Mesonephric adenocarcinoma in the uterine corpus: A case report and review of the literature.

Mesonephric adenocarcinoma (MNA) is a rare malignancy arising from the mesonephric remnant of the female reproductive tract, typically found in the cervix. MNA is uncommon in the uterine corpus, only 33 cases have been described in the literature. A 55-year-old postmenopausal woman presented with pink vaginal discharge and bilateral hip pain for 2 months, with the help of histopathologic observation and immunohistochemical staining, a diagnosis of "MNA" was made. The tumor invaded the whole layer of myometrium without endometrium involvement, mesonephric remnants and hyperplasia of the mesonephric duct were also found at the periphery of the neoplasm. After the operation, the patient was treated with 3 cycles of chemotherapy. The patient was followed for 6 months with disease. Further experience to diagnose and cure this rare tumor is warranted.

Activity of chemotherapy with carboplatin plus paclitaxel in a recurrent mesonephric adenocarcinoma of the uterine corpus.

BACKGROUND: Malignant lesions derived from mesonephric (Wolffian) remnants are uncommon. The course of these tumors is usually indolent, and the recurrence has only been documented in nine cases. Because of the small number of cases, no current recommendations exist regarding treatment, and little is known about the response to chemotherapeutic agents. CASE: A 33-year-old woman was diagnosed with a mesonephric adenocarcinoma arising in the uterine corpus. Ten months after initial surgery and radiotherapy she presented with local and pulmonary relapse. Salvage chemotherapy with carboplatin plus paclitaxel was administered with a good response. CONCLUSIONS: Mesonephric adenocarcinomas are uncommon neoplasms. Their treatment remains elusive. We report a case of a recurrent uterine mesonephric adenocarcinoma that presented a good response to therapy with carboplatin plus paclitaxel. A review of the previous literature is also presented.

[Therapy of testicular germ cell tumors. Current status of the MAHO studies]. / Therapie testikulärer Keimzelltumoren. Aktueller Stand der MAHO-Studien.

The GPOH-MAHO trials designed in 3/82 and 7/88 for treatment of childhood testicular germ cell tumors registered 3/92 105 pts. In MAHO 82 study 57 pts. and in MAHO 88 study 48 pts. were treated. Histologically 60% of the tumors revealed yolk sac tumors (YST), 22% teratomas (TD) and 18% malignant teratomas (MTI, MTU, MTT). Beside unilateral orchiectomy, according to stage and histology a stratified chemotherapy was administered. Standard chemotherapy consisted of 4 courses VLB, BLM, DDP, after 2 courses standard chemotherapy if vital tumor was suspected: explorative laparatomy. According to laparatomy some patients received salvage chemotherapy of 3 courses VP 16, IFO and DDP. The following results were obtained: YST: 59 pts. with stage I. Of these 10 received adjuvant chemotherapy with VLB, BML, DDP. 49 pts. were followed according to wait and see policy and not treated by adjuvant chemotherapy; 8 pts. had a relapse. 5 were treated with standard chemotherapy and 1 pt. with salvage therapy. 1 pt. had stage II and another stage III. Both received standard chemotherapy. The survival of all 61 pts. is 100%. Median observation time is 4 years. TD: 25 pts. had stage I. No chemotherapy was given. The relapse free survival is 100%. Median observation time is 4 years. Malignant teratomas (MTI, MTU, MTT): 8 pts. had stage I. Of these 3 received adjuvant chemotherapy, 5 lymphadenectomy without chemotherapy. All patients survived without relapse. 8 pts. had stage II and received standard chemotherapy, of these 4 pts. had stage IIc and explorative laparotomy was done. According to the result 2 pts. received salvage therapy. All pts. survived relapse free.(ABSTRACT TRUNCATED AT 250 WORDS)

Treatment of ovarian malignant germ cell tumors with preservation of fertility.

Conservative operation and postoperative chemotherapy were given to 15 patients with malignant germ cell tumors of the ovary with the preservation of fertility and ovarian functions. Four patients, one with endodermal sinus tumor and three immature teratoma, had full term deliveries after the operation. The possibility was discussed to preserve young women's fertility and ovarian function in treating their malignant germ cell tumors.

Endodermal sinus tumor of the vagina in an infant: magnetic resonance imaging evaluation.

Endodermal sinus tumor is a rare and highly malignant lesion. This report documents the clinical findings and magnetic resonance imaging (MRI) of an uncommon case of the endodermal sinus tumor arising in the vagina of a 6-month-old infant. MRI was extremely accurate in delineating the possible extent and location of the vaginal lesion. This is the first report to demonstrate MRI of vaginal endodermal sinus tumor.

