Primary bone lymphoma in children: <br /> diagnostic difficulties
Journal Title: Ortopedia Traumatologia Rehabilitacja - Year 2004, Vol 6, Issue 4
Abstract
Background. Primary localization of non-Hodgkins lymphoma (NHL) in the skeletal system is very rare in children. Bone tissue involvement most often points to metastasis from a primary focus localized elsewhere. The atypical localization and histopathological classification problems cause diagnostic difficulties, which delays the implementation of treatment until the children have reached a higher clinical stage (III or IV), leading to a poor prognosis.Case descriptions. We present 12 cases of pediatric NHL with primary localization in the skeleton (9 boys, 3 girls). In 10 of these cases only the skeletal system was involved, while in 2 cases there was simultaneous lymph node involvement. The average time from onset to correct diagnosis was 1.5 months. Initially these patients were diagnosed provisionally with localized or diffuse neoplasm of the bone. Of the 8 children in whom the diagnostic process lasted longer than 2 months, 7 died despite intensive treatment (BFM 86, LMB 89, ICE). One boy, additional treated with anti-CD20 and RT, is still alive and has been symptom-free for 4 months.Conclusions. Difficulties in histopathological diagnosis result not only from the crush effect, but also from the morphological picture, often differing from NHL, and sometimes resembling neoplastic or neuroectodermal tumors. The co-expression of neural or neuroendocrine markers can also be misleading. Given the ambiguous morphology, CD20 staining should be standard practice. In cases of local or dispersed skeletal pain one should also consider the possibility of neoplastic growth from the lymphatic system. The drugs now available seem to improve prognosis.
Authors and Affiliations
A. Raciborska, W. Wożniak, M. Rychłowska, J. Małdyk , B. Sopyło, T. Klepacka, J. Peregud-Pogorzelski
Keywords
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