Kartini Diani, Dasawala Filipus, Ham Maria Francisca
Department of Surgery, Surgical Oncology Subdivision, Dr. Cipto Mangunkusumo General Hospital, Faculty of Medicine, Universitas Indonesia, Jakarta, 10430, Indonesia.
Department of Surgery, Faculty of Medicine, Universitas Indonesia, Jakarta, 10430, Indonesia.
Int J Surg Case Rep. 2020;77:337-340. doi: 10.1016/j.ijscr.2020.10.140. Epub 2020 Nov 10.
Multiple endocrine neoplasia type 1 (MEN1) is a rare syndrome with common presenting signs and symptoms secondary to primary hyperparathyroidism (PHPT), which is managed surgically. Traditionally, either subtotal parathyroidectomy (SPTX) or total parathyroidectomy with autologous transplantation (TPTX) is the approach of choice. However, recent studies showed comparable persistence and recurrence rate in a subset of MEN1 patients (two or more concordant preoperative imaging results) who underwent less than subtotal parathyroidectomy (LSPTX).
We report a case of patient with PHPT and delayed diagnosis of MEN1, who underwent LSPTX without intraoperative parathyroid hormone (IOPTH) measurement. The approach was chosen based on the preoperative imaging studies. Unfortunately, the PHPT persisted and the patient was reoperated. To further elucidate the issue, a systematic search of the literature was conducted on Cochrane library, PubMed, and Scopus; articles relevant to the case were reviewed. Results are conflicting results with most of the studies showed LSPTX is inferior compared to the other two approaches.
Therefore, given the current body of evidence, we consider that subtotal or total parathyroidectomy is still the preferred surgical approach for the treatment of PHPT in MEN1.
Further studies are still needed to see whether LSPTX is comparable to SPTX or TPTX in regards to persistent and recurrent hyperparathyroidism if the conditions are met.
1型多发性内分泌腺瘤病(MEN1)是一种罕见综合征,常见的临床表现和症状继发于原发性甲状旁腺功能亢进症(PHPT),通常采用手术治疗。传统上,次全甲状旁腺切除术(SPTX)或甲状旁腺全切加自体移植术(TPTX)是首选的手术方式。然而,最近的研究表明,在一部分接受了小于次全甲状旁腺切除术(LSPTX)的MEN1患者(术前影像学检查结果两项或更多项一致)中,疾病持续存在和复发率相当。
我们报告一例PHPT患者,MEN1诊断延迟,该患者接受了LSPTX,术中未测量甲状旁腺激素(IOPTH)。手术方式是根据术前影像学检查确定的。不幸的是,PHPT持续存在,患者接受了再次手术。为了进一步阐明这个问题,我们在Cochrane图书馆、PubMed和Scopus上进行了系统的文献检索;对与该病例相关的文章进行了综述。结果相互矛盾,大多数研究表明LSPTX比其他两种手术方式效果差。
因此,鉴于目前的证据,我们认为次全或全甲状旁腺切除术仍然是治疗MEN1患者PHPT的首选手术方式。
如果条件满足,仍需要进一步研究来确定LSPTX在甲状旁腺功能亢进症持续存在和复发方面是否与SPTX或TPTX相当。