Mafucci syndrome is:
Correct Answer: Multiple enchondromas with soft tissue hemangiomas
Description: Ans- B.Whereas ,multiple osteochondromatosis is hereditary multiple exostosis.Ref: Apley's System of Orthopaedics and Fractures, 9th editionExplanation:MAFFUCCI SYNDROMEMultiple enchondromas.Soft-tissue hemangiomas.Lymphangiomas.Affects skin and musculoskeletal system.Usually bilateral.Most commonly seen in the hands and feet.SOME IMPORTANT NAMED MUSCULOSKELETAL CONDITIONSCodman's TumourBenign tumor of the cartilage and cartilageforming connective tissue.Affects epiphyses of the long bones.Most common sites: Femur, humerus and tibia.Askin TumorMalignant small cell tumor of the thoracopulmonary region.Most common sites: Soft tissue of chest wall, peripheral lung and ribs.Resembles Ewing sarcoma (EWS) in histology.Ewing Sarcoma - also called ' endothelioma of bone'.Baker CystMost common synovial cysts.Occur in the posterior thigh and knee.Caused by trapping of fluid in semimembranous bursa.Brodie AbscessSubaeute or chronic stage of pyogenic osteomyelitis.Usually found in metaphyses and diaphyses of tubular bones.Staphylococcus aureus is the most commonly isolated pathogen.Central area of suppurative necrosis lined by granulation tissue and surrounding sclerotic bone.X-ray appearance--Lytic lesion with surrounding sclerosis and cortical irregularities.Differential diagnoses--EWS, osteoid osteoma and eosinophilic granuloma.Imaging technique of choice for diagnosis--MRI.Penumbra sign in MRI: Thin layer of hyperintense T1 signal intensity (representing granulation tissue lining the cavity) relative to that of the abscess cavity.De Quervain TenosynovitisStenosing tenosynovitis of the abductor pollicis longus (EPL) and extensor pollicis brevis (EPB) tendons.Also called washerwoman's sprain and baby wrist.Cause: Idiopathic: Overuse and Microtrauma.Pathology--Chronic degeneration.Evans TumorLow-grade Fihromyxoid Sarcoma.Presents as intramuscular soft-tissue mass in the lower extremity or trunk.Most commonly seen in young to middle-aged adults.Kaposi SarcomaIdiopathic multiple pigmented sarcoma.Mesenchymal tumor involving blood and lymphatic vessels.Most common site is skin.It is an AIDS-defming illness.Virus associated with this tumor is herpes virus(HHV-8).Morel-Lavallee LesionClosed degloving injury.Abrupt separation of subcutaneous tissue from the underlying fascia in the presence of bleeding capillaries.Growing hemorrhagic collection.Most common site is proximal lateral thigh(dermis has rich vascular plexus piercing fascia lata).May require surgical debridement to prevent infection.Morton NeuromaPerineural fibrosis and nerve degeneration of the interdigital nerve at the level of the metatarsal heads.Not a true neuroma.Caused by compression and irritation of the nerve beneath the intermetatarsal ligament.Presents with intermittent pain in the distribution of the interdigital nerve.Most frequent location - between third and fourth metatarsals.Treatment: Local steroid injection.Diagnosis by MRI or ultrasound.Paget Disease"Osteitis deformans"; inflammatory condition of bone.Excessive absorption of bone followed by improper new bone production.Paramyxovirus implicated in one of the causes.Higher prevalence in Ashkenazi Jews.Serum marker HLA-DR2.Albers-Schonberg Disease--OsteopetrosisAlso called Marble bone disease (due to thick dense bone).Lobstein Syndrome--Osteogenesis Imperfecta Also called Brittle bone disease (due to tendency to repetitive spontaneous fractures).
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