Rhabdomyosarcomas of the Uterus WILLIAM R. HART, M.D., AND JOHN R. CRAIG, M.D., PH.D.
RHABDOMYOSARCOMAS infrequently originate in the female genital tract. Most rhabdomyosarcomas are associated with carcinoma and represent heterologous differentiation in a carcinosarcoma or malignant mixed mesodermal tumor. Pure rhabdomyosarcomas of the female genital tract generally occur during infancy or childhood, are of the botryoid embryonal type, and predominantly involve the vagina; in some reports they have been referred to as "sarcoma botryoides." 2 ' 0 - 1 2 1 8 Primary uterine sarcomas composed of rhabdomyosarcoma and not associated with carcinoma are especially rare in adults. 612 In the past such heterologous sarcomas often have been regarded as variants of malignant mixed mesodermal tumors. 1 7 1 8 Modern classifications of uterine sarcomas, however, separate the pure heterologous sarcomas, such as rhabdomyosarcoma, from those with carcinomatous elements, as well as from the pure homologous sarcomas (e.g., leiomyosarcoma, endometrial stromal sarcoma). 612 In this investigation we studied the clinicopathologic features of four cases of adult women who had primary rhabdomyosarcomas of the uterus. All were pure sarcomas. Two tumors were regarded as botryoid embryonal rhabdomyosarcomas and the other two as Received May 12, 1977; received revised manuscript August 1, 1977; accepted for publication August 1, 1977. Address reprint requests to Dr. Hart: Department of Pathology, University of Michigan Medical Center, 1335 E. Catherine St., Ann Arbor, Michigan 48109.
From the Departments of Pathology, The University of Michigan Medical Center, Ann Arbor, Michigan, and the Los Angeles County/University of Southern California Medical Center, Los Angeles, California
pleomorphic rhabdomyosarcomas. Follow-up information was available for each patient. Materials and Methods Cases 1 and 2 were encountered at the Los Angeles County/University of Southern California Medical Center. Cases 3 and 4 were diagnosed as rhabdomyosarcomas during a retrospective review of all patients with primary uterine sarcomas registered at The University of Michigan Medical Center. Our experiences with uterine leiomyosarcomas, endometrial stromal sarcomas and stromatosis are reported separately. 4 5 1 9 Microscopic slides of the primary tumors were available for study in each case. Additional slides were prepared from paraffin blocks in Cases 1, 2, and 4. Autopsy protocols, microscopic slides and paraffin blocks were utilized in Cases 3 and 4. In each case multiple histologic preparations were examined. We are reasonably confident that the tumors were sufficiently well sampled to ensure accurate interpretation and exclude examples of mixed mesodermal tumor, carcinosarcoma, adenosarcoma, and leiomyosarcoma. In addition to hematoxylin and eosin stains, trichrome and phosphotungstic acid hematoxylin (PTAH) methods were used for evaluation of cytoplasmic myofibrils and cross-striations. Results Case I Clinical Findings: A 22-year-old nulligravid Negro woman experienced repeated eposides of abnormal vaginal bleeding for about 16 months. On pelvic examination, a mass was seen protruding from her cervix. It was thought to be either an "aborting submucosal fibroid" or an endometrial polyp. Endometrial curettage was performed, and during the ensuing 11 months the patient had two additional endometrial curettages. Each time the pathologic interpretation was "endometrial polyps." Four months after the third curettage, pelvic examination revealed a friable mass protruding through the cervical os and extending to the introitus. Curettage of the endometrium was performed again
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Hart, William R., and Craig, John R.: Rhabdomyosarcomas of the uterus. Am J Clin Pathol 70: 217-223, 1978. The authors report the clinicopathologic findings in four cases of adult women with rhabdomyosarcomas that originated in the endometrium or cervix, or both. All tumors were pure sarcomas and not components of carcinosarcomas or malignant mixed mesodermal tumors. In two patients, the tumors were typical botryoid. embryonal rhabdomyosarcomas. Both patients were alive and well following combined treatments with surgery and chemotherapy or irradiation 3.0 and 2.7 years after operation, respectively, although one had a solitary pulmonary metastasis resected. The other two women had pleomorphic rhabdomyosarcomas, and both died rapidly of widespread tumor. Combination therapy rather than surgery alone probably should be used initially for patients with rhabdomyosarcomas of the uterus. (Key words: Rhabdomyosarcoma; Uterine sarcoma; Sarcoma botryoides; Endometrial sarcoma.)
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FIG. 1 (upper, left). Polypoid piece of botryoid embryonal rhabdomyosarcoma in endometrial curetting from Case 1. Several dilated normal endometrial glands are present. Extensive edema obscures the sarcoma cells. Hematoxylin and eosin. x40. FIG. 2 (upper, right). Botryoid embryonal rhabdomyosarcoma (Case 1). Aggregates of primitive small sarcoma cells encircle a normal nonneoplastic endometrial gland. Hematoxylin and eosin. x260. FIG. 3 (lower, left). Botryoid embryonal rhabdomyosarcoma (Case 1). Spindle-shaped sarcoma cells have uniform nuclei and indistinct cytoplasm. Hematoxylin and eosin. x406. FIG. 4 (lower, right). Botryoid embryonal rhabdomyosarcoma (Case 1). Composite photomicrograph shows well-differentiated rhabdomyoblasts of various shapes. Longitudinal and cross striations are barely visible in some cells at right. Hematoxylin and eosin. x406.
