1/172. Primary hepatic carcinoid in a renal transplant patient.There seems to be a world-wide increase in the incidence of tumors among immunosuppressed patients. Of 1350 renal allografts transplanted in the past 23 years at the Department of transplantation and Surgery, 56 cases were malignant tumors. The case of a 58-year-old female patient is reported, with disseminated primary carcinoid in the liver detected 86 days after renal transplantation. According to the literature only 39 patients with primary liver carcinoids have been reported until 1997, but this is the first where the carcinoid developed in an immunosuppressed patient. The rapid progression of the carcinoid could be associated with the immunosuppression.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
2/172. Extramammary Paget's disease with aggressive behavior: a report of two cases.Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
3/172. Case report: Hepatocellular carcinoma in type 1a glycogen storage disease with identification of a glucose-6-phosphatase gene mutation in one family.A 40-year-old man with glycogen storage disease type 1a (von Gierke disease, GSD1a) developed hepatocellular carcinoma (HCC). Cold single-strand conformation polymorphism (SSCP) with 12% glycerol identified the G727T mutation in the glucose-6-phosphatase (G6Pase) gene, which has been reported to be the most common mutation in Japanese GSD1a patients. This case report is the first documentation of HCC in a case with G727T mutation. Given the prevalence of HCC in GSD1a with various germline mutations, analysis is needed to confirm that the germline mutation in this case is really related to hepatocarcinogenesis. dna analysis of the family pedigree of this case, revealed three individuals with GSD1a and seven heterozygous carriers of the G727T mutation. As the diagnosis of GSD1a in this family was made only after these three patients reached adulthood, dna diagnosis may help early identification of GSD1a patients and prevention of the progression of the disease. This dna-based diagnosis permits prenatal diagnosis in at-risk patients and may facilitate screening and counselling of patients clinically suspected of having this disease.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
4/172. Phantom steatosis of the liver: report of a case.A patient, referred under a diagnosis of metastatic liver tumors, was found to have multiple areas of focal fatty change (FFC) which, during follow-up, exhibited discordant evolutions. To our knowledge, this phenomenon-regression of a FFC lesion with concurrent appearance or progression of other similar lesions in the same patient, has been reported in only one previous case. FFC can be strongly suggested by clinical, biochemical and radiologic criteria. However, an exact diagnosis can only be made with biopsy. To avoid misdiagnosing a malignancy as FFC and vice versa, biopsy should be performed without hesitation in all patients in whom a change in approach is possible.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
5/172. Congenital hemihypertrophy and epithelioid haemangioendothelioma in a 10-year-old boy: a case report.Epithelioid haemangioendothelioma has not been previously described in a patient with congenital hemihypertrophy and diabetes mellitus. Hepatic nodules were incidentally discovered on a routine US examination searching for known associated abnormalities. Pulmonary nodules were present on chest X-ray and CT of the lungs. The diagnosis was confirmed by open biopsy of a hepatic nodule. Despite significant disease progression the patient remains symptom free.- - - - - - - - - - ranking = 33.159514669126keywords = disease progression, progression (Clic here for more details about this article) |
6/172. Manifestations of hypercholesterolaemia, hypoglycaemia, erythrocytosis and hypercalcaemia in patients with hepatocellular carcinoma: report of two cases.BACKGROUND: Hypercholesterolaemia, hypoglycaemia, hypercalcaemia and erythrocytosis, are well-known paraneoplastic manifestations in patients with hepatocellular carcinoma (HCC). Hepatocellular carcinoma patients manifesting with two or three paraneoplastic manifestations have been previously reported. However, HCC patients presenting with four paraneoplastic syndromes have not been previously reported. methods AND RESULTS: This manuscript describes two HCC patients who manifested with hypercholesterolaemia, hypoglycaemia, hypercalcaemia and erythrocytosis during their clinical course. Erythrocytosis appeared early when HCC was diagnosed and declined to within normal range after optimal therapy for HCC. Hypercholesterolaemia manifested initially after the diagnosis in case 1, declined to within normal range after lobectomy and was re-elevated after tumour recurred. With disease progression, hypercalcaemia and hypoglycaemia occurred 1-3 months after diagnosis and the both patients subsequently died several days later. CONCLUSIONS: Similar features found in both patients were large tumour burden, high serum alpha-fetoprotein level and rapid downhill clinical course even with optimal therapy. The clinical significance of these paraneoplastic manifestations is discussed.- - - - - - - - - - ranking = 33.159514669126keywords = disease progression, progression (Clic here for more details about this article) |
