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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

Pathology of the Soft Tissues & Nerves

Arlene L. Santos, M.D.

SY 10-11

slide3

I. Fatty Tumors

  • II. Fibrous Tumors & Tumor-like Lesions
  • III. FibrohistiocyticTumors
  • IV. Tumors of the Skeletal Muscle
slide4

V. Tumors of the Smooth Muscle

  • VI. Tumors of the Skeletal Muscle
slide8

A benign tumor of fat

  • The most common soft tissue tumor of adulthood
  • Age group commonly affected: middle adulthood
pathologic findings conventional lipoma
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
slide14

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
Histopathology
  • Variants depending on morphologic features:
  • 1) Well-differentiated
  • 2) Myxoid/Round cell
  • 3) Pleomorphic
histopathology well differentiated liposarcoma
Histopathology-Well differentiated Liposarcoma
  • Tumor cells are recognized as lipocytes
  • Tumor cells contain supernumerary rings & giant rod chromosomes
histopathology myxoid pleomorphic variants
Histopathology-Myxoid & Pleomorphic Variants
  • Tumor contains lipoblasts (mimic fetal fat cells)
slide24

1) Superficial Fibromatoses (Palmar,

Plantar & Penile Fibromatoses)

  • 2) Deep-seated Fibromatoses (Desmoid

Tumors)

slide26

Characterized by nodular or poorly defined broad fascicles of fibroblasts surrounded by abundant dense collagen

  • Male predominance
variants of superficial fibromatoses
Variants of Superficial Fibromatoses
  • 1. Palmarfibromatosis
  • 2. Plantar fibromatosis
  • 3. Penile fibromatosis
palmar fibromatosis
Palmarfibromatosis
  • Also known as Dupuytren contracture
palmar fibromatosis1
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
Plantar Fibromatosis
  • Irregular or nodular thickening of plantar fascia
  • Flexion contractures are uncommon
penile fibromatoses
Penile Fibromatoses
  • Also known as Peyronie disease
  • Presents as a palpable induration or mass on dorsolateral aspect of the penis
penile fibromatosis
Penile Fibromatosis
  • May cause:

1) Abnormal curvature of the shaft

2) Constriction of the urethra

3) Both

slide36

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 setaed fibromatosis
Types of Deep-setaedFibromatosis
  • 1) Extra-abdominal
  • 2) Abdominal
  • 3) Intra-abdominal
  • W/ similar gross & histologic features
gross pathology
Gross Pathology
  • Gray-white, firm, poorly demarcated masses
  • Rubbery & tough
  • Infiltrative
extra abdominal fibromatosis gross pathology
Extra-abdominal Fibromatosis-Gross Pathology
  • Sites of Origin: Musculature of:

1) Shoulder

2) Chest wall

3) Back

4) Thigh

abdominal fibromatosis gross pathology
Abdominal Fibromatosis-Gross Pathology
  • Site of Origin: Musculoaponeurotic structures of anterior abdominal wall in ♀ during or after pregnancy
intra abdominal fibromatosis
Intra-abdominal Fibromatosis
  • Site: Mesentery or pelvic walls in individuals w/ familial adenomatouspolyposis (Gardner syndrome)
  • Mutations in APC or β-catenin genes
histopathology1
Histopathology
  • Plump fibroblasts arranged in broad sweeping fascicles w/c infiltrate the surrounding tissue
gross pathology1
Gross Pathology
  • Site: deep soft tissues of extremities
  • Unencapsulated, infiltrative, soft, fish-flesh masses
  • (+) hemorrhage & necrosis
histopathology2
Histopathology
  • All degrees of differentiation
  • Resemble cellular fibromatosis
  • MTC arranged in a herringbone pattern
  • Tumors w/ architectural disarray, pleomorphism, mitotic figures & necrosis
gross pathology2
Gross Pathology
  • Firm, small, mobile nodule
histopathology3
Histopathology
  • Variable mixture of :

-Histiocyte-like cells

▪ Some foamy

▪ Others multinucleated

▪ Others containing hemosiderin

-Fibroblast-like cells

histopathology4
Histopathology
  • Maybe:

-Extremely cellular

  • Mitotic activity usually scanty or absent
slide58

Phenotype of TC: fibroblastic

  • Now: dropped as a diagnostic entity
histopathology5
Histopathology
  • Soft tissue tumors characterized by:

-Marked cytologicpleomorphism

-Bizarre multinucleate cells

-Storiform architecture

types
Types
  • 1) Embryonal
  • 2) Alveolar
  • 3) Pleomorphic
histopathology6
Histopathology
  • Rhabdomyoblast

-Diagnostic cell in all types

-Eccentric eosinophilicgranular

cytoplasm

-Round or elongate

-(+)Cross-striations

embryonal rhabdomyosarcoma
EmbryonalRhabdomyosarcoma
  • Variants:

1) Sarcoma botryoides

2) Spindle cell type

3)Anaplastic type

  • Age predilection: < 10 y/o
embryonal rhabdomyosarcoma1
EmbryonalRhabdomyosarcoma
  • Sites of Origin:

1) Nasal cavity

2) Orbit

3) Middle ear

4) Prostate

5) Paratesticular region

sarcoma botryoides
Sarcoma Botryoides
  • Develops in wall of mucosal-lined structures s.a. :

