Hepatoblastoma: Review of Pathology, Diagnosis and Modern Treatment Strategies

2020 ◽  
Vol 16 (4) ◽  
pp. 276-291
Author(s):  
Adil A. Abbas ◽  
Alaa M.N. Samkari ◽  
Abeer S. Almehdar

Hepatoblastoma (HB) is the most common primary malignant hepatic tumor of childhood and, occurring predominantly in the first two years of life. Approximately 100 cases are diagnosed every year in the United States of America. The management of HB has changed markedly over the last three decades. Alfa feto protein (AFP) and beta human chorionic gonadotrophin (beta HCG) are the main tumor markers and are markers for diagnosis and follow up. International collaborative efforts have led to the implementation of the Pre - Treatment Extent of the Disease PRETEXT staging system consensus classification to assess upfront resectability. Complete surgical resection plays a key role in successful management. Overall, outcomes have greatly improved over the past decades mainly because of advances in chemotherapy (CTR) agents and administration protocols, newer surgical approaches and liver transplantation (LT). Targeted medications towards the newly discovered β-catenin and Wnt genetic pathways in tumor cells may soon become an option for treatment. The current disease free survival (DFS) rates are approaching 85%. For the 25% of patients with metastasis at presentation, the overall survival (OS) remains poor. A more individualized approach to treating the heterogeneous spectrum of HB may become the basis of successful treatment in complex cases. Newer medications and surgical techniques are being exploited. Here we present a comprehensive review of the recent advances in the management of HB. A wide literature search was made using internet search engines such as PubMed and Google scholar. More than 100 articles were reviewed and the information extrapolated was arranged to produce this review.

2019 ◽  
Author(s):  
Brian E Kadera ◽  
Michael D’Angelica

Metastatic colorectal cancer isolated to the liver is a common clinical presentation in the United States, occurring in an estimated 50,000 patients per year. Unlike most stage IV malignancies, surgery is an effective mainstay of therapy. In the past several decades, novel surgical approaches, improved systemic chemotherapy, and locoregional therapies such as ablation and hepatic arterial infusion chemotherapy have broadened the indications for resection. At the same time, advances in perioperative care and adoption of parenchymal-sparing surgical techniques have lowered the perioperative mortality of liver resection to approximately 1%. Surgical cure is possible and using 10-year disease-free survival as a definition, this can be achieved in approximately 20 to 30% of well-selected patients. The majority of patients recur; thus, active surveillance is appropriate to identify patients for potential salvage therapy, including in some cases repeat resections and/or ablation, which is associated with prolonged survival and potential cure. More research is needed in biomarker drivers of prognosis, as there are few reliable clinicopathologic indicators to identify those in whom surgery will not benefit. This review contains 7 figures, 7 tables, and 90 references. Key Words: colorectal cancer, FOLFOX, FOLFIRI, hepatic arterial infusion, hepatic resection, liver remnant, microwave ablation, portal vein ligation, ALPPS


2019 ◽  
Author(s):  
Brian E Kadera ◽  
Michael D’Angelica

Metastatic colorectal cancer isolated to the liver is a common clinical presentation in the United States, occurring in an estimated 50,000 patients per year. Unlike most stage IV malignancies, surgery is an effective mainstay of therapy. In the past several decades, novel surgical approaches, improved systemic chemotherapy, and locoregional therapies such as ablation and hepatic arterial infusion chemotherapy have broadened the indications for resection. At the same time, advances in perioperative care and adoption of parenchymal-sparing surgical techniques have lowered the perioperative mortality of liver resection to approximately 1%. Surgical cure is possible and using 10-year disease-free survival as a definition, this can be achieved in approximately 20 to 30% of well-selected patients. The majority of patients recur; thus, active surveillance is appropriate to identify patients for potential salvage therapy, including in some cases repeat resections and/or ablation, which is associated with prolonged survival and potential cure. More research is needed in biomarker drivers of prognosis, as there are few reliable clinicopathologic indicators to identify those in whom surgery will not benefit. This review contains 7 figures, 7 tables, and 90 references. Key Words: colorectal cancer, FOLFOX, FOLFIRI, hepatic arterial infusion, hepatic resection, liver remnant, microwave ablation, portal vein ligation, ALPPS


