RESUMO
Hepatocellular adenoma is considered to occur exclusively in non-fibrotic livers. It is a heterogeneous entity and a molecular classification is now widely accepted. The most frequent hepatocellular adenoma subtype, namely inflammatory adenoma, harbor somatic activating mutations of genes involved in the interleukin-6 pathway that lead to high C-reactive protein and serum amyloid A expression. The aim of our study was to investigate a series of benign hepatocellular neoplasms developed on cirrhotic livers and characterized by an unequivocal histological diagnosis. We performed a clinical, pathological, and molecular study of 10 benign hepatocellular neoplasms developed in three patients with cirrhosis. Markers allowing hepatocellular adenoma classification were assessed by quantitative real-time PCR and immunohistochemistry. Samples were sequenced for CTNNB1, HNF1A, IL6ST, GNAS, STAT3, and TERT (promoter) mutations. A control series of 32 classical macronodules developed in cirrhosis related to various etiologies was screened by immunohistochemistry and gene sequencing. The three patients had cirrhosis related to metabolic syndrome and/or alcohol intake; two had a single tumor, while the third developed more than 30 lesions. Microscopic examination showed well-differentiated neoplasms sharing features with inflammatory adenoma including inflammatory infiltrates, sinusoidal dilatation, and dystrophic vessels. Sequencing revealed classical hotspot somatic mutations (IL6ST, n=8; STAT3, n=1; and GNAS, n=1) known to be responsible for IL-6/JAK/STAT pathway activation. Two classical high-grade macronodules demonstrated high serum amyloid A and/or C-reactive protein expression, without gene mutations. Altogether, our findings support the existence of rare inflammatory adenoma developed in cirrhosis.
Assuntos
Adenoma de Células Hepáticas/patologia , Doença Hepática Terminal/patologia , Cirrose Hepática/patologia , Neoplasias Hepáticas/patologia , Fígado/patologia , Adenoma de Células Hepáticas/complicações , Adenoma de Células Hepáticas/genética , Adulto , Cromograninas , Receptor gp130 de Citocina/genética , Receptor gp130 de Citocina/metabolismo , Doença Hepática Terminal/complicações , Doença Hepática Terminal/genética , Feminino , Subunidades alfa Gs de Proteínas de Ligação ao GTP/genética , Subunidades alfa Gs de Proteínas de Ligação ao GTP/metabolismo , Fator 1-alfa Nuclear de Hepatócito/genética , Fator 1-alfa Nuclear de Hepatócito/metabolismo , Humanos , Fígado/metabolismo , Cirrose Hepática/complicações , Cirrose Hepática/genética , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/genética , Masculino , Pessoa de Meia-Idade , Mutação , Regiões Promotoras Genéticas , Fator de Transcrição STAT3/genética , Fator de Transcrição STAT3/metabolismo , Telomerase/genética , Telomerase/metabolismo , beta Catenina/genética , beta Catenina/metabolismoRESUMO
Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors that originate from interstitial cells of Cajal or their stem cell-like precursors. Generally, GISTs have specific c-KIT gene mutations. The incidence of GISTs is estimated to be 10 to 20 cases/one million individuals, and GISTs typically affect people over 50 years of age. The majority of GISTs are solitary. However, multifocal GISTs have been observed, especially in children. We report on two unusual adult cases of double GISTs that were treated by laparoscopic surgery. The first patient presented a polypoid mass of the fundus and a second isolated smaller tumor in the posterior wall of the lesser curvature of the stomach. A histopathological examination confirmed that both tumors were GISTs and were c-KIT-positive. A total laparoscopic gastrectomy was performed. In the second patient, GISTs were identified at the level of the fundus and the greater curvature of the stomach. A laparoscopic partial sleeve gastrectomy was performed. Both surgeries were successful with no complications or relapses at three to five years following surgery.
Assuntos
Gastrectomia , Tumores do Estroma Gastrointestinal/cirurgia , Laparoscopia , Neoplasias Gástricas/cirurgia , Feminino , Tumores do Estroma Gastrointestinal/patologia , Humanos , Pessoa de Meia-Idade , Prognóstico , Neoplasias Gástricas/patologiaRESUMO
The study aimed to compare laparoscopic wedge resection (LWR) versus open-wedge resections (OWR) for primary gastrointestinal stromal tumors (GISTs) of the stomach. Twenty-five patients who underwent LWR were matched by tumor size and location with 25 patients who underwent OWR. GISTs close to the pylorus or the esophagogastric junction, incidental, and metastatic GISTs were excluded. Demographic, clinical, and histologic variables did not differ between groups. Estimated blood loss, time to flatus, and duration of hospitalization were significantly lower in the LWR group. Overall, 6 patients developed minor postoperative complications that were medically treated. Mortality was nil. No group difference was observed for the incidence of diseases during the follow-up (average, 46.8 mo). The OWR procedure had significantly higher costs (+34%) than the LWR. Both techniques appear safe and oncologically feasible. However, laparoscopy is associated with faster recovery and shorter hospital stay, which reflect advantages in terms of contracted costs for the health care system.