Prepubertal testicular tumors: A 20‐year experience with 40 cases

Testicular tumors are rare in children. In addition, prepubertal testicular tumors (PTT) are fundamentally different from their postpubertal counterparts. We reviewed our 20‐year experience with 40 cases to suggest an algorithm for the appropriate surgical approach for patients with PTT. All tumors ultrasonographically suspected of being benign were confirmed to be benign postoperatively. There was a significant difference between median preoperative α‐fetoprotein (AFP) levels of infants with yolk sac tumor and teratoma. The majority of our cases were benign, with the most common histopathological subtype being teratoma, as recently reported. Testis‐sparing surgery was carried out in five patients with teratoma and three patients with epidermoid cyst. Other than one patient with teratoma, none of the patients developed recurrence or testicular atrophy after testis‐sparing surgery. When testis‐sparing surgery is considered, our algorithm based on ultrasonographic findings and preoperative AFP levels might be helpful.     

Testis sparing surgery for epidermoid cyst of the testis: A case report

A rare tumour of a prepubertal child, an epidermoid cyst, was excised with testicular preservation. Childhood testicular tumours are usually benign. Although epidermoid cysts of the testis may have teratomatous component, testicular teratomas are generally benign in the prepubertal child. For these reasons testis sparing surgery seems applicable in childhood epidermoid cysts.     

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.     

Testis sparing surgery for epidermoid cyst of the testis: A case report

A rare tumour of the prepubertal child, an epidermoid cyst was excised with testicular preservation. Childhood testis tumours are usually benign. Although epidermoid cystes of the testis may have teratomatous component, testicular teratomas are generally benign in the prepubertal child. For these reasons testis sparing surgery seems applicable in childhood epidermoid cysts.     

Testicular sparing surgery for prepubertal teratoma of the testis: a clinical and pathological study

We report on 5 patients 14 months to 6 1/2 years old with prepubertal teratoma of the testis treated by testicular sparing tumor enucleation. All 5 patients had no evidence of recurrence at a mean followup of 96 months. Recognizing that this is not accepted therapy for testis tumors, 17 orchiectomy specimens containing teratoma from children were histologically analyzed in cooperation with the Armed Forces Institute of Pathology Tumor Registry. All patients were prepubertal at orchiectomy (3 months to 8 years old) and all are well with a mean followup of 174 months. Histological examination revealed no foci of teratoma separate from the main tumor in any specimens. Immunohistochemical studies with placental alkaline phosphatase, a marker for malignant germ cells, were done to detect carcinoma in situ in the seminiferous tubules of these testes. This test did not reveal any intratubular malignant germ cells (carcinoma in situ). Based on our clinical experience with testicular sparing tumor enucleation, the histological findings on Armed Forces Institute of Pathology review demonstrating no associated carcinoma in situ and the universally benign behavior of prepubertal testicular teratomas, we recommend a testicular sparing operation rather than orchiectomy for testicular teratoma in prepubertal patients.     

The synchronous appearance of multiple testicular epidermoid cysts and of a malignant mixed germinal tumor in the contralateral testis]

We describe the simultaneous appearance of multiple epidermoid cysts in the right and an adult teratoma containing embryonal carcinoma and choriocarcinoma in the left testis. No similar case has previously been described. Epidermoid cysts of the testis are rare, accounting for about 1% of all testicular tumors. Epidermoid cysts are now regarded as monoepidermally developed teratomas of germ cell origin. Testicular teratomas in adults, however, are always malignant. Because epidermoid cysts are rare tumors, primary therapy often consists in ablation of the testis. In the case described, excision of the epidermoid cyst protected the patient from complete castration. This case demonstrates the simultaneous appearance of a malignant and a benign testicular germ cell tumor.     

