Alveolar Rhabdomyosarcoma of the Uterine Cervix. A Case Report Confirmed by FKHR Break-Apart Rearrangement Using a Fluorescence In Situ Hybridization Probe on Paraffin-Embedded Tissues

Rivasi, Francesco; Botticelli, Laura; Bettelli, Stefania; Masellis, Giuseppe

doi:10.1097/pgp.0b013e31816085ce

Cited by 18 publications

(8 citation statements)

References 11 publications

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“…Patients are usually postmenopausal or middle aged. Alveolar rhabdomyosarcoma is extremely rare and to the best of my knowledge, only two convincing cases of the uterine corpus 3,9 and three of the uterine cervix 4,7,10 have been described. Many of the older reported cases of rhabdomyosarcoma did not have confirmatory immunohistochemistry and may not have been adequately sampled.…”

Section: Discussionmentioning

confidence: 98%

“…The present case had a very aggressive clinical course. Three cases of alveolar rhabdomyosarcoma in the uterine cervix have been previously described 4,7,10 . One patient who underwent postoperative radiotherapy died of metastatic disease 3 months after surgery 4 .…”

Section: Discussionmentioning

confidence: 99%

“…One patient who underwent postoperative radiotherapy died of metastatic disease 3 months after surgery 4 . One patient remained disease free 18 months after surgery without chemotherapy 7 . The remaining patient who had postoperative chemotherapy and irradiation was disease free 3 years postoperatively 10 .…”

Section: Discussionmentioning

confidence: 99%

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Pure alveolar rhabdomyosarcoma of the uterine corpus

Fukunaga¹

2011

Pathology International

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Herein is presented a very rare case of alveolar rhabdomyosarcoma in the uterine corpus of a 72-year-old woman. The wall of the uterine corpus was replaced by multiple whitish-yellow, friable nodules, measuring up to 6 cm. Microscopically, the tumor was predominantly composed of round to polygonal cells arranged in an alveolar, papillary or nest pattern intermingled with multinuclear giant cells with abundant eosinophilic cytoplasm. Extensive sampling failed to show epithelial elements. Immunohistochemically, the tumor was positive for striated muscle markers such as myoglobin, myoD1 and myogenin. Metastatic lesions were found in the retroperitoneum and pelvic lymph nodes. The patient was treated by postoperative chemotherapy, but she died of systemic metastases 12 months after surgery.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

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Pure alveolar rhabdomyosarcoma of the uterine corpus

Fukunaga¹

2011

Pathology International

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“…Alveolar rhabdomyosarcoma is most commonly seen and consists of malignant cells arranged in an alveolar pattern surrounded by fibrous tissue with vascular tissue. The tumor cells contain large hyperchromatic nuclei and can be large polygonal or small spindle shaped (Rivasi et al 2008). Cytogenetic abnormalities t(2;13) (q35;q14), resulting in the fusion genes PAX3-FKHR and the less frequent t(1;13)(p36;q14), with fusion of PAX7-FKHR, are seen in majority of the cases.…”

Section: Mesenchymal Tumorsmentioning

confidence: 99%

Lesions of the Uterine Cervix

Walia¹,

Mhawech‐Fauceglia²

2016

Handbook of Gynecology

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“…Alveolar rhabdomyosarcomas are extraordinarily rare, [74][75][76][77] and probably display the same morphologic and cytogenetic attributes as alveolar rhabdomyosarcomas at other locations.…”

Section: Alveolar Rhabdomyosarcomasmentioning

confidence: 99%

Heterologous and Rare Homologous Sarcomas of the Uterine Corpus

Fadare¹

2011

Advances in Anatomic Pathology

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Pure sarcomas of the uterine corpus are uncommon, constituting less than 3% of all malignancies at this site, and most of them are leiomyosarcomas and endometrial stromal sarcomas. Rare histotypes of homologous sarcomas and heterologous sarcomas are occasionally encountered, and the absence of significant accumulated experience with these histotypes at this location may potentially raise diagnostic and patient management difficulties. In this article, the clinicopathologic attributes of all earlier reported sarcomas of the uterine corpus other than leiomyosarcomas and endometrial stromal sarcomas are summarized. Included are embryonal rhabdomyosarcoma, pleomorphic rhabdomyosarcoma, angiosarcoma, alveolar soft part sarcoma, malignant perivascular epithelioid cell tumors (PEComas), osteosarcoma, chondrosarcoma, liposarcomatous tumors, malignant extrarenal rhabdoid tumors, Ewing sarcoma/primitive neuroectodermal tumor, and other rare histotypes. Embryonal rhabdomyosarcoma (20%), Ewing sarcoma/primitive neuroectodermal tumor (17%), angiosarcoma (14%), and pleomorphic rhabdomyosarcoma (13%) appeared to be more common than the others, although there was no single overwhelmingly prevalent histotype in the group. A subset, including embryonal rhabdomyosarcoma, alveolar soft part sarcoma, and PEComas, peak in the premenopausal years, but most of the others were observed in postmenopausal women. Favorable outcomes have been reported for the patients diagnosed with alveolar soft part sarcoma, and the prognosis for their counterparts with PEComa remains a matter of debate. Multimodal therapeutic approaches to contemporary patients with embryonal rhabdomyosarcomas have resulted in significantly improved outcomes. Unfortunately, most of the other sarcomas have been associated with rapid tumor progression and unfavorable patient outcomes. The differential diagnosis for these sarcomas is often extensive and varies by histotype, but their accurate diagnosis fundamentally requires the careful exclusion of biphasic malignancies.

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Alveolar Rhabdomyosarcoma of the Uterine Cervix. A Case Report Confirmed by FKHR Break-Apart Rearrangement Using a Fluorescence In Situ Hybridization Probe on Paraffin-Embedded Tissues

Cited by 18 publications

References 11 publications

Pure alveolar rhabdomyosarcoma of the uterine corpus

Pure alveolar rhabdomyosarcoma of the uterine corpus

Lesions of the Uterine Cervix

Heterologous and Rare Homologous Sarcomas of the Uterine Corpus

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