Analysis of Recurrent Sacrococcygeal Teratoma in Children: Clinical Features, Relapse Risks, and Anorectal Functional Sequelae

Abstract

BackgroundSacrococcygeal teratoma (SCT) is a relatively uncommon tumor. Recurrence with poor survival and anorectal dysfunction are the 2 leading problems for patients. Here, we would review the clinic features of patients with SCTs in our hospital to identify risk factors of recurrent SCTs and to analyze anorectal functional sequelae.Material/MethodsA retrospective review of all patients with SCTs in our center between 2007 and 2013 was performed. We analyzed the recorded data on each patient and performed follow-up through phone calls.ResultsOur study included 105 inpatients (78 girls and 27 boys); 104 cases underwent surgical resection, and 62.5% cases had a mature histopathology. The proportion of malignant teratomas rose with increasing age. Fifteen children developed recurrent SCTs with a median of 11.5 months, and most of them had an elevation of AFP levels. Four recurrent children experienced a second tumor relapse. We observed a statistically significant difference in survival rate through Kaplan-Meier method between relapsed (66.7%) and non-relapsed (94.4%) patients. In univariate analysis, incomplete primary resection and malignant histology were proven to increase recurrence risks. Nearly half of patients had at least 1 of the parameters reflecting abnormal bowel function (e.g., involuntary bowel movements, fecal incontinence, and constipation). For those recurrent SCTs patients, difficulty defecating was a major problem.ConclusionsTumor recurrence affected the prognosis of children with SCT. In our research, incomplete resection and malignant histology were considered risk factors. Constipation was the main problem in anorectal functional sequelae for children who had recurrence.

International Society of Paediatric Surgical Oncology (IPSO) Surgical Practice Guidelines

“…Surgery is an essential component of treatment. Complete resection of the coccyx is vital to minimise tumour recurrence, which has been reported as 2%–35% with an odds ratio of 17.78 when complete resection is not possible [ 7 ]. Surgery may be facilitated by preoperative chemotherapy for malignant GCT.…”

Diagnostic accuracy of serum alpha-fetoprotein levels in diagnosing recurrent sacrococcygeal teratoma: A systematic review

“…In 93% of the patients, age ranged from two to 42 months. There were eight outliers: one developed recurrence at 59 months, three at 84 months, two at 96 months and two at 204 months of age [1,[9][10][11]17]. This histological diagnosis of the recurrent tumor was mature teratoma in one, immature teratoma in one and malignant in five patients.…”

“…Median latency period to recurrence was described in six studies; these ranged from 1.3 to 42 months [1,9,11,15,18,19]. The histological diagnosis of the recurrent tumor was mature teratoma in 24 (19.8%), immature teratoma in 10 (8.3%) and malignant in 85 (70.2%) patients.…”

“…The recurrence risk of SCTs after resection is 12.5% (range 2-35%) [5,7,8]. Mature SCT has a mean recurrence rate of 10%, and immature and malignant SCTs have mean recurrence rates of 33% and 18%, respectively [9]. Recurrence pathology shows a shift towards malignancy; 22-56% of recurrences have some evidence of malignant yolk sac tumor (YST) [1,[9][10][11].…”

“…Mature SCT has a mean recurrence rate of 10%, and immature and malignant SCTs have mean recurrence rates of 33% and 18%, respectively [9]. Recurrence pathology shows a shift towards malignancy; 22-56% of recurrences have some evidence of malignant yolk sac tumor (YST) [1,[9][10][11]. Established risk factors for local recurrence are incomplete resection of the tumor or coccyx, tumor spillage during primary resection and microfoci of YST on pathological evaluation [5].…”