Testicular and paratesticular neoplasms in prepubertal males

PURPOSE: We reviewed the current diagnosis, staging and management of testicular and paratesticular neoplasms in prepubertal males. MATERIALS AND METHODS: We performed a medical literature search in English using MEDLINE/PubMed that addressed testicular and/or paratesticular neoplasms in prepubertal males. We then analyzed the literature with respect to individual tumors. We present a concise approach toward the management of these individual tumors. RESULTS: There is still a predominance of yolk sac tumors in prepubertal males, although some studies suggest that teratomas are more common but underreported due to their benign course in children. Prepubertal males are diagnosed in a fashion similar to that in adult patients with an appropriate history and physical examination. A palpable, nontender mass suggests the diagnosis and prompts scrotal ultrasound and tumor markers. Although treatment for most primary tumors has historically been radical inguinal orchiectomy, most benign tumors can now be managed by testis sparing surgery. The addition of radiation, chemotherapy and/or retroperitoneal lymph node dissection depends on tumor stage and histological type. CONCLUSIONS: Although it is rare in children, any solid scrotal mass in prepubertal males warrants evaluation for possible testicular or paratesticular neoplasm.     

Prepubertal testicular tumours and efficacy of testicular preserving surgery

Study Type – Therapy (case series)
Level of Evidence 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.

OBJECTIVE

? To report our experience of testicular tumours in children aged ≤13 years, including our experience with testis‐sparing surgery.

PATIENTS AND METHODS

? 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.

RESULTS

? 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.

CONCLUSIONS

? 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.     

Enucleation for prepubertal leydig cell tumor

PURPOSE: Leydig cell tumors in children are rare, comprising only 4% to 9% of all primary testis tumors in prepubertal males. Almost all of these boys present with isosexual precocious pseudopuberty associated with increased testosterone, low gonadotropin levels and a testis mass. We present our experience with testis sparing enucleation of Leydig cell tumor in prepubertal boys. MATERIALS AND METHODS: Two patients presented with isosexual precocious puberty at ages 6 and 9 years. Each patient had a well circumscribed, painless testicular mass, increased serum testosterone (101 and 444 ng/dl [normal 0 to 25]), normal gonadotropins and negative alpha-fetoprotein levels. Both patients underwent successful enucleation of the testis mass following proper testis oncological surgical principles. RESULTS: Both patients had normalization of the serum testosterone following enucleation of the Leydig cell tumor. At 9 and 44 months of followup they have maintained normal ipsilateral testicular volume compared to the contralateral gonad, and 1 patient entered puberty spontaneously at 1 year postoperatively. Neither patient suffered any morbidity, and both have presumably benefited from preservation of the involved gonad with preserved testicular volume. CONCLUSIONS: Prepubertal boys with isosexual precocious pseudopuberty, an isolated testis mass, increased testosterone and low or normal gonadotropin levels can reliably be diagnosed with Leydig cell tumors. Based on the ability to establish the diagnosis preoperatively and the universal benign behavior of unilateral, prepubertal Leydig cell tumor, we believe these patients are best treated with testis sparing enucleation of the tumor. In view of the high likelihood that this tumor in prepubertal boys is benign, a transscrotal surgical approach should be considered.     

Testicular teratoma presenting as a transilluminating scrotal mass

Prepubertal testicular neoplasms are uncommon and differ from adult neoplasms in histopathologic features and presentation. The presented case describes a 3-year-old boy with an enlarged, transilluminating scrotal mass. Ultrasonography revealed a heterogeneous mass and absence of a hydrocele. After right radical orchiectomy, pathologic examination confirmed the mass as a mature teratoma. Although tumor registries cite yolk sac tumor as the most common prepubertal testis neoplasm, more recent studies have demonstrated benign tumors such as teratomas to be more common. Although rare, some prepubertal tumors such as teratomas may transilluminate. Ultrasonography should be used to evaluate hydroceles when the testis is impalpable.     

