Introduction: Malignant peripheral nerve sheath tumor (MPNST) is rare soft tissue sarcoma that arises from a peripheral nerve or shows nerve sheath differentiation.
Case Report: We present a case of a 43-year-old female diagnosed with neurofibromatosis type 1 and with a plexiform neurofibroma of the infratemporal fossa (IF) evolving for about four years. Examination revealed facial asymmetry with the prominence of the right hemiface, without facial pain, motor deficits or facial numbness. The magnetic resonance imaging (MRI) showed a lesion in right IF with well-defined limits and 18F-FDG positron emission tomography/computed tomography (PET/CT) showed intense metabolism that was attributed to malignant transformation. Cytology confirmed a MPNST. Excision of the IF lesion by maxillary swing approach was performed. The anatomopathological evaluation of the lesion revealed a 7×6×3 cm high grade MPNST arising within a neurofibroma. The excision was juxtalesional. The tumor was staged at pT3cN0M0, IIIB. The patient underwent adjuvant radiotherapy. Currently, after 12 months of follow-up, the patient is asymptomatic, without neurologic deficits. The MRI performed three months after the end of radiotherapy shows no evidence of tumor recurrence.
Conclusion: The only known definitive curative treatment for MPNST is complete surgical resection with wide negative margins. The maxillary swing approach delivers wide tumor exposure, providing controlled dissection with minimizing morbidity. Despite maximal efforts, MPNSTs are usually highly aggressive. Increased tumor size and high-grade morphology, features which were observed in our patient, have been identified as factors that negatively affect long-term survival. We encourage a multidisciplinary approach in the management of these rare tumors to optimize outcomes.
Introduction. Although uncommon from a population-based perspective, there is considerable morbidity and mortality associated with malignant tumors of the nose and paranasal sinuses.The objective of this study was to characterize the presentation, risk factors, management and survival of patients with these tumors treated at a single institution.Materials and methods. We retrospectively reviewed the clinical records of patients with malignancies of the nose and paranasal sinuses diagnosed between January 2010 and December 2014 at a tertiary cancer center. Univariate and multivariate analysis were performed.Results. Ninety patients were included in the study. Mean age at diagnosis was 62.8 years (range, 2–95 years) and mean follow-up was 44.5 months (range, 2–113 months). The maxillary sinus (33.3 %) and the nasal cavity (32.2 %) were the most frequent sites of origin. Squamous cell carcinoma (36.7 %), mucosal melanoma (15.6 %) and adenoid cystic carcinoma (10 %) were the most common histologic subtypes. Surgery was the primary treatment for 86.7 % of patients. Recurrence occurred in 45 patients (50 %). The overall 5‑year survival was 39.3 % and disease free-survival was 45.9 %. Survival was significantly decreased in non-smokers (p = 0.022), T3–4 tumors (p = 0.007), positive lymph nodes (p <0.001), nonepithelial tumors (p = 0.036) and positive margins (p = 0.032). Survival was not affected by surgical approach between endoscopic, open and combined approaches (p = 0.088).Conclusion. Prognosis is poor, with high recurrences and low survival, but clearly histology, location and stage-dependent. Sound oncologic principles, with complete resections and negative margins, result in a better outcome.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.