Fact-sheet: Eosinophilic granuloma

Updated on 10/20/2023 at 11:20 AM

Note : 0/10

Definition

Exclusively osseous involvement in Langerhans cell histiocytosis

Formerly Histiocytosis X
Multisystemic disease related to abnormal proliferation of Langerhans cells accumulating in various tissues
Preferentially affects children but can be seen in adults
Slight male predominance: sex ratio of 1.2
Annual incidence in children under 15 years of 4.6 cases per million
Most often localized to a single organ

Classification based on clinical presentation:

Eosinophilic granuloma (exclusively osseous involvement)
Hashimoto-Pritzker disease (cutaneous histiocytosis in the newborn)
Hand-Schuller-Christian disease (triad: diabetes insipidus, exophthalmos, and bone lesion)

Depending on location, limping, soft tissue swelling, spinal pain, painful scoliosis

Radiography:

Focal osteolysis with regular or irregular margins, +/- peripheral osteosclerosis, +/- periosteal reaction
Bone sequestrum
Extension into soft tissue
Variable appearance depending on chronicity and growth rate
Involvement of flat bones (50%): skull base (temporal bone) and calvaria (parietal bone), mandible, ribs, pelvis (acetabular roof)
Involvement of long bones (30%): femur, humerus, tibia
Exceptional involvement of the fibula and extremities

Focal osteolysis with regular or irregular margins, +/- peripheral osteosclerosis, +/- periosteal reaction
Bone sequestrum
Extension into soft tissue

Calvarial involvement

Punched-out, confluent lucency, absence of periosteal reaction and soft tissue involvement with possible intracranial extension
Sharp, polycyclic margins
Point of origin: diploe with lysis of the outer table more pronounced than the inner table, giving a beveled or double-contour appearance +/- bone sequestrum
Pseudo-abscess appearance

Nonspecific lesion of inflammatory appearance, hyperintense on T2, hypointense on T1, and enhancing, with a lobulated appearance and sometimes intralesional septa hypointense on T2 that enhance
Significant perilesional bone marrow edema

Hypermetabolism on PET-CT and on bone scintigraphy

Biopsy for histologic confirmation.

• Simple surveillance

• Local treatment: orthopedic, cutaneous, corticosteroid injection

• Systemic chemotherapy if bone involvement with functional threat

Chondroblastoma (epiphysis)

Osteoid osteoma

Osteoblastoma

Infection: Brodie's abscess, Osteomyelitis

Metastasis (neuroblastoma)