Intrarenal mixed germ cell tumor.
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A child treated with chemotherapy for disseminated yolk sac tumor had a residual retroperitoneal mass after normalization of serum alpha-fetoprotein. The mass was excised and histologically showed only fibrous tissue, necrosis and calcification. To our knowledge this is the first case reported of a prepubertal yolk sac tumor associated with a residual retroperitoneal mass.
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OBJECT: The goal of this study was to confirm the effectiveness of our novel treatment strategy, neoadjuvant therapy (NAT) consisting of combined chemo- and radiotherapy, which are performed before complete excision of residual tumor in patients with intracranial nongerminomatous malignant germ cell tumors (NGMGCTs). METHODS: The authors treated 11 consecutive patients with NGMGCTs by applying NAT consisting of combined platinum-based chemotherapy and radiotherapy, followed by complete excision of residual tumors. The pretreatment diagnosis, based on tumor markers with or without biopsy, was yolk sac tumor in five patients, embryonal carcinoma in one patient, immature teratoma in one patient, and mixed germ cell tumor containing malignant tumor components in four patients. Among the 11 patients, NAT achieved a complete response in two and a partial response in six patients; two patients manifested no change and one suffered disease progression. Residual tumors that occurred post-NAT were surgically removed in nine patients. Of the 11 patients, 10 are currently alive without recurrence of their disease, 30 to 177 months (mean 96 months) after diagnosis. In one patient a leptomeningeal tumor recurred and he died of the disease 21 months after diagnosis. CONCLUSIONS: Neoadjuvant therapy, consisting of combined chemo- and radiotherapy, followed by complete excision of residual tumors is highly effective in patients with intracranial NGMGCTs.
The present report describes an example of multifocal (two) yolk sac tumor (YST) with mesenchyme-like and enteroid patterns found in the placenta (730 g) of a newborn (4200 g) with Wiedemann-Beckwith syndrome (WBS) phenotype (macroglossia, omphalocele, hemihypertrophy, cardiomegaly, hypoglycemia). YST has not been previously reported to develop in the placenta. This case expands further the spectrum of alterations found in the placenta in the WBS and fits in the list of tumors related to WBS. ¿KW¿
Cytogenetic analysis has been carried out on 17 parallel short-term cultures from four malignant testicular teratomas of intermediate differentiation (MTI), two of them combined with seminomas. Clonal development was seen in three tumors, with most of the variation involving different rearrangements of chromosome 1. Two copies of the i(12p), characteristic of testicular germ-cell tumors, were present in the two tumors with yolk sac elements, a single i(12p) and a 12q- were found in an MTI that had metastasized, and a rearrangement of chromosome 12 containing centromeric chromatin from chromosome 18 was found instead of the i(12p) in a mixed tumor. A 13p+ marker containing Q-negative material was seen in two of the tumors, with a der(7)t(Y;7) in one. Chromosomes 1, 3, 6, 7, 8, 12, 17, and X were invariably overrepresented either as complete or partial trisomies, and chromosomes 4, 5, 13, and 18 were underrepresented in all four tumors, strengthening the idea that tumor suppressor genes on the latter four chromosomes, all of which show some loss of heterozygosity with DNA markers, may be important in tumor progression.
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.
PURPOSE: The Prepubertal Testis Tumor Registry was established by the Urologic Section of the American Academy of Pediatrics in 1980 to record data on a large number of prepubertal testis tumors regarding presentation, treatment and outcome to define appropriate management better. We reviewed the registry data in the context of other modern studies to elucidate the appropriate management of these rare tumors. MATERIALS AND METHODS: Relevant data in the prepubertal testis tumor registry were tabulated and analyzed. RESULTS: There were 395 prepubertal patients who had a primary testis tumor. Generally benign tumors accounted for 38% of cases. A significant proportion of tumors were benign regardless of patient age. alpha-Fetoprotein levels for patients with benign and malignant tumors overlapped in children younger than 6 months. Of the patients with yolk sac tumor 80% presented with stage 1 disease and overall survival was excellent. There were no metastases or deaths among the patients with teratoma. Of all patients with stromal tumors metastases developed in only 1. CONCLUSIONS: We recommend initial excisional biopsy for all amenable prepubertal testis tumors, except those with an alpha-fetoprotein level that is clearly increased for patient age. Patients with benign tumors may be released from oncological followup. Patients with stage I yolk sac tumor should be monitored closely, and those with recurrent or metastatic yolk sac tumor should be treated with chemotherapy. Retroperitoneal lymph node dissection is reserved for patients with recurrent retroperitoneal masses following chemotherapy. Aggressive treatment of metastatic Sertoli cell or undifferentiated stromal tumors is warranted.
