https://aasldpubs.onlinelibrary.wiley.com/doi/epdf/10.1002/hep.22734

Hepatocellular carcinomas in patients with metabolic syndrome often develop without significant liver fibrosis: A pathological analysis

https://aasldpubs.onlinelibrary.wiley.com/doi/pdf/10.1002/hep.22412

Excellent outcome following down-staging of hepatocellular carcinoma prior to liver transplantation: an intention-to-treat analysis.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1600-6143.2007.01965.x

Liver Transplantation for Hepatocellular Carcinoma: Validation of the UCSF-Expanded Criteria Based on Preoperative Imaging

Selected patients with hepatocellular carcinoma are candidates to receive potentially curative treatments, such as hepatic resection or liver transplantation, but nevertheless there is a high risk of tumor recurrence. Microvascular invasion is a histological feature of hepatocellular carcinoma related to aggressive biological behavior. We systematically reviewed 20 observational studies that addressed the prognostic impact of microvascular invasion, either after liver transplantation or resection. Outcomes were disease-free survival and overall survival. In liver transplantation, the presence of microvascular invasion shortened disease-free survival at 3 years (relative risk (RR) = 3.41 [2.05–5.7]; five studies, n = 651) and overall survival both at 3 years (RR = 2.41 [1.72–3.37]; five studies, n = 1,938) and 5 years (RR = 2.29 [1.85–2.83]; six studies, n = 2,003). After liver resection, microvascular invasion impacted disease-free survival at 3 and 5 years (RR = 1.82 [1.61–2.07] and RR = 1.51 [1.29–1.77]; four studies, n = 1,501 for both comparisons). However inter/intraobserver variability in reporting and the lack of definition and grading of microvascular invasion has led to great heterogeneity in evaluating this histological feature in hepatocellular carcinoma. Thus, there is an urgent need to clarify this issue, because determining prognosis and response to therapy have become important in the current management of hepatocellular carcinoma. In this systematic review, we summarize the diagnostic and prognostic data concerning microvascular invasion in hepatocellular carcinoma and present a basis for consensus on its definition.

A Systematic Review of Microvascular Invasion in Hepatocellular Carcinoma: Diagnostic and Prognostic Variability

Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel-Lindau disease (VHL), neurofibromatosis 1 (NF-1) (von Recklinghausen disease), and the tuberous sclerosis complex (TSC). The relative frequency with which patients who have these disorders develop PETs is MEN1>VHL>NF-1>TSC. Over the last few years, there have been major advances in the understanding of the genetics and molecular pathogenesis of these disorders as well in the localization and the medical and surgical treatment of PETs in such patients. The study of PETs in these disorders not only has provided insights into the possible pathogenesis of sporadic PETs but also has presented several unique management and treatment issues, some of which are applicable to patients with sporadic PETs. Therefore, the study of PETs in these uncommon disorders has provided valuable insights that, in many cases, are applicable to the general group of patients with sporadic PETs. In this article, these areas are reviewed briefly along with the current state of knowledge of the PETs in these disorders, and the controversies that exist in their management are summarized briefly and discussed.

/pdf/inherited-pancreatic-endocrine-tumor-syndromes-advances-in-16zs8fzwe0.pdf

Inherited pancreatic endocrine tumor syndromes: Advances in molecular pathogenesis, diagnosis, management, and controversies

https://aasldpubs.onlinelibrary.wiley.com/doi/pdf/10.1002/lt.20472

Tumor size predicts vascular invasion and histologic grade: Implications for selection of surgical treatment for hepatocellular carcinoma

Tumor size predicts vascular invasion and histologic grade

Von Hippel-Lindau disease (VHL) is a dominantly inherited multi-system syndrome. Although pheochromocytoma is the hallmark endocrine neoplasm, pancreatic lesions occur frequently, and their management can be complex. This report describes 26 patients from a single institution with pancreatic or adrenal lesions (or both) in the background of VHL. We reviewed records for all patients treated for adrenal (type 2 VHL) or pancreatic manifestations of VHL at our institution from 1990 to 2004. Forty patients with VHL were identified from 24 pedigrees; 26 (65%) had adrenal or pancreatic involvement (or both). Seven patients had isolated pheochromocytomas, 16 had isolated pancreatic lesions, and 3 had both. Of the 10 patients with pheochromocytomas, 4 had bilateral adrenal involvement. All but three pheochromocytomas were diagnosed in patients aged 40 years or younger. Of the 19 patients with pancreatic lesions, 8 had cystic lesions, 8 had neuroendocrine tumors, and 3 had both. Of 11 patients with neuroendocrine tumors, 4 had metastases (3 hepatic, 1 nodal). No patient with cystic pancreatic lesions developed carcinoma. VHL should be familiar to the endocrine surgeon because of the high incidence (65% in our series) of visceral endocrine lesions (pancreas 40%, adrenal gland 18%, both 7%). As seen in other hereditary endocrinopathy syndromes, pheochromocytomas are frequently bilateral; therefore,close follow-up of the contralateral gland in young patients with a unilateral lesion is critical. Cystic lesions of the pancreas may be closely monitored, whereas neuroendocrine tumors should be removed because of the risk of metastasis. Pancreatic pathology is not uncommon in VHL patients with pheochromocytoma and should be sought during the preoperative assessment.

Keith A. Delman

Papers

Tumor size predicts vascular invasion and histologic grade: Implications for selection of surgical treatment for hepatocellular carcinoma

Tumor size predicts vascular invasion and histologic grade

Abdominal Visceral Lesions in von Hippel-Lindau Disease: Incidence and Clinical Behavior of Pancreatic and Adrenal Lesions at a Single Center