Testicular germ cell tumors in prepubertal children

Pediatric testicular germ cell tumors are rare. Fifteen children, all less than 5 years of age, were evaluated and treated during February 1987 to July 1996. The median age was 18 months (range, 4-60 months). All were staged according to the Pediatric Oncology Group/Children's Cancer Study Group staging system. Seven patients had stage III disease. Histologically, 9 patients had pure endodermal sinus tumor, 1 had endodermal sinus tumor with embryonal carcinoma, 1 had embryonal carcinoma alone, 2 had immature teratoma, and 2 had mature teratoma. Six children were kept on surveillance. All others received chemotherapy with cisplatin, bleomycin, and vinblastine. The 10-year actuarial overall survival rate was 86.7%.     

Mixed germ cell tumours of the ovary in pediatric age groun

Summary Two cases of mixed germ cell tumours of the ovary in the pediatric age group are reported. The ages of the patients were 11 years and 6 years. The components of the tumours were dysgerminoma and teratoma in one case and dysgerminoma, teratoma, embryonal cell carcinoma and endodermal sinus tumour of Teilum in the other. From the Department of Pathology, Andhra Medical College, Visakhapatnam-2, A.P.     

Endodermal sinus tumor of the nasopharynx and previous mature congenital teratoma

Congenital teratoma of the nasopharynx is a rare tumor that has shown consistently benign behavior. In contrast, endodermal sinus tumor, even more unusual nasopharyngeal germ cell tumor, displays consistently aggressive behavior with most reports documenting early death of the patient. We report the clinicopathological features of a case of endodermal sinus tumor of the nasopharynx that developed in a 3-year-old girl who had a mature teratoma excised from the same site in the neonatal period. Local recurrence of the endodermal sinus tumor was followed by disseminated disease and death within 18 months.     

Endodermal Sinus Tumor of the Nasopharynx and Previous Mature Congenital Teratoma

Congenital teratoma of the nasopharynx is a rare tumor that has shown consistently benign behavior. In contrast, endodermal sinus tumor, even more unusual nasopharyngeal germ cell tumor, displays consistently aggressive behavior with most reports documenting early death of the patient. We report the clinicopathological features of a case of endodermal sinus tumor of the nasopharynx that developed in a 3-year-old girl who had a mature teratoma excised from the same site in the neonatal period. Local recurrence of the endodermal sinus tumor was followed by disseminated disease and death within 18 months.     

Mediastinal Germ Cell Tumors in Childhood

Background: Mediastinal germ cell tumors (GCTs) are rare and usually located in anterior mediastinum. We aimed to review clinical and pathological characteristics of these tumors. Procedure: Between 1973 and 2011, 24 children with mediastinal GCTs were diagnosed. Hospital files were reviewed for presenting complaints, clinical, radiological and other laboratory data, surgical practices, treatments, and outcomes. Results: Median age was 4.5 years (0.2–16) (male/female: 10/14). Most common initial complaints were dyspnea, cough, anorexia/fatigue, fever, and chest pain. Primary tumors were located in anterior mediastinum (n = 22), posterior mediastinum (n = 1), and sternum (n = 1). Thirteen of 24 cases had mature teratomas (54.2%); four (16.7%) endodermal sinus tumor (EST); four (16.7%) immature teratomas; and one (4.2%) each of embryonal carcinoma, teratocarcinoma, and malignant teratoma. Mature teratomas underwent only surgical resection and were under follow-up without disease. Four cases with ESTs received chemotherapy and radiotherapy (n = 3), three underwent surgical resections: three died, one was followed for 284 months in remission. All but one immature teratomas were treated with surgery and all were under follow-up without disease. Two patients with embryonal carcinoma and malignant teratoma didn't undergo surgery; both received chemotherapy and radiotherapy but died with disease. The patient with teratocarcinoma was treated with surgery and chemotherapy but died with disease. Conclusions: No adjuvant therapy is needed for mature teratomas. Immature teratomas must be under close follow-up for recurrences. Prognosis for mediastinal malignant GCTs was poor. These cases need intensive chemotherapies and effective local control measures as surgery –/+ radiotherapy to ensure long-term survival.     

