Prepubertal Testicular Tumors Research Articles

Surgical management of prepubertal testicular tumors: A 30-year study in our institution.

The 2021 European Association of Urology-European Society for Paediatric Urology guidelines on Pediatric Urology recommended testis-sparing surgery (TSS) as the primary approach to treat prepubertal testicular tumors exhibiting favorable preoperative ultrasound diagnoses. However, prepubertal testicular tumors are rare and clinical data regarding them is limited. Here, we analyzed the surgical management of prepubertal testicular tumors based on cases observed over approximately 30 years. Data were retrospectively reviewed from medical records of consecutive patients aged <14 years with testicular tumors who received treatment at our institution between 1987 and 2020. We compared patients by their clinical characteristics, namely, those who underwent TSS versus radical orchiectomy (RO) and those who received surgery in 2005 onward versus prior to 2005. We identified 17 patients, with a median age at surgery of 3.2 years (range 0.6-14.0) and a median tumor size of 15 mm (range 6-67). The tumor size was significantly smaller in patients who underwent TSS than in those who underwent RO (p=0.007). Patients treated in 2005 onward were more likely to undergo TSS than those treated prior to 2005 (71% vs. 10%, respectively), without significant differences in tumor size or the rate of preoperative ultrasound. No TSS cases required conversion to RO. Recent improvements in ultrasound imaging technology allow for more accurate clinical diagnosis. Therefore, the indications of TSS for prepubertal testicular tumors can be judged based not only on the tumor size but also on the diagnosis of benign tumors by preoperative ultrasound.

Clinical challenge: a case of testicular tumor disguised as a prenatal testicular torsion

BackgroundNeonatal tumors account for a minority of prepubertal testicular tumors. Similarly, testicular torsion in the neonate within the intrauterine or postnatal period is a rare event with an estimated incidence of 6 per 100,000. Testicular salvage is almost never achieved in cases of prenatal testicular torsion and there is still no consensus on the ideal management.Case presentationA neonate with prenatal testicular torsion that underwent inguinal orchiectomy. On pathological examination, an uncharacterizable, multicystic, and well-delineated testicular tumor was identified. Although rare, testicular tumor presenting as neonatal testicular torsion should be considered in the differential diagnosis of an acute neonatal scrotum.ConclusionsThis case highlights that an inguinal approach for surgical exploration in prenatal testicular torsions may be preferred given the risk, albeit extremely low, of an underlying testicular tumour.

Unusual Presentation, Relapse, and Metastasis of a Pediatric Testicular Yolk Sac Tumor: Case Report

Testicular tumors are not uncommon in children and represent 1%-2% of all pediatric malignancies. Prepubertal testicular yolk sac tumor is the most common childhood testicular cancer, accounting for 70%-80% of all cases. The clinical presentation varies from one patient to another; most common presentation is painless scrotal mass. Herein, we present a case of pediatric patient with a testicular yolk sac tumor who had unusual presentation followed by a local relapse and metastasis and continued to have high markers while he was on chemotherapy, then underwent retroperitoneal lymph node dissection and local recurrence excision.

Spermatogenesis in pre-pubertal boys with Leydig cell neoplasms suggests paracrine stimulation by testosterone

Pediatric Leydig cell tumors (LCTs) represent approximately 4% of pre-pubertal testicular tumors and are known to cause precocious puberty secondary to testosterone production. While gonadotropins and testosterone are known to initiate spermatogenesis beginning in puberty, it is yet to be determined if a similar phenomenon is triggered by isolated testosterone production in prepubescent boys. To determine if testicular pathology in pre-pubertal pediatric patients with LCTs exhibit spermatogenesis secondary to paracrine testosterone stimulation. We reviewed patients who underwent orchiectomy for a testicular tumor from 2003-17. We included patients with LCTs and compared them to children with non-LCT pathology (teratomas and epidermoid cysts). We excluded other pathologies and tumors in pubertal patients. Data were collected on the presence of spermatogenesis on pathology, tumor markers and serum hormone results. Orchiectomy for testicular tumors were completed in 66 patients, of which 20 were included in the non-LCT group and 9 in the LCT group. Two of the 9 LCT patients had bilateral pathology. Age at presentation was 6.3±5.8 years for the non-LCT group vs. 8.4±1.6 years for LCTs (p=0.261). Spermatogenesis was detected in 7 (64%) LCT specimens vs 2 (10%) non-LCT specimens (p=0.002). Age of the spermatogenesis patients in the non-LCT group (11.08±2.5 years) was older than LCT ones (8.3±2.0 years), suggesting that spermatogenesis in the non-LCT group may be due to early pubertal development. The summary figure demonstrates spermatogenesis identified in a pre-pubertal LCT patient. In this study, pre-pubertal males with LCTs were identified to have pathology evidence of spermatogenesis compared to controls with non-LCT pathology. This represents the first study assessing paracrine testosterone effects on spermatogenesis in pre-pubertal patients with LCTs. In contrast, adult literature on LCTs primarily report on resulting concerns for fertility, gynecomastia and testicular atrophy. LCTs can induce spermatogenesis in prepubertal patients. This reinforces the hypothesis that paracrine testosterone signaling plays a role in spermatogenesis. Our findings could help explore novel fertility preservation opportunities in children.

