Langerhans cell sarcoma: A systematic review

Howard, James; Dwivedi, Raghav C.; Masterson, Liam; Jani, Piyush

doi:10.1016/j.ctrv.2015.02.011

Cited by 53 publications

(79 citation statements)

References 47 publications

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“…LCS, once called malignant histiocytosis X, is considered a dendritic cell‐derived tumor with Langerhans cell differentiation according to the 2016 World Health Organization (WHO) classification . Howard et al systematically reviewed LCS, noting its extreme rarity, with approximately 70 reported cases . The median age was 41 years (range: 10–72 years) with 86.4% occurring in adults .…”

Section: Discussionmentioning

confidence: 99%

“…Langerhans cell sarcoma (LCS) is an extremely rare malignant dendritic cell neoplasm with a Langerhans cell immunophenotype . It commonly affects skin and underlying subcutaneous tissue and can also present in lymph nodes, lung, liver, spleen, bone, or multiple organs simultaneously . Histologically, there is an infiltrative proliferation of histiocytoid cells with more marked nuclear pleomorphism and atypia than that in Langerhans cell histiocytosis.…”

Section: Introductionmentioning

confidence: 99%

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Liquid‐based cytology in the diagnosis of Langerhans cell sarcoma: A case report

Iwasaki

Sakai

Mori

et al. 2018

Diagnostic Cytopathology

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Langerhans cell sarcoma (LCS) is an extremely rare malignant dendritic cell neoplasm with Langerhans cell differentiation. Conventional cytology, based on cell morphology alone, cannot render a cytological diagnosis of LCS because immunochemical analysis is essential to identify the Langerhans cell immunophenotype. We present a case illustrating the value of liquid-based cytology with immunocytochemistry as compared with conventional cytology, along with histological and immunohistochemical findings. A 92-year-old woman presented with a 1-month history of progressive right cervical lymphadenopathy. Cytology of a fine needle aspiration sample from the right superior internal jugular lymph node revealed proliferation of atypical, pleomorphic, and histiocytoid cells with one or more irregular-shaped nuclei. Compared with conventional cytology, liquid-based cytology demonstrated more clustered and spatial cells, slightly less marked nuclear atypia, more intense light green staining of the cytoplasm, and a clearer background. Immunocytochemical analysis of the abnormal cells revealed expression of vimentin, CD1a, langerin, CD68, and S-100. The combined morphologic and immunocytochemical results strongly indicated LCS. Histological and immunohistochemical examination of a subsequent excisional biopsy specimen closely coincided with the results of liquid-based cytology. Thus, this technique, including the use of immunocytochemistry, is very useful and valuable for the pathological diagnosis of nonepithelial and hematopoietic neoplasms. There are subtle but considerable differences in cell morphology between conventional and liquid-based cytology; these differences include clusterability, spatial findings, dyeability, and atypism as illustrated in this case of LCS.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Liquid‐based cytology in the diagnosis of Langerhans cell sarcoma: A case report

Iwasaki

Sakai

Mori

et al. 2018

Diagnostic Cytopathology

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“…LCS is a fatal disease, but little is known about this disease due to its scarcity. According to a report by Howard et al [4] which summarized 66 cases of LCS in 2015, the most common site of involvement in LCS was the lymph nodes (74.2%), followed by the skin (48.5%), lung (28.8%), and liver (16.7%). Its overall 5-year disease-specific survival was only 28%.…”

Section: Discussionmentioning

confidence: 99%

A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing

Kim¹,

Choi

Han³

et al. 2017

Acta Haematol

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Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) are clonal proliferations of Langerhans-type cells. Unlike in LCH, the pathophysiology and clinical course of LCS are unclear due to its rarity. Here, we report the case of a 73-year-old male patient who was diagnosed with cutaneous LCH and pulmonary LCS at the same time. Pathological review of these 2 tumors revealed similar immunohistochemical findings. However, the tumor cells in LCS had more aggressive cytological features than those in LCH. Results of BRAF mutation analysis using real-time PCR were negative for both tumors. In whole-exome sequencing (WES), stop-gain mutations in TP53 gene were discovered only in LCS cells. The mechanism of development of LCS from various progenitor cells is currently unclear. According to the results of the WES study, changes in TP53 gene might have contributed to the malignant features of LCS.

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“…In contrast to Langerhans cell histiocytosis (LCH), LCS is often observed in adults, and in general has a poor prognosis . The 5‐year disease specific survival is reported to be 28% . Although there have been reports on the effect of multimodality therapy that combines chemotherapy and radiation therapy, there is currently no specific chemotherapy regimen recommended …”

mentioning

confidence: 99%

“…The 5‐year disease specific survival is reported to be 28% . Although there have been reports on the effect of multimodality therapy that combines chemotherapy and radiation therapy, there is currently no specific chemotherapy regimen recommended …”

mentioning

confidence: 99%

Synchronous case of follicular lymphoma and Langerhans cell sarcoma in the same lymph node

Shimono

Miyoshi

Abe

et al. 2018

Pathology International

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Langerhans cell sarcoma (LCS) is a very rare histiocytic and dendritic cell neoplasm originating from Langerhans cells. There are case reports of histiocytic and dendritic cell neoplasms synchronously or sequentially observed in patients with malignant lymphoma. We present a case in which LCS and follicular lymphoma (FL) grade 3a were observed within the same lymph node. A 66‐year‐old male visited our hospital with a general malaise. Pleural effusion and systemic lymph adenopathy were detected. Biopsy of an inguinal lymph node was performed. The lymph node had regions with follicular structure and regions with acidophilic cytoplasm and large proliferating atypical cells. The tumor cells in the regions with follicular structure showed positivity for CD20 and BCL2 consistent with an FL grade 3a diagnosis. The tumor cells in the regions without follicular structure showed positivity for CD1a and S‐100 and were consistent with an LCS diagnosis. Both tumor cells showed the positivity of BCL6 split by FISH. PCR detected IgH clonality in DNA collected from each region, and direct sequence analysis of cells from both tumors detected almost identical amino acid sequences. This finding is important for future research on the development of very rare histiocytic and dendritic cell neoplasms.

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Langerhans cell sarcoma: A systematic review

Cited by 53 publications

References 47 publications

Liquid‐based cytology in the diagnosis of Langerhans cell sarcoma: A case report

Liquid‐based cytology in the diagnosis of Langerhans cell sarcoma: A case report

A Case of Pulmonary Langerhans Cell Sarcoma Simultaneously Diagnosed with Cutaneous Langerhans Cell Histiocytosis Studied by Whole-Exome Sequencing

Synchronous case of follicular lymphoma and Langerhans cell sarcoma in the same lymph node

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