Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report

Wilmar Ghuijs; Paul G. Kemps; Marta E. Capala; Robert M. Verdijk; Astrid G.S. van Halteren; Robert J.P. van der Wal; Jan A.M. van Laar

doi:10.1186/s13014-022-02108-0

Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report

Wilmar Ghuijs, Paul G. Kemps, Marta E. Capala, Robert M. Verdijk, Astrid G.S. van Halteren, Robert J.P. van der Wal, Jan A.M. van Laar

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Abstract

Background: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a⁺, CD207/Langerin⁺ histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken. Case presentation: In this case report, we describe the initial presentation of a single BRAF^V600E mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated. Conclusion: The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.

Original language	English
Article number	137
Journal	Radiation Oncology
Volume	17
Issue number	1
DOIs	https://doi.org/10.1186/s13014-022-02108-0
Publication status	Published - Dec 2022

Keywords

Bone
BRAF
Langerhans cell histiocytosis
LCH
Radiotherapy

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@article{3b5168ea1fb444909294636a4e218023,

title = "Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report",

abstract = "Background: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a+, CD207/Langerin+ histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken. Case presentation: In this case report, we describe the initial presentation of a single BRAFV600E mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated. Conclusion: The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.",

keywords = "Bone, BRAF, Langerhans cell histiocytosis, LCH, Radiotherapy",

author = "Wilmar Ghuijs and Kemps, {Paul G.} and Capala, {Marta E.} and Verdijk, {Robert M.} and {van Halteren}, {Astrid G.S.} and {van der Wal}, {Robert J.P.} and {van Laar}, {Jan A.M.}",

note = "Publisher Copyright: {\textcopyright} 2022, The Author(s).",

year = "2022",

month = dec,

doi = "10.1186/s13014-022-02108-0",

language = "English",

volume = "17",

journal = "Radiation Oncology",

issn = "1748-717X",

publisher = "BioMed Central Ltd.",

number = "1",

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T1 - Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy

T2 - a case report

AU - Ghuijs, Wilmar

AU - Kemps, Paul G.

AU - Capala, Marta E.

AU - Verdijk, Robert M.

AU - van Halteren, Astrid G.S.

AU - van der Wal, Robert J.P.

AU - van Laar, Jan A.M.

PY - 2022/12

Y1 - 2022/12

N2 - Background: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a+, CD207/Langerin+ histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken. Case presentation: In this case report, we describe the initial presentation of a single BRAFV600E mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated. Conclusion: The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.

AB - Background: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a+, CD207/Langerin+ histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken. Case presentation: In this case report, we describe the initial presentation of a single BRAFV600E mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated. Conclusion: The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.

KW - Bone

KW - BRAF

KW - Langerhans cell histiocytosis

KW - LCH

KW - Radiotherapy

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DO - 10.1186/s13014-022-02108-0

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AN - SCOPUS:85135302164

SN - 1748-717X

VL - 17

JO - Radiation Oncology

JF - Radiation Oncology

IS - 1

M1 - 137

ER -

Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report

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