15th December 2017, Volume 130 Number 1467

Timothy Little, Shareena Lala, Vipul Upadhyay

Cribb et al1 published a series of paediatric ovarian masses at our institution in 2014, finding a relatively large number of patients vis-à-vis international comparisons. We now analyse our experience of paediatric testicular tumours to ascertain if the prevalence is similarly higher than that of other published series and to compare the patterns of presentation, the types of tumour found and how they are managed.

The total male New Zealand population for the age range studied (0<16 years of age) grew steadily over the study period from 479,470 boys (2000) to 498,650 boys (2011). Estimates by region were only available for age 0<15 years. The catchment area of our institution grew from 223,700 boys (2000) to 239,800 (2011)—source: statistics New Zealand (stats.govt.nz).

Several series of paediatric testicular tumours have been published from major centres around the world,2–14 all demonstrating testicular tumours to be less common than ovarian tumours. Single-centre figures range from a mean of 1.7 to 6.3 cases per year and a reported incidence of 0.5–2/100,000 children. The series differ between including all tumours or just germ cell tumours.

Methods

The hospital’s pathology database was searched for all patients 0–15 years old with testis, testicle, testicular remnant and streak gonad submitted for analysis during a 12-year period (January 2000–December 2011). Only testicular tumours were then included and clinical records of these cases reviewed in detail with relevant aspects of each recorded in an Excel® spreadsheet. Demographic details were recorded, as well as presenting complaint, alpha-fetoprotein (aFP), beta human chorionic gonadotropin (bhCG) and lactate dehydrogenase (LDH), operation type and findings, histological analysis and follow-up.

Other world series were identified using MEDLINE and EMBASE.

Results

There were 33 children with testicular tumours, including five paratesticular tumours. There was no pattern to the ethnicity of children with particular tumours; the commonest two groups were New Zealand European and Māori.

Presentation

By far the commonest presentation (26 of 33) of these cases was of a unilateral intrascrotal swelling. In one case the swelling was thought to be acute (teratoma in a seven-month-old) and in another the increase in size was noted to be rapid, occurring over the few days prior to an acute presentation (yolk sac tumour in another infant). One 14-year-old presented with haemoptysis. It was only when metastases were seen on a plain chest film that the patient was further examined and investigated to find the testicular yolk sac tumour. Finally, the two patients with Leydig cell tumours presented with precocious puberty, in keeping with the diagnosis.

Histopathology

The breakdown of this 33 according to histological appearances is illustrated in Table 1. Of the 28 testicular tumours, 15 were malignant and 13 benign. The commonest tumour was mature teratoma, making up approximately a third (n=10) of the tumours over the study period. As expected, median aFP was higher in malignant tumours than in benign tumours (4,000 and 1.8 units respectively).

Table 1:Testicular and paratesticular tumours.

Tumour

Subtype

Number

%

aFP

(IU/ml)

bhCG

(mIU/ml)

LDH

(IU/L)

Germ cell tumours

Mature teratoma

10

36

3

NA

289.5

Immature teratoma

1

4

44.1

NA

NA

Yolk sac tumour

6

21

9,453

1,165

NA

Embryonal carcinoma

1

4

1

3

NA

Mixed embryonal carcinoma/teratoma

1

4

8,087

1,165

NA

Seminoma

1

4

NA

NA

NA

Sex cord stromal tumours

Granulosa cell tumour

3

11

4,064.5

NA

NA

Leydig cell tumour

2

7

0.85

NA

NA

Others

Lymphoma

2

7

NA

NA

NA

Lymphoblastic leukaemia

1

4

NA

NA

NA

Paratesticular tumours

Lipoblastoma

1

20

2.5

NA

262

Rhabdomyosarcoma

4

80

1.4

NA

307 

Table 2: Comparison with international experience.

Centre

Max age

Length of study (years)

No. of cases

Cases per year

Auckland, New Zealand

16

12

33

2.75

Melbourne, Australia (Sugita et al2)

18

30

68

2.27

Turkey (Cifti et al3)

17

30

51

1.7

France—multiple (Valla et al4)

15

15

273

n/a

USA—multiple (Ross et al5)

12

Not stated

395

n/a

Toronto, Canada (Metcalfe et al6)

18

18

51

2.83

Washington DC, Philadelphia, Boston, Toronto, USA/Canada (Pohl et al7)

12

Various

98

n/a

USA—multiple: SEER program (Walsh et al8)*

14

28

131

n/a

Leipzig, Germany (Troebs et al9)

18

25

24

0.96

Taipei, Taiwan (Chen et al10)*

12

25

34

1.36

Helsinki, Finland (Taskinen et al11)

18

25

34

1.36

Belgaum, India (Nerli et al12)

12

10

22

2.2

Hangzhou, China (Wang et al13)

13

10

63

6.3

Seoul, Korea (Baik et al14)

15

25

48

1.92

*germ cell tumours only. 

