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Original Article
Retroperitoneal Teratomas in Children Amit Chaudhary, Samir Misra, Ashish Wakhlu, R.K. Tandon and A.K. Wakhlu Department of Pediatric Surgery, King George Medical University, Lucknow, India
Abstract. Objective : This paper aims to highlight the clinical features, investigations and treatment of retroperitoneal teratomas condition. Methods : 12 patients (8 females and 4 males, age range-2 months to 14 yrs) of retroperitoneal teratoma admitted to the department of Pediatric Surgery, King George Medical University, Lucknow between 1980 and 2004 were studied. Investigations included hematology, plain X-ray of the abdomen, intravenous urography, ultrasound, computerised tomography (CT) of the abdomen (after 1990, 8 patients), and serum alpha-fetoprotein assay (after 1991, 6 patients, preoperatively). All patients underwent surgery. Serum alpha-fetoprotein assay was used during follow-up to detect recurrence. Results : Majority of the tumors were left pararenal in location. In two patients there was bilateral involvement. In all except one, the tumor could be excised easily preserving the kidneys. In one child with a massive cystic tumor with bilateral involvement, the tumor was marsupialised in the first stage and excised subsequently. One child died postoperatively, the other 11 children are well and there has been no tumor recurrence on follow-up. Conclusions : Retroperitoneal teratomas are uncommon lesions in children mostly arising in close relation to the kidneys. The majority are benign but complete excision is necessary for cure. Even large tumors with bilateral involvement of the retroperitoneum can be excised while preserving adjacent organs. Serum alphafetoprotein assay is a reliable method of detecting recurrence. [Indian J Pediatr 2006; 73 (3) : 221-223] E-mail :
[email protected]
Key words : Teratoma; Retroperitoneal; Germ cell tumors
Retroperitoneal teratomas are uncommon germ cell tumors in children.1 They present mainly as an abdominal mass with few other symptoms. Majority of the tumors are benign, situated on the left side and para renal in location; occasional lesions are bilateral. If diagnosed early, they are amenable to curative excision. MATERIALS AND METHODS Patients of retroperitoneal teratoma admitted to the Department of Pediatric Surgery between January 1980 and August 2004 were included in the study. Clinical history, examination findings, investigations and treatment details of the patients seen till January 2000 were obtained from hospital records (N=7) and, thereafter, recorded prospectively (N=5). Preoperative evaluation included plain X-ray, intravenous urography (IVU) (before 1990), abdominal ultrasound and contrastenhanced CT scan (after 1990) to delineate the nature and extent of the tumor. Hematological investigations and serum alpha-fetoprotein assay (AFP) (after 1991, 6 patients) were done to obtain preoperative values. Operation was through a transperitoneal approach in all
Correspondence and Reprint requests : Dr. Ashish Wakhlu, 1/147 Vivek Khand, Gomti Nagar, Lucknow–226 010 UP, India.
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cases. The excised specimens were subjected to detailed histological examination to detect malignant elements. The patients were reviewed at three weeks when serum AFP was estimated, it had returned to normal levels by this time in all 6 patients. Thereafter they were followed up in the Pediatric Oncology Unit with monthly estimation of serum AFP levels (for 1 year) to detect recurrence. RESULTS There were 12 patients aged 2 months to 14 years with a female preponderance (Table 1). The mode of presentation was abdominal enlargement with a palpable abdominal mass. The general condition of the children was good despite the presence of a large intra-abdominal tumor. Preoperative serum alpha-fetoprotein was elevated in all the 6 children evaluated. Imaging studies showed the presence of metacarpals in one patient (Fig 1) and a variegated tumor with solid and cystic areas in the others (Fig 2). Majority of the tumors were left-sided and closely-approximated to the kidney with the renal vessels stretched over the surface of the tumor. Two children had bilateral involvement (Fig. 3). All patients except one were operated through a supraumbilical transverse transperitoneal approach. The tumor was excised without difficulty; the renal vessels had to be carefully mobilized off the surface of the lesion, allowing preservation of the kidneys in all children, and in one instance a small rent in 221
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Amit Chaudhary et al TABLE 1. Patients Case no.
Age/Sex
Tumor
Imaging
Treatment/Nature
Outcome
1 2
3 yr/F 14 yr/F
Left para renal Bilateral
Plain X ray, IVU IVU
Excision/Benign Marsupialisation/Benign
4 yr/F 2 yr/F 6 yr/F 1 yr/M 5 yr/F 9 mth/F 2 mth/F 6 mth/M 2 yr/M 4 yr/M
Left para enal Left para renal Left para renal Left pararenal Right para renal Bilateral Right Pararenal Left Para renal Right para renal Left Para renal
Plain X ray, IVU CT scan CT scan CT scan CT scan CT Scan CT Scan CT Scan X ray, CT scan X ray CT Scan
Excision/Benign Excision/ Benign Excision/Benign Excision/Benign Excision/Benign Excision/Benign Excision/Benign Excision/Benign Excision/Benign Excision/Benign
Cured Reexcision Cured Cured Died Cured Cured Cured Cured Cured Cured Cured Cured
3 4 5 6 7 8 9 10 11 12
Fig 2. CT scan showing retroperitoneal teratoma involving both sides of the abdomen.
