Head and neck manifestation and prognosis of Langerhans’ cell histiocytosis in children

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Abstract

Objective

To appreciate the several head and neck manifestations of Langherans’ cell histiocytosis (LCH) in children and their multidisciplinary management and outcome.

Study design

Retrospective study.

Patients and methods

Clinical reports of 42 patients with LCH treated in the Departments of Paediatric Haematology, Paediatric Oncology and Paediatric Otorhinolaryngology of a tertiary care center were analyzed. Only cases where the disease was localized to the head and neck were considered. The age at diagnosis, gender, clinical presentation, extension of disease as well as response to treatment and outcome were recorded from the charts of each of these patients.

Results

Of the 42 patient charts reviewed, 31 (73.8%) presented with head and neck localization. 10 of these had an exclusive head and neck presentation. Multisystem LCH was mostly found in infants under 3-year-old (mean age: 2-year-old), and bony manifestations in older. All treatments delivered to patients were well-tolerated and the evolution good.

Discussion and conclusion

Head and neck involvement is known to be very frequent in LCH. There is no consensus about treatment but authors highlight that all teams in charge of patients presenting with LCH agree to remain as conservative as possible. For solitary large lesions looking like a tumor which resection could result in functional or cosmetic morbidity, it would be important to get first a biopsy. For multisystemic LHC, therapeutic trials with chemotherapy agents still in process should increase the rate of success.

Introduction

Langerhans’ cell histiocytosis (LCH) was first identified in 1893 by Hand who in a report to the Pennsylvanian Pathology Society described the case of a 3-year-old child who died after presenting with polyuria, exophtalmia and hepatosplenomegaly [1], [2]. In 1915, Schüller published a paper about the aetiology of cranial bone defects in childhood. In 1920, Christian reported a syndrome associating limbs bone defects with exophtalmia and diabetes insipidus. One year later, Hand wrote on the similarities between his own case and those related by Schüller and Christian [1], [2]. From these observations, the association of exophtalmia, diabetes insipidus and multiple bone defects became known as Hand–Schüller–Christian (HSC) disease. In 1924, Letterer reported the history of a 6-month-old infant presenting with an acute non-leukemoid reaction of the reticuloendothelial system, and Siwe reported a similar case 9 years later. The Letterer–Siwe (LS) disease, involving extra skeletal localizations was defined and thought to be a different entity from HSC [1], [2]. In 1940, the first cases of bony solitary eosinophilic granulomas were reported. Finally, in 1953, Lichenstein gathered those 3 diseases under the name of Histiocytosis X (X meaning that the cause remained unknown).

HSC most often begins in early childhood, after the age of 2 or 3 years, and patients present with diabetes insipidus, bone defects and exophtalmia. LS occurs before the age of 3 years, and skin, lymph nodes, liver and lungs are frequently involved and the prognosis is poor. Eosinophilic granulomas can be solitary as well as multiple, occur most commonly after the age of 20 years and have a good prognosis.

Histologically, Langerhans’ cells were first described in 1868. In 1961, Birbek using electronic microscopy, found the Histiocytosis X body (so called Birbek granule) in the Langerhans’ cells. Basset, in 1965 found intracytoplasmic granules in Histiocytosis X lung lesions and theorized that they were caused by a virus. Eight years later, Basset and Nezelof showed that those granules were the same as the Histiocytosis X bodies noted by Birbek. From this date, Histiocytosis X became Langerhans’ cell histiocytosis (LCH) [1], [2].

As shown through the previous historical overview, LCH can involve all the organs. Head and neck involvement is found in 55–73% of cases and is the third most frequent location after bones and skin [1]. The goal of this retrospective study is to analyze the head and neck presentations and to determine their consequence in the diagnosis, management and outcome of LCH.

Section snippets

Patients and methods

From 1994 to 2009, 42 children with LCH were diagnosed and treated at La Timone Children's Hospital in Marseille, France. 31 of these patients had head and neck manifestations of the disease. They were all managed by a multidisciplinary paediatric staff which consisted of paediatric haematologists, paediatric oncologists and paediatric head and neck surgeons. Charts of these 31 patients were reviewed. The age as well as gender, clinical presentations and outcome after treatment were recorded.

Results

In this series of 31 children with LCH head and neck localization, the sex ratio was 1 M/1.2 F (14 M/17 F) with no statistical difference between the groups. 11 out of these patients (35.5%) presented with primary head and neck disease. Each patient had a mean of 2.4 HN localizations. The most frequent HN localization was the dome of the skull which was involved in 17/31 (55%) (Fig. 1). The skull bones involved were the frontal bone in 11 cases (Fig. 2) and the temporal bone in 7 cases (Fig. 3

Discussion

LCH is a histiocytic proliferative disorder with an unknown etiology. Langerhans’ cells are usually monoclonal except in the single system pulmonary form [1]. This one is often a smoking-induced adult disease and considered as a separate LCH entity [1]. LCH cannot be considered as a usual neoplasm because lesions often regress spontaneously [1], especially when entering adulthood [2]. Some authors found Epstein-Barr virus DNA expression in LCH lesions and suggested that there is a viral

Conclusion

In LCH, head and neck involvement often occurs and is especially located to the cranial bones sometimes mimicking miscellaneous pathologies. Physician has to be aware and not forget LCH when an infant present with a history of chronic otorrhea, with an orbital tumor or a big lymph node mass. Imaging with CT-scan and MRI should be useful for the local evaluation. A general pediatric evaluation is mandatory for a good staging and an adequate treatment. Therapy can include wait-and-see as well as

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