Soft Tissue Tumors

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Soft Tissue Tumors
Lucy H. Liu M.D. Department of
Pathology University of Illinois at
Chicago e-mail lucyliu_at_uic.edu
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Soft Tissue Tumors
UIC College of Medicine M2 Pathology Course
Lecture 63 Tuesday January 28 2003 1130 am
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Soft Tissue Tumors
Lecture Goals 1. Nomenclature classification
system of tumors 2. Differences between benign
and malignant tumors 3. Clinical morphologic
features of common tumors 4. Approach to
diagnosis of soft tissue tumors
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Soft Tissue Tumor

• Neoplastic conditions arising in
• extraskeletal mesodermal tissues

-- Fibrous tissue
-- Smooth muscle
-- Skeletal muscle
-- Adipose tissue
-- Blood lymphatic vessels
-- Peripheral nerves - neuroectoderm
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Soft Tissue Tumor
General principles for diagnosis

• Location

-- Deep lesions tend to be malignant
-- Superficial lesions - benign

• Size

-- Larger tumors tend to be malignant

• Growth pattern

-- Rapidly growing - malignant
-- Infiltrating - malignant

• Metastasis

-- Malignant
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Soft Tissue Tumors
Approach to Diagnosis

• Histological morphology
• Immunohistochemistry
• Cytogenetic study
• Molecular analysis
• Ultrastructure

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Soft Tissue Tumors
Immunohistochemistry

• Cytokeratin
• Vimentin
• Smooth muscle actin
• Desmin
• S-100
• CD 31
• CD 34

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Soft Tissue Tumors
Cytogenetic Changes

• t( 213) Alveolar rhabdomyosarcoma
• t( 1122) Ewings sarcoma/PNET
• t( 1122) Desmoplastic small round cell tumor
• t( 1216) Myxoid liposarcoma
• t( 922) Myxoid chondrosarcoma
• t(1222) Clear cell sarcoma
• t( X18) Synovial sarcoma

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Nodular Fasciitis

• Benign reactive soft tissue lesion

• Probably secondary to trauma

• On forearm trunk back- well circumscribed
• Spindle cells ( fibroblasts myofibroblasts)
• in loose matrix -fibroblasts in tissue
  culture

• Rapid growth frequent mitotic figures

• Self-limited cured by excision

• Must differentiate from a sarcoma

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Nodular Fasciitis
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Fibromatosis

• Fibroproliferative lesions

• Fibroblasts Myofibroblasts

• Infiltrative growth pattern

• Tendency to recur

• Desmoid - Infiltrative masses
• in abdominal extra-abdominal
• intra-abdominal

• Palmar plantar fibromatosis
• Surgical excision

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Fibromatosis
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Fibrosarcoma

• Malignant tumor of fibroblast origin

• In adults in lower extremities
• upper extremities trunk

• Spindle cells in a herringbone pattern

• Increased cellularity high
• nuclear-cytoplasmic ratios

• Must exclude other tumors which
• resemble fibrosarcoma
• - Peripheral nerve sheath tumor
• - Synovial sarcoma

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Fibrosarcoma
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Dermatofibroma

• Benign fibrous histiocytoma

• Usually in skin

• Mixture of fibroblasts
• myofibroblasts histiocytes
• Surgical excision

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Dermatofibroma
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Dermatofibrosarcoma Protuberans (DFSP)

• Fibrohistiocytic tumor
• Intermediate malignant potential

• In skin subcutis
• Spindle cells in storiform pattern

• Local recurrence
• CD 34
• Transformation to fibrosarcoma
• Surgical excision

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Dermatofibrosarcoma Protuberans ( DFSP )
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Malignant Fibrous Histiocytoma

• Malignant soft tissue tumor with
• histocytic differentiation

• Most common type of soft-tissue sarcoma

• Most frequently encountered sarcoma
• post radiation therapy

• In adults in deep soft tissue
• - extremities retroperitoneum

• Marked pleomorphism spindle cells
• storiform myxoid

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MFH
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Leiomyoma

• Benign soft tissue tumor

• Arising in subcutaneous tissue
• or blood vessel wall

• Usually painful

• Fascicles of regular smooth muscle cells

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Leiomyoma
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Leiomyosarcoma

• Malignant soft tissue tumor

• Arising in extremities of blood
• vessel wall

• Necrosis hemorrhage

• Fascicles nuclear atypia

• High mitotic activity

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Leiomyosarcoma
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Rhabdomyosarcoma

• Malignant tumor of striated muscle
• differentiation

• In children young adults

• Several subtype
• - Embryonal
• - Alveolar
• - Botryoid
• - Pleomorphic

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Embryonal Rhabdomyosarcoma

• Most common in children
• in head neck
• Most common subtype

• Rhabdomyoblasts with
• cytoplasmic cross-striation

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Embryonal rhabdomyosarcoma
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Botryoid Rhabdomyosarcoma

• Most common in hollow visceral
• organs - genitourinary tract

• Polypoid grape-like tumor masses

• Scattered malignant cells in myxoid
• stroma

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Botryoid rhabdomyosarcoma
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Alveolar Rhabdomyosarcoma

• In extremities

• Fibrous septa with loose clusters of
• rounded cells in center
• - alveolar pattern

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Alveolar rhabdomyosarcoma
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Pleomorphic Rhabdomyosarcoma

• In skeletal muscles of older
• persons in thigh

• Marked pleomorphism

• Irregularly arranged cells

• Multinucleated giant cells

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Pleomorphic Sarcoma
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Lipoma

• Benign well-circumscribed tumor of
• well-differentiated adipocytes

• In adult upper back neck shoulder

• Usually subcutaneous any site of
• adipose tissue

• Most common type of benign soft
• tissue tumor

• Resemble normal adipose tissue
• Subtypesangiolipoma spindle cell lipoma

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Lipoma
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Liposarcoma

• Second most common sarcoma in adults

• In deep compartments of extremities
• retroperitoneum
• Lipoblasts

• Several subtypes

- Well differentiated/atypical lipoma - Myxoid
/ Round cell - Pleomorphic
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Liposarcoma
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Hemangioma

• Benign lesion
• Resemble normal blood vessels
• Congenital or non-congenital
• Most common in infants children

• Head neck internal organs - liver

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Angiosarcoma

• Malignant vascular tumor
• Many locations skin soft tissue
• breast live spleen

• Irregular channels atypical
• endothelial cells
• CD31- endothelial cell marker

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Angiosarcoma
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Schwannoma

• Benign tumor of neural differentiation
• - Schwann cells

• In association with large nerve trunks
• Head neck extremities

• Antoni A
• Antoni B

-- Cellular area palisaded nuclei

• -- Less cellular area myxoid background
• S-100

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Schwannoma
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Synovial sarcoma

• Malignant soft tissue tumor

• Unknown origin- misnomer

• Arising in region of a joint
• other sites of deep soft tissue

• Multilobular

• Biphasic pattern
• - spindle cells
• - epithelial - like cells
• t( X18 )

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Synovial sarcoma