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-sparing surgery for the prepubertal testicular tumor. Experience of two cases with large cell calcifying Sertoli cell tumors.

PURPOSE: We review prepubertal germ cell tumors of testis in our institute and the Japanese registry and present 2 cases with a large cell calcifying Sertoli cell tumor (LCCSCT) and discuss the possibility of testis-sparing surgery. MATERIALS AND METHODS: Incidence, age, pathology and clinical stages of prepubertal germ cell tumors are surveyed for 30 years at our department and 10 years of the malignant tumor registry of the Japanese Society of Pediatric Surgery. Two representative prepubertal boys with LCCSCT are presented. One of them was treated by partial orchiectomy. RESULTS: The majority of testicular germ cell tumors in the prepubertal age were composed of embryonal cell carcinoma/yolk sac tumors or teratoma, occurred in preschool age, were limited to clinical stage I and did not metastasize irrespective of histology. Benign behavior which included recovery from hormonal derangement, no tumor recurrence and negative antisperm antigen was observed in 2 cases with LCCSCT who underwent either radical orchiectomy or partial orchiectomy. CONCLUSION: Partial orchiectomy should be considered as a standard option in prepubertal schoolboys with a testicular mass if surgically feasible. This surgical treatment is safe and preserves fertility and is psychologically advantageous. It is not recommended for yolk sac tumors that may recur, however they are rare in prepubertal boys and can be differentiated preoperatively by prudent evaluation.     

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 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.     

Germinal testicular tumors in childhood. Report of observations and literature review

1,124 case observations, published from 1955 to 1981, and 45 of our own cases of childhood testicular tumor are evaluated. The age distribution, histology (WHO classification), stage and prognosis are compared to 1,062 adult cases. Diagnostic procedures are itemized. Priorities in the therapeutic approach and the effectiveness of various methods, depending on stage and histology, and toxic side effects and other complications are documented. In children, 29% of childhood testicular tumors are non-germinal, as compared to 8% in adults. 49% are yolk sac tumors. The age distribution differs depending on histology. Metastases occur less frequently (9%) than in adults (61%). Dissemination is predominantly hematogenic. Prognosis is best in teratoma which is cured by orchiectomy. Yolk sac tumor limited to the testicle, in infants less than 2 years old, is sufficiently treated with orchiectomy alone. Older children require adjuvant chemotherapy. Overall, chemotherapy was indicated in 15% of the evaluated cases of childhood testicular malignoma.     

CLINICAL EXPERIENCE WITH YOLK SAC TUMORS AND TERATOMA IN CHILDREN

Between 1971 and 1993, 12 children with testicular germ cell tumors were treated at the Department of Urology, Faculty of Medicine, Kyoto University. Seven patients had yolk sac tumors and 5 had mature teratoma. Of the 7 patients with yolk sac tumors, 6 had stage I and 1 had stage III tumors. Initial management of the stage I tumors consisted of high orchiectomy in 5 patients and high orchiectomy plus retroperitoneal lymph node dissection in 1 patient. Of these 6 patients, 4 were cured by surgery alone but lung metastases developed in the other 2 patients. One of them was salvaged with thoracotomy and chemotherapy but the other died of tumor. The patient with stage III tumor had bulky tumor spread to lung and retroperitoneum, but seems to have been cured by chemotherapy followed by resection of the residual mass although follow-up is still inadequate (14 months). Six of the 7 patients (85.7%) are alive 13 months to 21 years after diagnosis. Five patients with mature teratoma were treated by high orchiectomy or, more recently, enucleation and all are alive 4 months to 22 years after surgery.     

Yolk sac tumor of the testis in children: report of two cases]

Two cases of yolk sac tumor of the testis are presented. The patients were 17 months and 24 months old. The children were inflicted with painless swelling of their left scrotal content. alpha-Fetoprotein levels were elevated at presentation but decreased within normal limits after orchiectomy. Chest X-rays and CT scans were negative. The cases were diagnosed as stage I. Fifty six cases of testicular yolk sac tumor in children have been reported in Japan since 1981. There were no recurrent stage I cases. One patient with stage II and 3 patients with stage III died despite chemotherapy, while three children with stage II or stage III disease survived more than 36 months after a positive response to chemotherapy. We conclude that prepubertal stage I yolk sac tumor is treated best initially by orchiectomy alone. Aggressive chemotherapy has a major role in salvage of stage II or stage III patients.     

