Langerhans cell histiocytosis of bone in children: a clinicopathologic study of 108 cases

Wang, Jia; Wu, Xv; Xi, Zhengjun

doi:10.1007/s12519-010-0205-0

Cited by 25 publications

(23 citation statements)

References 13 publications

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“…These data confirm that a radiographic skeletal survey at the time of LCH diagnosis is important to evaluate extent of bone involvement. 20,22,[25][26][27] For our review, we included lung involvement in our definition of risk organs; however, recently lung involvement alone is no longer considered a negative prognostic factor. 20,28,29 Recent studies have shown that it rarely results in death and, if it does, it is usually secondary to mechanical complications, including pneumothorax or chronic emphysematous change.…”

Section: Discussionmentioning

confidence: 99%

Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Kamath

Arkader

Jubran

2014

Journal of Pediatric Orthopaedics

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Section: Discussionmentioning

confidence: 99%

Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Kamath

Arkader

Jubran

2014

Journal of Pediatric Orthopaedics

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“…While the classical hallmark of the skeletal involvement of ECD is osteosclerosis, occasionally, mixed sclerotic and lytic lesions have been described. It is imperative to recognize that bone lesions found in LCH are rather lytic than sclerotic [31] further complicating the diagnosis in cases which the typical bone changes are a tad less typical, in conjunction with osteolytic lesions. In their retrospective study encompassing 59 cases of ECD in 1996, Veyssier-Belot et al reported that 5%-8% of the patients in the study also had lytic lesions, either on the flat bones, like the ribs and skull, or on the long bones [5].…”

Section: Introductionmentioning

confidence: 99%

Erdheim-Chester Disease: a comprehensive review of the literature

Mazor

Manevich-Mazor

Shoenfeld

2013

Orphanet J Rare Dis

225

309

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Erdheim-Chester Disease (ECD) is a rare form of non Langerhans' cell histiocytosis. Individuals affected by this disease are typically adults between their 5th and 7th decades of life. Males and females are almost equally affected. The multi systemic form of ECD is associated with significant morbidity, which may arise due to histiocytic infiltration of critical organ systems. Among the more common sites of involvement are the skeleton, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum) and skin. The most common presenting symptom of ECD is bone pain. The etiology of ECD is unknown yet thought to be associated with an intense TH1 immune response. It may also be associated with the V600E BRAF mutation, as described in as many as half of the patients in recent studies. Bilateral symmetric increased tracer uptake on 99mTc bone scintigraphy affecting the periarticular regions of the long bones is highly suggestive of ECD. However, definite diagnosis of ECD is established only once CD68(+), CD1a(−) histiocytes are identified within a biopsy specimen. At present, this obscure ailment embodies numerous challenges to medical science. Given its rarity, it is diagnostically elusive and requires a high level of clinical suspicion. Therapeutically, it is of limited alternatives. Currently, interferon-α is the most extensively studied agent in the treatment of ECD and serves as the first line of treatment. Treatment with other agents is based on anecdotal case reports and on the basis of biological rationale. Nevertheless, cladribine (2CDA), anakinra and vemurafenib are currently advocated as promising second line treatments for patients whose response to interferon-α is unsatisfactory. Overall, the 5 year survival of ECD is 68%. Herein, the authors mustered and brought about a panoramic consolidation of all the relevant facts regarding ECD. This work highlights the different clinical, radiological and pathological manifestations associated with ECD, the differential diagnoses, the various treatment options and the acknowledged science explaining the disease.

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“…Patients often are admitted to the orthopedic department because of bone pain and enucleation of bone tumor is performed. LCH is often diagnosed based on postoperative histopathological examination [7]. Unlike eosinophilic granuloma, another form of LCH called Hand-Schüller-Christian disease (HSC) includes diabetes insip- ECD is characterized by an infiltration of lipid-laden macrophages and multinucleated giant cells [9].…”

Section: Introductionmentioning

confidence: 99%

Hand-Schüller-Christian Disease and Erdheim-Chester Disease: Coexistence and Discrepancy

Yin¹,

Zhang²,

Zhang³

et al. 2013

The Oncologist

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Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) share similar clinical features and mechanisms. In very rare circumstances, the two diseases coexist in the same patient. Here we report such a patient, who was first diagnosed with Hand-Schüller-Christian disease (HSC), a type of LCH. Several years later, the patient presented with severe exophthalmos and osteosclerosis on radiograph. New biopsy revealed ECD. We also analyze 54 cases of LCH and 6 cases of ECD diagnosed in our hospital, as well as their progression during a follow-up period of 8 years. In five cases of HSC (9.3% of LCH), a triad of central diabetes insipidus, hyperprolactinemia, and pituitary stalk thickening on magnetic resonance imaging (MRI) preceded the typical bone lesions by 4 -9 years. In addition, LCH was featured as elevated plasma alkaline phosphatase (ALP), which was normal in ECD. Combined with a literature review, several features are summarized to differentiate ECD from HSC. In patients with diabetes insipidus, concomitant hyperprolactinemia and pituitary stalk thickening on MRI indicate a possible HSC. Additionally, if osteosclerosis is observed in a patient with LCH, the coexistence of ECD should be considered. The Oncologist 2013;18:19 -24 Implications for Practice: Central diabetes insipitus (CDI) is usually the first or one of the first symptoms of Hand-Schüller-Christian disease (HSC). It is difficult to determine whether CDI is part of HSC at its onset. We propose a new triad of symptoms including central diabetes insipitus, hyperprolactinemia, and pituitary stalk thickening on MRI. If a patient is present with the triad, HSC should be considered. Bone scans are very useful to reveal HSC in the absence of bone pain. Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are featured with osteolytic lesions and osteosclerosis, respectively. If osteosclerosis is observed in a patient with LCH, coexistence of ECD should be considered. A new biopsy is helpful for the diagnosis.

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Langerhans cell histiocytosis of bone in children: a clinicopathologic study of 108 cases

Cited by 25 publications

References 13 publications

Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Outcomes of Children Younger Than 24 Months With Langerhans Cell Histiocytosis and Bone Involvement

Erdheim-Chester Disease: a comprehensive review of the literature

Hand-Schüller-Christian Disease and Erdheim-Chester Disease: Coexistence and Discrepancy

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