Urethral obstruction due to lobular mesonephric hyperplasia of the vagina--a case report.

The histological finding of persistent mesonephric duct remnants in the uterine cervix and vagina is uncommon and is usually an incidental finding in tissues excised for other reasons. Herein we present a case of lobular mesonephric hyperplasia in a 45 year old woman who presented with vaginal bleeding and difficulty passing urine.

Testicular tumor in patient with persistent müllerian duct syndrome.

A case of a yolk-sac tumor associated with an intra-abdominal testis in a patient with persistent müllerian duct syndrome is presented.

Mayer-Rokitansky-Kuster-Hauser syndrome associated with endodermal sinus tumor of the ovary.

Both EST and Mayer-Rokitansky-Kuster-Hauser syndrome are uncommon disorders. This report illustrates a case of these two rare conditions occurring in the same patient. Patients with Mayer-Rokitansky-Kuster-Hauser syndrome, although not appearing to be at increased risk for germ cell tumors, unlike testicular feminization patients, are not immune to ovarian cancer. Therefore, individuals with Mayer-Rokitansky-Kuster-Hauser syndrome should still be followed gynecologically because they are at least at the normal risk of developing ovarian as well as other gynecological neoplasms.

Intraabdominal testis with yolk sac tumor in a 2-year-old child.

A case of a large yolk sac tumor in an undescended testicle in a 2-year-old child is presented. No such similar finding has been reported in a young child. Despite the large size of the primary tumor, the high level of serum alpha-fetoprotein and the relatively late clinical presentation, this was a stage I lesion, and the child responded to surgical resection of the tumor and chemotherapy.

[Nephrogenic adenoma of the bladder associated with urinary tuberculosis. Case report]. / L'adenoma nefrogenico della vescica associato a tubercolosi urinaria. Contributo casisitico.

Nephrogenic adenoma is an uncommon benign lesion of the urinary tract, that histologically is characterised by glandular-like aspects resembling the distal part of the nephron. It is usually associated with antecedent inflammation, surgical procedures or other injuries. Personal experience with one additional case nephrogenic adenoma of the bladder in a patient with urinary tract tuberculosis is presented.

[A case of endodermal sinus tumor in the basal ganglia associated with Down's syndrome].

A case of Down's syndrome accompanied by endodermal sinus tumor of the left basal ganglia is reported. She was referred to us because of right hemiparesis. A CT scan showed a calcified mass in the left basal ganglia, which was well enhanced on contrast CT. Emergency removal of the tumor was performed because of its rapid growth, intratumoral hemorrhage, and acute hydrocephalus. The histological diagnosis established after surgery was endodermal sinus tumor. Alphafetoprotein-positive cells were seen in the resected tumor by light microscopy. Postoperative irradiation was very effective, and the high level of serum alphafetoprotein decreased during the course of irradiation. The residual tumor, enhanced on contrast CT, was treated by administration of cisplatin and etoposide after irradiation. Cases of malignant tumors other than leukemia, which are associated with Down's syndrome, are extremely rate. To our knowledge, including three cases of germ cell tumor, there have been only nine cases of brain tumors associated with Down's syndrome reported previously.

[Brain metastasis of testicular yolk sac tumor with massive hemorrhage: case report].