Testicular tumours in infancy and children

Introductionprepubertal testicular tumours are different from those that appear during adulthood. Traditionally, they were considered to be malignant, however benign testicular tumours are actually more frequent at this age.Materials and methodswe analysed our experience in the treatment of testicular tumours in children ≤ 13, with the intention of evaluating the use of partial orchiectomy. From 1984 to 2008, we diagnosed and treated 15 testicular tumours in children at our centre. We examined the therapeutic approach employed, underlining the possibility of testicular conservation in selected patients and we have analysed the results.Resultsthe clinical presentation in 80% of the cases was an increase in testicle size with palpable mass. We performed 4 radical orchiectomies (27%) and 11 tumourectomies (73%). All the benign lesions in the final pathological anatomy were treated with tumourectomy: four epidermoid cysts, one hemangioma, one lipoma, one fibrous hamartoma, one juvenile granulosa tumour and one splenogonadal fusion. We also successfully and conservatively treated two cases of teratoma. The cases that received radical treatment were a yolk sac tumour (Stage I), two mixed germ cell tumours and one gonadoblastoma.Conclusionsthere are more cases of benign testicular tumours than malignant tumours during puberty. In the event of a palpable testicular mass with negative tumour markers, conservative treatment by means of a tumourectomy may be considered. However, the lesion must be removed completely to prevent recurrence.     

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.     

Experience with testis sparing surgery for testicular teratoma

PURPOSE: Testicular teratoma is a rare neoplasm affecting the pediatric population and has classically been reported to be the second most common testis tumor in children behind yolk sac tumors. Testicular teratomas are benign and partial orchiectomy may be considered. We describe our single institution experience with testicular teratoma and definitive treatment with testis preserving surgery. MATERIALS AND METHODS: We reviewed the pathology records at our institution for all testicular and paratesticular tumors diagnosed between 1976 and November 2002 in males younger than 18 years. We specifically examined the prepubertal incidence of teratoma, including epidermoid cysts, and our experience with testis preserving surgery. Preoperative and postoperative ultrasonography images were used to calculate the atrophy index following surgery. Patients were contacted for long-term followup. RESULTS: Of 77 primary testicular and paratesticular tumors 38 were diagnosed in prepubertal boys (age younger than 13 years) including 11 mature teratomas and 5 epidermoid cysts. Mean patient age at treatment was 34.4 months (range 4 months to 10 years). All boys presented with a painless scrotal mass, cystic foci within an intratesticular mass on ultrasound and a normal alpha-fetoprotein level. Of the 16 boys with benign teratomas 13 (81%) were treated with a testis sparing procedure. At a mean 7-year followup no patient has presented with recurrent tumor in the ipsilateral or contralateral testicle. Postoperative physical examination and scrotal ultrasound were obtained in 9 patients at a median followup of 10.2 months, and there was no evidence of testicular atrophy or persistent discomfort. CONCLUSIONS: Unlike previously published series based on tumor registries, benign teratoma was the most common pediatric testicular tumor treated at our institution. Our single institution experience with testis preservation and long-term followup confirms the role and safety of this technique. Testis sparing surgery remains our technique of choice for testicular teratoma.     

Prepubertal testicular tumors

Summary Testicular tumors in prepubertal boys differ in type and behavior from those seen in adults. Teratomas and yolk sac tumors are the commonest histologic types seen. Teratomas before puberty behave benignly but yolk sac tumors are malignant. The management of yolk sac tumors is in flux and there is little reliable data upon which to base management. A pre-pubertal testicular tumor registry established by the Section on Urology of the American Academy of Pediatrics has provided preliminary data on over 160 boys with testicular tumors, 2/3 of which are yolk sac tumors. Some of this data is included in this overview of prepubertal testicular tumors.     

小儿睾丸卵黄囊瘤合并鞘膜积液诊治分析(附7例报告)

目的:提高小儿睾丸卵黄囊瘤的诊治水平,探讨小儿睾丸卵黄囊瘤合并鞘膜积液的临床特点及其中关联。方法:回顾性分析2008年9月至2012年4月收治7例睾丸卵黄囊瘤合并大量鞘膜积液患儿的临床资料。7例均初步诊断为睾丸卵黄囊瘤,临床Ⅰ期。术中快速病理证实为卵黄囊瘤后,行根治性高位精索睾丸切除术。7例术后随访时间3⁴1个月,按术后第1年每个月;第2年每3个月;第3年每6个月随访。内容包括常规体检、血清甲胎蛋白(AFP)、胸片、B超及CT。结果:7例术后病理均证实为睾丸卵黄囊瘤,未累及精索切缘端。6例术后1个月内血清AFP降至正常,诊断为临床Ⅰ期,未行化疗,无复发、转移;1例术后1个月血清AFP116μg/L,诊断为临床Ⅱ期,予PVB方案化疗,术后3个月失访。结论:小儿睾丸卵黄囊瘤合并鞘膜积液易于误诊,应常规检查B超。Ⅰ期患儿可单纯行根治性高位精索睾丸切除术,术后需密切随访;Ⅱ期患儿术后辅以联合化疗。目前尚无证据支持小儿睾丸卵黄囊瘤与鞘膜积液存在关联,其预后与同期病例相仿。     

Leydig cell tumor of testis.

