The Association of Metastasis Pattern and Management of Metastatic Disease with Oncological Outcomes in Patients with Malignant Peripheral Nerve Sheath Tumors: A Multicenter Cohort Study

Abstract

Purpose: This multicenter cohort study aimed to identify clinicopathologic and treatment-related factors associated with the development of distant metastasis (DM) and with overall survival (OS) after DM diagnosis in patients with malignant peripheral nerve sheath tumors (MPNST). Methods: All patients diagnosed with primary MPNST from 1988 to 2019 who were surgically treated for the primary tumor were included. Multivariable Cox regression analyses were performed to identify factors associated with DM and OS after DM diagnosis. Results: A total of 383 patients were included in this analysis, of which 150 developed metastatic disease. No differences in clinicopathologic characteristics and clinical outcome were found between patients with synchronous and metachronous DM. Neurofibromatosis type 1 (NF1), high grade, tumor size, triton and R2 resections were independent risk factors for the development of DM. NF1 and more than two metastasis sites were independently associated with worse OS after DM diagnosis. Metastasectomy, chemotherapy and the metastatic site category ‘other’ were associated with prolonged survival after DM diagnosis. Conclusion: This analysis provides important insights into clinicopathologic and treatment factors associated with outcomes in metastatic MPNST. Moreover, NF1-status is associated with a higher risk of DM; it is also independently associated with worse survival in metastatic MPNST.

Prediction tools for the personalized management of soft-tissue sarcomas of the extremity

“…For example, one may argue that the accuracy of the predictions of a generic eSTS model in a patient with a malignant peripheral nerve sheath tumour (MPNST) of the extremity, would be less than one based on a MPNST-specific prediction tool in which important MPNST-specific predictors, such as the presence of neurofibromatosis type 1 and rhabdomyoblastic differentiation (triton tumour), are incorporated. 63 A recent study showed that the discriminative ability of the Sarculator is less in MPNSTs compared with other histological subtypes, such as leiomyosarcomas (c-index: 0.66 vs 0.75, respectively). 52 This could be a reason to update the Sarculator in patients with MPNST with additional important MPNST-specific predictors.…”

Ovarian epithelioid malignant peripheral nerve sheath tumor with EWSR1‐CREM fusion: A case report and literature review

“…Although the carcinogenesis mechanisms underlining MPNST remain uncertain, more than half are sporadic diseases. Neurofibromatosis‐1 (NF‐1) is associated with 11.7% to 51% of all cases in previous studies, while the 10% are induced by radiotherapy 5–7,16 . MPNST usually affects patients in their fourth to fifth decades of life without sexuality dominance; the extremities, trunk, and head and neck areas are the most commonly involved sites, in descending prevalence 5,6,9 .…”

“…MPNST is a highly aggressive tumor with poor prognosis, nearly half of the patients will succumb to the disease 5 years after diagnosis and approximately two‐thirds experience disease progression or recurrence 6,9 . Moreover, it has been reported that approximately 40% of patients with MPNST developed distant metastases 16 . Tumor grade, tumor size, and Intergroup Rhabdomyosarcoma Study (IRS) groups are major prognostic predictors that have been identified in published research 5,6,8 .…”

“…However, most relevant studies showed no significant associations between better OS and disease‐free survival 5,6,9 . Yet 9.4% to 16% of patients with MPNST had distant metastatic disease at the time of diagnosis and most of them could not achieve satisfactory resection; chemotherapy or radiotherapy remain vital palliative therapy 5,8,9,16 . Published research has evaluated the therapeutic response rate in different chemotherapy regimens, while the combination of doxorubicin and ifosfamide had the best response rate and should be the preferred option in patients with MPNST 1,7,8 .…”

“…Neurofibromatosis-1 (NF-1) is associated with 11.7% to 51% of all cases in previous studies, while the 10% are induced by radiotherapy. [5][6][7]16 MPNST usually affects patients in their fourth to fifth decades of life without sexuality dominance; the extremities, trunk, and head and neck areas are the most commonly involved sites, in descending prevalence. 5,6,9 However, tumors that arise in the abdomen or viscera are particularly rare and only account for 1% of F I G U R E 1 Findings from positron emission tomography/computed tomography demonstrating multiple metastatic lesions involving the omentum, mesentery, peri-renal capsule, and spleen region in the abdominopelvic cavity.…”