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 review of 227 cases of hepatoblastoma, hepatic cell carcinoma in children seen in the United States over a 10-yr period is presented. Both tumors were seen most commonly in infancy, but the hepatocellular carcinoma shows a second peak of incidence around puberty. Males predominated in both diseases more so in hepatoblastoma. Presenting symptoms in both diseases were very similar, most commonly an upper abdominal mass or abdominal enlargement associated with anorexia and weight loss. In the preoperative evaluation the presence of alpha-feto protein was one of the most helpful diagnostic tests. Disturbances of liver function were usually mild but were more marked in those children with hepatocellular carcinoma. Preoperative x-rays were abnormal in a large percentage of cases with the hepatic arteriogram and vena cavagram being the most useful diagnostic x-rays for liver tumors. Liver scans were positive for liver tumor in 95% of the children when this test was carried out. The follow-up for these patients ranged from 2 to 10 yr. The size of the primary tumor did not appear to correlate with survival but bilateral location of the tumor, 33% in hepatoblastoma and 45% in hepatocellular carcinoma, made many of these tumors inoperable. Multicentric tumors were also found in a large number of patients, being more common in hepatocellular carcinoma. There was a high rate of local recurrence or local extension after operation in both diseases, and metastatic spread was similar being most common to the lungs and abdomen. A wide variety of surgical procedures were carried out in these patients from biopsy only to extended hepatic lobectomy. When incomplete excision or biopsy only was carried out no patient survived in either group. Among the hepatoblastoma patients, 45 of 78 patients who had complete excision are surviving. In the hepatocellular carcinoma patients where the operability rate was much lower 12 of 33 patients are surviving when tumor was completely excised. Complications were frequent, the most common being excessive blood loss at operation. There were eight operative deaths and 17 postoperative deaths in the combined group. There was no evidence that radiation therapy or chemotherapy controlled disease which could not be completely excised surgically. The only direct evidence of a favorable effect of radiation and chemotherapy were three cases of hepatoblastoma in which the tumor changed from inoperable to operable by a combination of radiation therapy and multiple drug chemotherapy. Both tumors are highly malignant, and 90% of the children who died of hepatoblastoma died within 12 mo of diagnosis. In the hepatocellular carcinoma 80% of the deaths occurred within 1 yr of diagnosis. At this time it seems that operative excision offers the only chance of cure in children with these tumors and cure rates of 60% can be expected with hepatoblastoma and 33% in hepatocellular carcinoma if the tumor can be completely excised.
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PMID:Liver tumors in children in the particular reference to hepatoblastoma and hepatocellular carcinoma: American Academy of Pediatrics Surgical Section Survey--1974. 4 16

The surgical evaluation and management of children with hepatoblastoma has changed with recent advances in imaging modalities and preoperative chemotherapy. Pediatric Oncology Group (POG) Study no. 8697 has followed 63 patients with hepatoblastoma from 1986 to 1991. Twenty-six patients underwent primary tumor resection followed by chemotherapy consisting of cisplatin, vincristine, and 5-fluorouracil (group I). Thirty-seven patients with "unresectable" tumors received preoperative chemotherapy. Twenty-nine of these patients responded to chemotherapy and 26 underwent delayed surgical resection (group II). Eight patients had an inadequate response to chemotherapy; two have had successful liver transplantation and six are dead of disease progression. "Unresectable tumor" involved both liver lobes (25 patients), encased the inferior vena cava (2), involved adjacent tissues (1), involved the hepatic veins (2), or was deemed too large for safe resection (7). Two patients had distant metastases. The reason for an unresectable designation was not reported in five patients. The determination for an unresectable designation included exploratory laparotomy in 14 patients, angiogram in 7, computed tomography scan in 20, and magnetic resonance imaging in 3 patients. Operative times and transfusion requirements were similar in both groups. Perioperative complications were higher in patients in group II. There was no mortality and only minor morbidity associated with chemotherapy in each group. In both groups 77% of the patients are in complete remission after 13 to 54 months. Preoperative chemotherapy can allow successful resection of initially "unresectable" hepatoblastoma. Primary resection that may result in exsanguination should be postponed and chemotherapy given.
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PMID:Chemotherapy can convert unresectable hepatoblastoma. 132 86

Although most children who die of liver malignancies do so as the result of complications of pulmonary metastases, little has been published regarding the efficacy of surgically excising such lesions. To the 12 previously reported cases of children who have undergone excision of pulmonary metastases of hepatic tumors, are added 5, 4 with hepatoblastoma and 1 with hepatocellular carcinoma. Total excision of a primary hepatic tumor leads to survival much more frequently than does incomplete excision. No patient had metastases at diagnosis. The length of time between resection of the primary tumor and the development of pulmonary disease resistant to chemotherapy is available for 9 of the 17 children; it was under 6 months for the 2 who died but over 6 months for the 7 who survived. Postoperative alpha-fetoprotein (AFP) levels accurately predicted the development of metastases in our 5 patients. Resection of metastases benefitted the 4 whose AFP levels had declined to less than 25 ng/mL following initial chemotherapy and who underwent operation before their levels increased above 1,000 ng/mL. They are alive and free of disease 4 to 83 months following excision of their lesions. Resection did not benefit the 1 nonsurvivor whose AFP level fell only to 5,000 ng/mL before beginning to increase, eventually reaching 58,000 ng/mL at the time of operation. Incomplete resection of metastases unresponsive to chemotherapy predictably leads to death. Multiple thoracotomies were successful in achieving the long-term survival of 4 children in this series.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Aggressive excision of pulmonary metastases is warranted in the management of childhood hepatic tumors. 171 81