RHABDOMYOSARCOMAS infrequently originate in the female genital tract. Most rhabdomyosarcomas are associated with carcinoma and represent heterologous differentiation in a carcinosarcoma or malignant mixed mesodermal tumor. Pure rhabdomyosarcomas of the female genital tract generally occur during infancy or childhood, are of the botryoid embryonal type, and predominantly involve the vagina; in some reports they have been referred to as "sarcoma botryoides." 2 ' 0 - 1 2 1 8 Primary uterine sarcomas composed of rhabdomyosarcoma and not associated with carcinoma are especially rare in adults. 612 In the past such heterologous sarcomas often have been regarded as variants of malignant mixed mesodermal tumors. 1 7 1 8 Modern classifications of uterine sarcomas, however, separate the pure heterologous sarcomas, such as rhabdomyosarcoma, from those with carcinomatous elements, as well as from the pure homologous sarcomas (e.g., leiomyosarcoma, endometrial stromal sarcoma). 612 In this investigation we studied the clinicopathologic features of four cases of adult women who had primary rhabdomyosarcomas of the uterus. All were pure sarcomas. Two tumors were regarded as botryoid embryonal rhabdomyosarcomas and the other two as Received May 12, 1977; received revised manuscript August 1, 1977; accepted for publication August 1, 1977. Address reprint requests to Dr. Hart: Department of Pathology, University of Michigan Medical Center, 1335 E. Catherine St., Ann Arbor, Michigan 48109.
From the Departments of Pathology, The University of Michigan Medical Center, Ann Arbor, Michigan, and the Los Angeles County/University of Southern California Medical Center, Los Angeles, California
pleomorphic rhabdomyosarcomas. Follow-up information was available for each patient. Materials and Methods Cases 1 and 2 were encountered at the Los Angeles County/University of Southern California Medical Center. Cases 3 and 4 were diagnosed as rhabdomyosarcomas during a retrospective review of all patients with primary uterine sarcomas registered at The University of Michigan Medical Center. Our experiences with uterine leiomyosarcomas, endometrial stromal sarcomas and stromatosis are reported separately. 4 5 1 9 Microscopic slides of the primary tumors were available for study in each case. Additional slides were prepared from paraffin blocks in Cases 1, 2, and 4. Autopsy protocols, microscopic slides and paraffin blocks were utilized in Cases 3 and 4. In each case multiple histologic preparations were examined. We are reasonably confident that the tumors were sufficiently well sampled to ensure accurate interpretation and exclude examples of mixed mesodermal tumor, carcinosarcoma, adenosarcoma, and leiomyosarcoma. In addition to hematoxylin and eosin stains, trichrome and phosphotungstic acid hematoxylin (PTAH) methods were used for evaluation of cytoplasmic myofibrils and cross-striations. Results Case I Clinical Findings: A 22-year-old nulligravid Negro woman experienced repeated eposides of abnormal vaginal bleeding for about 16 months. On pelvic examination, a mass was seen protruding from her cervix. It was thought to be either an "aborting submucosal fibroid" or an endometrial polyp. Endometrial curettage was performed, and during the ensuing 11 months the patient had two additional endometrial curettages. Each time the pathologic interpretation was "endometrial polyps." Four months after the third curettage, pelvic examination revealed a friable mass protruding through the cervical os and extending to the introitus. Curettage of the endometrium was performed again
0002-9173/78/0800/0217 $00.85 © American Society of Clinical Pathologists
217
Downloaded from http://ajcp.oxfordjournals.org/ by guest on June 5, 2016
Hart, William R., and Craig, John R.: Rhabdomyosarcomas of the uterus. Am J Clin Pathol 70: 217-223, 1978. The authors report the clinicopathologic findings in four cases of adult women with rhabdomyosarcomas that originated in the endometrium or cervix, or both. All tumors were pure sarcomas and not components of carcinosarcomas or malignant mixed mesodermal tumors. In two patients, the tumors were typical botryoid. embryonal rhabdomyosarcomas. Both patients were alive and well following combined treatments with surgery and chemotherapy or irradiation 3.0 and 2.7 years after operation, respectively, although one had a solitary pulmonary metastasis resected. The other two women had pleomorphic rhabdomyosarcomas, and both died rapidly of widespread tumor. Combination therapy rather than surgery alone probably should be used initially for patients with rhabdomyosarcomas of the uterus. (Key words: Rhabdomyosarcoma; Uterine sarcoma; Sarcoma botryoides; Endometrial sarcoma.)
y,
j
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vol. 70. No. 2
UTERINE RHABDOMYOSARCOMAS
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FIG. 1 (upper, left). Polypoid piece of botryoid embryonal rhabdomyosarcoma in endometrial curetting from Case 1. Several dilated normal endometrial glands are present. Extensive edema obscures the sarcoma cells. Hematoxylin and eosin. x40. FIG. 2 (upper, right). Botryoid embryonal rhabdomyosarcoma (Case 1). Aggregates of primitive small sarcoma cells encircle a normal nonneoplastic endometrial gland. Hematoxylin and eosin. x260. FIG. 3 (lower, left). Botryoid embryonal rhabdomyosarcoma (Case 1). Spindle-shaped sarcoma cells have uniform nuclei and indistinct cytoplasm. Hematoxylin and eosin. x406. FIG. 4 (lower, right). Botryoid embryonal rhabdomyosarcoma (Case 1). Composite photomicrograph shows well-differentiated rhabdomyoblasts of various shapes. Longitudinal and cross striations are barely visible in some cells at right. Hematoxylin and eosin. x406.