Fig. 1. Plain X ray showing bone formation in benign retroperitoneal teratoma.
the left renal vein was repaired. All the specimens were examined in theatre; among the contents were teeth, hair, bone and brown or pale fluid. In the oldest female child with massive bilateral cystic teratoma, the tumor was marsupialised onto the skin at the first operation because the organ of origin of the tumor was not clear. When the child returned three months later the size of the lesion had decreased, the residual tumor was excised uneventfully. Histological examination of the lesions showed that all 222
Fig. 3. Operative appearance of bilateral retroperitoneal teratoma.
were benign mature tumors. Postoperative recovery was uneventful in all patients except one who died in the immediate postoperative period due to inadequate recovery from anesthesia. All the patients have been followed for up to 6 yrs (longest duration). There has been no tumor recurrence in any child. Indian Journal of Pediatrics, Volume 73—March, 2006
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Retroperitoneal Teratomas in Children DISCUSSION Retroperitoneal teratomas comprise 3.5 – 4% of all germ cell tumors in children.1 Patients present with abdominal distension or a palpable mass. Occasionally, the tumor is present antenatally and diagnosed at birth, 2 these neonatal teratomas have a higher incidence of malignancy than those in older children2. Majority of the tumors in this study (7/12) were left-sided with a female preponderance; literature does not reveal a side or gender predilection and almost equal incidence in males and females have been reported. 1 All the patients in the present study had a mature benign tumor. Some of the reports on retroperitoneal teratoma highlight a diagnostic dilemma for these lesions. 3,4 In the present series the diagnostic algorithm was palpation of a solid flank mass, plain X-ray to demonstrate calcification or formed bony components like teeth and phalanges (which are pathognomonic). If these were present on X ray, ultrasound was sufficient to define the relationships of the tumor for planning surgery. If formed bony components were not visualised, CT scan was used to define the extent of the disease. Schey and Vesley5 have recommended only a plain abdominal X-ray and excision of the tumor if the characteristic calcification is demonstrated. Lack and Travis6 have also reported that the presence of bones or teeth on an X-ray was the most helpful in establishing a preoperative diagnosis. The authors suggest that CT scan is useful to delineate the extent of the disease in lesions occupying both sides of the retroperitoneum and those tumors where calcification is not seen on plain X-ray. However, it was seen in some of the patients under evaluation that the degree of tumor adherence to the adjacent structures suggested by CT was more than that found on exploration. CT findings should not therefore prevent surgical exploration of the tumor; even bilateral lesions are amenable to complete removal. Hayasaka and Yamada7 have reported internal homogeneity, fat density, cyst formation and calcification to be important predictors of a benign retroperitoneal tumor on CT. Papanicolau and Yoder 4 advocate angiography, inferior venacavography and needle biopsy for the accurate diagnosis of these tumors, which were not done in the present study. Serum alpha-fetoprotein was elevated preoperatively in six of the patients, it returned to normal after operation and formed a useful marker of monitoring recurrence. All reports agree that complete excision of the teratoma offers the best chance of cure.3 Malignancy is uncommon in retroperitoneal teratomas except endodermal sinus tumors and hence nonmutilating excision is possible and should be attempted even in lesions involving both sides of the abdomen. The
Indian Journal of Pediatrics, Volume 73—March, 2006
most important aspect of the excision is to remember the close relationship of these tumors with the kidneys. The renal vessels are invariably stretched out over the lesion, with care however, they can be separated from the teratoma. Removal of the kidney was not necessitated in any of the patients. Benign retroperitoneal teratomas are cured by complete removal. Lack and Travis 6 have reported guarded prognosis for unresectable lesions, immature teratomas and endodermal sinus tumors. Aggressive chemotherapy has been advocated for the latter lesions, which have a propensity for metastasis. Tapper and Lack8 reported that the single most important factor in prognosis was complete removal, no patient who did not undergo surgery or in whom only partial resection was possible, survived irrespective of the treatment used. CONCLUSION Retroperitoneal teratomas are uncommon tumors in children and majority of the lesions are benign. X-ray findings of calcification/ bone/ teeth are pathognomonic. Ultrasonography and CT scan are useful to delineate the extent of the tumor. Despite extensive local spread, the lesions are amenable to curative surgical excision. Recurrence can be monitored with tumor markers like serum alpha-fetoprotein. REFERENCES 1. Grosfeld JL, Ballantine TV, Lowe D, Bahener RL. Benign and malignant teratomas in children: Analysis of 85 Patients. Surgery 1976; 80: 297-305. 2. Auge B, Satge D, Sauvage P. Retroperitoneal teratomas in the perinatal period. Review of literature concerning a neonatal immature aggressive teratoma. Ann Pediatr 1993; 40: 613-621 3. Abdel Gauad G, Ivanchev I, Vasilev K. Retroperitoneal and pelvic presacral teratogenic tumors in early childhood – the diagnostic and surgical treatment problems. Khirurgiia (Sofia) 1990; 43: 1-5. 4. Papanicolau N, Yoder IC, Lee MJ. Primary retroperitoneal neoplasms: How close can we come in making the correct diagnosis. Urol Radiol 1992; 14: 221-228. 5. Schey WL, Vesly JJ, Radkowski MA. Shard like calcifications in retroperitoneal teratomas. Pediatr Radiol 1986; 16: 82-84. 6. Lack FE, Travis WD, Welch KJ. Retroperitoneal germ cell tumors in childhood. A clinical and pathological study of 11 cases. Cancer 1985; 56 : 602-608. 7. Hayasaka K, Yamada T, Saitoh Y, Sakura K. CT Evaluation of primary benign retroperitoneal tumor. Radiat Med 1994; 12: 115-120. 8. Tapper D, Lack EE. Teratomas in infancy and childhood. A 54 year experience at the Children’s Hospital Medical Center. Ann Surg 1983; 198 : 398-410.
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