Histiocytosis X (Eosinophilic Granuloma of Bone, Letterer-Siwe Disease, and Schüller-Christian Disease)

Lichtenstein, Louis

doi:10.2106/00004623-196446010-00007

Cited by 302 publications

(198 citation statements)

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“…It is now well established that the three syndromes eosinophilic granuloma, Hand-Schuiller-Christian disease and Letterer-Siwe disease--are closely related clinically and pathologically (Lichtenstein, 1953(Lichtenstein, , 1964 Birch, in preparation). Epithelial tumours, which are so frequent in adults, are extremely uncommon in childhood.…”

Section: Resultsmentioning

confidence: 99%

Incidence of malignant disease in childhood: a 24-year review of the Manchester Children's Tumour Registry data

Birch¹,

Marsden²,

Swindell³

1980

Br J Cancer

106

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The Manchester Children's Tumour Registry data for the period 1954-1977 have been analysed. The overall incidence of malignant disease in children aged 0-14 years in the north-west of England is estimated to be 100 per million person-years. The most common disease group is leukaemia, which forms about one third of the total number of cases. Among solid tumours, by far the most common presenting site is the central nervous system, representing nearly a quarter of all neoplasms. Wilms' tumour, neuroblastoma and soft-tissue sarcomas comprise approximately 5%, 6.5% and 6% respectively of the total. The tumours most frequently seen in adults (e.g. carcinoma of colon, lung and breast) are extremely rare in childhood. A significant excess of males was seen in acute lymphoid leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, medulloblastoma and hepatoblastoma. A female excess was found among germ-cell tumours. During the study period significant increases in incidence were seen among acute lymphoid leukaemia and epithelial tumours, and an increase in germ cell tumours approached significance.

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Section: Resultsmentioning

confidence: 99%

Incidence of malignant disease in childhood: a 24-year review of the Manchester Children's Tumour Registry data

Birch¹,

Marsden²,

Swindell³

1980

Br J Cancer

106

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“…Acute disseminated LCH (Letterer-Siwe disease), usually seen in children Ͻ3 years of age, can affect multiple organs and systems with a lethal outcome. 95 When the spine is involved, LCH most frequently presents with local back pain; the thoracic vertebrae is the most commonly affected region (54%), followed by the lumbar spine (35%) and cervical spine (11%). There may be associated leukocytosis and fever.…”

mentioning

confidence: 99%

Imaging of Back Pain in Children

Rodriguez¹,

Poussaint²

2009

AJNR Am J Neuroradiol

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SUMMARY:While back pain presents less frequently in children than in adults, it still poses a significant clinical challenge with respect to making a firm diagnosis and developing an effective treatment plan. When children have back pain and medical attention is sought, an underlying pathology is usually suspected. Pediatric patients are evaluated, first, with a complete clinical history and examination and, second, by an imaging work-up that is based on initial findings, including the child's age and size, signs and symptoms, and suspected etiology. This article describes 1) the epidemiology of back pain in children, 2) the imaging work-up used, and 3) the correlation of imaging findings with disease entities that may cause back pain in the pediatric patient. The list of diseases giving rise to back pain is not meant to be exhaustive but rather reflective of the most commonly identified pathologies and disorders among young children and adolescents, from athletic injuries to lethal cancers.ABBREVIATIONS: ALARA ϭ as low as reasonably achievable; ESR ϭ erythrocyte sedimentation rate; FSE ϭ fast spin-echo; 18 F ϭ fluorine 18; LCH ϭ Langerhans cell histiocytosis; LDH ϭ lactate dehydrogenase; MBP ϭ mechanical back pain; PET ϭ positron-emission tomography; RFA ϭ radio-frequency ablation, SPECT ϭ single-photon emission computed tomography; STIR ϭ short tau inversion recovery; 99m Tc-MDP ϭ technetium 99m methylene diphosphonate; WHO ϭ World Health Organization B ack pain in children presents less frequently than in adults. The incidence of back pain in adults has been estimated to be as high as 60%-80% 1 ; the actual incidence of back pain in children, however, is unknown. Back pain in children presenting to the emergency department was originally thought to be an uncommon complaint, 2 reflecting significant underlying pathology compared with adults. Although recent studies have shown an increased prevalence of back pain in children, [3][4][5][6][7] relatively few patients receive medical attention.

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“…It was first described as histiocytosis X in 1953 as a triad. This triad includes the solitary osseous lesion called an "Eosinophilic Granuloma"; eosinophilic granuloma with clinical symptoms of osteolysis, exophthalmus, diabetes insipitus called "Hand-Schuller-Christian Disease", and the malignant form of dissemination called "Letterer-Siwe Disease" (9). All of these clinical entities feature an abnormal proliferation of histiocytic cell type, that is, Langerhans cells (8,10).…”

Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis of the cervical spine in an adult: a case report

Sayhan

Altinel²,

Erguden³

et al. 2009

Turkish Neurosurgery

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We present a case of a 47-year-old-woman with a complaint of cervical pain with paresthetic appearance on her left arm. She was treated with analgetics. Further radiological evaluation because of the persistent pain revealed an osteolytic destruction of the fourth cervical vertebra. The patient underwent anterior cervical corpectomy with total excision of the tumor. Stabilization of the cervical spine was performed. Histology confirmed the diagnosis of Langerhans cell histiocytosis (LCH) of the cervical spine. This case report presents the histopathological evaluation, diagnostic work-up and the treatment procedures, because of rarity of cervical spinal LCH cases in the literature.

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Histiocytosis X (Eosinophilic Granuloma of Bone, Letterer-Siwe Disease, and Schüller-Christian Disease)

Cited by 302 publications

References 0 publications

Incidence of malignant disease in childhood: a 24-year review of the Manchester Children's Tumour Registry data

Incidence of malignant disease in childhood: a 24-year review of the Manchester Children's Tumour Registry data

Imaging of Back Pain in Children

Langerhans cell histiocytosis of the cervical spine in an adult: a case report

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