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Incidental finding of Langerhans cell histiocytosis of temporoparietal bone - A case report

INTRODUCTION AND IMPORTANCE: Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells...

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Detalles Bibliográficos
Autores principales: Mishra, Aakash, Gyawali, Sandesh, Kharel, Sanjeev, Mishra, Aman, Kuikel, Sandip, Pathak, Nibesh, Gurung, Ashim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8319439/
https://www.ncbi.nlm.nih.gov/pubmed/34274753
http://dx.doi.org/10.1016/j.ijscr.2021.106179
Descripción
Sumario:INTRODUCTION AND IMPORTANCE: Langerhans cell histiocytosis (LCH) is a rare haematological disorder affecting infants and young children and has an estimated incidence of 2-5 cases per million people per year. LCH invades the reticuloendothelial system and causes the proliferation of Langerhans cells and mature eosinophils. LCH involving the temporoparietal bone has rarely been reported in the literature. PRESENTATION OF CASE: A ten-year-old boy presented to the Neurosurgical outpatient clinic with a swelling on the right temporoparietal region following a fall from his bicycle. Local examination revealed a single, 3 × 3 cm, non-tender, cystic, immobile swelling in the right temporoparietal region. On evaluation for recent head trauma, an incidental finding of eosinophilic granuloma was discovered on a CT scan. The FNAC was suggestive of a histiocytic lesion pertaining to a diagnosis of LCH. The patient underwent wide excision of the mass and cranioplasty. A one-month follow-up CT scan of the head had no evidence of residual or recurrent disease. DISCUSSION: Eosinophilic granuloma is one of the three variants of LCH and has a relatively better prognosis. Clinical diagnosis can be challenging and mandates tissue sampling for histopathological examination. Treatment modalities including surgery, radiotherapy, chemotherapy, and steroid injection are used alone, or in combination, depending on the extent and severity of the disease. CONCLUSION: Examining a swelling in the temporoparietal region with no other characteristic symptoms could be a case of LCH. The timely diagnosis and surgical excision with other adjuvant treatment options of this rare pediatric disease would help in a better outcome.