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The postoperative course was uneventful and he left the hospital on postoperative day 14. The excess weight from the resected specimen was 1914 g. The tumor was one hundred mm in diameter with many satellite nodules. The key tumor contained septums and hemorrhagic regions. Additionally, it contained a capsule with some extracapsular invasion. Microscopic examination unveiled that the tumor cells had been swollen and polygonal with massive round or irregular nuclei with rough chromatin aggregations, apparent nucleoli and mitoses. Multinucleated tumor cells have been also discovered and many blood lakes had formed in the tumor. These findings resulted within the diagnosis of moderately differentiated HCC with intrahepatic metastases, whereas no adenomatous parts have been observed during the tumor. While in the non-tumorous liver parenchyma, cells that has a clear cytoplasm have been positive for periodic acid-Schiff (PAS) staining; this finding was consistent with that of GSD (Figure ?(Figure66). Figure 6 Giant tumor while in the suitable lobe. A: Resected specimen showed a giant tumor of ten cm in diameter within the cut surface. The tumor contained septums and hemorrhagic regions; B: hematoxylin and eosin stain (HE) staining exposed the capsule with some extracapsular ... The present case had several recurrences of HCC that were past the Milan criteria at roughly ten mo immediately after surgery. He was followed-up with transcatheter arterial chemoembolization. DISCUSSION GSD-Ia is definitely an autosomal recessive disorder induced by glucose-6-phosphatase deficiency within the liver, kidneys and intestinal mucosa. The ailment is characterized by the impaired conversion of glucose from glucose-6-phosphate while in the liver, leading to fasting hypoglycemia and lactic acidosis[1,2]. Inside the existing case, the development of hypoglycemia and lactic acidosis led for the opportunity to utilize imaging studies for your diagnosis of the hepatic tumor. Though US, CT, MRI and CT mixed with arterial portography and hepatic arteriography are generally regarded as to become successful to the diagnosis of HCC, preoperative diagnosis was difficult in this case. Each and every imaging examine exposed benign and malignant functions. Additionally, FDG-PET/CT unveiled the incredibly fascinating findings that FDG accumulated at high levels within the non-tumorous hepatic parenchyma and at reduced levels in the tumor. As GSD-I is characterized by glucose-6-phosphatase deficiency, it was hypothesized the FDG ingested by hepatic cells was phosphorylated by hexokinase to FDG-6-phosphate, which accumulated in non-tumorous cells without dephosphorylation by glucose-6-phosphatase. Liver tumors are common in GSD-I, nearly all which are HCAs. Talente et al described that 27 of 37 (73%) GSD-Ia patients had HCAs detected by US. Meanwhile, the concomitant occurrence of other tumor types is uncommon.
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