Coert, J. Henk

Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive sarcomas, with 40% associated with neurofibromatosis type 1 (NF1). Surgical excision is the main treatment for localized disease; however, local recurrence (LR) remains high. Radiotherapy (RTx) is increasingly used to enhance local control in STS, but its use remains controversial due to the potential for increased major wound complications and an increased risk of secondary malignancies in NF1 patients. This study investigated the use and impact of RTx on local control in MPNSTs, particularly in the NF1 setting. Methods Surgically treated primary MPNSTs from 1988 to 2019 in the MONACO multicenter cohort were included. Differences in demographics, treatment, and RTx use between NF1 and non-NF1 cases were examined. Factors influencing RTx administration and LR were assessed via multivariable analyses. Results Among 499 patients (33.1% NF1), 143 (28.7%) experienced LRs. Radiotherapy was administered to 56.3% of patients (57.0% in the NF1 group), with 27.3% receiving neoadjuvant and 72.7% adjuvant RTx. RTx was administered significantly more often to high-grade and extremity tumors. While RTx did not affect overall survival, it reduced LR risk in sporadic cases (hazard ratio [HR] 0.530; 95% confidence interval [CI], 0.354–0.793) but not in the NF1 subgroup (HR 1.00; 95% CI, 0.545–1.85). In NF1 patients, a microscopically positive margin (R1) was the only risk factor for LR development (HR 2.1; 95% CI, 1.19–3.79). Conclusions RTx is frequently used in the treatment of MPNSTs, regardless of NF1 status. While it may affect the LR rate in sporadic cases, its impact on NF1 patients is less clear.

Abstract Background This study aimed to assess the performance of currently available risk calculators in a cohort of patients with malignant peripheral nerve sheath tumors (MPNST) and to create an MPNST-specific prognostic model including type-specific predictors for overall survival (OS). Methods This is a retrospective multicenter cohort study of patients with MPNST from 11 secondary or tertiary centers in The Netherlands, Italy and the United States of America. All patients diagnosed with primary MPNST who underwent macroscopically complete surgical resection from 2000 to 2019 were included in this study. A multivariable Cox proportional hazard model for OS was estimated with prespecified predictors (age, grade, size, NF-1 status, triton status, depth, tumor location, and surgical margin). Model performance was assessed for the Sarculator and PERSARC calculators by examining discrimination (C-index) and calibration (calibration plots and observed-expected statistic; O/E-statistic). Internal–external cross-validation by different regions was performed to evaluate the generalizability of the model. Results A total of 507 patients with primary MPNSTs were included from 11 centers in 7 regions. During follow-up (median 8.7 years), 211 patients died. The C-index was 0.60 (95% CI 0.53–0.67) for both Sarculator and PERSARC. The MPNST-specific model had a pooled C-index of 0.69 (95%CI 0.65–0.73) at validation, with adequate discrimination and calibration across regions. Conclusions The MPNST-specific MONACO model can be used to predict 3-, 5-, and 10-year OS in patients with primary MPNST who underwent macroscopically complete surgical resection. Further validation may refine the model to inform patients and physicians on prognosis and support them in shared decision-making.

This retrospective analysis reports the mid-term (>2 years) outcomes of capsulodesis, (modified) Brunelli tenodesis and bone-ligament-bone reconstruction for scapholunate ligament instability. In total, 60 patients (64 wrists) returned the Patient-Rated Wrist Evaluation and Disabilities of the Arm, Shoulder, and Hand questionnaires. Of these participants, 42 (46 wrists) returned for a wrist examination and radiographs. In the questionnaire group, the mean follow-up for capsulodesis was 11.8 years, tenodesis 5.9 years and bone-ligament-bone 8.9 years. In the objective outcomes group, the mean follow-up was 10.2 years for capsulodesis, 5.7 for tenodesis and 8.9 for bone-ligament-bone. In all groups, some patients showed radiographic deterioration, which did not always correlate with poor functional outcome. With a mean follow-up greater than 5 years, there was no substantial difference in clinical outcomes and patient-based outcome questionnaires between capsulodesis, tenodesis or bone-ligament-bone reconstruction for the treatment of scapholunate instability. This study emphasizes the need for further research. Surgeons should use the technique that produces the fewest complications. Level of evidence III

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas. Their resection may lead to serious morbidity. Incidence of postoperative motor and sensory deficits is unknown, and reconstruction aimed at restoring such deficits is infrequently carried out. OBJECTIVE: To identify the incidence and risk factors of postoperative morbidity in MPNST as well as the use and outcomes of functional reconstructions in these patients. METHODS: Postoperative function and treatment of MPNSTs diagnosed from 1988 to 2019 in 10 cancer centers was obtained. Two models were constructed evaluating factors independently associated with postoperative motor (<M3) or critical sensory loss. Critical sensation was defined as partial or complete loss of hand, foot, or buttocks sensation. RESULTS: A total of 756 patients (33.4% neurofibromatosis type 1, NF1) were included. MPNSTs originated in 34.4% from a major nerve. Of 658 surgically treated patients, 27.2% had <M3 muscle power and 24.3% critical sensory loss. Amputations were carried out in 61 patients. Independent risk factors for motor and sensory loss included patients with NF1, symptomatic, deep-seated, extremity, or plexus tumors originating from major nerves (all P < .05). A total of 26 patients underwent functional reconstructions. The majority (64%) of these patients regained at least M3 muscle power and 33% M4 despite 86% receiving multimodal therapy. CONCLUSION: Resection of MPNSTs commonly results in motor and sensory deficits. Patients with NF1, symptomatic, deep-seated tumors, arising from major nerves were associated with a higher risk for developing postoperative morbidity. Functional reconstructions are infrequently performed but can improve functional outcomes.

Ten normal ring fingers from ten donors were used to determine the effect of flexor tendon repair on the gliding resistance between the tendon and the A2 pulley. Gliding resistance was measured for the intact FDP tendon and for the same tendon after it was cut transversely and repaired with a 4/0 Ticron core suture and a 6/0 running epitendinous nylon suture. After repair, the gliding pattern of the tendon through the A2 pulley changed significantly. The resistance and the friction coefficient were approximately doubled ( P<0.005).