Testicular tumors: wide spectrum in our short casuistics

Testicular tumors occur in 0.5 to 2 per 100,000 children. They are 1-2% of all solid tumors before puberty. The clinical history, testicular and abdominal ultrasonography, alpha-fetoprotein and human chorionic gonadotropin, estrogens and androgen levels, FSH and LH determine the diagnosis. The pathology determines the specific cell. We report seven cases, three germ cell tumors: a Yolk sac tumor in a child of 18 months and two mature teratomas in children between 2 and 11 years presenting as a painless testicular mass without other symptoms. Three tumors estrumales: one derived from Leydig cells and two of the granulosa cells, a palpable testicular mass was added precocious puberty in stage II-III of Tanner in the first, second gynecomastia in Tanner stage III and the third only with testicular mass. The seventh case, Lipoma para-testicular mass palpable. The treatment was radical orchiectomy in five cases. Testis-sparing surgery in Leydig cell tumor and resection of the paratesticular mass was performed through scrotal. The Yolk sac tumor requiring chemotherapy with good outcome. Retroperitoneal lymph node dissection is not recommended in prepubertal. Historically prepubertal testicular tumors have been treated in adults. Recent testicular preservation algorithms optimize and minimize the morbidity of adjuvant therapies. Many are benign and can be treated with preservation of the testis. Localized malignant tumors can be treated by orchiectomy.     

Benign testicular tumors

Testicular tumor is considered rare in Oriental people and benign testicular tumor has never been reported from this area. From 1969 to 1985 70 cases of intratesticular tumors were treated at Tri-Service General Hospital. 12 of them were benign in nature including 8 epidermoid cysts, 1 cavernous hemangioma, 1 foreign body granuloma with pseudocyst, 1 Sertoli cell tumor and 1 intratesticular simple cyst. A testis-sparing procedure was performed for 5 epidermoid cysts, the Sertoli cell tumor and the simple intratesticular cyst. The tumors were excised completely and the testes were preserved after careful and thorough frozen section pathological studies. One of the epidermoid cysts and the case of simple testicular cyst were diagnosed correctly before surgery by using scrotal ultrasound.     

Intraskrotales Lipoblastom im Kindesalter

Based on a case report of an intrascrotal lipoblastoma in childhood, we present the preoperative diagnostic algorithm and the main differential diagnoses in testicular and paratesticular tumors as well as their surgical management. An 8-week-old infant was admitted with a scrotal tumor known since birth. MRI showed a pinnate vessel supply, originating in the left internal iliac artery. The tumor was exposed operatively and could be completely removed. Intrascrotal lipoblastoma are rare. Like most testicular and paratesticular tumors in childhood lipoblastomas are benign. Today ultrasound and Doppler sonography are basic tools for diagnosis and surgical planning in testicular and scrotal tumors in childhood. In some cases MRI can provide additional important information for surgical planning. In contrast to adults testis sparing surgery is favoured in children.     

Benign intrascrotal lesions

PURPOSE: We summarize important clinical, pathological and diagnostic features of benign intrascrotal lesions, including paratesticular lesions (adenomatoid tumors, fibrous pseudotumors, cystadenomas, spermatoceles, hydroceles, varicoceles and hernias) and intratesticular lesions (tunica albuginea cysts, testicular simple cysts, epidermoid cysts, cystic ectasia of the rete testis, intratesticular varicocele, adrenal rest tumors and splenogonadal fusion). This review provides the reader with a better understanding of benign lesions that occur in the scrotum. MATERIALS AND METHODS: A directed MEDLINE literature review of benign scrotal lesions and of each individual lesion was performed. This information was enhanced with relevant information from select journals and texts. Particular emphasis was placed on clinical, pathological and diagnostic features. RESULTS: Intrascrotal lesions continue to provide a diagnostic challenge for physicians. A diagnosis can be made with a thorough history, physical examination and understanding of the pathophysiological processes of the structures contained within the scrotum. Lesions that are suspicious for malignancy should prompt urological consultation and radiological imaging. Ultrasound aids in the diagnosis in instances of uncertainty. Ultimately surgery may be necessary to make a histological diagnosis. CONCLUSIONS: Clinical assessment, physical examination and an understanding of benign intrascrotal processes are key to making a diagnosis. Ultrasound has an important role and adds essential information. If surgery is necessary and a benign process is recognized, a testis sparing procedure should be performed.     

