‫المملكة العربية السعودية‬

‫وزارة التعليم‬
‫جامعة نجران‬
‫كلية الطب‬

‫‪Soft Tissue Tumors‬‬

‫‪1445 - 2023‬‬

‫‪Dr. Abdulrahman Ibrahim‬‬

‫‪Dr. Amal Shediwah‬‬
Objectives
• Define soft tissue tumors.
• Discuss the Incidence and clinical presentation of soft tissue
tumors.
• Classify soft tissue tumors histologically.
• Identify the ancillary techniques used for diagnosis of soft
tissue tumors (Imaging, biopsy, immunohistochemistry).
• Identify the significance of histopathological analysis in the
grading and staging systems of soft tissue tumors for patient
management.
Definition
• Soft tissue refers to non-epithelial tissue excluding the
skeleton, joints, central nervous system, hematopoietic and
lymphoid tissues
• Voluntary muscles, fat, fibrous tissue, peripheral nervous
system
• Derived from mesoderm (and neuroectoderm)
Frequency
• Benign: Malignant = 100:1 “with exception”
• <1% of all cancers
• Sarcomas cause 2% of all cancer mortality “reflecting their
aggressive behavior”
• Most soft tissue tumors arise in the extremities, especially the
thigh
• ~15% arise in children but the incidence increases with age
• M> F
Etiology
• Unknown “sporadic”
• Genetic factors (associated with germline mutations in
tumor suppressor genes e.g.,neurofibromatosis 1,
Gardner syndrome, Li-Fraumeni syndrome, Osler-
Weber Rendu syndrome)
• Environmental factors (radiation, burns, or toxins)
• Viruses: Herpes virus 8 ➔ Kaposi sarcoma
Clinical Features
• Mass/ lump, usually painless
• Mass with pain may occur in some of the rapidly growing
sarcomas
• Less common: GIT bleeding, tubal organs (e.g. intestine)
obstruction, systemic symptoms (fever, loss of weight, fatigue &
anemia)
• N.B.:
– Based on clinical features it is difficult to differentiate between
benign and malignant tumors.
– Generally, superficial ones of >5cm diameter or deep seated
tumors are likely to be sarcomas
Investigation
• Biopsy and histopathology is the diagnostic test, H&E stained,
sometimes special stains may help, but of great help is IHC
(immunohistochemistry, what is it?)
• Cytogenetics: chromosomal analysis
• Genetic studies for certain mutations
OTHERS
• Plain X-ray
• CT scan
• MRI
• Bone scan
• Angiogram
Biopsy and histopathology
• What to see under the microscope?
Histopathology
Morphology of Cells in Soft Tissue Tumors
Spindle Cells
Small Round Cells
Epithelioid Cells
Architectural Patterns in Soft Tissue
Tumors
Synovial Sarcoma

Peripheral Nerve Sheath Tumor

Leiomyoma

Fibrosarcoma

Fibrohistiocytic

Plexiform Schwannoma

Alveolar Soft Part Sarcoma

Schwannoma
Classification
• Continues to evolve as new molecular genetic
abnormalities are identified
• Clinically:
• Benign “self-limited lesions that require minimal treatment”
• Intermediate “locally aggressive tumors with minimal metastatic risk”
• Malignant “sarcoma”
• Pathologically: integrates morphology (e.g., muscle
differentiation), immunohistochemistry, and molecular
diagnostics:
Classification
Prognostic Parameters
1- Histologic type
2- Grading based on:
a) Degree of differentiation
b) Average number of mitoses per high-power field
c) Cellularity
d) Pleomorphism
e) Necrosis
3- Tumor size
4- Depth: tumors arising in superficial locations (e.g., skin and
subcutis) have a better prognosis than deep-seated lesions
5- Stage (degree of spread) of the tumor
Grading
• Degree of differentiation: Well-differentiated anaplastic
• Mitotic activity
• Cellularity
• Pleomorphism
• The extent of necrosis
• Expansive, infiltrative, invasive growth
• Matrix formation (hemorrhage, calcification, collagen or mucoid)
 FNLCC Sarcoma grading system

