Extraosseous Ewing’s sarcoma of gynaecological origin: primitive neuroectodermal tumour of infrequent location. Case report and review of the literature

Authors

  • Claudia Isabel Vivas-Flores
  • Manuel Pantoja-Garrido
  • Zoraida Frías-Sánchez

DOI:

https://doi.org/10.18597/rcog.3023

Keywords:

Ewing’s sarcoma, primitive peripheral neuroectodermal tumours, neoplasm, neuroepithelioma

Abstract

Objective: To present the case of a female patient diagnosed with cervico-vaginal extraosseous Ewing’s sarcoma and to conduct a review of the literature, with special emphasis on histopathological diagnosis and therapeutic options for these neoplastic lesions. 

Materials and methods: Woman of a 53 year-old on follow-up due to a history of infiltrating ductal breast cancer, who presents with abnormal genital bleeding. On physical examination, a cervicovaginal mass extending to the pelvis was observed. A biopsy was taken and the result was consistent with Ewing’s sarcoma/primitive neuroectodermal tumour of the cervix. A search was conducted in the Medeline databases via PubMed, using the terms “sarcoma; ewing”, “neuroectodermal tumours”, “primitive”, “peripheral” and “uterine cervical neoplasms”, looking for reviews of the literature, case reports and clinical case series both in English and Spanish, published since 2009.

Results: Overall, 12 articles were found, including reviews of the literature, case reports and clinical case series, describing the most relevant aspects of the treatment, clinical manifestations and molecular, immunohistochemical and histopathological diagnosis of this disease.

Conclusions: Gynaecological extraosseous Ewing’s tumour is an extremely rare lesion whose diagnosis is based on the study of the histopathological morphology, immunohistochemical tests and genetic testing. A co mbination of surgery, chemotherapy and radiotherapy is considered the treatment of choice at the present time.

Author Biographies

Claudia Isabel Vivas-Flores

Médico especialista, Unidad de Gestión Clínica de Obstetricia y Ginecología, Hospital Universitario de Jerez de la Frontera, Cádiz (España).

Manuel Pantoja-Garrido

Médico especialista, Unidad de Gestión Clínica de Obstetricia y Ginecología, Hospital Universitario de Jerez de la Frontera, Cádiz (España). pantoja_manuel@hotmail.com

Zoraida Frías-Sánchez

Médico especialista, Unidad de Gestión Clínica de Obstetricia y Ginecología, Hospital Universitario Virgen Macarena, Sevilla (España).

References

Kelleher FC, Thomas DM. Molecular pathogenesis and targeted therapeutics in Ewing sarcoma/primitive neuroectodermal tumours. Clin Sarcoma Res. 2012;2:6. doi: 10.1186/2045-3329-2-6.

Li B, Ouyang L, Han X, Zhou Y, Tong X, Zhang S, et al. Primary primitive neuroectodermal tumor of the cervix. Onco Targets Ther. 2013;6:707-11. doi: 10.2147/OTT.S45889.

Khosla D, Patel FD, Kumar R. Sarcomas of the uterine cervix: A united and multidisciplinary approach is required. Women’s Health (Lond). 2013;9:501-4. doi: 10.2217/whe.13.59.

Guzmán Valdivia-Gómez G, Soto-Guerrero MT, Cedi¬llo-De la Cruz MI. Sarcoma de Ewing extraesquelético que semeja abdomen agudo. Cir Cir. 2010;78:163-6.

Yip CM, Hsu SS, Chang NJ, Wang JS, Liao WC, Chen JY, et al. Primary vaginal extraosseous Ewing sarcoma/ primitive neuroectodermal tumor with cranial metastasis. J Chin Med Assoc. 2009;72:332-5. doi: 10.1016/S1726-4901(09)70381-8.

Huamán-Guerrero M, Jauregui-Francia F, Bedoya- Vidal S, Espinoza-Bobadilla L, Guisado-Aguayo C, Kaway-Caceda H, et al. Tumor neuroectodérmico primitivo pélvico: a propósito de un caso y revisión de literatura. Revista de la Facultad de Medicina Humana de la Universidad Ricardo Palma. 2013;(1):33-5.

Mashriqi N, Gujjarlapudi JK, Sidhu J, Zur M, Yalamanchili M. Ewing’s sarcoma of the cervix, a diagnostic dilemma: A case report and review of the literature. J Med Case Rep. 2015;9:255. doi: 10.1186/ s13256-015-0733-2.

Yousefi Z, Sharifhi N, Hasanzadeh M, Mottaghi M, Bolandy S. Peripheral Primitive Neuroectodermal Tumor of the Pelvis. Iran J Med Sci. 2014;39:71-4.

Weissferdt A, Kalhor N, Moran CA. Ewing sarcoma with extensive neural differentiation a clinicopathologic, immunohistochemical, and molecular analysis of three cases. Am J Clin Pathol May. 2015;143:659-64. doi: 10.1309/AJCPFHFX07JFCRGU.

Loverro G, Resta L, Di Naro E, Caringella AM, Mastrolia SA, Vicino M, et al. Conservative Treatment of Ewing’s sarcoma of the uterus in young women. Case Rep Obstet Gynecol. 2015; 2015: 871821. doi: 10.1155/2015/871821.

Rekhi B, Qureshi S, Basak R, Desai SB, Medhi S, Kurkure P, et al. Primary vaginal Ewing’s sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report. J Med Case Reports. 2010;4:88. doi: 10.1186/1752-1947-4-88.

Dutta S, Dasgupta C, Choudhury K. Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor. Indian J Med Paediatr Oncol. 2011;32:171-3.

How to Cite

1.
Vivas-Flores CI, Pantoja-Garrido M, Frías-Sánchez Z. Extraosseous Ewing’s sarcoma of gynaecological origin: primitive neuroectodermal tumour of infrequent location. Case report and review of the literature. Rev. colomb. obstet. ginecol. [Internet]. 2017 Jun. 29 [cited 2024 May 17];68(2):135-41. Available from: https://revista.fecolsog.org/index.php/rcog/article/view/3023

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Published

2017-06-29

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Case Report
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