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.     

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.     

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 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.     

Präpubertäre Hodentumoren

Based on findings from the Prepubertal Testis Tumor Registry by the Urologic Section of the American Academy of Pediatrics and collaborative data in the literature, a modern algorithm for the surgical management of prepubertal testis tumors is presented. Following testicular surgery, patients with universally benign tumors, such as teratoma, may be released from oncological follow-up. Children with stage I yolk sac tumors should be monitored closely with periodic AFP tumor marker evaluation and imaging according to the primary dissemination (e.g., ultrasound, chest x-ray, and computed tomography). Patients with recurrent or metastatic yolk sac tumors should be treated with platinum-based chemotherapy and appropriate follow-up. Retroperitoneal lymph node dissection is not recommended except for patients with residual retroperitoneal masses following chemotherapy. Aggressive treatment is warranted for metastatic Sertoli cell and metastatic undifferentiated stromal tumors.     

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.     

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.     

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.     

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.     

Epidemiological and clinical behavior of prepubertal testicular tumors in Korea

PURPOSE: To our knowledge there is no multi-institutional report on prepubertal testicular tumors in Korea to date. We obtained demographic data for a better understanding of the biological behavior and optimal management of these tumors. MATERIALS AND METHODS: The prepubertal testicular tumor registry form was mailed to all 87 hospitals registered in the Korean Urology Association. We retrospectively reviewed recent 5-year medical records. RESULTS: A total of 209 patients were enrolled in this registry. The incidence was 0.98/100,000 children. Age was 1 to 142 months (median 18). Most patients were diagnosed with a scrotal mass before age 4 years. Serum alpha-fetoprotein and beta-human chorionic gonadotropin increased in as many as 62.9% and 2.7% of patients, including 94.7% and 2.2% in those with yolk sac tumor and 30.4% and 2.7% in those with teratoma, respectively. While potentially malignant tumors accounted for 52.5% of patients, the remainder were benign. Germ cell tumors were the most common (89.4% of cases), mainly with yolk sac tumor (47.8%) or teratoma (39.7%). Management after surgery included surveillance in 71.8% of cases, chemotherapy in 9.1%, combination therapy in 1.4% and other in 17.7%. Of the total patients 10.5% (5.9% of stage I yolk sac tumors) had progression to metastasis. The final results of treatment were complete remission (64.6% of cases), incomplete remission (2.9%), no response or disease progression (1.4%) and unknown (31.1%). Outcomes at the last followup (average 23.5 months) were 76.1% of patients alive, 0.9% dead and 23.0% of unknown status. CONCLUSIONS: Demographic data on pediatric testicular tumors in Korea will lead to a better understanding of these rare tumors and to optimal therapy in these children.     

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.     

Histology in mixed germ cell tumors. Is there a favorite pairing?

PURPOSE: Mixed germ cell tumors account for approximately 30% to 50% of testicular tumors. To our knowledge a systematic review with statistical analysis of the associations of histological subtypes in mixed germ cell tumors has not been done previously. It was our impression that such associations exist. Delineating concordant histological types may provide insight into the ontogeny of testicular tumors and also have important clinical implications. MATERIALS AND METHODS: We retrospectively reviewed the testis cancer data base at our institution. The primary tumor of orchiectomy specimens was examined in 2589 patients. Of these patients mixed histology was noted in 1765 (68.2%). ORs were calculated for all possible combinations of teratoma, embryonal carcinoma, yolk sac tumor, choriocarcinoma and seminoma. In addition, we evaluated the association of various histological types with teratoma at post-chemotherapy retroperitoneal lymph node dissection. RESULTS: Of 10 possible combinations of histological types in the primary tumor, positive correlations were noted in 4. The strongest correlation was found between teratoma and yolk sac tumor (OR 2.58, p <0.001). Teratoma or yolk sac tumor in the testis was associated with teratoma in the pathology specimen at post-chemotherapy retroperitoneal lymph node dissection. CONCLUSIONS: The strongest associations of histological subtypes in mixed germ cell tumors were seen between yolk sac tumor and teratoma. Similar associations are seen in late relapse and in some cases of prepubertal tumors. Further study of these associations may prove valuable in understanding the biology and clinical behavior of germ cell tumors.     

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.     

Current management of prepubertal yolk sac tumors of the testis

Over the last 3 decades, the management of pediatric yolk sac tumors of the testis has changed significantly. These changes reflect improvement in the treatment of testicular tumors in adults and children, such as the advent of platinum-based chemotherapy regimens. They also reflect increased understanding of the biology and natural history of prepubertal yolk sac tumors of the testis as a unique disease entity.     

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。     

Stromal testis tumors in children: a report from the prepubertal testis tumor registry.

