Solitary Plasmacytoma in the Setting of Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is an intriguing disorder characterized by the accumulation of specialized dendritic cells called Langerhans cells in several diverse tissues and body sites. It has been cited in numerous case reports to be associated with a wide variety of malignant neoplasms. Although many hypotheses have been suggested, the basis for such associations remains essentially unknown. We describe another association here that to our knowledge has not been reported thus far: a solitary plasmacytoma occurring at a site of previous involvement by LCH. This constitutes a new addition to the now fairly lengthy list of malignant neoplasms that have been reported to occur in the setting of LCH. The possible reasons for such an association are discussed along with a brief review of LCH.

Sinonasal extramedullary plasmacytoma: a systematic review of 175 patients

“…All 67 studies included individual patient data that was extractable and fit the minimum criteria for inclusion. The 67 studies are detailed in Table …”

Langerhans cell histiocytosis of the gingival mucosa with three recurrences over a 27 year period

“…Management of oral LCH depends on the size of the lesion, extent of tissue involvement and symptoms. ‘Mild systemic’ therapy with thalidomide, azathioprine or methotrexate, as well as local radiation and/or surgical excision have been proposed. Experience considering the management of oral soft tissue eosinophilic granuloma is limited, due to the small number of published cases.…”

“…The bones more commonly involved include the skull, pelvis, ribs, mandible, clavicle, spine and humerus. Occasionally, LCH may be associated with Hodgkin's disease, non‐Hodgkin's lymphoma and leukemia, and rarely with lung, thyroid, breast, liver, skin and stomach carcinomas, chondrosarcoma, osteosarcoma, astrocytoma, meningioma, retinoblastoma, apudoma and hepatoma.…”