Alveolar rhabdomyosarcoma in an adolescent male patient - case report and current perspectives.

ABSTRACT

Alveolar rhabdomyosarcoma (RMS) is a common pediatric malignant mesenchymal tumor, representing half of soft tissue sarcomas and approximately 5% of all cancers. We present the case of an adolescent male patient treated in our Department for a tumoral mass located in the middle third of the forearm. Magnetic resonance imaging (MRI) and angiography-computed tomography (angio-CT) showed a large mass located in the muscles of the anterior compartment of the forearm. Surgical treatment consisted of tumor ablation including segmental resection of the radial and ulnar arteries and of the median nerve, followed by saphenous autograft vascular bypass. The treatment plan was based on tumor type, histological grading (high), age, tumor size greater than 5 cm, unfavorable location, postoperative tumor, node, metastasis (TNM) stage II, presence of microscopic tumoral tissue in the margins of the resected piece, lymph node metastases (N1) and bone metastases (M1) found on positron-emission tomography (PET)-CT according to the German soft tissue sarcoma study (CWS)-IV 2002 protocol. The chemotherapy used Carboplatin and Topotecan. Survival was less than two years after the initial presentation. Adolescent extremity masses should raise suspicion to exclude serious malignancy. Despite early diagnosis and use of multimodal therapies, alveolar RMS prognostic remains unpredictable.