Soft Tissue Sarcoma: Pathology and Management
SEYI IDOWU — MCh MD FMCEM FWACS FMCOrtho
Learning Points / Outline
– Introduction
– Epidemiology and Etiologic Characteristics
– Synopsis of Pathological Features
– Clinical Presentation
– Diagnosis
– Surgical Management
– Adjuvant Therapy
– Follow‑up / Surveillance
– Future Prospects
Introduction
– Musculoskeletal neoplasms are diverse; soft tissue sarcomas (STS) are even more diverse.
– STS are malignant tumors of mesenchymal origin, excluding osseous and hematopoietic tissues.
Peculiar Challenges (Nigeria Context)
– Late presentation
– Limited facilities
– Limited specialists
– Inadequate funding
– Geographical disparity in access to care
Epidemiology
– 1% of adult cancers
– 15% of paediatric malignancies
– 1500 new cases yearly in UK
– 12000 new cases yearly in USA
– Benign:malignant = 300:1
– 4700 yearly deaths in USA
– More common in males
– 60% occur in extremities; 44% in thigh
Anatomic Location
– Thigh (48%)
– Leg (13%)
– Others: arm, forearm, gluteal, foot, shoulder
Histological Profile
– Liposarcoma (24%)
– Fibrosarcoma (24%)
– Leiomyosarcoma (18%)
– Others: MPNST, synovial sarcoma, rhabdomyosarcoma, angiosarcoma, epithelioid sarcoma
Grade Distribution
– High‑grade: 46%
– Intermediate: 16%
– Low‑grade: 38%
Nigerian Studies Summary
– 9 studies
– 1064 patients
– 60% male
– Mean age ≈ 34.4 yrs
– Symptoms duration: 21–58 months
Etiologic Factors
– Environmental: radiation, arsenic, phenols
– Genetic: NF1, Li‑Fraumeni, Retinoblastoma, Gardner syndrome
– Clinical: HIV, immunosuppression, chronic lymphedema, EBV
Classic Translocations
– t(X;18) → Synovial sarcoma
– t(2;13) → Alveolar rhabdomyosarcoma
– t(12;16) → Myxoid liposarcoma
Common Soft Tissue Sarcoma Types
– UPS (MFH)
– Liposarcoma
– Synovial sarcoma
– Leiomyosarcoma
– Rhabdomyosarcoma
– MPNST
– Angiosarcoma
– DFSP
Lymph Node–Metastasizing STS
– Rhabdomyosarcoma
– Angiosarcoma
– Clear‑cell sarcoma
– Epithelioid sarcoma
– Synovial sarcoma
Staging — AJCC
– T1 <5 cm | T2 >5 cm
– Superficial vs deep classification
– Grades G1–G4
– Stage I–IV
Staging — Enneking/MSTS
– IA: Low‑grade intracompartmental
– IB: Low‑grade extracompartmental
– IIA: High‑grade intracompartmental
– IIB: High‑grade extracompartmental
– III: Metastatic
Clinical Presentation
– Painless enlarging mass
– Centrifugal growth
– Pain, edema, neurological deficits
– Fungation in late stages
– Lesions >8 cm → worse prognosis
– Superficial mass >5 cm is suspicious
Diagnosis & Imaging
– X‑ray
– CT for staging
– MRI gold standard
– Ultrasound for biopsy guidance
– PET for recurrence
Biopsy Methods
– FNAC
– Core needle biopsy
– Open incisional biopsy
– Excisional biopsy
Surgical Management
– Wide local excision (2 cm margins)
– Staged reconstruction as needed
– Amputation still required in 5–10%
– Pulmonary metastasectomy when indicated
Radiotherapy
– Indicated for high‑grade tumors
– Neoadjuvant or postoperative
– Palliative options
– External beam or brachytherapy
Chemotherapy
– Used pre‑op or palliatively
– Doxorubicin‑responsive tumors: Ewing’s, small round cell tumors, leiomyosarcoma, angiosarcoma
Complications
– Radiation wound complications (20–30%)
– Delayed healing, infection, fibrosis
– Post‑radiation fractures
– Local recurrence <10% with combined therapy
– Unplanned excision → 74% residual disease
Follow‑up / Surveillance
– Baseline MRI
– Chest X‑ray every 3 months
– Every 3 months × 2 years
– Every 6 months × 3 years
– Yearly up to 10 years
Prognosis
– ~3490 deaths yearly
– Overall mortality ~50%
– Stage I: 90% survival | Stage II: 70% | Stage III: 50% | Stage IV: 10–20%
– Poor factors: high grade, mets at diagnosis, size >5 cm, deep tumors, delayed diagnosis, unplanned excision
Thank You
End of document.