Prepubertal testicular tumors in China: a 10-year experience with 67 cases.

Prepubertal testicular tumors are rare in children. We aim to present clinical and histological features of prepubertal testicular tumors through the analysis of the long-term experiences of a single medical center of China. A total of 67 children (≤ 14years) treated for testicular tumor at our institution from 2005 to 2015 were retrospectively reviewed. Data relating the clinical characteristics, histopathology findings, serum tumor markers, treatment method, and outcome were collected. The patients' median age at diagnosis was 18months (range 3-168months), and 49 cases (73.1%) were diagnosed at age younger than 3years. The most common clinical presentation was a painless scrotal mass or swelling. Regarding histology, 32 (47.8%) were teratomas and only one of these tumors presents immature teratomas, 20 (29.9%) were yolk sac tumors, 9 (13.4%) were epidermoid cyst, 1 (1.5%) was a Leydig cell tumor, 1 (1.5%) was a mixed malignant germ cell tumor, and 4 (8.3%) were paratesticular tumors. For germ cell tumors, the mean preoperative serum α-fetoprotein (AFP) level was significantly higher in patients with yolk sac tumor than in those with teratomas (2,078ng/mL vs 5.7ng/mL). Of all these patients, 37 (55.2%) were treated with radical inguinal orchiectomy and testis-sparing surgery was planned and achieved in 30 (44.8%). Surveillance was performed in 60 patients. None of the patients developed recurrence or testicular atrophy after appropriate treatment. The majority of our cases were benign, with the most common histopathological subtype being teratoma. A testis-sparing procedure should be performed in children with a palpable testicular mass and negative tumor markers. This study shows a better outlook for prepuberty patient with testicular tumors than their adult counterparts.

Characteristics, treatment decisions and outcomes of prepubertal testicular germ cell tumor: A descriptive analysis from a large Chinese center

Prepubertal testicular tumors are fundamentally distinct from their postpubertal and adult counterparts. Racial and ethnic differences contribute to the incidence, characteristics, and histological distribution of prepubertal testicular germ cell tumors (PTGCTs). To elucidate the characteristics and treatment outcomes of PTGCTs in a Chinese cohort. Data were retrospectively reviewed from consecutive PTGCT patients aged <12 years who received treatment at the current institution from January 2007 to December 2015. Exclusion criteria were: patients aged >12 years, non-primary testicular germ cell tumors, and para-testicular tumors. A total of 167 patients qualified for the study (Table). The median age at diagnosis was 20 months (range 1-142). Pathology revealed 87 (50.9%) teratomas, 50 (29.2%) yolk sac tumors (YSTs), 33 (19.3%) epidermoid cysts, and one (0.6%) mixed germ cell tumor. Of the teratomas, 70 (80.5%) mature teratomas (MTs) and 17 (19.5%) immature teratomas (ITs) were detected. ITs manifested at a younger age and were larger in size compared with MTs (P<0.001). Testis-sparing surgery (TSS) was performed for all MTs and 10 ITs. Radical orchiectomy (RO) was performed for seven ITs. No metastatic or local recurrence was detected in benign tumors during follow-up. Obviously elevated alpha fetoprotein (AFP) levels were detected in all the YSTs. Of these, 47 (94%) were diagnosed as Stage I, one (2%) as Stage II, and two (4%) as Stage IV. After RO, recurrence was detected in one Stage I patient with a median follow-up of 46 months. One Stage II and two Stage IV patients died due to tumor progression. Racial and ethnic differences were detected in PTGCTs. It is believed that the present study is the largest study detailing the clinicopathologic characteristics and treatment outcomes of PTGCTs in a Chinese cohort. Teratoma was the most common subtype of PTGCTs, followed by YST. ITs manifested as benign behavior and were more likely to be present at a younger age and have a larger tumor size compared with MTs. TSS was reliable for benign lesions. For Stage I YST, RO alone was valid. Salvage chemotherapy was effective and preventative retroperitoneal lymph node dissection may not be necessary for YST. The prognosis of PTGCTs was favorable.

