Scintigraphic evaluation of reduced-size liver transplant from living related donor in Byler''s disease

Byler's syndrome is a rare form of autosomal recessive intrahepatic cholestasis that is fatal in children. A 10-year-old girl diagnosed with Byler's syndrome underwent reduced-size liver transplantation using lateral segments of her living mother's liver. The donor's and the recipient's liver functions after transplantation were evaluated using Tc-99m disofenin and Tc-99m SC to investigate morphology, liver perfusion, and hepatobiliary function.     

Scintigraphic evaluation of malignant melanoma lesions with Tc-99m tetrofosmin

Two cases of malignant melanoma (primary and metastatic lesions) imaged with Tc-99m tetrofosmin are reported. One patient showed intense uptake in a primary skin lesion of the thorax, and the other patient had accumulation in skin, cerebellum, breast, and lymph node metastases. Like Tc-99m MIBI, Tc-99m tetrofosmin imaging may be of clinical relevance in the evaluation of suspicious skin lesions and in patients with known cutaneous malignant melanoma in the assessment of recurrent disease during follow-up evaluation.     

The use of imaging studies in the diagnosis and management of thyroid cancer and hyperparathyroidism

With the variety of radiopharmaceutical agents and refined imaging techniques, thyroid and parathyroid imaging provides much valuable clinical information. The use of imaging is most important in the follow-up of differentiated (DTC) and medullary thyroid cancer (MTC). Patients with DTC are followed with serum thyroidglobulin and 131I whole body scintigraphy when the serum thyroglobulin level is elevated. When the 131I scintigram is negative, 201Tl scintigraphy may best identify the site of recurrent DTC. Alternative radioisotopes, ultrasound, CT, and FDG PET are also useful in localizing the site of DTC metastases. MTC recurrences and metastases are more difficult to image. Selective venous catheterization is the most sensitive and specific method for detecting areas of recurrent MTC. High-resolution ultrasound, CT, MR imaging, and scintigraphy are all capable of, and useful in, detecting macroscopic foci of metastatic tumor. Somatostatin receptor scintigraphy and 99mTc DMSA have been the most frequently used nuclear imaging agents in patients with recurrent MTC. Imaging for hyperparathyroidism remains controversial. Sestambi has become the preferred isotope for parathyroid scintigraphy; whereas high-resolution ultrasound is also frequently used. Preoperative imaging is being used as a method to allow a unilateral neck exploration, more recently, in conjunction with intraoperative 1-84 PTH assay and with intraoperative use of the gamma probe. Most often, parathyroid imaging is performed before reoperation for persistent hyperparathyroidism.     

Intermittent cholestatic jaundice and nonmetastatic prostatic carcinoma

Hepatomegaly and abnormal liver function can occur in nonmetastatic malignancies. A patient with metastatic prostatic adenocarcinoma that had spared the liver and extrahepatic biliary tree is described. He had puzzling episodes of jaundice for a period of 2 1/2 years. The results of appropriate investigations and an exploratory laparotomy performed dlring the patient's four antemortem hospitalizations were indicative of "recurrent intrahepatic cholestasis," the cause of which remained an enigma even after exploratory laparotomy. At autopsy, no evidence of hepatic metastases or extrahepatic biliary obstruction was found. Alcohol, hepatotoxic drugs, toxins, viral and chronic active hepatitis, hemolysis, and extrahepatic biliary obstruction were eliminated as causes of the jaundice. We believe that the intermittent intrahepatic cholestasis is one of the nonmetastatic manifestations (nonmetastatic hepatopathy of malignancy) of the prostatic adenocarcinoma.     

