Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 11, 2017
Bilateral Temporal Langerhans Cell Histiocytosis
- Background:
- Langerhans Cell histiocytosis (LCH) is a rare disorder that has an unpredictable clinical course and multiple clinical presentations.
- The etiology and pathogenesis are still unclear.
- The involvement of the temporal bone has been described in 15–61% of all cases of Langerhans Cell histiocytosis.
- It most often affects children (1–4 years old).
- The involvement of the temporal bone can be seen at the onset of the disease or during its progression.
- In our case, the patient had a bitemporal isolated presentation.
- Isolated bilateral temporal bone involvement is less common (5–25%).
- Clinical Presentation:
- The most common symptom is persistent otorrhea in spite of medical treatment.
- Other symptoms include mastoid swelling, periauricular eczema, and erosion of external auditory canal.
- Sensorineural hearing loss, vertigo, and paralysis of the cranial nerves are unusual.
- Key Diagnostic Features:
- CT scan demonstrates well-demarcated temporal lytic with or without reactive sclerosis lesions.
- MRI is more useful to evaluate soft tissue and intracranial extension. The lesions are isointense on T1WI and hypertense on T2WI, with solid enhancement.
- Suspicion of LCH should be raised especially if the labyrinth is spared.
- Differential Diagnoses:
- The radiologic differential diagnoses includes mastoiditis, rhabdomyosarcoma, and metastasis; however, metastases are more common in older patients and extensive bone destruction with large soft-tissue masses are not typically seen in mastoiditis.
- The lytic lesion of the temporal bone, with sparing of the labyrinth, is a radiologic clue to the diagnosis.
- Rhabdomyosarcoma is not bilateral.
- Treatment:
- Solitary bone lesions will be treated surgically and locally by curettage or excision.
- Induction therapy, including vinblastine and prednisone, has been suggested for systemic disease and lesions that require prompt resolution or are not amenable to surgical therapy.
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