ABSTRACT

Cavernous hemangiomas/venous malformations

vening stroma, luminal thrombi, papillary endothelial hyperplasia (Masson lesion)

Gorham-Stout disease

proliferation of lymphatic vessels in bones leading to bone loss

FIBROUS SPINDLE CELL TUMORS OF BONE

Non-ossifying fibroma

demarcated ●● Bland uniform spindle cell proliferation,

interspersed giant cells, xanthomatous histiocytes

Desmoplastic fibroma

●● Similar to intraosseous desmoid-type fibromatosis

genized stroma, mitotic figures ●● High recurrence rate

Myofibromas

Cranial fasciitis

●● Fibroproliferative lesion resembling nodular fasciitis

●● Local growth erodes inner and outer table of skull

Congenital pseudoarthrosis

Fibrosarcoma

variant of osteosarcoma

Adamantinoma

●● Anterior diaphysis of tibia, well-circumscribed lesion

plasia ●● Bland spindle cell stroma, trabeculae of

woven bone and clusters of bland epithelial cells with peripheral palisading

●● Osteoblastic lining seen around bony trabeculae

basaloid, squamoid, pseudoglandular cells in myxoid stroma, may metastasize

ROUND CELL NEOPLASMS

Ewing sarcoma/primitive neuroectodermal tumor (PNET)

●● Second-most common pediatric malignant bone tumor

●● Imaging: Intramedullary, diaphyseal/ metadiaphyseal

with hyperchromatic nuclei, vacuolated to clear cytoplasm (glycogen)

●● Stain positively with PAS, CK, vimentin, CD99 (membranous), caveolin, FLI-1

Melanotic neuroectodermal tumor of infancy

●● Head and neck region: Maxilla, mandible, skull, brain/meninges

●● Larger epithelioid cells; glandular architecture, pigmented, positive for HMB45

●● Smaller cells; primitive, positive for neuroendocrine markers

Large B cell lymphoma

Langerhans cell histiocytosis (LCH)

●● Cells have abundant eosinophilic cytoplasm, folded nucleus, no processes

●● Infiltrate of Langerhans cells, eosinophils, Charcot-Leyden crystals, giant cells

disease ●● Acute disseminated LCH: Letterer-Siwe

disease

Chordoma

notochord ●● Cords and lobules of physaliferous cells

(vacuolated, bubbly cytoplasm) in fibromyxoid stroma

●● Stain positive for S100, CK, vimentin, EMA

OSTEOMYELITIS

●● Acute (neutrophils), subacute (mixture of neutrophils, lymphocytes, and plasma cells), chronic (lymphocytes, plasma cells, and macrophages)

●● Necrotic bone (sequestrum) and new bone formation (involucrum)

Acute osteomyelitis

●● Bacterial infection (Staphylococcus aureus); methicillin-resistant S. aureus (MRSA) or methicillin sensitive

●● In children with sickle cell disease = Salmonella

●● Tuberculous osteomyelitis is hematogenous, anterior portion of vertebral body (caseating granulomas)

●● Non-tuberculous mycobacterial infection in immunocompromised children (cystic fibrosis)

Subacute osteomyelitis

●● Brodie abscess (lytic lesion surrounded by osteosclerosis)

NON-BACTERIAL OSTEITIS

●● Auto-inflammatory disorder, chronic recurrent multifocal osteomyelitis

bony trabeculae, mosaic lines ●● Marrow fibrosis, chronic inflammation

ROSAI-DORFMAN DISEASE

●● Emperipolesis, background contains plasma cells, eosinophils, and histiocytes

INFANTILE CORTICAL HYPEROSTOSIS

condition ●● Soft-tissue swelling, inflammation, self-

limited course ●● Subperiosteal new bone formation, struc-

tural loss of cortical bone

OSTEONECROSIS

hemoglobinopathies (sickle cell disease) ●● Osteonecrosis of femoral head; seen in Legg-

Calvé-Perthes disease and Gaucher disease

●● Involved by infectious, metabolic, or autoimmune conditions

CHRONIC SYNOVITIS

●● Diffuse/nodular infiltrate of lymphocytes, plasma cells, mast cells

●● Hyperplasia of synovial cell lining, papillary architecture

Juvenile rheumatoid arthritis

large joints ●● Papillary synovial hyperplasia, lympho-

plasmacytic infiltrate, lymphoid follicles and germinal centers, multinucleated giant cells, fibrin and hemosiderin

HEMOPHILIC ARTHROPATHY

●● When factor VIII and IX levels <1% of normal

●● Infiltrates of lymphocytes, histiocytes, prominent hemosiderin deposits (synovial lining cells and histiocytes)

GRANULOMATOUS SYNOVITIS

HISTIOCYTIC SYNOVITIS

●● CD68 positive multinucleated giant cells and foamy macrophages

●● Non-tuberculous mycobacterial synovitis, arthropathy

TUMEFACTIVE LESIONS

●● Synovial chondromatosis, synovial lipomatosis, pigmented villonodular synovitis, giant cell tumor of tendon sheath, ganglion cyst

OSTEOARTHROPATHY

bolic disorder, osteonecrosis, abnormal skeletal development