From the University of Vermont Larner College of Medicine (S.S.H., R.J., C.J.A., J.C.D., B.T., A.A.T.), Burlington; Department of Surgery, Division of Neurosurgery (R.J., B.T.); Department of Radiology, Division of Radiation Oncology (C.J.A.); Department of Pathology and Laboratory Medicine (J.C.D.); and Department of Neurological Sciences (A.A.T.).
Neurology. 2022 Jul 18;99(3):117-122. doi: 10.1212/WNL.0000000000200748.
Meningeal melanocytomas are extremely rare, pigmented tumors of the CNS. They generally carry a favorable prognosis, although recurrence and transformation into the more aggressive malignant melanoma have been reported. We present a case of a patient who reported constipation and abdominal pain around the umbilicus, which progressed into cord compression with lower extremity weakness and gait instability. Spinal MRI revealed a tumor at the level of T11, and she underwent gross total resection of the mass. Pathology demonstrated a meningeal melanocytoma with intermediate features. She received postoperative radiation therapy and had stable disease for 3 years, at which time she developed new weakness and drop metastases. This case represents a rare presentation of a rare disease, in which a spinal cord tumor presented with constipation and abdominal distress. Intradural extramedullary tumors of the thoracic spine are most commonly nerve sheath tumors or meningiomas, but rare entities such as melanocytomas can present in this location; even more rarely, these tumors can have an aggressive course with delayed recurrence.
脑脊膜黑色素细胞瘤是极为罕见的 CNS 色素性肿瘤。它们通常具有良好的预后，尽管已有复发和转化为侵袭性更强的恶性黑色素瘤的报道。我们报告了一例患者，其主诉为脐周便秘和腹痛，随后进展为脊髓压迫，出现下肢无力和步态不稳。脊髓 MRI 显示 T11 水平有一肿瘤，行大体全切除。病理显示为具有中等特征的脑脊膜黑色素细胞瘤。患者接受了术后放疗，病情稳定 3 年，此时出现新的无力和颅神经转移。本例代表了一种罕见疾病的罕见表现，即脊髓肿瘤表现为便秘和腹部不适。胸段椎管内硬脊膜外肿瘤最常见的是神经鞘瘤或脑膜瘤，但黑色素细胞瘤等罕见实体也可出现在此部位；更罕见的是，这些肿瘤可能具有侵袭性病程，并出现延迟复发。