Follow-up

There were three deaths among the cohort. The first was a six-year-old patient with lymphoma who died four years after presentation and treatment with chemotherapy and palliative radiotherapy, having previously been treated for acute lymphoblastic leukaemia. The second died aged 17 years with lymphoblastic leukaemia, having presented six years prior, undergoing orchidectomy, chemotherapy and radiotherapy. The third was a 12-year-old with rhabdomyosarcoma who had presented a year prior and had undergone orchidectomy and radiotherapy.

The follow-up of the other patients was highly variable. However, in general, malignancies were followed by oncologists rather than surgeons and for longer than benign tumours (median follow-up five years and two years respectively).

International comparisons

We found 13 case series, including benign and malignant tumours. It must be noted that two series8,10 include only germ cell tumours. The age range also varies between studies, some considering paediatric to include up to the age of 18 years whereas others only up to 12 years so as to describe the series as pre-pubertal. The most common tumour varied between mature teratoma (as in our series) and yolk sac tumour.

Discussion

Our results show that the numbers of patients with testicular patients is similar to those published by other centres worldwide. The most common subtype was mature teratoma, also in line with other published series. During the work-up of patients presenting with a unilateral testicular mass, we found any elevation in tumour markers to be—as expected—predictive of a malignant lesion. Pre-operative aFP must, however, be interpreted with caution, both for the fact that elevated levels are physiological in the neonatal period and that yolk sac tumours do not always secrete aFP. Nevertheless, elevation in those aged older than six months warrants suspicion of malignancy.

Ultrasound has been used to assist in pre-operative planning, with some characteristics suggestive of a benign tumour, namely the findings of a well-circumscribed lesion, anechoic with a unilocular process,16 indeed one series found pre-operative ultrasound discriminated between benign and malignant tumours correctly in all cases.17 It is also helpful in indicating the volume and position of normal parenchyma remaining, thereby demonstrating the feasibility of testis-sparing surgery.

None of the patients in our series underwent testis-sparing surgery, but worldwide at least 10 centres advocate this approach where possible.2–12 The possibility of testis-sparing surgery was first raised in 1984 by Weissbach and colleagues,15 and Valla’s group in France4 advised on specific pre-operative and intra-operative criteria.

The disadvantages of testis-sparing surgery may be overstated. One argument is that the remaining tissue is not worth preserving and may even atrophy, but Shukla et al18 report no post-operative size alterations in their experience, and another group19 even reported compensatory growth of the remaining tissue.

Pain is not a significant feature reported in any of the series, even those with long-term follow-up. Perhaps more important than any other considerations is the fact that there were no recurrences or malignant transformations of incompletely resected tissue reported.

Of course, in practical terms, the practice relies on frozen section and therefore a well-resourced laboratory and local histopathological expertise in testicular tumours. Frozen section for testicular biopsies is, at least, highly accurate, with the literature reporting complete ability to discriminate malignant from benign pathology on frozen section.20, 21

In summary, Starship Children’s Hospital in Auckland treats children with testicular tumours as frequently as other major centres worldwide. It is useful to know that our most common lesion was mature teratoma. Many centres are moving to testis-sparing surgery and suitable candidates could be identified using tumour markers, pre-operative ultrasound and intra-operative frozen section.

Summary

Testicular tumours in children are rare. Our experience in managing such tumours is similar to that published by other major centres for paediatric surgery across the world. There may be scope to develop the practice of testicle-sparing surgery (ie, not removing the whole testicle when there is a tumour), but this has not been our practice thus far. Overall, survival rates are excellent.

Abstract

Aim

This is a 12-year, single-centre retrospective review of paediatric testicular tumours and review of the world literature on paediatric testicular tumours. The aim was to identify presenting features, the range of pathology and management of such tumours in comparison with other published series.

Method

The hospital’s pathology database was searched for all testicular and paratesticular tissue submitted for patients younger than 16 years of age during the 12-year study period January 2000 to December 2011, and patients with testicular tumours identified. A detailed review of clinical records was then completed and summary statistics calculated.

Results

There were 33 tumours and 22 (66.7%) were malignant. The most common tumour was mature teratoma. No tumours presented with a twisted gonad. The mean incidence per year was 2.75 cases. This is comparable to other reported series worldwide (median 1.92, range 1.7–6.3).

Conclusion

Testicular tumours in children are rare. In our centre, mature teratoma was the most common tumour, and malignant testicular tumours did not present with torsion. Our experience in managing gonadal tumours is similar to that published by major centres for paediatric surgery across the world. There is scope to develop the practice of testis-sparing surgery.

Author Information

Timothy Little, Paediatric Surgical Registrar, Department of Paediatrics and Child Health, Wellington Regional Hospital, Wellington; Shareena Lala, Paediatric Surgical Registrar, Department of Paediatric Surgery, Starship Children’s Hospital, Auckland; Vipul Upadhyay, Paediatric Surgeon and Paediatric Urologist, Department of Paediatric Surgery, Starship Children’s Hospital, Auckland.

