Bone, Cartilage, And Soft Tissue Tumors

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associated with asbestos exposure

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mesothelioma

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4 tumors that are more likely to initially spread to regional lymph nodes

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angiosarcoma
epithelioid sarcoma
clear cell sarcoma
alveolar rhabdomyosarcoma

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A metastatic process presenting as a lymph node metastasis is most likely what

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carcinoma rather than a sarcoma

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differentiate carcinomas and sarcomas

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1. carcinomas: epithelial tissue, spready via lymph or hematogenous route, distinct border, cell cohesiveness
2. Sarcoma: mesenchymal tissue, spready mainly via hematogenous route, subtle border, no cell cohesiveness

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reactive psueoneoplasm characterized by a haphazard proliferation of fibroblasts accompaned by inflammatory cells

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nodular fasciitis

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locally aggressive proliferation of fibroblasts

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fibromatosis

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characteristics of fibromatosis

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1. non-encapsulated
2. highly infiltrative peripheries in multiple recurrence
3. no metastasis

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Desmoid tumor

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variant of fibromatosis occuring in the abdominal musculature with prenancy

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Less aggressive variants of fibromatosis

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plantar fibromatosis
palmar fibromatosis - Dupuytren's contracture
fibromatosis colli - sternocleidomastoid muscle in children

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benign neoplasm of fibrous connect tissue

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fibroma - rarely encountered in the "pure" form

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pseudoneoplasm that is actually an accumulation of lipid-laden macrophages

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xanthoma

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benign proliferation of fibroblasts and histiocytes that are more common in superficial location

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Fibrous histocytoma

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high grade malignancy of late adulthood consisting of primitive mesenchymal cells

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malignant fibrous histiocytoma

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histiologic characteristics of malignant fibrous histiocytoma

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spindling
storiform growth patterns - cartweel appearance
bizzare pleomorphism

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characteristics of lipoma

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benign neoplasm of adipose tissue
encapsulated
most are superficial, subcutaeous moveable tumors in elderly

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hemartoma, usually renal, consisting of varying proportion of blood vessels and adipose tissue

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angiomyolipoma

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an adrenal lipoma containing bone marrow elements

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myelolipoma

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a lipoma showing vascular overgrowth

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angiolipoma

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characteristics of liposarcoma

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1. tumors arising in deep soft tissue of the thigh, hip, or retroperitoneum
2. rare in patients under 10
centrally occuring, no extremities

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benign tumor of smooth muscle most commonly found in the uterus

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Leiomyoma

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characteristics of Leiomyoma

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1. most commonly in the uterus
2. PAS stains show presence of glycogen
3. tumor cells grow in long, sweeping fascicles, contain glycogen, and have blunt-ended nuclei

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malignancy of smooth muscle

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Leiomyosarcoma

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differentiate leiomyosarcoma from leiomyoma

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mitotic activity
increased cellularity
necrosis and hemorrhage
pleomorphism

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differentiate leiomyosarcoma in the uterus compared to other soft tissues

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much more mitotic activity needs to be seen in the uterus for malignancy to be considered

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two soft tissue tumors associated with tuberous sclerosis

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angiomyolipoma
rhabdomyoma

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benign tumor of skeletal muscle

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rhabdomyoma

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malignancy of skeletal muscle found in the young

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rhabdomyosarcoma

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Botryoid rhabdomyosarcoma

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variant of embryonal rhabdomyosarcoma arising near mucous membranes - bladder, vagina, or prostate

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benign neoplasm of blood vessels

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hemangioma

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differentiate capillary and cavernous hemangioma

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1. Capillary: small, superficial, most regress by age seven
2. Cavernous: larger, more likely to be deep such as in the liver

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strawberry nevus

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variant of hemangioma found in the head and neck

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Kassabach-Merrit Syndrome

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platelets are sequestered and destroyed in a cavernous hemangioma causing thrombocytopenic purpura

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characteristics of angiosarcoma

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aggressive malignancy of endothelialc ells
stain positive for Human factor VIII, CD34, and CD31
metastasize to lymph nodes first

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assoacited with human factor VIII, CD34, and CD31

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angiosarcoma

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associated with malignant neoplasm of mitotically active spindle cells mixed with vascular element and extravasated red cells, HHV 8

