Salivary gland manifestations caused by Langerhans cell histiocytosis (LCH) are extremely rare even in multifocal disease. Only a few cases have been reported in the literature. For a general discussion of this disease, please refer to the Langerhans cell histiocytosis (LCH) article
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Epidemiology
Langerhans cell histiocytosis commonly affects children and young adults with the average age of onset being 1 to 3 years. It occurs more commonly in males 2.
Salivary gland involvement is exceedingly rare with only a few documented cases of Langerhans cell histiocytosis involving the parotid gland, submandibular gland and sublingual gland reported within the literature 2-9.
Clinical presentation
Presentation typically involves salivary gland swelling and may include non-specific symptoms such as cervical lymph node enlargement, intermittent fever and/or loss of appetite 8.
Radiographic features
Salivary gland manifestations commonly appear as non-specific hyperplastic changes on ultrasound, MRI and CT alongside associated increased metabolic activity in FDG-PET 7,8.
Treatment and prognosis
Prognosis varies depending on the extent of the disease, the severity at onset and age at diagnosis.
The treatment of salivary gland LCH is not well defined as there have only been a few case reports 2. Various treatment modalities have been described such as follow-up only, surgical resection, local injection of corticosteroids, low-dose radiation therapy, high dose systemic corticosteroids, chemotherapy, and, in more resistant cases, bone marrow transplantation and antibody therapy 3.
Differential diagnosis
lymphadenitis
suppurative parotitis
actinomycosis of parotid gland
parotid duct obstruction
myoepithelioma
metastatic disease (e.g. neuroblastoma, leukemia, lymphoma)
parotid hemangioma
uveoparotitis
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