Langerhans cell sarcoma involving skin and showing epidermotropism: A comprehensive review.


Journal

Journal of cutaneous pathology
ISSN: 1600-0560
Titre abrégé: J Cutan Pathol
Pays: United States
ID NLM: 0425124

Informations de publication

Date de publication:
Apr 2021
Historique:
revised: 25 06 2020
received: 04 05 2020
accepted: 05 07 2020
pubmed: 10 7 2020
medline: 3 11 2021
entrez: 10 7 2020
Statut: ppublish

Résumé

Langerhans cell sarcoma (LCS) is rare and aggressive; patients have an overall survival rate of less than 50%. We present a 62-year-old man with a history of superficial spreading melanoma of the upper back with sentinel lymph node metastasis, Langerhans cell histiocytosis, and LCS. The patient presented with erythematous papules and scaly areas on his face, neck, arms, chest, abdomen, and legs. A skin biopsy revealed a proliferation of large neoplastic cells involving the dermis and with epidermotropism. These cells had atypical bean-shaped nuclei, with ample cytoplasm and abundant mitotic figures including atypical forms. Immunohistochemical studies showed the tumor to be diffusely positive for CD1a, S100 protein, and langerin (CD207) and negative for melanocytic markers. Some tumor cells were positive for cyclin D1. A diagnosis of LCS involving the skin was established. The present study is a very unusual case of LCS showing epidermotropism. The patient's history of metastatic melanoma posed additional challenges for diagnosis, underlying the need of immunophenotyping in these cases. Consensus for optimal standard therapy has not been established in LCS, and thus, early recognition is important since these neoplasms tend to recur and metastasize. LCS in skin is discussed and published cases are comprehensively reviewed.

Identifiants

pubmed: 32644218
doi: 10.1111/cup.13803
doi:

Substances chimiques

S100 Proteins 0

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

547-557

Informations de copyright

© 2020 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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Auteurs

Shira Ronen (S)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Elizabeth Keiser (E)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Katrina M Collins (KM)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Phyu P Aung (PP)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Priyadharsini Nagarajan (P)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Michael T Tetzlaff (MT)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Jonathan L Curry (JL)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Doina Ivan (D)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Victor G Prieto (VG)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Sharon Hymes (S)

Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

L Jeffrey Medeiros (LJ)

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

Carlos A Torres-Cabala (CA)

Department of Pathology, Dermatopathology Section, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.
Department of Dermatology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA.

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