x12.3 cm sized posterior cul-de-sac mass composed of solid and cystic components. Cytoreductive surgery was performed to remove the mass and the histopathologic findings indicated ESS associated with extrauterine endometriosis. Six cycles of combination chemotherapy [ifosfamide (5 g/m(2)) with mesna (1 g/m(2)) and cisplatin (50 mg/m(2)) (IP)] were administered. After a six-month of disease-free interval, recurrent ESS developed in the pelvic cavity and in both lung fields. Megace medication decreased tumor marker CA-125 for six weeks. However, the patient expired sixteen months after the cytoreductive surgery. ESS associated with extrauterine endometriosis showed response to IP chemotherapy and megace.

Keyword

Endometriosis; Extrauterine; Endometrial stromal sarcoma; Chemotherapy

MeSH Terms

Adult
Ascites
Cisplatin
Drug Therapy, Combination
Endometriosis
Female
Humans
Lung
Megestrol Acetate
Mesna
Sarcoma, Endometrial Stromal
Cisplatin
Megestrol Acetate
Mesna

Figure

• 

  Fig. 1 Abdomino-pelvic CT finding shows profuse amount of ascites and 12.4×12.3 cm mass composed of solid and cystic components (‡).

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  Fig. 2 Gross specimens shows diffuse irregular soft tissue nodules on the external surfaces of the uterus, both ovaries and fallopian tubes. No intraparenchymal lesion is present in the right ovary (inset).

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  Fig. 3 (A) Extrauterine (mesenteric) tumor nodules show diffuse proliferation of small cells (Original magnification, ×40). (B) High magnification of the tumor shows diffuse proliferation of tumor cells with frequent mitosis and conspicuous spiral arteriole-like vessels resembling endometrial stroma (Original magnification, ×400). (C) The tumor is present at the ovarian surface (*) and foci of endometriosis (**) are often noted in continuity with the tumor (Original magnification, ×12.5).

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  Fig. 4 Immunohistochemical staining shows diffuse and strong nuclear immunoreactivity of estrogen receptor (A) and progesterone receptor (B). CD10 is negative in the tumor cells (C) (Original magnification, ×400).

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