Hepatoid adenocarcinoma of the lung:A case report

BACKGROUND Hepatoid adenocarcinoma of the lung(HAL)is a rare type of non-small cell lung cancer(NSCLC),histologically similar to hepatocellular carcinoma.HAL has high malignancy and poor prognosis,and a better treatment plan needs further study.CASE SUMMARY In order to deeply understand the occurrence and development of HAL,here we report a case of HAL with extensive metastasis of alpha fetoprotein negative KRAS A146T mutation.The patient refused chemotherapy and received one course of treatment(immune checkpoint inhibitors),and died three months later due to progressive disease.CONCLUSION HAL is a special type of NSCLC.The surgical treatment of HAL in the limited stage can achieve long-term survival,but most of them were in the advanced stage when they were found,and the prognosis was poor,which requires multidisciplinary comprehensive treatment.

Progress and current perspectives of diagnosis and treatment of hepatoid adenocarcinoma of the stomach

Hepatoid adenocarcinoma of the stomach(HAS)is a rare malignant gastric tumor exhibiting both hepatocellular and adenocarcinomatous differentiation.Patients are often diagnosed at an advanced stage,and their clinical symptoms closely resemble those of gastric adenocarcinoma.Because of its rarity,misdiagnosis and missed diagnoses are prevalent.Compared with gastric adenocarcinoma,HAS typically exhibits higher invasiveness and amore unfavorable prognosis.This review aimed to elaborate on the pathological features,potential mechanisms,clinical characteristics,diagnosis,and prognosis of HAS.The insights provided aimed to contribute robust guidance for the clinical management of patients with HAS.

Clinicopathological Characteristics and Prognosis of Hepatoid Adenocarcinoma of the Stomach:Evaluation of a Pooled Case Series

Hepatoid adenocarcinoma of the stomach (HAS)is an extremely rare and unique gastric malignancy.The present study aimed to examine the relevance of the clinicopathological characteristics of HAS with patient prognosis.We retrospectively reviewed clinical data of 34 HAS patients treated at our institution between January 2010 and December 2016,as well as 294 cases reported prior to 2017 in research databases.Among these patients,45.6%(115/252)had lesions in the gastric antrum and 77.0%(235/305)were male.Elevated levels of serum alpha-fetoprotein (AFP)were detected in most patients(75/93,80.6%).Vascular invasion(199/286,69.6%),lymph node metastasis (222/283,78.4%),and preoperative distant metastasis (121/328,36.9%)were commonly observed.The 5-year disease-free survival (DFS)and disease-specific survival (DSS) were 20.7%and 29.2%,respectively.DFS and DSS of patients receiving neoadjuvant therapy were significantly higher than those of patients receiving postoperative adjuvant therapy [DFS:P<0.001, hazard ratio (HR)=-1.831,95%confidence interval (CI):0.060-0.429;DSS:P<0.001,HR=-2.185, 95%CI:0.032-0.401].In conclusion,HAS exhibits distinct clinicopathological characteristics and a strikingly worse prognosis when compared with common gastric cancer.Complete surgery,early pTNM stage,and adjuvant therapy may predict a more favorable prognosis.Neoadjuvant therapy is strongly recommended for patients with lymph node metastasis or/and preoperative distant metastasis.

Hepatoid adenocarcinoma of the colon in a patient with inflammatory bowel disease

A case is presented of a 36-year-old male with primary sclerosing cholangitis-associated inflammatory bowel disease(PSC-IBD) and two synchronous stage 1 adenocarcinomata of the colon,who was initially treated with a subtotal colectomy with ileostomy.One year later,the patient presented with extensive intra-abdominal lymphadenopathy and peritoneal carcinomatosis,as well as a markedly elevated serum level of alpha-fetoprotein(AFP).Fine needle aspiration biopsy of a porta hepatis lymph node revealed a metastatic hepatoid adenocarcinoma.Subsequent review of the previous colectomy specimen showed that one of the previously identified adenocarcinomata had features suggestive of a hepatoid colonic adenocarcinoma.The patient was subsequently treated with a cytotoxic regimen of FOLFOX(oxaliplatin,leucovorin,5-fluorouracil) and bevacizumab,with stable results being achieved after six months.This case presents the first known report of PSC-IBD associated with synchronous typical and hepatoid adenocarcinomata of the colon and highlights the importance of considering hepatoid adenocarcinoma as a differential diagnosis in patients with an increasing serum AFP level.

