Abstract
Diagnosis: Langerhans cell histiocytosis (LCH) of the left temporal bone The patient was transferred to a skull base unit, where the findings of another biopsy led to the diagnosis of LCH. A whole-body bone scan showed that the disease was limited to the left temporal bone, and radiotherapy to the left side of skull was initiated. In 1953, Lichtenstein1observed that eosinophilic granuloma, Hand-Schüller-Christian disease, and Letterer-Siwe disease all have similar histologic features and coined the term histiocytosis X. In 1987, the name was changed to LCH, as recommended by the Writing Group of the Histiocytosis Society.2Langerhans cell histiocytosis is a rare, proliferative disorder in which there is accumulation of pathologic Langerhans cells, causing local tissue infiltration and destruction.3Histiocytes, which include dendritic cells and mononuclear phagocytes, are derived from bone marrow and migrate into the peripheral tissues after a period in the circulation. Langerhans cells are a subpopulation of dendritic histiocytes and are primarily located in the basal layer of the epidermis. Langerhans cell histiocytosis is rare in adults and principally affects children younger than 4 years.3It has an incidence of 0.5 to 5 cases per 1 million children per year.3Boys are affected twice as often as girls.3 The etiology of LCH is unknown and is still debated. There is little evidence to support an infectious cause. Abnormal immune regulation due to decreased T-cell function and increased production of cytokines has been suggested.3The monoclonal origin of all disease forms of LCH has also been demonstrated, which suggests a neoplastic process.4In adults, the infiltration of the temporal bone by Langerhans cells arises from the endolymphatic sac. The central role of the endolymphatic sac in the development of the inner ear immunity supports this hypothesis.3 Langerhans cell histiocytosis can affect all organs of the body. However, it has a predilection for the head and neck region. Cranial bone involvement is seen in 42% of all cases of LCH,5and temporal bone involvement occurs in 20% to 30% of cases.5When the disease affects the temporal bone, the patient may present with a range of otologic symptoms, including otorrhea, mastoid swelling, deafness, and aural polyps that are eroding the posterosuperior canal wall.3Facial nerve palsy, which has also been noted in association with LCH, is caused by destruction of the osseous canal, leading to compromised blood supply to the nerve.6The present case is unusual in that the patient presented only with pain and swelling above the ear. The absence of hearing loss and otorrhea is unusual in LCH that affects temporal bone, as are a normal tympanic membrane and normal middle ear function. Although facial nerve palsy has been reported in association with LCH of the temporal bone, we suspect that in this case the initial presentation with a left-sided lower motor neuron facial palsy was unrelated to the underlying disease process, as the patient made a full recovery with steroid treatment, and the facial palsy did not return despite progression of the underlying condition. There are no definitive clinical or radiologic findings that are exclusive to the diagnosis of LCH. Computed tomography can show a lytic lesion with well-defined borders without reactive sclerosis.3Magnetic resonance imaging may demonstrate an enhancing soft tissue mass that sometimes contains intralesional cysts, with a low to intermediate T1-weighted signal and a hyperintense T2-weighted signal.7It is helpful to differentiate LCH from cholesteatoma, which would not enhance with gadolinium, and from cholesterol granuloma, which has a high signal on T1-weighted images before contrast. The definitive of LCH diagnosis is made histologically. At biopsy, tissue is often reported to bleed profusely,8as in the present case. In 1987, the criteria for diagnosis of LCH were clearly established,2requiring the demonstration of CD1a antigens on the surface of the Langerhans cells or the identification of Birbeck granules (pentalaminar cytoplasmic inclusion bodies) in the lesional cells seen by electron microscopy. The treatment for LCH is variable, depending on the systems that are involved. For localized temporal bone lesions in adults, low-dose radiotherapy is commonly used,9as in this case, in which the patient underwent a 6-week course of irradiation after the diagnostic biopsy. Chemotherapy, including vinblastine plus prednisolone, is indicated in cases of multifocal bone involvement (>3 locations) and in disseminated disease.9The prognosis is good, with survival rates of more than 90% in patients with limited organ involvement.10Survival, however, is reduced in the presence of multisystem organ involvement and persistently active disease.10 References 1. Lichtenstein L Histiocytosis X. Arch Pathol 1953;5684- 102Google Scholar 2. Writing Group of the Histiocytosis Society, Histiocytosis syndromes in children. Lancet 1987;1 (8526) 208- 209PubMedGoogle Scholar 3. Mosnier IRondini-Gilli ECrosara PT et al. Langerhans' cell histiocytosis of the labyrinth in adults. Otol Neurotol 2004;25 (1) 27- 32PubMedGoogle ScholarCrossref 4. Willman CLBusque LGriffiths BB et al. Langerhans' cell histiocytosis (histiocytosis X): a clonal proliferative disease. N Engl J Med 1994;331 (3) 154- 160PubMedGoogle ScholarCrossref 5. Anonsen CKDonaldson SSStanfort S Langerhan's histiocytosis of head and neck. Laryngoscope 1987;97 (5) 537- 541PubMedGoogle ScholarCrossref 6. Irving RMBroadbent VJones NS Langerhans' cell histiocytosis in childhood: management of head and neck manifestations. Laryngoscope 1994;104 (1, pt 1) 64- 70PubMedGoogle ScholarCrossref 7. Koch BL Langerhans histiocytosis of temporal bone: role of magnetic resonance imaging. Top Magn Reson Imaging 2000;11 (1) 66- 74PubMedGoogle ScholarCrossref 8. Akisada THarada TYoshihiro TKawai A A case of bilateral eosinophilic granuloma in the temporal bone. Auris Nasus Larynx 1999;26 (3) 343- 348PubMedGoogle ScholarCrossref 9. Gulam IPegan BStancić VKruslin B Langerhans' cell granulomatosis in an adult: a 22-year follow-up. Eur Arch Otorhinolaryngol 2001;258 (4) 203- 207PubMedGoogle ScholarCrossref 10. Howarth DMGilchrist GSMullan BPWiseman GAEdmonson JHSchomberg PJ Langerhans' cell histiocytosis: diagnosis, natural history, management and outcome. 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Journal
Archives of Otolaryngology - Head & Neck Surgery
– American Medical Association
Published: Feb 1, 2008
Keywords: diagnostic radiologic examination,radiology specialty,temporal bone
