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Pathology of the Soft Tissues & Nerves. Arlene L. Santos, M.D. SY 10-11. Pathology of the Soft Tissues. I. Fatty Tumors II. Fibrous Tumors & Tumor -like Lesions III. Fibrohistiocytic Tumors IV. Tumors of the Skeletal Muscle. V. Tumors of the Smooth Muscle
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Pathology of the Soft Tissues & Nerves Arlene L. Santos, M.D. SY 10-11
I. Fatty Tumors • II. Fibrous Tumors & Tumor-like Lesions • III. FibrohistiocyticTumors • IV. Tumors of the Skeletal Muscle
V. Tumors of the Smooth Muscle • VI. Tumors of the Skeletal Muscle
A benign tumor of fat • The most common soft tissue tumor of adulthood • Age group commonly affected: middle adulthood
Pathologic Findings- Conventional Lipoma • Single or multiple subcutaneous growths that are soft, rounded or lobulated & movable against overlying skin • A well-encapsulated mass composed of sheets of mature fat cells • Arises in subcutis of proximal extremities & trunk
A malignant tumor of fat • Age group commonly affected: 40-60 y/o • Usually arises in the deep soft tissues of the proximal extremities & retroperitonemum
Histopathology • Variants depending on morphologic features: • 1) Well-differentiated • 2) Myxoid/Round cell • 3) Pleomorphic
Histopathology-Well differentiated Liposarcoma • Tumor cells are recognized as lipocytes • Tumor cells contain supernumerary rings & giant rod chromosomes
Histopathology-Myxoid & Pleomorphic Variants • Tumor contains lipoblasts (mimic fetal fat cells)
1) Superficial Fibromatoses (Palmar, Plantar & Penile Fibromatoses) • 2) Deep-seated Fibromatoses (Desmoid Tumors)
A1. Superficial Fibromatosis (Palmar, Plantar & Penile Fibromatoses)
Characterized by nodular or poorly defined broad fascicles of fibroblasts surrounded by abundant dense collagen • Male predominance
Variants of Superficial Fibromatoses • 1. Palmarfibromatosis • 2. Plantar fibromatosis • 3. Penile fibromatosis
Palmarfibromatosis • Also known as Dupuytren contracture
PalmarFibromatosis • Irregular thickening of palmar fascia ↓ W/ attachment to overlying skin ↓ • Puckering & dimpling of skin ↓ After some time • Progressive flexion contracture of 4th & 5th fingers of hand
Plantar Fibromatosis • Irregular or nodular thickening of plantar fascia • Flexion contractures are uncommon
Penile Fibromatoses • Also known as Peyronie disease • Presents as a palpable induration or mass on dorsolateral aspect of the penis
Penile Fibromatosis • May cause: 1) Abnormal curvature of the shaft 2) Constriction of the urethra 3) Both
Present as large, infiltrative masses that frequently recur after incomplete excision • Composed of well-differentiated fibroblasts • Age predilection: At any age but commonly in the teens-30 y/o
Types of Deep-setaedFibromatosis • 1) Extra-abdominal • 2) Abdominal • 3) Intra-abdominal • W/ similar gross & histologic features
Gross Pathology • Gray-white, firm, poorly demarcated masses • Rubbery & tough • Infiltrative
Extra-abdominal Fibromatosis-Gross Pathology • Sites of Origin: Musculature of: 1) Shoulder 2) Chest wall 3) Back 4) Thigh
Abdominal Fibromatosis-Gross Pathology • Site of Origin: Musculoaponeurotic structures of anterior abdominal wall in ♀ during or after pregnancy
Intra-abdominal Fibromatosis • Site: Mesentery or pelvic walls in individuals w/ familial adenomatouspolyposis (Gardner syndrome) • Mutations in APC or β-catenin genes
Histopathology • Plump fibroblasts arranged in broad sweeping fascicles w/c infiltrate the surrounding tissue
Gross Pathology • Site: deep soft tissues of extremities • Unencapsulated, infiltrative, soft, fish-flesh masses • (+) hemorrhage & necrosis
Histopathology • All degrees of differentiation • Resemble cellular fibromatosis • MTC arranged in a herringbone pattern • Tumors w/ architectural disarray, pleomorphism, mitotic figures & necrosis