Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

Udaka, Toru; Susa, Michiro; Kikuta, Kazutaka; Nishimoto, Kazumasa; Horiuchi, Keisuke; Sasaki, Aya; Kameyama, Kaori; Nakamura, Masaya; Matsumoto, Morio; Chiba, Kazuhiro; Morioka, Hideo

doi:10.1159/000441415

Cited by 7 publications

(10 citation statements)

References 13 publications

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“…The chest wall, particularly the clavicle and sternum, was the second most common site involved after the skull. We could not find exact data on clavicular involvement, but some studies have reported its incidence in LCH disease [ 7 , 9 , 37 – 39 ]. Consistent with the findings of these studies, we only found five cases of clavicular involvement.…”

Section: Discussionmentioning

confidence: 94%

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Reisi

Raeissi

Khalilabad

et al. 2021

Orphanet J Rare Dis

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Background Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs. Objective This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis. Methods We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included. Results We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid–fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups. Conclusions LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

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Section: Discussionmentioning

confidence: 94%

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Reisi

Raeissi

Khalilabad

et al. 2021

Orphanet J Rare Dis

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“…The typical imaging findings of LCH flat and irregular bone lesions reported in the literature are mainly dilated or lytic bone destruction. Rib lesions are often accompanied by pathological fractures, and soft tissue extension may cause extrapleural masses [ 24 , 25 ]. In our series, only 21.4% (3/14) of the cases were associated with pathological fractures, and 35.7% (5/14) were associated with soft tissue masses.…”

Section: Discussionmentioning

confidence: 99%

Radiologic findings that aid in the reduction of misdiagnoses of Langerhans cell histiocytosis of the bone: a retrospective study

et al. 2021

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Background This study aimed to identify the characteristic radiological signs for the diagnosis of Langerhans cell histiocytosis (LCH) of the bone. Methods We retrospectively studied 82 cases of LCH with bone lesions confirmed by pathology. Clinical and radiological features of the patients were analyzed. Results A total of 64 and 18 patients had single and multiple bone lesions, respectively. With regard to LCH with single bone lesions, 37.5% (24/64) of lesions were located in the skull and presented as bone destruction with or without soft tissue mass. The correct diagnosis rate of these lesions was 60.0% (9/15) in children and adolescents, but was only 22.2% (2/9) in adults. A total of 26.5% (17/64) of the solitary lesions were found in the spine. Of these, 88.2% (15/17) were located in the vertebral body and appeared to have different degrees of collapse, and 66.7% (10/15) of these lesions were correctly diagnosed. Of the unifocal lesions, 21.8% (14/64) were located in other flat and irregular bones and manifested as osteolysis. Only 21.4% (3/14) of these cases were correctly diagnosed. In total, 14.1% (9/64) of the isolated bone LCH lesions were located in the long bones. Of these, 77.8% (7/9) were located in the diaphysis and presented as central bone destruction with or without fusiform periosteal reaction and extensive peripheral edema, of which 42.9% (3/7) were correctly diagnosed before surgery or biopsy. With regard to LCH with multiple bony destructive lesions, 71.4% (10/14) of cases in children and adolescents were correctly diagnosed; however, all four cases among adults were misdiagnosed. Conclusion In all age groups, isolated diaphyseal destruction of the long bone with fusiform periosteal reaction and extensive peripheral edema, vertebra plana of the spine, and bevelled edge of skull defects accompanied by soft tissue masses strongly suggest LCH diagnosis. Moreover, the multiple bone osteolytic destruction in children and adolescents strongly suggests LCH diagnosis. Familiarity with these typical radiological signs of LCH is necessary to decrease misdiagnoses.

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“…In cases that Ukada et al reported in 2015, symptoms were relieved by bone curettage combined with biopsy [ 13 ] and in cases Shaowu et al reported in 2014, internal fixation with plate was performed on clavicles in addition to bone curettage with biopsy [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

Langerhans cell histiocytosis in the adult clavicle: A case report

Song

Nam

Lee

et al. 2020

International Journal of Surgery Case Reports

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Highlights Langerhans` Cell Histiocytosis is a rare disease involving proliferation of Langerhans-type cells, which shares immunophenotypic and ultrastructural similarities. LCH occurs dominantly in pediatric ages and involve cranium, pelvis, vertebrae, ribs, tubular bones and so forth. It is diagnosed by the means of histologic, immunochemical analysis. We encountered a very rare case of solitary LCH in an adult’s clavicle. We concluded LCH must be suspected as a diagnosis and ruled out when encountering primary tumorous lesion.

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Langerhans Cell Histiocytosis of the Clavicle in an Adult: A Case Report and Review of the Literature

Cited by 7 publications

References 13 publications

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Unusual sites of bone involvement in Langerhans cell histiocytosis: a systematic review of the literature

Radiologic findings that aid in the reduction of misdiagnoses of Langerhans cell histiocytosis of the bone: a retrospective study

Langerhans cell histiocytosis in the adult clavicle: A case report

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