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Kaposi's sarcoma

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benign neoplasm of lymph channels

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lymphangioma

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cystic hydroma

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lymphatic malformation of the posterior cervical triangle in children

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tumors arising in the setting of chronic edema

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lymphangiosarcoma

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Stewart-Treves syndrome

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development of lymphangiosarcoma in an edematous upper extremity following radical mastectomy

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may arise secondary to trauma or amputation when nerve fibers mis-align themselves in regenerative attempts

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Neuroma

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Morton's neuroma

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painful mass near the heads of metatarsal bones probably caused by repeated trauma

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malignant neoplasms of wavy spindle cells arising from peripheral nerve or neurofibromas

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malignant peripheral nerve sheath tumor

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1/2 show S100 protein positivity

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malignant peripheral nerve sheath tumor

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benign reactive pseudoneoplastic proliferation of bone usually in young athletes from trauma

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myositis ossificans

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high grade primitive round cell tumor with large amounts of glycogen (PAS positive), PNET small blue cells tumor

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Ewing's Sarcoma
soft tissue tumor of uncertain origin

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serum alkaline phosphatase reflects activity

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osteoblast

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serum acid phosphatase reflects activity

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osteoclast

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respond to PTH

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osteoclasts

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immature bone with haphazard arrrangement of collagen fibers mixed with osteoid

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woven bone

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most common site of most primary bone tumors

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metaphysis

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benign bone nodule arising in bones of the skull and face

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osteoma

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intestinal polyposis
soft tissue tumors
Multiple osteomas

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Gardner's syndrome

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benign developmental abnormality of bone

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fibrous dysplasia

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characteristics of fibrous dysplasia

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1. fibrous tissue surrounding woven bone
2. fishhook trabeculae
3. bone formation is arrested is a persistence of woven bone without maturation
4. seen in younger population
5. rare metastatizes, only if irradiated

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bone nodule composed of osteoblasts and osteoid trabeculae with varied degrees of mineralization

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osteoid osteoma

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associated with the metaphysis of the tibia or femur in younger patients

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osteoid osteoma

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high grade malignancy of bone forming cells

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osteosarcoma

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characteristics of osteosarcoma

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pain, swelling, pathologic fracture
early metastasis to lungs
metaphyseal region of long bone
Sun-burst appearance on x-ray
associated with Paget's disease and radiation exposure

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associated with paget's disease and radiation exposure

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osteosarcoma

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associated with early metastasis to the lungs

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osteosarcoma

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many multi-nuclated giant cells not associated with hyperparathyroidism, epiphysis of tibia and femur

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giant cell tumor (Osteoclastoma)

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tumor of hematopoietic derivation composed of plasma cells

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multiple myeloma

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associations of multiple myeloma

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1. multiple punched-out lesion in bone (skull usually)
2. hypercalcemia
3. bence jones proteins
4. older individuals

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associated with ends of spinal column, osseous destruction with extension into soft tissue, and physaliferous cells

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chordoma

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benign, cartilaged capped bony prominence - attempt to grow new bone

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osteochondroma

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benign, usually solitary intramedullary cartilaginous mass usually in small bones of hands and feet

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chondroma

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multiple form of chondroma

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Ollier's disease

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x-ray shows well demarcated hypodense lesion making hole in bone

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chondroma

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usually arising de novo

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chondrosarcoma

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microscopically muleiple lobules of disorganized cartilage, some with multiple cells in individual lacunae

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chondrosarcoma

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where is chondrosarcoma usually found

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pelvic bones or axial skeleton

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malformation, not a neoplasm, with a defect in the growth plate allowing cartilage to grow laterally to form another projection of bone covered by a cap of cartilage

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osteochondroma

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associated with severe nocturnal bone pain in 5-25 year olds which is alleviated by aspirin

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osteoid osteoma

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most common in in the metaphysis of long bones in kids and young adults, associated with a "sun-burst" pattern on x-ray

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osteosarcoma

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associated with rapid intra-medullary proliferation in long bones of kids, PAS stain positive because of presence of glycogen,

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Ewing Sarcoma

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chromosomal translocation is t(11:22) and primitive neuroectodermal cell tumor

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Ewing Sarcoma