Juvenile granulosa cell tumor of the testis:: contemporary clinical management and pathological diagnosis

PURPOSE: Juvenile granulosa cell tumor (JGCT) of the testis is a rarely diagnosed subset of testicular stromal tumors. Although this variant of testicular stromal tumor is predominantly a benign entity in prepubertal patients, limited experience precludes a complete understanding of its clinical presentation and pathological diagnosis. MATERIALS AND METHODS: We reviewed all cases of testicular tumors at Children's Hospital of Philadelphia between 1976 and 2002 in males younger than 18 years. We specifically reviewed our experience with JGCT in terms of presentation, surgical treatment and long-term outcome. We also reviewed the microscopic findings and histochemical techniques used to confirm the diagnosis. RESULTS: We identified 77 tumors during the defined interval, of which 3 (3.9%) were JGCTs. All 3 patients with JGCT were first noted to have a testis mass soon after birth. All presented with a firm, unilateral testicular mass. Ultrasonographic findings were consistent with a complex, multiseptated, hypoechoic mass. Two of the 3 patients underwent radical orchiectomy. Testis sparing mass excision was performed in 1 patient. Grossly the tumors were partially cystic masses. Histologically positive immunostaining with inhibin-alpha and negative staining for alpha-fetoprotein (AFP) reliably differentiated JGCTs from yolk sac tumors. At a mean followup of 8.5 years (range 5 to 14) no metastases or local tumor recurrences have been diagnosed. CONCLUSIONS: To our knowledge we report the first case of testis sparing enucleation of a JGCT with a 5-year recurrence-free followup. Testis sparing enucleation is now our procedure of choice for tumors in neonates and prepubertal children with serum AFP in the normal range for age. JGCT should be suspected in neonates presenting at birth with a complex, cystic mass of the testis. Positive immunostaining for inhibin-alpha and a lack of AFP staining have consistently corroborated the pathological diagnosis in our experience and they should be applied for pediatric testis tumors that may mimic yolk sac tumor pathology.     

Benigne zystische Raumforderungen des Hodens

Testicular cysts are increasingly diagnosed in the course of scrotal ultrasound examination. Among other things this is due to the general availability of modern high-resolution ultrasound devices. Benign and malignant diseases with testicular cyst formation need to be differentiated by differential diagnosis and by their aetiology. Benign diseases with cystic space-occupying lesions of the testicle are tubular ectasia of the rete testis, cystic dysplasia, epidermoid cysts, simple intraparenchymatous testicular cysts and cysts of the tunica albuginea. Testicular dermoid cyst was long misleadingly regarded as potentially malignant, but is now classified as benign. On diagnosis of a benign lesion of the testis an organ-conserving surgical therapy or an observational watch-and-wait strategy can be recommended in most cases.     

