Endometrial Stromal Sarcomas of the Uterus With Extensive Endometrioid Glandular Differentiation

Philip B. Clement, Robert E. Scully
In: International Journal of Gynecological Pathology · 1992 · vol. 11(3) , pp. 174 · doi:10.1097/00004347-199207000-00001 · W4322702027
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This report describes three cases of endometrial stromal sarcoma with extensive endometrioid glandular differentiation, presenting diagnostic challenges that require distinguishing them from various other uterine and extrauterine lesions.

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This report describes three cases of low-grade endometrial stromal sarcoma featuring extensive endometrioid glandular differentiation, which complicated differential diagnosis in patients aged 41 to 47 presenting with abnormal uterine bleeding or pelvic masses. The tumors exhibited varying degrees of glandular atypia, ranging from benign-appearing glands to carcinomatous changes, and were managed through hysterectomy with uneventful short-term follow-up for all patients. The authors emphasize the critical need to distinguish these entities from adenomyosis, endometriosis, adenosarcomas, and high-grade carcinomas to ensure accurate classification and treatment. Relevance to endometriosis: explicitly discussed as a key differential diagnosis that must be distinguished from endometrial stromal sarcomas with glandular differentiation, with extrauterine aggressive lesions potentially representing such sarcomas rather than true endometriosis.

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Abstract

Summary Three cases of endometrial stromal sarcoma (ESS) with prominent glandular differentiation within the primary or recurrent tumors are described. Each case posed a problem in diagnosis and classification. The patients, who ranged in age from 41 to 47 years, presented with abnormal uterine bleeding, a pelvic mass, or a combination thereof. All the patients underwent hysterectomy with or without bilateral salpingo-oophorectomy. There was no evidence of extrauterine spread of tumor in any case. Polypoid tumors involved the endometrium in two cases, and in one of them, tumor deeply invaded the myometrium. The tumor in the third case was an infiltrative mass that was confined to the myometrium and its vessels. On microscopic examination, the tumors were low-grade ESSs that contained large numbers of endometrioid glands, which were benign appearing in two cases, and in the third varied from atypical to carcinomatous. In one of the cases in the first group, the glandular component was present only in recurrent tumor excised 10 years after hysterectomy; prominent foci of sex-cord-like differentiation were also present in the recurrent tumor. This patient was clinically free of tumor 27 months later: follow-up in the other two patients was uneventful. ESSs with prominent numbers of benign-appearing glands should be distinguished from adenomyosis, endometriosis, ESSs arising in adenomyosis or endometriosis, ESSs with sex-cord-like differentiation, and müllerian adenosarcomas. ESSs with carcinomatous glands should be distinguished from endometrial adenocarcinomas and malignant müllerian mixed tumors (carcinosarcomas). Extrauterine lesions that have been designated “aggressive endometriosis” may be examples of extrauterine ESS with prominent glandular differentiation.
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Endometrial Stromal Sarcomas of the Uterus With Extensive Endometrioid Glandular Differentiation A Report of Three Cases That Caused Problems in Differential Diagnosis - Philip B. Clement - Robert E. Scully Summary Three cases of endometrial stromal sarcoma (ESS) with prominent glandular differentiation within the primary or recurrent tumors are described. Each case posed a problem in diagnosis and classification. The patients, who ranged in age from 41 to 47 years, presented with abnormal uterine bleeding, a pelvic mass, or a combination thereof. All the patients underwent hysterectomy with or without bilateral salpingo-oophorectomy. There was no evidence of extrauterine spread of tumor in any case. Polypoid tumors involved the endometrium in two cases, and in one of them, tumor deeply invaded the myometrium. The tumor in the third case was an infiltrative mass that was confined to the myometrium and its vessels. On microscopic examination, the tumors were low-grade ESSs that contained large numbers of endometrioid glands, which were benign appearing in two cases, and in the third varied from atypical to carcinomatous. In one of the cases in the first group, the glandular component was present only in recurrent tumor excised 10 years after hysterectomy; prominent foci of sex-cord-like differentiation were also present in the recurrent tumor. This patient was clinically free of tumor 27 months later: follow-up in the other two patients was uneventful. ESSs with prominent numbers of benign-appearing glands should be distinguished from adenomyosis, endometriosis, ESSs arising in adenomyosis or endometriosis, ESSs with sex-cord-like differentiation, and müllerian adenosarcomas. ESSs with carcinomatous glands should be distinguished from endometrial adenocarcinomas and malignant müllerian mixed tumors (carcinosarcomas). Extrauterine lesions that have been designated “aggressive endometriosis” may be examples of extrauterine ESS with prominent glandular differentiation.

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