Mandagere K A, Schimke R N, Kyner J L, Bhatia P S
Division of Metabolism, Endocrinology and Genetics, University of Kansas Medical Center, Kansas City, Kansas 66160-7318, USA.
Endocr Pract. 1998 Nov-Dec;4(6):382-6. doi: 10.4158/EP.4.6.382.
To describe a case of solitary intrasellar plasmacytoma in a patient with a preoperative diagnosis of a nonfunctioning pituitary adenoma.
A case of a solitary intrasellar plasmacytoma is presented, in which the clinical and laboratory findings are detailed and the response to treatment is discussed. Pertinent reports from the literature are reviewed.
A 53-year-old woman came to the neurology clinic with complaints of frontal headaches and intermittent blurry vision. Physical examination showed no remarkable findings. Computed tomography revealed an enhancing sellar and suprasellar mass, with extension into the sphenoid and cavernous sinuses. The patient had a preoperative diagnosis of a clinically nonfunctioning pituitary adenoma and underwent transsphenoidal resection. The biopsy specimen was heavily infiltrated with abnormal plasma cells, which stained exclusively for lambda light chain immunoglobulins. An extensive investigation failed to show evidence of multiple myeloma. In view of these findings, the diagnosis of solitary extramedullary plasmacytoma was made. Postoperatively, the patient received radiotherapy to the pituitary and has remained free of disease for 7 years. Review of the world literature disclosed only 17 previous reports of patients in whom a solitary plasmacytoma or multiple myeloma first appeared as a sellar mass. In each case, the plasmacytoma mimicked a clinically nonfunctioning pituitary adenoma.
Parasellar plasmacytomas are often mistaken for a nonfunctioning pituitary adenoma. The diagnosis is difficult to make in the absence of overt systemic myeloma. Nevertheless, normal pituitary function associated with severe destruction of the pituitary fossa, cranial neuropathies, and diabetes insipidus are clues that the primary lesion is outside the pituitary gland itself. The current patient is unique in terms of prolonged survival in the absence of systemic myeloma. Perhaps those patients with progression of disease did not have extramedullary tumors because such lesions rarely progress to overt myelomatous disease.
描述一例术前诊断为无功能垂体腺瘤患者的孤立性鞍内浆细胞瘤。
报告一例孤立性鞍内浆细胞瘤病例,详细阐述其临床和实验室检查结果,并讨论治疗反应。回顾相关文献报道。
一名53岁女性因前额头痛和间歇性视力模糊就诊于神经科门诊。体格检查未见明显异常。计算机断层扫描显示鞍内及鞍上有强化肿块,延伸至蝶窦和海绵窦。该患者术前诊断为临床无功能垂体腺瘤,接受了经蝶窦切除术。活检标本中大量浸润异常浆细胞,这些浆细胞仅对λ轻链免疫球蛋白染色。广泛检查未发现多发性骨髓瘤的证据。鉴于这些发现,诊断为孤立性髓外浆细胞瘤。术后,患者接受了垂体放疗,至今已无病生存7年。查阅世界文献发现,此前仅有17例关于孤立性浆细胞瘤或多发性骨髓瘤首次表现为鞍区肿块的患者报道。在每例病例中,浆细胞瘤均酷似临床无功能垂体腺瘤。
鞍旁浆细胞瘤常被误诊为无功能垂体腺瘤。在无明显系统性骨髓瘤的情况下,诊断较为困难。然而,垂体功能正常但垂体窝严重破坏、伴有颅神经病变和尿崩症,这些线索提示原发性病变位于垂体腺本身之外。本例患者在无系统性骨髓瘤的情况下长期生存,较为独特。也许那些疾病进展的患者并无髓外肿瘤,因为此类病变很少进展为明显的骨髓瘤疾病。