Acknowledgements

Mike Watson (Consultant Paediatric Histopathologist) and Daniel Wong from LabPLUS for their generosity in providing assistance with the database search. Steve Evans (Consultant Paediatric Surgeon) for reviewing the manuscript.

Correspondence

Tim Little, Department of Paediatrics & Child Health, Wellington Regional Hospital, Riddiford Street, Newtown, Wellington 6021.

Correspondence Email

mrtlittle@gmail.com

Competing Interests

Nil.

References

  1. Cribb B, Vishwanath N, Upadhyay V. Paediatric ovarian lesions-the experience at Starship Children’s Hospital, New Zealand. NZMJ. 2014; 127:41–51.
  2. Sugita Y, Clarnette TD, Cooke-Yarborough C. Testicular and paratesticular tumours in children: 30 years’ experience. ANZ J Surg. 1999; 69:505–508.
  3. Cifti AO, Bingol-Kologlu M, Senocak ME. Testicular tumors in children. J Pediatr Surg. 2001; 36(12):1796–1801.
  4. Valla JS, et al. Testis-sparing surgery for benign testicular tumors in children. J Urol. 2001; 165:2280–2283.
  5. Ross JH, Rybicki L, Kay R. Clinical behaviour and a contemporary management algorithm for prepubertal testis tumors: a summary of the prepubertal testis tumor registry. J Urol. 2002; 168:1675–1679.
  6. Metcalfe PD, Farivar-Mohseni H, Farhat W, et al. Pediatric testicular tumors: contemporary incidence and efficacy of testicular preserving surgery. J Urol. 2003; 170:2412–2416.
  7. Pohl HG, Shukla AR, Metcalf PD, et al. Prepubertal testis tumors: actual prevalence rate of histological types. J Urol. 2004; 172:2370–2372.
  8. Walsh TJ, Grady RW, Porter MP, et al. Incidence of testicular germ cell cancers in US children: SEER program experience 1973–2000. Urology. 2006; 68:402–405.
  9. Troebs RB, Krauss M, Geyer C, et al. Surgery in infants and children with testicular and paratesticular tumours: a single centre experience over a 25-year period. Klin Pediatr. 2007; 219(3):146–51.
  10. Chen Y-S, Kuo J-Y, Chin T-W et al. Prepubertal testicular germ cell tumors: 25 year experience in Taipei Veterans General Hospital. J Chin Med Assoc. 2008; 71(7):357–361.
  11. Taskinen S, Fagerholm R, Aronniemi J, Rintala R, Taskinen M. Testicular tumors in children and adolescents. J Pediatr Urol. 2008;4(2):134-7
  12. Nerli RB, Ajay G, Shivangouda P, Pravin P, Reddy M, Pujar VC. Prepubertal testicular tumors: our 10 years experience. Indian J Cancer. 2010; 47(3):292–5. doi: 10.4103/0019-509X.64730.
  13. Wang X, Xu S, Tang D, Li M, Wu D, Huang Y. Prepubertal testicular and paratesticular tumors in China: a single-center experience over a 10-year period. J Pediatr Surg. 2012; 47(8):1576–80. doi: 10.016/j.jpedsurg.2011.11.005.
  14. Baik K, Kang M, Park K, Choi H. Prepubertal testicular tumors in Korea: a single surgeon’s experience of more than 20 years. Korean J Urol. 2013; 54:399–403
  15. Weissbach L, Altwein JE, Stiens R. Germinal testicular tumors in childhood. Report of observations and literature review. Eur Urol 1984; 10:73–85.
  16. Peretsman, SI, Maldazys JD. Infantile testicular cyst: diagnosis and conservative surgical management. J Pediatr Surg, 30:1488, 1995.
  17. Tallen G, Hernaiz Driever P, Degenhardt P, Henze G, Riebel T. High reliability of scrotal ultrasonography in the management of childhood primary testicular neoplasms. Klin Padiatr. 2011; 223(3):131–7. doi: 10.1055/s-0031-1271813. Epub 2011 Apr 1.
  18. Shukla, AR, Woodard C, Carr MC. Experience with testis sparing surgery for testicular teratoma. J Urol. 2004; 171:161–3.
  19. Friend J, Barker A, Khosa J, Samnakay N. Benign scrotal masses in children - some new lessons learned. J Pediatr Surg. 2016; 51(10):1737–42. doi: 10.016/j.jpedsurg.2016.07.016. Epub Aug 5.
  20. Tokuc R, Sakr W, Pontes JE, Haas GP. Accuracy of frozen section examination of testicular tumors. Urology. 1992; 40(6):512–6.
  21. Elert A, Olbert P, Hegele A, Barth P, Hofmann R, Heidenreich A. Accuracy of frozen section examination of testicular tumors of uncertain origin. Eur Urol. 2002; 41(3):290–3.