Rationale and design of the peripheral nerve tumor registry: an observational cohort study

Dengler, Nora; Scholz, Christoph; Beck, Jürgen; Uerschels, Anne-Kathrin; Sure, Ullrich; Scheller, Christian; Strauß, Christian; Martín, Daniel; Schackert, Gabriele; Heinen, Christian; Woitzik, Johannes; McLean, Anna Lawson; Rosahl, Steffen K.; Kolbenschlag, Jonas; Heinzel, Johannes; Schuhmann, Martin U.; Tatagiba, Marco Soares; Guerra, Waltraud Kleist-Welch; Schroeder, Henry W. S.; Vetrano, Ignazio G.; Ahmadi, Rezvan; Unterberg, Andreas; Reinsch, Jennifer; Zdunczyk, Anna; Unteroberdoerster, Meike; Vajkoczy, Peter; Wehner, Sarah; Becker, Michael; Matthies, Cordula; Pérez-Tejón, Jose; Dubuisson, Annie; Barrone, Damiano G.; Trivedi, Rikin; Capone, Crescenzo; Ferraresi, Stefano; Kraschl, Jakob; Kretschmer, Thomas; Dombert, Thomas; Staub, Frank; Ronellenfitsch, Michael; Marquardt, Gerhard; Prinz, Vincent; Czabanka, Marcus; Carolus, Anne; Braun, Veit; König, Ralph; Antoniadis, Gregor; Wirtz, Christian Rainer; Rasulic, Lukas; Pedro, Maria Teresa

doi:10.1080/01616412.2022.2129762

Cited by 3 publications

(3 citation statements)

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“…Patient characteristics (age, sex) as well as disease (affected nerve, tumor location, histopathology), surgical treatment (type of treatment, pre- and postoperative symptoms), radiological imaging, diagnosis of neurofibromatosis (NF), data on health-related quality of life (HRQoL), return to work, and long-term follow-up data were analyzed. The long-term goal is to create uniform treatment recommendations ( 10 ).…”

Section: Methodsmentioning

confidence: 99%

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Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry

Grübel,

Antoniadis,

et al. 2024

Front. Oncol.

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ObjectivePeripheral nerve tumors (PNTs) are rare diseases. So far, no multicenter data on diagnostics, the efficacy of treatment, long-term outcomes, and health-related quality of life (HRQoL) exist. The establishment of the Peripheral Nerve Tumor Registry (PNTR) in 2015 allows for the systematic analysis of patients with tumors associated with peripheral nerves. The present study aims to investigate the impact of PNT on an individual’s HRQoL and the effect of surgery.MethodsHRQoL was pre- and postoperatively assessed by the Euro-Qol-5D-5L (EQ-5D-5L) and Euro-Qol visual analog scale (EQ-VAS) survey in the retrospective and prospective study arm in three active participating study centers. An index was calculated based on the EQ-5D-5L for the quantification of health state (0: worst possible state of health, 1: best possible state of health). The EQ-VAS ranges from 0% (worst imaginable health status) to 100% (best possible health status). Patient characteristics (age, sex), as well as disease (histopathological entity) and treatment (pre- and postoperative symptoms, type of treatment)-specific data, were analyzed.ResultsData from 171 patients from three high-volume centers were included, with schwannoma (70.8%, n = 121) and neurofibroma (15.8%, n = 27) being the most prevalent histopathological diagnoses. Both the median health index value (preoperative: 0.887, n = 167; postoperative: 0.910, n = 166) and the median EQ-VAS (preoperative: 75%, n = 167; postoperative: 85%, n = 166) of the entire cohort regarding all histopathological diagnosis improved significantly after surgical therapy (p < 0.001). Preoperatively, 12.3% (n = 21) reached the highest index score of 1.0 in EQ-5D-5L and 100% in the EQ-VAS score in 5.3% (n = 9) of all patients. Postoperatively, the highest index score of 1.0 and 100% in the EQ-VAS score increased significantly and were achieved in 33.3% (n = 57) and 11.1% (n = 19) of the patients, respectively (p < 0.001).ConclusionFor the first time, our study presents multicenter data on life quality and the effect of surgery in primarily benign peripheral nerve tumors. Early surgery at a specialized center could improve neurological outcomes and, in conclusion, better QoL. In summary, surgical therapy significantly improved the entire cohort’s QoL, VAS, and analgesia.

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Section: Methodsmentioning

confidence: 99%

“…This study was approved by our local ethics committees in Ulm (Nr. 249/17) and Berlin (EA4/058/17) and is registered with the German Trials Registry ( www.drks.de ) ( 10 ).…”

Section: Methodsmentioning

confidence: 99%

Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry

Grübel,

Antoniadis,

et al. 2024

Front. Oncol.