Gastric hepatoid adenocarcinoma: A computed tomography report of six cases

We describe the computed tomography(CT)imaging findings in six cases(five males and one female;age range 61-78 years;mean age 67.3 years)with histologically proven hepatoid adenocarcinoma of the stomach(HAS).Five of the six patients had elevated serum alpha-fetoprotein levels.The most common type of gross appearance HAS on CT is a polypoid mass(83%,5/6).The most common contrast enhancement pattern was heterogeneous.All six patients had a regional lymphadenopathy larger than 6 mm in its short axis.Liver metastases(n=3)were noted.Venous tumor thrombosis was identified in the portal vein(n=2)of the regions near primary gastric tumors or metastatic masses.Our findings suggest in an elderly,male patients with a large heterogeneous enhancement tumor,the presence of distant metastases,regional lymphadenopathy and characteristically increased serum alphafetoprotein levels indicates a high likelihood of HAS.

Liver metastasis from hepatoid adenocarcinoma of the stomach mimicking hepatocellular carcinoma: Dynamic computed tomography findings

AIM: To evaluate the dynamic computed tomography(CT) findings of liver metastasis from hepatoid adenocarcinoma of the stomach(HAS) and compared them with hepatocellular carcinoma(HCC).METHODS: Between January 2000 and January 2015, 8 patients with pathologically proven HAS and liver metastases were enrolled. Basic tumor status was evaluated for the primary tumor location and metastatic sites. The CT findings of the liver metastases were analyzed for tumor number and size, presence of tumor necrosis, hemorrhage, venous tumor thrombosis, and dynamic enhancing pattern.RESULTS: The body and antrum were the most common site for primary HAS(n = 7), and observed metastatic sites included the liver(n = 8), lymph nodes(n = 7), peritoneum(n = 4), and lung(n = 2). Most of the liver metastases exhibited tumor necrosis regardless of tumor size. By contrast, tumor hemorrhage was observed only in liver lesions larger than 5 cm(n = 4). Three patterns of venous tumor thrombosis were identified: direct venous invasion by the primary HAS(n = 1), direct venous invasion by the liver metastases(n = 7), and isolated portal vein tumor thrombosis(n = 2). Dynamic CT revealed arterial hyperattenuation and late phase washout in all the liver metastases.CONCLUSION: On dynamic CT, liver metastasis from HAS shared many imaging similarities with HCC. For liver nodules, the presence of isolated portal vein tumor thrombosis and a tendency for tumor necrosis are imaging clues that suggest the diagnosis of HAS.

Hepatoid adenocarcinoma of the stomach:Thirteen case reports and review of literature

BACKGROUND The aim of the present study was to examine the clinical characteristics of hepatoid adenocarcinoma of the stomach (HAS) and its diagnosis,treatment,and prognosis.CASE SUMMARY A retrospective analysis of 13 HAS cases was performed.The mean age of the 13patients was 66.08 years,and 10 of the 13 patients were male.Prior to treatment,the alpha-fetoprotein levels in the serum were elevated in 7 patients,the tumour was located in the distal or gastric body in 11 patients,and the gastroscopy pathological results showed that 3 patients had poorly differentiated tumours and that 8 patients had moderately/poorly differentiated tumours.Abdominal CT scans showed local stomach wall thickening,and enlarged lymph nodes were visible around the stomach in 8 patients.Of the 13 patients,11 underwent radical surgery.The clinical pathological staging was as follows:Stage Ⅱ in 2 cases;stage Ⅲ in 8 cases;and stage Ⅳ in 1 case.A total of 3 patients were lost to follow-up.Otherwise,as of the last follow-up,3 patients had survived for 56 mo,and the other 7 patients failed to achieve long-term survival (survival period of 1-56 mo).CONCLUSION HAS is a special type of gastric cancer,and the prognosis of HAS has improved compared with past prognoses.Measurement of alpha-fetoprotein,early diagnosis,active surgical treatment,and application of new diagnostic and treatment techniques are conducive to improving the prognosis of HAS.

