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

Abstract

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.

Langerhans cell histiocytosis of the skull in 23 children

“…The CT scan image generally has single or multiple, round to oval osteolytic lesions with sharp borders giving a punched out appearance with indistinct bone margins in association with a homogeneous soft tissue mass. A “Double-contour” or “beveled edge” appearance due to involvement of both the inner and outer tables may be seen [ 12 ]. The diagnostic criteria of LCH that Langerhans cell-specific protein is the gold standard for diagnosis were defined by The Histiocyte Society Writing group in 1787 [ 13 ].…”

Spontaneous remission of skull Langerhans cell histiocytosis that had developed by repeated head injury: illustrative case

“…There have been several reports of LCH subsequent to head trauma. 2,[4][5][6][7][8][9][10][11][12] However, rapid enlargement of the skull LCH within 1 month after a head injury is extremely rare. LCH preceded by head injury may have been caused by an inflammatory mechanism; however, to the best of our knowledge, there have been no reports demonstrating a local inflammatory response by histopathology.…”