Actual half-life of alpha-fetoprotein as a prognostic tool in pediatric malignant tumors

In a retrospective study, the prognostic value of monitoring the decay of alpha-fetoprotein (AFP) was assessed. Serum AFP was determined serially in 18 children with malignant germ-cell or hepatic tumors: 7 endodermal sinus tumor, 3 embryonal carcinoma, 5 malignant teratoma, 2 hepatoblastomas, and 1 hepatocellular carcinoma. The actual half-life (AHL) of AFP was computed after surgical resection of the tumor. In group 1, which had complete resection and no recurrence during follow-up (n = 13), the AHL of AFP was 4.0 ± 0.9 days. In group 2, which had incomplete resection or recurrence during follow-up (n = 5), the AHL of AFP was 24.8 ± 20 days, significantly longer than that of group 1 (P = 0.0026). The increased AHL of AFP indicated residual active tumor after surgical resection. The AHL of AFP may be more sensitive than serial monitoring of AFP in detecting preclinical recurrence after surgical resection of AFP-secreting tumors. Treatment strategies can be based on AFP clearance, and prospective clinical trials are warranted.     

Teratoma Associated with Endodermal Sinus Tumor

A 3-1/2-year-old girl was diagnosed as having a mediastinal endodermal sinus tumor with pulmonary, bony, and hilar lymph node metastases. Following 7 months of treatment with chemotherapy, thoracic CT (computerized tomogram) scan showed the presence of a residual mass. Upon further surgical exploration the residual mass, which was found to be intrapericardial and arising from the ascending aorta, was resected. This second lesion was shown to be a benign cystic teratoma.

The combination of an intrapericardial teratoma and a mediastinal endodermal sinus tumor in a female child is probably unique. The possible etiology for the combination of these two pathologies is discussed.     

Malignant mediastinal germ-cell tumors in childhood: a report of two cases achieving long-term disease-free survival

Two cases of malignant mediastinal germ-cell tumor are reported. Case 1 is a 20-month-old girl with endodermal sinus tumor (EST) and is, to our best knowledge, the third female pediatric case of mediastinal EST in the literature. Case 2 is a 15-year-old boy with malignant teratoma (mature teratoma with poorly differentiated squamous cell carcinoma). Both cases were treated by primary combination chemotherapy of vincristine, actinomycin D, and cyclophosphamide or vinblastine, cisplatin, and bleomycin, secondary operation (complete resection), radiation to the tumor bed, and some courses of consolidation chemotherapy. Malignant mediastinal germ-cell tumors have been considered to have a poor prognosis, being different from gonadal tumors; however, long-term survivals with no evidence of recurrence were achieved both in case 1 (for 55 months) and in case 2 (for 56 months).     

Clinical manifestations and MRI features of vaginal endodermal sinus tumors in four children

Background

Vaginal endodermal sinus tumor is a rare entity.

Objective

The purpose of this study was to report the clinical manifestations and MRI features in a case series.

Materials and methods

Children with vaginal endodermal sinus tumor admitted to our hospitals between January 2008 and August 2012 were included. MRI was performed in all four children and diffusion-weighted imaging was performed in two children.

Results

Four children, mean age 14 months, were included. All had a history of vaginal bleeding. Serum alpha-fetoprotein was significantly elevated on admission. Relative to muscle, the vaginal masses were uniformly isointense on T1-weighted images, heterogeneously hyperintense on T2-weighted images and heterogeneously enhancing on contrast-enhanced images. The vaginal masses were obviously hyperintense on diffusion-weighted images (b value, 800 s/mm²). Extravaginal invasion was observed in three children. Pelvic lymphadenopathy was noted in two children and pulmonary metastasis was found in one child.

