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Experience With Testis Sparing Surgery for Testicular Teratoma
0022-5347/04/1711-0161/0 THE JOURNAL OF UROLOGY® Copyright © 2004 by AMERICAN UROLOGICAL ASSOCIATION
Vol. 171, 161–163, January 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000101185.90327.b4
EXPERIENCE WITH TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA ASEEM R. SHUKLA, CHRISTOPHER WOODARD, MICHAEL C. CARR, DALE S. HUFF, DOUGLAS A. CANNING,* STEPHEN A. ZDERIC, THOMAS F. KOLON AND HOWARD M. SNYDER, III From the Divisions of Pediatric Urology and Pathology (DSH), The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
ABSTRACT
Purpose: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery. Materials and Methods: We reviewed the pathology records at our institution for all testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We specifically examined the prepubertal incidence of teratoma, including epidermoid cysts, and our experience with testis preserving surgery. Preoperative and postoperative ultrasonography images were used to calculate the atrophy index following surgery. Patients were contacted for long-term followup. Results: Of 77 primary testicular and paratesticular tumors 38 were diagnosed in prepubertal boys (age younger than 13 years) including 11 mature teratomas and 5 epidermoid cysts. Mean patient age at treatment was 34.4 months (range 4 months to 10 years). All boys presented with a painless scrotal mass, cystic foci within an intratesticular mass on ultrasound and a normal ␣-fetoprotein level. Of the 16 boys with benign teratomas 13 (81%) were treated with a testis sparing procedure. At a mean 7-year followup no patient has presented with recurrent tumor in the ipsilateral or contralateral testicle. Postoperative physical examination and scrotal ultrasound were obtained in 9 patients at a median followup of 10.2 months, and there was no evidence of testicular atrophy or persistent discomfort. Conclusions: Unlike previously published series based on tumor registries, benign teratoma was the most common pediatric testicular tumor treated at our institution. Our single institution experience with testis preservation and long-term followup confirms the role and safety of this technique. Testis sparing surgery remains our technique of choice for testicular teratoma. KEY WORDS: testicular neoplasms, teratoma, surgery
Testicular tumors in infants and prepubertal children comprise only 1% of all pediatric solid neoplasms.1 The rarity of their presentation and limited experience with treatment outcomes at a single institution prompted the establishment of the Prepubertal Testis Tumor Registry by the American Academy of Pediatrics in 1980. The experience of this registry has confirmed clear differences in the incidence, presentation, natural history and pathological subtype of testicular tumors between the prepubertal and adult population.2, 3 The overall incidence of germ cell tumors is lower in children than in adults, malignant subtypes such as seminoma and embryonal carcinoma are absent in prepubertal patients but common in adults and teratomas, which are uniformly benign in children, may be malignant in adults.3, 4 We describe the incidence of various pediatric testicular tumor subtypes during 25 years at the Children’s Hospital of Philadelphia and the consistent differences in pathobiology compared to the adult testicular tumors. We found, as previously noted by Rushton and Belman,5 that testicular teratoma and its epidermoid cyst subtype were often clearly
distinguishable from normal adjacent parenchyma in children. The distinct appearance of the lesion and the uniformly benign outcome for testicular teratoma after orchiectomy encouraged us to implement a testis preserving approach for select patients. We present our single center experience with the incidence of testicular teratoma and definitive treatment with testis preserving surgery. MATERIALS AND METHODS
We reviewed the pathology records at our institution for all 77 testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We obtained permission from the Institutional Review Board at our hospital, and then specifically analyzed the records of only prepubertal patients (age less than 13 years) with testicular teratoma and epidermoid cysts. While all patients presenting with teratoma or epidermoid cyst underwent radical orchiectomy before 1990, a testis preserving surgery was selectively performed in subsequently treated patients. We recorded age at presentation, preoperative and postoperative ultrasonography images, and individual treatment outcomes for boys who underwent testis preserving surgery. Those patients were contacted to affirm recurrence-free status and for subjective evidence of testicular atrophy. The cosmetic, sonographic and recurrence-free outcome was evaluated with
