Routine MRI prior to surgery is not warranted in most cases of DFSP. In pediatric DFSP cases where delineation of tumor from critical anatomic structures is ambiguous, MRI has aided in appropriate determining extent of tumor, surgical planning, and post-therapy follow-up [114, 115]. MRI has also been utilized in intraoperative resection of DFSP. Gould et al. used MRI to confirm clearance of DFSP after excision in three cases of DFSP with subcutaneous extension. They concluded that MRIguided resection is a valuable tool for achieving surgical clearance of soft tissue sarcomas while reducing the amount of non-involved tissue removed and the need for subsequent reconstructive surgery [116]. No studies exist in the current literature regarding the accuracy and limitations of MRI in predicting tumor extension of DFSP or its utility in postoperative surveillance.

Summary: Prognosis

•Metastases are rare with lung being the most common site.

•Indicators of poor prognosis are fibrosarcomatous variant, high mitotic index, and increased cellularity, history of prior recurrence, age greater than 50, and anatomic location of head and neck.

•Patients with history of recurrent tumors or probable metastatic disease should undergo physical examination with elicitation of review of systems.

•A thorough metastatic workup, including imaging of chest, abdomen, and pelvis, may be performed if signs and symptoms of malignancy are present.

19.8Prognosis

Generally, the prognosis for DFSP is good. Metastases rarely occur with DFSP with an estimated rate of 1%. In the SEER database study, 57% cases of DFSP were classified as “localized” disease restricted to skin, while only 0.4% were reported as having distant metastases. The remaining 43% exhibited “regional” disease limited to localized lymph nodes [1]. Lung is the most common site of metastases [40]. Metastatic foci can be evaluated in suspicious cases via CT or MRI scans. In addition, tumor activity can also be detected on fluorodeoxyglucose (FDG)-positron emission tomography

with increased FDG uptake signifying hypermetabolic state of tumor formation [117].

Due to its infiltrative nature, DFSP has high potential for recurrence. Most recurrences occurring within a mean of 3 years [40, 74]. Several factors are associated with increased morbidity from recurrence. Most commonly, inadequate resection margins are implicated in high rates of local recurrence. Histologically, the fibrosarcomatous variant of DFSP, high mitotic index, and increased cellularity are predictors of poor clinical outcome. History of prior recurrence, age greater than 50, and location of head and neck are also unfavorable. Interestingly, tumor size or depth and gender do not confer an increased risk for local recurrence [74].

After complete excision of DFSP, patients should be closely monitored for signs of recurrence. The NCCN recommends cutaneous examination intervals of 6–12 months for inspection of the primary DFSP site and biopsy of any suspicious areas [118]. Patients with history of recurrent tumors or probable metastatic disease should undergo physical examination with elicitation of review of systems. A thorough metastatic workup, including imaging of chest, abdomen, and pelvis, may be performed if signs and symptoms of malignancy are present. It has been suggested that the histopathological diagnosis of DFSP should be confirmed by cytogenetics for PDGFB overexpression, and inoperable tumors expressing PDGFB should receive imatinib before surgical resection [119].

Summary: Conclusion

•DFSP is a rare and locally aggressive soft tissue sarcoma with varying clinical presentation and high rate of recurrence.

•The mainstay of DFSP therapy is margincontrolled surgical resection by either MMS or WLE.

•All DFSP patients need careful monitoring of disease recurrence and regular clinical follow-up.

19.9Conclusion

DFSP is a rare and locally aggressive soft tissue sarcoma with varying clinical presentation and high rate of recurrence. It most commonly presents in adults, and it is increasingly being reported in children. Early diagnosis and prompt pathological evaluation can aid in institution of appropriate treatment. The mainstay of DFSP

therapy is margin-controlled surgical resection by either MMS or WLE. Radiotherapy provides limited benefit, and its utility is controversial. Imatinib is approved for use in adults patients with unresectable, recurrent, and metastatic DFSP. It is unclear whether imatinib is beneficial as neoadjuvant therapy. All DFSP patients need careful monitoring of disease recurrence and regular clinical follow-up to promote tumor-free survival.

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