Conclusion

MRI may contribute in the evaluation of vaginal endodermal sinus tumors.     

Endodermal sinus (yolk sac) tumor in infants and children. A clinical and pathologic study: an 11 year review

We reviewed the clinical features, treatment, and results of children with gonadal and extragonadal yolk sac (endodermal sinus) tumors seen in the King Faisal Specialist Hospital and Research Centre between 1976 and 1987. There were nine children (seven girls and two boys) with ages ranging from 7 months to 12 years (median of 3.5 years). Sites of origin included the vagina (two cases), face (two cases), sacrum (two cases), mediastinum (one case), ovary (one case), and testicle (1 case). All children had elevated alpha-fetoprotein (AFP) at diagnosis. One girl had complete surgical excision of an ovarian tumor at the time of diagnosis, and one boy had surgical excision of the testis. In the remaining seven children, the tumor was unresectable. Surgery was limited to a biopsy in six children. All patients received different combinations of chemotherapy, including vincristine (VCR), actinomycin D (Act-D), cyclophosphamide (Cyclo), adriamycin (Adria), bleomycin (Bleo), cis-platinum (CDDP), vinblastine (VBL), and VP-16. Of the nine patients, one was lost to follow-up while in remission, five died, one was lost to follow-up, and three are alive and disease-free at 15, 55, and 67 months from diagnosis. This review demonstrates an unusual preponderance of the extragonadal form of endodermal sinus tumor among our patients.     

小儿性腺外内胚窦瘤3例诊断与治疗分析

目的总结小儿原发性腺外内胚窦瘤的诊断与治疗经验。方法回顾性分析我院2003年10月-2011年9月收治的3例性腺外内胚窦瘤患儿的病历资料。结果术前均行PEB或JEB方案新辅助化疗,根治性手术切除,术后病理均为完全缓解,术后继续化疗。随访3个月～8年,2例无病存活,1例原位复发,经术前化疗、再次手术切除及术后化疗后,现随访25个月,无病存活。结论甲胎蛋白水平对小儿性腺外内胚窦瘤诊断及术后复发监测灵敏度较高,化疗与手术结合治疗效果良好。     

小儿骶尾部内胚窦瘤的治疗

目的探讨骶尾部内胚窦瘤的治疗和预后。方法回顾性分析1999年1月-2005年12月收治12例骶尾部内胚窦瘤患儿。该组病例术前经B超、CT或MRI检查发现骶尾部肿瘤,经检测血清甲胎蛋白均升高,采用手术切除全部或大部分肿瘤,所有患儿均采用BEP（顺铂十足叶乙甙＋博来霉素）方案化疗。结果该组病例术前经B超或CT检查发现骶尾部肿瘤,经检测血清甲胎蛋白（AFP）均升高,采用手术切除全部或大部分肿瘤,病理证实为内胚窦瘤。患儿均采用BEP（顺铂＋足叶乙甙＋博来霉素）方案化疗。随访6年,生存9例,死亡3例,复发5例。结论以顺铂为基础的联合化疗可改善内胚窦瘤疗效,AFP检测及B超联合应用对小儿内胚窦瘤诊断及复发监测有重要意义,选择恰当的手术方式并辅以联合化疗可提高患儿长期生存率。     

Metastatic endodermal sinus tumor: CT appearances

A one-week-old boy had undergone resection of a sacrococcygeal benign cystic teratoma. At the age of 12 months, he had a serum alpha-fetoprotein level of 139,000 IU/ml and a recurrent pelvic mass which was removed, and the microscopic examination revealed endodermal sinus tumor. Postoperatively, massively enlarged inguinal lymph nodes and abdominal distention developed. Computerized tomography displayed enlarged inguinal lymph nodes, metastatic lesions in the liver, and a pelvic recurrent mass. He received BEP (bleomycin, etoposide, cisplatin) chemotherapy regimen, and a complete remission was achieved with a normal serum alpha-fetoprotein. Close follow-up and serum alpha-fetoprotein monitoring are mandatory after the resection of a sacrococcygeal teratoma.     

