Langerhans Cell Histiocytosis

Clinical History

A 20 year old lady had a three month history of left sided jaw pain and paraesthesia. After referral to a maxillofacial outpatient clinic she had an orthopantomogram, CT mandible, chest X-ray and high resolution CT chest examination.

Imaging Findings

A lyic ill defined lesion is seen on the left anterior mandible on the orthopantomogram. The lesion is centred at the root of the lower left first molar. There is no marginal sclerosis and a wide zone of transition. Greater soft tissue and bony detail is seen on CT. Chest x-ray shows a reticulonodular pattern to the lungs with preponderance for the upper zones. High resolution CT chest confirms nodules of varying size and associated cystic change. A biopsy of the mandibular lesion confirmed Langerhans cells.

Discussion

Langerhans Cell Histiocytosis (LCH) is a disease of unknown aetiology with a prognosis related to the age of presentaion, location and extent of organ involvement. The disease encompasses the childhood syndromes of Hand-Schueller-Christian and Letterer-Siwe. Eosinophilic granuloma describes the clinical scenario of a solitary bone lesion and is common in young adults with a greater preponderance in males. LCH is characterised histologically by the presence of Langerhans cells, histiocytic cells with Birbeck granules. The bones commonly affected are the flat bones - ribs, vertebrae and skull and long bones. The mandible is more commonly affected than the maxilla and both are more commonly involved in patients less than 20 years. Approximately 30% of patients with maxillofacial bone involvement will have systemic disease at the time of presentation (1). The presenting feature is typically jaw pain. Classically osteolysis results in a 'floating tooth' on orthopantomogram. The appearance can be confused with osteomyelitis or neoplasm. Within the chest there is an upper zone predominance of irregular cysts and nodules. Cysts result from the destruction of bronchiolar wall and subsequent dilatation of the lumen. Lung volumes are preserved and there is sparing of the costophrenic angles. Advanced disease results in honeycomb lung with pulmonary hypertension. The patient underwent an aggressive chemotherapeutic regime after referral to the National Specialist Treatment Centre.

Differential Diagnosis List

Final Diagnosis

Langerhans Cell Histiocytosis

URL:	https://www.eurorad.org/case/6700
DOI:	10.1594/EURORAD/CASE.6700
ISSN:	1563-4086