Langerhans Cell Sarcoma

Rare malignant neoplasm of dendritic LANGERHANS CELLS exhibiting atypical cytology, frequent mitoses, and aggressive clinical behavior. They can be distinguished from other histiocytic and dendritic proliferations by immunohistochemical and ultrastructure studies. Cytologically benign proliferations of Langerhans cells are called LANGERHANS CELL HISTIOCYTOSIS.

Also Known As:

Networked: 26 relevant articles (3 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Precursor Cell Lymphoblastic Leukemia-Lymphoma (Acute Lymphoblastic Leukemia)
2.	Hairy Cell Leukemia
3.	Neoplasms (Cancer)
4.	Langerhans-Cell Histiocytosis (Histiocytosis, Langerhans Cell)
5.	Erdheim-Chester Disease

Experts

1.	Shen, Chentian: 1 article (04/2022)
2.	Sun, Zhenkui: 1 article (04/2022)
3.	Abeykoon, Jithma P: 1 article (01/2022)
4.	Go, Ronald S: 1 article (01/2022)
5.	Goyal, Gaurav: 1 article (01/2022)
6.	Kozelsky, Timothy F: 1 article (01/2022)
7.	Ravindran, Aishwarya: 1 article (01/2022)
8.	Rech, Karen L: 1 article (01/2022)
9.	Young, Jason R: 1 article (01/2022)
10.	Zanwar, Saurabh: 1 article (01/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Langerhans Cell Sarcoma:

1.	pembrolizumabIBA 01/01/2022 - "Prolonged remission with pembrolizumab and radiation therapy in a patient with multisystem Langerhans cell sarcoma."
2.	DNA (Deoxyribonucleic Acid)IBA 09/01/2010 - "Molecular studies of IGH- and TCRG-gene rearrangements were performed with DNA from the Langerhans' cell sarcoma and the cryopreserved cells from the acute B-lymphoblastic leukemia. " 11/01/2015 - "The authors describe the first known case of a primary cutaneous Langerhans cell sarcoma with lineage infidelity and use comparative genomic hybridization to investigate the genetic composition of the tumor and detect DNA copy number alterations throughout its entire genome. "
3.	Neural Cell Adhesion Molecules (Neural Cell Adhesion Molecule)IBA 05/01/2005 - "CD56/NCAM-positive Langerhans cell sarcoma: a clinicopathologic study of 4 cases." 05/01/2005 - "This report concerns the clinicopathologic features of 4 patients with CD56/neural cell adhesion molecule (NCAM)-positive Langerhans cell sarcoma (LCS). "
4.	Immunoglobulin Heavy Chains (Immunoglobulin Heavy Chain)IBA 06/01/2012 - "The bone marrow biopsy containing hairy cell leukemia and skin biopsies of Langerhans cell sarcoma were evaluated by routine histology, immunohistochemistry, flow cytometric immunophenotyping and PCR-based gene rearrangement studies of the immunoglobulin heavy chain and kappa genes. "
5.	S100 Proteins (S 100 Protein)IBA 06/01/2008 - "A 57-year-old man became aware of left supraclavicular lymph node swelling, which was subsequently diagnosed as Langerhans cell sarcoma, based on a positive immunophenotype for CD1a, S-100 protein, and langerin, and histologically bizarre pleomorphism. " 06/01/2010 - "The Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) samples were positive for S-100 protein, CD1a, Langerin, fascin, DEC-205 and DC-SIGN. "
6.	DesminIBA 11/13/2019 - "The tumor cells were positive for CD68, S100, CD4 and lymphocyte common antigen, while epithelial membrane antigen, HMB-45, CK AE1/AE3, myogenin, desmin, CD1a, CD21 and SALL-4 were negative thus ruling out rhabdomyosarcoma, extrarenal rhabdoid tumor, Langerhans cell sarcoma and malignant melanoma." 06/01/2016 - "H and E stained sections revealed a Langerhans cell sarcoma, and immunohistochemistry was performed additionally; CD68, CD163, CD14, fascin, HLA-DR, lysozyme, S100 CD1a and langerin showed a positive reaction, while CD20, CD30, CD34, CD31, pan-cytokeratin, AE/1AE3, SMA, desmin, EMA, ERG, INI-1, CD21, CD4, PLAP, MPO and CD117c were negative. "
7.	AntigensIBA 11/01/2015 - "Langerhans cell sarcoma is a very rare and aggressive tumor of Langerhans cell lineage, for which aberrant expression of T-cell-related antigens has not yet been reported in a primary skin tumor. " 11/13/2019 - "The tumor cells were positive for CD68, S100, CD4 and lymphocyte common antigen, while epithelial membrane antigen, HMB-45, CK AE1/AE3, myogenin, desmin, CD1a, CD21 and SALL-4 were negative thus ruling out rhabdomyosarcoma, extrarenal rhabdoid tumor, Langerhans cell sarcoma and malignant melanoma."
8.	dabrafenibIBA 12/10/2015 - "Dramatic transient improvement of metastatic BRAF(V600E)-mutated Langerhans cell sarcoma under treatment with dabrafenib." 12/01/2016 - "Sustained response to salvage therapy for dabrafenib-resistant metastatic Langerhans cell sarcoma."
9.	fascinIBA 06/01/2010 - "The Langerhans cell histiocytosis (LCH) and Langerhans cell sarcoma (LCS) samples were positive for S-100 protein, CD1a, Langerin, fascin, DEC-205 and DC-SIGN. " 06/01/2016 - "H and E stained sections revealed a Langerhans cell sarcoma, and immunohistochemistry was performed additionally; CD68, CD163, CD14, fascin, HLA-DR, lysozyme, S100 CD1a and langerin showed a positive reaction, while CD20, CD30, CD34, CD31, pan-cytokeratin, AE/1AE3, SMA, desmin, EMA, ERG, INI-1, CD21, CD4, PLAP, MPO and CD117c were negative. "
10.	Mucin-1 (CA 15-3 Antigen)IBA 11/13/2019 - "The tumor cells were positive for CD68, S100, CD4 and lymphocyte common antigen, while epithelial membrane antigen, HMB-45, CK AE1/AE3, myogenin, desmin, CD1a, CD21 and SALL-4 were negative thus ruling out rhabdomyosarcoma, extrarenal rhabdoid tumor, Langerhans cell sarcoma and malignant melanoma."

