Malignant hemangioendothelioma of the uterus

doi:10.1016/0090-8258(79)90048-9

Gynecologic Oncology

Volume 8, Issue 3, December 1979, Pages 376-383

https://doi.org/10.1016/0090-8258(79)90048-9 Get rights and content

Abstract

In the entire world literature, there are only seven reported cases of malignant hemangioendothelioma arising in the uterus. An eighth case, reported here, was a 17-year-old girl who lived for 8 years after curettings and subsequent hysterectomy revealed malignant hemangioendothelioma of the endometrium with extension to the left ovary. Pelvic recurrences were temporarily controlled with chemotherapy, X-ray treatment, and repeated surgery. However, the patient eventually succumbed to massive growth of malignant hemangioendothelioma in the pelvis, with extension into the inferior vena cava and left external iliac vein. Growth within blood vessel lumens was also striking in the surgical specimens of recurrent tumor. Microscopically, the important diagnostic feature was the presence of numerous capillaries lined by malignant endothelial cells.

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Methods. A case report is presented with a review of the English language literature via a Medline search. The key phrases used in the search were uterine angiosarcoma, hemangiosarcoma, hemangioendothelioma, and primary uterine neoplasm.
Results. Since the first report in 1902, there have been 19 reported cases of primary uterine angiosarcoma considered valid. Many early cases are questioned due to the lack of ultrastructural and immunohistochemical evidence available in later cases. Seventy-four percent (14 of 19) of these patients are perimenopausal with a mean age of 55 years (range 17–76 years). The common presenting findings are a pelvic mass, menorrhagia, and weight loss. Varying regimens of surgery, chemotherapy, and radiation have been utilized with limited success.
Conclusions. Primary uterine angiosarcomas tend to exhibit a highly malignant behavior. The predominant prognostic factor seems to be the size of the tumor at diagnosis and the presence of extrapelvic disease. Recurrence occurs on average at 8.2 months. Of evaluable patients (n = 14), at 12 months the survival was only 43%. Although radiation and chemotherapy are options being utilized, no consensus exists for optimal therapy given the few cases from which to draw conclusions. Regardless of treatment, outcome is usually poor.
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Case reportMalignant hemangioendothelioma of the uterus

Abstract

Vascular tumors of the female genital tract

Obstet. Gynecol.

Morphological and clinical aspects of malignant vascular tumors of the uterus

Arch. Gynäkol.

Hemangioendothelioma of uterus

Urol. Cutan. Rev.

Haemangioendothelioma of the uterus

Ann. Chir. Gynaecol. Fenn.

Hemangio-endothelioma: A tumor of blood vessels featuring vascular endothelial cells

Ann. Surg.

Case report
Malignant hemangioendothelioma of the uterus