Gandhi Chaitanya, Koumna Stella, Chik Constance
Division of Endocrinology and Metabolism, Department of Medicine, University of Alberta, Edmonton, AB T6G 2G3, Canada.
Department of Radiology and Diagnostic Imaging, Cross Cancer Institute, Edmonton, AB T6G 1Z2, Canada.
JCEM Case Rep. 2024 Jul 15;2(7):luae123. doi: 10.1210/jcemcr/luae123. eCollection 2024 Jul.
Aggressive pituitary neuroendocrine tumors (PitNETs) present significant morbidity, and multimodal therapies including surgery, radiotherapy, and medications are frequently required. Chemotherapy, particularly temozolomide, is often pursued for tumors that progress despite these treatments. Although peptide receptor radionuclide therapy (PRRT) using radiolabeled somatostatin analogs is approved for the treatment of well-differentiated gastrointestinal neuroendocrine tumors, its use in aggressive PitNETs is limited. We describe the case of a 65-year-old man who presented with vision changes and hypopituitarism at age 33 secondary to a nonfunctioning gonadotroph PitNET. His initial treatment included a craniotomy followed by radiation therapy. With tumor regrowth, he required transsphenoidal surgeries at age 44 and age 52. At age 56, further tumor regrowth and a positive octreotide scan prompted treatment with long-acting octreotide for 1 year. Given absent tumor response, 12 cycles (4 treatment cycles and 8 maintenance cycles) of PRRT with Lutetium-DOTATATE were pursued. This resulted in partial response with significant tumor shrinkage. Notably, there was no tumor regrowth 40 months after treatment discontinuation. This is only the second report on the effectiveness of PRRT in patients with aggressive gonadotroph PitNETs. We also provide an overview of PRRT for PitNETs and describe clinical outcomes previously reported in the literature.
侵袭性垂体神经内分泌肿瘤（PitNETs）具有较高的发病率，通常需要手术、放疗和药物等多模式治疗。对于尽管接受了这些治疗仍进展的肿瘤，常采用化疗，尤其是替莫唑胺。虽然使用放射性标记生长抑素类似物的肽受体放射性核素治疗（PRRT）已被批准用于治疗高分化胃肠神经内分泌肿瘤，但其在侵袭性PitNETs中的应用有限。我们描述了一名65岁男性的病例，他在33岁时因无功能促性腺激素PitNET继发视力改变和垂体功能减退。他的初始治疗包括开颅手术，随后进行放射治疗。随着肿瘤复发，他在44岁和52岁时需要进行经蝶窦手术。56岁时，肿瘤进一步复发且奥曲肽扫描呈阳性，促使其接受长效奥曲肽治疗1年。鉴于肿瘤无反应，采用了12个周期（4个治疗周期和8个维持周期）的镥-奥曲肽PRRT治疗。这导致了部分反应，肿瘤显著缩小。值得注意的是，治疗中断40个月后肿瘤未复发。这是关于PRRT在侵袭性促性腺激素PitNETs患者中有效性的第二篇报道。我们还概述了PitNETs的PRRT，并描述了文献中先前报道的临床结果。
