Villegas Ma Celine Isobel A, Dungca Leona Bettina P
Division of Pediatric Surgery, Department of Surgery, Philippine General Hospital, University of the Philippines Manila.
Acta Med Philipp. 2024 Oct 15;58(18):85-90. doi: 10.47895/amp.vi0.5698. eCollection 2024.
Sacrococcygeal teratomas (SCT) are the most common extragonadal tumors of early childhood. Their clinical characteristics and outcomes of patients with sacrococcygeal tumors who underwent excision in the Philippines has never been described, while numerous retrospective studies have been conducted in other countries.
This was a retrospective, descriptive study over a four-year period (December 2014 to November 2018). The study described the patients' demographic data, manner of delivery, clinical presentation, prenatal diagnosis of tumor, Altman classification, and alpha fetoprotein levels. These information were obtained from the medical records of the patients. Additional data from the operative technique include the surgical approach, size of the mass, and gross involvement of adjacent structures and the final histopathologic results. Outcomes include the 30-day mortality and morbidity, and tumor recurrence.
A total of 29 patients were included in the study with 22 females (75.86%) and seven males (24.14%). Twenty-five out of the 29 (86.21%) had a sacral or gluteal mass at birth while other presenting factors include a palpable abdominal mass (1), constipation (1), difficulty in urination (1), and an elevated AFP in one postoperative patient. Even if 27 out of the 29 patients underwent a maternal ultrasound, only three patients (10.34%) had a correct ultrasound interpretation of sacrococcygeal teratoma. Age at presentation was problematic, with 12 presenting at greater than one year of age while 10 were brought for consultation at greater than one month old. Only seven presented at the neonatal period. CT scan was the most common imaging tool utilized (37.93%), followed by ultrasound (27.59%). AFP was elevated in ten patients (34.48%). Six of the patients with elevated AFP had mature teratoma, two had yolk sac tumor, one had fibroepithelial polyp, and one was post chemotherapy but had mature teratoma based on the final histopathology report. Fifteen out of the 29 patients had Altman type I tumors (51.72%), seven (24.14%) had type II tumors, six (20.69%) had type III tumors, and only one patient had type IV tumor. Sacral approach in the excision of the sacrococcygeal tumor was performed in 25 patients (86.21%). There was no reported perioperative mortality for patients who underwent surgery for SCT during the study period. Twelve out of the 29 had postop morbidities, three with surgical site infection and three with rectal or vaginal perforation. Five patients had tumor recurrence occurring from two months to three years postoperatively.
Early detection of sacrococcygeal teratomas even in the prenatal period is the norm in certain areas of the world, but in our country, prenatal detection is still a challenge. Even if the majority of the patients presented with a gluteal mass at birth, less than a third were brought to our tertiary government hospital in neonatal life. The sacral approach for SCT excision was employed for the great majority of our patients, but due to the advanced age at diagnosis and locally advanced disease, morbidities occurred in about a third of the patients. Therefore, early detection prenatally and early referral to a pediatric surgical center should be achievable goals for physicians dealing with these patients.
骶尾部畸胎瘤(SCT)是幼儿期最常见的性腺外肿瘤。菲律宾骶尾部肿瘤患者接受切除术后的临床特征和预后从未被描述过,而其他国家已经进行了大量的回顾性研究。
这是一项为期四年(2014年12月至2018年11月)的回顾性描述性研究。该研究描述了患者的人口统计学数据、分娩方式、临床表现、肿瘤的产前诊断、奥特曼分类以及甲胎蛋白水平。这些信息从患者的病历中获取。手术技术的其他数据包括手术入路、肿块大小、相邻结构的大体受累情况以及最终的组织病理学结果。结局包括30天死亡率和发病率以及肿瘤复发情况。
该研究共纳入29例患者,其中女性22例(75.86%),男性7例(24.14%)。29例中有25例(86.21%)出生时骶部或臀部有肿块,其他表现因素包括可触及的腹部肿块(1例)、便秘(1例)、排尿困难(1例)以及1例术后患者甲胎蛋白升高。尽管29例患者中有27例接受了产前超声检查,但只有3例患者(10.34%)超声正确诊断为骶尾部畸胎瘤。就诊年龄存在问题,12例患者就诊时年龄大于1岁,10例患者在大于1月龄时前来咨询。只有7例在新生儿期就诊。CT扫描是最常用的影像学检查工具(37.93%),其次是超声(27.59%)。10例患者(34.48%)甲胎蛋白升高。甲胎蛋白升高的患者中6例为成熟畸胎瘤,2例为卵黄囊瘤,1例为纤维上皮息肉,1例为化疗后患者,但根据最终组织病理学报告为成熟畸胎瘤。29例患者中有15例为奥特曼I型肿瘤(51.72%),7例(24.14%)为II型肿瘤,6例(20.69%)为III型肿瘤,只有1例患者为IV型肿瘤。25例患者(86.21%)采用骶部入路切除骶尾部肿瘤。研究期间接受SCT手术的患者未报告围手术期死亡情况。29例中有12例术后出现并发症,3例手术部位感染,3例直肠或阴道穿孔。5例患者术后2个月至3年出现肿瘤复发。
在世界某些地区,即使在孕期早期发现骶尾部畸胎瘤是常态,但在我国,产前检测仍是一项挑战。即使大多数患者出生时臀部有肿块,但不到三分之一的患者在新生儿期被送至我们的三级政府医院。大多数患者采用骶部入路切除SCT,但由于诊断时年龄较大且疾病局部进展,约三分之一的患者出现了并发症。因此,对于处理这些患者的医生来说,产前早期检测和早期转诊至小儿外科中心应该是可以实现的目标。