Misperfusion of the liver during hepatic artery infusion chemotherapy: value of preoperative angiography and postoperative pump scintigraphy

OBJECTIVE: One purpose of this study was to determine if patients who have anatomic variations in their hepatic arteries are at increased risk for complications associated with the use of intrahepatic arterial infusion pumps. We also tried to determine the value of perfusion studies obtained with 99mTc-microspheres or 99mTc-macroaggregated albumin in detecting postoperative hepatic or visceral misperfusion and in predicting complications in patients with anatomic variants despite pre- or intraoperative attempts to correct the arterial abnormality. SUBJECTS AND METHODS: We prospectively compared findings on scintigrams obtained after delivering the radionuclide through intrahepatic arterial infusion pumps with anatomic variations in hepatic arteries seen on celiac and superior mesenteric hepatic arteriograms obtained before placement of the pump in 49 consecutive patients with colon carcinoma metastatic to the liver. RESULTS: Despite pre- or intraoperative attempts to correct arterial abnormalities to ensure optimal perfusion of the liver in 24 patients with hepatic arterial anomalies seen on preoperative arteriograms, only two patients had normal findings on postoperative perfusion studies performed with 99mTc-microspheres and/or 99mTc-macroaggregated albumin. Abnormalities included perfusion of extrahepatic organs, including the spleen in 12 patients, stomach in seven, bowel in four, and pancreas in three. Eight patients had no perfusion of the left lobe of the liver, and three had no perfusion of the right lobe. Two patients had minimal or no perfusion of both lobes. In 23 of 25 patients with no demonstrable variations in vascular anatomy on preoperative celiac and superior mesenteric arteriograms, findings on hepatic pump scintigrams were normal. Of the 24 patients with abnormal scintigraphic findings, 20 had subsequent clinical complications. However, only two of the 25 patients with normal scintigraphic findings had clinical complications. CONCLUSION: Our results indicate that patients with anatomic variations in the hepatic arterial system are at high risk for misperfusion during chemotherapy despite pre- or intraoperative efforts to alter the perfusion for chemotherapeutic agents delivered by intrahepatic arterial infusion pumps. Misperfusion can be detected by using pump scintigraphy, and therefore patients should be closely monitored with 99mTc-macroaggregated albumin perfusion studies to ensure successful delivery of the chemotherapeutic agents and to avoid serious clinical complications caused by inadvertent perfusion of other organs.     

Histopathological diagnosis of intra- and extrahepatic neonatal cholestasis

The histopathology of the liver is fundamental for the differential diagnosis between intra- and extrahepatic causes of neonatal cholestasis. However, histopathological findings may overlap and there is disagreement among authors concerning those which could discriminate between intra- and extrahepatic cholestasis. Forty-six liver biopsies (35 wedge biopsies and 11 percutaneous biopsies) and one specimen from a postmortem examination, all from patients hospitalized for neonatal cholestasis in the Pediatrics Service of Hospital de Clínicas de Porto Alegre, were prospectively studied using a specially designed histopathological protocol. At least 4 of 5 different stains were used, and 46 hepatic histopathological variables related to the differential diagnosis of neonatal cholestasis were studied. The findings were scored for severity on a scale from 0 to 4. Sections which showed less than 3 portal spaces were excluded from the study. Sections were examined by a pathologist who was unaware of the final diagnosis of each case. Bile tract permeability was defined by scintigraphy of the bile ducts and operative cholangiography. The F test and discriminant analysis were used as statistical methods for the study of the hepatic histopathological variables. The chi-square method with Yates correction was used to relate the age of the patients on the date of the histopathological study to the discriminatory variables between intra- and extrahepatic cholestasis selected by the discriminant function test. The most valuable hepatic histopathological variables for the discrimination between intra- and extrahepatic cholestasis, in decreasing order of importance, were periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules, foci of myeloid metaplasia, and portal-portal bridges. The only variable which pointed to the diagnosis of intrahepatic cholestasis was myeloid metaplasia. Due to the small number of patients who were younger than 60 days on the date of the histopathological study (N = 6), no variable discriminated between intra- and extrahepatic cholestasis before the age of 2 months and all of them, except for the portal expansion, were discriminatory after this age. In infants with cholestasis, foci of myeloid metaplasia, whenever present in the liver biopsy, suggested intrahepatic cholestasis. Periportal ductal proliferation, portal ductal proliferation, portal expansion, cholestasis in neoductules, portal cholestasis and portal-portal bridges suggested extrahepatic obstructive cholestasis.     

