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The authors describe a 6-year-old boy diagnosed with alveolar rhabdomyosarcoma located in the thigh, with distal metastases to lungs, bones, and bone marrow. A very good partial response to first-line chemotherapy was obtained, but the child developed fatal leptomeningeal dissemination immediately after complete resection of the primary tumor. This case demonstrates the rapidity with which leptomeningeal spread of extracranial metastatic alveolar rhabdomyosarcoma can occur and underscores the importance of diagnostic lumbar puncture and brain radiological investigations at diagnosis, even when the tumors are not in the parameningeal location. [ABSTRACT FROM AUTHOR]

The authors describe the case of a 19-year-old female patient with a primary primitive neuroectodermal tumor (PNET) of the thoraco-lumbar spinal cord, who presented with acute urinary retention and back pain for 2 months. Magnetic resonance imaging revealed an intradural extramedullary tumor, 6.5 cm long, in the region of the conus medullaris. Histological examination disclosed a small round cell tumor with immunohistochemical characteristics of a peripheral PNET. Metastatic workup showed no evidence of an intracranial tumor or metastases outside the neuroaxis. The patient received multidisciplinary treatment, including surgical excision, irradiation of the entire cranio-spinal axis, and high-dose chemotherapy with autologous stem cell rescue. Presently, 24 months after diagnosis, the patient remains in complete remission. [ABSTRACT FROM AUTHOR]

High doses of methotrexate (MTX) have been incorporated in the treatment of osteogenic sarcoma since the 1970s. Unfortunately, high-dose MTX (HD-MTX) can cause untoward side effects that may complicate the proper management of these patients. Two cases of MTX-induced acral erythema are described and the possible implications of this complication are reviewed, with a discussion of the pathophysiology of this adverse effect. Two young female patients suffering from osteogenic sarcoma received HD-MTX (12 g/m²) according to the chemotherapeutic protocol. A 17-year-old patient with osteogenic sarcoma of the head developed painful acral erythema of her palms and soles after the fifth dose of MTX, and a 22-year-old patient with osteosarcoma of the femur developed severe acral erythema after the sixth dose. The severity of the reaction was significant enough to cancel further treatment with HD-MTX in both patients. All symptoms resolved without sequellae several weeks later in both cases. Acral erythema may pose a significant albeit transient phenomenon adversely influencing chemotherapy in patients suffering from osteosarcoma and treated with HD-MTX. The pathophysiology of this side effect is not completely understood and further investigation of the pharmacokinetics of HD-MTX in this patient population is needed. [ABSTRACT FROM AUTHOR]

A 14-year old girl was diagnosed as suffering from an immature teratoma. As removal of the teratoma was technically unfeasible, the patient was started on interferon α2β, 3 MU/day, 5 days per week. On this therapy, the growth of the teratoma was stopped and the patient has been well for the past 50 months. She continues to be treated with interferon α2β, with almost no side effects and no restrictions of her everyday activities, with a good quality of life. She is able to attend school regularly, travels abroad frequently, and participates fully in everyday activities normal for her age and social conditions. Her Karnofsky performance status is 100. This case demonstrates the efficacy of interferon α2β therapy in certain instances of growing teratoma syndrome, even when the tumorous mass is initially quite large. [ABSTRACT FROM AUTHOR]