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

Chen, Ding-Bao

Atlas of Genetics and Cytogenetics in Oncology and Haematology

English

I-Revues

Leukaemia Section 
Short Communication 
Atlas Genet Cytogenet Oncol Haematol. 2020; 24(10) 369 
Atlas of Genetics and Cytogenetics 
in Oncology and Haematology 
INIST-CNRS OPEN ACCESS JOURNAL 
Langerhans cell sarcoma 
Ding-Bao Chen 
Department of Pathology, Peking University People's Hospital, Beijing 100044, People's Republic of 
China; cdingbao@163.com 
Published in Atlas Database: November 2019 
Online updated version : http://AtlasGeneticsOncology.org/Anomalies/LangerhansCellSarcomaID1731.html 
Printable original version : http://documents.irevues.inist.fr/bitstream/handle/2042/70818/11-2019-LangerhansCellSarcomaID1731.pdf 
DOI: 10.4267/2042/70818
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 2.0 France Licence. 
© 2020 Atlas of Genetics and Cytogenetics in Oncology and Haematology 
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 
Identity 
Other names 
Dendritic/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). 
Langerhans cell sarcoma Chen DB 
 
 
 
 
 
Atlas Genet Cytogenet Oncol Haematol. 2020; 24(10) 370 
 
 
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. 
Langerhans cell sarcoma Chen DB 
 
 
 
 
 
Atlas Genet Cytogenet Oncol Haematol. 2020; 24(10) 371 
 
 
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. 
 
Langerhans cell sarcoma Chen DB 
Atlas Genet Cytogenet Oncol Haematol. 2020; 24(10) 372 
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. 
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 
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) 
References 
Bohn OL, Ruiz-Argüelles G, Navarro L, Saldivar J, 
Sanchez-Sosa S. Cutaneous Langerhans cell sarcoma: a 
case report and review of the literature. Int J Hematol. 2007 
Feb;85(2):116-20 
Chen W, Jaffe R, Zhang L, Hill C, Block AM, Sait S, Song 
B, Liu Y, Cai D. Langerhans Cell Sarcoma Arising from 
Chronic Lymphocytic Lymphoma/Small Lymphocytic 
Leukemia: Lineage Analysis and BRAF V600E Mutation 
Study. N Am J Med Sci. 2013 Jun;5(6):386-91 
Karai LJ, Sanik E, Ricotti CA, Susa J, Sinkre P, Aleodor AA. 
Langerhans cell sarcoma with lineage infidelity/plasticity: a 
diagnostic challenge and insight into the pathobiology of the 
disease. Am J Dermatopathol. 2015 Nov;37(11):854-61 
Keklik M, Sivgin S, Kontas O, Abdulrezzak U, Kaynar L, 
Cetin M. Langerhans cell sarcoma of the nasopharynx: a 
rare case Scott Med J  2013 Nov;58(4):e17-20 
Matsukawa T, Suto K, Miyoshi H, Oshimi K, Ohshima K, 
Miyagishima T. Successful treatment of an elderly 
Langerhans cell sarcoma patient by EPOCH (etoposide, 
prednisone, vincristine, cyclophosphamide, and 
doxorubicin) chemotherapy J Clin  Exp Hematop  2018 Dec 
13;58(4):184-187 
Nakamine H, Yamakawa M, Yoshino T, Fukumoto T, 
Enomoto Y, Matsumura I. Langerhans Cell Histiocytosis 
and Langerhans Cell Sarcoma: Current Understanding  and 
Differential Diagnosis J Clin Exp Hematop  2016;56(2):109-
118 
Nakayama M, Takahashi K, Hori M, Okumura T, Saito M, 
Yamakawa M, Tabuchi K, Hara A. Langerhans cell sarcoma 
of the cervical lymph node: a case report and literature 
review Auris Nasus Larynx  2010 Dec;37(6):750-3 
Ropponen A, Silventoinen K, Hublin C, Svedberg P, 
Koskenvuo M, Kaprio J. Sleep patterns as predictors for 
disability pension due to low back diagnoses: a 23-year 
longitudinal study of Finnish twins Sleep  2013 Jun 
1;36(6):891-7 
Swerdlow SH,Campo E,Harris NL,et al,eds.. WHO 
Classification of Tumours of Haematopoietic and Lymphoid 
Tissues. Lyon :IARC:;2017:470-473. ISBN:978-92-832-
4494-3 
Yi W, Chen WY, Yang TX, Lan JP, Liang WN. Langerhans 
cell sarcoma arising from antecedent langerhans cell 
histiocytosis: A case report Medicine (Baltimore)  2019 
Mar;98(10):e14531 
Zwerdling T, Won E, Shane L, Javahara R, Jaffe R. 
Langerhans cell sarcoma: case report and review of world 
literature J Pediatr Hematol Oncol  2014 Aug;36(6):419-25 
alli AO, Morgül Y, Alacaciolu , Bener S, Payzin B. 
Langerhans Cell Sarcoma  of the Axillary Lymph Node: A 
Case Report and Review of the Literature Turk J Haematol 
2013 Jun 5;30(2):198-203 
This article should be referenced as such: 
Chen DB. Langerhans cell sarcoma. Atlas Genet 
Cytogenet Oncol Haematol. 2020; 24(10):369-372. 


Langerhans cell sarcoma

http://documents.irevues.inist.fr/bitstream/2042/70818/1/11-2019-LangerhansCellSarcomaID1731.pdf

Langerhans cell sarcoma

Abstract

Similar works

Full text

Available Versions

I-Revues