Systemic rhabdomyosarcoma with diffuse bone marrow involvement. Case report of an unusual presentation.
Rhabdomyosarcoma presenting as a systemic disease is rare. This report concerns a 12-year-old girl who came to medical attention for what was thought to be a hematologic malignancy. Diffuse lytic bone lesions and large primitive cells in the bone marrow exhibiting erythrophagocytosis supported this diagnosis. During the course of the disease, a soft tissue mass in the region of the left ankle was removed and was a typical alveolar rhabdomyosarcoma. Retrospective review of the marrow, including electron microscopy, demonstrated that the primitive marrow cells were probably rhabdomyoblasts as well. The clinical course was a rapid downhill one in which lytic bone lesions and hypercalcemia were prominent. Although rhabdomyosarcoma eventually may disseminate, initial widespread disease without a clinically apparent primary can be a diagnostic dilemma. This clinical presentation, in combination with the recognized aggressiveness of the alveolar histologic subtype, identifies a rare subgroup of patients with rapidly fatal disease.
D000842:Ankle; D000971:Antineoplastic Combined Chemotherapy Protocols; D001856:Bone Marrow Examination; D001859:Bone Neoplasms / Q000473:pathology*; D002648:Child; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008727:Methotrexate / Q000008:administration & dosage; D014411:Neoplastic Stem Cells / Q000276:immunology; D010587:Phagocytosis; D011241:Prednisone / Q000008:administration & dosage; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D003520:Cyclophosphamide; D011241:Prednisone; D008727:Methotrexate