Correspondence: Essam Elhalaby, M.D, Department of Pediatric Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt, eselhalaby@idsc.net.eg

 

Annals of Pediatric Surgery Vol 5, No 2, April 2009, PP 119-125

Original Article

Sacrococcygeal Teratoma: Management and Outcomes Amel Hashish A, Hisham Fayad, Ashraf El-attar A, Mohamed Moursi Radwan , Khalid Ismael ,

Mohamed H M Ashour, Essam Elhalaby Department of Pediatric Surgery, Facullty of Medicine, Tanta University, Tanta, Egypt

Background/Purpose: Sacrococcygeal teratoma (SCT) is a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the various patterns of clinical presentation, and factors affecting the outcome of patients with SCT.

Materials & Methods: A total of 35 patients with SCT were received during the period from January 1998 to November 2008 at Tanta University Hospital and its affiliated hospitals. A retrospective review of all recorded data concerning maternal history, mode of delivery, age at presentation, patient sex, presenting clinical features, associated anomalies, various laboratory and radiological investigations, operative details, and tumor histopathology were performed. Early and late postoperative functional results and complications were also reviewed. The patients were divided into 2 groups according to the age at presentation. Group I involved 21 patients presented at the neonatal period, while group II included 14 patients presented after the first month of life. The follow up period ranged between 3 months and 8 years

Results: One patient died prior to surgery. Excision of the tumors was achieved in the remaining 34 patients. Living patients in group I (n=20) were operated upon at a median age of 2 days. The histology proved to be benign in all patients in this group, and no recurrence was noted during a follow period ranging between 3 months and 8 years. The mean age at surgery was 6+ 3 months in group II patients (n= 14). In this group, three patients proved to have malignant changes in the excised specimens and postoperative recurrence occurred in 4 patients.

Conclusion: A prenatal diagnosis of SCT is essential to avoid early mortality. This study stressed on the importance of: early diagnosis, early complete en-block resection of the tumor along with the coccyx, and the avoidance of intraoperative spillage of the tumor as critical factors in prognosis. Delayed presentation and the presence of malignant changes continued to be poor prognostic factors. Close follow-up of these patients is mandatory to deal with the potential postoperative sequelae of surgery.

Index Word: Sacrococcygeal Teratoma, children, complications.

INTRODUCTION

acrococcygeal teratomas (SCT) represent a group of benign and malignant tumors deriving from the perimordial germ cells. They 

are believed to originate from embryological pluripotent cell during embryogenesis. 1-3 Most germ cell tumors in the neonatal period are benign and are classified as either mature or immature teratomas.4,5 SCT are the most common neonatal tumors, with an

incidence of one in 35,000 to 40.000 live births.6 Females are affected more frequently with a female to male ratio of almost 4:1.7,8 Sacrococcygeal teratoma can be diagnosed during antenatal ultrasonography. Polyhydraminos is found in up to 20% of cases.9 Previous studies have shown a significant relationship between age of the patients at diagnosis and outcome of treatment of SCT. The

S

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incidence of malignancy at the neonatal period is approximately 10%, against almost 100% at the age of 3 years.3,10 Therefore, early surgical intervention, the use of combined abdomino-perineal approach when indicated to assure devascularization of the tumor, and complete excision of the tumor are all important prognostic factors in the outcome. Prognosis depends also on the tumor size, the histological type as well as the degree of prematurity.11 Excision of the coccyx is mandatory in all cases. Failure to remove the coccyx results in 30- 40% recurrence rate, with a higher probability of malignancy.12

Recurrence after resection varies from 2-35%. This may result from incomplete surgical excision with the presence of microscopic residues, non resection of the entire coccyx and/ or tumor spillage.13-15 The reappearance of the tumor in patients in whom no complete macroscopic control of primary tumor could be achieved is usually considered as progression of the disease rather than recurrence.16

Most of the tumor-related mortalities are attributed to the hyperdynamic state caused by the arteriovenous shunting within the extremely vascular tumor, which results in high output cardiac failure.17

The purpose of this study was to analyze the patient's characteristics, surgical technique, histology of the resected tumor and the impact on early and delayed outcome of patients with SCT treated at our institution.

PATIENTS AND METHODS A total of 35 patients with SCT were received during the period from January 1998 to November 2008 at Tanta University Hospital and its affiliated hospitals. A retrospective review of all recorded data concerning maternal history, mode of delivery, age at presentation, patient sex, presenting clinical features, associated anomalies, various laboratory and radiological investigations, operative details, and tumor histology with respect to type and resection margins were performed. Early and late postoperative functional results and complications were also reviewed. Each patient was evaluated with regard to her/his recurrence free survival, postoperative bowel and urinary tract function. The follow up period ranged between 3 months and 8 years .

For analytical purposes, patients were divided into 2 groups, according to the age at presentation. Group I involved 21 patients who presented at the neonatal period, while group II included 14 patients who presented after the first month of life.

