Atlas of Genetics and Cytogenetics in Oncology and Haematology


Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

X Y 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 NA

Langerhans cell sarcoma

Written2019-11Ding-Bao Chen
Department of Pathology, Peking University People's Hospital, Beijing 100044, People's Republic of China; cdingbao@163.com

Abstract Tumours derived from Langerhans cells (LCs) are divided into two main subgroups, according to the degree of cytological atypia and clinical aggressiveness: LC histiocytosis (LCH) and LC sarcoma (LCS). LCS is a high-grade neoplasm with obviously malignant cytologic features and the Langerhans cell phenotype, which is rare. Here the clinic-pathological of LCS will be discussed based on reported cases in the literature.

Keywords  Langerhans cell sarcoma; CD1a; immunophenotype; Cytogenetics

(Note : for Links provided by Atlas : click)

Identity

ICD-Topo C420,C421,C424
ICD-Morpho 9756/3 Langerhans cell sarcoma
Atlas_Id 1731
Other namesDendritic/histiocytic sarcoma,Langerhans cell type
Malignat histiocytosis X

Clinics and Pathology

Disease Tumours derived from Langerhans cells (LCs) are divided into two main subgroups, according to the degree of cytological atypia and clinical aggressiveness: LC histiocytosis (LCH) and LC sarcoma (LCS). Both subgroups maintain the phenotypic profile and ultrastructural features of LC. LCS is a high-grade neoplasm with obviously malignant cytologic features and the Langerhans cell phenotype. Birbeck granules are present, but desmosomes/junctional specializations are absent (Swerdlow, et al ,2008. Swerdlow, et al, 2017. Nakamine, 2016). It is reported that LCS can arise from LCH (Yi, 2019).
Phenotype / cell stem origin The neoplastic cells of LCS may expresses CD1a, langerin, and S100 protein. However, the staining of individual markers may be focal and patchy. The Langerhans cells are derived from mononuclear phagocytes macrophages and dendritic cells) or histiocytes. (Swerdlow, et al ,2008. Swerdlow, et al ,2017)
LCS may arise de novo or be observed in other disorders. Several cases have been reported in myeloproliferative syndromes, other histiocytic disorders, B-lineage leukemia, follicular lymphoma, dermal lentigines, and after liver transplantation. (Zwerdling 2014).
Epidemiology LCS is rare, and the reported cases are mainly in adults. The median age is 39 years ( range, 10-72 years). There is a male predilection, with a male-to- female ratio of 2:1. Rare cases may be associated with follicular lymphoma (West, 2013. Swerdlow, et al ,2008. Swerdlow, et al ,2017 ).
Clinics Most cases involve skin, bone and multifoci, and lymph node in 22%. Other sites include soft tissue, lung, liver and spleen. 44% of disease is high-grade (stage III-IV), Hepatosplenomegly is seen in 22% and pancytopenia in 11% masses (Swerdlow, et al ,2008. Swerdlow, et al ,2017).
Pathology The most prominent feature is the obviously malignant cytology of a pleomorphic tumour, and only the phenotype and/or chromatin is clumped and nucleoli are conspicuous. Some cells may have the complex grooves of the LCH cell, which is an important clue to the diagnosis. The mitotic rate is high, usually > 50 mitoses/10 HPF. Scattered eosinophils can be seen. Birbeck granules are present, whereas desmosomes/junctional specializations are absent.
 
Figure 1. Langerhans cell sarcoma involving the mediastinum of a female. Hypercellular proliferation of cells with oval-shaped nuclei and overt atypia can be seen. Scattered eosinophils are in the background.
 
Figure 2. Langerhans cell sarcoma. Hypercellularity, nuclear atypia, and mitotic cells are noted. The proliferating cells are rather uniform in size having fairly abundant, weakly eosinophilic cytoplasm.
 
Figure 3. The tumor cells are positive for CD1a in the membrane.
 
Figure 4. The proliferation index of tumor cells is high shown by Ki67.
Treatment An optimal treatment strategy for LCS has not been established, owing to its rarity; however, aggressive surgery, chemotherapy, and additional local control with radiation appear to be good option for localized lesions or confined nodal disease (Call, 2013. Zwerdling, 2014). Radiotherapy may be effective in treating minimally invasive LCS lesions (Nakayama, 2010). The successful treatment of advanced LCS with multipleorgan involvement is feasible with a variety of chemotherapeutic regimens. Systemic combination chemotherapy, such as the CHOP or CHOP-like regimens, may be helpful in some cases (Bohn, 2007). Current data indicate that the ESHAP regimen may be partially effective in treating relapsed patients (Keklik, 2013). Etoposide-containing chemotherapy, EPOCH, may be efficacious for LCS due in part to the similar pathogenic mechanisms of LCS with MCC and MCPyV infection, and it may be safe for elderly patients (Matsukawa, 2018).
Prognosis LCS is an aggressive, high-grade malignancy, with > 50% mortality from progressive disease (Chen, 2013). Patients presenting with multisite/multiorgan disease fared very poorly with 64% dead (Zwerdling, 2014).