Testicular tumors in prepubescent patients

Background/purposePediatric testicular tumors are rare, constituting only 1% of all pediatric solid tumors. Single-institution studies addressing pediatric testicular tumors published to date have been limited in the number of patients. MethodsWe utilized the National Cancer Data Base (1998–2012) to review all prepubescent patients (≤12 years old) with testicular neoplasms. Demographics, tumor characteristics, treatment modalities, and outcomes were abstracted. ResultsA total of 479 patients were identified, with a median age of 3 years (IQR 0–4) at diagnosis. 67% of cases were diagnosed by 3 years of age. Yolk sac tumors were the most common histology (202 patients, 42.2%). Most tumors were diagnosed at a low stage. Resection was performed in 465 boys, with 75% having undergone radical orchiectomies. Chemotherapy was utilized in 28% of cases and radiotherapy in 7%. With mean follow-up of 5.6 years, mortality rate was 3%. No difference in mortality was noted based on histology or extent of surgical resection. ConclusionsThis series of prepubertal testicular tumors is the largest yet reported and highlights the patient demographics, tumor characteristics, treatment modalities and outcomes for these tumors. Type of studyPrognosis study Level of evidenceII.

Perspectives in Pediatric Pathology, Chapter 25. Testicular and Paratesticular Tumors in the Pediatric Age Group.

Testicular tumors in the prepubertal age are relatively rare, representing only 9.4% of the total testicular and paratesticular specimens from a 20-year review performed at a large pediatric hospital [ 1 ]. They account for 1% to 2% of all solid tumors in the pediatric age group, with an annual incidence between 0.5/100 000 and 2/100 000 boys according to Coppes et al [ 2 ] and data from the Prepubertal Testicular Tumor Registry [ 3 ]. Similar to other neoplasms afflicting children, a bimodal age distribution is observed. The first peak is between birth and 3 years of age, and a second one occurs at the onset of puberty, extending to the fourth decade. Reports on their frequency vary because some investigators include the adolescent period, while others do not [ 4 ]. The vast majority of testicular tumors are germ cell neoplasms, accounting for 95% across all ages [ 5 ]. In children, germ cell tumors also predominate, representing 71% of all testicular neoplasms. These include yolk sac tumors (49%), teratomas (13%), seminomas and mixed germ cell tumors (9%), and sex-cord stromal tumors (29%). Malignant potential is significantly lower (less than 70%) in the pediatric age group compared to adults (90%) [ 6 ]. According to Pohl et al, 74% of prepubertal testicular tumors are benign [ 7 ].

Congenital juvenile granulosa cell tumor of the testis: Case report and literature review

Juvenile granulosa cell tumor (JGCT) is a very rarely diagnosed benign tumor, accounting for 1.2% of all prepubertal testicular tumors. A full-term healthy neonate was diagnosed with a painless left scrotal mass. During evaluation it was identified to have about two times the volume of the contralateral testis, presenting a firm consistency, not as hard as the consistency of a prenatal testicular torsion. Doppler ultrasound detected a multicystic left testicular mass, with normal blood flow, but failed in detecting normal-appearing testis. Human chorionic gonadotropin (?-HCG) and serum alpha-fetoprotein (AFP) were normal. Inguinal approach was performed, section of the lesion was sent to frozen biopsy and excluded yolk sac tumor, and however the impossibility of detecting normal testis tissue indicated orchiectomy with high ligation of the spermatic cord. Histological evaluation demonstrated gray testicular parenchyma with multicystic aspect fulfilled with yellow fluid. The usual clinical presentation of JGCT is a painless scrotal mass, radiological imaging demonstrates a multicystic tumor. Tumoral markers levels are normal and the standard treatment is the inguinal orchiectomy.

