Rhabdomyosarcoma with heterologous cartilage of the uterine cervix: A clinicopathologic and immunohistochemical study of an aggressive neoplasm in a young female

Perrone, Theresa; Carson, Linda F.; Dehner, Louis P.

doi:10.1002/mpo.2950180116

Cited by 27 publications

(18 citation statements)

References 20 publications

(22 reference statements)

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“…11–13 This was true in 12 of 14 cases in this study, where these neoplasms grew into the vaginal vault from the cervix and had microscopically evident cambium layers of malignant cells underlying the surface epithelium. The other two cases (Cases 8 and 14) presented as infiltrative masses rather than polyps and had complex patterns with focal areas of embryonal rhabdomyosarcoma with a periglandular orientation of small primitive rhabdomyoblasts, and additionally other patterns of spindle cell rhabdomyosarcoma and anaplasia.…”

Section: Discussionmentioning

confidence: 58%

Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations

2012

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Embryonal rhabdomyosarcoma of the uterine cervix is an uncommon presentation of the most common soft-tissue sarcoma in the first decades of life. Unlike embryonal rhabdomyosarcoma in other anatomic sites, in which 70–80% of cases present before 9 years of age, the average age in our series of 14 cervical cases was 12.4 years (median, 13 years), with an age range of 9 months to 32 years at diagnosis. Of the 14 cases, 12 presented as a polyp at the cervical os; two patients had an infiltrative mass in the cervix without a botryoid polyp. The polyps measured 1.5–5 cm and all had the histopathological pattern of the sarcoma botryoides variant of embryonal rhabdomyosarcoma, with condensations of primitive and differentiated rhabdomyoblasts beneath the surface epithelium and around endocervical glands. Nodules of benign-appearing cartilage were present in the stroma of six cases (43%). One of the embyronal rhabdomyosarcomas from the youngest patient, 9 months old, also had a distinctive microscopic focus of immature tubular profiles in a primitive stroma; these tubules expressed epithelial and neuroendocrine markers. Two patients had a pleuropulmonary blastoma, one diagnosed 9 years before the embryonal rhabdomyosarcoma of the cervix and the other recognized synchronously. This latter 9-year old had a DICER1 germline mutation. One patient presented with hirsutism and had a Sertoli–Leydig cell tumor, an incidentally detected cervical embryonal rhabdomyosarcoma, and nodular hyperplasia of the thyroid. Although a pleuropulmonary blastoma was not documented in the latter patient, ovarian sex-cord stromal tumors and nodular hyperplasia of the thyroid are manifestations of the pleuropulmonary blastoma family tumor and dysplasia syndrome (OMIM 601200). Embryonal rhabdomyosarcoma of the cervix must be distinguished from other rare entities, including adenosarcoma, malignant mixed Mullerian tumor and low-grade stromal sarcoma, as the former has a better prognosis; 12 of our 14 patients remain disease-free following conservative surgery and chemotherapy. Our study suggests that cervical embryonal rhabdomyosarcoma may be another pathological manifestation in the spectrum of extrapulmonary pathology in the setting of pleuropulmonary blastoma.

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Section: Discussionmentioning

confidence: 58%

Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations

2012

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“…5 Overall, the botryoides type of RMS has a rather good prognosis with surgical and chemotherapeutic treatment; however, deep myometrial invasion, lymphatic invasion, and focal alveolar pattern are histopathologic findings associated with a poorer prognosis. 6,7 The patient in case 1 had invasion of the inner half of the myometrium, with no lymphatic or vascular invasion and adjuvant chemotherapy that portends a better overall survival. 8 Review of early literature indicated that original treatment was solely aggressive surgical resection for all embryonal RMS located at genitourinary sites.…”

Section: Discussionmentioning

confidence: 99%

Embryonal Rhabdomyosarcoma

Ballard

Tedjarati²,

Robinson³

et al. 2010

International Journal of Gynecological Cancer

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The aim of this retrospective study was to evaluate differences in treatment of embryonal rhabdomyosarcoma (RMS) of the uterus in 2 premenopausal women. We discuss adjuvant chemotherapy and use of ChemoFx Assay (Precision Therapeutics, Pittsburgh, PA) to guide choice of active chemotherapeutic agents. Two premenopausal patients were identified with a pathologic diagnosis of embryonal RMS of the uterus. Both met inclusion criteria for the study. A 21-year-old woman underwent a staging abdominal hysterectomy for a variant of embryonal RMS. Vincristine, actinomycin D, and cyclophosphamide were given adjunctively for a complete response. A 20-year-old woman underwent a diagnostic dilation and curettage revealing embryonal RMS. Initial treatment included an abdominal hysterectomy and nodal sampling. Presentation to a subsequent gynecologic oncologist 7 months later revealed recurrence. Carboplatin, doxorubicin, and paclitaxel provided a partial response. After a second surgical resection, ChemoFx Assay identified ifosfamide and mitomycin C as active agents and resulted in a complete response. Recommended treatment includes surgery and chemotherapy with possible radiation therapy if deemed necessary. The benefit of adding neoadjuvant or adjuvant chemotherapy and radiation therapy allows for a conservative surgical approach and improved survival. Choosing active chemotherapy agents can be aided by ChemoFx Assay. The chemotherapy most commonly used for treatment of embryonal RMS is a combination of vincristine, actinomycin D, and cyclophosphamide.

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“…The prognosis of patients with cervical sarcoma botryoides is rather favourable when compared to other genital rhabdomyosarcomas, particularly when the tumour arises in a single polypoid lesion and when the polyp is completely removed or resected (Perrone et al, 1990;Zeisler et al, 1998). The surgical aggressiveness for the management of this tumour has progressively decreased from pelvic exenteration (often used until the 1970s) to abdominal hysterectomy.…”

Section: Discussionmentioning

confidence: 99%

“…Some authors (Perrone et al, 1990) described a woman with local and distant recurrence after radical hysterectomy for a sarcoma botryoides with deep infiltration into the cervical stroma. Since one of our patients had local recurrence despite receiving chemotherapy, the effectiveness of this chemotherapy regimen in sterilizing distant micrometastases remains debatable.…”

Section: Discussionmentioning

confidence: 99%

Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females

et al. 1999

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Summary Sarcoma botryoides of the cervix is an extremely rare tumour and seems to be associated with a better prognosis than its vaginal counterpart. Recent studies have suggested that it is possible to limit surgery to local excision in stage I cases. We report three cases of young subjects treated successfully with polypectomy or diathermy loop excision followed by adjuvant chemotherapy. One patient had a local recurrence which was treated with further local excision. All subjects remain alive without evidence of recurrence and with normal menstrual function 36, 38 and 38 months following initial diagnosis. A conservative surgical approach to early cervical sarcoma botryoides is possible. The efficacy of adjuvant chemotherapy and the regimen of choice still need to be investigated.

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Rhabdomyosarcoma with heterologous cartilage of the uterine cervix: A clinicopathologic and immunohistochemical study of an aggressive neoplasm in a young female

Cited by 27 publications

References 20 publications

Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations

Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations

Embryonal Rhabdomyosarcoma

Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females

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