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 I. Fatty Tumors  II. Fibrous Tumors & Tumor-like Lesions  III. Fibrohistiocytic Tumors  IV. Tumors of the Skeletal Muscle.

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Presentation on theme: " I. Fatty Tumors  II. Fibrous Tumors & Tumor-like Lesions  III. Fibrohistiocytic Tumors  IV. Tumors of the Skeletal Muscle."— Presentation transcript:

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3  I. Fatty Tumors  II. Fibrous Tumors & Tumor-like Lesions  III. Fibrohistiocytic Tumors  IV. Tumors of the Skeletal Muscle

4  V. Tumors of the Smooth Muscle  VI. Tumors of the Skeletal Muscle

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8  A benign tumor of fat  The most common soft tissue tumor of adulthood  Age group commonly affected: middle adulthood

9  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

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14  A malignant tumor of fat  Age group commonly affected: y/o  Usually arises in the deep soft tissues of the proximal extremities & retroperitonemum

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16  Variants depending on morphologic features:  1) Well-differentiated  2) Myxoid/Round cell  3) Pleomorphic

17  Tumor cells are recognized as lipocytes  Tumor cells contain supernumerary rings & giant rod chromosomes

18  Tumor contains lipoblasts (mimic fetal fat cells)

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24  1) Superficial Fibromatoses (Palmar, Plantar & Penile Fibromatoses)  2) Deep-seated Fibromatoses (Desmoid Tumors)

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26  Characterized by nodular or poorly defined broad fascicles of fibroblasts surrounded by abundant dense collagen  Male predominance

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28  1. Palmar fibromatosis  2. Plantar fibromatosis  3. Penile fibromatosis

29  Also known as Dupuytren contracture

30  Irregular thickening of palmar fascia ↓ W/ attachment to overlying skin ↓  Puckering & dimpling of skin ↓ After some time  Progressive flexion contracture of 4 th & 5 th fingers of hand

31  Irregular or nodular thickening of plantar fascia  Flexion contractures are uncommon

32  Also known as Peyronie disease  Presents as a palpable induration or mass on dorsolateral aspect of the penis

33  May cause: 1) Abnormal curvature of the shaft 2) Constriction of the urethra 3) Both

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36  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

37  1) Extra-abdominal  2) Abdominal  3) Intra-abdominal  W/ similar gross & histologic features

38  Gray-white, firm, poorly demarcated masses  Rubbery & tough  Infiltrative

39  Sites of Origin: Musculature of: 1) Shoulder 2) Chest wall 3) Back 4) Thigh

40  Site of Origin: Musculoaponeurotic structures of anterior abdominal wall in ♀ during or after pregnancy

41  Site: Mesentery or pelvic walls in individuals w/ familial adenomatous polyposis (Gardner syndrome)  Mutations in APC or β- catenin genes

42  Plump fibroblasts arranged in broad sweeping fascicles w/c infiltrate the surrounding tissue

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45  Site: deep soft tissues of extremities  Unencapsulated, infiltrative, soft, fish- flesh masses  (+) hemorrhage & necrosis

46  All degrees of differentiation  Resemble cellular fibromatosis  MTC arranged in a herringbone pattern  Tumors w/ architectural disarray, pleomorphism, mitotic figures & necrosis

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52  Benign tumor of the soft tissue w/ a fibrohistiocytic origin

53  Firm, small, mobile nodule

54  Variable mixture of : -Histiocyte-like cells ▪ Some foamy ▪ Others multinucleated ▪ Others containing hemosiderin -Fibroblast-like cells

55  Maybe: -Extremely cellular  Mitotic activity usually scanty or absent

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58  Phenotype of TC: fibroblastic  Now: dropped as a diagnostic entity

59  Soft tissue tumors characterized by: -Marked cytologic pleomorphism -Bizarre multinucleate cells -Storiform architecture

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63  1) Embryonal  2) Alveolar  3) Pleomorphic

64  Rhabdomyoblast -Diagnostic cell in all types -Eccentric eosinophilic granular cytoplasm -Round or elongate -(+)Cross-striations

