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ORIGINAL ARTICLE
Year : 2010  |  Volume : 47  |  Issue : 3  |  Page : 292-295
 

Prepubertal testicular tumors: Our 10 years experience


1 Department of Pediatric Urology, KLES Kidney Foundation, Belgaum, India
2 Department of Pediatric Surgery, KLE University's JN Medical College & KLES Dr. Prabhakar Kore Hospital, & MRC, Belgaum, India

Date of Web Publication28-Jun-2010

Correspondence Address:
R B Nerli
Department of Pediatric Urology, KLES Kidney Foundation, Belgaum
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.64730

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 » Abstract 

Background: Testicular tumors in the pediatric population are distinct from those of the adults. In contrast to the prevalence data reported in tumor registries, several studies have shown that a majority of the prepubertal testis tumors are benign. We retrospectively analyzed a series of prepubertal testicular tumors. Materials and Methods: A retrospective review of all testicular tumors at our institution was done from Jan 1999 to Dec 2008. Data relating to presentation, evaluation, and management were collected. Results: A total of 22 children with prepubertal testicular tumors were identified. The mean age at presentation was 4.6 years. Mature teratoma, epidermoid cysts, immature teratoma, and yolk sac tumor accounted for 49.94%, 13.62%, 9.08%, and 18.16%, respectively. All surgeries were successful with respect to cancer control and testicular preservation. Conclusions: Benign tumors formed the majority (72.64%) of the tumors that were encountered, with yolk sac tumors (18.16%) being a minority. Testicular preserving surgery appears to be a feasible option for benign tumors and is safe and efficacious in long-term follow-up.


Keywords: Surgery, testicular neoplasm, testis


How to cite this article:
Nerli R B, Ajay G, Shivangouda P, Pravin P, Reddy M, Pujar V C. Prepubertal testicular tumors: Our 10 years experience. Indian J Cancer 2010;47:292-5

How to cite this URL:
Nerli R B, Ajay G, Shivangouda P, Pravin P, Reddy M, Pujar V C. Prepubertal testicular tumors: Our 10 years experience. Indian J Cancer [serial online] 2010 [cited 2019 Jul 17];47:292-5. Available from: http://www.indianjcancer.com/text.asp?2010/47/3/292/64730



 » Introduction Top


Until recently, little data was available regarding the natural history and appropriate management of prepubertal testicular tumors. Prepubertal tumors were managed in much the same way as testicular tumors in adults, with radical orchiectomy and retroperitoneal lymph node dissection playing major roles. The incidence of testicular tumors in children is only 0.5-2.0 per 100,000. [1] The age distribution of testicular tumors suggests that pediatric and adult tumors are distinct. Rather than one large age distribution curve with children at the extreme left end, there is a bimodal age distribution of testicular tumors, with a large peak for young adults and a much smaller, but distinct, peak for the first 3 years of life. [2]

Benign lesions represent a greater percentage of cases in children than in adults. The American Academy of Pediatrics Prepubertal Testis Tumor Registry reported that benign tumors accounted for 38% of cases. [3] Large series from single institutions report a higher percentage of benign lesions, thus suggesting that these entities were underreported to the Tumor Registry. [4],[5] A recent multicenter report found that 74% of primary testis tumors in prepubertal children were benign. [6]

We retrospectively analyzed a series of testicular tumors in children presenting to us in the last 10 years.


 » Materials and Methods Top


With permission obtained from the Institutional Ethical Committee, we reviewed the case records of all children less than 13 years of age, diagnosed with testicular tumors between Jan 1999 and Dec 2008. We recorded age at presentation, pre- and postoperative images, and individual treatment outcomes for all the children. The tumor review criteria included presentation, radiologic investigations, and tumor markers. The outcome data included histopathology, evidence of metastases, treatment modalities, and outcomes. These children/parents/guardians were contacted to reaffirm recurrence-free status. All the children who underwent testes preserving surgery were evaluated for subjective evidence of testicular atrophy. The cosmetic, sonographic, and recurrence-free outcome was evaluated with a physical examination and follow-up ultrasonography. Testicular size was calculated in cubic centimeters by the Lambert formula as (length Χ width Χ breadth) Χ 0.71. [5] 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) Χ 100, expressed as a percentage. [5]


