Multisystem Langerhans cell histiocytosis: Literature review and case report

Cong, Cung-Van; Duc, Nguyen Minh

doi:10.1016/j.radcr.2022.02.024

Cited by 6 publications

(3 citation statements)

References 21 publications

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“…This group of pediatric patients showed a variety of clavicular issues across different age ranges. In the 0-3 years group, eosinophilic granuloma was the most common, which matches what we know about eosinophilic granuloma occurring in early childhood [9]. We also found Kaposiform hemangioendothelioma in this age group, which is typically known to appear early in life [10], but its occurrence in the clavicle is a new finding.…”

Section: Discussionsupporting

confidence: 86%

Clinical Characteristics of Pediatric Clavicular Lesions: A Retrospective Analysis of 20 Cases

Jiang,

Li,

Guo

et al. 2024

Cureus

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Background This research aims to study the diagnostic patterns, anatomical locations, and age-related trends in pediatric clavicular lesions, filling a gap in pediatric-specific data for these conditions. Methodology A retrospective study of 20 pediatric patients (aged ≤14 years) with clavicular lesions was conducted based on inclusion and exclusion criteria emphasizing confirmed diagnosis and treatment specifics. The diagnostic process relied on open biopsy, followed by excision or curettage and histopathological examination. Results The study primarily involved patients with an average age of 7.1 ± 3.8 years. Eosinophilic granuloma was the most common diagnosis (30% of cases), particularly in the age group of 0-3 years. Clavicular lesions predominantly manifested as either a palpable lump or localized swelling with pain. The medial of the clavicle was the most frequent lesion location. No malignant tumors were found, and the functional outcomes post-treatment were satisfactory. Conclusions Pediatric clavicular lesions exhibit distinct diagnostic and anatomical characteristics compared to adults. Eosinophilic granuloma is significantly prevalent in early childhood, necessitating age-specific diagnostic and therapeutic approaches. The study advocates for multidisciplinary collaboration in the treatment and improved understanding of these lesions, which are vital for pediatric orthopedic oncology.

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Section: Discussionsupporting

confidence: 86%

Clinical Characteristics of Pediatric Clavicular Lesions: A Retrospective Analysis of 20 Cases

Jiang,

Li,

Guo

et al. 2024

Cureus

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“…The formation of early-stage granulomas in the lung is easy to observe. However, nodule formation in other organs, especially solid organs, can be difficult to detect on imaging, despite the similar nature of the lesions (20). Owing to the rarity of LCH in adults, coupled with its nonspecific and diverse clinical presentation, it often leads to missed and delayed diagnosis (3,21).…”

Section: Discussionmentioning

confidence: 99%

Multi-System Langerhans Cell Histiocytosis as a Mimic of IgG4-Related Disease: A Case Report and Literature Review

et al. 2022

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Langerhans cell histiocytosis (LCH) is a rare disease characterized by the clonal accumulation and/or proliferation of specific dendritic cells resembling normal epidermal Langerhans cells (LCs). Clinical manifestations are variable, depending on the affected tissues or organs, however, LCH with elevated serum IgG4 has not been reported. Herein, we reported a 26-year-old Chinese female multi-system LCH (MS-LCH) who first presented with central diabetes insipidus (CDI), accompanied by panhypopituitarism and hepatic dysfunction. Diagnostic investigations were strongly suspicious of IgG4-RD because of elevated serum IgG4 levels during the process. Furtherly, thyroid and lymph node involvement and biopsy led to the diagnosis of MS-LCH; the strongly positive staining of CD1a, S100, CD207 (langerin), and Ki67 was found. Moreover, after systemic treatment with five cycles of chemotherapy, many lesions were greatly improved. Since both LCH and IgG4-RD are orphan diseases that can affect any organ, the differential diagnosis is challenging, especially when LCH is associated with unexplained serum IgG4 elevation. In this article, the case of a young woman suffering from MS-LCH that affected organs including the pituitary, thyroid, lymph node, and liver was summarized, and relevant literature was reviewed to better equip the diagnosis and treatment in its early stages.

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“…About 70 % of LCH cases are confined to the single-organ system at the time of diagnosis. However, some patients develop multi-system LCH, often with protracted and debilitating clinical course [8] . Medical management of patients with LCH is quite heterogeneous, and includes close observation, surgery, radiotherapy and pharmacological interventions: systemic and topical chemotherapy, glucocorticoids, non-steroidal anti-inflammatory drugs such as indomethacin, and signaling pathway inhibitors [9] .…”

Section: Introductionmentioning

confidence: 99%

Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

Hu¹,

Buhtoiarov²,

Wang³

et al. 2022

Journal of Bone Oncology

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Multisystem Langerhans cell histiocytosis: Literature review and case report

Cited by 6 publications

References 21 publications

Clinical Characteristics of Pediatric Clavicular Lesions: A Retrospective Analysis of 20 Cases

Clinical Characteristics of Pediatric Clavicular Lesions: A Retrospective Analysis of 20 Cases

Multi-System Langerhans Cell Histiocytosis as a Mimic of IgG4-Related Disease: A Case Report and Literature Review

Langerhans Cell Histiocytosis: A Population-based Study of Anatomical Distribution and Treatment Patterns

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