Therapies and Procedures

1.	Therapeutics 06/01/2008 - "ESHAP therapy effective in a patient with Langerhans cell sarcoma." 04/01/2012 - "To explorer the clinical features, diagnosis and therapy of Langerhans cell sarcoma (LCS). " 08/01/2014 - "Langerhans Cell Sarcoma in a Chronic Myelogenous Leukemia Patient Undergoing Imatinib Mesylate Therapy: A Case Study and Review of the Literature."
2.	Radiotherapy 01/01/2010 - "We present the case of a patient with progressive Langerhans cell sarcoma whose cutaneous lesions and nodal masses were treated with palliative radiotherapy. " 01/01/2010 - "Langerhans cell sarcoma: Response to radiotherapy." 01/01/2022 - "Prolonged remission with pembrolizumab and radiation therapy in a patient with multisystem Langerhans cell sarcoma."
3.	Hematopoietic Stem Cell Transplantation 01/01/2019 - "[Durable remission of Langerhans cell sarcoma attained by autologous hematopoietic stem cell transplantation following surgical resection]."
4.	Drug Therapy (Chemotherapy) 01/01/2014 - "His LCH was refractory to conventional chemotherapy, and thus worsened to Langerhans cell sarcoma (LCS) in May 2011. " 03/01/2009 - "Chemotherapy for Langerhans cell sarcoma." 01/01/2016 - "[Successful treatment with total cranial irradiation for central nervous system involvement of Langerhans cell sarcoma during chemotherapy]." 11/01/2013 - "We chose the 2-chlorodeoxyadenosine (2-CDA) regimen as first-line chemotherapy, and clinical improvement of Langerhans cell sarcoma was obtained. " 12/13/2018 - "Successful treatment of an elderly Langerhans cell sarcoma patient by EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) chemotherapy."
5.	Breast Implants (Breast Implant) 01/02/2023 - "The patient initially presented with leakage of bilateral breast implants as discovered by a positron emission tomography (PET)-computerized tomography (CT) scan performed as part of a workup for small bowel Langerhans cell sarcoma metastases. "