Elsevier

The Lancet Oncology

Volume 11, Issue 5, May 2010, Pages 476-483
The Lancet Oncology

Review
Testicular and paratesticular tumours in the prepubertal population

https://doi.org/10.1016/S1470-2045(10)70012-7Get rights and content

Summary

Prepubertal testicular and paratesticular tumours are a rare group of tumours, distinct from postpubertal paediatric and adult tumours of this region. Tumours within this group are testicular germ-cell tumours (such as benign teratoma, epidermoid cyst and malignant yolk-sac tumours) and stromal tumours (such as juvenile granulosa-cell, leydig-cell, and sertoli-cell tumours). Paratesticular tumours can be benign (lipoma, leiomyoma, haemangioma) or malignant (rhabdomyosarcoma, melanotic neuroectodermal tumour of infancy). Because of their rarity, centralised pathology and treatment, and national collaborative clinical trials have been important in establishing the optimum management of malignant tumours in this group. We provide an up-to-date and comprehensive review of the clinical presentation, imaging, pathology, and clinical management of prepubertal paratesticular and testicular tumours.

Introduction

Prepubertal testicular and paratesticular tumours are a group of rare but distinct entities with regard to epidemiology, natural history, histological types, and management, compared with pathologies encountered in adult or postpubertal populations. Previously, adolescent and prepubertal cases were grouped together as a paediatric population; however, the current recommendation is to regard the prepubertal group as a distinct entity.1 As with many rare childhood cancers, centralised data collection by registries (such as the Prepubertal Testis Tumor Registry by the Section of Urology of the American Academy of Pediatrics in 1980 and the Armed Forces Institute of Pathology American Testicular Tumor Registry) and national cooperative group trials (such as the Children's Oncology Group in the USA, the Children's Cancer Study Group in the UK, and the German Society of Pediatric Oncology) have been fundamental in improving the management of patients with these tumours. Here, we provide a comprehensive review of the clinical presentation (figure 1A), diagnosis, pathogenesis, and management (figure 1B) of the common prepubertal testicular and paratesticular tumours likely to be encountered by paediatric surgeons and oncologists (table).

Section snippets

Epidemiology

Prepubertal testicular and paratesticular tumours represent 1–2% of all solid paediatric neoplastic lesions with an incidence of 0·5–2 per 100 000 children.2, 3 Testicular tumours show a bimodal age distribution, with a large peak in young adults and a much smaller, but distinct, peak in the first 3 years of life.4, 5 In fact, although the peak age at presentation in this group is 2 years, 60% of tumours present earlier, and the median age for presentation of yolk-sac tumours is 16 months and

Clinical presentation

The most common presentation (>90%) is of a painless scrotal mass, with a history of trauma or bruising and hydrocoele or hernia in <10%.10 Examination usually reveals a palpable solid mass, although occasionally the mass may be impalpable and deep within the testicular tissue. A hydrocoele is associated with a testicular tumour in 15–50% of cases, and a thorough examination is needed to determine the presence of other pathology, such as testicular torsion, epididymitis, inguinal hernia, and

Diagnosis and staging

Ultrasonography with doppler studies at a high working frequency of 7·5–10 MHz is required to distinguish particular features of testicular and paratesticular lesions, and the detection of testicular neoplasms with ultrasonography approaches 100%.11, 12, 13 Benign tumours tend to be well circumscribed with sharp borders and decreased blood flow on doppler studies. Epidermoid cysts present as firm, well defined intratesticular lesions that, on ultrasound, have a central hypoechoic region or

Teratomas

Teratomas are almost always benign in prepubertal children but may be malignant in adults.18 Teratomas present at a median age of 13 months, ranging from birth to 18 months. Histologically, prepubertal teratomas can consist of any combination of the three primitive embryological germ-cell layers (ectoderm, mesoderm and endoderm, giving rise to epithelium, cartilage, fat, bone, muscle, and neural elements), which accounts for their heterogeneous appearance on ultrasound, with areas of

Prepubertal paratesticular tumours

The paratesticular region consists of the spermatic cord, epididymis, vestigial remnants, and tunica vaginalis. These tissues can very rarely give rise to benign (leiomyoma, fibroma, lipoma, and haemangioma) and malignant (rhabdomyosarcoma and melanotic neuroectodermal tumour of infancy) lesions in the prepubertal age group. Paratesticular masses that are small (<3 cm), and for which normal testicular tissue can be preserved, usually warrant inguinal exploration with on-table frozen section to

Conclusion

Testicular and paratesticular tumours in childhood are rare but are a substantial burden when they do occur, both from the malignant effects of local infiltration and metastatic spread, as well as the effects of treatment on fertility and body image. Emphasis in recent years has been on reducing treatment-related morbidity for those with benign tumours, using enucleation, and aggressive treatment of boys with malignant tumours to increase survival.

Search strategy and selection criteria

Searches of Medline and PubMed were done by use of the search terms “paediatric or pediatric” and “paratesticular” and/or “testicular” and specific search terms for each tumour described in this review. Only papers published in English between Jan 1, 1999, and May 31, 2009, were included. The search was supplemented with manual searches of the bibliographies of relevant papers.

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