Updated: 5/1/2020

Undifferentiated Pleomorphic Sarcoma

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Introduction
  • Overview
    • Undifferentiated pleomorphic sarcoma (UPS; previously malignant fibrous histiocytoma) is a malignant fibrogenic tumor typically seen in patients 30-80 years of age
      • treatment is usually wide-margin local excision with adjuvant radiotherapy
  • Epidemiology
    • incidence
      • 1-9/100,000
    • demographics
      • most common soft-tissue sarcoma in adults 55-80 years
      • caucasian > African American 
      • male > female
    • location
      • extremities (lower > upper)
      • retroperitoneum
  • Genetics
    • mutations
      • programmed cell death protein 1 (PD-1) expression in 30%
  • Prognosis
    • survival with treatment
      • 50-60% 5-year survival
Classification

AJCC Staging System for Soft Tissue Sarcoma of the Extremity or Trunk
Stage Size Lymph Node Involvement
Metastasis
Grade
IA
< 5 cm
none/unknown none low
IB
> 5 cm none/unknown none low
II < 5 cm none/unknown none intermediate/high
IIIA 5-10 cm none/unknown none intermediate/high
IIIB > 10 cm none/unknown none intermediate/high
IVA any
regional none any
IVB any
any
present any
 
Presentation
  • History
    • may have history of trauma that draws attention to the mass
  • Symptoms
    • may present with fever
    • common symptoms
      • slow-growing, painless mass
    • location
      • lower > upper extremities > retroperitoneum
  • Physical exam
    • inspection
      • painless soft tissue mass may be palpable
      • lack of ecchymosis suggests an encapsulated mass 
        • could contradict diagnosis of hematoma, which typically presents with ecchymosis after trauma
    • neurovascular
      • mass effect may cause neurovascular symptoms
Imaging
  • Radiograph 
    • indications
      • obtain plain radiographs with orthogonal views for initial workup
  • MRI 
    • indications
      • mandatory to evaluate soft tissue lesions in the extremities and determine treatment algorithm
    • findings
      • T1: low signal intensity (isointense with muscle) 
      • T2: high signal intensity 
Studies
  • Serum labs
    • may present with hypoglycemia
    • may present with elevated WBC
  • Histology
    • gross anatomy
      • nodular, gray-white 
    • histology 
      • 3 subtypes
        • pleomorphic (80-85%)  
        • giant cell (10%)
        • inflammatory (<10%)
      • spindled, histiocytic, and multinucleated eosinophilic giant cells 
      • cells arranged in storiform (cartwheel) pattern arranged around small vessels
      • atypia and mitoses present
Treatment
  • Operative
    • biopsy
      • indications
        • mass concerning for malignancy after clinical and radiological workup
      • outcomes
        • best results if done at the institution where definitive surgery will take place, under a multidisciplinary team
    • wide surgical resection & radiation therapy
      •  indications
        • standard of care in most cases 
        • radiation is an important adjunct to surgery decreasing local recurrence 
        • chemotherapy may be administered in some cases
      • outcomes
        • 50-60% 5-year survival
    • amputation
      • indications
        • when otherwise unable to resect tumor with negative margins
        • resection would result in irreversible damage to major nerves
        • patient comorbidities limit potential for recovery after limb-sparing surgery 
      • outcomes
        • good prognosis in absence of metastatic disease
Techniques
  • Biopsy 
    • approach
      • longitudinal incision 
        • transverse incisions are contraindicated due to a need for wider resection at time of surgery
    • technique
      • use anatomic route that limits contamination of compartments and neurovascular structures
      • maintain meticulous hemostasis
      • use knife or curette to remove tissue
      • drains should be placed distal and in line with the surgical incision to allow removal of drain site with extensile incision
  • Wide resection & radiation therapy  
    • approach
      • elliptical incision used to incorporate biopsy and drain sites
    • technique
      • resection
        • use of tourniquet without exsanguination
        • maintain meticulous hemostasis
        • meticulous dissection to ensure wide margins, must confirm border free of disease with histology
          • frozen section can be used to ensure margins are free of tumor
      • radiation
        • 50 Gy pre-operative and 66 Gy postoperative is the standard dose for soft-tissue sarcomas 
  • Amputation 
    • approach
      • level and incision guided by advanced imaging to ensure negative margins
    • technique
      • use of tourniquet without exsanguination
      • maintain meticulous hemostasis
      • meticulous dissection to ensure wide margins, must confirm border free of disease with histology
        • frozen section can be used to ensure margins are free of tumor
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)

Case A

tibia
 
 
 
(1) - histology does not always correspond to case 


 

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(OBQ13.4) A 64-year-old man presents with forearm swelling. A gadolinium-enhanced MRI of his forearm is shown in Figure A and a CT scan of his chest is shown in Figure B. The lesion is resected and its gross and cellular histological appearance is shown in Figure C and D respectively. Fluorescence-in-situ-hybridization is negative for t(X:18) and t(12:16). Immunohistochemistry is negative for desmin, myogenin and CD34. What is the most likely pathologic diagnosis? Tested Concept

QID: 4639
FIGURES:
1

Alveolar rhabdomyosarcoma

11%

(325/3070)

2

Myxoid liposarcoma

9%

(265/3070)

3

Dermatofibrosarcoma protuberans

9%

(286/3070)

4

Synovial sarcoma

7%

(221/3070)

5

High-grade pleomorphic undifferentiated sarcoma

64%

(1951/3070)

L 4 B

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