Simultaneous Occurrence of Hodgkin's Lymphoma and Langerhans Cell Histiocytosis of the Spine : A Rare Combination

Abstract

Hodgkin's disease presenting with spinal cord compression owing to extradural and bone involvement is extremely unusual. A 48-year-old man presented with progressive lower extremity weakness resulting from spinal cord compression attributable to an epidural mass in the thoracic vertebrae. The patient underwent decompressive surgery, and was then treated with chemotherapy for Langerhans cell histiocytosis. However, the disease progressed, and we performed second decompressive surgery with stabilization. Subsequent histopathological investigations revealed Hodgkin's lymphoma of the bone. Here, we describe an unusual case of spinal Hodgkin's lymphoma and Langerhans cell histiocytosis to draw attention to this combination as a possible diagnosis in patients with mixed inflammatory cell infiltrate lesions in the spine.

Keywords: Hodgkin's disease; Langerhans cell histiocytosis; Lymphoma; Spinal cord compression.

Fig. 1

Preoperative images (A-D). (A) Axial computed tomographic (CT) scan showing a bone-destructive lesion. Gadolinium-enhanced T1-weighted MR sagittal (B) and axial (C) images showing thoracic spinal cord compression. (D) The CT scan of the thorax shows a mediastinal mass (arrow). (E) A postoperative axial CT scan following the first surgery demonstrates a T3 laminotomy.

Fig. 2

Magnetic resonance images (A and B) and whole-body positron emission tomographic scan (C and D) images obtained 1 year after surgery, demonstrating progression of the thoracic spine lesion with spinal cord compression.

Fig. 3

(A) Scanning power view shows irregular osteolytic fibrous lesion with extensive inflammation (original magnification, ×20, H&E stain). (B) High power view shows cellular lesion composed of a mixture of histiocyte, Lagerhans cells, eosinophils, and fibroblasts in the densely sclerotic background (original magnification, ×400, H&E stain). Insets : The Langerhans cells stain positively with S100 and CD1a. (C) Adjacent area shows numerous Reed-Sternberg cells, with their characteristic bi-lobed mirror image nuclei containing owl-eyed nucleoli among small lymphocytes, and plasma cells (original magnification, ×400, H&E stain). Inset : Immunostain for CD30 (Ki-1 antigen).