The case of a child with a stage I perianal embryonal botryoid rhabdomyosarcoma is presented, with description of coordinated management utilizing local excision, irradiation, and cyclic chemotherapy resulting in a 3-year tumor-free survival. Previously reported pediatric cases are summarized.

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http://dx.doi.org/10.1002/1097-0142(197608)38:2<1008::aid-cncr2820380253>3.0.co;2-iDOI Listing

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Article Synopsis
  • Rhabdomyosarcoma (RMS) is a rare and aggressive pediatric cancer that commonly affects children and adolescents, with alveolar rhabdomyosarcoma (ARMS) often developing in areas like the trunk and head/neck.
  • An 11-year-old girl diagnosed with ARMS presented with a perianal mass, showing aggressive metastasis to the pleura, lymph nodes, and even bones, which is less common.
  • The case underscores the difficulties in diagnosing and treating ARMS, stressing the importance of early detection, advanced imaging techniques, and the need for ongoing research to develop better treatment options.
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The Other Site of Rhabdomyosarcoma.

Cancer Med

October 2024

Department of Pediatric Hematology and Oncology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.

Background: Rhabdomyosarcoma (RMS) is a rare malignant soft tissue sarcoma (STS), accounting for almost 50% of pediatric STSs. Due to its heterogeneity, RMS presents challenges in diagnosis and treatment, with prognosis varying depending on multiple factors. Tumors localized in the other site (OTH)-including the paraspinal, perianal, thoracic, abdominal, pelvic, and perineal regions-are generally classified as unfavorable.

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Article Synopsis
  • Perianal rhabdomyosarcoma (P)RMS is a rare cancer in young children with a generally poor prognosis, and there's limited data on effective treatments.
  • This study reviewed treatment outcomes for 6 children who received surgery and high-dose rate (HDR) brachytherapy since 2009, with the majority achieving successful organ preservation and survival.
  • The findings suggest that combining surgery and HDR brachytherapy is a feasible treatment option that can improve survival rates and maintain organ function in young patients with (P)RMS.
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Perianal alveolar rhabdomyosarcoma is a rare sarcoma that requires a high index of suspicion along with tissue biopsy for accurate diagnosis. Successful treatment, even in the setting of recurrence, requires a multidisciplinary approach.

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Purpose: To describe clinical features, risk factors, and outcomes of patients with perineal and perianal rhabdomyosarcoma.

Methods: The records of 51 patients (38 perineal and 13 perianal) enrolled on Children's Oncology Group clinical trials between 1997 and 2012 were reviewed.

Results: At presentation, 53% were female, 65% were older than 10 years of age, 76% were alveolar histology, 76% were more than 5 cm, 84% were invasive, 65% were regional node positive by imaging, 49% were metastatic, only 16% were grossly resected upfront, and 25% of patients had a delayed excision.

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