-Nasopharynx

-Common bile duct

-Bladder

-Vagina

  • Histopathology:Cambium layer
alveolar rhabdomyosarcoma gross pathology
Alveolar Rhabdomyosarcoma –Gross Pathology
  • Site of origin: deep musculature of extremities
alveolar rhabdomyosarcoma histopathology
Alveolar Rhabdomyosarcoma-Histopathology
  • 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

pleomorphic rhabdomyosarcoma gross pathology
PleomorphicRhabdomyosarcoma-Gross Pathology
  • Site of origin: Deep soft tissue of adults
pleomorphic rhabdomyosarcoma histopathology
PleomorphicRhabdomyosarcoma-Histopathology
  • TC-large, maybe multinucleated, bizarre & eosinophilic
types1
Types
  • 1) Cutaneous
  • 2) Genital
  • 3) Vascular
gross pathology3
Gross Pathology
  • Yellow-yellowish pink
  • Sharply circumscribed
  • Fairly firm
histopathology7
Histopathology
  • Intersecting fascicles of smooth muscle cells:

-Encircle vascular lumina lined by

normal endothelial cells

  • No:

-Mitotic activity

-Necrosis

-Hemorrhage

slide85

A malignant mesenchymaltumor w/ predominantly smooth muscle differentiation

  • Typically :

-Adults

-Elderly

gross pathology4
Gross Pathology
  • Location: anywhere but mostly in extremities
  • May arise from walls of arteries, veins, venules & arterioles
gross pathology5
Gross Pathology
  • Maybe as well-circumscribed as leiomyoma but:

-Larger

-Softer

-Tendency for:

▪ Tumor necrosis

▪ Hemorrhage

▪ Cystic degeneration

histopathology8
Histopathology
  • Pattern of Growth:

-Predominantly fascicular

-Tumor bundles intersect at wide angles

histopathology9
Histopathology
  • Cellular features:

-Elongated blunt-ended nuclei

-Acidophilic fibrillary cytoplasm

slide94

Cell of origin is unclear

  • Age predilection: Mostly 20-40 y/o
gross pathology6
Gross Pathology
  • Location:

1) Deep soft tissue (frequently the lower

extremity)

2) Head & Neck

3) Viscera

histopathology10
Histopathology
  • Maybe monophasic or biphasic
  • (+) Calcified concretions
monophasic synovial sarcoma
Monophasic Synovial Sarcoma
  • Spindle cells only
  • Epithelial cells only
biphasic synovial sarcoma
Biphasic Synovial Sarcoma
  • Both epithelial-like cells & mesenchymal-like cells
  • Epithelial cells:

-Cuboidal to columnar

-Pattern: form glands, cords or

aggregates

biphasic synovial sarcoma1
Biphasic Synovial Sarcoma
  • Spindle cells:

-Form fascicles that surround epithelial

cells

slide103

Peripheral Nerve Sheath Tumors

A. Schwannoma

B. Neurofibroma

C. Malignant Peripheral Nerve Sheath

Tumor

slide105

Usually adulthood

  • Location:

-Cerebellopontine angle

▪Nearly all produce hearing loss

▪Bilateral eighth nerve examples are

defining feature of neurofibromatosis

type 2

slide106

Location:

-Can present as interventricular masses

-May involve cranial nerves other than

acoustic

gross pathology7
Gross Pathology
  • Cerebellopontine angle:

-Nearly all originate in vestibular branch

of cranial nerve VII (Acoustic

schwannoma or neuroma)

gross pathology8
Gross Pathology
  • Lumbosacral spinal extramedullaryspace

-Predilection for sensory divisions,

typically the posterior roots

-Often “dumbbell” configuration

gross pathology9
Gross Pathology
  • NF-2-associated variant:

-Often multilobulated growth pattern

histopathology11
Histopathology
  • Antoni A and B structure
  • Verocay bodies
  • Infiltration by foamy macrophages
  • Vascular hyalinization
types2
Types
  • 1) CutaneousNeurofibroma
  • 2) PlexiformNeurofibroma
cutaneous neurofibroma gross pathology
CutaneousNeurofibroma-Gross Pathology
  • Location: demis & subcutaneous fat
  • Unencapsulated masses
cutaneous neurofibroma histopathology
CutaneousNeurofibroma-Histopathology
  • Spindle cells
  • W/ a collagenousstroma w/ little myxoid material
plexiform neurofibroma gross pathology
PlexiformNeurofibroma-Gross Pathology
  • Site of origin: anywhere along a nerve
  • Affected nerves are irregularly expanded
plexiform neurofibroma histopathology
PlexiformNeurofibroma-Histopathology
  • Cell types present:

-Schwann cells

-Fibroblastic sells

-Inflammatory cells

  • Loose myxoidstroma w/ areas containing collagen bundles
slide124

Originate in:

-Cranial nerve roots

-Spinal Nerve roots

  • May arise in:

-Cerebral parenchyma

-Lateral ventricle

histopathology12
Histopathology
  • Variable
  • Majority - fibrosarcomatous, herringbone pattern is detectable focally
  • Densely cellular tumor
  • Frequent mitotic figures
histopathology13
Histopathology
  • Geographic necrosis
histopathology14
Histopathology
  • Cytologic features:

-Elongated nuclei w/ tapered ends