Accepted for publication August 1, 2003. Study received Institutional Review Board approval. * Correspondence: Division of Pediatric Urology, Children’s Hospital of Philadelphia, 3rd Floor Wood Building, Children’s Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, Pennsylvania 19104-4399 (telephone: 215-590-2769; FAX: 215-5903985). 161
162
TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA
a physical examination and followup ultrasonography. Testicular size was calculated in cc by the Lambert formula as (length ⫻ width ⫻ breadth) ⫻ 0.71.6 An atrophy index for the enucleated testis was calculated after modification of the formula commonly used for assessing differential testicular growth in boys with varicoceles: (contralateral testicular volume—affected testicular volume)/(contralateral testicular volume) ⫻ 100, expressed as a percent.7 The testis preserving procedure consisted of an inguinal incision and testis mobilization with the cord compressed at the internal ring. The surgical field was then carefully draped with towels, the tunica vaginalis was incised and the testicle was exposed. If the tumor appeared consistent with teratoma with an encapsulated, cystic and peripherally located lesion, occlusion on the spermatic cord was removed to maintain optimal vascular flow to the affected testicle. The tumor was excised using the edge of a scalpel to enucleate it from the bordering normal parenchyma. If an infiltrative lesion was noted, the child was peripubertal or postpubertal, or the location and size of the mass precluded a testis sparing approach, then radical orchiectomy was performed. RESULTS
A total of 77 primary testicular and paratesticular tumors were encountered in males younger than 18 years at our institution between 1976 and November 2002. The tumor types and frequency of presentation are shown on table 1. Of the 43 germ cell tumors 22 (51.5%) were teratomas (16 mature teratomas, 1 immature teratoma and 5 epidermoid cysts) and 10 (23.2%) were yolk sac tumors. A total of 38 boys were prepubertal at the time of presentation, and pathology and distribution of germ cell tumors are shown in table 2. We identified mature teratomas and epidermoid cysts in 11 and 5 boys, respectively, with a mean age of 34.5 months (range 4 months to 10 years). All of these prepubertal patients presented with a painless scrotal mass and ␣-fetoprotein (AFP) levels that were normal for age. Ultrasound was available for 14 (87.5%) patients preoperatively and demonstrated a complex, welldemarcated hypoechoic lesion distinct from adjacent normal appearing testicular parenchyma. A testis preserving procedure was used to treat 13 of 14 prepubertal boys who presented with a teratoma or epidermoid cyst after 1990. Radical orchiectomy was performed for 1 prepubertal boy who presented with a large teratoma in the testis mediastinum that precluded a testis sparing approach. Of the 13 tumors removed by testis preserving surgery 8 were mature teratomas and 5 were epidermoid cysts. Frozen section analysis was requested following exposure of the mass in 10 cases (77%). Final diagnosis correlated with the frozen section report in all cases. One patient had an enlarging hematoma in the immediate postoperative period and was returned to surgery for hemostasis. At a mean 88 months following surgery (range 5 months to 22
TABLE 1. Classification of 77 pediatric testicular tumors Histology Germ cell tumors: Teratoma (including epidermoid cysts) Yolk sac tumor Embryonal Ca Mixed malignant germ cell tumor Monophasic chorioca Seminoma Ca in situ Not otherwise specified Stromal tumors Paratesticular tumors Primary lymphomas Gonadoblastomas Miscellaneous (regressed tumor, cystic dysplasia)
Total No. (%) 43 (57) 22 (30) 10 (13) 2 (2.5) 4 (5) 1 (1) 2 (3) 1 (1) 1 (1) 9 (12) 8 (10) 5 (7) 1 (1) 11 (14)
TABLE 2. Frequency of prepubertal germ cell tumors and treatment performed Germ Cell Tumor Type
Testis Sparing Procedure After 1990
Orchiectomy
Total No. (% all prepubertal tumors)
Yolk sac tumor Mature teratoma Epidermoid cyst
0 8 5
8 3 0
8 (21) 11 (29) 5 (13)
