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1/15. Pancreatoblastoma treated by delayed operation after effective chemotherapy.

    A case of pancreatoblastoma in a 4-year-old girl is presented. She was admitted with an abdominal mass and vomiting in August 1994. Computed tomography scan showed a 10- x 8- x 7-cm mass occupying both the head and body of the pancreas. serum alpha-fetoprotein (AFP) level was 9,600 ng/mL (normal, <8.3 ng/mL). Results of open biopsy of the tumor showed pancreatoblastoma. Chemotherapy was administered using the new A-1 regimen consisting of cyclophosphamide, etoposide, pirarubicin, and cisplatin. After 3 cycles of chemotherapy, the size of the tumor was reduced to 5 x 4 x 3 cm, the portal vein became patent, and the AFP value decreased to 98.1 ng/mL. Total removal of the tumor was performed leaving the head and tail of the pancreas. Postoperative chemotherapy continued for 2 years. The patient has been disease free for 5 years, and her serum AFP remained within normal levels.
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2/15. Successful treatment of retrograde ejaculation with sperm recovered from bladder washings. A report of two cases.

    BACKGROUND: Retrograde ejaculation causes < 2% of male infertility but is the leading cause of aspermia. The incidence of retrograde ejaculation is increasing due to the aggressiveness of modern urologic cancer surgery and an increase in diabetes mellitus. Generally, the only adverse effect is on fertility. Various approaches have been proposed for treatment, ranging from insemination with sperm-rich urine obtained after masturbation to intracytoplasmic sperm injection (ICSI). We used a protocol involving bladder washing. CASES: Case 1 involved a man with retrograde ejaculation secondary to a successful right orchiectomy and retroperitoneal lymph node dissection for stage B1 embryonal cell carcinoma. He was treated with bladder washing and intrauterine insemination. He fathered three children from six insemination cycles. Case 2 involved a man with idiopathic retrograde ejaculation and a wife with ovulatory dysfunction. He received treatment similar to that in case 1 and fathered one child from two insemination cycles. CONCLUSION: Larger studies need to be done specifically comparing treatments. Our method resulted in four normal infants in two couples over eight total insemination cycles and, taken together with other results from the literature, seems a good choice for clinicians who are treating retrograde ejaculation for the first time. We agree with others who have recommended that in vitro fertilization/ICSI not be the first step for treating the usual couples with retrograde ejaculation.
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3/15. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature.

    Undifferentiated (embryonal) sarcoma of the liver (USL) is a highly malignant tumor of early life. Treatment choices for USL, especially with intraperitoneal rupture, are uncertain. Outcomes have been almost uniformly poor until recently. We describe two 7-year-old girls treated for ruptured USL. In the more recent patient, operative biopsy was followed by three cycles of cisplatin (CDDP), adriamycin (ADR), and cyclophosphamide (CPM). A fluid-filled cavity in the tumor showed enlargement and was drained. Two cycles of CDDP, ADR, vincristine (VCR), and ifosfamide were accompanied by reduction in tumor size, and trisegmentectomy was performed. She has no evidence of disease 3.5 years after surgery. In the other patient, left lobectomy was followed by a less intensive regimen, including CPM, VCR, and fluorouracil. This patient died of dissemination within 5 months. In 170 reported pediatric patients with USL, the 2-year disease-free survival was 17%. For the 96 such patients reported since 1980, 2-year disease-free survival had improved to 27%. More aggressive chemotherapy has been associated with this change. Of 8 patients with tumor rupture whose details have been reported (including the 2 present patients) after resection of the tumor, 4 died, 1 was alive with disease, and 3 were free of disease at 8, 49, and 58 months, respectively, after diagnosis. Ruptured USL should be treated with combination chemotherapy including CDDP and ADR, as well as with curative resection.
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4/15. gynecomastia following chemotherapy for testicular cancer.

    A 22-year-old man received 4 cycles of bleomycin, etoposide and cisplatin combination chemotherapy for clinical stage IIA embryonal cell carcinoma of the right testis. The treatment resulted in complete remission. Five months following cessation of the chemotherapy first on the left and then 2 weeks later on the right side painful gynecomastia developed. His hormonal values are all normal with no evidence of recurrence of the cancer. gynecomastia on both sides resolved in 8 months spontaneously without any treatment. He is still in clinical remission 14 months after completion of the chemotherapy. We should be aware that gynecomastia following cytotoxic chemotherapy in a young man does not necessarily mean the return of the cancer.
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5/15. Retroaortic left renal vein in testicular cancer patient: potential staging and treatment pitfall.

    A 20-year-old man was diagnosed with a left mixed germ cell testicular tumor and clinical staging with computerized tomography suggested left para-aortic subhilar retroperitoneal adenopathy. The patient received 4 cycles of cisplatin, vinblastine and bleomycin chemotherapy but the mass in the left renal hilus area remained unchanged. Subsequent retroperitoneal lymphadenectomy revealed the mass to be a retroaoritc left renal vein type 2. Further confusion occurred during followup in differentiating this anomaly from recurrent neoplasm necessitating evaluation by magnetic resonance imaging. Retroaortic left renal vein represents a potential imaging pitfall in testicular cancer that may facilitate suboptimal staging, treatment and followup.
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6/15. Signet ring cell adenocarcinoma of the urachus.