Prepubertal testicular tumors: a single-center experience of 44 years

PurposeTo present the clinical and histological features of prepubertal testicular tumors (PTTs), the long-term experience of a single institution was reviewed. Materials and methodsA total of 62 prepubertal children who were treated for testicular tumors at Kanagawa Children's Medical Center from 1971 to 2014 were retrospectively reviewed. Histopathological findings, age at operation, clinical stage, and outcomes were analyzed. Clinical findings between the two eras, 1971–1990 and 1991–2014, were also compared. ResultThe median age at operation was 17months. Pathology revealed 29 teratomas (47%), 26 yolk sac tumors (42%), 5 epidermoid cysts (8%), 1 Sertoli cell tumor (1.5%), and 1 benign cyst (1.5%). Teratoma was the most common tumor in this series, and children with immature teratomas were operated at a significantly younger age than those with mature teratomas. Yolk sac tumor was the second most common. The clinical stages of yolk sac tumors were stage I in 23 (89%) and stage II in 3 (11%). Clinical findings were not significantly different between the early and late eras. ConclusionsTo the best of our knowledge, this is the largest single-center study of PTTs in Japan. The most common PTT in this study was teratoma, followed by yolk sac tumor. There was no significant difference in the histological distribution of PTTs between the two eras. Compared with the current data of single-center series in North America, the incidence of yolk sac tumor was markedly higher in the present study. This discrepancy is possibly explained by racial differences.

Juvenile Granulosa Cell Tumor of the Testis: A Bilateral and Synchronous Case. Should Testis-sparing Surgery Be Mandatory?

Granulosa cell tumor of the testis is an infrequent stromal cell tumor that can be distinguished into adult and juvenile, the latter being more common. Juvenile granulosa cell tumor of the testis is a rare pathologic finding, accounting for 1.2%-3.9% of prepubertal testicular tumors. It is considered as a benign stromal sex cord tumor and is usually unilateral. Although radical surgery was previously considered the treatment of choice, testis-sparing surgery is now recommended in all cases where applicable. We report a bilateral synchronous juvenile granulosa cell tumor in a 6-month-old child treated with testis-sparing surgery and provide a review of the literature.

Overview of pediatric testicular tumors in Korea.

Prepubertal testicular tumors are rare compared with postpubertal testicular tumors. The incidence of prepubertal testicular tumors peaks at 2 years of age, tapers off after 4 years of age, and then begins to rise again at puberty. Prepubertal and postpubertal testicular tumors show many differences, including the typical tumor histology, molecular biological differences, and the malignant potential of tumors at different ages. Pediatric testicular tumors are classified as benign or malignant on the basis of their clinical behavior and histologically are divided into germ cell and gonadal stromal (nongerm cell) tumors. Many histological and biological studies have further confirmed the distinct nature of prepubertal and postpubertal testicular tumors. These differences have led to various management strategies for prepubertal and postpubertal tumors. Because overall about 75% of prepubertal testicular tumors are benign, a testis-sparing approach is becoming more common in children. Orchiectomy and observation with very selective use of chemotherapy has become the standard approach when a malignant tumor is identified. Retroperitoneal lymph node dissection and radiation therapy play very limited roles.

Prepubertal testicular tumours: Should testicular-sparing surgery be considered? A single-institution experience and review of the literature