years) no patient presented with recurrent tumor in the ipsilateral or contralateral testicle. A total of 10 (77%) patients treated with testis sparing surgery were successfully contacted for longterm followup at a median of 72 months (mean 65, range 15 to 120). All patients reported that they were free of recurrence, testicular atrophy or persistent orchalgia. No patient reported dissatisfaction with the cosmetic appearance or any abnormalities on palpation. Patient report was confirmed by a physician administered physical examination in 4 of these 10 patients at a mean of 38.8 months (range 18 to 60). Two of these 4 patients were prepubertal in age and development at the time of the last followup physical examination. Postoperative testicular examination and scrotal ultrasound were obtained for 9 (69%) patients who underwent testis preserving surgery at a mean of 10.2 months following surgery (range 5 to 60). There was no evidence of testicular dysmorphia, although the affected testicle was smaller with a mean atrophy index for 7 patients of 10.3%. Affected testicles in the 2 remaining patients sustained a mean 35% increase in volume compared to the normal contralateral testicle. Imaging of the entire testicle confirmed normal appearing parenchyma with no evidence of previous surgery or of recurrent lesions. DISCUSSION
Teratoma was the most commonly encountered pediatric testicular germ cell tumor at our institution comprising more than 50% of all tumor types. Among prepubertal boys teratomas were 50% more frequent than yolk sac tumors. This finding differs substantively with that of the Prepubertal Testicular Tumor Registry. The tumor registry compiled 244 cases of yolk sac tumor (64% of all germ cell tumors) compared to 92 (23%) teratomas concurring with the traditional view that yolk sac tumor is the most common germ cell tumor of the testicle in the prepubertal male.3 We, however, agree with others that teratoma is more likely the most common prepubertal testicular tumor, and discrepancy with the registry is due to underrepresentation of benign tumors.3, 8, 9 Testicular teratoma is a uniformly benign entity in prepubertal children with a clear distinction from the adult population in which malignancy may be present.1–3, 5, 10, 11 The benign course of the tumor has encouraged implementation of a testis preserving approach to treatment. While Altadonna et al reported testis preserving surgery as a definitive treatment option for excision of benign testicular cysts,12 Weissbach first applied this approach to 2 patients with teratoma.13 Rushton et al provided extensive pathological analysis of 5 teratoma specimens removed by testis sparing procedure to ascertain the intrinsic safety of this approach.14 The authors found that testicular parenchyma adjacent to the enucleated tumor was normal along with no evidence of tumor multifocality in distant testicular tissue. This report further corroborated our approach to testis preservation, which became our treatment of choice for suspected teratomas and epidermoid cysts in prepubertal children after 1990. Testis preserving surgery may be rendered impossible, however, as it was in 1 of our patients, if the tumor burden is exceedingly large and centered near the mediastinum of the testicle. Initially, we obtained a frozen section analysis following enucleation of the lesion to confirm teratoma histology for all
TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA
patients undergoing testis preserving surgery. As our experience progressed, however, we increased our reliance on the preoperative AFP level, physical examination, and initial sonographic and intraoperative visual appearance of the lesion to dictate the need for frozen sections. When frozen section was performed, we found, as have others, that final pathology was in concordance with frozen section in all cases.14, 15 For our 3 cases that did not have a frozen section report, final pathology confirmed a benign lesion. In our experience cystic lesions of the testicle are always benign (benign simple cysts, epidermoid or dermoid cysts, or teratomas) and a frozen section analysis may be safely omitted when confronted with a predominantly cystic testicular tumor in a prepubertal child. We do not routinely excise testicular tissue adjacent to the lesion for biopsy due to the negligible risk for tumor multifocality.14 –16 A report of the finding of intratubular germ cell neoplasia adjacent to a mature testis teratoma in a 3-year-old boy raises the question of the need to biopsy the adjacent testicular tissue in these patients.17 However, the clinical implication of carcinoma in situ in the prepubertal testis is unclear. The Pediatric Oncology Group study of prepubertal testis and its relationship to intratubular germ cell neoplasia found that germ cells adjacent to infantile germ cell tumors are commonly proliferative but not neoplastic.16 Following testis preserving surgery, the remaining testicular parenchyma appears to be largely