Survival of malignant peripheral nerve sheath tumor of the trachea, mediastinum, and other respiratory organs: Analysis of SEER database.

Abstract

e23550 Background: Malignant peripheral nerve sheath tumor (MPNST) is a rare form of sarcoma arising from Schwann cells with an incidence rate of about 1 case per 100,000 people. Most MPNSTs occur in the extremities; MPNST of respiratory organs is exceedingly rare and has only been reported in case studies previously, with little being known about its incidence and treatment outcomes. Methods: Patients with MPNST of the “trachea, mediastinum and other respiratory organs” were identified from the Surveillance, Epidemiology, and End Results (SEER) database (2000-2020) using SEER*Stat. Patient demographics, tumor stage, treatment, and survival data were extracted. Survival outcomes were compared by stage, chemotherapy (any chemotherapy vs. none/unknown), radiation (beam radiation vs. no/unknown), and surgery (any surgery vs. none). Results: 43 patients (mean age: 44.7 ± 18.7) with MPNST of the trachea, mediastinum and other respiratory organs were extracted. Most patients (27; 63%) were male. Most patients were white (34; 79%), followed by black (5; 12%) and other races (4; 9.3%). Earlier tumor stages (p=0.04) and receiving surgery (p=0.02) were significantly associated with longer median survival (Table). Not receiving chemotherapy and receiving beam radiation also appeared to be associated with longer median survival, although these were not statistically significant. Conclusions: This study identified that tumor stage and receiving surgery are associated with improved survival outcomes among patients with MPNST of the trachea, mediastinum and other respiratory organs. The poor survival associated with distant metastasis and lack of surgical intervention highlight the need for novel systemic therapeutics and alternative local intervention options to surgery given the location of the disease which is often unresectable. Median survival of MPNST based on stage and treatment. Number of cases (%) Median survival (months) p-value Stage (n=34) LocalizedN=15 (44%) 30 p=0.04 RegionalN=10 (29%) 10.5 DistantN=9 (26%) 4 Chemotherapy (n=37) YesN=15 (41%) 10 p=0.2 None/unknownN=22 (59%) 20 Radiation (n=38) Beam radiationN=20 (53%) 13.5 p=0.3 None/unknownN=18 (47%) 12 Surgery (n=38) YesN=26 (68%) 20 p=0.02 NoneN=12 (32%) 4.5