Clinical signs:
It is a rare disease characterized by skeletal and visceral accumulation of foamy histiocytes leading to fibrosis. Predominantly affected are long bones of the extremities and lungs.
Histology:
The bone marrow shows loose fibrosis and infiltration by foamy histiocytes. Touton giant cells and plasma cells may be present.
Pictures
Erdheimova-Chester disease:
Erdheim Chester, HE 40x (12376)
Erdheim Chester, HE 40x (12377)
Erdheimova-Chester disease:
Erdheim Chester, HE 40x (12675)
Clinical signs:
Metaphyseal fibrous defect is a small lytic intracortical lesion of children which often disappears in a course of few years. Occasional progression with extension into the medullary cavity are features of nonossifying fibroma. Distal femur, distal tibia and proximal tibia are the most common sites of involvement.
Macroscopic appearance:
Curetted fragments of fibrous tissue have shades of gray, brown or yellow.
Histology:
Fibrous tissue has areas with storiform arrangement. Aggregates of foamy macophages, siderophages and scattered multinucleated osteoclast-like giant cells are present in varying proporions.
Pictures
Metaphyseal fibrous defect:
Fibrous defect of the bone, HE 40x (12387)
Clinical signs:
Fibrous dysplasia is a bone defect with benign proliferation of fibrous tissue and bone. Lesions of fibrous dysplasia may be solitary (monostotic form) or multiple (polyostotic form). Fibrous dysplasia usually presents clinically during childhood or adolescence. Skeletal sites commonly involved include craniofacial bones, femur, tibia and ribs.
Macroscopic appearance:
The tissue is firm, fibrous, occasionally gritty in consistency. The colour is white or red.
Histology:
Irregularly shaped trabeculae of woven bone lacking any meanigful pattern are situated in a backround of moderately cellular fibrous stroma. The bony trabeculae may assume varying configurations, reminiscent of "C" shapes or circles. Osteoblasts rimming edges of bony trabeculae are inconspicuous. Fibrous stromal component is composed of cytologically bland spindle cell and collagen fibres. Mitotic activity is low.
Pictures
Fibrous dysplasia:
Fibrous dysplasia of the bone, HE 40x (12388)
Clinical signs:
OFD presents as a painless enlargement of the tibia that can lead to bowing or pathologic fracture. The radiograph discloses ill defined lucencies in the diaphyseal portion of the long bone. The lesion is usually diagnosed in children in the first decade of life.
Macroscopic appearance:
There is a focal substitution of the cortex by whitish to reddish fibrous tissue, soft or gritty in consistency.
Histology:
Histologically, there is fibrous spindle cell proliferation and bone trabeculae. The distinguishing feature of trabeculae in osteofibrous dysplasia is prominent osteoblastic rimming. Less mature central portion contains fibroblastic proliferation and immature spicules of woven bone. At the periphery of the lesion the bony trabeculae mature, become lamellar and more numerous. The differences between osteofibrous dysplasia and OFD-like adamantinoma are very subtle and the dictinction can be difficult.
Pictures
Osteofibrous dysplasia:
Osteofibrous dysplasia, HE 40x (12495)
Clinical signs:
Simple bone cyst occurs in patients in the first and second decades. There is predilection for proximal humerus and proximal femur. Bone cyst may produce pain and pathological fracture.
Macroscopic appearance:
Intramedullary non-expansive unilocular cavity containing serous fluid.
Histology:
Inner lining of the cyst consists of a thin fibrous membrane. The lining is composed of spindle cells and few scattered giant cells. It may contain hemosiderin and reactive new bone formation. A peculiar amorphous material, probably mineralized fibrin resembling cementum is often seen.
Clinical signs:
Is a nonneoplastic expanding osteolytic lesion composed of blood filled spaces separated by fibrous septae. Primary aneurysmal bone cyst arises de novo in previously uninvolved bone. Secondary aneurysmal bone cyst arise in association with precursor benign neoplasm.
Macroscopic appearance:
Removed usually by curettage, red-brown tissue fragment with discernible fibrous septa contain cavernous cystic spaces with blood.
Histology:
Vascular spaces of varying size are separated by fibrous septae that lack endothelium and smooth muscle wall. Septae contain fibroblasts, mononuclear histiocytes, strands of osteoid and scattered multinucleated osteclast-like giant cells. The cells are mitoticaly active.
Pictures
Aneurysmatic cyst of the bone:
Aneurysmatic cyst of the bone, HE 40x (12319)
Aneurysmatic cyst of the bone:
Aneurysmatic cyst of the bone, HE 40x (12325)Aneurysmatic cyst of the bone, HE 40x (12326)
Aneurysmatic cyst of the bone, HE frozen section and formol fixes slide:
Aneurysmatic cyst of the bone, frozen HE 40x (72956)Aneurysmatic cyst of the bone, HE 40x (72957)
Clinical signs:
Is a neoplastic proliferation of Langerhans cells causing lytic lesion usually centered in the medullary cavity. Any bone may be affected. More than half of cases occur in the first decade.
Macroscopic appearance:
The gross appearance is not distinctive, the tissue is soft, gray or brown.
Histology:
Langerhans cells are found in nests, clusters or sheets. The prototypic Langerhans cells have more or less abundant eosinophilis cytoplasm and well-demarcated cell margins. Nuclei are typically grooved, with coffee bean appearance. Some nuclei are reniform in shape with well-defined nuclear membrane and fine chromatin. Langerhans cells have characteristic immunophenotype (CD1a+; S100 Protein+). Electron microscopic examination shows unique intracytoplasmic inclusions called Bierbeck granules. Background inflammatory cells population include many eosinophils, lymfocytes and multinucleated giant osteoclast-like cells.
Pictures
Eosinophilic granuloma of the bone, HE and langerin:
Eosinophilic granuloma of the bone, HE 40x (12042)Eosinophilic granuloma of the bone, Langerin 40x (12043)
Eosinophilic granuloma of the bone:
Eosinophilic granuloma, HE 40x (12372)