Updated: 6/22/2021

Undifferentiated Pleomorphic Sarcoma

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  • summary
    • Undifferentiated Pleomorphic Sarcoma, previously known as malignant fibrous histiocytoma, is a high-grade, aggressive, malignant fibrogenic tumor.
    • The condition is typically seen in patients 55-80 years of age who present with a slow-growing, painless mass.
    • Diagnosis is made with a biopsy showing spindled, histiocytic, and multinucleated eosinophilic giant cells arranged in a storiform pattern arranged around small vessels.
    • Treatment is usually wide-margin surgical excision with radiotherapy.
  • Epidemiology
    • Incidence
      • 1-9/100,000
    • Demographics
      • most common soft-tissue sarcoma in adults 55-80 years
      • caucasian > African American
      • male > female
    • Anatomic location
      • extremities (lower > upper)
      • retroperitoneum
  • Etiology
    • Genetics
      • mutations
        • programmed cell death protein 1 (PD-1) expression in 30%
  • 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
            • Neoadjuvant chemotherapy has shown improved 5-year recurrence-free survival and overall survival in tumors >10 cm 
        • 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
  • Prognosis
    • Survival with treatment
      • 50-60% 5-year survival
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(OBQ18.135) A 63-year-old man presents with an enlarging mass in his left thigh. Radiographs and an MRI are shown in Figures A, B, and C. A biopsy is performed and returns with the histology shown in Figure D. Which of the following is the best next step in treatment?

QID: 213031
FIGURES:

Observation

0%

(8/1950)

Marginal resection

4%

(78/1950)

Marginal resection and radiation

5%

(93/1950)

Wide resection and chemotherapy

20%

(388/1950)

Wide resection and radiation

70%

(1363/1950)

L 2 A

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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?

QID: 4639
FIGURES:

Alveolar rhabdomyosarcoma

11%

(396/3601)

Myxoid liposarcoma

9%

(326/3601)

Dermatofibrosarcoma protuberans

9%

(338/3601)

Synovial sarcoma

7%

(252/3601)

High-grade pleomorphic undifferentiated sarcoma

63%

(2263/3601)

L 4 B

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