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“…B. Perineuriome, Lipome, Desmoidtumoren, Paragangliome, oder Hämangiomen zugeordnet werden 4 . Um diese sehr seltenen Entitäten besser zu verstehen, ist eine multizentrische Datensammlung wünschenswert 5 . Eine andere seltene Entität, die keine typisch „tumuröse“ Genese hat, aber in diesem Artikel aufgrund der Ähnlichkeit der klinischen Apparenz trotzdem beschrieben wird, sind die Ganglionzysten.…”

Section: Introductionunclassified

Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen

Dengler,

Pedro,

Kolbenschlag

2024

Handchir Mikrochir Plast Chir

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ZusammenfassungTumuröse Läsionen oder tumorähnliche Läsionen peripherer Nerven sind insgesamt selten, heterogen und herausfordernd in Diagnose und Therapie. Sie können durch tastbare Schwellungen in Nähe zu Nerven, sensible und/oder motorische Defizite, Berührungsschmerz oder neuropathischen Schmerz auffällig werden. In 91% der Fälle sind Tumore gutartig. Vor diesem Hintergrund sind eine Differenzierung der Entitäten, ihrer Charakteristika und eine funktionserhaltene Resektionsstrategie hochgradig relevant. Fehldiagnosen können gravierende Effekte für die Patienten mit schweren neurologischen Schäden und Schmerzsyndromen haben. Gutartige Tumore sind Schwannome und Neurofibrome, die sporadisch auftreten können aber auch bei Häufung mit neurogenetischen Tumordispositionssyndromen vergesellschaftet sein können. Seltenere gutartige Nerventumore sind Perineuriome, Lipome, die aggressive Fibrose (Desmoidtumor), Paragangliome und Hämangiome. Als tumorähnliche Läsionen werden die Ganglienzyste beschrieben. Die Assoziation von Nerventumoren zu neurogenetischen Syndromen und eine richtige Zuordnung von möglicherweise malignen Läsionen, wie dem MPNST (Maligne Periphere Nervenscheidentumor) oder den Zwischenstufen wie z. B. den ANNUBPs (atypische neurofibromatöse Neoplasien mit unbekanntem biologischem Potential) stellen besondere Herausforderungen dar. Die Interdisziplinarität hat eine hohe Relevanz für die klinische Behandlung und korrekte Diagnosestellung. Das Ziel unserer Arbeit ist die Übersicht über die relevanten Entitäten, die Diagnostik und zeitgemäße Therapiestrategien anhand der aktuellen Datenlage und unter Berücksichtigung der kürzlich erschienen interdisziplinären AWMF S2K-Leitlinie „Diagnostik und Therapie peripherer Nerventumore“.

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Collection of Rare Peripheral Nerve Tumors—Insights from the German Registry

Grübel,

Antoniadis,

Uerschels

et al. 2024

Cancers

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The most common peripheral nerve tumors are of a benign nature and include schwannoma or neurofibroma. In rare cases, other tumors or non-tumorous lesions can mimic peripheral nerve tumors clinically or radiologically. Based on data from the multicentric German Peripheral Nerve Tumor Registry (PNTR), which encompasses current information on 315 surgically treated patients from three high-volume centers, we present 61 cases of rare tumors and lesions that mimic tumors associated with peripheral nerves. This cohort displays considerable heterogeneity, featuring a broad spectrum of morphological features and biological potentials. Histopathological diagnoses include various intrinsic peripheral nerve tumors such as malignant peripheral nerve tumors (MPNSTs) (n = 13), perineurioma (n = 17), and hybrid nerve sheath tumors (HPNSTs, comprising schwannoma/perineurioma and schwannoma/neurofibroma) (n = 14), as well as atypical neurofibromatous neoplasm with unknown biological potential (ANNUBP) (n = 1). Additionally, the cohort encompasses extrinsic tumorous lesions like lymphoma (n = 3), lymphangioma (n = 2), hemangioma (n = 2), solitary fibrous tumor (n = 2), metastatic disease (n = 1), and single cases of other rare tumor entities (n = 6). An overview of the underlying pathology, imaging features, and clinical presentation is provided, with a brief description of each entity. A definitive preoperative differentiation between benign peripheral nerve tumors and rare intrinsic and extrinsic tumors is often not possible. Clinical examination and subtle imaging clues can at least indicate the possibility of a rare entity. The basic requirement is close cooperation between radiologists, neurologists, neuropathologists, and neurosurgeons at a specialized center to develop a multidisciplinary concept and offer the patient the best therapeutic approaches.

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Rationale and design of the peripheral nerve tumor registry: an observational cohort study

Cited by 3 publications

References 11 publications

Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry

Health-related quality of life in patients with peripheral nerve tumors: results from the German multicentric Peripheral Nerve Tumor Registry

Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen

Collection of Rare Peripheral Nerve Tumors—Insights from the German Registry

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