unaffected by surgical manipulation. We calculated an atrophy index when postoperative sonography was available in 9 patients to calibrate the differential testicular size following surgery. A mean atrophy index of 10.2% in 7 patients affirmed our clinical suspicion that no substantial size alterations were distinguishable postoperatively in most cases. We believe that the large testicular tumor likely compresses the normal testicular parenchyma but when the benign tumor is shelled out, the compressed parenchyma usually regains a near normal testicular size. We did find an actual increased volume in the postoperative testis compared to the unaffected contralateral testicle in 2 cases. Both of these patients underwent followup sonography at 6 months and we speculate that the short interval may reflect ongoing postoperative changes. Further imaging and physical examinations following a longer interval may reveal eventual resolution of size discrepancy in these cases. After a median of 6 years following testis preserving surgery, there have been no tumor recurrences of teratoma or epidermoid cysts since 1976. Our single institution experience with 13 cases concurs with the recent report by Valla on the results of testis sparing surgery at 19 institutions.18 A total of 16 teratomas and 13 epidermoid cysts were treated by 22 surgeons in that study with no recurrences, testicular atrophy or discordant frozen section final pathology cases at a mean followup of 56 months. The safety of testis sparing surgery for prepubertal children with mature teratoma or epidermoid cysts has now been confirmed by our experience and that of others, and should be considered the primary treatment of choice.3, 9, 18, 19 Ross et al have suggested an algorithm in which testis sparing surgery is recommended for prepubertal children with a normal serum AFP level, ultrasound with adequate normal testis parenchyma and after exclusion of yolk sac tumor by frozen section.3 Physiologically normal increases in serum AFP are expected for infants, and the authors suggest excluding from testis sparing surgery those patients between 6 and 12 months old with AFP greater than 100 ng./ml. Indeed, the pure histological nature of almost all prepubertal germ cell tumors has encouraged application of testis sparing surgery to all prepubertal benign tumors, including teratomas, epidermoid cysts, Sertoli cell, Leydig cell and juvenile granulosa cell tumors, with excellent results.3, 18 –20 While our experience with other benign testicular tumors has been limited, we suggest that our experience broadly supports this approach.
163
CONCLUSIONS
Testicular teratoma was the most common pediatric testicular tumor treated at our institution, contrary to previously published series based on tumor registries. Under-reporting of benign tumors by most institutions may explain this discrepancy. Our single institution experience with testis sparing surgery and long-term followup confirms the role and safety of this technique. Testis preserving surgery remains our technique of choice for the treatment of testicular teratoma, including epidermoid cysts.
REFERENCES
1. Brosman, S. A.: Testicular tumors in prepubertal children. Urology, 13: 581, 1979 2. Kay, R.: Prepubertal testicular tumor registry. J Urol, 150: 671, 1993 3. Ross, J. H., Rybicki, L. and Kay, R.: Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol, 168: 1675, 2002 4. Zdeb, M. S.: The probability of developing cancer. Am J Epidemiol, 106: 6, 1977 5. Rushton, H. G. and Belman, A. B.: Testis-sparing surgery for benign lesions of the prepubertal testis. Urol Clin North Am, 20: 27, 1993 6. Taskinen, S., Taavitsainen, M. and Wikstro¨ m, S.: Measurement of testicular volume: comparison of 3 different methods. J Urol, 155: 930, 1996 7. Paduch, D. A. and Niedzielski, J.: Repair versus observation in adolescent varicocele: a prospective study. J Urol, 158: 1128, 1997 8. Farivar-Mohseni, H., Ba¨ gli, D. J., McLorie, G., Pippi Salle, J. and Khoury, A.: Prepubertal testicular & paratesticular tumours. J Urol, suppl., 155: 392A, abstract 325, 1996 9. Walsh, C. and Rushton, H. G.: Diagnosis and management of teratomas and epidermoid cysts. Urol Clin North Am, 27: 509, 2000 10. Grady, R. W., Ross, J. H. and Kay, R.: Epidemiological features of testicular teratoma in a prepubertal population. J Urol, 158: 1191, 1997 11. Mostofi, P. K. and Price, E. B., Jr.: Tumors of the male genital system. Armed Forces Inst Pathol, 8: 28, 1970 12. Altadonna, V., Snyder, H. M., III, Rosenberg, H. K. and Duckett, J. W.: Simple cysts of the testis in children: preoperative diagnosis by ultrasound and excision with