Metastatic pulmonary, gastric, and renal calcification demonstrated on bone scintigraphy in a patient with malignant melanoma and renal failure

PURPOSE: This case report describes the clinical, scintigraphic, and pathologic findings in a patient with an unexpected finding of a cutaneous malignant melanoma. METHOD: Multiple imaging studies were done, as was a pathologic examination of a suspicious pigmented lesion on the patient's back. RESULT: A Tc-99m MDP bone scan showed diffuse uptake in the skeleton, lungs, kidneys, and stomach. CONCLUSION: Metastatic calcification, as shown by isotope scintigraphy, is an unusual manifestation of metastatic cancer from a primary cutaneous melanoma.     

Intraoperative determinants of unresectability for patients with colorectal hepatic metastases

BACKGROUND: Intrahepatic and extrahepatic factors are utilized by the surgeon in the decision-making process for the performance of hepatic resection for patients with colorectal metastases. Accurate preoperative and intraoperative staging are mandatory to avoid unnecessary surgery. In this report the intraoperative determinants of hepatic unresectability were evaluated. METHODS: This was a retrospective review of medical records from January 1985 to March 1996 of 62 patients with colorectal hepatic metastases who at the time of exploratory laparotomy were deemed to have unresectable disease based on intrahepatic or extrahepatic factors. The stage of the primary tumor, disease free interval, preoperative carcinoembryonic antigen, computed tomography portography, intraoperative ultrasound, and assessment of intrahepatic and extrahepatic tumor extension were evaluated. RESULTS: Intraoperative determination of the extent of required hepatic resection, including trisegmentectomy (9 patients; 15%) and total hepatectomy (10 patients; 16%), accounted for the majority of unresectable patients. Patients with > 4 metastases (8 patients; 13%) and satellitosis (6 patients; 10%) accounted for 23% of unresectable patients. Four patients had extensive nonmalignant hepatic parenchymal disease precluding resection. Thorough abdominal exploration revealed extrahepatic disease in 13 of 62 patients (21%). Routine periportal/celiac lymph node biopsies revealed metastases in an additional 12 patients (19%), 7 of whom (11%) had only periportal/celiac lymph node metastases. CONCLUSIONS: A meticulous abdominal exploration prior to hepatic resection for patients with colorectal metastases is essential to identify those patients with extrahepatic disease. Periportal and celiac lymph nodes commonly are involved by tumor. Therefore, routine periportal/celiac lymph node biopsies should be performed in the absence of other extrahepatic disease.     

Technetium-99m tetrofosmin and technetium-99m sestamibi imaging of multiple metastases from differentiated thyroid carcinoma

A 79-year-old male with follicular thyroid carcinoma metastasizing to the lung, bone and lymph nodes was subjected to whole-body scintigraphy using technetium-99m tetrofosmin and 99mTc-sestamibi. Both agents delineated the metastatic lesions and the two image qualities were comparable. We believe that 99mTc-tetrofosmin and 99mTc-sestamibi images may be helpful in localizing metastatic foci and substitute for thallium-201 in the follow-up of patients with differentiated thyroid carcinoma.     

Primary biliary cirrhosis complicated by severe hypoxemia

The case reported here is of a 61-year-old woman diagnosed with hepatopulmonary syndrome. She had had severe hypoxemia for 5 years. In room air, her arterial oxygen tension was low, and although we anticipated that treatment with oxygen would increase it, the treatment failed to do so. Pulmonary perfusion imaging with Tc-99m-labelled macroaggregated albumin showed an arterio-venous shunt in the lungs. Blood tests and liver scanning with Tc-99m-labelled galactosyl human serum albumin showed abnormalities of liver function, and per-rectal portal scintigraphy with Tc-99m pertechnetate showed severe portal hypertension. Inspection of a biopsy specimen taken under ultrasonographic guidance showed primary biliary cirrhosis of Scheuer stage I. The causes of hepatopulmonary syndrome are unknown, but seem to include a decrease in hepatic functional reserve, portal hypertension, or other factors. The development of hepatopulmonary syndrome due to portal hypertension in this patient is of interest.     

