Correspondence to: Kamal Abd El-Elah Aly, MD, Pediatric Surgery Unit, Faculty of Medicine, Mansoura University, Mansoura, Egypt Tel.:+20 12 244 7637 , e-mail: kamalaly5@yahoo.com

Original Article

Annals of Pediatric Surgery, Vol 2, No 2, April 2006, PP 106-111

Sacrococcygeal Teratoma: A Neonatal Surgical Problem Kamal Abd Elelah Aly, Mahmoud Shoier, Tarek Badrawy

Pediatric Surgery Unit, Department of General Surgery, Faculty of Medicine, Mansoura University, Egypt

Bacground/ Purpose: Sacrococcygeal teratoma is the most common congenital tumor, which may present at birth or in the prenatal period. It is reported in approximately 1/35000 to 1/40000 live births. The Aim of the Work was to revise the clinical characters, pathological features, management options and outcome of sacrococcygeal teratoma treated at Mansoura University Children Hospital (MUCH)

Patients and Methods: Fifteen patients with sacrococcygeal teratomas were treated at (MUCH) during the period from January 2001 through June 2005. All cases were analyzed regarding prenatal and neonatal variables, tumor characteristics, and details of surgical treatment. Numerical data are expressed in the form of mean ± standard deviation using the appropriate statistical methods.

Results: The mean birth weight was 3137.27 ± 781.74 gm and the mean gestational age was 37.0 ± 2.8 weeks. There were 3 (20.0 %) males and 12 (80.0 %) females; 73.3 % of them were delivered by CS and 20.0 % had associated with congenital anomalies. According to Altman classification, there were: type I: 12 (80.0 %), type II: 2 (13.3 %) and type III: 1 (6.7 %). Macroscopically, there were 6 (40.0 %) solid tumors, 3 (20.0 %) cystic tumors and 6 (40.0 %) mixed tumors. Histologically, only 2 (13.3 %) tumors had immature tissues. Most tumors (93.3 %) were accessed sacrally, while 6.7 % were accessed abdomio-sacrally. 14 patients were tumor-free after surgery, while one patient had later recurrence.

Conclusion: Sacrcoccygeal teratoma, in spite rare, constitutes a considerable part of the neonatal surgical problems at our institution. The proper management of these cases necessitates quite attention and experience regarding the tumor presentation, management options and postoperative results with special emphasis on buttock contouring, bladder and anorectal functions.

Index Word: Sacrococcygeal Teratoma, Neonatal tumors, Germ cell tumor

INTRODUCTIONacrococcygeal teratoma (SCT) is a congenital tumor, which may present in the prenatal period

or at birth.1 It is the most common congenital tumor in the neonate, reported in approximately 1/35000 to 1/40000 live births. Approximately 80% of affected infants are females. Female to male preponderance is 4:1.2 Eighteen % of these infants have additional congenital anomalies. 3

SCT have tissues derived from ectoderm, mesoderm, and endoderm. Although their embryonic origin is still uncertain, they are believed to arise early in gestation (at around the late second or early third week) from the totipotential cells of Hensen’s node (also called the primitive knot), a remnant of the primitive streak in the coccygeal region.4

The tumor may be classified as benign (mature) and malignant or immature (composed of embryonic

S

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elements). Mature teratomas are more common in both neonates (68%) and older children (73%).5

The American Academy of Pediatrics surgical section (AAPSS) classification helps in grading the extent of SCT, as follows: Type I (47%): predominantly external with minimal presacral component; Type II (35%): present externally but with significant intrapelvic extension. Type III (8%): apparent externally but predominantly a pelvic mass extending into the abdomen. Type IV (10%): Presacral with no external presentation.6

The management of SCT depends on fetal gestational age, the presence of associated anomalies, and tumor vascularity assessed by color flow Doppler.7 Newborns with SCT have an excellent prognosis in contrast to the fetus with SCT that remains at high risk for perinatal complications and death.8-11

The high perinatal/neonatal mortality and morbidity rates are attributed to preterm delivery and the complications as malignant invasion, hemorrhage into the tumor, obstruction of the umbilical flow, high output cardiac failure, non-immune hydrops and bladder outlet obstruction.11,12 Some of these complications can be detected and treated prenatally.13

