Malignant Peripheral Nerve Sheath Tumor of the Cervical Spine Treated with Surgical Resection Followed by X-ray Radiotherapy or Carbon Ion Radiotherapy: A Report of Three Cases

Honda, Akira; Iizuka, Yoichi; Okamoto, Masahiko; Shiba, Shintaro; Koshi, Hiromi; Mieda, Tokue; Ishiwata, Sho; Kakuta, Yohei; Tajika, Tsuyoshi; Ohno, Tatsuya; Chikuda, Hirotaka

doi:10.22603/ssrr.2019-0100

Cited by 3 publications

(6 citation statements)

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“…MPNSTs are highly aggressive and locally invasive rare malignancies with an incidence of 0.0001% in the general population and 3%–5% in patients with neurofibromatosis type 1 (NF1) ( 2 ). Lesions are most frequently found on the trunk, extremities, and head and neck.…”

Section: Discussionmentioning

confidence: 99%

“…The best adjuvant treatment remains poorly defined due to the lack of prospective trials. Previous literature suggests that adjuvant radiotherapy after surgery could be an effective treatment for patients, especially in lesions larger than 5 cm in size or with residual tumor, which is critical in the prognosis of primary spinal intradural MPNSTs ( 2 , 7 , 8 , 31 ). However, our study revealed that radiotherapy is ineffective in controlling recurrence and does not appear to affect overall survival, which may be due to the bias caused by the fact that more aggressive tumors are more likely to undergo radiotherapy.…”

Section: Discussionmentioning

confidence: 99%

“…MPNST (1 case in ten million) is an unusual disease and represents 2% to 4% of all soft tissue sarcomas and 23% to 51% of these tumors were associated with neurofibromatosis type 1 (NF1) ( 1 ). Spinal MPNSTs accounted for 2%–3% of all MPNSTs ( 2 ). Primary sporadic intradural MPNST in the spinal canal is even more exceptional, and it is easy to be misdiagnosed as central nervous system tumors or other types of soft tissue sarcomas.…”

Section: Introductionmentioning

confidence: 99%

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Epidemiology, Characteristic, and Prognostic Factors of Primary Sporadic Intradural Malignant Peripheral Nerve Sheath Tumor in the Spinal Canal: A Systematic Literature Review

et al. 2022

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PurposePrimary sporadic intradural malignant peripheral nerve sheath tumor (MPNST) in the spinal canal is a type of rare neoplasm with challenging diagnosis and therapy. The overall prognosis of this tumor is markedly different from that of the usual spinal intradural tumors. The purpose of this systematic review is to reduce the misdiagnosis and enhance the prognosis of the disease by reviewing the literature.MethodsPubMed, Medline, and Embase databases were searched for articles in English language published from 1980 to May 2021, yielding 500 potentially relevant articles. The keywords were as follows: “spinal”, “malignant peripheral nerve sheath tumor”, “neurosarcoma”, “malignant schwannoma”, and “malignant neurofibroma”. Thirteen papers met the eligibility criteria, including 55 cases with spinal intradural primary sporadic MPNSTs, which were confirmed by post-operation pathology. We further analyzed the clinical manifestations, radiological manifestations, pathological features, comprehensive treatment strategies, and prognosis.ResultsFifty-five spinal intradural primary sporadic MPNSTs from 30 (54.5%) male and 25 (45.5%) female patients with an average age at diagnosis of 40 years (range, 3–70 years) were included in the study. The most common clinical manifestations were local or radicular pain and motor disturbance. All tumors had significant enhancement and heterogeneous enhancement was more common. Out of 18 lesions, 14 were diagnosed as high grade and the remaining 4 were diagnosed as low grade. The ki-67 labeling index ranged from 5% to 60%. The median recurrence and survival time were 36 and 72 months, respectively. The log-rank tests indicated that significant predictors of OS were patient age (≤30 vs. >30 years) at the time of diagnosis and the presence of metastatic disease, and similar analyses for RFS demonstrated that the presence of metastatic disease was the only significant predictor (60 vs. 10 months). The multivariate Cox proportional hazards regression analysis revealed that absence of metastasis was an independent factor for predicting a favorable prognosis.ConclusionsSpinal intradural primary sporadic MPNSTs are challenging malignant tumors without a systematic treatment plan. The factors affecting its prognosis are not clear. Even after surgical treatment and adjuvant treatment, the recurrence rate and mortality rate are still high. Clinicians should be alert to the possibility of this disease and achieve early detection and treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Epidemiology, Characteristic, and Prognostic Factors of Primary Sporadic Intradural Malignant Peripheral Nerve Sheath Tumor in the Spinal Canal: A Systematic Literature Review

et al. 2022

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“…To the best of our knowledge, this is the first report of the oncological outcome and toxicity rates of mPNST/PNST patients treated with protons using a PBS delivery paradigm. Of note, two retrospective studies and two case reports analyzing mPNSTs treated with carbon ion (C12) particles were published ( 18 – 20 ). Jensen et al., 2015 ( 18 ) reported on the outcome and toxicity of 11 patients with unresected or incompletely resected mPNSTs treated with C12 irradiation.…”