A rare case of yolk sac tumor of the testis which metastasized to the brain parenchyma with repeated intra-tumoral hemorrhage is reported. The patient was a 38 year-old-man admitted to the Department of Urology with the chief complaint of painless swelling of the left testis for a period of one year. He underwent orchiectomy with highly suspected malignancy, because chest X-ray examination demonstrated metastatic coin lesions. Pathological examination of the operated specimen showed typical yolk sac tumor of the testis. Postoperative clinical course was uneventful. However, on the 4th postoperative day, the patient suddenly fell into a coma with left hemiplegia and dilated right pupil. CT scan demonstrated an intra-parenchymal hematoma in the right parietal lobe. Emergency craniotomy with evacuation of the hematoma clot was performed. Histological examination of the hematoma clot showed the same histology (yolk sac tumor) as found in the operative specimens of the left testis. After regaining full consciousness, chemotherapy (PEB and PVB) was started. It was effective to reduce the high serum AFP level and the size of the metastatic coin lesions in the lung. However, it was not effective in preventing brain metastasis. He again relapsed into coma due to a newly-developed intra-tumoral hematoma with multiple brain metastasis and died 6 months after the orchiectomy. Yolk sac tumor of the testis is rare in adult Japanese and there is no previous report of its metastasis to the brain parenchyma with intratumoral hemorrhage. We have reviewed the previously reported cases and discussed the brain metastasis of this malignant urogenic tumor.

Mesonephric hyperplasia can cause abnormal cervical smears: report of three cases with review of literature.

BACKGROUND: Hyperplastic mesonephric remnants are an incidental finding in occasional uterine or cervical surgical specimens. We describe three cases in which such remnants were postulated to be the source of abnormal glandular cells in cervical smears. CASES: In all three cases abnormal glandular cells were seen in cervical smears. Subsequent histology showed the presence of hyperplastic mesonephric remnants that communicated with the endocervical canal and were likely to be the source of the abnormal glandular cells. We believe that the key features of these cells, which may aid their distinction from other causes of glandular abnormalities, are their loose clustering, lack of significant anisocytosis and cuboidal outlines. CONCLUSION: We aim to document mesonephric hyperplasia as a possible source for abnormal glandular cells in cervical smears.

Idiopathic thrombocytopenia in association with extragonadal germ cell cancer.

Thrombocytopenia was associated with primary extragonadal germ cell cancer in three patients. All had embryonal cell cancer with anterior superior mediastinal masses. Severe thrombocytopenia was noted initially and persisted throughout the course of the disease. Bone marrow showed increased megakaryocytes. There was no evidence of immune destruction of platelets or sequestration by the mediastinal tumor masses. Therapy with prednisone and splenectomy did not increase platelet counts and chemotherapy was extremely difficult. Idiopathic thrombocytopenia may be an adverse prognostic feature for patients with extragonadal germ cell cancer.

Hepatoid yolk sac tumor of the ovary (endodermal sinus tumor with hepatoid differentiation): a light microscopic, ultrastructural and immunohistochemical study of seven cases.

Seven cases of ovarian yolk sac tumor (endodermal sinus tumor) with patterns resembling those of hepatocellular carcinoma were encountered in patients 7-43 years of age. Two of the patients had gonadal dysgenesis with a 46XY karyotype. At operation three tumors were confined to the ovary and four were associated with intra-abdominal metastases. Two of the Stage I tumors recurred within one year. The hepatoid pattern was a prominent feature of all the tumors and was exclusive in four of them. In one specimen it merged almost imperceptibly with a polyvesicular vitelline pattern. The hepatoid component of the tumors was characterized by discrete masses, nests and/or broad bands of large polyhedral cells with central nuclei and prominent nucleoli; gland-like spaces, some of which contained mucin, were occasionally evident. Each tumor contained numerous PAS-positive, diastase-resistant intracytoplasmic and extracytoplasmic hyaline bodies. Alpha-fetoprotein and alpha-1-antitrypsin were identified by immunoperoxidase and immunofluorescence techniques in four tumors and albumin in two. Immunoperoxidase stains for chorionic gonadotropin were negative in four cases. Ultrastructural analysis of two specimens disclosed features similar to those of hepatocellular carcinoma.

[Case of yolk sac tumor of the anterior mediastinum with superior vena cava syndrome].

