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Retroperitoneoscopic adrenalectomy (RA) provides a direct approach to the adrenal gland. RA represents a complex approach with unique orientation that is less intuitive. The authors objectively evaluated the impact of mentorship on the performance of RA and also compared it with laparoscopic adrenalectomy (LA). After implementing the use of RA, a retrospective review of the operative experience of two high-volume endocrine surgeons was performed. Both surgeons participated in a hands-on RA mentorship. Clinical presentation and perioperative outcomes were compared. Subgroup analysis was used to compare RA pre- and postmentorship and with LA. Sixty-one LAs and 31 RAs were included in the analysis. The mean operative time was 115 for LA versus 90 minutes for RA (P = 0.002). Blood loss was greater for LA versus RA (56 vs 22 mL; P = 0.001). Length of stay (LOS) for LA was 2.2 versus 1.5 days for RA (P = 0.029). Ten patients were treated by RA in the prementorship era versus 21 in the postmentorship era. The mean operative time for the prementorship group was 118 minutes, which decreased to 77 minutes postmentorship (P < 0.0001). LOS also decreased from 2.0 to 1.2 days (P = 0.04) in the postmentorship era. RA demonstrates a shorter operative time, less blood loss, and decrease length of hospital stay as compared with standard LA. After proper mentorship and patient selection, RA may represent a superior option for removal of small, benign adrenal tumors.
PURPOSE - Create a Raman spectroscopic database with potential to diagnose cancer and investigate two different diagnostic methodologies. Raman spectroscopy measures the energy of photons scattered inelastically by molecules. These molecular signatures form the basis of identifying complex biomolecules and can be used to differentiate normal from neoplastic tissue.
METHODS - 1,352 spectra from 55 specimens were collected from fresh or frozen normal brain, kidney and adrenal gland and their malignancies. Spectra were obtained utilizing a Renishaw Raman microscope (RM1000) at 785 nm excitation wavelength with an exposure time of 10 to 20 s/spectrum over three accumulations. Spectra were preprocessed and discriminant function analysis was used to classify spectra based on pathological gold standard.
RESULTS - The results of leave 25 % out training/testing validation were as follows: 94.3 % accuracy for training and 91.5 % for testing adrenal, 95.1 % accuracy for training and 88.9 % for testing group of brain, and 100 % accuracy for kidney training/testing groups when tissue origin was assumed. A generalized database not assuming tissue origin provided 88 % training and 85.5 % testing accuracy.
CONCLUSION - A database can be made from Raman spectra to classify and grade normal from cancerous tissue. This database has the potential for real time diagnosis of fresh tissue and can potentially be applied to the operating room in vivo.
BACKGROUND - Despite significant advances in the treatment of intrahepatic lesions, the prognosis for patients with hepatocellular carcinoma (HCC) who have extrahepatic metastasis remains poor. The objective of this study was to further elucidate the clinical course and prognostic determinants of patients with this disease.
METHODS - In total, 342 patients who had HCC with extrahepatic metastasis were enrolled. The metastases were diagnosed at initial presentation with HCC in 28 patients and during follow-up in the remaining patients. The authors analyzed clinical features, prognoses, and treatments and established a scoring system to predict prognosis using a split-sample method with a testing set and a training set.
RESULTS - The most frequent site of extrahepatic metastasis was the lung followed by lymph nodes, bone, and adrenal glands. These metastases were related directly to death in only 23 patients (7.6%). The median survival after diagnosis of extrahepatic metastasis was 8.1 months (range, 0.03-108.7 months). In univariate analysis of the training set (n = 171), performance status, Child-Pugh classification, the number and size of intrahepatic lesions, macroscopic vascular invasion, symptomatic extrahepatic metastases, α-fetoprotein levels, and complete responses to treatment were associated significantly with prognosis. On the basis of multivariate analysis, a scoring system was developed to predict prognosis that assessed uncontrollable intrahepatic lesions, extent of vascular invasion, and performance status. This scoring system was validated in the testing set (n = 171) and produced a concordance index of 0.73.
