MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities

can LCH really look like anything?

Jonathan D Samet*, Joanna Lynn Weinstein, Laura M. Fayad

*Corresponding author for this work

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Objective: To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. Materials and methods: The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. Results: The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). Conclusions: The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH.

Original languageEnglish (US)
Pages (from-to)607-613
Number of pages7
JournalSkeletal Radiology
Volume45
Issue number5
DOIs
StatePublished - May 1 2016

Fingerprint

Langerhans Cell Histiocytosis
Pelvis
Extremities
Blood Sedimentation
Leukocyte Count
C-Reactive Protein
Edema
Ilium
Scapula
Clavicle
Spontaneous Fractures
Humerus
Tibia
Femur
Differential Diagnosis
Fever
Bone Marrow
Biopsy
Bone and Bones
Pain

Keywords

  • Extremities
  • LCH
  • Langerhans cell histiocytosis
  • MRI
  • Pediatric bone lesion
  • Pelvis

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

Cite this

@article{54bb0225123c470aa064d138407dbb62,
title = "MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?",
abstract = "Objective: To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. Materials and methods: The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. Results: The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 {\%}). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 {\%}), 8/12 (67 {\%}), and 4/10 (40 {\%}) of cases, respectively. Fever was documented in 1/15 (7 {\%}) patients and pain was reported in 15/15 (100 {\%}). Conclusions: The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH.",
keywords = "Extremities, LCH, Langerhans cell histiocytosis, MRI, Pediatric bone lesion, Pelvis",
author = "Samet, {Jonathan D} and Weinstein, {Joanna Lynn} and Fayad, {Laura M.}",
year = "2016",
month = "5",
day = "1",
doi = "10.1007/s00256-016-2330-x",
language = "English (US)",
volume = "45",
pages = "607--613",
journal = "Skeletal Radiology",
issn = "0364-2348",
publisher = "Springer Verlag",
number = "5",

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TY - JOUR

T1 - MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities

T2 - can LCH really look like anything?

AU - Samet, Jonathan D

AU - Weinstein, Joanna Lynn

AU - Fayad, Laura M.

PY - 2016/5/1

Y1 - 2016/5/1

N2 - Objective: To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. Materials and methods: The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. Results: The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). Conclusions: The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH.

AB - Objective: To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. Materials and methods: The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. Results: The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). Conclusions: The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH.

KW - Extremities

KW - LCH

KW - Langerhans cell histiocytosis

KW - MRI

KW - Pediatric bone lesion

KW - Pelvis

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JO - Skeletal Radiology

JF - Skeletal Radiology

SN - 0364-2348

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