7/172. Primary hepatic carcinoid tumors confirmed with long-term follow-up after resection.Primary carcinoid tumor of the liver is very rare. Only 30 cases have been reported in the English literature. However, most of those cases were diagnosed only on the basis of diagnostic imaging and surgical exploration, their follow-up periods being up to 5 years. Considering the slow progression of the tumor, long-term follow-up is required to exclude occult extrahepatic primaries which may manifest afterwards, and to determine the clinical course of this disease. We experienced 3 patients with primary hepatic carcinoid tumors who underwent total resections. They all survived more than 7 years and were clinically confirmed as hepatic primary by failing to detect other primaries during their courses. One patient is disease-free for more than 7 years after resection. The other 2 were found to have recurrence in the remnant liver; 1 underwent transcatheter arterial embolization several times and is alive more than 4 years after recurrence, and the other underwent 2 more operations and survived 8 years after the 1st recurrence. Surgery might offer a possible chance of cure for primary hepatic carcinoid tumor, and transcatheter arterial embolization might be a good treatment option when an unresectable disease is confined to the liver.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
8/172. Generalized intraperitoneal seeding of hepatocellular carcinoma after microwave coagulation therapy: a case report.We first describe a case of generalized intraperitoneal seeding of hepatocellular carcinoma (HCC) after microwave coagulation therapy (MCT). A 61 year-old man underwent operative MCT for an exophytic HCC, 60 mm in diameter, in segment IV of his cirrhotic liver. Despite successful tumor ablation, the serum alpha-fetoprotein levels continuously rose after MCT. Five months later, radiographic examinations delineated several perihepatic masses with hypervascularity, and the patient presented with constipation. At the second laparotomy, there were numerous small peritoneal metastases involving the entire peritoneal cavity and slightly bloody ascites. An omental mass, 50 mm in diameter, involved the transverse colon. Most of these intraabdominal masses were removed together with the involved colon. Histologically, the initial tumor was a moderately differentiated HCC, and the peritoneal masses were poorly differentiated HCCs. The patient died of rapid tumor progression and bleeding 2 months later. In conclusion, we should be aware of the possible occurrence of peritoneal seeding after MCT for HCC. Every effort should be made to prevent this serious complication, particularly in cases of superficial, large, and less differentiated HCCs.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
9/172. Neuroendocrine carcinoma of the sigmoid colon: report of a case.Neuroendocrine carcinoma of the colon is a rare entity; however, this type of tumor is known for its aggressive progression and poor prognosis. A case of a 56-year-old Japanese male is presented in this report. A huge, child's head-sized tumor was found to have grown extraluminally on the sigmoid colon with multiple liver metastases. The tumor measured 16.5 x 15 x 8.2 cm in size and weighed 1 300 g. The patient died of hepatic failure due to massive liver metastases 6 months after operation. The pathological findings including an electron microscopic analysis were correlated with those of neuroendocrine carcinoma. We reviewed the English literature, and analyzed 94 cases of neuroendocrine carcinoma which had been reported previously. The nomenclature and definition of this disease still remains somewhat unclear, and not a small population of this disease may thus have been misdiagnosed and treated as other less aggressive entities. The necessity to make an accurate differential diagnosis in such cases is thus emphasized.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
10/172. A case with intrahepatic double cancer: hepatocellular carcinoma and cholangiocarcinoma associated with multiple von Meyenburg complexes.Combined hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC) is sometimes found in resected livers, however, cases with double cancer of HCC and CC are very rare. As well, the rarity of CC arising in von Meyenburg complexes (VMCs) is appreciated. We report the case of a 74-year-old man found to have intrahepatic double cancer composed of well-differentiated HCC and CC which exhibited a histologic progression from VMCs to adenomatous lesions and CC. To our knowledge, this is the first case report published in the literature of a double HCC and CC associated with multiple VMCs. The pathogenesis and previous associated reports of these lesions are discussed.- - - - - - - - - - ranking = 1keywords = progression (Clic here for more details about this article) |
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