CONCLUSIONS - The controllability of intrahepatic lesions and performance status were identified as important prognostic factors in patients with advanced HCC who had extrahepatic metastasis.
Copyright © 2011 American Cancer Society.
Germline mutations in the von Hippel-Lindau disease (VHL) and succinate dehydrogenase subunit B (SDHB) genes can cause inherited phaeochromocytoma and/or renal cell carcinoma (RCC). Dysregulation of the hypoxia-inducible factor (HIF) transcription factors has been linked to VHL and SDHB-related RCC; both HIF dysregulation and disordered function of a prolyl hydroxylase domain isoform 3 (PHD3/EGLN3)-related pathway of neuronal apoptosis have been linked to the development of phaeochromocytoma. The 2-oxoglutarate-dependent prolyl hydroxylase enzymes PHD1 (EGLN2), PHD2 (EGLN1) and PHD3 (EGLN3) have a key role in regulating the stability of HIF-α subunits (and hence expression of the HIF-α transcription factors). A germline PHD2 mutation has been reported in association with congenital erythrocytosis and recurrent extra-adrenal phaeochromocytoma. We undertook mutation analysis of PHD1, PHD2 and PHD3 in two cohorts of patients with features of inherited phaeochromocytoma (n=82) and inherited RCC (n=64) and no evidence of germline mutations in known susceptibility genes. No confirmed pathogenic mutations were detected suggesting that mutations in these genes are not a frequent cause of inherited phaeochromocytoma or RCC.
Paragangliomas are catecholamine-secreting tumors arising from the chromaffin cells of the sympathetic ganglia, and are known as extra-adrenal pheochromocytomas. These tumors commonly present with episodic hypertension, tachycardia, headache, and diaphoresis, and can be either benign or malignant. Diagnosis is made by serum and urine analysis for catecholamines and metanephrines, and confirmed with imaging studies including computed tomography scanning, magnetic resonance imaging, or 123-I metaiodobenzylguanidine imaging. Although the majority of paragangliomas are sporadic, a growing percentage of cases are found to be part of a familial genetic syndrome. Genetic testing should be offered to patients diagnosed with paraganglioma, particularly in patients who are young, have multiple tumors, or have a family history of malignancy. Management of paraganglioma is predicated on surgical resection, and careful perioperative management with alpha- and beta-adrenergic blockade is imperative for optimal outcomes. The majority of these tumors are benign, but for patients with malignant disease, chemotherapy, and radiation therapy may provide modest benefit. Long-term follow-up is essential, as paragangliomas can recur many years after initial diagnosis. Ongoing research into the genetic underpinnings of this tumor may allow for more targeted molecular therapies in the future.
BACKGROUND - Extrahepatic metastasis of hepatocellular carcinoma (HCC) is of growing importance as the survival of patients has been improved owing to advances in treatments to intrahepatic lesions.
METHODS - To elucidate the incidence and risk factors of extrahepatic metastasis of HCC, we enrolled 1573 (1131 treatment-naïve and 442 previously treated on referral) patients with HCC without extrahepatic tumour spread treated at the authors' department between 1990 and 2003. Patients received medical treatment including percutaneous ablation and transcatheter arterial chemoembolization, and followed by dynamic computed tomography (CT) or magnetic resonance imaging (MRI) and tumour markers every 3-4 months. Extrahepatic metastasis was diagnosed by plain X-ray, CT, MRI and scintigraphy. Clinical parameters at the time of treatment to intrahepatic lesions were evaluated as a predictor of subsequent extrahepatic metastasis among the 1131 treatment-naïve patients by Cox's proportional hazard model.
RESULTS - During the average observation period of 3.9 years, extrahepatic metastasis was diagnosed in 123 in the treatment-naïve and 53 in the patients treated previously. The incidence rate of extrahepatic metastasis, as detected during the lifetime after medical treatment of HCC, was approximately 13% at 5 years. Multivariate analysis with Cox proportional hazard model revealed that positivity for viral markers, lager tumour diameter, multiple tumour nodules, presence of vascular tumour invasion and elevated tumour markers were associated with the development of extrahepatic metastasis.