Tumour-to-normal uptake ratio of 90Y microspheres in hepatic cancer assessed with 99Tcm macroaggregated albumin

The selective delivery of a high dose of radiation to malignant hepatic tumours by infusion of non-biodegradable yttrium-90 (90Y) microspheres via the hepatic artery while sparing the non-tumorous liver parenchyma depends on the tumour-to-normal uptake ratio (T/N) of the therapeutic radiopharmaceutical. Using intrahepatic arterial technetium-99m macroaggregated albumin (99Tcm-MAA), the effect of tumour type, tumour vascularity assessed by hepatic angiography (HAG), tumour size and the degree of extrahepatic shunting on the T/N was investigated in 377 patients with hepatocellular carcinoma (HCC) and 25 patients with colorectal liver metastases. HCC was shown to have a wider range of T/N (0.2-26.5) compared with liver metastases (2.3-7.2). HCC with vascularity grade 1 on HAG had significantly lower T/N but there was no significant difference in HCC with higher vascularity grades. This confirmed that vascularity on HAG does not predict T/N. Overall there was no correlation between T/N and tumour size. Large tumours (> 20 cm) had a significantly lower T/N, probably due to necrosis in the tumour centres. A decrease in mean T/N with increasing percentages of lung shunting was observed in HCC. Determination of T/N by simulation with 99Tcm-MAA is recommended before internal radiation therapy with 90Y microspheres.     

Radioimmunoimaging assay of ovarian tumor with 99mTc labeled anti-carcinoembryonic antigen monoclonal antibody

Radioimmunoimaging (RI I) assay with 99mTc labeled anti-CEA monoclonal antibody was performed in 31 patients with suspected primary or recurrent malignant ovarian tumor. The radiation dose raging from 920MBq to 110MBq(1mgIgG) was administered i.v. for each patient. Anterior-posterior views of the pelvis and abdomen were obtained routinely with gamma-camera during 18-24 hour after the application of antibody. The results of RII were compared with the findings in operation or CT examination. Sensitivity in diagnosis was 100% (13/13), specificity was 94%(16/17) and metastatic foci sensitivity was 63%(10/16). The serum CA125 in this study appeared to be correlated with the results of RII. However, in 2 patients with normal serum CA125 titers RII revealed true positive. The preliminary clinical results of RII with 99mTc-MAb showed that RII is a promising method for the detection of malignant ovarian tumors.     

Fetal oxygen saturation and uterine contractions during labor

In the majority of cases, carcinoma of the thyroid presents as a cold nodule by radioiodine and Tc-99m sodium pertechnetate scintigraphy. Whereas the presence of a hot nodule usually implies a benign entity, it does not provide complete assurance against thyroid malignancy. Presented is a rare case of metastatic pure papillary thyroid carcinoma appearing as a hot nodule on Tc-99m sodium pertechnetate and I-123 sodium iodide scintigraphy. The implications of such a case, its management, and review of the pertinent literature are discussed.     

Better visualization of retinoblastoma metastases with Tc-99m sestamibi versus Tl-201

Tc-99m sestamibi has been shown to accumulate in several primary malignant tumors, but data regarding its use in the detection of distant metastases are limited. Despite its physical limitations, Tl-201 now has a definite place in the routine evaluation of certain primary and metastatic tumors. This report describes the value of Tc-99m sestamibi and its superiority to Tl-201 in the visualization of distant metastases in a 2-year-old girl with bilateral retinoblastoma. Three sites of soft tissue and bone metastasis were demonstrated by Tc-99m sestamibi imaging. Of these metastases, the one in the parieto-occipital region showed evident Tl-201 uptake, while the other two in the right arm and chest wall showed only slightly increased uptake, which could hardly be spotted without the confirmation of a later Tc-99m sestamibi scan. The scintigraphic findings were confirmed with histopathologic examination. Tc-99m sestamibi scan is effective and superior to Tl-201 in the detection of distant soft tissue and bone metastases from retinoblastoma.     

