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Abstract Background Hepatoid adenocarcinoma of the stomach (HAS) is an extremely rare and unique malignant gastric tumor with a significantly worse prognosis than non‐hepatoid adenocarcinoma of the stomach (non‐HAS). The present study explored the clinicopathological features of HAS and non‐HAS patients to provide insights into HAS treatment strategies. Methods From December 26, 2023, we performed a comprehensive search of the PubMed, Web of Science, Cochrane Library, and Embase.com databases for relevant studies. Two authors independently screened the studies, evaluated their quality, extracted data, and performed the analyses. This study was registered with PROSPERO on January 2, 2024. Results Nine retrospective studies were included for analysis after screening 833 articles. A total of 350 and 924 patients were enrolled in the HAS and non‐HAS groups, respectively. While no significant differences were observed in age, sex, tumor size, T3 or T4 stage, and N2 or N3 stage between the two groups, the HAS group exhibited higher rates of lymph node metastasis (OR = 1.93, 95% CI: 1.19–3.13, p = 0.007), liver metastasis (OR = 3.45, 95% CI: 2.26–5.28, p

Hepatoid adenocarcinoma (HAC) is a rare form of adenocarcinoma that is diagnosed based on immuno-histochemical findings reminiscent of hepatocellular carcinoma (HCC). The clinical characteristics of HAC include increased levels of serum alpha-fetoprotein and a poor prognosis due to early liver metastasis. In particular, diagnosing liver metastasis of HAC can be challenging owing to radiological findings similar to those of HCC. Although HAC can occur in various organs, the stomach is the most common site. We present the case of a 64-year-old femalewho presented with multiple tumors in the liver. During subsequent examination, rectal cancer was identified and diagnosed as HAC through a biopsy. Herein, we report this case along with a literature review.

Abstract Background Hepatoid adenocarcinoma (HAC) is rare in the urinary system, with only 7 reported cases in upper urinary tract. This report aimed to explore the genetic characteristics of ureteral HAC for first time, and to describe the treatment prognosis of ureteral HAC. Case presentation We present a rare case of ureteral HAC in a 53-year-old female, showing elevated serum levels of AFP and CEA, prolonged chronic irritation may be an important cause of her ureteral HAC. Radical nephroureterectomy was performed, the serum levels of AFP and CEA decreased significantly, and metastasis in lymph nodes was found at 9 months after surgery, she had no related symptoms after 18 months postoperatively without adjuvant chemotherapy. Three driver somatic mutations in cancer were identified by NGS testing, including: TP53D281H, KMT2DL1211Ifs*2, KMT2DT1843Nfs*5, demonstrating that ureteral HAC has the similar mutational features to upper tract urothelial carcinoma. Homologous-recombination deficiency (HRD) was positive in this tumor with no mutations in HRD-related genes, which was possibly induced by the copy number deletion of SETD2 gene. Conclusions We report a rare case of ureteral HAC with elevated serum levels of AFP and CEA. NGS testing demonstrated that ureteral HAC has the similar mutational features to upper tract urothelial carcinoma, which is an important guide for the diagnosis and treatment of ureteral HAC.

Abstract Hepatoid adenocarcinoma of the lung is a special type of primary origin in the lung with obvious pathological features and short survival time. However, standard treatment guidelines have not yet been established. Herein, we report a case of hepatoid adenocarcinoma with the primary lesion located in the left upper lung. The tumour size was reduced after four cycles of combined therapy. Subsequent postoperative pathology confirmed complete remission.

Abstract Background To investigate the association between CT signs and clinicopathological features and disease recurrence in patients with hepatoid adenocarcinoma of stomach (HAS). Methods Forty nine HAS patients undergoing radical surgery were retrospectively collected. Association between CT and clinicopathological features and disease recurrence was analyzed. Multivariate logistic model was constructed and evaluated for predicting recurrence by using receiver operating characteristic (ROC) curve. Survival curves between model-defined risk groups was compared using Kaplan–Meier method. Results 24(49.0%) patients developed disease recurrence. Multivariate logistic analysis results showed elevated serum CEA level, peritumoral fatty space invasion and positive pathological vascular tumor thrombus were independent factors for disease recurrence. Odds ratios were 10.87 (95%CI, 1.14–103.66), 6.83 (95%CI, 1.08–43.08) and 42.67 (95%CI, 3.66–496.85), respectively. The constructed model showed an area under ROC of 0.912 (95%CI,0.825–0.999). The model-defined high-risk group showed poorer overall survival and recurrence-free survival than the low-risk group (both P

