Ectopic dopamine agonist-resistant macroprolactinoma to the clivus masquerading as a chordoma - A case report.

Q3 Medicine Endocrine regulations Pub Date : 2025-03-12 Print Date: 2025-01-01 DOI:10.2478/enr-2025-0002
Ines Bayar, Atef Ben Nsir, Sana Abid, Bilel Ben Amor, Hela Marmouch, Asma Ben Mabrouk, Kais Maamri, Jamel Saad, Mehdi Darmoul, Ines Khochtali
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Abstract

Objective. Pituitary neuroendocrine tumors (PitNETS) are common intracranial tumors, but extrasellar or ectopic PitNETS are very rare and supposed to originate from some pituitary remnants. They are mostly found in sphenoidal sinus. But particularly, ectopic clival PitNETS are highly aggressive and can cause bone invasion and can be misdiagnosed as other lesions of the skull base such as chordomas. Case Report. We report a challenging case of an ectopic prolactin-secreting PitNET arising in the clivus in a young female presenting with secondary amenorrhea and sellar mass effect symptoms. On magnetic resonance imaging (MRI), the tumor showed osteolytic features that firstly oriented towards chordoma. Regarding the very high levels of prolactin that constantly exceeded 200 ng/mL, prolactinoma was indeed very presumable. Dopamine agonist treatment was progressively introduced to its maximal tolerated dose, but with neither hormonal response nor size reduction. Hence, surgical resection was decided and the patient underwent an endoscopic transsphenoidal resection of the tumor that was purely ectopic to the clivus. The diagnosis of prolactinoma was confirmed by pathological examination and immunohistochemical staining was intensely and diffusely positive for prolactin and focally for follicle-stimulating hormone (FSH) and luteinizing hormone (LH). The surgery succeeded to normalize prolactin level, but with residual tumor on the fourth month MRI control. Conclusion. Management of these rare tumors should be individualized with multidisciplinary collaboration.

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目的。垂体神经内分泌瘤(PitNETS)是常见的颅内肿瘤,但星状或异位垂体神经内分泌瘤非常罕见,应该源自某些垂体残余。它们多见于蝶窦。但特别是异位蝶窦PitNETS具有高度侵袭性,可导致骨侵袭,并可能被误诊为脊索瘤等其他颅底病变。病例报告。我们报告了一例具有挑战性的病例,一名年轻女性因继发性闭经和蝶窦肿块症状而被诊断为异位催乳素分泌型 PitNET,该病例发生在蝶窦。磁共振成像(MRI)显示,该肿瘤具有溶骨特征,首先倾向于脊索瘤。泌乳素水平非常高,经常超过 200 纳克/毫升,因此很有可能是泌乳素瘤。多巴胺受体激动剂的治疗剂量逐渐达到最大耐受剂量,但既没有激素反应,体积也没有缩小。因此,患者决定接受手术切除,并在内窥镜下接受了经蝶窦肿瘤切除术,该肿瘤完全异位于蝶窦。病理检查证实了催乳素瘤的诊断,免疫组化染色显示催乳素呈强阳性和弥漫性阳性,卵泡刺激素(FSH)和黄体生成素(LH)呈灶性阳性。手术成功地使催乳素水平恢复正常,但在第四个月的磁共振成像对照中仍有肿瘤残留。结论。对这类罕见肿瘤的治疗应在多学科协作下进行个体化治疗。
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来源期刊
Endocrine regulations
Endocrine regulations Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
2.70
自引率
0.00%
发文量
33
审稿时长
8 weeks
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