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LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

HISTIOCITOSE DE CÉLULAS DE LANGERHANS DA ESCÁPULA - DIAGNÓSTICO E OPÇÕES DE TRATAMENTO

HISTIOCITOSIS DE CÉLULAS DE LANGERHANS DE LA ESCÁPULA - DIAGNÓSTICO Y OPCIONES DE TRATAMIENTO

ABSTRACT

Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

Keywords:
Histiocytosis, Langerhans-cell; Scapula; Immunohistochemistry

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