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.

Successful treatment of a primary endodermal sinus tumor of the liver.

A 27-year-old woman had a large hepatic tumor and a markedly increased serum alpha-fetoprotein (AFP) level. A diagnosis of endodermal sinus tumor was made after a needle biopsy was performed on the liver. Clinical and radiologic examinations did not show an alternative primary site. Treatment with cisplatin, etoposide, and bleomycin was started, but, after three cycles, was changed to cisplatin, vincristine, methotrexate, bleomycin, dactinomycin, cyclophosphamide, and etoposide because the serum AFP level was decreasing too slowly. After additional chemotherapy was given, the patient was well but had an increased AFP level and a large residual mass in the liver. A right hemihepatectomy was performed, but no viable tumor was present. The patient is alive and disease-free 5 years later. Thus, AFP levels may be misleading in the presence of large necrotic tumors. The authors stress the need to make a diagnosis of these rare tumors early because aggressive treatment with combination chemotherapy may result in cure.

Primary endodermal sinus tumor of the penis: a case report.

We report a case of primary extragonadal germ cell tumor of the penis in a 2-year-old child with elevated serum alpha-fetoprotein. The patient responded well to a combination of bleomycin, etoposide and cisplatin, achieving a complete response within 3 cycles. He was alive and disease-free 8 months after diagnosis. To our knowledge this is the first case of an endodermal sinus tumor of the penis reported in the literature. The possible histogenesis of this tumor at such a rare site is discussed.

The role of second look laparotomy and tumor markers in the follow-up of endodermal sinus tumor of the ovary. A case report and review of the literature.

Endodermal sinus tumor (EST) of the ovary is extremely rare and little information exists about therapy and the role of second-look laparotomy in the management of this entity. A case of EST of the ovary in a 21 year old woman is reported. She received conservative surgery and six courses of combination therapy consisting of Vincristine, Actinomycin D and Cyclophosphamide before second-look laparotomy. Due to progression of the disease second-line polychemotherapy with Vinblastine, Bleomycin and Cisplatin was administered. This new regimen reduced the alpha-fetoprotein to normal levels although the patient was not free of disease on second-look laparotomy. Precise guidelines for the management of this disease, especially in advanced stages, are still lacking.

Primary chemotherapy in the management of low stage (IIA and IIB) non-seminomatous germ cell testicular tumours.

In a prospective study a total of 65 patients in clinical stages IIA and IIB nonseminomatous testicular tumours were treated by primary chemotherapy followed by retroperitoneal lymphadenectomy in cases with residual disease. The patients were given a combination of cisplatin, vinblastine and bleomycin, or also etoposide. Sixty-two patients (95.4%) achieved complete response: 39 (60%) by chemotherapy alone and 23 (35.4%) following surgical removal of residual disease. Three patients died; there were two drug-related deaths during PVB chemotherapy, one patient had progression of disease following chemotherapy and died as a result of disease dissemination. Three patients relapsed from complete response following chemotherapy, two of them died within 19 and 29 months after the onset of therapy. The third patient received second-line chemotherapy and gained complete response again. Of the 65 patients, 60 (92.3%) survive with no evidence of disease. The follow-up period ranged from 6 to 79 months (mean 39.4 months, median 39 months).

POMB/ACE chemotherapy in non-seminomatous germ cell tumours: outcome and importance of dose intensity.

This study reports the outcome of POMB/ACE (cisplatin, vincristine, methotrexate, bleomycin, actinomycin D, cyclophosphamide, etoposide) chemotherapy in 53 male patients with metastatic non-seminomatous germ cell tumour (NSGCT) treated between 1983 and 1989 in one centre. The overall complete response (CR) rate was 62% [95% confidence interval (CI) 49-75%), and for patients with large or very large volume disease (L/VL, MRC criteria), the CR rate was 56% (95% CI 41-71%). The overall 5 year survival was 61%, and for L/VL volume disease 67%. Comparison with previous studies suggests that POMB/ACE chemotherapy is not superior to BEP, even in patients with adverse prognostic factors. Increased average relative dose intensity and increased relative dose intensity of cisplatin over the first seven courses were not associated with improved survival. However, in patients receiving a relative dose intensity of etoposide greater than or equal to 0.75, survival at 5 years was significantly improved compared with those in whom this parameter was less than 0.75 (79% vs. 44%, P less than 0.05), suggesting that dose intensity of etoposide may be an important determinant of outcome in the chemotherapy of metastatic NSGCT.

Expression of a human multidrug resistance gene in human ovarian carcinoma cell lines.