ObjectivesTo review our 10-year experience with uncommon testicular tumours in children (prepubertal testicular and paratesticular), to review previous reports, and to determine the appropriate management of these rare tumours, specifically the role of testicular-sparing surgery. Patients and methodsWe retrospectively reviewed all cases of testicular tumours managed at our institution between 1999 and 2009. Boys aged <16years were included in the study. The patients’ characteristics, presentation, the diagnostic tools, tumour markers, mode of treatment, pathological findings and outcome were assessed. We reviewed previous reports that addressed testicular and paratesticular tumours in prepubertal boys, using a Medline/PubMed search. ResultsFrom 80 patients, 13 boys (median age 8.7years) presented with testicular tumours (16%) and were included in the study. Two boys presented with precocious puberty and one with gynaecomastia. The level of α-fetoprotein was high in three boys, and the human chorionic gonadotrophin level was elevated in one, and both markers were high in one. Testicular-sparing surgery was performed in three boys. Six of the 13 tumours were malignant and seven were benign. None of the patients developed a recurrence or testicular atrophy after testicular-sparing surgery. From the review of previous reports we devised an evidence-based algorithm for managing prepubertal testicular tumours. ConclusionsPaediatric testicular tumours are rare but they require an inguinal approach for either orchidectomy or testicular-sparing surgery; we recommend the latter option as long as frozen sections are analysed and a safety margin is maintained.

Expression analysis of the pluripotency marker UTF-1 for determining the applicability of testis-sparing surgery for prepubertal testis tumors

Testis-sparing surgery (TSS) is widely performed for prepubertal testicular tumors because most of these tumors are benign and have biological characteristics that make them amenable to local treatment. Here, we report a case of prepubertal teratoma enucleation. In order to evaluate the proliferative potential of the tumor and the applicability of testis-sparing surgery, we investigated the expression of UTF-1—a specific marker of pluripotency and self-renewal—using real-time reverse transcription-polymerase chain reaction and immunohistochemistry. Because the enucleated tissue did not demonstrate the expression of UTF-1, the applicability of TSS was verified, in the present case.

Testicular Teratoma, Mimicking a Simple Testicular Cyst, in an Infant

Prepubertal testicular tumors are rare, and teratoma is the second most frequent histologic type. Its typical features are those of a hard and painless scrotal mass at clinical examination, and nonhomogeneous, echoic, often with calcifications at ultrasonography. Rare but reported is the atypical presentation as a transilluminating scrotal mass, due to the presence of some internal cystic areas, detectable at ultrasonography. We report the case of an infant with a transilluminating scrotal mass, mimicking at ultrasonography and surgery a simple, fully liquid cyst, which the pathologic examination revealed to be mature cystic testicular teratoma.

Prepubertal Testicular Tumors in Korea: A Single Surgeon's Experience of More Than 20 Years

PurposeTo present clinical and histological features of prepubertal testicular tumors through the analysis of the long-term experiences of a single surgeon.Materials and MethodsThe charts of 48 children treated for testicular tumors from 1986 to 2010 were retrospectively reviewed. All patients underwent radical orchiectomy. The patients' ages, clinical presentations, histopathological findings, kinetics of tumor markers, and outcomes were recorded.ResultsThe patients' median age at the initial diagnosis was 19.5 months (range, 3 to 84 months). All patients presented with either a palpable mass (76%) or scrotal size discrepancy (24%). Compared with a palpable mass, scrotal size discrepancy led to delay in diagnosis by 5 months. Regarding histology, yolk sac tumors and teratomas accounted for 53% and 36% of the tumors, respectively. The mean preoperative alpha-fetoprotein (AFP) level was significantly higher in patients with yolk sac tumors than in those with teratomas (4,600 ng/mL vs. 6.3 ng/mL), and only one patient with a teratoma had a preoperative AFP value higher than 20 ng/mL. Following radical orchiectomy, 72%, 8%, and 16% of patients with a yolk sac tumor showed normalization, persistent elevation, and relapse after transient lowering of AFP, respectively. Preoperative AFP was greater in patients with non-normalization than in those with normalization. Five of six patients with non-normalization showed evidence of either vascular invasion or endolymphatic tumor emboli.ConclusionsWe found a higher number of yolk sac tumors than teratomas in patients with prepubertal testicular tumors. AFP was the most useful marker in the diagnosis and follow-up of childhood yolk sac tumors. Relapsed yolk sac tumors often showed pathological evidence of aggressiveness.

