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Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.
Should rare subtypes of pancreatic cancer be treated as its most common variant (ductal adenocarcinoma)? The most common type of pancreatic cancer is ductal adenocarcinoma. While much attention has been given to the molecular aspects and treatment aspects of this disease, rare variants of pancreatic cancer have been underappreciated. Some of them present unique molecular features that suggest different treatment approaches could lead to better outcomes. In this review, we summarize information on the clinical, pathological, and molecular features of rare subtypes of pancreatic cancer, along with subtype-specific data on treatment.
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OBJECTIVE: The objective of this study is to assess the clinical pathological attributes of Hepatoid Adenocarcinoma of the Stomach (HAS) and to delineate the differential diagnostic considerations about it. METHOD: The investigation involved analyzing 31 HAS cases using histomorphological assessment, immunohistochemical profiling, and relevant gene detection methodologies. RESULTS: Among the 31 HAS cases, 9 (29.0%) were of trabecular hepatoid adenocarcinoma of the stomach, 7 (22.6%) were of glandular hepatoid adenocarcinoma of the stomach, 4 (12.9%) were of nesting hepatoid adenocarcinoma of the stomach, 3 (9.7%) were of clear cell hepatoid adenocarcinoma of the stomach, and 8 (25.8%) were of diverse hepatoid adenocarcinoma of the stomach. Of these 31 cases, 24 were male, accounting for 77.4% of the cases. Serum alpha-fetoprotein (AFP) levels were notably elevated, with radioimmunoassay results reaching 1240 ng/ml; 28 out of 31 cases had AFP levels below 25 µg/l, accounting for 90.3%. Related genes: HER2 protein indicated positive expression on the cell membrane in 35.5% (11/31) of the cases; HER2 gene amplification detected by the FISH technique was 12.9% (4/31). Tumoral stromal lymphocytes exhibited a PD-1 positive expression rate of 58.1% (18/31). In gastric cancer tissues, the PD-L1 positive rate was 45.1% (14/31). CONCLUSION: HAS represents a distinctive subtype of gastric cancer with a propensity for mimicking other forms of tumors, underscoring the significance of discerning its unique histopathological attributes for accurate differential diagnosis and tailored therapeutic interventions.
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Adenocarcinoma , Neoplasias Gástricas , alfa-Fetoproteínas , Humanos , Neoplasias Gástricas/genética , Neoplasias Gástricas/patologia , Neoplasias Gástricas/metabolismo , Masculino , Adenocarcinoma/genética , Adenocarcinoma/patologia , Feminino , Pessoa de Meia-Idade , Idoso , alfa-Fetoproteínas/metabolismo , alfa-Fetoproteínas/análise , Idoso de 80 Anos ou mais , Adulto , Biomarcadores Tumorais/genética , Diagnóstico Diferencial , Receptor ErbB-2/metabolismo , Receptor ErbB-2/genéticaRESUMO
Different cancers have multiple genetic mutations, which vary depending on the affected tumour tissue. Small biopsies may not always represent all the genetic landscape of the tumour. To improve the chances of identifying mutations at different disease stages (early, during the disease course, and refractory stage), liquid biopsies offer an advantage to traditional tissue biopsy. In addition, it is possible to detect mutations related to metastatic events depending on the cancer types analysed as will be discussed in this case report, which describes a patient with brain metastasis and lung cancer that harboured K-RAS mutations both in the brain tumour and in the ctDNA present in the bloodstream.