Most patients in group I were investigated by routine laboratory tests only, while plain X-ray, ultrasonography, CT and/ or MRI scans were performed to older patients presented after the neonatal period (group II). Four patients had serum alphafetoprotein (AFP) assessment preoperatively. Thirty four patients underwent surgical intervention. One neonate died on the first day of life following rupture of his massive tumor prior to surgical intervention .

RESULTS

The study included 11 boys and 24 girls. The diagnosis of SCT had been made antenatally in 7, at birth in 14, and after the neonatal period in 14 patients. The main presenting clinical features are summarized in table 1. The presence of mass in the sacrococcygeal region was the main symptom in the majority of cases.

Table 1. Clinical presentation

Main Presentation Number

Presacral mass 28

Pelviabdominal mass 3

Constipation 2

Urinary tract symptoms 2

Associated malformations were detected in 2 patients. One patient had spinal malformation and another one had sacral anomalies.

The tumors were classified according to the criteria proposed by the Surgical Section of the American Academy of Pediatrics 6 as follows :

� Type I: (n=15, 45.8%) predominantly external with minimal presacral component. One patient died shortly after birth prior to surgical intervention as mentioned before .

� Type II: (n=10, 34%) external but with significant intrapelvic extension .

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� Type III: (n=6, 17.1%) apparently external but predominantly pelvic extending into the abdomen .

� Type IV: (n=4, 11.4%) presacral with no external component .

Perineal or abdomino-perineal en-block dissection of the tumors together with the coccyx was performed in 34 patients. Fourteen patients of type I, and 5 of the 10 patients of type II were successfully operated through an entirely perineal approach. Transabdominal mobilization was required in the remaining 5 type II patients, as well as all type III and type IV patients. Coccygectomy was performed for all patients. Spillage due to rupture of the tumor occurred in 3 patients.

Patients were followed for a period ranged between 3 months and 8 years. No recurrence was reported in group I (20 followed up patients) while recurrence occurred in 4 patients in group II (14 patients). Two of the three patients with proved malignant changes in the excised tumor died. Among the 32 survivors, 2 patients had bladder dysfunction (one had urinary straining and the other had urinary incontinence), two had fecal incontinence, 3 had chronic

constipation, one had motor weakness of the lower limbs. Good postoperative cosmetic results were noted in 26 out of the 34 patients (76.5%). The cosmetic results were considered acceptable in 5, while 3 patients will need revision of the scar due to poor cosmetic outcome.

Histological analysis of the resected specimen showed mature teratoma in 20 patients and various foci of immature teratoma in 14 patients. The different histopathological grades are shown in table 2.

Table 2. Histopathological grades of 34 resected tumors

Grade No

Grade 0 (Tumor contains only mature tissue) 20

Grade 1 (Rare foci of immature tissues) 7

Grade 2 (Moderate quantities of immature

tissues) 5

Grade 3 (Large quantities of immature tissue –

malignant yolk sac elements) 2

 

 

 

Fig 1a: preoperative view of a type I the tumor is predominately external 

 Fig 1b: Tumor bed after complete excision of the tumor with attached coccyx

 

 

 

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 Figure 2a: preoperative view of a type II tumor 

Figure 2b: the tumor has significant intrapelvic extension 

 Fig 3a: preoperative view of a type III tumor. 

 Fig 3b: Although the tumor is apparently external, it is predominately pelvic intraoperatively 

 Fig 4a: CT of a type IV tumor. 

Fig 4b: the tumor is totally presacral stretching the rectosigmoid, uterus, and urinary bladder infront of it during abdominal exploration. 

   

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DISCUSSION 

Sacrococcygeal teratoma is the most common neonatal tumor. A female preponderance has been consistently noticed in the published series. This also proved true in the current series, albeit the female to male ratio of 2.2:1 is less than the previously reported 4:1 ratio in the other series.7,8

Teratomas in children originate most commonly in the Sacrococcygeal region (SCT), but they may also occur in the gonads, the retroperitoneal region or even in the brain or the liver.18 In this study, associated congenital anomalies were found in 5.7% of our cases. Billman and Billman reported 20% incidence of associated congenital anomalies in their series of 25 patients.19

Antenatal diagnosis can be achieved in more than 50% of patients.20 Ultrasonography done prenatally can detect SCT that appears as a caudal mass with or without intraabdominal extension. Antenatal diagnosis facilitates the decision of elective cesarean delivery. In the current study, only seven (20%) patients were diagnosed antenally. Six of them had elective cesarean section. There should be more awareness among obstetric-gynecologist regarding the importance of antenatal diagnosis of these cases. In our study, the only case that died preoperatively had a huge mass that was not detected antenatally. Vaginal delivery resulted in severe intra-tumoral hemorrhage and death of the child. Clearly, such death was preventable if the case was diagnosed during antenatal screening. Roberson et al17 stated that successful management of fetal SCT requires very close obstetric observation because the rate of tumor growth is unpredictable and the hyperdynamic state may develop precipitously. They recommended weekly sonogram to detect early signs of decompensation such as increase aortic flow and dilated cardiac chambers or IVC.