2008 ◽  
Vol 132 (10) ◽  
pp. 1600-1607 ◽  
Author(s):  
Mary Kay Washington

Abstract Context.—Colorectal carcinoma is one of the most common types of cancer in Western countries and is consistently ranked among the top 3 causes of cancer-related deaths, with approximately 150 000 new cases in the United States and 55 000 deaths in 2006. The pathologist's assessment of tumor stage and stage-independent morphologic features, such as vascular/lymphatic invasion, influences treatment strategies for the individual patient, such as the decision to offer adjuvant therapy after surgery. However, although the pathologist influences clinical care in colorectal cancer, certain aspects of staging and evaluation of prognostic factors remain challenging and confusing. Objectives.—To present the currently used colorectal cancer staging system; to address challenging areas in pathologic staging, including T category considerations and recommendations for the minimum number of lymph nodes sampled; and to discuss assessment of selected stage-independent prognostic factors, such as vascular/ lymphatic invasion. Data Sources.—This review is based on the current staging manual from the American Joint Committee on Cancer, the College of American Pathologists Protocol for Examination of Specimens From Patients With Primary Carcinomas of the Colon and Rectum, and selected articles pertaining to colorectal carcinoma staging and prognostic factors accessible through Ovid Medline (National Library of Medicine, Bethesda, Md). Conclusions.—Proper assessment of pathologic staging for colorectal cancer and of morphologic prognostic factors requires a thorough understanding of staging guidelines and careful specimen dissection and sampling.


1992 ◽  
Vol 78 (2) ◽  
pp. 87-93 ◽  
Author(s):  
Radhakrishna Pillai ◽  
Prabha Balaram ◽  
M. Krishnan Nair

Cancer of the uterine cervix is the single largest female malignancy in India and also remains a major problem facing oncologists in other parts of the world. While advances in radiation therapy and surgical techniques have made the treatment of cervical carcinoma impressive, limitations to successful management still remain. In fact, the 5-year survival rate, stage for stage, has not improved in the United States or world wide in the past 40 years. With an estimated half a million women developing this disease annually, this lack of improved survival poses an international unresolved health problem. Immune response has been shown to be a major factor involved In the course of the disease for this cancer. Immunologic monitoring was also shown to be of effective value in assessing the prognosis for cervical carcinoma. We studied the various immunologic abnormalities in cervical cancer, the effects of radiation therapy on immune function, prospects of an immunologic staging system, the relationship between human papillomavirus infection and the Immune response, and the possibility of using in vitro Immunologic assessment to provide a better framework for more effective management of cancer of the uterine cervix.


Cancers ◽  
2020 ◽  
Vol 12 (5) ◽  
pp. 1347 ◽  
Author(s):  
Jeffrey Norton ◽  
Deshka Foster ◽  
Malini Chinta ◽  
Ashley Titan ◽  
Michael Longaker

Pancreatic cancer is the 4th leading cause of cancer deaths in the United States. The pancreatic cancer phenotype is primarily a consequence of oncogenes disturbing the resident pancreas parenchymal cell repair program. Many solid tumor types including pancreatic cancer have severe tumor fibrosis called desmoplasia. Desmoplastic stroma is coopted by the tumor as a support structure and CAFs aid in tumor growth, invasion, and metastases. This stroma is caused by cancer associated fibroblasts (CAFs), which lay down extensive connective tissue in and around the tumor cells. CAFs represent a heterogeneous population of cells that produce various paracrine molecules such as transforming growth factor-beta (TGF-beta) and platelet derived growth factors (PDGFs) that aid tumor growth, local invasion, and development of metastases. The hard, fibrotic shell of desmoplasia serves as a barrier to the infiltration of both chemo- and immunotherapy drugs and host immune cells to the tumor. Although there have been recent improvements in chemotherapy and surgical techniques for management of pancreatic cancer, the majority of patients will die from this disease. Therefore, new treatment strategies are clearly needed. CAFs represent an under-explored potential therapeutic target. This paper discusses what we know about the role of CAFs in pancreatic cancer cell growth, invasion, and metastases. Additionally, we present different strategies that are being and could be explored as anti-CAF treatments for pancreatic cancer.


2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 9503-9503 ◽  
Author(s):  
S. L. Cohn ◽  
W. B. London ◽  
T. Monclair ◽  
K. K. Matthay ◽  
P. F. Ambros ◽  
...  