The use of Alphafetoprotein (AFP) as a tumor marker is well established, and persistent elevated level may indicate a residual tumor, recurrence or malignant degeneration.14,15 However, it should be done with caution in infants because its levels are normally elevated in the first 8 months of life.16The mean time required for AFP to be normalized after SCT resection is about 9 months.17

The standered treatment of SCT is, early surgical resection to prevent tumor ulceration and hemorrhage, and to reduce the risk of malignant changes whose incidence increase with the age of the infant at the time of diagnosis.7 Complete excision of the coccyx is mandatory; because microscopic nests of neoplastic cells are commonly found in or immediately adjacent to the coccyx.18 So, a recurrence rate of 37.0 % was reported when the coccyx wasn’t completely removed.19

MATERIALS AND METHODS The medical records of fifteen patients with SCT

were reviewed retrospectively at Mansoura University Children's Hospital (MUCH) during the period from January 2001 through June, 2005. After confirmation of the diagnosis, preoperative preparation included: routine laboratory tests to prove or exclude the presence of consumption coagulopathy secondary to intratumor bleeding in utero, during labor or after delivery, serum alpha-fetoprotein (AFP) estimation and Color flow Doppler US was performed to define the vascularity of the huge cases.

The birth weight of these patients ranged between 2250-5350 gm (mean: 3137.27 ± 781.74), the gestational age ranged between 34-42 weeks (mean: 37.0 ± 2.8). Other Demographic data are shown in Table 1. The mean weight of excised tumors was 679.7 ± 538.9 gm. (range: 80.0 – 2125 gm). Other tumor characteristics are shown in Table 2. The details of Surgical approach, extent of resection, recurrence, mortality and morbidity were recorded (Table 3). In all cases, the Altman classification, weight of the tumor, histological appearance, anorectal and bladder function and cosmetic outcome were documented.

The follow up period ranged from 18 days to 34 months (mean, 18.5 months). The postoperative follow up included: wound complications as disruption or necrosis, serum AFP evaluation every 3 months and stabilization of cardiac condition after excision of the huge highly vascular cases.

Numerical data are expressed in the form of mean ± standard deviation using the appropriate statistical method

RESULTS Surgical treatment had been carried out in all the

patients. The age at operation ranged from 3 days to 60 days (mean, 27.6 ± 17.0). Surgery was deferred in two of the three huge cases till control of cardiac failure caused by hyperdynamic circulation that associates these giant, highly vascular tumors.

Sacrococcygeal approach was performed for 14 cases (Altman I & II). A one-stage combined abdomino-sacral surgical intervention was performed in one case (Altman III). Lazy S incision was performed for the huge cases (Fig. 1[A,B],2[A,B])

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Fig 1A&B. Huge sacrococcygeal teratoma

Fig 2 A Gluteus maximus dissected exposing the tumor mass, B. Closure of the incision after resection of the huge tumor

Fig 3 A. Chevron incision Fig3 B. Rectum exposed after tumor excision

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Table 1. Demographic data of the studied cases

No %

Male 3 (20.0 %) Sex Female 12 (80.0 %)

Vaginal 4 (26.7 %)

Delivery CS 11 (73.3 %)

Present: PS VSD PDA & Ectopic Kidney

1 1 1

(20 %) Associated Anomalies

Absent 12 (80 %)

Table 2. Tumor Characteristics

No %

Type I 12 80.0 % Type II 2 13.3 % Altman

Classification Type III 1 6.7 %

Solid 6 40.0 % Cystic 3 20.0 %

Macroscopic Features

Mixed 6 40.0 % Mature 13 86.7 %

Histology Immature 2 13.3 %

Table 3. Access and Outcome

No %

Sacral 14 93.3 % Access Abdomino-sacral 1 6.7 %

Tumor-free 14 93.3 %

Outcome Recurrence 1 6.7 %

Fecal soiling 4 26.7 % 3) Functional impairment Poor cosmetic

results 6 40.0 %

while Chevron incision was performed in 12 (80%)

cases (Fig.3A), coccygectomy was performed in 13 (86.7 %) of cases (Fig. 5) Then the tumor was excised exposing the rectum (Fig. 3B).

