Orthopedic Oncology cases Langerhans cell histiocytosis

An 8-year-old female complains of pain in her lower back. She is an active, otherwise healthy child, and the pain started indolently about 4 weeks ago, without history of trauma. Her parents report that it keeps her up at night, and her performance in sports has begun to suffer as result of the pain. She denies fever, chills, or constitutional symptoms. She denies pain, weakness, or sensory changes in the legs, and denies bladder/bowel dysfunction. X-rays of the spine shows vertebra plana of one of the lumbar vertebrae. A biopsy is performed and the histology is shown in Figure 8–42.

 

 

 

Figure 8–42

 

What is the most likely diagnosis?

1. Osteomyelitis

2. Congenital vertebral anomaly

3. Langerhans cell histiocytosis

4. Acute fracture

 

Discussion

The correct answer is (C). Wafer-shaped collapsed vertebra (termed “vertebra plana”) in a child is typical of histiocytosis. The disk height above and below the involved level is increased. This can cause back pain but in the absence of acute trauma should not be confused with an acute compression fracture. Vertebral osteomyelitis is less likely to cause such a compression, unless it is chronic and destructive over years. This patient has no signs of osteomyelitis (fever, chills, etc.). A congenital vertebral anomaly would usually have caused symptoms prior to age 8, as well as a coronal plane deformity which the patient does not have.

Langerhans cell histiocytosis/eosinophilic granuloma is the most common tumor of the:

1. Clavicle

2. Scapula

3. Fibula

4. Distal radius

 

Discussion

The correct answer is (A). This benign but painful lytic bone lesion is commonly in children and is the most common tumor of the clavicle.

A high power biopsy slide is shown in Figure 8–43. Which of the following characteristic findings is not usually seen with Langerhans cell histiocytosis?

1. Hemosiderin laden tissues

2. Indented and lobular nuclei

3. Bilobed or coffee bean shaped nuclei

4. High proportion of eosinophils

 

 

 

Figure 8–43

 

Discussion

The correct answer is (A). Histopathologic examination of this lesion reveals eosinophils with typical eosinophilic cytoplasm and bilobed nucleus and the diagnostic Langerhans cells, which have an indented and lobular nucleus.

What is the most appropriate recommendation for treatment in this patient?

1. In situ posterior spinal fusion

2. Cortisone injection

3. Observation

4. Bisphosphonates

 

Discussion

The correct answer is (C). In the absence of cord compression, no treatment is necessary for vertebra plana. Eighty percent of patients will at least partially spontaneously reconstitute vertebral height at the affected level.

 

Objectives: Did you learn...?

 

To recognize vertebra plana?

 

 

To recognize Langerhans cell histiocytosis? Treatment of LCH?