Section: Discussionmentioning

confidence: 99%

Clinical Outcome After Pencil Beam Scanning Proton Therapy of Patients With Non-Metastatic Malignant and Benign Peripheral Nerve Sheath Tumors

et al. 2022

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ObjectivePeripheral nerve sheath tumors (PNSTs) commonly arise from peripheral nerve roots and grow locally invasive. Malignant PNSTs (mPNSTs) represent aggressive sarcomas of neural origin that can originate from PNSTs. Radiation therapy is commonly used as part of the required multimodal treatment. However, both entities tend to occur early in life and are associated with the genetic disorder neurofibromatosis type 1 (NF-1), which is known to cause increased radiosensitivity. Pencil beam scanning proton therapy (PBSPT) allows for a minimization of the dose delivered to organs at risk and the integral dose and, thus, potentially also a reduction of radiation-induced adverse events. We report the clinical outcome and toxicity rates of patients with (m)PNSTs treated with PBSPT.MethodsWe retrospectively reviewed 36 patients who received PBSPT (median dose, 64 GyRBE) with curative intent for (m)PNSTs between 1999 and 2020 at our institute. Twenty-eight (78%) and 8 (22%) patients were treated at diagnosis and for tumor recurrence/progression, respectively. The median age was 32 years (range, 3–75), and 25 (69%) patients were male. mPNST and PNST were diagnosed in 31 (86%) and 5 (14%) patients, respectively. Underlying NF-1 disease was found in 8 (22%) patients. Acute and late toxicities were recorded according to Common Terminology Criteria for Adverse Events, version 4.1 (CTCAE v4.1). Overall survival (OS), local control (LC), and distant control (DC) were estimated using the Kaplan–Meier method.ResultsWith a median follow-up time of 31 months (range, 4–194), 13 (36%) patients died from a progressive disease, 8 (22%) experienced local failure, and 14 (39%) experienced distant failure after PBSPT. Estimated 2-year OS, LC, and DC were 75.5%, 73.5%, and 61.2%, respectively. Acute grade 3 toxicity (dermatitis, mucositis, and pain) was observed in 5 (14%) patients. Late grade 3 cataract and osteonecrosis were both observed in 1 (3%) patient at 34 and 194 months after PBSPT, respectively. There was no late grade >3 toxicity or radiation-induced secondary cancer.ConclusionTo our knowledge, this is the first study to analyze the outcome of (m)PNSTs treated with proton therapy using a PBS delivery paradigm. In our cohort, consisting mainly of patients with mPNSTs, we report reasonable oncological outcomes and low toxicity rates after PBSPT.

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“…The main goal of surgical treatment is to remove the tumor, relieve nerve compression and rebuild the stability of the spine. Spinal tumor surgery usually involved long operation time, large area of soft tissue resection and long wound, and was prone to hypothermia during the operation [ 1 – 6 ].…”

Section: Introductionmentioning

confidence: 99%

Implementation of multi-mode nursing insulation program for patients receiving surgery for spine tumor: a propensity score-matched analysis

Liu

Gao

et al. 2022

BMC Surg

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Background Spinal tumor surgery usually involved long operation time, large area of soft tissue resection and long wound, and was prone to hypothermia during the operation. Therefore, actively promoting insulation and optimizing the intraoperative insulation program have great potential in reducing the incidence of hypothermia and reducing the incidence of postoperative complications. In this study, we compared patients who did not implement multi-mode nursing insulation program (MNIP) with those who implemented MNIP, observing and comparing clinical outcomes, and complications in both groups, with the aim of developing an optimal management plan for the preoperative, intraoperative, and postoperative periods, respectively. Methods We selected 2 periods of 1 year, before (n = 120 patients) and after MINP implementation (n = 120 patients). Data were collected on patient demographics, operative, perioperative details, temperature changes, anesthesia recovery effect, incidence of postoperative wound infection, length of hospital stay and complications. PS analyses were used for dealing with confounding bias in this retrospective observational study. Results After PS matching, the outcomes of 120 well-balanced pairs of patients were compared (No-MNIP vs MNIP). There was no significant difference concerning the satisfaction survey. The results indicated that the MNIP had better insulation effect at 90 min, 120 min, 150 min after anesthesia induction and after surgery. There were 16 cases of complications in the No-MNIP group and 5 cases in the MNIP group postoperative, which have significant statistical difference. Conclusion In this study, the incidence of intraoperative hypothermia was effectively reduced by adopting the multi-mode insulation scheme, thus reducing the incidence of incision infection and shortening the length of hospital stay of patients.

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Malignant Peripheral Nerve Sheath Tumor of the Cervical Spine Treated with Surgical Resection Followed by X-ray Radiotherapy or Carbon Ion Radiotherapy: A Report of Three Cases

Abstract: This case report was approved by the institutional review boards at Gunma University and the institutional review board overseeing the central data management center.

Cited by 3 publications

References 14 publications

Epidemiology, Characteristic, and Prognostic Factors of Primary Sporadic Intradural Malignant Peripheral Nerve Sheath Tumor in the Spinal Canal: A Systematic Literature Review

Epidemiology, Characteristic, and Prognostic Factors of Primary Sporadic Intradural Malignant Peripheral Nerve Sheath Tumor in the Spinal Canal: A Systematic Literature Review

Clinical Outcome After Pencil Beam Scanning Proton Therapy of Patients With Non-Metastatic Malignant and Benign Peripheral Nerve Sheath Tumors

Implementation of multi-mode nursing insulation program for patients receiving surgery for spine tumor: a propensity score-matched analysis

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