Hepatoid carcinoma of the pancreas:A case report and review of the literature

BACKGROUND Hepatoid carcinoma(HC)is an extremely rare neoplasm that is morphologicallysimilar to hepatocellular carcinoma.HC has been described in various organs;however,HC of the pancreas is extremely rare.To our knowledge,only 38 caseshave been reported.We present a case of HC of the pancreas in a 36-year-oldmale patient.CASE SUMMARY A 36-year-old cachexic man with no significant past medical history wastransferred to our hospital with a history of painless jaundice,elevated bloodglucose and significant weight loss.Lab tests showed elevated serumtransaminases,bilirubin and alpha-fetoprotein levels.Magnetic resonanceimaging of the upper abdomen showed a diffusely enlarged pancreas,appearing“sausage-shaped”.Magnetic resonance cholangiopancreatography showedupstream ductal dilation secondary to stricture of the main pancreatic duct andthe common bile duct,which were not visible.Immunohistochemistry ofbiopsied tissue from a percutaneous pancreatic biopsy showed tumor cellpositivity for HepPar1,polyclonal carcinoembryonic antigen and CK19,suggestive of HC of the pancreas.The characteristics of 39 patients with HC ofthe pancreas were reviewed.CONCLUSION HC of the pancreas is more prevalent in males,and patients have a median age of57 years.It is most commonly asymptomatic or presents as abdominal back pain,and the pancreatic tail is the most common location.At the time of diagnosis,liver metastasis is often present.

Treatment of gastric hepatoid adenocarcinoma with pembrolizumab and bevacizumab combination chemotherapy:A case report

BACKGROUND Gastric hepatoid adenocarcinoma(GHA)is a rare and aggressive cancer that is characterized by foci with features of both hepatocellular differentiation and adenomatous differentiation.However,there is currently no standard treatment for this disease,which has a poor prognosis.CASE SUMMARY A 72-year-old male with a body mass index of 20.9 was diagnosed with GHA with perigastric lymph node and liver metastasis.He underwent first-line chemotherapy but that failed.Pembrolizumab and bevacizumab with chemotherapy were used in the second-line treatment.The progression-free survival and overall survival were 14 mo and 16 mo,respectively,after treatment.In addition,the main adverse reaction was tolerable.The patient did not die of tumor progression.CONCLUSION The combination of pembrolizumab and bevacizumab with chemotherapy is an effective and safe regimen for GHA and may be recommended as a new choice for GHA treatment.Further studies should evaluate this treatment in a larger cohort or a randomized controlled trial.

Alpha-fetoprotein-producing hepatoid adenocarcinoma of the lung responsive to sorafenib after multiline treatment:A case report

BACKGROUND Hepatoid adenocarcinoma of the lung(HAL) is an extremely rare malignant tumor,and many patients with HAL exhibit high levels of alpha-fetoprotein(AFP) expression.Currently,there is no standardized treatment strategy for advanced HAL and its prognosis is poor.CASE SUMMARY We report a 55-year-old man with unresectable AFP-related HAL.The largest cross-sectional area of the mass in the upper lobe of the left lung at the beginning of treatment was 8.46 cm × 6.53 cm.The patient’s serum AFP level was 9283 ng/m L.The mass increased in size to 8.86 cm × 8.21 cm after two courses of platinum-based combination chemotherapy and immunotherapy,and serum AFP reached its highest level(71232.2 ng/m L).The patient was treated with sorafenib(400 mg twice daily,per os).Forty days later,the mass was reduced to 5.63 cm × 5.29 cm and serum AFP level dropped to 786.8 ng/m L.The patient achieved partial remission for > 9 mo with sorafenib and an excellent biomarker response,as well as survival > 13 mo,which is among the longest reported for unresectable stage IV HAL.CONCLUSION This is the first report to document successful treatment of unresectable AFPrelated HAL with single-agent sorafenib after multiline therapy.

Hepatoid Carcinoma of the Pancreas:a Case Report

A 37-year old female patient suffering from an upper abdominal pain, accompanied by recent emaciation and anorexia that resulted from a two-month epigastric lump, visited a doctor in our hospital in January, 2007. Physical examination of the patient by palpation identified a 10.0 × 8.0 cm^2 lump from the xiphoid process above the middle and superior abdomen. The lesion was hard with a rough surface, the upper boundary unsharp, and the inferior and right boundary clearly apparent. The pain at the site of the lump was only apparent with touching at that site.

Gastric adenocarcinoma with hepatoid adenocarcinoma:A case report and literature review

Objective Hepatoid adenocarcinoma of the stomach is exceedingly rare. Only a few cases have been reported worldwide. In this report, we describe our pathological findings along with a review of the literature to improve our understanding of the disease and prevent misdiagnosis, as well as to provide evidence for its treatment and prognosis.Methods A 68-year-old male patient was admitted to our hospital(Dalian Municipal Central Hospital, Dalian, China) complaining of upper abdominal pain. Physical examination and regular laboratory blood tests showed no obvious abnormalities. A contrast-enhanced computed tomography scan displayed a 3.0 cm × 3.0 cm mass in the gastric antrum, but no metastasis was found in the adjacent organs.Results A radical gastrectomy was performed and postoperative histopathology showed a moderately differentiated adenocarcinoma with partial hepatoid adenocarcinoma.Conclusion Hepatoid adenocarcinoma of the stomach is a rare malignant neoplasm. The diagnostic criteria for hepatoid adenocarcinoma of the stomach depends mainly on the clinical, radiographic, and histopathological findings. Pathomorphology and immunohistochemical staining can be utilized to confirm the diagnosis.

