Soft Tissue Pathology Flashcards
(27 cards)
What is the pathogeneses of soft tissue tumors
The majority are sporadic - germline mutations, some environmental
Mostly pluripotent mesenchymal stem cell
Rate and characteristics of simple Karyotype tumors
15-20%
Single chromosomal change
common in younger
Rate and characteristics of complex karyotype
80-85%
Aneuploid or polyploid with multiple chromosome abnormalities
common in adults
Characteristics of benign tumors
Superficial Small Slow growing Avascular (may have calcification)
Characteristics of malignant tumors
Deep Large Rapidly growing Hypervascular (may have calcifications)
Characteristics of Lipomas
Benign Common Soft, mobile, painless Well circumscribed Proximal extremities and trunk Mature Adipocytes Cured with excision
Characteristics of Liposarcomas
Most common sarcoma Occur in 50-60 yo Deep tissue of proximal extremities and retroperitoneum Slow growing From lipoblast
What are liposarcoma morphologies
Well differentiated Myxoid/round cell Pleomorphic (large, bizarre tumor cells with variable amounts of lipoblasts) Fibrous bands Various size
Characteristics of Nodular Fasciitis
Benign, self-limited Fibroblastic and myofibroblastic Young adults (10-40) History of trauma (25%) grows rapidly over weeks Superficial Cured with excision
Characteristics of Fibromatosis
Locally aggressive proliferation Slow growing Occur anywhere Male > Female Incidence with diabetes, alcoholism, epilepsy Wnt pathway - Beta Catenin
What are the 3 types of common superficial fibromatosis
Palmar - Thickenig in palmar facia (Dupuytrans), more common older
Plantar - Young people, unilateral
Penile - Peyronie disease, induration of mass on dorsolateral aspect, abnormal curvature
Deep fibromatosis
Desmoid tumors Large, infiltrative mass Some painful Facia and musculature Do not metastisize
Rhabdomyoma
Rare, benign tumor of muscle
Cardiac rhabdomyoma with tuberous sclerosis complex
Rhabdomyosarcoma
Malignant mesenchymal tumor
Most common soft tissue sarcoma in children
Aggressive neoplasia
Tx: surgery, chemo +/- radiation
Leiomyoma
Benign smooth muscle tumor Often in uterus (Female> Male) 1-2 cm Interesting fascicles of uniform spindled cells with cigar shaped nuclei Markers of smooth muscle
Leiomyosarcoma
Malignant tumors 10-20% of soft tissue sarcomas Adult onset Femal > Male Deep soft tissue extremities and retroperitoneum Metastasize late in life cycle Smooth muscle markers: actin and Desmin
Synovial Sarcoma
Not from the synovium Highly malignant Most common in deep soft tissue near joints occur in 20-40 yo Painful mass Chromosomal translocation of Chromosome X and 18 5 year survival 25-62% metastasize to lung
Myasthenia Gravis demographics
150-200 per million
2:1 F>M young; more common in Older Males
Myasthenia Gravis presentation
Anti-Ach ab
Eye symptoms: Ptosis, diplopia
Association with thymic abnormalities
Muscle weakness: worse with activity, better with rest
Lambert-Eaton Myasthenic Syndrome
Autoantibodies that block the presynaptic Ca2+ channels
Underlying malignancy such as Neuroendrocrine (small cell) carcinoma
Muscle weakness NOT IN THE EYES
Muscle weakness DOES NOT IMPROVE WITH REST
Toxins that bock Ach receptors
Botulinum Ethanol Steroids Chloroquine Statins
Dermatomyositis
Proximal muscles first
Systemic autoimmune dz
Associated with carcinoma
Skin involvement: heliotrope rash, malar rash, red papules on elbow knuckles and knees
Dermatomyositis Lab findings
Increased creatinine kinase
ANA+
Anti-Jo-1 ab
infiltration of mononuclear inflammatory cells (CD4 T ) around perimysium
Polymyositis
Like dermatomyositis but lacks skin involvement
CD8 T Cells infiltrate endomysium
