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Objective: The purpose of the report is to describe a patient with hereditary osteochondromatosis and spinal cord compression at the thoracic level.Clinical features: An 8-year-old patient with hereditary osteochondromatosis inherited from his father presented paraparesis in the left foot, leading to complete paralysis in both legs.Intervention: In a CT scan, a bony tumor rising from the posterior wall of the T3 body narrowing the spinal canal, and the MRI spinal cord compression at the same level and the hydrosyringomyelic cavity extended to the conus medullaris; with an anterior thoracic approach to T2–T4, the fibro-cartilaginous tumor was removed, and the stabilization was completed with bone graft and a plate. Two months after surgery, the patient recovered strength in both legs.Conclusions: A detailed family history through examination-guided advanced imaging and biopsy provides useful information for diagnosis and appropriate management of occupative lesions in patients affected with multiple hereditary exostosis. [ABSTRACT FROM AUTHOR]

Background: The prognosis of medulloblastoma in children under 3 years of age is poor. Methods: A retrospective analysis was performed to evaluate children under 3 years of age with medulloblastoma. Overall survival (OS) and progression-free survival (PFS) were assessed in children with and without metastasis. Results: A total of 534 children were diagnosed with medulloblastoma during the study period, 49 (9.1%) of whom were under 3 years of age and were evaluated. Their ages ranged from 5 to 35 months with a mean of 18.5 months. In 39 (79.6%) of these patients the tumors were staged as T3M0 or under, while 10 (20.4%) had metastasis at diagnosis. The OS was 38% and PFS 37% in the whole series, while PFS was 32% in those with metastasis and 40% in those without ( P=0.78). For those who received radiotherapy the PFS was 62%, and in those not treated with radiotherapy PFS was nil ( P=0.0001). When the children were divided into those who received surgical treatment plus chemotherapy and those who received surgery plus radiotherapy plus chemotherapy, the PFS was nil and 66%, respectively ( P=0.00001). Conclusion: Because of the high morbidity of radiotherapy in children under 3 years old, surgery continues to be the basis of improved prognosis, followed by chemotherapy. [ABSTRACT FROM AUTHOR]

Five hundred and seventy primary central nervous system (CNS) tumors from the Department of Pathology at the National Institute of Pediatrics in Mexico City, collected from 1970 to 1989, were histologically reclassified in order to find out their relative incidence as well as their outstanding features. With this, we could establish a frame of reference for our local population, contributing to the epidemiological analysis of these entities. All the tumors were examined independently by two pathologists (C.R. and M.R.), using the classification of Rorke et al. Histological type, patient age and sex, and tumor location were analyzed. CNS tumors were the secondmost frequently encountered solid tumors, after lymphomas, and were increasing in incidence at a rate of 2.2 annually. Children in the age group 0-9 years were most often affected, and there was a predominance of male patients. Astrocytoma and medulloblastoma were the most common tumor types. The infratentorial region was the most frequent tumor location in the 2-to 9-year age group. By contrast, in the under 2-year-olds a supratentorial location was more frequent, and the incidence of germ cell tumors was proportionally high. In general, some histological types seemed to be associated with particular age groups. Although we found primitive neuroectodermal tumors to be the fifth most common at all ages (except for medulloblastoma), many other authors do not report a similar finding. [ABSTRACT FROM AUTHOR]