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.     

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.     

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.     

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.     

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.     

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 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.     

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.     

Serum lactate dehydrogenase and its isoenzymes in men with maldescended testes

Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were determined as a screen for testicular germ cell neoplasia in 130 men with maldescended testes. A testicular tumor was found on clinical examination in 1 patient, which was revealed to be embryonal carcinoma, teratoma, yolk sac tumor and carcinoma in situ on orchiectomy. Subclinical testicular germ cell neoplasia was found on testicular biopsy in 3 men (1 with microinvasive seminoma and 2 with carcinoma in situ). These 4 patients had normal serum lactate dehydrogenase and serum lactate dehydrogenase isoenzymes. Elevated serum lactate dehydrogenase was noted in 3 men without testicular germ cell neoplasia: 1 had predominantly increased serum lactate dehydrogenase isoenzymes 1 to 3 and 2 had slightly increased serum lactate dehydrogenase isoenzymes 3 and 4. Serum lactate dehydrogenase and lactate dehydrogenase isoenzymes were not sensitive to detect testicular germ cell tumors in a subclinical stage.     

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.     

Glypican 3: a novel marker in testicular germ cell tumors

Glypican 3 (GPC3), a membrane-bound heparin sulfate proteoglycan, may play a role in promoting embryonic cell growth and differentiation. GPC3 is mutated in Simpson-Golabi-Behmel syndrome, characterized by tissue overgrowth and an increased risk of embryonal malignancies. Recently, GPC3 was reported to be one of the over-expressed genes in testicular yolk sac tumors by gene expression microarray analysis. However, the presence of the GPC3 protein in germ cell tumors has never been investigated. The purpose of the study was to investigate the GPC3 expression in various histologic components of testicular germ cell tumors using immunohistochemistry and to assess its possible utility as a diagnostic marker. Tumors from 71 patients were examined, including components of 42 seminomas, 37 embryonal carcinomas, 24 yolk sac tumors, 20 teratomas with mature elements, 16 teratomas with immature elements, and 7 choriocarcinomas. Cytoplasmic and membranous immunoreactivity was semiquantitatively evaluated. All yolk sac tumor (24/24) and choriocarcinoma (7/7) components were immunoreactive for GPC3, whereas only 38% of teratomas with immature elements and 8% of embryonal carcinomas expressed GPC3. There was no immunoreactivity in benign testicular tissue, intratubular germ cell neoplasia, seminomas (0/42), or teratomas with mature elements (0/20). We conclude that the oncofetal protein GPC3 is a novel immunohistochemical marker in testicular germ cell tumors with differential expression in defined histologic subtypes. Our findings suggest a possible role of GPC3 in tumor cell differentiation. Furthermore, GPC immunostaining may be useful in the pathologic diagnosis of nonseminomatous germ cell tumors, particularly yolk sac tumor, and choriocarcinoma.     

Diffuse embryoma of the testis. A distinctive form of mixed germ cell tumor

Two testicular tumors characterized by a diffuse, orderly arrangement of embryonal, yolk sac, and trophoblastic elements are described as examples of a newly recognized form of mixed germ cell neoplasia. In one case, ribbons of embryonal carcinoma and yolk sac tumor wound around one another to create a distinctive necklace pattern. In the second case, differentiation of the yolk sac component was more advanced with the formation of numerous clusters of cells resembling hepatocytes. Tumors with these patterns are appropriately designated diffuse embryomas to distinguish them from polyembryomas and other forms of malignant mixed germ cell tumor.     

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.     