testicular preservation. J Urol, 140: 1505, 1988 13. Weissbach, L., Altwein, J. E. and Stiens, R.: Germinal testicular tumors in childhood. Report of observations and literature review. Eur Urol, 10: 73, 1984 14. Rushton, H. G., Belman, A. B., Sesterhenn, I., Patterson, K. and Mostofi, F. K.: Testicular sparing surgery for prepubertal teratoma of the testis: a clinical and pathological study. J Urol, 144: 726, 1990 15. Tokuc, R., Sakr, W., Pontes, J. E. and Haas, G. P.: Accuracy of frozen section examination of testicular tumors. Urology, 40: 512, 1992 16. Hawkins, E., Heifetz, S. A., Giller, R. and Cushing, B.: The prepubertal testis (prenatal and postnatal): its relationship to intratubular germ cell neoplasia: a combined Pediatric Oncology Group and Children’s Cancer Study Group. Hum Pathol, 28: 404, 1997 17. Renedo, D. E. and Trainer, T. D.: Intratubular germ cell neoplasia (ITGCN) with p53 and PCNA expression and adjacent mature teratoma in an infant testis. An immunohistochemical and morphologic study with a review of the literature. Am J Surg Pathol, 18: 947, 1994 18. Valla, J. S. for the Group D’Etude en Urologie Pe´ diatrique: Testis-sparing surgery for benign testicular tumors in children. J Urol, 165: 2280, 2001 19. Sugita, Y., Clarnette, T. D., Cooke-Yarborough, C. C., Chow, C. W., Waters, K. and Hutson, J. M.: Testicular and paratesticular tumours in children: 30 years’ experience. Aust N Z J Surg, 69: 505, 1999 20. Nonomura, K., Koyama, T., Kakizaki, H., Murakumo, M., Shinohara, N. and Koyanagi, T.: Testicular-sparing surgery for the prepubertal testicular tumor. Experience of two cases with large cell calcifying Sertoli cell tumors. Eur Urol, 40: 699, 2001
Vol. 171, 161–163, January 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000101185.90327.b4
EXPERIENCE WITH TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA ASEEM R. SHUKLA, CHRISTOPHER WOODARD, MICHAEL C. CARR, DALE S. HUFF, DOUGLAS A. CANNING,* STEPHEN A. ZDERIC, THOMAS F. KOLON AND HOWARD M. SNYDER, III From the Divisions of Pediatric Urology and Pathology (DSH), The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
ABSTRACT
Purpose: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery. Materials and Methods: We reviewed the pathology records at our institution for all testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We specifically examined the prepubertal incidence of teratoma, including epidermoid cysts, and our experience with testis preserving surgery. Preoperative and postoperative ultrasonography images were used to calculate the atrophy index following surgery. Patients were contacted for long-term followup. Results: Of 77 primary testicular and paratesticular tumors 38 were diagnosed in prepubertal boys (age younger than 13 years) including 11 mature teratomas and 5 epidermoid cysts. Mean patient age at treatment was 34.4 months (range 4 months to 10 years). All boys presented with a painless scrotal mass, cystic foci within an intratesticular mass on ultrasound and a normal ␣-fetoprotein level. Of the 16 boys with benign teratomas 13 (81%) were treated with a testis sparing procedure. At a mean 7-year followup no patient has presented with recurrent tumor in the ipsilateral or contralateral testicle. Postoperative physical examination and scrotal ultrasound were obtained in 9 patients at a median followup of 10.2 months, and there was no evidence of testicular atrophy or persistent discomfort. Conclusions: Unlike previously published series based on tumor registries, benign teratoma was the most common pediatric testicular tumor treated at our institution. Our single institution experience with testis preservation and long-term followup confirms the role and safety of this technique. Testis sparing surgery remains our technique of choice for testicular teratoma. KEY WORDS: testicular neoplasms, teratoma, surgery
Testicular tumors in infants and prepubertal children comprise only 1% of all pediatric solid neoplasms.1 The rarity of their presentation and limited experience with treatment outcomes at a single institution prompted the establishment of the Prepubertal Testis Tumor Registry by the American Academy of Pediatrics in 1980. The experience of this registry has confirmed clear differences in the incidence, presentation, natural history and pathological subtype of testicular tumors between the prepubertal and adult population.2, 3 The overall incidence of germ cell tumors is lower in children than in adults, malignant subtypes such as seminoma and embryonal carcinoma are absent in prepubertal patients but common in adults and teratomas, which are uniformly benign in children, may be malignant in adults.3, 4 We describe the incidence of various pediatric testicular tumor subtypes during 25 years at the Children’s Hospital of Philadelphia and the consistent differences in pathobiology compared to the adult testicular tumors. We found, as previously noted by Rushton and Belman,5 that testicular teratoma and its epidermoid cyst subtype were often clearly