Chemotherapy was used to treat 11 children with hepatoblastoma that was judged to be unresectable because of tumor tissue in both lobes of the liver (eight patients) or because of size of the primary tumor (three patients). Three with bilobar involvement also had metastatic disease. Adriamycin was used in all patients. In nine, it was used in combination with cisplatin. A combination of other agents was used in four of these children. After two to six cycles (mean, 4 cycles), eight primary tumors exhibited marked response with greater than 50% reduction in size. Metastases disappeared in two patients. Complete resection of residual tumor was attempted in eight cases, and was successful in seven. One patient died at the time of surgery during an extended right hepatectomy. Two children had anaplastic hepatoblastomas that did not respond to chemotherapy, and the children died. One responder with giant cell hepatitis died from a severe coagulopathy and bleeding during chemotherapy before surgery. With preoperative chemotherapy, seven of 11 children with "unresectable" hepatoblastoma are now alive without disease 4 to 42 months following successful resection.
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PMID:Preoperative chemotherapy in 'unresectable' hepatoblastoma. 254 11

We present herein a rare case of hepatoblastoma occurring in an adult male. The patient was 22 years old and his laboratory investigations on admission showed a marked elevation of alpha-fetoprotein in the serum. CT scan and other examinations revealed a primary tumor, 6.5 cm in size, in the left hepatic lobe with metastasis in the head of the pancreas. Thus left hepatic lobectomy and pancreaticoduodenectomy were performed, but metastasis to the right hepatic lobe, left lung and abdominal skin were found 2 months later. Despite repeated courses of chemotherapy with adriamycin and cisplatin, the patient died 9 months after his operation. Pathological findings revealed poorly differentiated type hepatoblastoma. A review of the literature revealed only twelve other such cases.
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PMID:A case of hepatoblastoma occurring in an adult. 255 43

Delayed primary operation has been started since 1982 in our department and 19 cases of solid tumor in childhood were treated with this method. They included 11 cases of advanced neuroblastoma, 5 cases of hepatoblastoma and 3 cases of yolk sac tumor in sacrococcygeal area. We discussed effectiveness of preoperative chemotherapy to the primary tumor and metastatic lesions, the best timing of operation after chemotherapy and the operative technique in delayed primary operation. Preoperative chemotherapy were useful in almost all cases of delayed primary operation, so the primary lesion and lymph nodes metastases were removed easily. We recommended it was the best timing of operation when 2 or 3 courses of preoperative chemotherapy were finished. CUSA (Cavitron Ultrasonic Surgical Aspirator) was the useful instrument to remove the primary tumor and lymph node metastases completely in advanced neuroblastoma preserving both kidneys.
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PMID:[Delayed primary operation of solid tumors in children]. 284 15

Four children with major hepatoblastoma were treated with extensive surgical excision and chemotherapy. Two right lobectomies and two left extended hepatectomies were performed. The first patient only received postoperative chemotherapy. The others three had preoperative chemotherapy for unresectable hepatoblastoma. All these children exhibited an initial response with marked reduction in size of their primary tumor. The only child who died during operation had disease involving both lobes of the liver, inferior vena cava and lungs metastasis. The follow up of the three other children is 26 to 32 months. Two of them are free of all disease. The third had lung isolated metastasis removed and is actually free of disease with follow up of 32 months. Authors discuss chemotherapy interest and limits in major hepatoblastoma.
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PMID:[Possibilities for surgical excision of diffuse hepatoblastomas. Contribution and limitations of chemotherapy]. 298 73

A case of an unusual type of hepatoblastoma in a 5-month-old male infant is described. The tumor showed the following unusual features as compared with the epithelial and mixed types of hepatoblastoma: (1) The tumor cells presented a primitive anaplastic appearance without any resemblance in terms of cytologic features or arrangement to embryonal or fetal liver. (2) Abundant mucoid material containing acid mucopolysaccharide was present giving a slimy, gelatinous, gross appearance to the tumor and its metastases. (3) Well-defined tubular structures were present in some foci within the tumor parenchyma. (4) The tumor resulted in a rapidly fatal course, with metastases to the lungs and widespread peritoneal seeding despite complete surgical resection of the primary tumor. Yolk sac carcinoma and undifferentiated (embryonal) sarcoma of the liver were considered in the differential diagnosis. On electron microscopic examination, the tumor cells showed cytoplasmic features and junctional complexes consistent with their epithelial origin. Alpha-fetoprotein in the blood, which was markedly elevated prior to surgery, returned to a normal level postoperatively. Hepatoblastoma with the combination of features described above has not been previously reported. Because of the two striking and easily recognizable features viz. total lack of differentiation of tumor cells and presence of abundant mucoid material, the authors designated the tumor as mucoid anaplastic hepatoblastoma.
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PMID:Mucoid anaplastic hepatoblastoma. A case report. 609 5

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


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