Hepatoid adenocarcinoma of the stomach with neuroendocrine differentiation:A case report and review of literature

BACKGROUND Both hepatoid adenocarcinoma of the stomach(HAS)and neuroendocrine differentiation(NED)are rare histological subtypes of gastric cancer with unique clinicopathological features and unfavorable outcomes.HAS with NED is even rarer.CASE SUMMARY Here,we report a 61-year-old man with HAS with NED,as detected by gastric wall thickening by positron emission tomography/computed tomography for a pulmonary nodule.Distal gastrectomy was performed,and pathological examination led to the diagnosis of HAS with NED.However,liver metastases occurred 6 mo later despite adjuvant chemotherapy,and the patient died 27 mo postoperatively.CONCLUSION We treated a patient with HAS with NED who underwent adjuvant chemotherapy after radical surgery and still developed liver metastases.We first report the detailed processes of the treatment and development of HAS with NED,providing an important reference for the clinical diagnosis and treatment of this condition.

Multi-Metastatic Hepatoid Adenocarcinoma of the Lung: Report of Case

Hepatoid adenocarcinoma of the lung is a rare tumor with a poor prognosis. There are no specific treatment-recommendations for this cancer. We report a 59-year-old man, a former smoker, with this condition. He presented with a cough and computed tomography revealed a pulmonary nodule (50 × 47 × 48 mm) with a right pleural effusion, together with a cerebral right temporal-lobe lesion and a right adrenal lesion. The serum alpha-fetoprotein value was normal. Histological examination via bronchoscopic biopsy revealed an adenocarcinoma. Positive CK7, AE1/AE2, and TTF1 with granular cytoplasmic staining on immunohistochemical study confirmed the diagnosis of hepatoid adenocarcinoma of the lung, stage IV-B. The patient had received chemotherapies (carboplatin-pemetrexed, gemcitabine) and cerebral radiotherapy, but he died at eight months of the treatment as a result of tumor progression.

Clinicopathological and prognostic features of hepatoid adenocarcinoma of the stomach

Background Hepatoid adenocarcinoma of the stomach （HAS） is a rare type of gastric carcinoma, which has its unique clinicopathological features and poorer prognosis than that of the ordinary gastric adenocarcinoma. At present, there is still a lack of understanding about this disease. The current study aimed to summarize and discuss the clinical,pathological, immunohistochemical, and prognostic features of this disease.Methods A total of 20 patients of HAS were retrospectively studied. All the patients were treated in Cancer Hospital of Chinese Academy of Medical Sciences between March 1998 and October 2009. Statistical analysis, including the Kaplan-Meier method, log-rank test and Cox model, were performed by the SPSS 15.0 software.Results Seventeen patients （85%） had at least 1 lymph node metastases; 17 patients （85%） received postoperative immunohistochemical examinations, with an alpha-fetoprotein （AFP） positive rate of 94.1% （16/17）; 14 patients had distant metastases （including 12 liver metastases, 1 lung metastasis, and 1 celiac widespread metastases）, and one simultaneously had anastomotic recurrence and liver metastases. The overall survival time was 2-99 months （median：12.0 months）. The 3-year survival rate of the 20 patients was 17.2%. The 3-year survival rate of patients with complete hepatocyte-like regions and those with both hepatocellular carcinoma and adenocarcinoma regions was 20.0% and 17.5%, respectively （P=0.361）. The survival difference among the radical surgery group, palliative surgery group and no surgery group was statistically significant （P=0.022）. The Kaplan-Meier method and log-rank test showed that surgery,pTNM stages, and adjuvant chemotherapy were associated with prognosis （P 〈0.05）. The Cox model only confirmed that the pTNM stages and adjuvant chemotherapy had statistical significance for the prognosis of HAS （P〈0.05） due to the limited cases.Conclusions HAS is a special type of gastric carcinoma and has a poor prognosis. The pTNM stage is an independent risk factor for HAS. Multidisciplinary therapy, including surgery and chemotherapy, may improve the prognosis of HAS.