distinguishable from normal adjacent parenchyma in children. The distinct appearance of the lesion and the uniformly benign outcome for testicular teratoma after orchiectomy encouraged us to implement a testis preserving approach for select patients. We present our single center experience with the incidence of testicular teratoma and definitive treatment with testis preserving surgery. MATERIALS AND METHODS
We reviewed the pathology records at our institution for all 77 testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We obtained permission from the Institutional Review Board at our hospital, and then specifically analyzed the records of only prepubertal patients (age less than 13 years) with testicular teratoma and epidermoid cysts. While all patients presenting with teratoma or epidermoid cyst underwent radical orchiectomy before 1990, a testis preserving surgery was selectively performed in subsequently treated patients. We recorded age at presentation, preoperative and postoperative ultrasonography images, and individual treatment outcomes for boys who underwent testis preserving surgery. Those patients were contacted to affirm recurrence-free status and for subjective evidence of testicular atrophy. The cosmetic, sonographic and recurrence-free outcome was evaluated with
Accepted for publication August 1, 2003. Study received Institutional Review Board approval. * Correspondence: Division of Pediatric Urology, Children’s Hospital of Philadelphia, 3rd Floor Wood Building, Children’s Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, Pennsylvania 19104-4399 (telephone: 215-590-2769; FAX: 215-5903985). 161
162
TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA
a physical examination and followup ultrasonography. Testicular size was calculated in cc by the Lambert formula as (length ⫻ width ⫻ breadth) ⫻ 0.71.6 An atrophy index for the enucleated testis was calculated after modification of the formula commonly used for assessing differential testicular growth in boys with varicoceles: (contralateral testicular volume—affected testicular volume)/(contralateral testicular volume) ⫻ 100, expressed as a percent.7 The testis preserving procedure consisted of an inguinal incision and testis mobilization with the cord compressed at the internal ring. The surgical field was then carefully draped with towels, the tunica vaginalis was incised and the testicle was exposed. If the tumor appeared consistent with teratoma with an encapsulated, cystic and peripherally located lesion, occlusion on the spermatic cord was removed to maintain optimal vascular flow to the affected testicle. The tumor was excised using the edge of a scalpel to enucleate it from the bordering normal parenchyma. If an infiltrative lesion was noted, the child was peripubertal or postpubertal, or the location and size of the mass precluded a testis sparing approach, then radical orchiectomy was performed. RESULTS
A total of 77 primary testicular and paratesticular tumors were encountered in males younger than 18 years at our institution between 1976 and November 2002. The tumor types and frequency of presentation are shown on table 1. Of the 43 germ cell tumors 22 (51.5%) were teratomas (16 mature teratomas, 1 immature teratoma and 5 epidermoid cysts) and 10 (23.2%) were yolk sac tumors. A total of 38 boys were prepubertal at the time of presentation, and pathology and distribution of germ cell tumors are shown in table 2. We identified mature teratomas and epidermoid cysts in 11 and 5 boys, respectively, with a mean age of 34.5 months (range 4 months to 10 years). All of these prepubertal patients presented with a painless scrotal mass and ␣-fetoprotein (AFP) levels that were normal for age. Ultrasound was available for 14 (87.5%) patients preoperatively and demonstrated a complex, welldemarcated hypoechoic lesion distinct from adjacent normal appearing testicular parenchyma. A testis preserving procedure was used to treat 13 of 14 prepubertal boys who presented with a teratoma or epidermoid cyst after 1990. Radical orchiectomy was performed for 1 prepubertal boy who presented with a large teratoma in the testis mediastinum that precluded a testis sparing approach. Of the 13 tumors removed by testis preserving surgery 8 were mature teratomas and 5 were epidermoid cysts. Frozen section analysis was requested following exposure of the mass in 10 cases (77%). Final diagnosis correlated with the frozen section report in all cases. One patient had an enlarging hematoma in the immediate postoperative period and was returned to surgery for hemostasis. At a mean 88 months following surgery (range 5 months to 22
