Langerhans' cell granulomatosis in an adult: a 22-year follow up

Abstract

A 57-year-old patient with Langerhans' cell granulomatosis (LCG) is presented. During 22 years of treatment, there were five relapsing infiltrations at different sites of the skull bones, which were treated by surgery, local radiotherapy, and chemotherapy. During the last relapse, the right temporal bone was infiltrated by granuloma and the petrous bone was destroyed with an intimate spread to the internal carotid artery. After two palliative surgical resections and ineffective radiotherapy, 12 cycles of chemotherapy (vinblastine plus prednisolone) were applied and a clinical remission of the disease was achieved. Special attention is paid to the complexity of diagnosis and choice of therapy. It is concluded that the behavior of LCG may change with time, and assume an aggressive form of the disease. Chemotherapy is the treatment of choice for this type of multifocal malignant form of LCG. Prognosis of the disease is unpredictable.

Petrous Bone Langerhans Cell Histiocytosis Treated with Radiosurgery

“…Radiotherapy is usually administered at low dosages, ranging between 5 and 20 Gy [8,9] , and is indicated for large destructive lesions or growing unifocal lesions [2] . More recently, some patients have been treated with intralesional steroids with excellent local control [3,11] . In general, low-dose radiation has been considered the treatment of choice for accessible LCH of the temporal bone [2,9] .…”

“…Single bone lesions have been reported to resolve spontaneously after diagnostic biopsy, with or without curettage. Consequently most authors agree that these lesions should be locally treated when possible [3,8,9] . Radiotherapy is usually administered at low dosages, ranging between 5 and 20 Gy [8,9] , and is indicated for large destructive lesions or growing unifocal lesions [2] .…”

“…Various areas of the temporal bone may be affected, including the squama, the mastoid and the petrous apex [3,4,9] . We present a patient with monosystemic LCH limited to the posterior surface of the petrous bone, extending to the IAC.…”

“…The term includes eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. Eosinophilic granuloma, also known as monosystemic LCH, is the localized form of LCH and is the least aggressive of the 3 clinical entities [3,4] . LCH is usually diagnosed in patients between 5 and 15 years old [1,5] and is characterized by the presence of osteolytic lesions.…”

Pulmonary Langerhans' Cell Histiocytosis in Adults

“…Changes in the form of painful osteolytic defects and nodulated masses spreading to the soft tissues are usually discovered in the cranial vault, bony face, particularly in the lower jaw, orbital bones, mastoid process, ribs, shoulder-blade and pelvis. Of particular danger is the involvement of the bones of the spinal column, the base of the scull and orbit, which is signaled by the pressure and damage to the surrounding structures (Figs 3-5) [3,23].…”