Carbon Ion irradiation in the treatment of grossly incomplete or unresectable malignant peripheral nerve sheaths tumors:
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RESEARCH
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Carbon Ion irradiation in the treatment of grossly incomplete or unresectable malignant peripheral nerve sheaths tumors: acute toxicity and preliminary outcome Alexandra D Jensen1*, Matthias Uhl1, Naved Chaudhri2, Klaus K Herfarth1, Juergen Debus1,4 and Falk Roeder1,3
Abstract Background: To report our early experience with carbon ion irradiation in the treatment of gross residual or unresectable malignant peripheral nerve sheath tumors (MPNST). Methods: We retrospectively analysed 11 patients (pts) with MPNST, who have been treated with carbon ion irradiation (C12) at our institution between 2010 and 2013. All pts had measurable gross disease at the initiation of radiation treatment. Median age was 47 years (29-79). Tumors were mainly located in the pelvic/sacral (5 pts) and sinunasal/orbital region (5 pts). 5 pts presented already in recurrent situation, 3 pts had been previously irradiated, and in 3 pts MPNST were neurofibromatosis type 1 (NF1) associated. Median cumulative dose was 60 GyE. Treatment was carried out either as a combination of IMRT plus C12 boost (4 pts) or C12 only (7 pts). Results: Median follow-up was 17 months (3-31 months). We observed 3 local progressions, translating into estimated 1- and 2-year local control rates of 65%. One patient developed distant failure, resulting in estimated 1- and 2-year PFS rates of 56%. Two patients have died, therefore the estimated 1- and 2-year OS rates are 75%. Acute radiation related toxicities were generally mild, no grade 3 side effects were observed. Severe late toxicity (grade 3) was scored in 2 patients (trismus, wound healing delays). Conclusion: Carbon ion irradiation yields very promising short term local control and overall survival rates with low morbidity in patients suffering from gross residual or unresectable malignant peripheral nerve sheath tumors and should be further investigated in a prospective trial.
Background Malignant peripheral nerve sheath tumors (MPNSTs) comprise a rare group of tumors that arise from different cells found in the sheath of peripheral nerves including Schwann cells, perineural fibroblasts or fibroblasts, formerly known as malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma or malignant neurilemoma [1]. The reported incidence is 1:1000000 per year [2], representing 5-10% of all malignant soft tissue tumors [3]. The most important risk factor is neurofibromatosis type I (NF I), which accounts for about half of the cases [1]. The incidence seems equal in males and females and * Correspondence: [email protected] 1 Department of Radiation Oncology, University of Heidelberg, INF 400, 69120 Heidelberg, Germany Full list of author information is available at the end of the article
the most common age at diagnosis is 20 to 50 years [2]. Predominant sites of presentation are the proximal extremities and the pelvis [4]. They usually present as enlarging mass, which may be associated with pain or neurological symptoms [2]. However, MPNSTs are highly aggressive tumors characterized by rapid,
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