Sacrococcygeal teratoma: clinical course and prognosis with a special view to long-term functional results

Abstract

From 1976 to 1995, 23 children, 4 boys and 19 girls, were treated at our department for sacrococcygeal teratomas (SCT). Their records were analyzed retrospectively, considering age at operation, histopathology, recurrences, and long-term evolution. One died on the 1st day of life following tumor rupture with hemorrhagic shock without surgical intervention. All others were operated upon at a mean age of 4.2 days for those 19 (= 82%) who were diagnosed in the neonatal period and whose histology proved benign. In the remaining 3 children, in whom tumor manifestation did not occur before 11 months, 13 months, and 10 years of age, respectively, histopathologic evaluation revealed 2 carcinomas and 1 yolk-sac tumor, and all 3 recurred. Overall, 5 patients died, the 1 mentioned above, 1 due to volvulus after laparotomy, and 1 from multiple associated congenital malformations. Two deaths were related to malignancy, whereby only 1 was a malignant teratoma diagnosed at the original operation. Eight children had recurrences, 2 were benign and 6 malignant, with 3 of the latter having been graded benign on histology of the primary tumor. Of the 18 surviving patients, 17 (93.5%) returned for clinical review following a standardized protocol. The average interval from the primary surgery was 12.3 years (range 3.5-22 years). Four had malignant tumors with a recurrence-free period of from 9 to 14 years; 5 (29.4%) had urinary or anorectal functional impairment. One child with a patulous anus presented with fecal soiling. Two reported nocturnal enuresis, 1 associated with perineal anesthesia. One had a neurogenic bladder with overflow voiding and bilateral third-degree vesicoureteral reflux. Second-degree reflux was found in the last patient. We conclude that follow-up after surgery for SCT should not only search for tumor recurrence but include the diagnosis and treatment of possible secondary urinary and/or fecal incontinence.

Sacrococcygeal teratoma: Clinical characteristics and long-term outcome in Nigerian children

“…Four of our patients had malignant histology on presentation; two were lost to follow-up after initial excision and chemotherapy, while two were well at 12 and 24 months after surgical excision and chemotherapy. Even though our patients with malignant disease were few, it would appear that they do well after chemotherapy and excision as noted by Wakhlu et al [8] A number of reports have attempted to assess longterm outcome after treatment of SCT; [8,10,11,[15][16][17] which include continence, quality of life, and sexual function. In one UK study on the long-term followup of children treated for SCT, [18] a third had some functional problems, and Malone et al, found as many as 41% of children treated for SCT with some long-term functional sequelae.…”

“…Currarino later described three more cases and suggested that the etiology may be related to adhesions between the ectoderm and neural endoderm causing a split notochord. [17] In another report from North-Eastern Nigeria, associated congenital anomalies were noted in three of the patients. [6] In this present report, two children had associated congenital anomalies: one child with congenital heart disease and the other child with anal stenosis with tethering of the cord.…”

Long-term functional results following resection of neonatal sacrococcygeal teratoma

“…The treatment of choice for SCT is early surgical resection including complete excision of the coccyx. Patients with malignant SCT may be treated with chemotherapy and irradiation in addition to surgical resection [10].…”

Internal Sacrococcygeal Teratoma Causing Urinary Retention in a Newborn

“…Incomplete excision, incision and drainage of tumour is associated with risk of recurrence and malignant change [8]. The probability of recurrence of tumour is high in the first 2 years after the initial surgery [9]. The recurrence rate is between 3-35% [1,6].…”