Testicular tumours in children: a single-institutional experience

OBJECTIVE: To report our experience of testicular and paratesticular tumours in children, as such tumours are rare, and historically yolk sac tumour has been described as the most common lesion in children, but recent reports suggest that benign testicular lesions might be more common. PATIENTS AND METHODS: We reviewed retrospectively the records of children treated for testicular tumours from 1998 to 2005. The patients' age, clinical presentation, diagnostic procedures, treatment methods, histopathological findings, and outcome were recorded. Patients aged>144 months and those with non-primary metastatic lesions were excluded. RESULTS: In all, 11 patients met our criteria, with a mean age of 37 months (range 9 days to 144 months). Pathological analysis revealed teratoma in four patients, yolk sac tumour in two, epidermoid cysts in two, extrarenal nephroblastomatosis in one, and paratesticular rhabdomyosarcomas in two. The most common clinical presentation was a painless testicular mass. Depending on the clinical presentation and pathology, scrotal ultrasonography, tumour markers (alpha-fetoprotein and beta-human chorionic gonadotrophin), and/or staging computed tomography (CT) were obtained in eight patients. All patients had a radical orchidectomy. Three patients had elevated tumour markers that normalized after orchidectomy. CT revealed extensive mediastinal adenopathy in one patient with rhabdomyosarcoma. Chemotherapy was administered to both patients with rhabdomyosarcoma. CONCLUSION: Although there were few patients, most of the lesions were benign tumours, with the most common histological subtype being teratoma. As both malignant and paratesticular lesions occurred at a significant frequency, we would continue to advocate an initial radical inguinal approach at which time testis-sparing could be considered if the preoperative evaluation was favourable, and frozen-section analysis at the time of surgery confirms a benign lesion.     

Testicular and paratesticular prepuberal tumors: our experience and update on the topic

Objetivesto evaluate the importance of testicular and paratesticular prepubertal tumors in our center and to make an update on the topic.Methods and patientsdata from all patients diagnosed of testicular and paratesticular prepubertal tumors and treated in our pediatric oncology unit from January 1^st 1998 to December 31^st 2003 have been revised.Resultsseven cases are reported among one hundred and ninety patients (represents 3,68 percent of all treated tumors): five tumors affecting the testis and two cases of paratesticular tumors. Pathology classification was as follows: one yolk salk tumor, one mature teratoma, two nongerminomatous testicular tumors (one Sertoli cell tumor and one unclassifiable), one Burkitt’s lymphoma and two paratesticular rhabdomyosarcomas. Primary approach was inguinal radical orchiectomy in all cases except neoadjuvant chemotherapy in the case of lymphoma and partial escrotectomy in one patient previously managed with transcrotal orchiectomy. Rhabdomyosarcoma cases received adjuvant chemotherapy. All patients are alive and well after a follow-up period ranging from 17 to 74 months.Conclusionstesticular and paratesticular prepubertal tumors are rare. Except for one patient affected of lymphoma, surgical primary approach have been essential for treatment. The prognoses in this series has been excellent.     

Unusual tumors of the testis. Apropos of 22 cases]

The authors report their experience of rare non-germ-cell tumours of the testis over a period of 10 years. The criteria of benign disease, justifying testicular preservation in 5 out of 7 cases of epidermoid cysts, are defined. Four cases of Leydig cell tumours, including 3 with gynaecomastia, are described. Three cases of adenomatoid tumours with conservative surgery, 3 lymphomas, 2 mature teratomas and 2 cysts of the rete testis are also reported. The place of ultrasonography is defined and the possibility of conservative surgery is discussed in relation to a review of the recent literature.     

Cystic testicular lesions in the pediatric population

PURPOSE: We present the etiology, histological evaluation and management of all cystic lesions of the pediatric testis. MATERIALS AND METHODS: Illustrative cases from our experience are reported with a literature review of all possible diagnoses. RESULTS: Included in the differential diagnosis of cystic testis lesions in children are epidermoid cyst, dermoid cyst, prepubertal teratoma, juvenile granulosa cell tumor, cystic dysplasia of the rete testis, testicular cystic lymphangioma, simple cyst and cystic degeneration after torsion. Testis sparing surgery is feasible in many circumstances. CONCLUSIONS: Cystic lesions of the pediatric testis are rare but represent an interesting group of diagnoses. Patient age at presentation, examination features, tumor markers and sonographic appearance may assist in making a presumptive and occasionally definitive diagnosis preoperatively. Based on the likely diagnosis enucleation or partial orchiectomy may be considered when performed with frozen section histological assessment. A thorough understanding of potentially cystic testis lesions in children leads to the best management choices and often to preservation of a substantial portion of the affected testis.