Histopathological examination revealed differentiated benign SCTs in 13 (86.7 %); while 2 (13.3 %) showed immature tissue in some parts but no evidence of teratocarcinoma in either of them. Examination of the recurrent case (6.7 %) was benign similar to the primary lesion.

AFP decreased to normal levels 4-6 months postoperatively but elevated again when tested in the recurrent case

As regards the anorectal function, preservation of normal anal tone was noted in 11 (73.3 %) cases while 4 (26.7 %) cases showed weak tone with manifest soiling.

Unsatisfactory cosmetic results (presence of dimples, hypertrophic scars or disfiguring scars causing limited mobility of the buttock region) were observed in 6 (40.0 %) cases. Three of them showed wound infection and partial skin disruption that was left to heal with secondary intention.

DISCUSSION Sacrococcygeal teratoma is the most common tumor

diagnosed among newborns. In our series, female to male ratio was 4:1; which is consistent with that noted by Winderl and Silverman. The mean birth weight of the studied cases was 3137.27 ± 781.74 gm with a range of 2250-5350 gm. This wide range is attributed to the relatively huge tumors weighing 1850, 2050, and 2125 gm that shifted our results from those demonstrated by Perrelli et al,21 who had a mean birth weight of 2916 gm with a range of 1770-3640 gm. The gestational age at birth in our series (37.0 ± 2.8 weeks) was comparable with those reported by authors (35.0 ± 3.1 weeks).21

Holterman et al,11 recommend caesarean section (CS) for delivery when the tumor exceeds the diameter of 5 cm. This because the large uterine incision will minimize trauma to the tumor during delivery avoiding massive bleeding.22 In our series, 11 (73.3 %) of cases were delivered by CS. In Perrelli et al., series, it was 75 %.21

The incidence of congenital anomalies associated with sacrococcygeal teratoma varies from 5 to 26 %.20 In our series, congenital anomalies in the form of PS, VSD, PDA and ectopic kidney were recorded in 20 % of cases.

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In our series there were 12 (80.0 %) tumors with type I extent, 2 (13.3%) tumors with type II extent and one (6.7 %) tumor with type III extent, according to Altman classification. These results slightly vary from those reported by Perrelli et al, who had 75 % type I, 18.75 % type II and 6.25% type III.21

Macroscopically, 40% of the sacrococcygeal teratomas were solid, 20% were cystic and 40% were mixed. These notes differ from those stated by Keslar et al, who recorded 62 % of tumors assessed in their series as mixed tumors.5

Sacrococcygeal teratomas are graded according the presence of immature tissues. Grading of sacrococcygeal teratomas doesn’t seem to correlate directly with prognosis.5 In this series tumors that have immature tissues (13.4 %) are relatively fewer when compared with other studies with about 32 % of tumors containing immature cells.5

The treatment of sacrococcygeal teratoma is early surgical resection with complete excision of the coccyx.18 In the current study, all tumors were resected via sacral access apart from one tumor that necessitated abdomino-sacral approach.

Recurrence has been attributed to several possible factors, including, failure to achieve complete resection of the tumor, incomplete enbloc removal of the coccyx along with the tumor, tumor spillage, and failure to detect malignant components within the tumor.23 All cases of our series were tumor-free except one case that had later recurrence one year after excision of mature teratoma. These results are in accordance with those obtained by Bilik et al,15 who reported a 7.5 % recurrence rate. In our case, recurrence was benign similar to that of the primary lesion, and coccygectomy had been performed. It was caused by spillage of the tumor during resection.

Anorectal dysfunction occurred in 26.7% of cases in our series. On the other hand it was noted only in 20% of Bittmann and Bittmann series24.

Poor cosmetic results were the most common long-term complication after surgery for SCT in the literature. 24 Azizkhan and Caty reported cosmetic impairment in 29 % of cases.25 It was 40 % in our series because of infection and skin disruption in 3 cases and the huge tumour size in the another 3 patients.

CONCLUSION Sacrococcygeal teratoma constitutes a

considerable part of the neonatal surgical problems in our unit. It necessitates quite attention and experience regarding the tumor presentation, management options and postoperative results with special emphasis on buttock contouring, bladder and anorectal functions. Desirable cosmoses are not always attainable especially in huge disfiguring types

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