Undifferentiated Pleomorphic Sarcoma of the Chest Wall

Author: Paul Schipper, MD
Institution: OHSU
Date Reviewed: March 2025
Original Case: Dale Han, MD
Learning Domain: General Thoracic
Learning Objective: Diagnosis and management of undifferentiated pleomorphic sarcoma
PowerPoint File: File Undifferentiated Pleomorphic Sarcoma of the Chest Wall.pptx

 

History 

  • 72 year-old female with history of right breast cancer treated with lumpectomy and radiation.  Eight years later, she develops a right anterolateral chest wall mass  
  • PMH:
    • Right breast cancer, hypertension, pancreatitis 
  • PSH:
    • Right breast lumpectomy, hysterectomy, umbilical hernia repair, bilateral tubal ligation
  • SH:
    • 10 pack year smoking history.  Quit 45 years ago

 

Exam and Labs

Pertinent Physical Exam

  • Lungs clear bilaterally
  • Heart rate regular
  • Right chest in lower right axilla with an approximately 5 cm hard fixed mass, no skin changes

Labs 

  • Normal white cell count

 

Chest CT

CT chest: 4.6 cm lobulated mass in right axilla next  to 6th and 7th ribs, no pulmonary nodules

 

Additional Studies

  • Brain MRI
  • PET scan

 

Discussion Points for Conference

  • Differential Diagnosis?
  • Diagnostic Approaches ?
    • Core Needle Biopsy
    • Incisional Biopsy
    • Excisional Biopsy
  • Therapeutic Options ?
    • Radiation Therapy
    • Wide excision
    • Combined modalities
  • Stage?

 

Further Work Up

  • Core needle biopsy of mass: atypical spindle cells
  • Excisional biopsy performed resecting mass down to and including portion of serratus anterior muscle
    • Pathology: undifferentiated pleomorphic sarcoma
    • 4.2 cm
    • Grade 2
    • Positive deep and caudal margins
  • MRI brain: negative for metastases
  • PET scan: negative for metastases

 

Surgery

  • Patient subsequently referred and evaluated for definitive management of right chest wall sarcoma
  • Right chest radical resection of excisional biopsy cavity with en bloc resection of right ribs 4, 5, 6, 7
  • Wedge resections of 2 right lower lobe pulmonary nodules found intra-operatively
  • Reconstruction of chest wall with mesh and latissimus dorsi flap

Pathology: Residual cells with morphologic appearance similar to original sarcoma.  Margins negative.  Pulmonary wedge resections negative for malignancy.

 

Further Discussion Points

  • Development of sarcoma in radiated tissue
  • Use of excisional biopsy versus upfront radical resection of chest wall
  • Extent of resection: en-bloc resection of ribs for positive deep margin after resection of serratus anterior muscle
  • Reconstruction of defect
  • Adjuvant therapy for chest wall sarcomas

 

Learning Points

  • Undifferentiated pleomorphic sarcoma previously known as malignant fibrous histiocytoma
  • Attempt needle biopsy as opposed to excisional biopsy to minimize extent of final resection
    • Atypical spindle cells on needle biopsy should be assumed to be sarcoma in the appropriate clinical setting
  • Radical wide excision is treatment with resection of involved structures such as ribs and chest wall
  • Local recurrence common (20-45%)
  • Distant recurrence common (30-50%)
  • Adjuvant therapy has not been shown to definitively improve survival