GEIS-SEHOP clinical practice guidelines for the treatment of rhabdomyosarcoma

Rhabdomyosarcoma of the kidney: A case report

“…Targeted radiotherapy may reduce radiation-related complications, such as external proton radiotherapy or brachytherapy. 4 Systemic chemotherapy is the basis of multimodal treatment for RMS patient, 5 with vincristine, actinomycin, and alkylating agents (ifosfamide or cyclophosphamide) as the main drugs. For very high-risk RMS patients, such as those in disease remission, maintenance chemotherapy is also recommended after the end of consolidation chemotherapy.…”

Radioresistance in rhabdomyosarcomas: Much more than a question of dose

“…At the onset, hematuria and urinary obstruction represent the signs and symptoms more frequent, whereas 10%–20% of pediatric and 40% of adult patients present distant metastases. RMS diagnosis requires, in addition to standard laboratory (complete blood counts, electrolytes, renal function tests, liver function tests, and urinalysis), the direct evaluation of tumor tissue derived from either an incisional/excisional biopsy or a core needle biopsy ( 1 ) magnetic resonance imaging, for local staging, and computed tomography (CT) and [F-18]2-fluoro-2-deoxyglucose positron emission tomography (18F-FDG PET)/CT), for systemic staging and the risk stratification ( 14 , 15 ). TNM staging is based on the anatomic location and invasiveness of the primary tumor, tumor size, nodal status, and extent of metastasis ( Tables 1 , 2 ).…”

“…Treatment of locally and locally advanced RMS is mainly based on surgery ( 14 , 15 ), although, aggressive surgery, often necessary to achieve tumor debulking and negative microscopic margins, is no longer recommended ( 19 ). This is particularly true for genitourinary RMS, to avoid significant long-term morbidities such as urinary diversion, infertility, and sexual dysfunction particularly.…”

“…This is particularly true for genitourinary RMS, to avoid significant long-term morbidities such as urinary diversion, infertility, and sexual dysfunction particularly. Therefore, except for paratesticular tumors ( 20 ), the standard care of RMS, genitourinary and non-genitourinary, usually provides neoadjuvant chemotherapy (CHT) followed by RT or concomitant CHT/RT followed or not by excision ( 14 , 15 ). In 20% of patients with genitourinary RMS, a close follow-up with imaging is a reasonable alternative to aggressive surgery ( 14 , 15 ).…”

“…Therefore, except for paratesticular tumors ( 20 ), the standard care of RMS, genitourinary and non-genitourinary, usually provides neoadjuvant chemotherapy (CHT) followed by RT or concomitant CHT/RT followed or not by excision ( 14 , 15 ). In 20% of patients with genitourinary RMS, a close follow-up with imaging is a reasonable alternative to aggressive surgery ( 14 , 15 ). In support of a delayed surgery, it has been shown that, despite RMS can persist after a neoadjuvant approach ( 21 , 22 ), the rhabdomyoblasts found in subsequent biopsies progressively decrease and their presence do not necessarily predict local recurrence ( 23 , 24 ).…”