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.
最常见的周围神经肿瘤性质为良性，包括神经鞘瘤或神经纤维瘤。在罕见情况下，其他肿瘤或非肿瘤性病变在临床或影像学上可模拟周围神经肿瘤。基于德国多中心周围神经肿瘤登记处（PNTR）的数据——该登记处汇集了来自三个高手术量中心的315例手术患者的当前信息，我们呈现了61例罕见肿瘤及模拟周围神经肿瘤的病变病例。该队列显示出显著的异质性，涵盖了广泛的形态学特征和生物学潜能。组织病理学诊断包括多种内在性周围神经肿瘤，如恶性周围神经鞘瘤（MPNSTs）（n=13）、神经束膜瘤（n=17）和混合性神经鞘瘤（HPNSTs，包括神经鞘瘤/神经束膜瘤和神经鞘瘤/神经纤维瘤）（n=14），以及具有未知生物学潜能的不典型神经纤维瘤性肿瘤（ANNUBP）（n=1）。此外，该队列还包括外在性肿瘤样病变，如淋巴瘤（n=3）、淋巴管瘤（n=2）、血管瘤（n=2）、孤立性纤维性肿瘤（n=2）、转移性疾病（n=1）以及其他罕见肿瘤实体的个别病例（n=6）。本文概述了其基础病理学、影像学特征和临床表现，并对每种实体进行了简要描述。术前明确区分良性周围神经肿瘤与罕见的内在性和外在性肿瘤通常难以实现。临床检查和细微的影像学线索至少可以提示罕见实体的可能性。基本要求是专科中心的放射科医师、神经科医师、神经病理学家和神经外科医师之间密切合作，制定多学科诊疗方案，为患者提供最佳治疗策略。
Collection of Rare Peripheral Nerve Tumors—Insights from the German Registry
肿瘤(癌症)患者之家