并发乳头状颅咽管瘤和分泌生长激素的垂体腺瘤:一种罕见的侵袭性碰撞肿瘤

Q3 Medicine
Alyssa J. Mancini MD , Ribu Mathew MD , Jaymie Oentoro MD , Alma M. Devine MD , Carolyn Maxwell MD, ECNU , Igor Kravets MD, FACP, FACE
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引用次数: 0

摘要

背景/目的由颅咽管瘤和垂体腺瘤组成的碰撞性肿瘤极为罕见。我们报告了一起由乳头状颅咽管瘤和分泌生长激素的垂体腺瘤组成的碰撞性肿瘤,据我们所知,这是首次报告此类肿瘤。磁共振成像(MRI)显示有一个 4.1 厘米的蝶鞍/鞍上肿块,肿块影响视交叉。肿瘤经过两次开颅手术切除,第二次是由于间隔生长,两次手术后的病理结果均显示为乳头状颅咽管瘤。IGF-1为517纳克/毫升(68-225),生长激素抑制试验呈阳性。复查磁共振成像显示肿瘤残留,并对视丘产生持续的肿块效应,于是开始进行放射治疗。核磁共振成像显示肿块呈间歇性生长,IGF-1升至700纳克/毫升,随后患者接受了经蝶窦肿瘤切除术;病理显示为残留乳头状颅咽管瘤和PIT1系腺瘤,大部分细胞表达生长激素。讨论蝶鞍撞击瘤的临床表现往往具有侵袭性,因为它们往往在术前未被诊断出来,从而降低了完全切除的可能性,导致颅咽管瘤的复发率升高。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Concurrent Papillary Craniopharyngioma and Growth Hormone-Secreting Pituitary Adenoma: A Rare and Aggressive Collision Tumor

Background/Objective

Collision tumors composed of craniopharyngiomas and pituitary adenomas are extremely rare. We report a collision tumor formed by a papillary craniopharyngioma and a growth hormone-secreting pituitary adenoma, which is the first report of such a tumor, to the best of our knowledge.

Case Report

A 49-year-old man presented with 2 months of headaches and blurry vision. An exam demonstrated frontal bossing, enlarged jaw and hands, macroglossia, and bitemporal hemianopsia, and magnetic resonance imaging (MRI) showed a 4.1 cm sellar/suprasellar mass with mass effect on the optic chiasm. The tumor was resected twice via a craniotomy, the second time due to interval growth, with the pathology after both surgeries showing a papillary craniopharyngioma. IGF-1 was 517 ng/mL (68-225) and growth hormone suppression test was positive. Repeat MRI showed residual tumor with ongoing mass effect on the optic chiasm and radiation therapy was initiated. MRI showed interval growth of the mass and IGF-1 rose to 700 ng/mL after which the patient underwent a transsphenoidal resection of the tumor; the pathology showed a residual papillary craniopharyngioma and a PIT1 lineage adenoma with most cells expressing growth hormone. After developing numerous complications, the patient passed away.

Discussion

Collision tumors of the sella are often associated with an aggressive clinical course, as they often go undiagnosed preoperatively, thus reducing the likelihood of total resection and leading to higher rates of craniopharyngioma recurrence.

Conclusion

A pituitary mass with an aggressive clinical course should prompt a high index of suspicion for a sellar collision tumor, though prognosis remains poor.

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来源期刊
AACE Clinical Case Reports
AACE Clinical Case Reports Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
2.30
自引率
0.00%
发文量
61
审稿时长
55 days
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