    Signet ring cell adenocarcinoma of the urachus is an extremely rare variety of adenocarcinoma of the urachus; only 23 cases have been reported in the literature to date. In the present report we discuss two more cases of this type and review the literature. For our first case, a 17-year-old man was treated with partial cystectomy with en-bloc excision of the urachus and umbilicus. He received local radiotherapy because of positive surgical margins. Local recurrence occurred after 21 months of diagnosis and he was lost to follow up. For our second case, a 31-year-old man was found to have peritoneal carcinomatosis at the time of surgery and partial cystectomy was carried out as a palliative procedure for hematuria. He received six cycles of chemotherapy, a combination of methotrexate, vinblastine, adriamycin and cisplatinum. The patient's response to the chemotherapy lasted for 8 months, after which his disease progressed and he died 19 months after diagnosis.
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7/15. Different transformation of mature teratoma in a patient with mixed germ cell tumor of the testis.

    A 44-year-old male was referred with a left supraclavicular lymphadenopathy. A biopsy of the lymph node showed metastatic embryonal carcinoma. Tumor markers were present at high levels: alpha-fetoprotein 253.9 ng/mL, beta-human chorionic gonadotrophin 62 ng/mL. Computed tomography (CT) showed retroperitoneal adenopathy. High orchiectomy was done. The patient was treated with three cycles of etoposide plus cisplatin, achieved normalization of the serum tumor markers and underwent retroperitoneal lymph node dissection. Pathological findings of multiple lymph nodes showed teratomatous glands without viable cells. At follow-ups performed every 3 months, tumor markers remained within normal limits and no evidence of recurrence was observed. Eight years after first admission a CT scan revealed a cystic tumor 1 cm in diameter in the para-aortic region. The cystic tumor continued to slowly grow, expanding by 1 cm in diameter per year without elevation of tumor markers. The para-aortic tumor had grown to 4 cm in diameter and a left supraclavicular lymphadennopathy recurred. A resection of the supraclavicular cystic tumor showed mucinous cystadenocarcinoma, but a cystic tumor in the para-aortic region revealed mature teratoma. Here we report a case of mature teratoma with metastases at supraclavicular and para-aortic lymph nodes which had different transformations in spite of both regions consisting of cystic tumors.
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8/15. Successful treatment of disseminated extragonadal germ cell cancer with intensive conventional chemotherapy after first-line high-dose chemotherapy.

    High-dose chemotherapy (HDCT) with peripheral blood stem cell (PBSC) support has been investigated as a first-line treatment in patients with poor risk germ cell cancer. However, effective management of patients with residual cancer after HDCT has not been well addressed, and the outcome in such patients is poor. Here, we report a case of disseminated germ cell cancer successfully treated with intensive conventional chemotherapy after HDCT. A 31-year-old man presented with a bulky mass at the retroperitoneum, which had invaded the lumbar and sacral vertebra, and multiple lung and liver metastases. The patient's serum beta subunit of human chorionic gonadotrophin (beta-hCG) was elevated to 2600 IU (cut-off value <0.1 IU). At the time of diagnosis of poor risk germ cell cancer of extragonadal origin, he underwent two cycles of BEP (bleomycin, etoposide, and cisplatin) chemotherapy and PBSC harvest followed by three cycles of HDCT with PBSC transplantation. The liver metastases disappeared. The retroperitoneal bulky mass and multiple lung metastases shrank but were still present, and the serum beta-hCG level was not completely normalized. An additional three courses of BEP and five courses of VIP (cisplatin, ifosfamide, etoposide) normalized the beta-hCG level. Pathological evaluation of the residual masses revealed no viable cancer cells at either site. The patient is alive without disease recurrence 5 years after completion of chemotherapy.
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9/15. Retroperitoneal extragonadal germ cell tumor presenting as a bulky pelvic mass of the obturator fossa.

    Extragonadal germ cell tumors (EGGCT) are commonly found in the midline of the body. We report a rare case of a retroperitoneal EGGCT presenting as a bulky pelvic mass located between the left internal and external iliac arteries in a 29-year-old man. His alpha-fetoprotein level was 12946.2 ng/mL and the biopsy of the tumor revealed a nonseminomatous germ cell tumor. After three cycles of chemotherapy (bleomycin/etoposide/cisplatin) followed by resection of all residual tumors, one cycle of salvage chemotherapy (etoposide/ifosphamide/cisplatin) was added. The patient was disease free at 21-month follow up.
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10/15. Contralateral testicular cancer in spite of TIN-negative double biopsies and interval cisplatin chemotherapy.

    BACKGROUND: Current models of tumorigenesis postulate that testicular germ cell cancer uniformly develops through a preinvasive lesion termed testicular intraepithelial neoplasia (TIN). An open testicular biopsy is a simple and highly sensitive method to diagnose TIN, and this procedure constitutes the basis for curative treatment of TIN. patients with testis cancer carry a significantly increased risk of developing contralateral testicular tumors. Therefore, a contralateral biopsy has been recommended in these patients. A negative biopsy was assumed to exclude the risk of a subsequent germ cell cancer in the testis due to the high sensitivity of the method. Reports on false-negative biopsies gave rise to the idea that TIN is not uniformly distributed throughout the testis. Consequently, double biopsies are thought to increase the diagnostic sensitivity. CASE REPORT: A 24-year-old patient with nonseminomatous testis cancer is reported. The patient had TIN-negative double biopsies in the contralateral testis. He received three cycles of standard PEB (cisplatin, etoposide, bleomycin) chemotherapy for visceral metastasis. 1 year after treatment the patient developed a nonseminomatous contralateral testis cancer which was treated by partial orchiectomy and subsequent local radiotherapy with 20 Gy. CONCLUSION: The case presented here highlights some clinically important aspects: a) even double biopsies of the testis may fail to detect TIN. b) Systemic cisplatin-based chemotherapy may fail to prevent contralateral testicular germ cell cancer. c) A metachronous contralateral testis cancer may-in contrast to common clinical perception-develop even soon after the diagnosis of the first testis tumor. Furthermore, the case could foster the hypothesis that testicular germ cell tumors may in some cases develop without a preceding stage of TIN.
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