Chapter 2 Cysts and Other Benign Lesions
LANGERHANS CELL HISTIOCYTOSIS
EPIDEMIOLOGY
• The umbrella phrase LCH is now used in lieu of the previous clinical diagnoses of eosinophilic granuloma (localized, unifocal, or multifocal bony involvement), Hand–Schüller–Christian disease (localized bone [usually skull] involvement, exophthalmos, and diabetes insipidus), and Letterer–Siwe disease (disseminated disease).
• There has been a long-standing debate whether LCH represents a true neoplasm or merely an inflammatory process.1
• LCH is often considered a disease of childhood, but in a larger series,2 approximately 40% of the study population was younger than 20 years old; overall, LCH lesions were marginally more common in females than in males.
DISTRIBUTION
HISTOLOGY/GRADING
• The histologic feature of LCH consists of Langerhans cells, featured with grooved nuclei and faintly eosinophilic cytoplasm, and variable amounts of inflammatory cells including eosinophils, lymphocytes, plasma cells, and neutrophils (Fig. 2-1).
ANEURSYMAL BONE CYST
EPIDEMIOLOGY
• Aneurysmal bone cysts (ABCs) are characterized by small and large cavernous spaces filled with clotted-blood fluid.
DISTRIBUTION
• The cervical, thoracic, and lumbar spines are similarly affected and in most of the reported cases, the posterior elements of the vertebrae were the site of the lesion. Hay et al6 reported a series of 12 sacral lesions out of 92 cases of ABC in the spine.
• The etiology of ABCs is obscure, although the following theory is generally accepted. The aneurysmal bone cysts may be caused by development of a vicious hemodynamic cycle in a bone that is the site of a congenital vascular anomaly. This cycle is triggered by trauma or the development of other pathology, which interferes with venous drainage of the area. As a result, subsequent venous pressure increases, and a dilated and engorged vascular bed develops in the affected bone.4
HISTOLOGY
• Pathologically, ABCs contain multiple fluid-filled cavities separated by multiple fibrous septa. The new bone is commonly parallel to fibrous septa (Fig. 2-6). These large spaces filled with blood do not have an endothelial lining.7
• The fibrous septa are composed of a moderately dense cellular proliferation of bland fibroblasts, with scattered osteoclastic giant cells and reactive new bone (Fig. 2-7). In approximately one-third of cases, the bone is basophilic and has been termed “blue bone” (Fig. 2-8).
• The majority of secondary ABCs develop in association with benign neoplasms, most commonly giant cell tumor of bone, osteoblastoma, chondroblastoma, and fibrous dysplasia.