Fercho Justyna Małgorzata, Chasles Oskar G, Chamier-Gliszczyński Jakub, Płaza Dominik Ryszard, Jabłoński Bogdan, Kokot Klaudia, Mielczarek Maciej, Małłek-Grabowska Małgorzata, Szypenbejl Jacek, Szkudlarek Adrian, Szmuda Tomasz, Siemiński Mariusz, Furtak Jacek
Neurosurgery Department, 10th Military Research Hospital and PolyClinic SPZOZ, Bydgoszcz, Poland.
Department of Emergency Medicine, Medical University of Gdańsk, Gdańsk, Poland.
Front Endocrinol (Lausanne). 2025 Aug 25;16:1633411. doi: 10.3389/fendo.2025.1633411. eCollection 2025.
Pheochromocytoma (PCC) is a rare neuroendocrine tumor, with 10-15% of cases showing malignant behavior defined by metastatic spread, including exceptionally rare central nervous system (CNS) involvement. Brain metastases present unique diagnostic and therapeutic challenges due to their potential to impair neurological function. This study reports a case of malignant PCC (mPCC) with CNS metastases and a systematic review to clarify the clinical patterns, management strategies, and prognostic factors.
We describe the surgically managed case of a 41-year-old man with right frontoparietal brain metastasis. A systematic review, adhering to the PRISMA 2020 guidelines, searched PubMed, Scopus, and Web of Science for peer-reviewed studies on mPCC with brain or spinal metastases confirmed by radiology or histopathology. Data on demographics, symptoms, imaging, treatments, and outcomes were extracted and descriptively analyzed using Python-generated graphics.
This review identified 18 cranial (1948-2022) and 60 spinal (1977-2024) metastasis cases from 53 studies. Cranial metastases were present at a mean age of 46.6 years (SD 14.1), commonly with headaches (44.4%) and neurological deficits, such as weakness, presented in our case, with 72.2% surgically treated. Spinal metastases occurred at a mean age of 44.5 years (SD, 16.0), often with hypertension (51.7%) or pain, with a mean of 1.7 lesions (SD 1.5). The patient achieved short-term symptom relief post-resection, but incomplete follow-up (33.3% cranial) and reporting gaps (63.3% spinal laterality) limited the prognostic insights. MRI and PET improved the diagnostic accuracy over historical non-contrast CT use (41.7% spinal cases).
CNS mPCC metastases are exceedingly rare with distinct neurological (cranial) and structural (spinal) presentations. Advanced imaging, particularly magnetic resonance imaging (MRI) and positron emission tomography (PET), is critical for accurate diagnosis and surgical planning. Sparse data underscores the need for registries and prospective studies to standardize care and improve outcomes.
嗜铬细胞瘤（PCC）是一种罕见的神经内分泌肿瘤，10%-15%的病例表现出由转移扩散定义的恶性行为，包括极其罕见的中枢神经系统（CNS）受累。脑转移瘤因其可能损害神经功能而带来独特的诊断和治疗挑战。本研究报告了一例伴有CNS转移的恶性PCC（mPCC）病例，并进行了系统综述，以阐明临床模式、管理策略和预后因素。
我们描述了一例41岁男性右额顶叶脑转移瘤的手术治疗病例。按照PRISMA 2020指南进行系统综述，在PubMed、Scopus和科学网中检索经放射学或组织病理学证实的伴有脑或脊髓转移的mPCC的同行评审研究。提取有关人口统计学、症状、影像学、治疗和结局的数据，并使用Python生成的图形进行描述性分析。
本综述从53项研究中确定了18例颅内转移（1948 - 2022年）和60例脊髓转移（1977 - 2024年）病例。颅内转移的平均年龄为46.6岁（标准差14.1），常见症状为头痛（44.4%）和神经功能缺损，如本病例中的肌无力，72.2%接受了手术治疗。脊髓转移的平均年龄为44.5岁（标准差16.0），常伴有高血压（51.7%）或疼痛，平均有1.7个病灶（标准差1.5）。患者术后短期内症状缓解，但随访不完整（颅内33.3%）和报告缺失（脊髓侧别63.3%）限制了对预后的了解。与以往使用的非增强CT相比，MRI和PET提高了诊断准确性（脊髓病例中为41.7%）。
CNS mPCC转移极其罕见，有独特的神经学（颅内）和结构学（脊髓）表现。先进的影像学检查，尤其是磁共振成像（MRI）和正电子发射断层扫描（PET），对于准确诊断和手术规划至关重要。数据稀少凸显了建立登记系统和开展前瞻性研究以规范治疗并改善结局的必要性。