The patient was a 21-year-old man with SVC syndrome due to a large anterior mediastinal mass. Following radiotherapy which improved his condition, thoracotomy was performed, however, total resection failed. Despite postoperative re-irradiation, his condition worsened rapidly and he died five months after the onset of his symptoms. The neoplasm was composed of a yolk sac tumor producing alpha-fetoprotein. Yolk sac tumors of the mediastinum are rare and lethal. Recent reports indicate that cis-platinum may be highly effective in germ cell tumors. Therefore, radiotherapy combined with cis-platinum may be improved the survival of patients with yolk sac tumor.

Nephroid metaplasia of the urinary tract. A survey of the literature, with the contribution of 5 new immunohistochemically studied cases, including one case examined by electron microscopy.

Nephroid metaplasia is an unusual lesion confined to the lamina propria of the lower urinary tract. It is defined by a characteristic histologic picture of tubular structures, formed by a single layer of cuboidal cells, surrounded by a thick basement membrane. Two main theories concerning the histogenesis of the condition have been proposed: embryonic origin, or metaplasia. Five cases of nephroid metaplasia were studied light-microscopically and by immunohistochemistry for the content of Tamm Horsfall's uromucoprotein. In addition, one case was examined by electron microscopy. The results were compared to current knowledge of the lesion obtained from a survey of the literature, with special reference to histogenesis. Morphologically, one case of nephroid metaplasia was associated with mesonephroid adenocarcinoma. It is concluded that nephroid metaplasia arises as a metaplastic lesion, nearly always in previously traumatized urothelial mucosa. The natural history of the typical nephroid metaplasia is benign, but a possible relationship to mesonephroid adenocarcinoma, representing the malignant counterpart of the lesion, is discussed in relation to the histological findings, suggesting a rare but possible malignant potential of nephroid metaplasia. The diverse nomenclature used for this histologic entity needs re-evaluation and should be changed to: nephroid metaplasia.

Ataxia-telangiectasia and endodermal sinus tumor of the ovary: report of a case.

Ataxia-telangiectasia, an inherited disorder characterized by progressive cerebellar ataxia and telangiectasias, is often associated with primary immunodeficiency and high incidence of malignancies, mostly of the lymphoreticular type. Endodermal sinus tumor is a rare germ cell tumor of the ovary characterized by an extremely rapid growth and poor prognosis. Both these diseases are associated with an abnormal production of alpha-fetoprotein. Primary tumors of the ovary in patients with ataxia-telangiectasia are extremely rare and the association of an endodermal sinus tumor and ataxia-telangiectasia has never been reported in the literature. This case report serves to focus on the particular problems encountered in the diagnosis and management of two diseases both characterized by the same serum marker.

45,XO/46,X,dic(Yq) mosaicism in Turner's phenotype with endodermal sinus tumor of the ovary.

A case of a endodermal sinus tumor of the ovary in a patient with 45,XO/46,X,dic(Y) mosaicism is reported because of the rarity of the karyotype and condition. A 15-year-old girl was admitted to Yamagata University Hospital because of constipation for several days. Physical examination showed webbing of the neck, cubitus valgus and short stature. Her abdomen was bulging. Chromosomal analysis showed 45,XO/46,X,dic(Yq) mosaicism in karyotype. alpha-Fetoprotein and CA-125 in the serum were high. A left ovarian tumor was found by laparotomy; however, the right ovary was a streak gonad and the uterus was hypoplastic. An endodermal sinus tumor was diagnosed by a pathologist. After operation, cisplatin-vinblastin-bleomycin chemotherapy was instituted and the tumor marker went down. This patient is still healthy and under observation at the outpatient clinic.

Congenital anomalies in children with testicular germ cell tumor.

The incidence and type of congenital anomalies associated with childhood testicular germ cell tumors were studied retrospectively in 25 patients (20 cases of yolk sac tumor and 5 cases of teratoma). Congenital anomalies were observed in 3 patients with yolk sac tumors and in 1 patient who had a mature teratoma. The abnormalities observed included individual cases of retrocaval ureter, diverticulum of the bladder, Down's syndrome and an ipsilateral inguinal hernia. Children with a testicular tumor should be examined closely for congenital abnormalities.