The management and treatment of a complex soft tissue sarcoma.

1. Tony Weaver, DO
General Surgery
“IN THE FLESH”
SOFT TISSUE SARCOMA

2. CASE PRESENTATION:
HPI: 52 y.o. female presents w/ a >40 yr hx of a “keloid” on the right
upper posterolateral thigh. She first noticed it following an immunization
as around age 12. Her only symptoms were intermittent erythema and
she now states, “My pants won’t fit just right.”
ROS: All neg except HPI.
PMedHx: HTN, Morbid Obesity
PSurgHx: Appendectomy, Lap Chole
Fam/Soc Hx: No hx of skin cancer or genetic d/o
Sun Exposure Hx: 1-2 Peeling sunburns as a teen, avoids sun
exposure since 20s.
Meds: Atenolol Allergies: None

4. • Physical exam
Ht 177cm, Wt 111.1kg, T 36.4, HR 56, BP 132/81
Right superior lateral thigh- irregular multinodular 13cm x
10 cm mass, w/ areas of erythema, telangectasia,
induration & mild hyperpigmentation.
Firm, non-tender, mobile

5. DIFFERENTIAL DIAGNOSIS?

6. Imaging, Labs & Tests
● Punch biopsy- “spindle cell neoplasm consistent with
DFSP”
● CXR- calcified granuloma, otherwise negative
● MRI (preoperative assessment)- lobulated cutaneous
mass w/ no evidence of deep extension
● CBC, electrolytes, renal and liver test all WNL

8. TREATMENT
Radical resection of Right Hip Mass
● Wide excision with 3cm margins
● Post-op Defect size - 23cm x 17cm
● Preliminary frozen section analysis of margins- negative
● Wound vac with interval posterior lateral thigh rotational
Flap.

12. DERMATOFIBROSARCOMA
PROTUBERANS
Rare, slow growing, locally aggressive cutaneous tumor of
mesenchymal origin.
● Originates from dermal fibroblasts.
● Low grade to intermediate grade. Rarely metastasizes,
but high recurrence rate 21%
● Occurs primarily in adults aged 20-50s
○ 0.8-5 cases per million population per year
● Distribution- Trunk (47%), limbs (38%), head and neck
area (14%)

13. CLINICAL FEATURES
● Early DFSP- plaque-like areas
of cutaneous thickening that are
frequently skin-colored, red-
brown, or violaceous
● As the tumor slowly enlarges, it
becomes raised, firm, and
nodular; the surrounding skin
may be telangiectatic. The
nodule is often fixed to the
dermis but moves freely over
deeper tissues

14. GENETIC & HISTOLOGIC FACTORS
Possible correlation to soft tissue trauma
Cytogenetic abnormality
• Detected by RT-PCR and FISH
• Chromosomal Translocation t(17;22)
• Fusion gene is formed that binds COL1A1-PDGF-B
Histology
• Monomorphic, benign-appearing spindle cells
• Spindle cells arranged in an irregularly storiform, cart-wheel like
pattern
• Stains positively for CD34, hyalunorate, and vimentin; negatively for
factor XIIIa

17. WORK UP & TREATMENT:
1. Obtain Tissue Diagnosis:
Incisional Biopsy for Lesions >4 cm
Excisional Biopsy for Lesions <4 cm
2. Determine Extent/Rule out Metastatic Disease:
CXR or MRI
LUNGS MOST COMMON SITE OF METASTASIS
3. Operative Planning:
Consultation: Oncology or Plastics?

18. CURRENT TREATMENT
Surgical Resection:
○ Mohs Micrographic surgery-less recurrence rate.
○ Wide local excision- faster procedure, better for larger tumors.
○ Adjuvant radiotherapy if positive margins little benefit.
Molecularly targeted chemotherapy:
○ Used for positive margins or advanced disease.
○ Imatinib (Gleevec)- Tyrosine Kinase inhibitor
■ PDFG-B receptor (tyrosine kinase dependent)

19. REFERENCES
Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM,
Brennan MF, Lewis JJ. Dermatofibrosarcoma protuberans: A
clinicopathologic analysis of patients treated and followed at a single
institution. Cancer. 2000 Jun
Gloster HM Jr, Harris KR, Roenigk RK. “A comparison between Mohs
micrographic surgery and wide surgical excision for the treatment of
dermatofibrosarcoma protuberans.” J Am Acad Dermatol 1996; 35:82.
Lemm D, Mügge LO, Mentzel T, Höffken K. “Current treatment options in
dermatofibrosarcoma protuberans”. J Cancer Res Clin Oncol 2009; 135:653.