A 24-year-old man presented with right scrotal swelling and was diagnosed as having a testicular cancer. A high inguinal orchiectomy was carried out. Histological examination revealed immature teratoma and yolk sac tumor of the testis. Eight months later, 2 separate metastatic lesions appeared in the lungs, both of which were removed by operation after a course of combination chemotherapy with Etoposide and Cisplatin. The histology showed one to be composed of immature teratoma, and the another of yolk sac tumor.
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BACKGROUND: There is a therapeutic dilemma for the pregnant patient with malignancy requiring chemotherapy. CASE: We had a case of a 30-year-old pregnant woman with a yolk sac tumor who underwent right salpingo-oophorectomy at 22 weeks gestation. Chemotherapy during pregnancy was postponed because of concern with the risks of the chemotherapy to the fetus. After the operation, L2 fraction of alpha-fetoprotein (AFP) depending on the affinity for Lens culinaris agglutinin (LCA) as an indicator for a maternal source of AFP had been detectable since 30 weeks gestation and elevated rapidly with advancing gestation. Tumor recurrence was confirmed clinically at 34 weeks gestation. She was induced, delivering a healthy baby, and received seven complete chemotherapy courses (cisplatin, etoposide, and peplomycin). Currently, the mother (39 months after last chemotherapy) and child are doing well. CONCLUSION: AFP-L2 may be a sensitive indicator for a yolk sac tumor derived AFP, leading to an earlier detection of tumor recurrence during pregnancy.
Testicular germ cell cancer is a common cancer in young adults and its incidence has risen dramatically over the past several decades in Western countries. Because RET finger protein (RFP), which belongs to the large B-box RING finger protein family, has been reported to be expressed in different stages of spermatogenesis, we investigated its expression in testicular germ cell tumors. These comprised 13 pure seminomas, five pure non-seminomatous germ cell tumors (NSGCT) and seven mixed germ cell tumors, four of which contained seminomatous component. In normal adult testis, the expression of RFP was strong in the germ cells, particularly in spermatogonia and primary spermatocytes. RFP immunoreactivity was seen uniformly and specifically in 12 of the 13 pure seminomas examined. It was also detected in seminomatous components of mixed germ cell tumors, whereas pure NSGCT were negative for RFP expression. The expression of RFP in male germ cells and seminomas together with the lack of its expression observed in highly aggressive NSGCT suggested that RFP could be associated with the regulation of germ cell proliferation and/or histological-type of germ cell tumors.
A case of recurrent yolk sac tumor in a three-year-and five-month old child is reported. Left high orchiectomy was performed two years earlier at which time no evidence of metastatic disease was demonstrated. Twenty-four months postoperatively, serum alpha-fetoprotein (alpha-FP) was elevated and recurrent mass appeared in the left para aortic region. Treatment by VAC regimen consisting of vincristine, actinomycin D and cyclophosphamide was performed. Complete response was obtained after two courses of this therapy, which was confirmed by retroperitoneal lymphadenectomy. We believe that this chemotherapy is effective and safe for the treatment of yolk sac tumor of infantile testis.
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OBJECTIVE AND IMPORTANCE: We report the extremely rare occurrence of a second germ-cell tumor at a different site and with different histological types long after total resolution of a pineal germinoma. CLINICAL PRESENTATION: A 21-year-old man who presented with headache and diplopia was admitted to our hospital. Neuroradiological studies revealed a tumor in the pineal region. The tumor was biopsied with endoscope, and third ventriculostomy was performed. Histologically, the tumor proved to be a germinoma. The patient received 3 cycles of combination chemotherapy consisting of carboplatin and etoposide with radiotherapy. The tumor was totally resolute. Twelve months later, he was readmitted with headache and diplopia. Neuroradiological studies showed a tumor in the right temporal lobe. INTERVENTION: The second tumor was totally removed. Histologically, the tumor proved to be a mixed germ-cell tumor, which consisted a yolk-sac tumor and a germinoma. After the second course of chemotherapy, magnetic resonance image studies revealed no evidence of the tumor. CONCLUSION: The second tumor was considered to be a metachronous neoplasm rather than a recurrence of the original mixed germ-cell tumor, which consisted a yolk-sac tumor and a germinoma.