rare bone tumors 2

Rare Bone Tumors - Part 2

Vinod NaneriaConsultant Orthopaedic Surgeon

Choithram Hospital & Research Centre,Indore, M.P., India

Fibrous Dysplasia

• Developmental anomaly.

• Monostotic, or polyostotic.

• X-ray: cystic lesion with ground glass appearance.

• Curettage + grafting + fixation

Shepherd Crooked Deformity

Fibrous dysplasia of 9th Rib

Monomelic

Polycystic

Melorheostosis

It is usually apparent in early childhood and even in the first few days of life. About 50% of persons affected will develop the symptoms by the time they are 20 years of age.

On x-rays, the disease's sclerotic bone lesions looks like wax dripping down the side of a candle. Hence its name; which is derived from the greek melos "limb" and rhein "to flow".

Osteopoikilosis• rare, autosomal dominant bone disorder

• multiple, small, circumscribed round or ovoid areas of increased bone density, widely distributed, caused by condensations of the spongiosa

• NO symptoms

• Associated- scleroderma, syndactyly

• Dwarfism, endocrine abnormalities, Melorrheostosis, cleft palate.

Pycnodysostosis

• An inherited disorder of the bone.

• Causes short stature.

• Causes abnormally dense brittle bones.

• Causes the "soft spot" of a baby's skull to stay widely open.

• Due to defect in an enzyme: cathepsin K.

• Growth hormone treatment may increase growth in pycnodysostosis.

Osteopetrosis

• marble bones, brittle bones, osteosclerosis fragilis

• abnormally dense bones

• brittle, fracture easily

• "rugger jersey" spine

• may be cause of anemia (d/t obliterated marrow space)

Aneurysmal Bone Cyst

• Radiolucent lesion w/ expanded cortex arising in medullary canal of metaphysis, aneurysmal expanded appearance of cortex is contained by periosteum & thin shell of bone;Marked cortical thinning and erosion and periosteal elevation;Lesion rarely penetrates the articular surface or growth plate.

Aneurysmal cyst arising from 10th Dorsal Vertebra.

Congenital Syphilis

• Defective collagen synthesis leads to defective dentine formation.

• Hemorrhaging into the gums, and loss of teeth. • Bone involvement is typical. • Calcification of the growth cartilage at the end of the long

bones continues, leading to the thickening of the growth plate. The typical invasion of the growth cartilage by the capillaries does not occur. Preexisting bone becomes brittle and undergoes resorption at a normal rate, resulting in microscopic fractures of the spicules between the shaft and calcified cartilage. With these fractures, the periosteum becomes loosened, resulting in the classic subperiosteal hemorrhage at the ends of the long bones.

Scurvy

Gout• Soft tissue - Calcific deposits eccentric juxta-

articular lobulated soft-tissue masses (hand, foot, ankle, elbow, knee)

• Joints - Preservation of joint space initially, absence of periarticular demineralization, erosion of joint margins with sclerosis, cartilage destruction late in course of disease, periarticular swelling, Chondrocalcinosis (menisci, articular cartilage of knee) resulting in secondary osteoarthritis

• ·      

•   Bone findings

• "Punched-out" lytic bone lesion ± sclerosis of margin

• "Mouse / rat bite" from erosion of long-standing soft-tissue tophus

• "Overhanging margin" (40%)

• Ischemic necrosis of femoral / humeral heads

• Bone infarction

Lithium

• Common side effects include muscle tremors, twitching, ataxia, hyperparathyroidism (bone loss, hypercalcemia, hypertension, etc,), kidney damage, nephrogenic diabetes insipidus (polyuria and polydipsia) and seizures. Many of the side-effects are a result caused by the increased elimination of potassium

Thalassemia• cortical thinning, osteopenia, and coarsening of the

trabeculae due to long standing anaemia.• phalanges reveal sausage-shaped configuration.• "rib-within-a-rib“, & subcortical lucency.• Premature fusion of the epiphyses is a characteristic

finding in thalassemia.

• Classical radiological features are rare due to treatment.

• Joint involvement is very common and often difficult to differentiate from Tuberculosis.

DISCLAIMER• Information contained and transmitted by this presentation is based

on personal experience and collection of cases at Choithram Hospital & Research centre, Indore, India, during last 25 years.

• It is intended for use only by the students of orthopaedic surgery. • Views and opinion expressed in this presentation are personal

opinion.• Depending upon the x-rays and clinical presentations, viewers can

make their own opinion.• For any confusion please contact the sole author for clarification.• Every body is allowed to copy or download and use the material

best suited to him. I am not responsible for any controversies arise out of this presentation.

• For any correction or suggestion please contact• naneria@yahoo.com