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Primary peritoneal malignant mixed Müllerian tumors
Author(s) -
Shen DanHua,
Khoo UiSoon,
Xue WeiCheng,
Ngan Hextan Y. S.,
Wang Jian Liu,
Liu Vincent W. S.,
Chan YuenKwong,
Cheung Annie N. Y.
Publication year - 2001
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(20010301)91:5<1052::aid-cncr1097>3.0.co;2-a
Subject(s) - medicine , pathology , immunohistochemistry , carcinoma , adenocarcinoma , cancer
BACKGROUND Primary peritoneal malignant mixed Müllerian tumors (MMMTs) are rarely reported in the literature. METHODS The clinical, pathologic, and immunohistochemical features of five cases of MMMT of female peritoneum were analyzed. The tumors were also investigated for expression of hormone receptors, specific BRCA‐1 mutations, and clonality. RESULTS The patients' ages ranged from 33 to 67 years. They presented with abdominal pain or mass. One case of peritoneal MMMT was associated with a synchronous endometrial carcinoma whereas another case was detected 2 years after the diagnosis of a primary adenocarcinoma of the fallopian tube. One patient died 1 month after diagnosis whereas 2 patients died with disease within 1 year. Both carcinomatous and sarcomatous elements are present in all the tumors. Squamous differentiation was noted in two cases. Heterologous elements, including chondroid, rhabodomyoblastic, and osteoid differentiation were detected in all tumors. Immunohistochemical studies confirm the biphasic differentiation with variable demonstration of neural and smooth muscle differentiation. All five MMMTs were negative for estrogen and progestogen receptors although the related endometrial and tubal carcinomas were positive. Heteroduplex analysis used to screen for specific BRCA‐1 mutations were negative in all five MMMTs. Clonality study of the two MMMTs found in association with endometrial carcinoma and tubal carcinoma was inconclusive. CONCLUSIONS Our study confirmed that primary peritoneal MMMTs were aggressive tumors with poor prognosis. The presence of synchronous or metachronous genital carcinomas suggests multifocal tumorigenesis from tissue of same embryologic origin. The lack of hormone receptor in these tumors indicates deviation from hormonal control. Specific BRCA‐1 mutations found in ovarian carcinoma in Chinese patients could not be detected in our series. Cancer 2001;91:1052–60. © 2001 American Cancer Society.