9503 Background: Modern treatment strategies for neuroblastoma (NB) are tailored according to patient risk. However, it is not currently possible to compare the results of clinical studies conducted around the globe because the criteria used to define risk are not uniform. A committee of international investigators with expertise in NB have worked during the past 2 years to develop a uniform International NB Risk Group (INRG) Classification System for pre-treatment stratification. Methods: Investigators from North America and Australia (COG); Europe (SIOPEN and Germany), and Japan collated data on 8,800 children with NB diagnosed between 1990 and 2002. Survival tree regression analyses tested 13 potential prognostic factors. Tumor differentiation, MKI, and diagnostic category were evaluated individually in lieu of the International NB Pathology Classification (INPC) system to determine if these histologic features had prognostic value independent from age. To stage patients at the time of diagnosis prior to surgery, a new staging system was developed (INRGSS) based on the presence or absence of image-defined risk factors (IDRFs) and metastases. Results: Since statistical analyses demonstrated support for an optimal age cut- off between 14–19 months, 18 months was selected. In addition to age, stage, MYCN amplification, tumor differentiation, ploidy, and genetic aberrations of 11q were found to be the most highly prognostically significant factors. These clinical and biological factors were combined to define 15 INRG pre-treatment groups. Patients with low- (3 groups), intermediate- (4 groups), high- (4 groups), or ultra-high-risk NB (4 groups) had EFS of ≥85%, >70–85%, >50–70%, or <50%, respectively. Conclusion: International collaborative studies in NB will be greatly facilitated by the INRG classification system which will allow comparisons of different risk-based therapeutic approaches in homogeneous patient cohorts. No significant financial relationships to disclose.


2003 ◽  
Vol 15 (5) ◽  
pp. 1-6 ◽  
Author(s):  
Kevin C. Yao ◽  
Stefano Boriani ◽  
Ziya L. Gokaslan ◽  
Narayan Sundaresan

Object Spinal metastases are prevalent in the population of patients with cancer. Effective cancer therapy must incorporate treatment strategies for these lesions. Increasingly, surgery is being recognized as an effective treatment modality both for the patient's quality of life and potential oncological cure. En bloc spondylectomy is the surgical procedure of choice to obtain these goals. The purpose of this study was to examine critically the rationale, indications, and outcomes of en bloc spondylectomy for spinal metastases. Methods Outcomes in the authors' series of patients who underwent en bloc spondylectomy for spinal metastases are critically analyzed. The rationale and indications for this procedure are discussed. The Weinstein, Boriani, and Biagini surgical staging system for spinal tumors is described. A review of the literature is performed to examine further the rationale underlying this aggressive surgical approach to metastatic spinal disease. Conclusions En bloc spondylectomy is the treatment of choice for solitary and oligometastatic spinal metastases with biologically favorable histological findings. In appropriately selected patients, neurological outcome, pain control, and oncological control are significantly better after en bloc spondylectomy compared with radiation therapy. Oncological outcomes also exceed those of intralesional techniques. The Weinstein, Boriani, and Biagini surgical staging system provides a standard with which to plan surgical approaches and to compare surgical outcomes.


Neurosurgery ◽  
2007 ◽  
Vol 61 (suppl_1) ◽  
pp. SHC-375-SHC-389 ◽  
Author(s):  
Nobuo Hashimoto ◽  
Kazuhiko Nozaki ◽  
Yasushi Takagi ◽  
Ken-ichiro Kikuta ◽  
Nobuhiro Mikuni

Abstract Despite remarkable progress, the microsurgical extirpation of cerebral arteriovenous malformations (AVMs) even by experienced neurosurgeons is not always easy or safe. This article focuses on how to render AVM surgery safer, and offers strategies and tactics for avoiding perilous bleeding and preserving postoperative neurological function. Our treatment strategies and surgical techniques are offered from the operating surgeon's perspective. An understanding of pathophysiology of cerebral AVMs is important for their appropriate surgical treatment. Sophisticated neuroimaging techniques and scrupulous neurophysiological examinations alert to possible complications, and improved surgical approaches help to minimize the sequelae of unanticipated complications. At the early stage of cerebral AVM surgery, extensive dissection of the sulci, fissures, and subarachnoid cistern should be performed to expose feeders, nidus, and drainers. Problems with the surgery of large and/or deep-seated lesions are exacerbated when arterial bleeding from the nidus continues even after all major feeders are thought to have been occluded. We routinely place catheters for angiography at the surgery of complex AVMs to find missing feeding arteries or to identify the real-time hemodynamic status of the lesion. Temporary clip application on feeders and less coagulation of the nidus is necessary to control intranidal pressure and to avoid uncontrollable bleeding from the nidus and adjacent brain. Intraoperative navigation images superimposed on tractography images can provide us with valuable information to minimize neurological deficits. Deeper insight into AVM nature and into events that occur during AVM surgery as well as the inclusion of molecular biological approaches will open new horizons for the safe and effective treatment of AVMs.