The pathology of late recurrence of testicular germ cell tumors

A total of 91 men had histologically documented late recurrences of testicular germ cell tumors characterized by a complete response to treatment with a subsequent disease-free interval of at least 2 years and no evidence of a second primary lesion. Ninety percent of the patients for whom information was available received chemotherapy shortly after their initial diagnosis of testicular germ cell tumors; most of the other patients were known to have stage I disease initially. Overall, 60% of patients had teratoma in their late recurrences, including 20 patients (22%) in whom teratoma was the only element. Thus, teratoma was the most common type of neoplasm in late recurrences. Excluding teratoma coexisting with other types of neoplasms, yolk sac tumor was the most frequent type of tumor in patients with late recurrence. It occurred in 47% of patients, either alone or with teratoma, another nonteratomatous germ cell tumor type, or a "nongerm cell malignant tumor." Unusual types of yolk sac tumor, including glandular, parietal, clear cell, and pleomorphic patterns, were seen frequently in late recurrences and often raised differential diagnostic problems with "nongerm cell" carcinomas. A smaller number of late recurrences consisted of other types of neoplasms. Twenty percent of patients with late recurrence had a nonteratomatous germ cell tumor other than yolk sac tumor, either alone, with yolk sac tumor, or with a "nongerm cell malignant tumor." Most of these nonteratomatous germ cell tumors other than yolk sac tumor were embryonal carcinoma, although rarely seminoma and choriocarcinoma were encountered. "Nongerm cell malignant tumors," including both sarcomas and carcinomas of various types, occurred in 23% of late-recurrence patients, either alone or with a nonteratomatous germ cell tumor. Late recurrences were seen in many different sites in these patients, including the retroperitoneum, abdomen, pelvis, liver, mediastinum, lung, bone (femur, vertebra, and rib), lymph nodes outside the retroperitoneum and mediastinum (supraclavicular, neck, and axillary regions), scrotum and inguinal regions, adrenal gland, chest wall, and buttocks. Follow-up data were available for 79 of the 91 patients studied. Duration of follow-up ranged from 2 months to 13 years after the patient's first late recurrences; the mean length of follow-up was 4.8 years. Patients whose late recurrences consisted of teratoma only had the most favorable outcomes, with 79% having no evidence of disease at last follow-up. Patients whose late recurrences consisted of pure "nongerm cell malignant tumor" or pure germ cell tumor (yolk sac tumor or other types) had a much worse prognosis: Only 36% to 37% were alive with no evidence of disease. Patients with two different types of nonteratomatous malignancies in their late recurrences had a dismal clinical course: Only 17% with both yolk sac tumor and other nonteratomatous germ cell tumor had no evidence of disease, whereas no patient with both nonteratomatous germ cell tumor and "nongerm cell malignant tumor" was disease free. Late recurrences consisting of teratoma alone often have a favorable outcome, but the prognosis in all other patients is poor. Furthermore, late recurrence is not likely to respond to chemotherapy and is best treated by surgical excision when possible.     

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.     

睾丸原发性卵黄囊瘤临床病理分析(附8例报告)

目的:探讨睾丸原发性卵黄囊瘤的临床病理特征、诊断、治疗及预后。方法:运用光镜、免疫组化对8例睾丸原发性卵黄囊瘤进行检测。结果:8例睾丸原发性卵黄囊瘤来自我院1998~2013年诊治的病例(2例为外院会诊病例),占我院同期睾丸生殖细胞肿瘤的10.7%(8/75),患者年龄7~43岁,平均23.9岁,8例患者临床表现均为患侧睾丸无痛性肿大,均发生于单侧睾丸。组织学:全部病例肿瘤组织均见微囊或网状结构和嗜酸性透明滴,而作为本瘤结构的S-D小体有5例。8例中仅1例为单纯性卵黄囊瘤,其余7例均为混合性卵黄囊瘤。免疫表型:AFP为其特征性标记物。结论:原发于睾丸的卵黄囊瘤是罕见的恶性肿瘤,术前AFP检测有助诊断,确诊依赖于病理检查。以手术加放、化疗的综合治疗措施,可以延长生存期。     

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.     

Testicular seminoma occurring 8 years after treatment of a metastatic extragonadal germ cell tumor

A 30-year-old man was admitted with a chief complaint of left-sided scrotal enlargement, and was diagnosed as having testicular seminoma after orchiectomy. Eight years earlier, he had been treated with chemotherapy for an extragonadal germ cell tumor, without orchiectomy, leading to complete remission. His histological diagnosis at that time was a germ cell tumor, composed of choriocarcinoma and embryonal carcinoma. He was followed up without testicular biopsy. Routine pretreatment testicular biopsy in patients with extragonadal germ cell tumor is controversial, but regular long-term follow up and information on the risk of developing a metachronous testicular tumor are needed after treatment of extragonadal germ cell tumors, even when there seems to be a partial or complete clinical response.     

Clinical features of testicular tumors in children

AIM: Testicular tumors are not common pediatric solid tumors, especially in Asian children. There have been few reviews of cases in Japan to date. We present the clinical features of 14 pediatric testicular tumor patients. METHODS: Clinical features of 14 testicular tumor patients, such as chief complaints, age at diagnosis, pathology, stages, treatments and prognosis, were examined from medical records. Two patients had their semen tested at adolescence. RESULTS: Of the 14 prepubescent patients, 12 (85.7%) patients were diagnosed before 3 years of age. Ten cases (71.4%) were diagnosed as yolk sac tumors, three (21.4%) as mature teratomas and one case as an epidermoid cyst. Nine cases (90.0%) among the 10 cases of yolk sac tumor were diagnosed as stage I and one case was stage IV. One stage I yolk sac tumor patient developed lung metastasis later. Eventually, two yolk sac tumor patients died, despite chemotherapy. While all the cases with a diagnosis before 2 years of age survived, 67% (2/3) of cases with a diagnosis after the age of 2 died of tumors. Semen analysis in two patients showed normospermia. CONCLUSION: In the present study, the most common testicular tumors were yolk sac tumors and the patients diagnosed before 2 years of age showed favorable results. Age could be a relapse risk factor in yolk sac tumors. Guidelines for handling testicular tumors in children is not yet well established in Japan. An organized system seems necessary to gather and accumulate the results of the cases in Japan in order to develop better guidelines for treatment.