Resection for extrahepatic recurrence of hepatocellular carcinoma

A total of 277 patients with hepatocellular carcinoma (HCC) underwent hepatic resection over a 20-year period. Twelve of 36 patients with recurrence confined to extrahepatic organs underwent surgical resection. There were no complications but one patient died in hospital from secondary intrahepatic recurrence. The 1-, 2- and 5-year survival rates for these 12 patients after hepatic resection were 92, 52 and 26 per cent respectively and were better than those of 24 patients who did not undergo resection for recurrence. The mean survival following resection for recurrent disease was 19.7 months and the longest survival time was nearly 8 years. Secondary recurrence after resection of metastases developed more commonly in the liver than in extrahepatic organs. Among the eight patients who survived for more than 4 months after the second operation, secondary recurrence developed in the liver and extrahepatic organs in eight and four patients respectively. In selected patients with isolated extrahepatic recurrence of HCC, surgery is effective in controlling extrahepatic disease and offers the only chance of long-term survival.     

Comparison of metabolic and receptor imaging in recurrent medullary thyroid carcinoma with histopathological findings

Early diagnosis of metastases of medullary thyroid carcinoma (MTC) provides the optimal condition for curative outcome. The aim of this study was to appraise the detection of metastases in patients with recurrent MTC using [111In-DTPA-d-Phe1]-pentetreotide and pentavalent technetium-99m dimercaptosuccinic acid [99mTc(V)-DMSA] in comparison with histopathological findings. Eighteen MTC patients with persistently elevated tumour marker (calcitonin, carcinoembryonic antigen) levels underwent somatostatin receptor scintigraphy using [111In-DTPA-d-Phe1]-pentetreotide (222 MBq) with early (4 h after injection) and delayed (24 h) whole-body scans and single-photon emission tomography (SPET) imaging. Metabolic whole-body and SPET imaging using 500 MBq 99mTc(V)-DMSA was performed 4 h after injection. Metabolic and receptor imaging revealed 51 sites of focal accumulation in the 18 patients investigated. Comparison with histological findings revealed that metabolic and receptor imaging had a sensitivity of 84% for the diagnosis of MTC. Using [111In-DTPA-d-Phe1]-pentetreotide, SPET discovered four lymph node metastases in two patients in whom planar views had previously identified only one lymph node metastasis, and provided no new information in the other 16 patients. In comparison, SPET studies [using 99mTc(V)-DMSA] additionally localized eight lymph node metastases in four patients and confirmed the diagnosis of hepatic metastases (n=5) in another patient in whom conventional imaging modalities and planar views had previously detected only three liver metastases. Overall, lesion detection sensitivities for 99mTc(V)-DMSA and [111In-DTPA-D-Phe1]-pentetreotide were 69% and 29%, respectively. Five surgically removed foci were adjudged false-positive with respect to MTC metastases. False-positve results were caused by lymphadenitis, an enchondroma and a pheochromocytoma (histologically proven). The smallest lesion identified by metabolic imaging was a 6 mm in diameter lymph node metastasis located in the upper mediastinum. Somatostatin receptor scintigraphy only demonstrated tumour sizes more than 1 cm in diameter. These preliminary results suggest that the combination of metabolic [99mTc(V)-DMSA] and receptor ([111In-DTPA-D-Phe1]-pentetreotide) imaging is more sensitive for tumour localization in patients with recurrent MTC than the use of only one radiopharmaceutical. However, neither 99mTc(V)-DMSA nor [111In-DTPA-D-Phe1]-pentetreotide is specific for MTC and false-positive scintigraphic findings have to be considered. Furthermore, somatostatin receptor scintigraphy cannot visualize small tumour sites (<1 cm). Further studies are needed to evaluate the role of combined metabolic and receptor imaging in the management of patients with recurrent MTC.     

Arteriovenous malformation mimicking recurrent medulloblastoma

Here we report on the case of a 51-year-old man who presented with a high-grade fever six weeks prior to hepatic resection. The resected specimen of the liver showed a well encapsulated and completely necrotic mass and some intrahepatic metastatic disease. Widespread multinodular recurrence in the liver remnant and metastatic foci in the bones were detected approximately one year after surgery. This case suggests that transcatheter arterial embolization--which aims to bring about necrosis of the tumor--followed by hepatic resection might increase the risk of early recurrence of hepatocellular carcinoma, due to the fact that tumor necrosis can facilitate the release of cancer cells from the primary tumor.     