TABLE 1. Classification of 77 pediatric testicular tumors Histology Germ cell tumors: Teratoma (including epidermoid cysts) Yolk sac tumor Embryonal Ca Mixed malignant germ cell tumor Monophasic chorioca Seminoma Ca in situ Not otherwise specified Stromal tumors Paratesticular tumors Primary lymphomas Gonadoblastomas Miscellaneous (regressed tumor, cystic dysplasia)
Total No. (%) 43 (57) 22 (30) 10 (13) 2 (2.5) 4 (5) 1 (1) 2 (3) 1 (1) 1 (1) 9 (12) 8 (10) 5 (7) 1 (1) 11 (14)
TABLE 2. Frequency of prepubertal germ cell tumors and treatment performed Germ Cell Tumor Type
Testis Sparing Procedure After 1990
Orchiectomy
Total No. (% all prepubertal tumors)
Yolk sac tumor Mature teratoma Epidermoid cyst
0 8 5
8 3 0
8 (21) 11 (29) 5 (13)
years) no patient presented with recurrent tumor in the ipsilateral or contralateral testicle. A total of 10 (77%) patients treated with testis sparing surgery were successfully contacted for longterm followup at a median of 72 months (mean 65, range 15 to 120). All patients reported that they were free of recurrence, testicular atrophy or persistent orchalgia. No patient reported dissatisfaction with the cosmetic appearance or any abnormalities on palpation. Patient report was confirmed by a physician administered physical examination in 4 of these 10 patients at a mean of 38.8 months (range 18 to 60). Two of these 4 patients were prepubertal in age and development at the time of the last followup physical examination. Postoperative testicular examination and scrotal ultrasound were obtained for 9 (69%) patients who underwent testis preserving surgery at a mean of 10.2 months following surgery (range 5 to 60). There was no evidence of testicular dysmorphia, although the affected testicle was smaller with a mean atrophy index for 7 patients of 10.3%. Affected testicles in the 2 remaining patients sustained a mean 35% increase in volume compared to the normal contralateral testicle. Imaging of the entire testicle confirmed normal appearing parenchyma with no evidence of previous surgery or of recurrent lesions. DISCUSSION
Teratoma was the most commonly encountered pediatric testicular germ cell tumor at our institution comprising more than 50% of all tumor types. Among prepubertal boys teratomas were 50% more frequent than yolk sac tumors. This finding differs substantively with that of the Prepubertal Testicular Tumor Registry. The tumor registry compiled 244 cases of yolk sac tumor (64% of all germ cell tumors) compared to 92 (23%) teratomas concurring with the traditional view that yolk sac tumor is the most common germ cell tumor of the testicle in the prepubertal male.3 We, however, agree with others that teratoma is more likely the most common prepubertal testicular tumor, and discrepancy with the registry is due to underrepresentation of benign tumors.3, 8, 9 Testicular teratoma is a uniformly benign entity in prepubertal children with a clear distinction from the adult population in which malignancy may be present.1–3, 5, 10, 11 The benign course of the tumor has encouraged implementation of a testis preserving approach to treatment. While Altadonna et al reported testis preserving surgery as a definitive treatment option for excision of benign testicular cysts,12 Weissbach first applied this approach to 2 patients with teratoma.13 Rushton et al provided extensive pathological analysis of 5 teratoma specimens removed by testis sparing procedure to ascertain the intrinsic safety of this approach.14 The authors found that testicular parenchyma adjacent to the enucleated tumor was normal along with no evidence of tumor multifocality in distant testicular tissue. This report further corroborated our approach to testis preservation, which became our treatment of choice for suspected teratomas and epidermoid cysts in prepubertal children after 1990. Testis preserving surgery may be rendered impossible, however, as it was in 1 of our patients, if the tumor burden is exceedingly large and centered near the mediastinum of the testicle. Initially, we obtained a frozen section analysis following enucleation of the lesion to confirm teratoma histology for all
TESTIS SPARING SURGERY FOR TESTICULAR TERATOMA