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66  Variants: 1) Sarcoma botryoides 2) Spindle cell type 3)Anaplastic type  Age predilection: < 10 y/o

67  Sites of Origin: 1) Nasal cavity 2) Orbit 3) Middle ear 4) Prostate 5) Paratesticular region

68  Develops in wall of mucosal-lined structures s.a. : -Nasopharynx -Common bile duct -Bladder -Vagina  Histopathology:Cambium layer

69  Site of origin: deep musculature of extremities

70  Fibrous septae w/c divide cell into clusters or aggregates (pulmonary alveoli)  TC-moderate size & w/ little cytoplasm  Center- dyscohesive -Periphery-TC line the septae

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72  Site of origin: Deep soft tissue of adults

73  TC-large, maybe multinucleated, bizarre & eosinophilic

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77  A benign stromal tumor mainly composed of mature smooth muscle bundles

78  1) Cutaneous  2) Genital  3) Vascular

79  Yellow-yellowish pink  Sharply circumscribed  Fairly firm

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81  Intersecting fascicles of smooth muscle cells: -Encircle vascular lumina lined by normal endothelial cells  No: -Mitotic activity -Necrosis -Hemorrhage

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85  A malignant mesenchymal tumor w/ predominantly smooth muscle differentiation  Typically : -Adults -Elderly

86  Location: anywhere but mostly in extremities  May arise from walls of arteries, veins, venules & arterioles

87  Maybe as well-circumscribed as leiomyoma but: -Larger -Softer -Tendency for: ▪ Tumor necrosis ▪ Hemorrhage ▪ Cystic degeneration

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89  Pattern of Growth: -Predominantly fascicular -Tumor bundles intersect at wide angles

90  Cellular features: -Elongated blunt-ended nuclei -Acidophilic fibrillary cytoplasm

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94  Cell of origin is unclear  Age predilection: Mostly y/o

95  Location: 1) Deep soft tissue (frequently the lower extremity) 2) Head & Neck 3) Viscera

96  Maybe monophasic or biphasic  (+) Calcified concretions

97  Spindle cells only  Epithelial cells only

98  Both epithelial-like cells & mesenchymal- like cells  Epithelial cells: -Cuboidal to columnar -Pattern: form glands, cords or aggregates

99  Spindle cells: -Form fascicles that surround epithelial cells

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103 I. Peripheral Nerve Sheath Tumors A. Schwannoma B. Neurofibroma C. Malignant Peripheral Nerve Sheath Tumor

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105  Usually adulthood  Location: -Cerebellopontine angle ▪ Nearly all produce hearing loss ▪ Bilateral eighth nerve examples are defining feature of neurofibromatosis type 2

106  Location: -Can present as interventricular masses -May involve cranial nerves other than acoustic

107  Cerebellopontine angle: -Nearly all originate in vestibular branch of cranial nerve VII (Acoustic schwannoma or neuroma)

108  Lumbosacral spinal extramedullary space -Predilection for sensory divisions, typically the posterior roots -Often “dumbbell” configuration

109  NF-2-associated variant: -Often multilobulated growth pattern

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111  Antoni A and B structure  Verocay bodies  Infiltration by foamy macrophages  Vascular hyalinization

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115  Most are manifestations of neurofibromatosis type 1

116  1) Cutaneous Neurofibroma  2) Plexiform Neurofibroma

117  Location: demis & subcutaneous fat  Unencapsulated masses

118  Spindle cells  W/ a collagenous stroma w/ little myxoid material

119  Site of origin: anywhere along a nerve  Affected nerves are irregularly expanded

120  Cell types present: -Schwann cells -Fibroblastic sells -Inflammatory cells  Loose myxoid stroma w/ areas containing collagen bundles

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124  Originate in: -Cranial nerve roots -Spinal Nerve roots  May arise in: -Cerebral parenchyma -Lateral ventricle

125  Often originate in neurofibroma

126  Variable  Majority - fibrosarcomatous, herringbone pattern is detectable focally  Densely cellular tumor  Frequent mitotic figures

127  Geographic necrosis

128  Cytologic features: -Elongated nuclei w/ tapered ends

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