Langerhans’ cell histiocytosis: report of a case with temporal localization

Aimoni, Claudia; Trevisani, Michele; Marangoni, Paola

doi:10.1016/s0165-5876(00)00374-8

Cited by 31 publications

(44 citation statements)

References 16 publications

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“…As mentioned previously, there are no specific signs and symptoms for LCH involving the skull, and the most common presentation is a painful and immobile scalp mass, which may be palpable in certain cases (5,8). In the present study, the patient experienced left temporal pain and headache, however, there was no obvious palpable mass.…”

Section: Discussionmentioning

confidence: 40%

“…The abnormal accumulation of histiocytes may occur in almost every organ, including the central nervous system, skin, bone, bone marrow, lung, liver, spleen and lymph nodes, and may cause associated signs and symptoms (4). Therefore, LCH may be classified as monosystemic or plurisystemic, according to the number of organs involved (5). The diagnosis of LCH is based on pathological examination.…”

Section: Introductionmentioning

confidence: 99%

“…LCH is typically considered to be an extremely rare disease of childhood, with an incidence rate of ~2-5/1,000,000 children/year (7). There are no specific signs and symptoms associated with LCH involving the skull, and the most common presentation is a painful and immobile scalp mass, which may be palpable in certain cases (5,8). Epistaxis or otorrhagia may be exhibited when the lesion invades the paranasal sinuses or external ear canal (5,8).…”

Section: Introductionmentioning

confidence: 99%

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Langerhans' cell histiocytosis of the temporal fossa: A case report

Chen

Liang

et al. 2016

Oncology Letters

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Abstract. Langerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations, varying from an isolated lesion to systemic involvement. The etiology of this disease remains to be elucidated. The present study reports a case of LCH with temporal fossa localization in an 8-year-old male patient, who had exhibited left temporal pain and headache for 1 month. Physical examination revealed slight exophthalmos and conjunctival hemorrhage in the patient's left eye, and non-contrast computed tomography imaging of the head revealed a soft tissue mass with unclear margins located in the left temporal fossa, as well as a wide bony defect. Magnetic resonance imaging revealed a heterogeneously contrast-enhanced mass near the left temporal pole, which eroded into the patient's left orbit and maxillary sinus. The lesion was totally excised and confirmed to be LCH through biopsy. IntroductionLangerhans' cell histiocytosis (LCH) is a rare disease with a wide spectrum of clinical manifestations (1). The primary pathogenesis of this disease is an abnormal proliferation of Langerhans' cells, which are typically present only in the dermis (1). A total of three different terms, namely eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease, were used in the past to describe a group of disorders characterized by increased numbers of histiocytes (2). In 1953, Lichtenstein (3) summarized these disorders as histiocytosis X, due to their similar histopathologies, and in 1987, the term LCH was recommended by The Writing Group of the Histiocyte Society as the official definition for this syndrome (4). The clinical appearance of LCH is dependent on the location of the lesion. The abnormal accumulation of histiocytes may occur in almost every organ, including the central nervous system, skin, bone, bone marrow, lung, liver, spleen and lymph nodes, and may cause associated signs and symptoms (4). Therefore, LCH may be classified as monosystemic or plurisystemic, according to the number of organs involved (5). The diagnosis of LCH is based on pathological examination. Typical accumulation of histiocytes, electron microscopic observation of Birbeck granules and the presence of Langerhans' cell-associated markers, including cluster of differentiation (CD)1a and S-100 protein, are crucial for establishing a diagnosis of LCH (6). LCH is typically considered to be an extremely rare disease of childhood, with an incidence rate of ~2-5/1,000,000 children/year (7). There are no specific signs and symptoms associated with LCH involving the skull, and the most common presentation is a painful and immobile scalp mass, which may be palpable in certain cases (5,8). Epistaxis or otorrhagia may be exhibited when the lesion invades the paranasal sinuses or external ear canal (5,8).The present study reports the case of an 8-year-old male patient with an LCH lesion in his left temporal fossa, and aims to provide clinical experience in the diagnosis and treatment of intracranial LCH. Case reportAn 8-yea...

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Section: Discussionmentioning

confidence: 40%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Langerhans' cell histiocytosis of the temporal fossa: A case report

Chen

Liang

et al. 2016

Oncology Letters

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“…Nevertheless, a number of authors prefer the application of chemotherapy. Different protocols, in addition to the use of systemic corticosteroids, cytostatic therapies like 2-chlorodesoxiademosin (2CdA-cladribine), cyclosporine, thalidomide and myeloablative therapies were prescribed (19,(21)(22)(23). Single system disease can be treated with local injectable intratumoral steroids (11,18).…”

Section: Discussionmentioning

confidence: 99%

“…Single system disease can be treated with local injectable intratumoral steroids (11,18). Following surgery, numerous investigators recommend low-dose radiotherapy (10-20 Gy) in cases in which the tissue involved cannot be completely excised (21,24).…”

Section: Discussionmentioning

confidence: 99%

Langerhans' cell histiocytosis of the temporal bone: A case report

Yang

2017

Experimental and Therapeutic Medicine

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Abstract. Langerhansmporal histiocytosis (LCH) refers to a group of diseases that are characterized by the primary pathogenesis of an abnormal polyclonal proliferation of Langerhans cells that affect different structures of the human body, including the temporal bone. Thus far, the etiology of LCH remains unclear. Diagnosis of LCH is based on a synthetic analysis of the clinical presen tations, as well as on features of imaging and histopathology. The clinical features, diagnosis, treatment and prognosis of LCH remain obscure, and temporal LCH is often confused with ear inflammatory lesions and malignant tumors. There are several therapeutic modalities for temporal bone LCH that include surgery, chemotherapy, radiotherapy and steroidal injections. The present study reports the case of an infant presenting a 1-month history of worsening left facial paralysis and a slowly enlarging post auricular mass followed by a 1-month history of postauricular swelling in the left ear. Computed tomography demonstrated a large mass of organized tissue. Moreover, the diagnosis of LCH was confirmed by histopathological and immunohistochemical examinations. The patient also suffered from multiple organ failure, including the liver, kidney, lymphatic system, skin, hematopoietic system and lungs. Following surgical intervention with mastoidotympanectomy, the parents of the patient refused further chemotherapy, and the patient succumbed to the disease ~6 months later.

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Paediatric Head and Neck Pathology and Surgery

Saniasiaya

Lazim

2022

Head and Neck Surgery : Surgical Landmark and Dissection Guide

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Langerhans’ cell histiocytosis: report of a case with temporal localization

Cited by 31 publications

References 16 publications

Langerhans' cell histiocytosis of the temporal fossa: A case report

Langerhans' cell histiocytosis of the temporal fossa: A case report

Langerhans' cell histiocytosis of the temporal bone: A case report

Paediatric Head and Neck Pathology and Surgery

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