ObjectiveThis study aimed to investigate the effectiveness of tumor markers and contrast-enhanced computed tomography (CE-CT) in differentiating gastric hepatoid adenocarcinoma (GHA) from gastric adenocarcinoma (GA).MethodsThis retrospective study included 160 patients (44 with GHA vs. 116 with GA) who underwent preoperative CE-CT. Preoperative serum concentrations of tumor biomarkers and CT imaging features were analyzed, including alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen 19-9 (CA19-9), carbohydrate antigen 125 (CA125), tumor location, growth pattern, size, enhancement pattern, cystic changes, and mass contrast enhancement. Multivariate logistic regression analyses were performed to evaluate useful tumor markers and CT imaging features for differentiating GHA from GA.ResultsWhen compared to GA, GHA showed a higher serum AFP [13.27 ng/ml (5.2–340.1) vs. 2.7 ng/ml (2.2–3.98), P

Hepatic metastasis from hepatoid adenocarcinoma of the stomach (HAS) is a rare malignant tumor with hepatocellular differentiation. For the hepatic tumor in middle-aged and elderly people, the image presence of hepatocellular carcinoma (HCC) and production of large amounts of alpha fetoprotein (AFP) and the presence of stomach tumor, that suggest the diagnosis of hepatic metastasis from HAS. Here, the authors report a case of hepatic metastasis from HAS. The characteristics of the disease were analyzed on the basis of clinical symptoms, MR imaging findings, laboratory examinations and pathological diagnosis results. The imaging features and differential diagnosis methods of the disease were summarized combined with literature review, aiming to improve the understanding and diagnostic ability of the disease.

Abstract Hepatoid adenocarcinoma (HAC) is a rare malignancy that may mimic hepatocellular carcinoma (HCC) given its hepatoid histologic appearance and production of alpha fetoprotein. Clinical management and prognosis of HAC varies considerably from HCC. We present two cases of metastatic HAC in a cirrhotic liver diagnosed after biopsy and histopathological evaluation. The cases underscore the importance of recognizing the limitations of diagnostic imaging and pursuing a biopsy where there is diagnostic uncertainty.

BackgroundHepatoid adenocarcinoma of the stomach (HAS) is a rare subtype of gastric cancer with poor prognosis, and its clinicopathological features are not well understood, so the pathology from the clinical biopsy is easily misdiagnosed, especially for special or atypical HAS. We present an extremely rare early HAS with signet ring cell carcinoma and evaluate its clinicopathological features.Case presentationA 51-year-old female patient of Chinese Han ethnicity with upper abdominal pain for 5 years and worsened abdominal pain for 1 month was admitted to our hospital. Esophagogastroduodenoscopy showed a submucosal tumor-like elevated lesion with central depression in the greater curvature of the junction between the antrum and body. Histopathological examination from the biopsy revealed medium–low-differentiation adenocarcinoma with signet ring cell carcinoma. Radical gastrectomy was performed, and the final diagnosis was early HAS with signet ring cell carcinoma.ConclusionsHAS with signet ring cell carcinoma is a special type of HAS and extremely rare. It is first presented for this extremely rare type of HAS, which contributes to strengthen the understanding for the clinicopathological characteristics of HAS and especially promote early detection of HAS.

Hepatoid adenocarcinoma of the duodenum is a rare special type of adenocarcinoma, featured by hepatocyte components in primary adenocarcinoma of the duodenum. It has the characteristics of high malignancy, invasiveness, rapid progress, and poor prognosis. An abnormal elevation of serum alpha-fetoprotein (AFP) may occur in most cases. The diagnosis is mainly based on pathological morphology. Here, we reported a case of hepatic adenocarcinoma of the duodenum. The middle-aged female patient had an ampulla mass at diagnosis and received radical pancreaticoduodenectomy. The postoperative pathology was stage IIIA duodenal adenocarcinoma. At 1 month after surgery, she had multiple intrahepatic metastases and retroperitoneal lymph node metastasis; the AFP level was 300 ng/ml at that time. As she refused target therapy, two cycles of capecitabine-oxaliplatin (XELOX) chemotherapy were performed. However, the AFP elevated from 300 to 1,931.90 ng/ml, and the disease progressed rapidly. Immunohistochemistry (IHC) of tissue samples from presurgical endoscopic ultrasound guided fine needle aspiration (EUS-FNA), surgery, and liver biopsy showed positive AFP staining. Combining the abnormal elevation of serum AFP and microscopic pathological morphology, this case is diagnosed as hepatoid adenocarcinoma of the duodenum with liver metastasis. The physical condition of this patient was too poor to receive follow-up treatment. She died of the rapid disease progression with an overall survival time of 161 days. Considering that in most patients with hepatoid adenocarcinoma the abnormal elevation of serum AFP occurs preoperatively and returns to normal postoperatively rather than normal before surgery and increased after surgery, the primary lesion is located in the stomach rather than the intestine, and the patients are more often older men rather than middle-aged women; this case is rare particularly. Therefore, reporting this case with complete case data may be helpful to further study, so as to improve the understanding of this special type of malignant tumor.

