Soft tissue tumors

Dr Kavita Karmacharya
 Soft tissue
• Non-epithelial tissue
• Excluding
– Skeleton,
– Jonts
– CNS
– Hematopoietic
– Lymphoid tissues
• Benign >>> Malignant – Sarcoma by 100 fold

• Sarcomas – aggressive behaviour – 2% of all

cancer mortality

• Extremities – esp thigh

• Incidence increases with age – 15% in children

 Pathogenesis
• Most sarcomas – Sporadic
• No known predisposing cause
• Some minority of soft tissue neoplasms
– Associated with germline mutation
• Tumor suppressor genes
• (neurofibromatosis 1, Gardner syndrome, Li-
Fraumeni syndrome etc)
– Environmental cause
• Radiation, burns or toxins
– Molecular genetic abnormalities:
Classification
Tumors of Adipose tissue
• LIPOMA
• LIPOSARCOMA
 LIPOMA
• Benign tumor of fat
• Most common soft tissue tumor
• Adulthood
• Subclassified
– Conventional lipoma
– Fibrolipoma
– Angiolipoma
– Spindle cell lipoma
– Myelolipoma
 Morphology
• Conventional Lipoma
– Most common subtype
– Well encapsulated mass
– Mature adipocytes
– Subcutis of proximal extremities
– Trunk
– Middle adulthood
– Large/intramuscular/poorly
circumscribed
 Clinical Feature
• Soft/mobile/painless

• Angiolipoma – painful

• Cured by simple excision

 LIPOSARCOMA
• Most common sarcomas of adulthood
• 50s to 60s
• Deep soft tissue of proximal extremities
• Retroperitoneum
• Well differentiated – 12q13-q15  MDM2
gene amplification  inhibits p53
• Myxoid liposarcoma – t(12;16)
 Morphology
• Well-differentiated Liposarcoma
– Adipocytes with scattered atypical spindle cells
– Lipoblasts
• Myxoid liposarcoma
– Abundant basophilic
extracellular matrix

– Arborizing capillaries

– Primitive cells at various

stages of adipocyte
differentiation

– Reminiscent of fetal fat

• Pleomorphic Liposarcoma
– Sheets of anaplastic cells

– Bizzrre nuclei

– Immature adipocytes

– Lipoblasts
SMOOTH MUSCLE TUMORS
• Leiomyoma
• Leiomyosarcoma
 Leiomyoma
• Benign tumor of smooth muscle
• Often – Uterus
• Uterine leiomyomas
– most common neoplasm in women
– 77% women
– Variety of symptoms/infertiliy
• Pilar leiomyomas
– Skin, nipples, scrotum, labia
– Multiple, apinful
– Cutaneous leiomyomas - Autosomal dominant trait –
associated with uterine leiomyomas and renal cell carcinoma
 Morphology
• Soft tissue leiomyomas
– 1-2 cm
• Composed of
– Fascicles of densely
eosinophilic intersecting
spindle cells
– Intersecting each other at
right angles
– Tumor cells – blunt end,
elongated nuclei
– Minimal atypia/ few mitotic
figures
– Surgery – solitary lesion
 Leiomyosarcoma
• 10-20% of soft tissue sarcomas
• Adults
• Women
• Deep soft tissues of extremities
• Retroperitoneum
• Deadly form arises from great vessels – esp IVC
• Complex genotypes – underlying defects that
lead to profound genomic instability
 Morphology
• Painless, firm masses
• Retroperitoneal tumors
– large and bulky
– Abdominal symptoms
• Eosinophilic spindle cells
with blunt end
• Hyperchromatic nuclei
arranged in interweaving
fascicles
• Ultrastructurally
– Bundles of thin filaments
– With dense bodies
– Pinocytic vesicles
– Individual cells are
surrounded by basal
lamina

• Immunohistochemically
– Stain with antibodies to
SMA and Desmin
SKELETAL MUSCLE TUMORS
• Almost all malignant

• Rhabdomyoma – frequently seen in

individuals with tuberous sclerosis

• Rhabdomyosarcoma
 RHABDOMYOSARCOMA
• Malignant mesenchymal tumor

• Skeletal muscle differentiation

• Three subtypes
– Alveolar (20%)
Children, adolesence/<20 yrs
– Embryonal (60%)
– Pleomorphic (20%) - Adults
 Pathogenesis
• Alveolar rhabdomyosarcoma
– Fusions of FOXO1 gene to either PAX3 or PAX7
gene  interferes with skeletal muscle
differentiation

– Rearrangements of (2;13) or (1;13)

– Translocations
 Morphology
• Embryonal
rhabdomyosarcoma
– Soft gray infiltrative mass

– Tumor cells – mimic skeletal

muscle at various stages of
embryogeneis

– Consists of both primitive

round and spindled cells in
myxoid stroma

– Rhabdomyoblasts – visible
cross-striations may be
present
• Alveolar rhabdomyosarcoma
– Traversed by a network of
fibrous septae
– Divide the cells into clusters or
aggregates
– Creating a crude resemblance
to pulmonary alveoli
– Discohesive cells in the cells
– Cells adhere to the septae at
– Uniform round cell, with little
cytoplasm
– Cross striations are not a
• Pleomorphic rhabdomyosarcoma
– numerous large, sometimes multinucleated,
bizarre eosinophilic tumor cells
– can resemble other pleomorphic sarcomas
– Immunohistochemistry (e.g., myogenin +)
 FIBROUS TUMORS
• Nodular fascitis
• Fibromatoses
• Deep fibromatosis
 TUMORS OF UNCERTAIN ORIGIN
• Synovial Sarcoma
• Undifferentiated Pleomorphic Sarcoma