Conservative management of a perianal rhabdomyosarcoma in a 2-year old child by Papillon’s technique

Abstract

Rhabdomyosarcoma (RMS) is the most common sarcoma in paediatric patients. A perianal site is unusual and is associated with a low cure rate. The few cases of reported perianal RMS have been associated with sequelae. Here, we report the case of a 29-month-old male child who received sequential treatment by surgery, chemotherapy and radiotherapy inspired by Papillon’s irradiation of adult anal/low-rectum cancers (including external beam radiotherapy in the gynecological exam position followed by brachytherapy) and who remains in complete remission 49 months post treatment with no sphincter or other anorectal disorders.

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation: A series of three consecutive children

“…Anus saving surgery was considered in all patients. Brachytherapy was considered but abandoned because all three still had a substantial infiltrating residual tumor and because experience from brachytherapy in PRMS in children was sparse [6,7]. The decision for abdominoperineal excision and permanent colostomy was eventually made in agreement with all three patients and their caregivers as it was felt that anus saving surgery would carry also a high risk of tumor recurrence and anal incontinence.…”

“…Conservative surgery with brachytherapy or with proton beam radiation has also been used in perineal and PRMS but due to the rarity of the disease the number of reported cases is low. Fuchs et al (2016) [6] reported two patients and Demoor-Goldschmidt et al (2015) [7] one patient with PRMS treated with combined surgery and brachytherapy. The experience with the proton beam therapy is also limited to a few patients [16,17].…”

“…A combination of conservative surgery and brachytherapy may provide better chances of organ saving surgery without compromising survival or recurrence [7]. Successful management of anal and distal rectal cancer with brachytherapy with or without conservative surgery has already been reported [13].…”

“…Treatment options have included chemotherapy, conservative or radical excision of the tumor and local radiotherapy. In selected patients a combination of brachytherapy in the treatment protocol may improve the chances for non-mutilating surgery [6][7][8][9].…”

Pediatric perianal rhabdomyosarcoma: Multimodal therapy for tumor control

“…Primary perineal and perianal RMS (PRMS) comprise only 2% of all RMS. 3,4 PRMS are often misdiagnosed as gluteal or perirectal abscess causing a delay in the diagnosis resulting in locoregional or distant metastasis at presentation. The importance of delayed diagnosis leading to unfavorable outcomes in PRMS patients has been emphasized by many authors.…”