Successful treatment of medically and surgically refractory lymphocytic hypophysitis with fractionated stereotactic radiotherapy: a single-center experience and systematic literature review.

IF 3.3 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM
Pituitary Pub Date : 2024-04-01 Epub Date: 2024-01-25 DOI:10.1007/s11102-023-01367-8
Mehdi Khaleghi, Guilherme Finger, Kyle C Wu, Vikas Munjal, Luma Ghalib, Peter Kobalka, Dukagjin Blakaj, Khaled Dibs, Ricardo Carrau, Daniel Prevedello
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Abstract

Purpose: To explore the potential role of focused radiotherapy in managing the lymphocytic hypophysitis (LH) refractory to medical therapy and surgery.

Method: A systematic literature review was conducted following PRISMA guidelines to identify the studies on radiation treatment for hypophysitis, along with the experience in our institution.

Results: The study included eight patients, three from our institution and five from existing literature. The age at presentation ranged from 37 to 75 years old, with a median age of 58. The presenting symptoms involved headache in seven patients and diplopia in two patients. Pre-radiation visual field defects were noticed in four patients. All patients exhibited variable degrees of hypopituitarism before radiation, with oral corticosteroids being the initial medical treatment. Immunosuppressive therapy was attempted in two patients prior to radiation. Seven patients had a history of transsphenoidal surgery with a histologically confirmed LH. Three patients underwent stereotactic radiosurgery (SRS), while the remaining received FSRT, with a mean irradiation volume of 2.2 cm3. A single-session total dose of 12 -15 Gy was administered in the SRS group. In the FSRT group, doses ranged from 24 to 30 Gy with a median dose of 25 Gy, delivered in 2 Gy fractions. Four patients achieved a resolution of visual field defects, while another two patients demonstrated improvement in their associated focal neurologic deficits. No change in pre-existing endocrine status was shown after radiation, except in one patient. Clinical response was achieved in seven patients after a single course of radiation, while one patient required the second course. Six patients remained stable on low-dose glucocorticoid during at least a 12-month follow-up period, and one discontinued it entirely without experiencing relapse. Three patients demonstrated a complete radiologic response, while the remaining showed a partial radiologic response.

Conclusions: Focused radiation, including FSRT, can play a role in symptomatic relief, effective mass shrinkage, and minimizing radiation exposure to critical surrounding structures in patients with refractory LH. However, further research efforts are necessary to better clarify its effects and optimal dose planning.

Abstract Image

分次立体定向放射治疗成功治疗药物和手术难治性淋巴细胞性肾上腺皮质功能减退症:单中心经验和系统性文献综述。
目的:探讨聚焦放射治疗在治疗药物治疗和手术治疗难治性淋巴细胞性肾上腺皮质功能减退症(LH)中的潜在作用:方法:按照PRISMA指南进行了系统性文献回顾,以确定有关放射治疗肾上腺皮质功能减退症的研究以及本机构的经验:研究共纳入八名患者,其中三名来自本院,五名来自现有文献。患者的发病年龄从 37 岁到 75 岁不等,中位年龄为 58 岁。七名患者的主要症状为头痛,两名患者为复视。四名患者在放射治疗前出现视野缺损。所有患者在放射治疗前都出现了不同程度的垂体功能减退,最初的药物治疗是口服皮质类固醇。两名患者在放射治疗前尝试了免疫抑制疗法。七名患者曾接受过组织学确诊的经蝶窦手术。三名患者接受了立体定向放射外科手术(SRS),其余患者接受了FSRT,平均照射体积为2.2立方厘米。SRS 组的单次总剂量为 12-15 Gy。在 FSRT 组中,剂量从 24 Gy 到 30 Gy 不等,中位剂量为 25 Gy,分 2 Gy 次照射。四名患者的视野缺损得到缓解,另外两名患者的相关局灶性神经功能缺损得到改善。除一名患者外,其他患者放疗后原有的内分泌状况没有发生变化。7 名患者在接受一个疗程的放射治疗后获得了临床反应,1 名患者需要接受第二个疗程的治疗。六名患者在至少 12 个月的随访期间一直稳定服用小剂量糖皮质激素,其中一名患者完全停用糖皮质激素后未出现复发。三名患者显示出完全的放射学反应,其余患者显示出部分放射学反应:包括 FSRT 在内的聚焦放射治疗可以缓解难治性 LH 患者的症状,有效缩小肿块,并最大限度地减少对周围重要结构的辐射照射。然而,要更好地阐明其效果和最佳剂量规划,还需要进一步的研究努力。
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来源期刊
Pituitary
Pituitary 医学-内分泌学与代谢
CiteScore
7.10
自引率
7.90%
发文量
90
审稿时长
6 months
期刊介绍: Pituitary is an international publication devoted to basic and clinical aspects of the pituitary gland. It is designed to publish original, high quality research in both basic and pituitary function as well as clinical pituitary disease. The journal considers: Biology of Pituitary Tumors Mechanisms of Pituitary Hormone Secretion Regulation of Pituitary Function Prospective Clinical Studies of Pituitary Disease Critical Basic and Clinical Reviews Pituitary is directed at basic investigators, physiologists, clinical adult and pediatric endocrinologists, neurosurgeons and reproductive endocrinologists interested in the broad field of the pituitary and its disorders. The Editorial Board has been drawn from international experts in basic and clinical endocrinology. The journal offers a rapid turnaround time for review of manuscripts, and the high standard of the journal is maintained by a selective peer-review process which aims to publish only the highest quality manuscripts. Pituitary will foster the publication of creative scholarship as it pertains to the pituitary and will provide a forum for basic scientists and clinicians to publish their high quality pituitary-related work.
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