 » Results Top


A total of 22 patients met the inclusion criteria. The mean age of the children presenting with testicular tumor was 4.6 years (range 1-11 years). The presenting symptom was a painless testicular mass gradually increasing in size. The tumor types and frequency of presentation are shown in [Table 1]. Among the 22 children with prepubertal testicular tumors, 11 (49.94%) had mature teratomas, 3 (13.62%) epidermoid cysts, 2 (9.08%) immature teratoma, 4 (18.16%) had yolk sac tumors, and the remaining 2 (9.08%) had other tumors.

Alpha-fetoprotein (AFP) was elevated in all the 4 (18.16%) children with yolk sac tumors, and the levels were normal for age in the rest. The children with yolk sac tumors underwent radical inguinal orchiectomy. The clinical stage was I in all the patients, and all these 4 children were under close follow-up ranging from 18 to 54 months. None of these children have had any recurrence in the retroperitoneum or in the lungs. None of these children had any adjuvant therapy.

Overall 16 (72.64%) of the tumors were benign on histopathologic examination [Figure 1]. In 7 of the 16 benign tumors, inguinal radical orchiectomy was done as the frozen section was not diagnostic. Nine of the 16 children with benign lesions underwent testicular preserving surgery. Seven had mature teratomas and the remaining 2 had epidermoid cysts. All these 9 children were successfully contacted on a follow-up at a median of 47 months. All these children were free of recurrence, testicular atrophy, or persistent orchalgia. None of these children reported dissatisfaction with the cosmetic appearance or any abnormalities on palpation. This was confirmed on physical examination in all these 9 children. Scrotal ultrasonography was done in all of them. There was no evidence of testicular dysmorphia, although the affected testicle was smaller with a mean atrophy index of 15%. Imaging of the entire testicle confirmed normal appearing parenchyma with no evidence of previous surgery or recurrent lesions [Figure 2].


 » Discussion Top


Prepubertal testicular tumors are distinct from those of adults in histologic characteristics, molecular biology, and clinical behavior. Although germ cell tumors are far more common than stromal tumors in both the age groups, the vast majority of adult tumors are malignant with histologic features of either seminoma or mixed germ cells. In contrast, the most common histologic features for prepubertal tumors are pure yolk sac and teratoma. In the Prepubertal Testis Tumor Registry, it was noted that malignancies appeared to be less common in children than in adults. The most common tumor reported in the registry was pure yolk sac tumor, which accounted for 62% of the reported neoplasms. [3] Benign tumors, primarily teratomas, made up nearly all the remainder. The registry data seemed to be inherently biased toward malignancies and unusual tumors. Pohl et al[6] studied the histopathologic features of all pediatric testicular tumors removed at several major referral centers. They found that the incidence of malignant tumors in children was even less than that described in the Prepubertal Testis Tumor Registry. Teratoma accounted for 48% of the tumors, with yolk sac tumors accounting for only 15%. Overall, 74% of the tumors in that series were benign.

Molecular studies have given additional evidence of the distinct nature of prepubertal testicular tumors vis-a-vis those of adults. A chromosomal gain in the short arm of chromosome 12p has been found in nearly all malignant adult germ cell tumors. In contrast, prepubertal yolk sac tumors show no changes at chromosome 12p but are characterized by other changes on chromosomes 1, 6, and 20. [7] Similarly, although postpubertal teratomas might show complex cytogenetic aberrations, pure teratomas in childhood fail to show any genetic changes detectable with conventional chromosomal analysis or chromosomal and array-based hybridization studies, consistent with their universally benign behavior in children. [7] Carcinoma in situ or intratubular germ cell neoplasia (ITGCN) is a common finding in the ipsilateral and contralateral testes of men with malignant germ cell tumors. However, ITGCN does not appear to occur in testes harboring prepubertal yolk sac tumors. [8]