Sacrococcygeal teratoma is classified according to its anatomical location and extension, following the American Academy of Pediatric Surgical Section classification. In the literature, Altman type I is present in 47%, type II in 35% type III in 8% and type IV in 10% of cases.6 In the current study, the incidence of the four types was 45.8%, 34%, 17.1%, and 11.4% respectively. This is comparable to that reported in the American Academy of Pediatrics survey.6

As expected, patients with type 1 and type 2 SCT present early, while delayed diagnosis may occur in

type 3 and 4 patients, as the presacral element can pass unnoticed. In the current study, 21 patients presented in the neonatal period. These patients suffered either type I, II or III SCT. Fourteen patients presented after the neonatal period, all of them had type III or IV SCT.

Rescorla et al14 reported that all children presenting after the age of one year had malignant tumors. Gabra et al21 reported that 71% of their patients presenting after the neonatal period were malignant. In our study, 8.6 % of the cases were malignant and all of them presented after the neonatal period.

Patients with SCT may present with sacral and presacral anomalies (Currarino's triad), with symptoms of bladder or bowel dysfunction.22 In our series, none of our patients had Currarino's triad. However, bladder dysfunction with retention and dribbling of urine due to stretching and compression on bladder neck occurred in some patients. Another 2 patients presented with fecal impaction.

Management of SCT is mainly surgical. Not only that the earlier the diagnosis, the better the prognosis. But also, the earlier the surgical intervention (for neonates diagnosed prenatally), the better the prognosis.11 In our study, early resection was performed in 20 patients (group I). There was no postoperative mortality in this group and the prognosis was excellent. A complete surgical excision of the tumor including coccygectomy was accomplished in all these cases to avoid recurrence as recommended in previous studies.11,23

Robertson et al17 recommended staged resection, by initial devascularization through ligation of the internal iliac arteries and middle sacral artery to correct the hyperdynamic state, lessens the risk of hemorrhage and decrease the blood loss during the subsequent tumor resection. But they also reported that staged resection carried the theoretical risk of malignant transformation in the retained tumor. Therefore, they recommended using histological grading as guide to urgency of resection of the residual tumor. We believe that such approach is not necessary, and one-stage approach, whether entirely transperineally or combined transabdominal/ perineal can be safely accomplished in all cases .

Many reports highlighted the potential postoperative complications after SCT resection. Postoperative rectal dysfunction was found in up to 40% of cases in some seies.7.,24 Constipation and fecal incontinence occurred in 14.7% of our cases. The

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frequency of postoperative bladder dysfunction in our series (5.9%) is quite favorable with the previously reported literature, in which it ranged between 20% to 50% of patients.24,25 A long-term observation and follow up is required in these patients to deal with any urinary or bowel dysfunction and the appearance of delayed complications .

There are few publications concerning the long-term cosmetic outcome after SCT surgery. Bittmann and Bittmann19 reported a poor cosmetic result in more than half of their patients after a follow up period of 396 months. They recommended that cosmetic results should be integrated with bladder, anorectal and sexual function assessment, in the follow up programs of such patients. A good cosmetic results were obtained in 26 of the 34 operated patients in the current series (76.5% .(

Teratomas are divided into immature and mature tumors depending on the amount of immature elements in them, mostly neuroepthelium.26 Tumor recurrence was reported in 2- 35% of patients in the different series. Recurrence was due to incomplete resection of the tumor, failure of the en-block removal of the coccyx along with the tumor, tumor spillage or the presence of immature tumors. Mature teratoma should not recur if complete surgical excision and coccygectomy were achieved properly.16,27 De Backer et al16 reported a recurrence rate ranging between 0-26% for mature teratomas and 12-55% for immature teratomas in the literature. In our study, Recurrence of the tumor occurred in 4 patients (1.8%). All these patients had immature teratomas and were operated after the age of 5 months. Diagnosis of recurrence in our patients was confirmed by imaging studies, and increasing AFP. The use of AFP as a tumor marker in the follow up of those patients has been recommended by many authors.28 Persistently elevated AFP levels may indicate a residual, or recurrent tumor. On the other hand, Brewer and Tank 29 reported that the level of AFP is normally elevated in the first 8 months of life; therefore such high levels should be interpreted with caution in infants. Repeated AFP assessment was feasible only in 4 cases, 3 in group I, and in only one patient in group 2. There was no increasing titre or recurrence in any of these patients .

CONCLUSION

A prenatal diagnosis of SCT is essential to avoid early mortality. This study stressed on the importance of: early diagnosis, early complete en-block resection of the tumor along with the coccyx, and the avoidance of intraoperative spillage of the tumor as critical factors in prognosis. Delayed presentation and the presence of malignant changes continued to be poor prognostic factors. Close follow-up of these patients is mandatory to deal with the potential postoperative sequelae of surgery.

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