Hepatoid carcinoma of the pancreas:a case report

In 1970, a gastric adenocarcinoma patient with increased alpha fetoprotein （AFP） in his serum and liver metastatic tumor was reported by Bourreilile et al. At that time, it was realized that a gastric adenocarcinoma could produce AFP. In 1985, Ishikura et al reported a hepatocellular carcinoma （HCC）-like differentiation in a primary gastric tumor and first proposed the conception of a ＂hepatoid carcinoma＂. Subsequent documentation of this unique histopathologic feature has been made in other extrahepatic sites including the esophagus, papilla of Vater, colon, lung, gallbladder, adrenal gland, kidney, urinary bladder, ovary, uterus, vagina, testicle, and small intestine. However, the number of cases is very small. Hepatoid carcinoma is a primary extrahepatic neoplasm exhibiting features of hepatocellular carcinoma in terms of morphology, immunohistochemistry and biological behavior. But histopathologically it is very rare. Many but not all cases, present with an elevated serum AFP. Taking a wide view of various literatures it is evident that hepatoid carcinoma has mostly been found in the stomach and very rarely in the pancreas. To date, only eight cases of hepatoid carcinoma of the pancreas have been reported in the literature. We present a case of hepatoid carcinoma of the pancreas arising in an elderly man without evidence of residual disease eight months after local resection of the tumor.

Liver metastasis from hepatoid adenocarcinoma of the esophagus mimicking hepatocellular carcinoma

Alpha-fetoprotein(AFP)-producing adenocarcinoma,histologically mimicking hepatocellular carcinoma(HCC),is a distinct entity known as hepatoid adenocarcinoma(HAC).Reported cases of HAC arising from the esophagus are extremely rare.Due to common liver metastasis and elevated AFP levels in patients with esophageal HAC,differentiation of HAC with liver metastasis from HCC could be challenging.We describe a case of esophageal HAC that presented with a liver mass showing hepatoid features and elevated serum AFP levels.Initial presentation was suspicious for HCC.Upon further diagnostic work-up,the patient was diagnosed with esophageal HAC with liver metastasis.The distinction between these two entities is particularly important because HAC is more aggressive,and its therapeutic options are very limited.

胃肝样腺癌的CT特征

目的分析胃肝样腺癌的临床和CT特征,提高对本病的认识。资料与方法回顾性分析2012年9月—2023年4月苏州大学附属第一医院经病理证实的38例胃肝样腺癌患者的临床病理资料、实验室检查、CT资料,分析病灶大小、形态、密度、边界、强化方式、转移及侵犯等情况,总结其临床及CT特征。结果38例患者中,血清甲胎蛋白水平升高24例,免疫组化甲胎蛋白表达阳性32例。CT表现为胃壁增厚,门静脉期病变最大截面长径2.38~11.95cm,中位数为5.200(3.365,7.215)cm,23例伴溃疡,20例内见坏死,25例周围侵犯,14例出现肝脏转移,5例出现门静脉系统癌栓。结论胃肝样腺癌为罕见肿瘤,血清甲胎蛋白常增高,CT增强检查肿瘤常较大,可见坏死,渐进性或持续强化,易发生转移、侵犯门静脉,认识这些特征有助于提高诊断水平。

^(18)F-FDG PET/CT在胃肝样腺癌诊断中的价值

目的分析胃肝样腺癌的^(18)F-FDG PET/CT显像特征,结合血清AFP水平,两者联合提高对该病认识及诊断准确率。方法选取5例经病理学证实胃肝样腺癌患者的^(18)F-FDG PET/CT检查资料,分析患者的临床表现、原发病灶位置及形态、最大标准摄取值(SUVmax)、肿瘤标志物水平、预后情况。结果原发病灶位于胃窦3例,位于贲门-胃体处2例。5例病灶的SUVmax范围为7.58~23.6,平均值13.8±6.4。5例中位生存期为8个月。结论胃肝样腺癌是一种特殊类型癌,侵袭性强,临床表现无特异性,明确诊断有赖于影像学与病理结合,无肝脏病变时患者血清AFP升高具有重要的诊断提示意义。

原发子宫肝样腺癌2例临床病理分析及文献复习

肝样腺癌(hepatoid adenocarcinoma,HAC)是一种非常罕见的发生于肝外的特殊类型腺癌,因其形态学特征和免疫表型与肝细胞癌相似而得名。发生于子宫的HAC极为少见,检索国内外文献,目前原发于宫颈的HAC仅见2例报道^([1-2]),原发于子宫内膜的HAC共16例报道^([3-17])。本文收集2例原发性子宫HAC病例,并对以往文献进行了复习,探讨其临床病理特征,以期提高对子宫HAC的认识,为该病的治疗及预后提供依据。