RESUMEN
BACKGROUND: Langerhans cell histiocytosis (LCH) is a disease that primarily affects bone but can be associated with a clinical spectrum that ranges from a solitary bone lesion with a favorable natural history to a multisystem, life-threatening disease process. AIM: We analyzed our single institutional experience of managing children with LCH. SETTINGS AND DESIGN: A total of 40 children of LCH, managed in tertiary cancer center in South India in the period from 2001 to 2005, were evaluated retrospectively. MATERIALS AND METHODS: Clinicopathological features, laboratory findings, treatment modalities and long-term outcome were analyzed. RESULTS: Children were aged between 2 months and 12 years, with a mean of 3 years. Majority of the children were below 5 years of age. Group B constituted a bulk of children. Disseminated cases were less (five patients). Liver function dysfunction was seen in four (10%) children. Pulmonary interstitial infiltrates were seen in two (5%) cases. Diabetes insipidus manifested in three patients. There was one death. CONCLUSION: A better understanding of the etiology and pathogenesis of LCH will result in more directed and efficacious treatment regimens.
RESUMEN
We report a case of myxoinflammatory fibroblastic sarcoma in a thirteen year old girl who presented with a tender swelling in the left upper back. The tumor consisted of varying proportions of inflammatory, myxoid and hyalinized areas. Large bizarre cells with virocyte like inclusions and lipoblast like cells were present. To the best of our knowledge this is the first reported case of myxoinflammatory fibroblastic sarcoma of the back, the extremities being the commonest site of involvement. Due to its varied histologic appearance, the tumor should be differentiated from various benign and malignant soft tissue lesions.