Influence of tumor site and histology on long-term survival in 193 children with extracranial germ cell tumors

AIMS: Although germ cell tumors (GCT) supposedly share the same cell type of origin, their clinical course differs considerably depending on tumor site and histology. The aim of this work was to study long-term survival stratified for tumor site and tumor histology. MATERIALS AND METHODS: The medical records of 193 consecutive infants and children with extracranial GCT were studied. The GCT arose in the following anatomical sites: sacrococcygeal (n = 70), ovary (n = 66), testis (n = 20), retroperitoneum (n = 12), neck (n = 8), mediastinum (n = 7), and miscellaneous (n = 10). Histological analysis revealed 152 teratomas (mature: 115, immature: 37), 27 yolk sac tumors, 8 mixed tumors, 2 dysgerminomas, 2 gonadoblastomas, 1 choriocarcinoma and 1 embryonal carcinoma. RESULTS: Overall survival (OS) for the whole patient group was 0.91 +/- 0.02, and event-free survival (EFS) was 0.88 +/- 0.02 at ten years. Patients with gonadal GCT had a higher probability of OS than those with extragonadal GCT (p = 0.029). Patients with cervical and mediastinal tumors had a lower probability of EFS than those with gonadal, retroperitoneal or sacrococcygeal GCT (p = 0.018). Patients with choriocarcinoma, embryonal carcinoma, immature teratoma, yolk sac tumor and mixed GCT had a lower probability of EFS than patients with mature teratoma or gonadoblastoma (p < 0.001). CONCLUSIONS: Mortality in children with extracranial germ cell tumors is not only dictated by malignant histology, but also, as in the case of mature teratomas, by occurrence at certain sites.     

Emergence of mature teratoma following treatment of sacrococcygeal endodermal sinus tumor: CT and MR imaging with pathological correlation

We report a case of pseudoretroconversion in which, clinically and pathologically, it appeared that an endodermal sinus tumor retroconverted to mature teratoma. An 8-month-old white female infant with a sacrococcygeal tumor, diagnosed by biopsy as endodermal sinus tumor (yolk sac tumor), was treated with chemotherapy. The residual tumor after treatment was mature teratoma confirmed by imaging and by pathological examination. We present the computerized tomography (CT) and magnetic resonance imaging (MRI) findings with pathological correlation.     

Primary testicular tumors in childhood. Observations in 21 cases and a discussion of the necessity of metastasis prevention]

A retrospective analysis of 21 primary testicular tumors in childhood is presented. Histologic review revealed 4 differentiated teratomas, 14 yolk sac tumors, 1 rhabdomyosarcoma of testicular envelopes and 1 paratesticular sarcoma. One tumor could not be classified. Two patients with yolk sac tumor and the patient with the paratesticular sarcoma died. In 4 of the 14 patients with yolk sac tumor iliac and/or paraaortic lymphnodes were removed 8-15 days after hemicastration but no tumor cells were found. Of 3 children with yolk sac tumor who developed metastases, one had had prophylactic resection and another one prophylactic irradiation of the draining lymphnodes. 8 patients with yolk sac tumor received prophylactic chemotherapy, and none developed metastases. For patients with yolk sac tumor prophylactic chemotherapy is indicated, particularly if more than 2 months have elapsed between the first detection of a testicular mass and operation. In prepubertal boys with testicular teratoma no prophylactic therapy to prevent dissemination is necessary. Patients with yolk sac tumor have an age distribution comparable to that of patients with an embryonal tumor.     