CONCLUSION - The incidence of extrahepatic metastasis of HCC diagnosed during clinical course was not frequent. Advanced intrahepatic lesions, presence of vascular tumour invasion, elevated tumour markers and presence of viral hepatitis were risk factors for extrahepatic metastasis.
BACKGROUND - Serious complications of adrenalectomy are rare but the incidence may be underestimated if they occur outside major referral centers. We report five cases of high-grade complications after adrenalectomy that have not been previously described.
METHODS - The records of five cases of adrenalectomy performed at outside hospitals were reviewed. Four cases were referred for management of complications and one for medical-legal review. The nature of the adrenal lesion, operative approach, complication(s), and subsequent clinical course and complication management were assessed. Both open adrenalectomy (OA) and laparoscopic adrenalectomy (LA) cases were included.
RESULTS - Operative indications were pheochromocytoma (N = 3), aldosteronoma (N = 1), and a nonfunctioning 6-cm hypervascular mass (N = 1). Complications of adrenalectomy included: case 1--complete transection of the porta hepatitis during right LA resulting in hepatic failure requiring emergent liver transplantation; case 2--ligation of the hepatic artery during right OA resulting in recurrent cholangitis and bile duct sclerosis requiring liver transplantation; case 3--ligation of the left ureter during LA resulting in postoperative hydronephrosis and loss of renal function; case 4--loss of left kidney function after OA, likely secondary to renal artery ligation ultimately requiring laparoscopic nephrectomy; case 5--LA of a normal adrenal gland for a 6-cm hypervascular mass thought to be arising from the adrenal gland. Three-month postoperative imaging demonstrated a persistent mass and the patient underwent hand-assisted laparoscopic nephrectomy for a left upper pole renal cell carcinoma that was missed at the time of LA.
CONCLUSION - Despite the generally low morbidity of adrenalectomy, serious and potentially life-threatening complications can occur. Surgeon inexperience may be a factor in the occurrence of some of these complications which have not been previously described.
BACKGROUND - Laparoscopic adrenalectomy (LA) is the preferred surgical approach for pheochromocytomas. We have investigated the changes in diagnosis, management and outcome of pheochromocytomas treated since the widespread advent of LA.
METHODS - Data were collected retrospectively from 96 patients with pheochromocytomas that had been surgically treated at three tertiary referral centers.
RESULTS - There were 53 females. Mean age was 47 years (10-81). Tumors were found incidentally in 40% of patients. Of the 96 patients, 12 (13%) had familial syndromes. CT or MRI localized the adrenal lesion in all patients. MIBG scans obtained from 32 patients were concordant with the CT/MRI in 19, were false negative in 9 and misleading in 1, and altered management in only 3 patients. Mean tumor size was 5.6 cm (1.8-17). There were 92 adrenal pheochromocytomas and 9 paragangliomas. Laparoscopy was successful in 67 of 74 (91%) patients, with 20 of 67 (30%) having tumors of 6 cm or greater in size. Conversions to open procedures were performed in patients with 4 left, 2 right pheochromocytomas and 1 paraganglioma. Of the patients, 22 had an open procedure due to suspicion of malignancy or large tumors. Malignancy was observed in 4 of 92 (4.3%) pheochromocytomas and 4 of 9 (44%) paragangliomas. Average follow-up was 22 months (1-122). There were seven recurrences. Postoperative biochemical tests available in 64 patients were normal in 90%.
CONCLUSIONS - The diagnosis of pheochromocytoma was made incidentally in 40% of patients. MIBG is not necessary for unilateral non-hereditary pheochromocytomas localized by CT/MRI. LA is possible with excellent results in most patients, including for treatment of lesions 6 cm or greater in size with no signs of invasion. Laparoscopy should be used cautiously for paragangliomas because of a high rate of malignancy.