Langerhans’ cell histiocytosis of the temporal bone in children

Saliba, Issam; Sidani, K.; Fata, Fouad El; Arcand, Pierre; Quintal, Marie‐Claude; Abela, Anthony

doi:10.1016/j.ijporl.2008.02.001

Cited by 75 publications

(93 citation statements)

References 39 publications

(104 reference statements)

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“…The indices of response, recurrence and complications may vary due to different schemes. Moreover, the prognosis of temporal bone LCH is excellent in patients with limited organ involvement (3). Besides the extent of the disease, the age of the patient at the time of diagnosis is an important factor.…”

Section: Discussionmentioning

confidence: 99%

“…LCH is primarily a pediatric disease, which is rarely reported in adults. Moreover, the peak age of the patients range between 1 and 4 years old, and patients with focal lesions are generally older (0.1-15.1 years) than those with multisystemic disease (0.09-14.8 years) (3). Histiocytosis of the temporal bone occurs in 15-60% of the sample cases, and it is higher when radiological findings are included in asymptomatic patients; its bilateral occurrence is described in up to 30% of the cases (4) and is more frequent in the multisystemic diseases (5,6).…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Yang

2017

Experimental and Therapeutic Medicine

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“…Langerhans' 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).…”

Section: Introductionmentioning

confidence: 99%

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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“…Langerhans cell histiocytosis (LCH), as renamed from histiocytosis X by the Histiocyte Society in 1987 [23], refers to a group of conditions with different clinical courses, characterized by abnormal proliferation of pathologic Langerhans cells (LCs) [27]. Although the stem cell has been identified, the pathogenesis remains unclear [10,23].…”

Section: Introductionmentioning

confidence: 99%

“…Although the stem cell has been identified, the pathogenesis remains unclear [10,23]. While it has been suggested LCH is a primary neoplastic disorder of the LC [29], more recent evidence points toward an immunologic aberration [2,5,10].…”

Section: Introductionmentioning

confidence: 99%

Does Adjunctive Chemotherapy Reduce Remission Rates Compared to Cortisone Alone in Unifocal or Multifocal Histiocytosis of Bone?

Baptista

Camargo

et al. 2012

Clinical Orthopaedics &Amp; Related Research

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Background Langerhans cell histiocytosis (LCH) is a rare disorder that can affect almost any organ, including bone. Treatment options include local corticosteroid infiltration in isolated bone lesions and oral corticosteroids and chemotherapy in multifocal bone lesions. Several studies show local corticosteroid injection in unifocal bone lesions heal in more than 75% of patients with minimal side effects. Therefore, it is unclear whether chemotherapy adds materially to the healing rate. Questions/purposes We therefore compared overall survival, remission rate, and recurrence rate in patients with bone LCH treated with chemotherapy and corticosteroids or corticosteroids alone. Methods We retrospectively reviewed the records of 198 patients with LCH since 1950. Median age at diagnosis was 5 years, male-to-female ratio was 1.33, and the most frequent symptom was local pain (95%). We recorded the disease presentation, demographics, treatment, and clinical evolution of each patient. Minimum followup was 4 months (median, 24 months; range, 4-360 months). Results The survival rate of the systemic disease group was 76.5% (65 of 85) while the survival rate in the unifocal and multifocal bone involvement groups was 100% at a median 5-year followup. All patients with unifocal bone involvement and 40 of 43 (93%) with multifocal bone involvement had complete remission. One of 30 patients with multifocal bone involvement treated with chemotherapy and oral corticosteroids did not achieve remission whereas two of six receiving only corticosteroids did not achieve remission. Conclusions Our observations suggest intralesional corticosteroid injection without adjunctive chemotherapy achieves remission in unifocal bone LCH but may not do so in multifocal single-system bone involvement. Larger series would be required to confirm this observation.

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Langerhans’ cell histiocytosis of the temporal bone in children

Cited by 75 publications

References 39 publications

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

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

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

Does Adjunctive Chemotherapy Reduce Remission Rates Compared to Cortisone Alone in Unifocal or Multifocal Histiocytosis of Bone?

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