In adult patients with Leydig cell tumor of the testis, endocrinologic signs occur in 30 per cent of the cases and often precede the onset of a palpable testicular mass. Gynecomastia is the most common endocrinologic manifestation and probably is due to increased estrogen secretion by the Leydig cells. In the patient with adrenogenital syndrome and testicular enlargement it is difficult to distinguish Leydig cell tumor from adrenal rest hypertrophy. Four patients with Leydig cell tumor and endocrinologic manifestations are discussed; three are adults who presented with gynecomastia and the fourth is a patient with congenital adrenogenital syndrome. In the adult patient inguinal orchiectomy is the treatment of choice, while in the patient with adrenogenital syndrome initial management by high-dose steroid suppression should be attempted prior to testicular exploration.     

Testicular tumors in prepubertal children

A clinical analysis was made of 556 prepubertal children with testis tumors. Germinal tumors comprised 76 per cent of this group, and the most common of these tumors was the yolk sac carcinoma. Although many appellations have been used to describe this tumor, experimental and clinical evidence support the use of this term. The over-all two-year survival was 72 per cent and was better in children less than two years of age. The tumor is distinctly different from the embryonal carcinoma of the adult. The role of lymphadenectomy needs to be reassessed in view of the low incidence of lymph node metastases and the propensity for pulmonary metastases. Chemotherapy appears to be of benefit. Tumor markers, particularly alpha-fetoprotein, may be of assistance in planning therapy.The other germinal tumor is the teratoma. This is a benign, nonmetastasizing disease in children and requires only orchiectomy.Nongerminal tumors comprised 24 per cent of tumors and require only orchiectomy. Sertoli cell tumors are rare, and there has been only one report of metastatic disease. Leydig cell tumors present with endocrine manifestations and are benign in children. They may be difficult to distinguish from tumors associated with congenital adrenal hyperplasia.Rhabdomyosarcomas should be treated with a combination of surgery, chemotherapy, and radiation. The over-all survival of 74 per cent for these patients indicates the benefits of aggressive therapy.     

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.     

Organ-sparing surgery for bilateral leydig cell tumor of the testis

Leydig cell tumors of the testis are uncommon, and bilateral lesions are extremely rare. We report a case of bilateral Leydig cell tumor of the testis treated with radical orchiectomy and contralateral subtotal orchiectomy with the intent of preservation of hormonal function and fertility.     

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.     

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.     

Testicular neoplasms in the prepubertal male

Unlike adult neoplasms, prepubertal testicular tumors are frequently benign. Yolk sac tumors, which are always malignant, constitute the majority among the histological types of neoplasm reported in the literature. Teratomas are benign in the pediatric population and are the second-most commonly reported testicular tumors. The remaining tumors are exceedingly rare and almost always benign; however, juvenile granulosa cell tumors, undifferentiated stromal tumors, and Sertoli cell tumors may rarely possess metastatic potential. Therefore, testicular neoplasms are varied, rare and often benign. However, even when they are malignant, long-term survival is excellent with prompt diagnosis and treatment. Following an English-based Medline literature search, this article reviews a concise approach towards the management of these individual tumors based on an analysis of the literature in the English language that addresses testicular neoplasms in prepubertal males.     

Testis risk and prognostic factors. The pathologist's perspective.

Pathologic analysis is invaluable in defining the levels of risk for the development of a testicular tumor or for metastasis in a patient with an established testicular cancer. The identification of IGCNU in testicular biopsies defines a group of patients at high risk for subsequent invasive germ cell tumor unless they are treated by orchiectomy or radiotherapy. This method for defining the risk for the development of a testicular tumor is not effective in prepubertal patients, except for those with intersex syndromes. Pathologic analysis of testicular germ cell tumors in patients with clinical stage I disease may allow their stratification into high- and low-risk groups for occult metastases. This would provide a rational basis for recommending intervention or surveillance, respectively. The precise classification of postchemotherapy lesions permits an assessment of the patient's risk for subsequent recurrence and progressive tumor.