Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2019.104
Autopsy and Case Reports
Article / Clinical Case Report

Uterine cavity embryonal rhabdomyosarcoma

Marina Gomes Pereira Sardinha; Fábio Morozetti Ramajo; Cesar Cilento Ponce; Camila Franzin Marques; Carolina Marques Fontes Bittencourt; Fernando Gardin Caldano; José Matheus Frizzo Lopes Moço; Otávio de Lacquila Yano; Pedro Marques da Rosa Reis; Vinicius Silva Malaguti; Catarina Tealdi Reno Gandra de Sousa; Roberto Cesar Nogueira Junior

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Abstract

Rhabdomyosarcoma (RMS) is a rare solid tumor in childhood and adolescence. The higher incidence is predominant during the first two decades of life. According to the Intergroup RMS Study Group, the embryonal RMS (ERMS), botryoidal variant, constitutes a histological subtype characterized as a “grape-like” lesion of 2.0 cm to 9.5 cm. The treatment involves chemotherapy, surgery, and/or radiotherapy. We present the case of a 14-year-old female patient diagnosed with ERMS, botryoidal variant, which originated in the uterine cervix with vaginal externalization. The initial therapeutic approach comprised an initial prolapsed mass excision followed by Wertheim–Meigs surgery due to the tumor extension. No consensual protocol to ERMS treatment is found in the medical literature; however, a combined approach seems to offer a better result. The postoperative time period was uneventful and the patient followed an adjuvant therapy with vincristine, d-actinomycin, and cyclophosphamide. A comprehensive evaluation of the therapeutic options preserving the reproductive function—unfortunately not always possible—is part of a multi-disciplined care team concerning the pediatric patients.

Keywords

Rhabdomyosarcoma, Embryonal, Uterine Cervical Neoplasms, Cervix Uteri

References

Raney RB, Maurer HM, Anderson JR, et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): major lessons from the IRS-I through IRS-IV studies as background for the current IRS-V treatment protocols. Sarcoma. 2001;5(1):9-15. 10.1080/13577140120048890.18521303

Chintagumpala MM. Soft-tissue sarcomas. In: McMillan JA, Feigin RD, DeAngelis CD, Jones MD Jr, editors. Oski’s pediatrics: principles and practice. 4th ed. Philadelphia: Williams & Wilkins; 2006. p. 1781-6.

Maurer HM. The Intergroup Rhabdomyosarcoma study: update. Natl Cancer Inst Monogr. 1981;56(56):61-8.7029297

Semczuk A, Baranowski W, Berbeć H, Marzec B, Skomra D, Miturski R. Analysis of p53 and K-ras genes and their proteins in a sarcoma botryoides of the uterine cervix. Eur J Gynaecol Oncol. 1999;20(4):311-4.10475130

Behtash N, Mousavi A, Tehranian A, Khanafshar N, Hanjani P. Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature. Gynecol Oncol. 2003;91(2):452-5. 10.1016/S0090-8258(03)00539-0.14599884

Parham DM. Pathologic classification of rhabdomyosarcomas and correlations with molecular studies. Mod Pathol. 2001;14(5):506-14. 10.1038/modpathol.3880339.11353062

Zeisler H, Mayerhofer K, Joura EA, et al. Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature. Gynecol Oncol. 1998;69(1):78-83. 10.1006/gyno.1998.4962.9571003

Gonzalez-Crussi F. Atlas of tumor pathology. 2nd ed. Washington: Armed Forces Institute of Pathology; 1982. Extragonadal teratomas.

Arndt CA, Donaldson SS, Anderson JR, et al. What constitutes optimal therapy for patients with rhabdomyosarcoma of the female genital tract? Cancer. 2001;91(12):2454-68. 10.1002/1097-0142(20010615)91:12<2454::AID-CNCR1281>3.0.CO;2-C.11413538

Kayton ML, Wexler LH, Lewin SN, Park KJ, La Quaglia MP, Abu-Rustum NR. Pediatric radical abdominal trachelectomy for anaplastic embryonal rhabdomyosarcoma of the uterine cervix: an alternative to radical hysterectomy. J Pediatr Surg. 2009;44(4):862-7. 10.1016/j.jpedsurg.2008.11.038.19361657

Daya DA, Scully RE. Sarcoma botryoides of the uterine cervix in young women: a clinicopathological study of 13 cases. Gynecol Oncol. 1988;29(3):290-304. 10.1016/0090-8258(88)90228-4.3278956

Stankovic ZB, Djuricić S, Stanković DS, Zdravković S, Gazikalović S, Sedlecki K. Minimal invasive treatment of cervical rhabdomyosarcoma in an adolescent girl. J BUON. 2007;12(1):121-3.17436413

Mousavi A, Akhavan S. Sarcoma botryoides (embryonal rhabdomyosarcoma) of the uterine cervix in sisters. J Gynecol Oncol. 2010;21(4):273-5. 10.3802/jgo.2010.21.4.273.21278891

Dehner LP, Jarzembowski JA, Hill DA. Embryonal rhabdomyosarcoma of the uterine cervix: a report of 14 cases and a discussion of its unusual clinicopathological associations. Mod Pathol. 2012;25(4):602-14. 10.1038/modpathol.2011.185.22157934

Goldblum JR, Lamps LW, McKenney JK, Myers JL. Rosai & Ackerman’s surgical pathology. 9th ed. St. Louis: Elsevier; 2004. Volume 2; p. 2302-3.

Barros DG, Barbosa MS, Carneiro MAGQ, et al. Rabdomiossarcoma embrionário uterino em paciente adulta jovem: um relato de caso. Rev. Saúde HSI. 2018;(2):28-30.

Khosla D, Gupta R, Srinivasan R, Patel FD, Rajwanshi A. Sarcomas of uterine cervix: clinicopathological features, treatment, and outcome. Int J Gynecol Cancer. 2012;22(6):1026-30. 10.1097/IGC.0b013e31825a97f6.22740005

Zanetta G, Rota SM, Lissoni A, Bratina G, Mangioni C. Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females. Br J Cancer. 1999;80(3-4):403-6. 10.1038/sj.bjc.6690370.10408845

 

 

 


Publication date:
07/12/2019

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