2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 6504-6504 ◽  
Author(s):  
W. Stock ◽  
B. Moser ◽  
D. A. Sher ◽  
E. Schachter-Tokarz ◽  
M. Myers ◽  
...  

6504 Background: Refinement of prognostic groups using molecular genetic features of the disease may facilitate the design of future risk-adapted trials for APL. Previous analyses of the prognostic value of PML-RARα transcript levels and isoform type have generated conflicting results. We sought to determine the clinical significance of measuring PML-RARα transcript level at the time of diagnosis using real-time quantitative RT-PCR (RQ-PCR) of the long (L) and short (S) isoforms of the fusion gene. Methods: PML-RARα transcript levels were measured in pretreatment bone marrow (BM) and/or paired blood (B) samples of 139 patients (pts) with newly diagnosed APL registered to C-9710, the intergroup phase III randomized study of concurrent tretinoin and chemotherapy with or without arsenic trioxide as initial consolidation therapy. Transcript levels were expressed as the normalized quotient (NQ) of PML-RARα/ABL or PML-RARα/GAPDH. Correlations between pre-treatment NQ values and other pre-treatment characteristics including age, presenting WBC and platelet count, and L or S isoform were explored. Results: Pre-treatment NQ values using ABL vs. GAPDH showed significant correlation (p < 0.0001). Pre-treatment transcript levels in B and BM for 57 pts with S-form were significantly higher than 82 L-form patients (p = 0.02) when NQ was determined using GAPDH as the control gene (but not with ABL). With a median follow-up of 24.3 months for all patients, there have been 19 events. Median DFS for either isoform has not been reached, however, DFS was significantly shorter for patients with S-isoform compared to those with L-isoform (p = 0.017) with an estimated hazard ratio of 3.28. Using a multivariate proportional hazard model, no other significant relationships were found between DFS and NQ level at diagnosis, age, presenting WBC or platelet count. Conclusions: The S-isoform of the PML-RARa fusion gene was associated with shorter DFS in a subset of patients entered onto C-9710. If confirmed in a larger cohort of C-9710 pts, presence of the S-isoform should be considered as a high-risk feature in the design of future risk-adapted treatment strategies for APL. No significant financial relationships to disclose.


2018 ◽  
Vol 36 (2) ◽  
pp. 85-90
Author(s):  
Adam Honeybrook ◽  
Matthew Crowson ◽  
Charles Woodard ◽  
Jamil Asaria ◽  
Dane Barrett

Since Eugene Holländer performed the first rhytidectomy in 1901, face-lift surgery has dramatically transformed over 100+ years. Our objective was to assess conceptual themes and content evolution in the published rhytidectomy literature. A bibliometric rhytidectomy analysis was performed on English-language literature between the period of 1951 and 2017. Analyses included most productive authors, countries, journals, most-cited articles, and keywords. K-means clustering was used to discern trends in topics. A total of 1927 rhytidectomy abstracts were mined from the Scopus® bibliographic database over the period of 1951-2017. These abstracts originated from 500 different source publications and 3744 different authors. The annual growth rate of rhytidectomy literature is 6.87 articles/year. The most productive countries were the United States (856 publications), France (107 publications), and Brazil (67 publications). Between-country collaborations were rare. From 1995-2015, the recent literature centered on younger adults, surgical approaches, and complications. Older literature investigated surgical techniques, facial skin, aging, and lipectomy. Two distinct concept clusters were discovered: (1) surgical outcomes and techniques/approaches, and (2) study design. Bibliometric analyses are useful in understanding intellectual structure, gaps, and opportunities for knowledge advancement. The recent rhytidectomy literature is focused on new techniques/approaches, complications, and younger adults and is dominated by US-based clinicians.


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