Current concepts for the diagnosis and management of eosinophilic granuloma of bone.

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Abstract

This review summarizes current concepts in the diagnosis and management of the patients with eosinophilic granuloma. Given the benign biology, the clinical course, and the pediatric group of patients that this condition more commonly affects, a treatment approach that carries a lower risk of complications while ensuring a successful cure is desirable. Variable treatment options have been reported with satisfactory results and a recurrence rate of less than 20 %. In this setting, symptomatic lesions that are accessible in the spine or the extremities may be treated with intralesional methylprednisolone injection after tissue biopsy for histological diagnosis.

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Most cited references 62

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Langerhans'-cell histiocytosis (histiocytosis X)--a clonal proliferative disease.

Alden Griffith, L Busque, K McClain … (1994)

The lesions of Langerhans'-cell histiocytosis (histiocytosis X), a proliferative histiocytic disorder of unknown cause, contain histiocytes similar in phenotype to dendritic Langerhans' cells. The disease ranges in severity from a fatal leukemia-like disorder to an isolated lytic lesion of bone. Intermediate forms of the disease are usually characterized by multiorgan involvement, diabetes insipidus, and a chronic course. To determine whether Langerhans' histiocytosis is a polyclonal reactive disease or a clonal disorder, we used X-linked polymorphic DNA probes (HUMARA, PGK, M27 beta[DXS255], and HPRT) to assess clonality in lesional tissues and control leukocytes from 10 female patients with various forms of the disease. Lymphoid clonality was also assessed by analysis of rearrangements at immunoglobulin and T-cell-receptor gene loci. The HUMARA assay detected clonal cells in the lesions of 9 of the 10 patients: 3 patients had acute disseminated disease, 3 had unifocal disease, and 3 had intermediate forms. The percentage of clonal cells closely approximated the percentage of CD1a-positive histiocytes in each lesion. Clonality was also confirmed in two of nine cases with the PGK or M27 beta probe. Extreme constitutional lyonization precluded assessment of clonality in the 10th case. Lymphoid clonality was ruled out in all cases. The detection of clonal histiocytes in all forms of Langerhans'-cell histiocytosis indicates that this disease is probably a clonal neoplastic disorder with highly variable biologic behavior. Thus, genetic mutations that promote clonal expansion of Langerhans' cells or their precursors may now be identified.

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Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society.

B E Favara, A Feller, M. Pauli … (1997)

Pathologists and pediatric hematologist/ oncologists of the World Health Organization's Committee on Histiocytic/Reticulum Cell Proliferations and the Reclassification Working Group of the Histiocyte Society present a classification of the histiocytic disorders that primarily affect children. Nosology, based on the lineage of lesional cells and biological behavior, is related to the ontogeny of histiocytes (macrophages and dendritic cells of the immune system). Dendritic cell-related disorders of varied biological behavior are dominated by Langerhans cell histiocytosis, but separate secondary proliferations of dendritic cells must be differentiated. Juvenile xanthogranuloma represents a disorder of dermal dendrocytes, another dendritic cell of skin. The hemophagocytic syndromes are the most common of the macrophage-related disorders of varied biological behavior. Guidelines for distinguishing the exceedingly rare malignant diseases of histiocytes from large cell lymphomas through the use of a battery of special studies are provided.

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Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement.

A Podda, E Azouz, J. L. Rodriguez … (2005)

Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a "button sequestrum." Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement.

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Author and article information

Journal

Journal ID (iso-abbrev): J Orthop Traumatol

Title: Journal of orthopaedics and traumatology : official journal of the Italian Society of Orthopaedics and Traumatology

Publisher: Springer Nature

ISSN (Electronic): 1590-9999

ISSN (Print): 1590-9921

Publication date (Electronic): Jun 2017

Volume: 18

Issue: 2

Affiliations

[1 ] Department of Orthopedics, University of Bologna, Istituto Ortopedico Rizzoli, Via Pupilli, 40136, Bologna, Italy. andrea.angelini83@yahoo.it.

[2 ] First Department of Orthopaedics, Athens University Medical School, ATTIKON University Hospital, Athens, Greece.

[3 ] Department of Radiology and Interventional Angiographic Radiology, Istituto Ortopedico Rizzoli, Bologna, Italy.

[4 ] Department of Orthopedics and Orthopedic Oncology, University of Padova, Padova, Italy.

Article

Publisher Item ID: 10.1007/s10195-016-0434-7

DOI: 10.1007/s10195-016-0434-7

PMC ID: 5429252

PubMed ID: 27770337

SO-VID: 44fc7164-4bdf-4b36-8a2b-209a2dfc41f5

History

Keywords: Bone tumors,Eosinophilic granuloma,Extremities,Methylprednisolone injection,Spine

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Keywords: Bone tumors, Eosinophilic granuloma, Extremities, Methylprednisolone injection, Spine

Current concepts for the diagnosis and management of eosinophilic granuloma of bone.

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Most cited references 62

Langerhans'-cell histiocytosis (histiocytosis X)--a clonal proliferative disease.

Contemporary classification of histiocytic disorders. The WHO Committee On Histiocytic/Reticulum Cell Proliferations. Reclassification Working Group of the Histiocyte Society.

Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement.

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