What are soft tissue tumours?
• ‘Soft tissue’ is defined as non-epithelial extraskeletal tissue:
∘ Muscle
∘ Fat
∘ Fibrous tissue
∘ Vessels.
• By convention, peripheral nerves are also defined as soft tissue.
How are soft tissue tumours broadly classified?
What types of benign soft tissue tumours are there?
• Resemble normal tissue: ∘ Lipoma. ∘ Leiomyoma. ∘ Rhabdomyoma. ∘ Lymphangioma. ∘ Schwannoma. • Limited capacity for autonomous growth. • Not locally invasive; low rate of recurrence following treatment.
What are malignant soft tissue tumours?
What are tumours of intermediate malignancy? Give some examples.
• These are tumours of borderline or low malignant potential.
• May have high local recurrence rate but low risk of metastasis.
• Examples:
∘ Dermatofibrosarcoma protuberans (DFSP)
∘ Kaposiform haemangioendothelioma
∘ Atypical lipoma
∘ Haemangiopericytoma.
What is the definition of sarcoma?
• Peripheral nerve malignancies although of ectodermal origin, are also considered sarcomas.
What is the epidemiology of sarcomas?
What is the aetiology of sarcomas?
Sporadic (majority).
Potential causative factors:
∘ Genetic
(a) NF-1: up to 10% lifetime risk of malignant transformation (MPNST: malignant peripheral nerve sheath tumours).
(b) Gardner syndrome (familial polyposis coli) –> desmoid tumours.
(c) Hereditary retinoblastoma survivors –> STS.
(d) Gorlin’s syndrome –> fibrosarcoma and rhabdomyosarcoma.
(e) Li Fraumeni syndrome (germline mutation in p53) –> STS.
∘ Radiation (e.g. breast) –> angiosarcoma (16-fold) & STS (2-fold increase)
∘ Chronic lymphoedema –> lymphangiosarcoma.
∘ Chronic burn scar.
∘ Chemical exposure: phenoxyacetic herbicides, Agent Orange, arsenic.
∘ Viral infection: herpes virus or HIV –> Kaposi sarcoma; Epstein-Barr virus (EBV) –> smooth muscle sarcoma.
How do sarcomas present?
Features
1 feature - 16%; 2 - 43%; 3 - 65%; 4 - 86% chance of sarcoma.
What is the diagnostic pathway for sarcomas?
What is the appropriate management for suspected sarcomas?
What is covered in the clinical assessment?
∘ Extent of resection
∘ Regional lymphadenopathy (rare in sarcoma)
∘ Reconstruction required.
What is the purposes of different radiological assessments and how should they be ordered?
∘ Imaging usually done before biopsy to minimise artefact from bleeding or oedema.
∘ Localises and characterises tumour.
∘ Stages the disease.
What different biopsies may be performed?
Biopsies, incisions and drain sites are placed within the zone of future excision.
How is treatment planned after 1,2,3?
∘ Neoadjuvant and adjuvant therapies.
∘ Surgery and reconstruction.
How are sarcomas classified?
∘ Histological type
∘ TNMG classification
∘ Stage.
Name some different histological types of sarcomas.
Too many! Few e.g.s
∘ Smooth muscle - leiomyosarcoma
∘ Striated muscle - rhabdomyosarcoma
∘ Fat - liposarcoma
∘ Blood and lymph vessels - angiosarcoma, Kaposi sarcoma
∘ Fibrous tissue - malignant fibrous histiocytoma (MFH)
∘ Nerve - malignant peripheral nerve sheath tumour (MPNST)
∘ Synovium - synovial sarcoma
∘ Skin - atypical fibroxanthoma (AFX).
What is the TNMG classification of sarcomas?
T
Primary tumour (T) • Tx: Cannot be assessed • T0: No evidence of primary tumour • T1: Tumour ≤5 cm ∘ a: Superficial ∘ b: Deep • T2: Tumour >5 cm ∘ a: Superficial ∘ b: Deep
(Superficial tumour = superficial to fascia).
What is the TNMG classification of sarcomas?
N
Regional lymph nodes:
• Nx: Cannot be assessed.
• N0: No mets.
• N1: RLN metastasis.
What is the TNMG classification of sarcomas?
M
Distant metastasis (M)
• M0: No distant metastasis
• M1: Distant metastasis.
What is the TNMG classification of sarcomas?
G
Trojani grade (G) • Based on number of mitoses, presence of necrosis, degree of differentiation. • G1: low-grade tumour • G2: intermediate-grade tumour • G3: high-grade tumour.
What is the clinical staging for sarcomas?
.
What is the Enneking Classification for benign sarcomas?
.
What is the Enneking Classification for malignant sarcomas?
.