Soft Tissue Tumors

1
Soft Tissue Tumors
Lucy H. Liu, M.D. Department of
Pathology University of Illinois at
Chicago e-mail lucyliu_at_uic.edu
2
Soft Tissue Tumors
UIC College of Medicine M2 Pathology Course,
Lecture 63 Tuesday, January 28, 2003 1130 am
3
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
4
Soft Tissue Tumor

• Neoplastic conditions arising in
• extraskeletal mesodermal tissues

-- Fibrous tissue
-- Smooth muscle
-- Skeletal muscle
-- Adipose tissue
-- Blood lymphatic vessels
-- Peripheral nerves - neuroectoderm
5
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
6
Soft Tissue Tumors
Approach to Diagnosis

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

7
Soft Tissue Tumors
Immunohistochemistry

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

8
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

9
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

10
Nodular Fasciitis
11
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

12
Fibromatosis
13
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

14
Fibrosarcoma
15
Dermatofibroma

• Benign fibrous histiocytoma

• Usually in skin

• Mixture of fibroblasts,
• myofibroblasts, histiocytes
• Surgical excision

16
Dermatofibroma
17
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

18
Dermatofibrosarcoma Protuberans ( DFSP )
19
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

20
MFH
21
Leiomyoma

• Benign soft tissue tumor

• Arising in subcutaneous tissue
• or blood vessel wall

• Usually painful

• Fascicles of regular smooth muscle cells

22
Leiomyoma
23
Leiomyosarcoma

• Malignant soft tissue tumor

• Arising in extremities of blood
• vessel wall

• Necrosis, hemorrhage

• Fascicles, nuclear atypia

• High mitotic activity

24
Leiomyosarcoma
25
Rhabdomyosarcoma

• Malignant tumor of striated muscle
• differentiation

• In children young adults

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

26
(No Transcript)
27
Embryonal Rhabdomyosarcoma

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

• Rhabdomyoblasts with
• cytoplasmic cross-striation

28
Embryonal rhabdomyosarcoma
29
Botryoid Rhabdomyosarcoma

• Most common in hollow visceral
• organs - genitourinary tract

• Polypoid, grape-like tumor masses

• Scattered malignant cells in myxoid
• stroma

30
Botryoid rhabdomyosarcoma
31
Alveolar Rhabdomyosarcoma

• In extremities

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

32
Alveolar rhabdomyosarcoma
33
Pleomorphic Rhabdomyosarcoma

• In skeletal muscles of older
• persons, in thigh

• Marked pleomorphism

• Irregularly arranged cells

• Multinucleated giant cells

34
Pleomorphic Sarcoma
35
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

36
Lipoma
37
Liposarcoma

• Second most common sarcoma in adults

• In deep compartments of extremities
• retroperitoneum
• Lipoblasts

• Several subtypes

- Well differentiated/atypical lipoma - Myxoid
/ Round cell - Pleomorphic
38
Liposarcoma
39
Hemangioma

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

• Head neck, internal organs - liver

40
(No Transcript)
41
Angiosarcoma

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

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

42
Angiosarcoma
43
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

44
Schwannoma
45
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 )

46
Synovial sarcoma