Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Intraabdominal testis with yolk sac tumor in a 2-year-old child. 290 6

Two undifferentiated (embryonal) sarcomas of liver were studied ultrastructurally and immunohistochemically. Electron microscopic examination of the pleomorphic tumor cells revealed fibroblastic and histiocytic characteristics. There were no specific findings to support rhabdomyoblastic, leiomyoblastic, or epithelial differentiation. Cytoplasmic peroxidase-antiperoxidase (PAP) immunohistochemical staining for vimentin, alpha1-antitrypsin, and alpha1-antichymotrypsin was found. No staining for epidermal or internal organ cytokeratins, desmin, myoglobin, or alpha-fetoprotein was observed. The ultrastructural correlates of the cytoplasmic periodic acid-Schiff-positive, diastase-resistant hyaline globules were large, membrane-bound, heterogenous electron-dense inclusions, probably lysosomal in origin. These inclusions did not react on either alpha1-antitrypsin or alpha1-antichymotrypsin PAP staining. Tumor specimens from two metastatic sites were also examined. Neither contained the ducts or cysts that characterized the primary tumor. These studies confirm the mesenchymal nature of this uncommon childhood neoplasm and support the suggestion that the cytoplasmic hyaline globules represent a degenerative phenomenon. There are ultrastructural and immunohistochemical similarities with malignant fibrous histiocytoma.
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PMID:Undifferentiated (embryonal) sarcoma of the liver: ultrastructural and immunohistochemical similarities with malignant fibrous histiocytoma. 298 50

The current report describes a secondary malignancy developing in a retroperitoneal mature residual lesion 6 years after chemotherapeutic treatment of a disseminated nonseminomatous testicular tumor. The histologically malignant component was not present in the primary tumor and consisted of polygonal and fusiform cells with focal tubular formations, resembling primitive neuroectodermal tissue. Immunoperoxidase staining for alpha-fetoprotein and the beta-subunit of human chorionic gonadotropin remained negative, whereas focal positivity for S100 protein was observed. Neuron specific enolase positivity was equivocal. The DNA contents of both the mature components in the primary and the metastatic retroperitoneal tumor and in the various malignant components of the primary tumor, were in the hypotriploid range. In the malignant component of the retroperitoneal metastasis, a hypertriploid peak was observed. These findings suggest further clonal evolution in a phenotypically mature, genotypically abnormal residual metastatic tumor after chemotherapy. It is stressed that the mature appearance of the residual lesions may be deceiving and that these lesions are highly susceptible to resume malignant behavior.
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PMID:Histology and DNA contents of a secondary malignancy arising in a mature residual lesion six years after chemotherapy for a disseminated nonseminomatous testicular tumor. 371 20

Excluding patients with bulky stages II or III disease, 73 patients with nonseminomatous germ cell testicular tumors were evaluated between September 1979 and April 1983 for a protocol omitting retroperitoneal lymph node dissection. Patient eligibility required clinical stage I (T1 category) disease based upon normal post-orchiectomy serum tumor markers (alpha-fetoprotein, human chorionic gonadotropin and lactic dehydrogenase), chest x-ray, ipsilateral lymphangiography, and a computerized tomography scan of the abdomen and pelvis. Of the 73 patients 10 (14 per cent) were entered and followed for more than 2 years (3 had relapse within 7 months but were salvaged with retroperitoneal lymph node dissection and chemotherapy). Analysis of failures showed embryonal carcinoma in all 3 patients, with vascular invasion in the primary tumor in 1 and undetected spermatic cord involvement in 1, while 1 had a slower than expected decrease to normal of an elevated human chorionic gonadotropin level after orchiectomy. There were 63 patients (86 per cent) excluded from the protocol for various reasons: 2 (3 per cent) refused treatment, 16 (25 per cent) had suspicious or positive lymphangiography, 22 (40 per cent) had a positive CT scan, 6 (9 per cent) had elevated tumor markers, 3 (5 per cent) were less than 15 or more than 15 or more than 40 years old, 8 (13 per cent) had had a prior orchiopexy or scrotal violation, 4 (6 per cent) had extension to the spermatic cord and 2 (3 per cent) were unavailable for monthly followup. These 63 patients underwent retroperitoneal lymph node dissection, and 36 (57 per cent) had negative and 27 (43 per cent) had positive nodes (8 had stage N1, 10 stage N2A, 6 stage N2B and 3 stage N3 disease). Average interval from orchiectomy to final staging was 6 weeks. The results suggest that assessment of local tumor extent and potential sites of metastases via all available means are necessary in an effort to reduce the risk of tumor recurrence in patients who are followed expectantly.
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PMID:Selection of testicular tumor patients for omission of retroperitoneal lymph node dissection. 394 94