Testicular tumors in children and adolescents

ObjectiveTo analyze the spectrum of testicular tumors in children in an unselected population-based series, as well as the results of testis-preserving surgery.Patients and methodsOur hospital database was analyzed for operations for testicular tumors from 1981 to 2006. The clinical data and findings during follow up (4.7 years) were recorded.ResultsThirty-four patients were operated on because of testicular tumors. In 23 (68%) the tumor was benign: benign teratoma (16), Leydig-cell tumor (2), epidermoid cyst (2), Sertoli-cell tumor (1), cystic dyplasia (1), intratesticular focal fibrosis (1). Eleven patients (32%) had a malignant tumor: yolk-sac tumor (6), embryonal carcinoma (5). Twenty out of the 26 (77%) prepubertal boys had a benign tumor in contrast to only three of the eight (38%) adolescent males (P = 0.079). Testis-preserving surgery was performed in 10 patients. In eight, the tumor was curatively excised and remaining testis preserved. Two patients with benign teratoma had a recurrence due to incomplete primary resection. In one patient who underwent orchiectomy for benign teratoma, two metachronous teratomas were detected in the contralateral testis 6 years after primary surgery.ConclusionsIn children, most testicular tumors are benign, especially before puberty. If testis-preserving surgery is contemplated, complete excision of the tumor should be ascertained. The possibility of metachronous bilateral tumors should be considered in the follow up of testicular teratomas.     

儿童内胚窦瘤临床治疗研究

目的 探讨儿童内胚窦瘤的治疗与转归。方法 回顾性分析2000 年4 月至2013 年7 月收治的12 例内胚窦瘤患儿的临床资料。其中男7 例,女5 例;11 例年龄在1~3.3 岁之间,1 例11 岁。Ⅰ期2 例,Ⅱ期4 例,Ⅲ期2 例,Ⅳ期4 例。1 例采用单纯手术,1 例采用手术联合VAC（长春新碱+ 放线菌素D+ 环磷酰胺）方案化疗,10 例采用手术联合以PEB（顺铂+依托泊苷+博来霉素）方案为主的化疗。结果 成功随访11 例,目前存活10 例,生存期为4.5~66 个月。10 例接受了PEB 方案化疗的患儿,8 例达到完全缓解,1 例达到部分缓解,1 例失访。PEB 方案治疗并发症主要包括骨髓抑制及胃肠道反应,未发现远期并发症。结论 手术联合PEB 化疗方案是有效、安全的治疗儿童内胚窦瘤的方法,但需积累更多病例进一步证实。     

Endodermal sinus tumor. Histological changes induced by chemotherapy

The case of a child affected at birth sacrococcygeal teratoma is reported. Twenty-eight months following surgical resection, the tumor relapsed locally and liver metastases occurred. A biopsy of the sacrococcygeal mass was performed and histologic examination proved it to be an endodermal sinus (yolk sac) tumor. Chemotherapy consisting of etoposide and high-dose carboplatin was started. When a second operation was performed the mass had disappeared and the macroscopically involved areas of liver were removed. Histologic examination demonstrated that the complete necrosis of the tumor was partially replaced by a fibrous scar tissue. The child underwent adjuvant chemotherapy and now is in complete remission fourteen months after the last operation.     

小儿纵隔肿瘤的CT影像和临床分析

目的 研究小儿从隔肿瘤的ＣＴ诊断特征和临床特点。方法 １５例小儿纵隔肿瘤、其中神经母细胞瘤５例,神经节细胞瘤３例,神经鞘瘤１例,胸腺瘤及畸胎瘤各２例,淋巴管瘤和内胚实瘤各１例。各地术前、化疗前行胸部ＣＴ平扫和增强扫描,３例神经线细胞瘤治疗前、后作尿ＶＭＡ测定和同步ＣＴ扫描。结果 畸胎瘤和胸腺瘤位于前纵隔,神经源性肿瘤和内胚窦瘤发生于后纵隔。神经母细胞瘤瘤呈呈浸润性生长、钙化、破坏及骨质破坏多见,胸