Musculoskeletal Path Bone Tumors/vasculitides - Firstaid

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Interlacing trabeculae of woven bone, surrounded by osteoblasts; <2 cm

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Osteoid osteoma

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Where are osteoid osteomas found?

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Proximal tibia and Femur

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Bigger version of Osteoid osteomas; where are these found?

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Osteoblastomas - vertebral column

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What is the most COMMON benign bone tumor?

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Osteochondroma! Exostosis

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What sex/age most often get Osteochondromas?

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Males <25 yrs old

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What do Osteochondromas consist of?

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Mature bone with a cartilage cap

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What part of the bone do Osteochondromas originate from?

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Long METAPHYSIS

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Osteochondromas RARELY do what:

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Transform into malignancy

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What benign bone tumor occurs in women more? At what age?

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Giant cell tumor
20-40 yrs old

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Giant cell tumors occur WHERE:

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EPIPHYSIS of long bones

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Though Giant cell tumors are benign, they are:

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LOCALLY AGGRESSIVE

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Most common bones in which Giant cell tumors form; X-ray hallmarks?

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-Proximal tibia
-Distal femur
(same as for Osteochondroma)
Double bubble/Soap bubble

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Histologic features of Giant cell tumors:

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Spindle shaped cells among Multinucleated Giant cells

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Benign cartilaginous neoplasm found in intramedullary bone:

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Endochondroma

Front 15

Endochondromas are usually found in what bones?

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Distal extremities

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Most common primary malignant bone tumor:

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Osteosarcoma

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Age/sex of osteosarcomas:

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Males 10-20

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What part of the bone do Osteosarcomas originate in?

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Metaphysis of long bones - same as for benign Osteochondromas

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4 predisposing factors to developing Osteosarcoma:

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Pagets (older people!)
Bone infarcts
Radiation
Familial Retinoblastoma!

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HAllmark lab finding in Osteosarcoma

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greatly elevated ALP

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Xray finding in Osteosarcoma:

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Codman's triangle

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Anaplastic small round blue cell tumor of bone:

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Ewing's sarcoma

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Most common sex/age of Ewing's sarcoma:

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Boys <15

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Progression of Ewing sarcoma:

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AGGRESSIVE
-Mets early
-Chemo response though!

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Hallmark characteristics of Ewing sarcoma; what part of bone does it form in:

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-Onion skin appearance
-DIAPHYSIS

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Translocation associated with Ewing sarcoma:

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11,22

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Malignant cartilaginous tumor:

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Chondrosarcoma

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Sex/age associated with Chondrosarcoma:

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Males, 30-60

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2 ways that Chondrosarcomas may develop:

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-Primary (most common)
-From Osteochondroma/exostosis transformation (rare)

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Hallmark appearance of Chondrosarcoma:

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Expansile glistening mass withIN the medullary cavity

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FAN MY SKIN On Wednesday:

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Takayasu's arteritis

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What does FAN MY SKIN On Wednesday stand for?

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-Fever
-Arthritis
-Night sweats
-Myalgia
-SKIN nodules
-Ocular disturbances
-Weak pulses in upper extrem.

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What IS Takayasu's arteritis?

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GRANULOMATOUS thickening of the aorta and proximal great vessels! Med and LARGE arteries

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Takayasu's is typically seen in:

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Asian females under 40 yrs old

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Takayasu's is also know as the ____ disease because of ____

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Pulseless disease - weak pulses in upper extremities from aortic thickening

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Most common vasculitis/what pateints?

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Giant cell arteritis - Temporal arteritis; in ELDERLY

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Size of vessels affected in Temporal arteritis:

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-small
-medium

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What arteries are typically affected in Temporal arteritis?

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Carotid artery branches - show focal granulomatous infiltrates

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What are 4 common findings in Temporal arteritis?

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-Unilateral HEADACHES
-Jaw claudication
-Impaired vision from OPTHALMIC artery occlusion
-Polymyalgia rheumatica

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What causes the polymyalgica rheumatica in temporal arteritis? How is it treated?

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The elevated ESR
Txmt: steroids

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Vasculitis seen in heavy smokers:

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Buerger's disease

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Buerger's disease is aka:

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Thromboangiitis obliterans

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Txmt for Buerger's:

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quit smoking

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Main symptoms of Buerger's disease:

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Severe pain/claudication
Raynaud's/cold insensitivity

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Acute/self limiting vasculitis in Children:

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Kawasaki disease!

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What is seen histopathologically in Kawasaki? What is it AKA?

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Acute NECROTIZING VASCULITIS of small/med vessels
-Aka Mucocutaneous lymph node syndrome.

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What are the signs/sx of Kawasaki disease? What can it be complicated by?

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-Fever/cong conjunctiva, lip and oral mucosal changes; LN edema
-May develop CORONARY ANEURYSMS

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What is the treatment for Kawasaki disease?

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It is self limiting.

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What is the MOST COMMON vasculitis in children and what is the main difference from Kawasaki?

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Henoch-Schonlein Purpura - this is HYPERSENSITIVITY Induced

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What is Henoch Schonlein in children comparable to in adults?

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Serum sickness

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What does the hypersensitivity in Henoch Schonlein consist of?

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Immune complex formation in serum resulting in Leukocytoclastic vasculitis

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What is the hallmark finding in henoch Schonlein lesions?

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They are all the same age - in contrast to those in PAN

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What are the clinical findings in Henoch Schonlein?

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APAIM
-Arthralgia
-Palpable purpura - rash
-Abdominal pain due to
-Intestinal hemorrhage
-Melana

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In what vasculitide are the lesions of DIFFERENT ages?

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Polyarteritis nodosa

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What is PAN caused by?

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Necrotizing immune complex inflammation of med-sized muscular arteries

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What vessels are typically involved in PAN?

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RENAL
VISCERAL

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What other condition is associated with 30% of PAN cases?

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Hepatitis B

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What can be found on arteriograms of patients with PAN?

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MULTIPLE aneurysms
Constrictions

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What can be positive serotypically in in PAN?

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P-ANCA - perinuclear

P is for PAN

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What are 2 drugs that are used for treatment of PAN?

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-Corticosteroids
-Cyclophosphamide

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What vasculitide is more widespread/systemic, and pos for C-ANCA strongly?

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WEGENER'S granulomatosis

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Are granulomas found in PAN?

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No

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What is the characteristic TRIAD of findings in Wegener's granulomatosis?

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-Focal necrotizing vasculitis
-Necrotizing granulomas in lungs and upper airways (sinuses)
-Necrotizing glomerulonephritis - crescentic!

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What are 3 common clinical manifestations of Wegener's?

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-Nasal septum perforation
-Hemoptysis (lung involvement)
-Hematuria (renal disease)

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Like Wegener's, lacks granulomas, P-ANCA pos:

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Microscopic polyangiitis

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ANCA pos vasculitis LIMITED to the kidney:

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Primary pauci-immune crescentic glomerulonephritis

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Granulomatous vasculitis with EOSINOPHILIA in atopic patients:

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Churg-Strauss syndrome

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What vessels are often involved in the vasculitis in Churg-Strauss syndrome?

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Pulmonary vessels