Summation of
scores
And Grading
result
Adipocytic
• Benign:
• Lipoma

• Malignant:
• Liposarcoma
 Lipoma
• The most common soft tissue tumor of adulthood
• May be single or multiple
• Superficial or deep
• Types:conventional lipoma, fibrolipoma, angiolipoma, spindle cell lipoma, myelolipoma, and pleomorphic
Pathology :
1) Well-encapsulated mass of mature adipocytes
• Soft, well-circumscribed, thinly-encapsulated rounded masses
• Few millimeters to > 10 cm
2) Site: Subcutis of the proximal extremities and trunk
3) Histologically, they consist of mature white fat cells with no pleomorphism
Signs/Symptoms:
• Painless (except angiolipoma),soft, solitary, mobile mass
• Age:
• Adults (40-60 years)
• Rare in 1st 2 decades
• Sex:
• M>F
Liposarcoma
• One of the most common sarcomas of adulthood
• Age:
• Late adult life (60-70 years), uncommon in children
• Myxoid and round cell subtypes occur in 5th decade
• Anatomic Distribution: Arise in the deep soft tissues of the
proximal extremities and retroperitoneum and are notorious for
developing into large tumors
• Signs/Symptoms:
• Extremity tumors develop as slow-growing masses
• Retroperitoneal tumors are associated with abdominal symptoms
Liposarcoma
Pathology:
• Histological variants are:
1) Well-differentiated variant
is relatively indolent
2) Myxoid type is intermediate
in its malignant behavior
3) Round cell : aggressive
4) Pleomorphic: aggressive
Skeletal Muscles
• Benign :
• Rhabdomyoma, very
rare and if it occurs it
does so in the heart
• Malignant
• Rhabdomyosarcoma
Rhabdomyosarcoma
• Most common soft tissue sarcoma of childhood and adolescence
• Locations that do not normally contain much skeletal muscle (do
not arise from mature, terminally differentiated mesenchymal
cells)
• Three types
• Alveolar (20%): extremities, typically forearm
• Embryonal (60%): head and neck or the reproductive
“Sarcoma Botryoid in vaginal wall”
• and urinary organs
• Pleomorphic (20%): extremities
Rhabdomyosarcoma
• The rhabdomyoblasts may be
round or elongate; the latter
are known as tadpole or strap
cells and may contain cross-
striations visible by light
microscopy
• Immunohistochemically they
stain with antibodies to the
myogenic markers: desmin,
MYOD1, and myogenin
Rhabdomyoblasts
Fibroblastic/Myofibroblastic
• Benign
• Nodular Fasciitis
REACTIVE PSEUDOSARCOMATOUS PROLIFERATIONS
• Myositis ossificans
• Palmar fibromatosis
• Abdominal fibromatosis (desmoid tumor)

• Malignant
• Fibrosarcoma
Fibrosarcoma
• Anatomic Distribution:
• Adult-type
» Deep soft tissues of lower extremities
» Upper extremities; head & neck
» Rare organ involvement (heart, lung, liver, CNS)
• Infantile-type
» Distal extremities
• Gross Findings:
• Solitary, lobulated, circumscribed and frequently encapsulated
• May invade adjacent structures
Nerve Sheath
• Benign:
• Schwannoma
(Neurilemoma)
• Neurofibroma

• Malignant:
• Malignant Peripheral
Nerve Sheath Tumor
(MPNST)
Schwannoma
• 90% of tumors are sporadic
• Signs/Symptoms:
• Freely mobile, non-painful
lesion
• Age:
• All ages; most common
between 20-50 years
• Sex:
• M=F
• Anatomic Distribution:
• Head & neck, flexor surfaces
Palisading
of upper and lower
extremities
 Neurofibroma
• Growth pattern may be :
a. Localized: are sporadic and
not associated with NF1
b. Diffuse
c. Plexiform

• Diffuse and plexiform tumors

occur in the setting of
neurofibromatosis type I gene
(NF1) aka, von Recklinghausen’s
disease
Malignant Peripheral Nerve Sheath Tumor
• 25-50% of tumors found in patients with NF1
• Signs/Symptoms:
• Mass lesion
• Anatomic Distribution:
• Most arise in association with major nerve trunks (sciatic
nerve, brachial and sacral plexi)
• Gross Findings:
• Eccentric masses (> 5 cm) with hemorrhage and necrosis