To investigate the possible role of the multidrug resistance phenotype to chemoresistance in human ovarian carcinoma, we have analyzed human multidrug resistance gene (mdr 1) expression in 8 human ovarian adenocarcinoma cell lines. An increase in P-glycoprotein level specific to multidrug-resistant tumor cells was not apparently associated with the increase in resistance to vincristine (VCR) or doxorubicin (Adriamycin). Mdr 1 transcripts (4.5 kilobases) were observed in the RNA preparation obtained from only one cell line (SHIN-3) that showed the highest resistance to both drugs in vitro and in vivo. No cell lines showed mdr 1 DNA amplification. These results suggest that the insensitivity of human ovarian carcinoma to chemotherapy could be partly explained by the expression of mdr 1.

Primary endodermal sinus (yolk sac) tumor of the anterior mediastinum: a report of a successfully treated eleven-year-old boy.

An asymptomatic 11-year-old Japanese boy with an endodermal sinus (yolk sac) tumor of the anterior mediastinum was successfully treated with cisplatinum-based combination chemotherapy and subsequent surgical excision. The patient is alive and in good health 60 months from diagnosis.

[The prognostic significance of serum alpha 1-fetoprotein in children and adolescents with malignant extracranial non-testicular germ cell tumors]. / Die prognostische Bedeutung des Serum alpha 1-Fetoproteins (AFP) bei Kindern und Jugendlichen mit malignen extrakranialen nichttestikulären Keimzelltumoren.

The cooperative therapy study MAKEI 83/86 included an examination of the prognostic value of the AFP in children and adolescents with extracranial non-testicular yolk sac tumors. The serum AFP values of 72 protocol- and follow-up-patients were documented at diagnosis and up to the ninth month of treatment. 32 of these patients had saccrococcygeal tumors, 27 had tumors of the ovary and 13 suffered from extragonadal germ cell-tumors. 4 children showed progressive disease under initial chemotherapy and 1 patient died of therapy, 10 of 72 patients relapsed. The AFP measurements were plotted on semilogarithmic charts. They were compared to the measurements of healthy children up to the age of 1 year. According to the development of the patients' AFP values compared to the reference curves the following classifications could be made: 1. Patients with a normal AFP-decrease id est 50% in less than or equal to 6 days during the 1st month of therapy: 48/72 patients 2. Patients with slow AFP-decrease: 17/72 patients 3. Patients with transient AFP-decrease: 5/72 patients 4. Patients with no AFP-decrease: 2/72 patients According to Kaplan-Meier life table analysis, patients with a normal AFP-decrease had an event-free survival of 89% +/- 4%, whereas all other patients showed an event-free survival of 63% +/- 10% (p less than 0.05). Regarding primary therapy id est tumor resection or preoperative chemotherapy an equal distribution of the patients among those with a normal and slow AFP-decrease was observed.(ABSTRACT TRUNCATED AT 250 WORDS)

[Clinical study of bilateral metachronous testicular tumors with different histological findings].

Seventy-seven patients with primary malignant testicular tumors were treated in our hospital. Twenty-five of them were given antineoplastic agents containing cis diamine dichloro platinum (CDDP). In three long-term survivors, new malignant testicular tumors developed meta-chronously and had different histological findings from those of the initial tumors. Case 1. A 28-year-old patient with a yolk sac tumor of the left testicle, stage IIO, developed metastasis to the supraclavicular lymph nodes five years after radiation. Chemotherapy containing of VP-16 (837 mg), CDDP (1050 mg), vincristine (32 mg), bleomycin (480 mg), and actinomycin-D (16 mg) achieved complete remission. Four years 11 months later a seminoma of the contralateral testicle, stage I, was disclosed and he died of cancer 11 years and four months after the onset of the initial disease. Case 2. A 30-year-old patient with testicular teratoma, stage IIIA, on the right side gained complete remission after a CDDP containing chemotherapy. One year and four months after the beginning of the CDDP use (1,300 mg totally as CDDP) a seminoma on the contralateral side, stage I, was detected. He died of cancer eight years and two months after his initial tumor was detected. Case 3. A 37-year-old patient with combined tumor of seminoma and yolk sac tumor of the right testicle, stage IIIO, was free from disease for six years and five months under chemotherapy. At this point a seminoma, stage I, of the contralateral testicle was newly found and treated by radiation.(ABSTRACT TRUNCATED AT 250 WORDS)

The growing teratoma syndrome.

Thirteen patients with metastatic non-seminomatous germ cell tumours and enlarging metastases consisting of teratoma differentiated only were identified. Patients were managed with surgical resection soon after the growing lesions were documented. Surgical morbidity was minimal and 12 patients are alive (10 are disease-free) at a median follow-up of 28 months.