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表现为无功能侵袭性垂体大腺瘤的鞍内浆细胞瘤:病例报告及文献综述

Intrasellar plasmacytoma presenting as a non-functional invasive pituitary macro-adenoma: case report & literature review.

作者信息

Sinnott B P, Hatipoglu B, Sarne D H

机构信息

University of Illinois at Chicago, 1819 West Polk Street, M/C 640, Chicago, IL 60612, USA.

出版信息

Pituitary. 2006;9(1):65-72. doi: 10.1007/s11102-006-8281-9.

Abstract

We report an uncommon case of an intrasellar plasmacytoma presenting as a non-functional invasive pituitary macro-adenoma as the first manifestation of multiple myeloma. A 57 year old woman was referred to our department with a presumed diagnosis of a non-functioning pituitary macro-adenoma. She reported a 3 month history of headaches and a 2 week history of sudden onset of right facial numbness. Preoperative endocrine evaluation was remarkable only for a modestly elevated serum prolactin. A magnetic resonance imaging (MRI) scan revealed 3.6 x 5 x 4 cm enhancing homogeneous intrasellar mass with extension into the sphenoid and cavernous sinuses bilaterally; the optic chiasm was not displaced. She underwent transphenoidal surgery of the sellar lesion. The surgical specimen was heavily infiltrated with abnormal plasma cells, which stained almost exclusively for Kappa light chain immunoglobulins. An extensive investigation was undertaken to look for occult myelomatous disease. A diagnosis of multiple myeloma was made 1 month later based on a combination of clinical, pathological and radiologic features. She underwent radiation therapy directed towards the residual sellar tumor, followed by chemotherapy and autologous stem cell transplantation. Review of the world literature revealed only 22 previous reports of patients in whom a solitary plasmacytoma or multiple myeloma first presented as a sellar mass; in all cases mimicking clinically and radiologically a non-functioning invasive pituitary adenoma however with additional cranial nerve involvement. Intrasellar plasma cell tumors are rare tumors which may mimic non-functioning invasive pituitary tumors. The diagnosis should be suspected in patients with well preserved anterior pituitary function and cranial nerve neuropathies in the presence of significant sellar destruction.

摘要

我们报告了一例罕见的鞍内浆细胞瘤,表现为无功能侵袭性垂体大腺瘤,是多发性骨髓瘤的首发表现。一名57岁女性因疑似无功能垂体大腺瘤转诊至我科。她自述有3个月头痛病史和2周突发右侧面部麻木病史。术前内分泌评估仅发现血清催乳素轻度升高。磁共振成像(MRI)扫描显示鞍内有一3.6×5×4 cm大小的均匀强化肿块,双侧延伸至蝶窦和海绵窦;视交叉未移位。她接受了经蝶窦手术切除鞍区病变。手术标本中大量浸润异常浆细胞,几乎仅对κ轻链免疫球蛋白染色。进行了广泛检查以寻找隐匿性骨髓瘤病。1个月后,根据临床、病理和影像学特征综合诊断为多发性骨髓瘤。她接受了针对残留鞍区肿瘤的放射治疗,随后进行化疗和自体干细胞移植。查阅世界文献发现,此前仅有22例关于孤立性浆细胞瘤或多发性骨髓瘤首发表现为鞍区肿块的患者报道;所有病例在临床和影像学上均酷似无功能侵袭性垂体腺瘤,但伴有额外的颅神经受累。鞍内浆细胞瘤是罕见肿瘤,可能酷似无功能侵袭性垂体肿瘤。对于垂体前叶功能良好且存在明显鞍区破坏并有颅神经病变的患者,应怀疑此病。

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