A painless testicular mass is the most common finding in a child with a testicular tumor. Disorders that must be excluded are epididymitis, hydrocele, hernia, and spermatic cord torsion. The latter might manifest as a painless mass in a neonate with little scrotal wall inflammation if the event occurred prenatally. Acute abdominal pain could be the initial symptom with torsion of an abdominal undescended testicle containing a tumor. Some children with hormonally active tumors may have small intratesticular lesions that are not palpable on physical examination. Testicular ultrasound is routinely performed for the evaluation of testicular masses in children. Color Doppler ultrasound has been reported to be more effective than gray-scale ultrasound in detecting intratesticular neoplasms in the pediatric population. [9] There are no sonographic features that can reliably distinguish benign and malignant tumors, but the finding of anechoic cystic lesions can suggest a benign lesion, such as a simple cyst, cystic dysplasia, teratoma, or cystic granulosa cell tumor. The ultrasound findings of epidermoid cyst are also unique. There is a heterogenous intratesticular mass with concentric rings of alternating hypoechoic and hyperechoic layers that give rise to an "onion-skin" appearance. This corresponds to the multiple layers of keratin debris within the lesion. The mass is surrounded by a hypoechoic or echogenic rim and the absence of flow on color Doppler sonography. [10] Ultrasound can also assess whether there is enough normal testis parenchyma remaining to warrant salvage. If a child is identified with a cystic lesion on ultrasound and the serum AFP level is normal, the surgeon can plan for a testis-sparing procedure. [4],[5],[11],[12],[13] Magnetic resonance imaging may detect small, functioning Leydig cell tumors that are not evident on ultrasound. [14]

Testicular tumor markers are important tools in the evaluation of testicular tumors in children. Because yolk sac tumor is the only important malignant germ cell tumor in children, human chorionic gonadotropin is not a helpful marker. In contrast, AFP is very important because it is elaborated by 90% of yolk sac tumors in children. An important caveat in the use of AFP for these patients is that the serum AFP level is normally very high in infancy. It measures in the tens of thousands in newborns and does not decrease to normal adult levels until nearly 1 year of age. [15] Therefore, although elevated AFP in a child older than 1 year with a testicular tumor almost always reflects the presence of a yolk sac tumor, an "elevated" level in infants can also occur in the case of benign tumors.

In children, increasing consideration has been given to performing testis-sparing surgery for benign testicular tumors. [3] This is particularly attractive in prepubertal children because many, if not most, tumors are benign in this population. The preoperative evaluation plays a significant role in patient selection for testis-sparing surgery. An elevated AFP level in a child older than 1 year, virtually always reflects the presence of a yolk sac tumor and precludes a testis-sparing approach. However, in all infants, and in older children with a normal AFP level, the likelihood of a benign tumor is considerable. For children, inguinal exploration should be considered so that testis-sparing surgery can be performed if a benign histologic type is confirmed. If a malignancy is detected, or frozen section analysis is nondiagnostic, orchiectomy should be performed.

The appropriate adjuvant therapy for children who have a malignant tumor differs from that for adults. Prepubertal patients are less likely than adults to have metastases limited to the retroperitoneum. Furthermore, 80% of prepubertal patients have clinical stage I disease and 20% will develop a recurrence with no therapy other than orchiectomy. [16] Given these facts, retroperitoneal surgery in children has been reserved for biopsy of radiographically equivocal nodes or for resection of a persistent retroperitoneal mass after chemotherapy.

In the absence of an age-adjusted elevation in the AFP levels, the initial surgical approach for pediatric testicular tumors should be inguinal exploration with excisional biopsy and frozen section analysis. Most of these patients will have a benign tumor and will require no additional evaluation or treatment. Those children with the most common malignancy-yolk sac tumor-usually have stage I disease that can be managed with observation. Children with metastatic disease and those children with stage I disease that progresses during observation can almost always be cured with platinum-based multiagent chemotherapy. The outlook for prepubertal patients with testicular tumors is even better than that for adults, and the emphasis in recent years has been on reducing the morbidity of both the surgical and the adjuvant therapies for these children. [17]


 » Conclusions Top


Prepubertal testicular tumors are distinct from those of adults in histologic characteristics, molecular biology, and clinical behavior. Benign lesions represent the majority of primary testis tumors (72.64%) with the most common histologic type being mature teratoma. Testicular preservation is possible in most cases of benign mature teratomas, and long-term follow-up confirms the safety of this technique.