Cytogenetics

Note One reported case has been found to harbor the BRAF V600E mutation. (Chen, et al., 2013; Swerdlow et al ,2016). Whole genome analysis for copy number changes and loss of heterozygosity showed a complex karyotype with variable hyperdiploidy and numerous allelic imbalances. Significant findings included a homozygous deletion at 9p21 involving the CDKN2A and loss of heterozygosity at 17p involving TP53 gene, coupled with a TP53 missense mutation. (Karai, 2015)

Bibliography

Cutaneous Langerhans cell sarcoma: a case report and review of the literature
Bohn OL, Ruiz-Argüelles G, Navarro L, Saldivar J, Sanchez-Sosa S
Int J Hematol 2007 Feb;85(2):116-20
PMID 17321988
 
Langerhans Cell Sarcoma Arising from Chronic Lymphocytic Lymphoma/Small Lymphocytic Leukemia: Lineage Analysis and BRAF V600E Mutation Study
Chen W, Jaffe R, Zhang L, Hill C, Block AM, Sait S, Song B, Liu Y, Cai D
N Am J Med Sci 2013 Jun;5(6):386-91
PMID 23923114
 
Langerhans cell sarcoma with lineage infidelity/plasticity: a diagnostic challenge and insight into the pathobiology of the disease
Karai LJ, Sanik E, Ricotti CA, Susa J, Sinkre P, Aleodor AA
Am J Dermatopathol 2015 Nov;37(11):854-61
PMID 26368646
 
Langerhans cell sarcoma of the nasopharynx: a rare case
Keklik M, Sivgin S, Kontas O, Abdulrezzak U, Kaynar L, Cetin M
Scott Med J 2013 Nov;58(4):e17-20
PMID 24215052
 
Successful treatment of an elderly Langerhans cell sarcoma patient by EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin) chemotherapy
Matsukawa T, Suto K, Miyoshi H, Oshimi K, Ohshima K, Miyagishima T
J Clin Exp Hematop 2018 Dec 13;58(4):184-187
PMID 30305476
 
Langerhans Cell Histiocytosis and Langerhans Cell Sarcoma: Current Understanding and Differential Diagnosis
Nakamine H, Yamakawa M, Yoshino T, Fukumoto T, Enomoto Y, Matsumura I
J Clin Exp Hematop 2016;56(2):109-118
PMID 27980300
 
Langerhans cell sarcoma of the cervical lymph node: a case report and literature review
Nakayama M, Takahashi K, Hori M, Okumura T, Saito M, Yamakawa M, Tabuchi K, Hara A
Auris Nasus Larynx 2010 Dec;37(6):750-3
PMID 20554413
 
Sleep patterns as predictors for disability pension due to low back diagnoses: a 23-year longitudinal study of Finnish twins
Ropponen A, Silventoinen K, Hublin C, Svedberg P, Koskenvuo M, Kaprio J
Sleep 2013 Jun 1;36(6):891-7
PMID 23729932
 
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.
Swerdlow SH,Campo E,Harris NL,et al,eds.
Lyon :IARC:;2017:470-473. ISBN:978-92-832-4494-3
 
Langerhans cell sarcoma arising from antecedent langerhans cell histiocytosis: A case report
Yi W, Chen WY, Yang TX, Lan JP, Liang WN
Medicine (Baltimore) 2019 Mar;98(10):e14531
PMID 30855438
 
Langerhans cell sarcoma: case report and review of world literature
Zwerdling T, Won E, Shane L, Javahara R, Jaffe R
J Pediatr Hematol Oncol 2014 Aug;36(6):419-25
PMID 24942035
 
Langerhans Cell Sarcoma of the Axillary Lymph Node: A Case Report and Review of the Literature
alli AO, Morgül Y, Alacaciolu , Bener S, Payzin B
Turk J Haematol 2013 Jun 5;30(2):198-203
PMID 26923635
 

Citation

This paper should be referenced as such :
Chen DB
Langerhans cell sarcoma;
Atlas Genet Cytogenet Oncol Haematol. in press
On line version : http://AtlasGeneticsOncology.org/Anomalies/LangerhansCellSarcomaID1731.html



External links

arrayMap (UZH-SIB Zurich)Morph ( 9756/3) -   [auto + random 100 samples .. if exist ]   [tabulated segments]
 
 
REVIEW articlesautomatic search in PubMed
Last year articlesautomatic search in PubMed
All articlesautomatic search in PubMed


© Atlas of Genetics and Cytogenetics in Oncology and Haematology
indexed on : Mon May 11 15:50:42 CEST 2020


Home   Genes   Leukemias   Solid Tumors   Cancer-Prone   Deep Insight   Case Reports   Journals  Portal   Teaching   

For comments and suggestions or contributions, please contact us

jlhuret@AtlasGeneticsOncology.org.