Enrico Martin

284 Chapter 12 find clinical subgroups of patients each of which benefiting from different oncological treatment approaches; i.e. more/less aggressive resections, use of radiotherapy, and use of chemotherapy. Retroperitoneal MPNST for instance, had a drastically worse survival compared to non-retroperitoneal MPNST in Chapter 3 , and are known to have the highest risk of recurrence and metastasis. 39,40 Safety of less extensive and function- preserving surgery in patients can subsequently be determined. Also, as depicted in Chapter 10 , patients with a prognosis of at least three years can be calculated to whom functional reconstructions can be offered, even though Chapter 2-4 already show promisingly longer overall median survival for localized MPNST. The resulting choice for reconstruction will depend on numerous factors, but ideal techniques are best discussed in the multidisciplinary setting involving both an oncological and a reconstructive surgeon. Preferably, a surgeon with knowledge of nerve reconstruction possibilities should be involved as well. Contrarily, in selected cases a wider excision can be planned, possibly avoiding the need for radiotherapy, when all possibilities are known to reconstruct anticipated deficits. Overall, such collaboration may lead to a balanced treatment strategy. While clinical characteristics are able to predict survival moderately-well, tumor-specific biology may enhance predictive value. Several studies have suggested that immunohistochemical markers and specific genetic alterations may improve prognostication in MPNST. 2,41,42 Future studies should be encouraged to test the most valuable of these markers in a large cohort of patients and ideally observe if it enhances prediction of the MONACO calculator. The somewhat somber outcomes of MPNST, both oncological and functional, raise the question if MPNST-related surgeries should be centralized even more than other STS. Other possibilities may include an MPNST-specific multidisciplinary team that is available for digital consultation to any STS-dedicated center.

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