 
 » References Top

1.Coppes M, Rackley R, Kay R. Primary testicular and paratesticular tumors of childhood. Med Pediatr Oncol 1994;22:329-40.  Back to cited text no. 1      
2.Schneider DT, Calaminus G, Koch S, Teske C, Schmidt P, Haas RJ, et al. Epidemiologic analysis of 1,442 children and adolescents registered in the German germ cell tumor protocols. Pediatr Blood Cancer. 2004;42:169-75.  Back to cited text no. 2      
3.Ross JH, Rybicki L, Kay R. Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: A summary of the prepubertal Testis Tumor Registry. J Urol 2002;168:1675-8.   Back to cited text no. 3  [PUBMED]    
4.Metcalfe PD, Farivar-Mohseni H, Farhat W, McLorie G, Khoury A, Bagli DJ. Pediatric testicular tumors: Contemporary incidence and efficacy of testicular preserving surgery. J Urol 2003;170:2412-5.  Back to cited text no. 4      
5.Shukla AR, Woodard C, Carr MC, Huff DS, Canning DA, Zderic SA, et al. Experience with testis sparing surgery for testicular teratoma. J Urol 2004;171:161-3.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]  
6.Pohl HG, Shukla AR, Metcalfe PD, Cilento BG, Retik AB, Bagli DJ, et al. Prepubertal testis tumors: Actual prevalence rate of histological types. J Urol 2004;172:2370-2.  Back to cited text no. 6      
7.Harms D, Zahn S, Gobel U, Schneider DT. Pathology and molecular biology of teratomas in childhood and adolescence. Klin Padiatr 2006;218:296-302.  Back to cited text no. 7      
8.Hawkins E, Heifetz SA, Giller R, 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 1997;28:404-10.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]  
9.Luker GD, Siegel MJ. Pediatric testicular tumors: Evaluation with gray-scale and color Doppler US. Radiology 1994;191:561-4.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]  
10.Langer JE, Ramchandani P, Siegelman ES, Banner MP. Epidermoid cysts of the testicle: Sonographic and MR imaging features. AJR Am J Roentgenol 1999;173:1295-9.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]  
11.Rushton HG, Belman AB, Sesterhenn I, Patterson K, Mostofi FK. Testicular sparing surgery for prepubertal teratoma of the testis: A clinical and pathological study. J Urol 1990;144:726-30.  Back to cited text no. 11  [PUBMED]    
12.Grunert RT, Van Every MJ, Uehling DT. Bilateral epidermoid cysts of the testicle. J Urol 1992;147:1599-601.  Back to cited text no. 12  [PUBMED]    
13.Valla JS for the Group D'Etude en Urologie Pediatrique. Testis-sparing surgery for benign testicular tumors in children. J Urol 2001;165:2280-3.   Back to cited text no. 13      
14.Kaufman E, Akiya F, Foucar E. Viralization due to Leydig cell tumor diagnosis by magnetic resonance imaging: Case management report. Clin Pediatr (Phila) 1990;29:414-7.   Back to cited text no. 14      
15.Wu JT, Book L, Sudar K. Serum alpha fetoprotein (AFP) levels in normal infants. Pediatr Res 1981;15:50-2.  Back to cited text no. 15  [PUBMED]    
16.Schlatter M, Rescorla F, Giller R, Cushing B, Vinocur C, Colombani P, et al. Excellent outcome in patients with stage I germ cell tumors of the testes: A study of the Children's cancer group/Pediatric oncology group. J Pediatr Surg 2003;38:319-24.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]  
17.Ross JH. Prepubertal testicular tumors. Urology 2009;74:94-9.  Back to cited text no. 17  [PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]

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