Animals ; Brachytherapy ; Combined Modality Therapy ; Humans ; Male ; Prognosis ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar/drug therapy*

Rhabdomyosarcoma originating from the chest wall is a rare malignant tumor in children and was considered to be guarded in prognosis previously. However, recent advances in multidisciplinary treatment of rhabdomyosarcoma in children have improved the disease free survival rate. Authors report a case of alveolar rhabdomyosarcoma, stage III, originating from the chest wall who is surviving free of disease for 15 months with aggressive chemotherapy and radiotherapy.
Child ; Disease-Free Survival ; Drug Therapy ; Humans ; Prognosis ; Radiotherapy ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Thoracic Wall* ; Thorax*

It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.
Adolescent ; Blood Platelets ; Blood Transfusion ; Child ; Cyclophosphamide ; Dacarbazine ; Disease Progression ; Disseminated Intravascular Coagulation ; Doxorubicin ; Drug Therapy* ; Drug Therapy, Combination ; Epistaxis ; Erythrocytes ; Hematuria ; Hemorrhage ; Humans ; Male ; Plasma ; Protease Inhibitors ; Rhabdomyosarcoma ; Rhabdomyosarcoma, Alveolar* ; Tissue Donors ; Vincristine

Primary tumors of the lung are uncommon in pediatric patients, particularly bronchioloalveolar carcinoma (BAC). An 11-year-old female suffering from back pain for 1 month was referred to Seoul St. Mary's Hospital for treatment of a pathologic fracture of the lumbar spine. Comprehensive evaluation disclosed numerous pulmonary metastases of rhabdomyosarcoma (stage IV). During chemotherapy, most of the lung lesions regressed, with the exception of two nodules. Wedge resections, intended for diagnosis and cure, yielded a histologic diagnosis of BAC.
Adenocarcinoma, Bronchiolo-Alveolar* ; Back Pain ; Child ; Diagnosis ; Drug Therapy ; Female ; Fractures, Spontaneous ; Humans ; Lung ; Lung Neoplasms ; Neoplasm Metastasis ; Rhabdomyosarcoma ; Seoul ; Spine

OBJECTIVEAlbeit rare, rhabdomyosarcoma (RMS) may occur in middle to old-aged adults. The purpose of this study was to investigate the clinicopathological characteristics and prognosis of RMS in patients ≥ 40 years of age.

METHODSThe clinical, pathological and immunohistochemical profiles of the 76 cases were reviewed, and the outcomes were analyzed.

RESULTSThere were 46 males and 30 females with a median age of 55 years. Sixty cases occurred in somatic soft tissues, including extremities (n = 25), head and neck (n = 19) and trunk (n = 16). Sixteen developed in the internal organs with predilection for the urogenital tract. Histologically, 38 cases (50.0%) were of embryonal RMS (ERMS) subtype, 29 cases (38.2%) of pleomorphic RMS (PRMS), 7 cases (9.2%) of poorly-differentiated RMS (PdRMS), and 2 cases (2.6%) of alveolar RMS (ARMS). Immunohistiochemically, the tumor cells showed diffuse staining of desmin (68/68, 100%), with a variable expression of myogenin (30/43, 69.8%), MyoD1 (33/51, 64.7%) and MSA (29/40, 72.5%). The therapy data available in 54 cases showed that three inoperable cases were treated with chemotherapy and radiotherapy, 51 cases underwent local excision, including 10 cases with chemotherapy, 9 cases with radiotherapy, 8 cases with chemotherapy and radiotherapy after surgery, and the remaining 24 cases were not administrered with postoperative adjuvant therapy. The follow-up data available in 54 cases showed that 22 patients were alive with unresectable or recurrent/disease and 32 patients were alive with no evidence of disease. The median disease-free and overall survivals were 6.0 months and 7.0 months, respectively. Twenty-seven patients exhibited progression of disease with recurrence in 8 cases and metastasis in 19 cases. The median interval to progression was 6.0 months.

CONCLUSIONSThe most common subtypes of RMS in middle to old aged patients are ERMS and PRMS. In general, Rhabdomyosarcomas of middle to old aged patients shows a highly aggressive behavior. Patients may benefit from multi-disciplinary therapy.

Adult ; Aged ; Aged, 80 and over ; Chemotherapy, Adjuvant ; Desmin ; metabolism ; Disease-Free Survival ; Extremities ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Humans ; Male ; Middle Aged ; MyoD Protein ; metabolism ; Myogenin ; metabolism ; Neoplasm Recurrence, Local ; Radiotherapy, Adjuvant ; Rhabdomyosarcoma ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Rhabdomyosarcoma, Alveolar ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Rhabdomyosarcoma, Embryonal ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Soft Tissue Neoplasms ; drug therapy ; metabolism ; pathology ; radiotherapy ; surgery ; Survival Rate