Prognosis of hepatocellular carcinoma patients with extrahepatic metastases

BACKGROUND/AIMS: There is no effective treatment for hepatocellular carcinoma (HCC) with extrahepatic metastases. This study investigated the survival and causes of death in HCC patients with extrahepatic metastases. MATERIALS AND METHODS: We retrospectively analyzed 34 HCC patients with extrahepatic metastases who received systemic chemotherapy without other anticancer treatment except prior hepatectomy. We classified causes of death as cancer death and death from other causes, and subclassified cancer deaths into hepatic cause, extrahepatic cause and cachectic cause. Each cause of death was analyzed in the two subgroups comprised of 10 patients with bone metastases alone and 22 patients with metastatic lesions in sites other than bone. RESULTS: Thirty-two of the 34 patients had died at the time of analysis. The median survival time and the 1-year survival rate were 4.6 mo and 20.3%, respectively. Incidence of hepatic cause, extrahepatic cause, cachectic cause and death from other causes were 21 (66%), 7 (22%), 2 (6%) and 2 (6%), respectively. In the subgroup of 10 patients with bone metastases alone, nine (90%) died from hepatic causes, but none died from extrahepatic causes. In the group of 22 patients with metastatic lesions in sites other than bone, 7 (32%) patients died from extrahepatic causes. CONCLUSION: The causes of death in HCC patients with extrahepatic metastases depended on metastatic site at the time of diagnosis. The results of this study may be useful in the design and analysis of future clinical trials of the HCC therapy.     

Potentially multicentric hepatocellular carcinoma: clinicopathologic characteristics and postoperative prognosis

When multiple hepatic tumors are present, it is sometimes difficult to distinguish between metastatic and multicentric hepatocellular carcinoma (HCC). To identify the important clinicopathologic features of multicentric HCC, we evaluated the clinical characteristics of patients with multicentric HCC and examined the usefulness of surgical treatment in those patients. A total of 99 patients with multiple HCCs were classified into one of the following two groups according to whether their tumors were multicentric or metastatic: Group MO consisted of 18 patients with tumors thought to have developed synchronously from multicentric origins. Group IM consisted of 64 patients with intrahepatic metastases. In this study 18% of the patients with multiple HCCs were thought to have presented with multicentric tumors. This study revealed that synchronous multicentric HCCs often affected multiple segments of the liver and responded relatively well to partial hepatectomy of individual tumor-affected areas. To appropriately treat potentially multicentric HCC, it is important to understand the histopathologic characteristics of multicentric HCC and diagnose during preoperative and intraoperative ultra-sonography, so surgical treatment may be useful.     

Metastasis and invasion of hepatocellular carcinoma mimicking a right adrenal tumor

A 60-year-old man presented with a large right adrenal mass. Adrenal primary carcinoma invading the liver and retrohepatic inferior vena cava was suspected after preoperative imagings, which included ultrasonography, computed tomography, selective hepatic and adrenal angiography, and magnetic resonance imaging. An en bloc resection of the right kidney, right adrenal gland, posterior hepatic segment, and laterodorsal of the vena cava was performed using an active veno-venous bypass. The defect of the inferior vena cava was closed using a 6 x 10 cm patch of horse pericardium. The cut surface of the resected specimens revealed a smaller necrotic intrahepatic tumor as well as a large extrahepatic tumor which involved the right adrenal gland and extended continuously to the liver, mimicking an adrenal tumor. As the histological features of the two tumors disclosed the same moderately differentiated hepatocellular carcinoma with a trabecular or pseudoglandular pattern, a huge mass of the right adrenal gland with invasion into the right lobe of the liver, which mimicked a primary adrenal tumor, was diagnosed as metastatic hepatocellular carcinoma from a primary hepatic tumor.