patients undergoing testis preserving surgery. As our experience progressed, however, we increased our reliance on the preoperative AFP level, physical examination, and initial sonographic and intraoperative visual appearance of the lesion to dictate the need for frozen sections. When frozen section was performed, we found, as have others, that final pathology was in concordance with frozen section in all cases.14, 15 For our 3 cases that did not have a frozen section report, final pathology confirmed a benign lesion. In our experience cystic lesions of the testicle are always benign (benign simple cysts, epidermoid or dermoid cysts, or teratomas) and a frozen section analysis may be safely omitted when confronted with a predominantly cystic testicular tumor in a prepubertal child. We do not routinely excise testicular tissue adjacent to the lesion for biopsy due to the negligible risk for tumor multifocality.14 –16 A report of the finding of intratubular germ cell neoplasia adjacent to a mature testis teratoma in a 3-year-old boy raises the question of the need to biopsy the adjacent testicular tissue in these patients.17 However, the clinical implication of carcinoma in situ in the prepubertal testis is unclear. The Pediatric Oncology Group study of prepubertal testis and its relationship to intratubular germ cell neoplasia found that germ cells adjacent to infantile germ cell tumors are commonly proliferative but not neoplastic.16 Following testis preserving surgery, the remaining testicular parenchyma appears to be largely unaffected by surgical manipulation. We calculated an atrophy index when postoperative sonography was available in 9 patients to calibrate the differential testicular size following surgery. A mean atrophy index of 10.2% in 7 patients affirmed our clinical suspicion that no substantial size alterations were distinguishable postoperatively in most cases. We believe that the large testicular tumor likely compresses the normal testicular parenchyma but when the benign tumor is shelled out, the compressed parenchyma usually regains a near normal testicular size. We did find an actual increased volume in the postoperative testis compared to the unaffected contralateral testicle in 2 cases. Both of these patients underwent followup sonography at 6 months and we speculate that the short interval may reflect ongoing postoperative changes. Further imaging and physical examinations following a longer interval may reveal eventual resolution of size discrepancy in these cases. After a median of 6 years following testis preserving surgery, there have been no tumor recurrences of teratoma or epidermoid cysts since 1976. Our single institution experience with 13 cases concurs with the recent report by Valla on the results of testis sparing surgery at 19 institutions.18 A total of 16 teratomas and 13 epidermoid cysts were treated by 22 surgeons in that study with no recurrences, testicular atrophy or discordant frozen section final pathology cases at a mean followup of 56 months. The safety of testis sparing surgery for prepubertal children with mature teratoma or epidermoid cysts has now been confirmed by our experience and that of others, and should be considered the primary treatment of choice.3, 9, 18, 19 Ross et al have suggested an algorithm in which testis sparing surgery is recommended for prepubertal children with a normal serum AFP level, ultrasound with adequate normal testis parenchyma and after exclusion of yolk sac tumor by frozen section.3 Physiologically normal increases in serum AFP are expected for infants, and the authors suggest excluding from testis sparing surgery those patients between 6 and 12 months old with AFP greater than 100 ng./ml. Indeed, the pure histological nature of almost all prepubertal germ cell tumors has encouraged application of testis sparing surgery to all prepubertal benign tumors, including teratomas, epidermoid cysts, Sertoli cell, Leydig cell and juvenile granulosa cell tumors, with excellent results.3, 18 –20 While our experience with other benign testicular tumors has been limited, we suggest that our experience broadly supports this approach.
163
CONCLUSIONS
Testicular teratoma was the most common pediatric testicular tumor treated at our institution, contrary to previously published series based on tumor registries. Under-reporting of benign tumors by most institutions may explain this discrepancy. Our single institution experience with testis sparing surgery and long-term followup confirms the role and safety of this technique. Testis preserving surgery remains our technique of choice for the treatment of testicular teratoma, including epidermoid cysts.
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