Rhabdomyosarcoma of the cervix in teenagers – Is fertility preservation a feasible option?

Abstract

Highlights Embryonal rhabdomyosarcoma (RMS) of the cervix. Subtypes of embryonal RMS. Disease of adolescence. Fertility preservation. Lack of standard treatment.

Embryonal rhabdomyosarcoma of the uterine cervix in an adult female

“…The current WHO classification subclassifies RMS into embryonal, alveolar, spindle cell/sclerosing, and pleomorphic variants and does not separate the botryoid subtype. 2,6,7 In recent years, anaplastic or pleomorphic and embryonal RMS have emerged as the two main subtypes of RMS based on the microscopic features of the cells distributed around a central area or cells resembling immature skeletal myoblasts. This distinction is supported by the recognition that the anaplastic variant is often associated with balanced chromosomal translocations involving chromosomes 2 or 1 and 13 (referred to here as t(2;13) and t(1;13)), originally detected by cytogenetics.…”

“…9 Embryonal RMS is the most common type and accounts for 60% of all rhabdomyosarcomas. 7 It is a malicious variant of RMS and arises from the embryonal mesenchyme. It accounts for 4-6% of all childhood malignancies.…”

“…It accounts for 4-6% of all childhood malignancies. 7 It is further categorized into sarcoma botryoides and anaplastic variants. The anaplastic variant is an uncommon variant of embryonal RMS and comprises roughly 8-27% of all embryonal RMS.…”

“…The primary principle for surgical management of cervical embryonal RMS is complete resection of the primary tumor with the surrounding margin of normal tissue. 7 The spectrum of surgical therapy now includes radical hysterectomy with or without lymphadenectomy, vaginectomy, cervicectomy, polypectomy, local excisions, and diathermy loop excisions. Embryonal RMS of the cervix are mostly either treated with surgery alone or with adjuvant chemotherapy and/or radiotherapy.…”

“…48 The literature indicates regional lymph node removal as an option in patients presenting with low-risk disease manifestations but a recommended compulsion in patients with high-risk disease. 7 Additionally, RMS is also known to be a chemosensitive tumor. Adjuvant chemotherapy targets micro lymphatic metastases if any.…”