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Neoplasias Encefálicas , Neoplasias Pulmonares , Humanos , Biópsia Líquida , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/diagnóstico , Mutação , Biópsia , Neoplasias Encefálicas/genética , Biomarcadores TumoraisRESUMO
The reported prevalence of canine hepatoid gland tumor (HGT) varies widely owing mainly to the lack of well-defined diagnostic criteria and poor interobserver agreement. The aim of the present study was to improve the level of diagnostic agreement among pathologists in canine HGT. Five pathologists diagnosed and classified morphological features in 57 cases of canine HGT and, based on their reports, diagnostic algorithms were devised using recursive partitioning analysis. The proportion of diagnoses of malignant hepatoid neoplasia among the five pathologists ranged from 26.3 to 50.9%. Interobserver diagnostic agreement was classified as fair (κ = 0.54) but improved to good (κ ~ 0.65) following application of two novel diagnostic algorithms based on histomorphological features as sebaceous differentiation, mitotic count, atypical mitosis and cellular atypia. This study has demonstrated that interobserver agreement in the diagnosis of canine HGT could be improved using novel algorithms. Further analyses are warranted to validate the proposed classification systems applying a higher sampling of canine HGTs.(AU)
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Animais , Variações Dependentes do Observador , Cães , Neoplasias das Glândulas Anais/diagnósticoRESUMO
Hepatoid adenocarcinoma of the stomach is an uncommon subtype of gastric cancer remarkably similar to hepatocellular carcinoma in histopathological analysis. It is also commonly associated with high serum alfa-fetoprotein and a poorer prognosis, despite the emergence of new therapeutic options. In recent years, next generation sequencing (NGS) technology has made it possible to identify and describe the genes and molecular alterations common to gastric cancer thereby contributing to the advancement of targeted therapies. A 62-year-old patient, with no prior risk factor for hepatocellular carcinoma (HCC), presented to the emergency room with dysphagia for solids, abdominal pain and weight loss of about 3 kilograms over 3 months. Histopathological analysis presented with disparities regarding HER2 and programmed death-ligand 1 (PD-L1) status in the primary and metastatic sites. We describe a case of a de novo metastatic, human epidermal growth factor receptor 2 (HER2) positive esophagogastric junction hepatoid adenocarcinoma. Although this is a rare subgroup of gastric cancer, treatment strategies were based in recent studies in immunotherapy and guided therapy, taking into consideration the molecular findings from the patient's tumor NGS analysis. Data about HER2 and PDL1 heterogeneity were also reviewed. Despite the aggressiveness and rarity of this histology, the patient had a good response to treatment.
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Hepatoid adenocarcinoma of the lung is a rare subtype of lung cancer. We report a case of a metastatic hepatoid adenocarcinoma of the lung with aggressive behavior, including biopsy and autopsy findings. The pulmonary tumors showed features indistinguishable from hepatocellular carcinoma and were diffusely positive for Hepatocyte Paraffin 1.
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Hepatoid adenocarcinoma of the lung is a rare subtype of lung cancer. We report a case of a metastatic hepatoid adenocarcinoma of the lung with aggressive behavior, including biopsy and autopsy findings. The pulmonary tumors showed features indistinguishable from hepatocellular carcinoma and were diffusely positive for Hepatocyte Paraffin 1.
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Humanos , Masculino , Pessoa de Meia-Idade , Adenocarcinoma , Carcinoma Pulmonar de Células não Pequenas , Carcinoma Hepatocelular , Neoplasias Pulmonares , Autopsia , Evolução FatalRESUMO
We present the case of a 27-year old female with an ovarian tumor and alpha-fetoprotein (AFP) of 1210â¯ng/m, a right salpingo-oophorectomy was performed and had conservative complementary staging by gynecologic oncologists. The histopathological report was primary hepatoid carcinoma of the ovary (HCO), clinical stage IA, complementary treatment was adjuvant chemotherapy with BEP and remains clinical, imaging and biochemically disease free in three years follow up.
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Neoplasias das Glândulas Anais/patologia , Carcinoma/veterinária , Doenças do Cão/patologia , Neoplasias Pélvicas/veterinária , Neoplasias das Glândulas Anais/diagnóstico por imagem , Neoplasias das Glândulas Anais/tratamento farmacológico , Sacos Anais , Animais , Antineoplásicos/uso terapêutico , Carcinoma/diagnóstico , Carcinoma/tratamento farmacológico , Carcinoma/patologia , Doenças do Cão/diagnóstico por imagem , Doenças do Cão/tratamento farmacológico , Cães , Imuno-Histoquímica/veterinária , Neoplasias Pélvicas/diagnóstico por imagem , Neoplasias Pélvicas/tratamento farmacológico , Neoplasias Pélvicas/secundário , Pelve/diagnóstico por imagem , Pelve/patologiaRESUMO
El adenocarcinoma hepatoide gástrico es un tumor maligno que tiene una morfología que se asemeja al carcinoma hepatocelular, y algunos poseen alfa feto proteína inmunorreactiva. Es de mal pronóstico, agresivo y letal. Se presenta el caso de una paciente de 84 años de edad que padecía un adenocarcinoma gástrico variedad hepatoide. El tratamiento consistió en una gastrectomía subtotal distal ampliada con linfadenectomía tipo D2. El objetivo de este trabajo es la presentación de un caso poco frecuente y la revisión de la literatura sobre este tema(AU)
Hepatoid gastric adenocarcinoma is a malignant tumor having morphology similar to that of the hepatocellulary carcinoma, and some of them have immunoreactive alpha fetoprotein. It is aggressive, lethal and its prognosis is bad. Here is a 84 years-old female patient who suffered hepatoid-type gastric adenocarcinoma. The treatment was subtotal distal gastrectomy plus D2-type lymphadenectomy. The objective of this paper was to present this unusual case and a literature review on this topic(AU)