Background And Aim: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy.
Methods: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports.
Results: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient #3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had free margins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months.
Conclusion: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management.
Level Of Evidence: IV (Treatment Study, Case Series with no Comparison Group).
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http://dx.doi.org/10.1016/j.jpedsurg.2018.10.090 | DOI Listing |
Front Med (Lausanne)
December 2024
Department of Nuclear Medicine, Peking University First Hospital, Beijing, China.
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.
View Article and Find Full Text PDFClin Transl Radiat Oncol
November 2024
Department of Pediatric Surgery and Pediatric Urology, University Children's Hospital, Eberhard Karls University Tuebingen, 72076 Tuebingen, Germany.
Clin Case Rep
September 2024
Department of Surgery East Carolina University, Brody School of Medicine Greenville North Carolina USA.
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.
View Article and Find Full Text PDFPediatr Blood Cancer
November 2024
Division of Pediatric Surgery, University of Kentucky Medical College, Lexington, Kentucky, USA.
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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