Updated: 6/22/2021

Eosinophilic Granuloma

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  • summary
    • Eosinophilic Granulomas are self-limiting benign histiocytic lesions that can occur in isolation or as a part of 2 systemic syndromes: Hand-Schuller-Christian (HSC) disease and Letterer-Siwe disease (LSD). 
    • Isolated lesions typically present with pain in the thoracic spine or long bones in patients < 20 years of age. HSC presents with diabetes insipidus and exophthalmos, while LSD is fatal in early childhood.  
    • Diagnosis is made with biopsy showing mononuclear histiocyte-like cells with oval nuclei with well-defined round cytoplasm with electron microscopy revealing Birbeck granules seen inside Langerhan's cells.
    • Treatment for isolated Eosinophilic Granulomasis is usually observation with pain management as the lesions typically resolve over time. Surgical management is indicated for lesions associated with impending fracture. Treatment for HSC and LSD is focused on the medical management of the syndrome.
  • Epidemiology
    • Demographics
      • most commonly occurs in children (80% of afflicted < 20 years of age)
      • HSC disease presents in children > 3 years of age
      • LSD occurs in children < 3 years of age
      • Male to female ratio of 2:1
    • Anatomic location
      • eosinophilic granuloma
        • commonly presents in the skull, ribs, clavicle, scapula, mandible
        • isolated lesions of the spine (thoracic most common)
        • can also occur in diaphyseal regions of long bones and the pelvis
      • HSC
        • multiple bony sites
        • multiple lytic skull lesions
        • visceral involvement of the lungs, spleen, liver, skin, lymph nodes
  • Etiology
    • A spectrum of diseases of the reticuloendothelial system with one of three general presentations
      • Eosinophilic granuloma (EG)
        • usually a single self-limited lesion found in younger patients
      • Hand-Schuller-Christian disease (HSC)
        • chronic, disseminated form with bone and visceral lesions
        • also known as Langerhans cell histiocytosis with visceral involvement
      • Letterer-Siwe disease (LSD)
        • fatal form that occurs in young children
    • Genetics
      • no clear genetic pattern of inheritance or locus has been determined
  • Presentation
    • Symptoms
      • skeletal involvement
        • pain and swelling at the region of involvement
        • limping can be seen with pelvic or lower extremity involvement
      • vertebral involvement
        • localized or diffuse back pain
        • increasingly kyphotic posture
        • radiculopathy can occur with more aggressive lesions
        • classic triad of
          • multiple lytic skull lesions
          • diabetes insipidus
            • increased thirst and water intake
          • exopthalmos
        • visceral involvement
          • diffuse or nonspecific abdominal or chest pain
  • Imaging
    • Radiographs
      • general
        • known as "the great mimicker" as it appears similar to many lesions
        • radiographic differential includes osteomyelitis, leukemia, lymphoma, fibrous dysplasia, or Ewing's sarcoma
      • diaphyseal lesions
        • well defined intramedullary lytic or "punched-out" lesion
        • cortex may be thinned, expanded, or destroyed
        • may have periosteal reaction
      • metaphyseal lesions
        • extend up to but not through the physis
        • less central location than diaphyseal lesions
      • spinal lesions
        • vertebra plana (flattened vertebrae) in spine
        • increased kyphosis
      • cranial involvement
        • multiple "punched-out" lytic lesions
    • MRI
      • may show a soft tissue mass adjacent to boney lesions
    • Bone scan
      • generally shows increased uptake in the region of boney lesion
  • Studies
    • Histology
      • Langerhan's cells
        • mononuclear histiocyte-like cells with oval nuclei with well-defined round or oval cytoplasm.
        • a prominent nuclear groove (coffee bean nuclei) can be seen in most of the nuclei
        • eosinophilic cytoplasm (pink generally)
        • stain with CD1A
        • electronmicroscopy
          • birbeck granules seen inside Langerhan's cells
      • mixture of inflammatory cells also present
      • giant cells are present
      • lack of nuclear atypia and atypical mitoses
        • differentiates this condition from malignant conditions such as Ewings sarcoma, lymphoma of bone, and metastatic neuroblastoma, which may look similar based on the round cells alone
  • Differential
    • Eosinophilic Granuloma Differential
      Destructive lesion in young patients
      Multiple lesions in young patients
      Treatment is Observation alone 
      Eosinophilic granuloma
      Osteomyelitis
      Osteosarcoma
      Ewing's sarcoma
      Desmoplastic fibroma
      Lymphoma
      Leukemia
      Fibrous dysplasia
      Enchondroma / Olliers / Maffucci's
      Osteochondroma / MHE
      Hemangioendothelioma
      NOF /Jaffe-Campanacci syndrome
      Paget's
  • Treatment
    • Nonoperative
      • observation alone
        • indications
          • a self-limited process and it is reasonable to treat with observation alone
      • bracing
        • indications
          • to prevent progressive kyphosis of the spine
        • outcomes
          • will correct deformity in 90% of patients
          • vertebral lesions generally regain 50% of their height
      • low dose irradiation (600-800 cGy)
        • indications
          • indicated for lesions in the spine that compromise stability, neurologic status
          • lesions not amenable to injection or open treatment
        • outcomes
          • effective for most lesions
      • chemotherapy
        • indications
          • diffuse HSC
        • outcomes
          • prognosis is improved with less severe extraskeletal involvement
      • corticosteroid injection
        • indications
          • isolated lesions
          • can be performed after curettage as well
    • Operative
      • curettage and bone grafting
        • indications
          • for lesions that endanger the articular surface or are a risk for impending fractures
      • spinal deformity correction
        • indications
          • progressive spine deformity refractory to bracing
            • approximately 10% of patients with spine lesion will need operative intervention for deformity correction
  • Prognosis
    • EG
      • isolated involvement generally treatable with local management
      • spine lesions can spontaneously resolve
    • HSC
      • prognosis depends on response to chemotherapy
      • worsening prognosis with increasing extraskeletal involvement
    • LSD
      • generally fatal in children < 3 years of age