PURPOSE: Stromal testis tumors are rare and generally exhibit a benign behavior in prepubertal patients. We reviewed the Prepubertal Testis Tumor Registry to elucidate further the behavior of these tumors. MATERIALS AND METHODS: Epidemiological and clinical information on stromal testis tumors was compiled and reviewed from the Prepubertal Testis Tumor Registry. In addition, original pathology reports were requested for all patients registered as having undifferentiated stromal tumors. RESULTS: There were 43 patients registered with stromal tumors. Of the 21 patients with unspecified stromal tumors pathology reports were obtained on 11. Eight patients had truly mixed or undifferentiated stromal tumors. Mean patient age at presentation was 38 months (Leydig cell 70, Sertoli cell 52.5, juvenile granulosa cell 1.5 and mixed/undifferentiated 41.2). No patient with a Leydig cell, Sertoli cell or juvenile granulosa cell tumor had metastases at presentation or metastatic disease during an average 24.6 months of followup. One undifferentiated tumor demonstrated malignant behavior by presenting with metastatic disease. Pathological examination revealed a poorly differentiated tumor with extension into the adjacent tunica and frequent mitotic figures. While other stromal tumors displayed mitotic figures, none showed local invasion. CONCLUSIONS: Stromal testis tumors are rare. Data from the Prepubertal Testis Tumor Registry confirms the benign behavior of most of these tumors. However, undifferentiated stromal tumors may exhibit metastatic behavior. A high index of suspicion is appropriate when there are a large number of mitotic figures, the tumor is poorly differentiated or when local invasion is present in the primary tumor. Metastatic evaluation and close followup are warranted for this select group of patients.     

Retroperitoneal germ cell tumor with testicular calcification indicating tiny testicular origin: consideration of the origin of retroperitoneal germ cell tumors: report of two cases

Two cases of germ cell neoplasm retrospectively considered to have been of testicular origin are reported. Case 1. A 19-year-old male with brain, liver and retroperitoneal tumors was diagnosed with yolk sac tumor by retroperitoneal tumor biopsy. After multidisciplinary treatment, a region of calcification was detected in the left testis on scrotal sonography and left high inguinal orchiectomy was performed. Case 2. A 57-year-old male with neck, lung and retroperitoneal tumors was diagnosed with yolk sac tumor by supraclavicular biopsy. From initial examination, scrotal sonography revealed a small calcified lesion in the right testis. After chemotherapy, high inguinal orchiectomy and retroperitoneal lymphadenectomy were simultaneously performed. Pathologic evaluation of these testicular specimens revealed calcification and a fibrous scar in correspondence with the clinical diagnosis. These changes were considered as scars of the primary testicular tumor due to burned-out tumor or the result of reaction to chemotherapy. Since a primary tumor of testicular origin may exist in the extragonadal germ cell tumor, it is important to examine the intrascrotal contents in detail in the case of so-called extragonadal germ cell tumors with palpably normal testes. In such cases, there are two possible conditions, an occult testicular tumor and a burned-out testicular tumor. We briefly reviewed 42 such cases in the Japanese literature. It appears that there are very few true extragonadal germ cell tumors, and that the possibility of primary testicular origin metastasizing from viable occult testicular tumor or burned-out testicular tumor with spontaneous regression is high in retroperitoneal germ cell tumors.     

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.     

A case of mediastinal yolk sac tumor successfully treated with chemotherapy and surgery

A 28-year-old man was admitted due to increasing respiratory symptoms. X-ray examination of the chest showed a tumor mass in the anterior mediastinum with possible invasion into the chest wall and upper lobe of right lung. No tumor was found in the testis. Serum alpha-fetoprotein (AFP) concentration was 6400 ng/ml. Serum levels of CEA and HCG were within normal limits. Percutaneous biopsy of the tumor strongly suggested yolk sac tumor with an evidence of AFP by an immunoenzyme labelling technique. The serum AFP rapidly decreased after two courses of combination chemotherapy. En bloc resection of the tumor was successfully performed and third chemotherapy was added. Mediastinal yolk sac tumors should be treated with combination chemotherapy and surgical resection.     

The problems in making a diagnosis and arriving at a therapeutic procedure in a patient with retroperitoneal volk sage tumor. Case report and review of the literature

Summary The case of a 26 years old patient with a primary paraaortic extragonadal yolk sac tumor is presented. After operative reduction of the tumor polychemotherapy with PEB was performed. Following chemotherapy testicular biopsy did not show histological changes typical for testicular intraepithelial neoplasia (TIN). Three years after the initial manifestation of the yolk sac tumor, a malignant tumor of the testis with components of undifferentiated teratoma and yolk sac tumor was observed. In this paper the problems of development of testicular malignancy after polychemotherapy and the validity of a small exploratory excision of testicular tissue in retroperitoneal germ cell tumors and following chemotherapy are discussed.