Forty-two patients with advanced testis carcinoma without previous chemotherapy were treated with VAB-4, and 41 were evaluable. The program consisted of three in-hospital inductions 16 weeks apart, and outpatient treatments every three weeks. Of the patients, 80% achieved complete remissions (CR). Chemotherapy alone induced CR in 61%, partial remissions (PR), in 24% and minor response (MR), in 15%. An additional 20% of patients (six PRs and 2 MRs) achieved CR following resection of residual tumor deposits. With a median follow-up of 27 months, the median duration of CR has not been reached. Of those achieving CR to chemotherapy alone, 12% had relapses. Bulk and extent of metastatic disease, histology of primary tumor, and tumor markers at the beginning of therapy influenced the frequency of CR. Of those with minimal disease, 90% achieved CR. The CR rate was 67% for those with advanced thoracic disease and 29% for those with advanced abdominal disease. Patients who had embryonal carcinoma and those who had no elevation of alpha-fetoprotein had a higher frequency of CRs. Myelosuppression with a leukocyte count drop less than 1000/mm3 occurred in three patients, and no patient had chronic renal failure or pulmonary fibrosis. One patient died from sepsis while in complete remission.
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PMID:VAB-4 combination chemotherapy in the treatment of metastatic testis tumors. 616 66

A 42/3 year old boy with hepatoblastoma presented with precocious sexual development and an abdominal mass. During the course of disease, serial endocrinologic laboratory investigations were done, along with alpha-fetoprotein levels. A significant correlation is noted in these values at diagnosis, postsurgery, and later during a relapse. Ectopic production of chorionic gonadotropins by the tumor is evident. After extensive surgical resection, chemotherapy was started because of metastases. Although the primary tumor failed to respond, the pulmonary metastatic disease showed a greater than 50 percent response rate with cis-platinum.
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PMID:Virilizing hepatoblastoma: precocious sexual development and partial response of pulmonary metastases to cis-platinum. 618 Aug 13

A one-year-five-month-old boy with hepatoblastoma producing both human chorionic gonadotropin (hCG) and alpha-fetoprotein (AFP) is presented. Histologically, the primary tumor was mainly composed of well differentiated hepatoblastoma cells, with minor areas of poorly differentiated cells. Immunoperoxidase staining of the tumor for hCG and AFP showed that a few well differentiated, fetal type cells and multinucleated giant cells were positive for hCG, and AFP was never stained in the same cells. In areas where cells were poorly differentiated, positive reactions for either hCG or AFP were not observed. Electron microscopic studies revealed focal aggregates of cytoplasmic cored vesicles in some tumor cells, similar to secretory granules.
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PMID:Immunocytochemical identification of human chorionic gonadotropin- and alpha-fetoprotein-producing cells of hepatoblastoma associated with precocious puberty. 618 19

Primary hepatocellular carcinoma metastasizing to abdominal lymph nodes and to the left lung was observed in a 16-year-old male patient. No clinically apparent chronic liver disease preceded the carcinoma and no signs of cirrhosis were detectable in the nonneoplastic liver. Hepatitis B surface antigen, hepatitis B e antigen and antibody to hepatitis B core antigen were found to be positive in the serum. By immunohistochemistry (peroxidase-antiperoxidase technique) hepatitis B surface antigen could be demonstrated in the nontumorous liver parenchyma, but not in the primary hepatocellular carcinoma itself. Serum alpha-fetoprotein was only moderately elevated (75 ng/ml), but immunohistochemically primary hepatocellular carcinoma revealed a considerable number of alpha-fetoprotein-containing cells, whereas nontumorous parenchyma did not. Carcinoembryonic antigen could be demonstrated immunohistochemically in some tumor cells of a lymph node metastasis, but not in the primary tumor or in the nontumorous liver parenchyma. We propose that primary hepatocellular carcinoma developed in this case in a symptomless hepatitis B virus carrier without preceding cirrhosis, an we exclude a simultaneous acute hepatitis B.
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PMID:Primary hepatocellular carcinoma with hepatitis B virus infection in a 16-year-old noncirrhotic patient. 618 92

Radioimmunodetection with an 131I anti-alpha-fetoprotein (alpha FP) has been performed in 5 patients with germ cell tumors. In 3 of them, 5 of 6 recognized or suspected tumor sites could be demonstrated. In 1 of these patients, a clinically non-apparent primary tumor was detected by this method. The 2 other patients had received intensive chemotherapy and had a persistent elevation of serum alpha FP, although usual paraclinical examinations were normal. In these patients, the labelled antibody scan did not show any abnormality. These results suggest that radioimmunodetection can be useful in patients with nonseminomatous gonadal and extragonadal tumors at the time of presentation. Usefulness of radioimmunodetection in the follow-up of these patients remains to be determined. Even in our limited series, radioimmunodetection was of no help on localizing the sites of alpha FP secretion in treated patients in whom other methods had also failed to recognize the site of abnormal alpha FP synthesis.
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PMID:Radioimmunodetection of primary and metastatic germ cell tumors containing alpha-fetoprotein. 620 26

A mixed tumor of the omentum majus found at autopsy of a 28-year-old man with a huge liver tumor is described. The primary tumor was identified in the omentum majus by macroscopic, histologic and electron-=microscopic observations. The tumor was finally diagnosed as a mixed tumor which resembled a hepatoblastoma consisting of immature and embryonal types of epithelial component and of mesodermal tumor including hemangioma, osteoid and myxomatous tissues in the primary lesion. Widespread multiple metastases of the epithelial component mainly to the liver, and partially in the lungs, diaphragm and lymph nodes were noted. The levels of alpha-fetoprotein and carcinoembryonic antigen were within normal limits, but the epithelial component showed a resemblance to primitive hepatocytes. This tumor was discussed related to hepatoblastoma in an adult, with the features compatible with description of the same tumors reported previously in the literature.
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PMID:A mixed tumor of the omentum majus in an adult with discussion related to hepatoblastoma. 626 97


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