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Flashcards (7)
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Questions (7)

(OBQ13.47) A 2-year-old boy is brought in by his mother after an unwitnessed fall. She reports the child is constantly thirsty. His clinical appearance and radiographs are shown in Figures A through D. What is the most likely diagnosis?

QID: 4682
FIGURES:
1

Child abuse

2%

(60/2764)

2

Neurofibromatosis

9%

(242/2764)

3

Metastatic neuroblastoma

15%

(421/2764)

4

Adamantinoma

2%

(63/2764)

5

Langerhans cell histiocytosis

71%

(1959/2764)

L 4 B

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(OBQ13.220) A 6-year-old boy presented to the emergency department with a suspected head injury after a fall. His parents attribute the injury to a loss of balance as they have noticed a worsening left sided limp over the past 2 months. Radiographs of the head and pelvis are seen in Figures A and B. Further questioning revealed the child has recently been worked up for increasing thirst and non-specific abdominal pain. A histology specimen can be seen in Figure C. What is the most likely diagnosis?

QID: 4855
FIGURES:
1

Hand-Schuller-Christian disease

67%

(1988/2980)

2

McCune-Albright syndrome

17%

(496/2980)

3

Maffucci's syndrome

5%

(156/2980)

4

Neurofibromatosis

7%

(218/2980)

5

Ollier disease

3%

(98/2980)

L 3 B

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(OBQ11.32) A 5-year-old child presents with skin lesions and a limp. He has met all developmental milestones accordingly but his parents have noticed he has recently stopped gaining weight. Clinical photograph of his skin lesion, radiograph of his left hip, and histology slide representative of his hip lesion are shown in Figures A, B, and C respectively. What is the most likely diagnosis?

QID: 3455
FIGURES:
1

Neuroblastoma

5%

(111/2102)

2

Ewing's sarcoma

5%

(99/2102)

3

Eosinophilic granuloma

77%

(1623/2102)

4

Acute lymphoblastic leukemia

10%

(209/2102)

5

Fifth's disease

3%

(54/2102)

L 2 C

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(OBQ10.275) A 7-year-old boy complains of pain in the right thigh for 1 month with no history of traumatic injury. A radiograph is provided in Figure A. A histology specimen from incisional biopsy is provided in Figures B and C. Which of the following is the most appropriate management?

QID: 3363
FIGURES:
1

Conservative treatment with crutches and repeat radiographs in 6 weeks

19%

(288/1501)

2

Wide excision

5%

(79/1501)

3

Wide excision and chemotherapy

62%

(927/1501)

4

Radiation therapy alone

7%

(98/1501)

5

Curettage and bone grafting

7%

(100/1501)

L 5 C

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