Prepubertal testicular and paratesticular tumors in China: a single-center experience over a 10-year period

ObjectivePrepubertal testicular tumors are rare and fundamentally distinct from adult testicular tumors. We reviewed our 11-year experience in a single medical center of China. Material and MethodsThis study reports the clinical characteristics, histopathologic diagnosis, treatment methods, and outcome in a series of 63 prepubertal boys who were treated between 1997 and 2008. ResultsA total of 63 primary prepubertal testicular and paratesticular tumors were identified. The median age at presentation was 11 months. Of these tumors, 27 (42.9%) were mature teratomas, 5 (7.9%) were immature teratomas, 21 (33.3%) were yolk sac tumors, 4 (6.3%) were epidermoid cyst, 2 (3.2%) were Leydig cell tumors, 1 (1.6%) was a mixed malignant germ cell tumor, and 3 (4.8%) were paratesticular tumors. The most common clinical presentation (95.2%) was a painless scrotal mass or swelling. Forty-eight tumors were treated with radical inguinal orchiectomy, and 15, with a testis-sparing procedure. Follow-up was available in 59 cases, range from 4 to 128 months (median, 50 months). One patient with yolk sac tumor had recurrence and progression to metastasis at the end of 4 months after surgery. Other patients were disease free at last follow-up. ConclusionsMost of the prepubertal testicular lesions were benign, and the most common histologic subtype was teratoma. Our experience with testis-sparing procedures supports the current trends that less invasive treatment should be performed for benign lesions. This study confirms the excellent cure rates obtained in children with prepubertal testicular tumors.

Tumorregister und Realität - Differenzialdiagnose von Hodentumoren im Kindesalter - Übersicht mit eigenem Patientengut

Painless scrotal swelling must always be considered as a potential malignant testicular tumour until proven otherwise. Prepubertal testicular tumours are seldom entities, for their differential diagnosis testicular sonography is gaining increasing importance. Although, according to national and international tumour registries, most patients tend to have malignant tumours, some clinical series from paediatric centres confirm that prepubertal testicular tumours are mainly benign lesions, especially up to the age of 12 years. Testis-sparing procedures are favoured when AFP is in a normal range and testicular parenchyma is detected sonographically. Oncological principles should be applied to paediatric patients consequently, staging examinations should be requested when a definite histology is proven. Cases should be reported to national tumour registries and, in addition to close surveillance, in some cases adjuvant therapy is necessary. In addition to the clinical guidelines from the tumour registries, differential diagnosis, testis-sparing surgery and the presence of TIN in the paediatric population should be discussed.

An Unusual Cause of Infantile Gynecomastia

Prepubertal testicular masses are relatively rare. Sertoli cell tumors account for 2% of prepubertal testicular tumors and very few have occurred in the first decade of life. Gynecomastia can be seen in approximately 5% of patients with testicular mass. We present an 8-month-old boy admitted with bilateral gynecomastia and unilateral testicular mass.

Prepubertal testicular tumours and efficacy of testicular preserving surgery

Therapy (case series). 4. What's known on the subject? and What does the study add? Testicular tumours in childhood are very rare. Historically, most of these tumours have been considered malignant, but more recent studies indicate that benign lesions, particularly teratoma, are much more frequent than previously thought. Testicular tumours in this age group have traditionally been treated with inguinal radical orchiectomy, but more conservative management has been proposed in view of the higher frequency of benign tumours. In children, most testicular tumours are benign, especially before puberty. A testis-sparing procedure should be performed in children with a palpable testicular mass and negative tumour markers. To report our experience of testicular tumours in children aged≤13 years, including our experience with testis-sparing surgery. A retrospective study was performed of 15 patients with testicular tumours aged≤13 years who presented at our centre between 1984 and 2008. The use of testis-preserving surgery according to indication was investigated and outcomes were recorded. The clinical presentation was increased testicular size with a palpable mass in 80% of the cases. All 15 patients underwent surgery. The tumour was benign in 12 (80%) patients and malignant in three (20%) patients. Organ-preserving surgery was planned and achieved in 11 patients (73%). Pathology of the tumourectomy specimens disclosed benign tumours in all cases: four epidermoid cysts, two teratomas, one juvenile granulosa cell tumour, one haemangioma, one lipoma, one fibrous hamartoma and one splenogonadal fusion. In four patients who underwent radical orchiectomy, pathology identified one yolk sac tumour (stage I), two mixed germ cell tumours and one gonadoblastoma. In children, most testicular tumours are benign, especially before puberty. A testis-sparing procedure should be performed in children with a palpable testicular mass and negative tumour markers. The lesion, however, should be thoroughly excised to avoid recurrences.