Hepatoid adenocarcinoma is a rare type of extra-hepatic adenocarcinoma which exhibits morphological, functional, and immunohistochemical features of hepatocellular carcinoma, hence correct diagnosis poses a challenge. The most frequent site of this tumor is stomach and rarely occurs in ovaries, lung, gallbladder, pancreas, uterus, and other sites. We present four cases of hepatoid adenocarcinoma of our hospital at rare sites like lungs and gallbladder along with literature review and a simplified approach to diagnosis. In absence of adequate immunohistochemistry or radiological findings often this entity is missed or misdiagnosed as metastatic hepatocellular carcinoma. We have analyzed these hepatoid adenocarcinoma cases in terms of various clinical, serological, histo-morphological, and immunohistochemical parameters, and propose a systematic approach to correctly diagnose this entity.

Alpha-fetoprotein (AFP)-producing gastric carcinoma is a rare subtype of gastric cancer that produces AFP. As an oncogenic glycoprotein that is normally expressed during gestation, AFP is also used as a biomarker for yolk sac tumors, tumors of gonadal origin, hepatocellular carcinoma, and certain gastric carcinomas. Compared with other gastric cancer subtypes, AFP-producing gastric carcinoma is one of the most aggressive gastric tumors due to its high incidence of early liver and lymph node metastasis. Early metastasis and poor prognosis are attributed to the effect of AFP as a growth factor. Tissue biopsy with immunohistochemical staining is helpful in arriving at the diagnosis and differentiating it from metastatic hepatocellular carcinoma. Although currently there is no standard treatment for AFP-producing gastric carcinoma, surgical resection and chemotherapy have been used.

Lung hepatoid adenocarcinoma (HAC) is a rare primary lung carcinoma pathologically characterized by hepatocellular carcinoma-like tumor cells, the majority of which produce alpha-fetoprotein (AFP). The clinical prognosis of lung HAC is generally poor, and effective therapeutic regimens for inoperable or recurrent cases have not been established. Here, we report a case of AFP-producing lung HAC with brain metastasis with long-term disease control, treated with the 5-fluorouracil-derived regimen S-1. The patient was a 66-year-old male admitted to the hospital with alexia. Chest X-ray revealed a massive tumor in the left upper lobe, and a head CT scan revealed a metastasis in the left parietal lobe. The laboratory data showed a remarkably elevated AFP level (97,561 ng/mL). Pathological assessment of the resected brain tumor revealed HAC, which was compatible with the lung biopsies. Together with the absence of other metastatic lesions, a final diagnosis of primary lung HAC, stage IV T4N3M1b, was given. The patient first underwent non-small cell lung cancer chemotherapy regimens (carboplatin and paclitaxel as the first line, and pemetrexed as the second line), but had clinical progression. After third-line oral S-1 (tegafur/gimeracil/oteracil) administration, the serum AFP level significantly dropped and the patient achieved long-term disease control without relapse, surviving more than 19 months after disease presentation. The autopsy result was consistent with the diagnosis of primary lung HAC, and immunohistochemical staining was AFP+, glypican 3+, and spalt-like transcription factor 4+. Here, we report the case of a rare primary lung HAC with apparent disease control on S-1 therapy, together with a literature review.

Hepatoid adenocarcinoma (HAC) is an extremely rare extrahepatic carcinoma, which is pathologically featured by hepatocellular carcinoma (HCC) and marked by producing alpha-fetoprotein (AFP). HAC of mediastinum is extremely rare. For inoperable patients, the curative treatment options have not been established, and the outcome of HAC is usually poor. Here, we present a case of mediastinal HAC with normal serum AFP level who achieved well-controlled and good response after local–regional interventional approach combined with systemic PD-1 inhibitor. A 53-year-old male who complained of chest pain was admitted to our hospital in February 2021. A chest CT scan revealed several tumors in his mediastinum. The laboratory data showed normal serum AFP level. HAC was diagnosed through pathological assessment of biopsy. Surgery was not available due to the infiltration of sternum. Local regional FOLFOX chemotherapy was given by transarterial infusion, followed by transcatheter arterial chemoembolization, and thereafter combined with systemic anti-PD-1 treatment. The patient achieved favorable disease control and apparent symptom relief. So transarterial interventional therapy combined immunotherapy may be a possible and promising treatment for mediastinal HAC.

Introduction: Hepatoid adenocarcinoma (HAC) is a rare neoplasm with a poor prognosis that shows morphological and biochemical similarities to hepatocellular carcinoma. It can occur at any anatomical site, predominantly in the stomach. So far, HAC of soft tissue origin has only been reported once in the literature. Case presentation: We report an unusual case of a 64-year-old male patient diagnosed with a HAC of the soft tissue, with production of alpha fetoprotein. The patient was treated with systemic therapy and responded clinically, radiologically and biochemically to treatment with cisplatin and etoposide. Conclusion: When a patient presents with an extra-hepatic mass, morphologically mimicking hepatocellular carcinoma, the diagnosis of HAC should be considered, especially in case of elevated serum levels of alpha fetoprotein. The disease may be sensitive to systemic treatment.

Alpha-fetoprotein (AFP)-producing gastric carcinomas (AFPGCs) are relatively rare tumors known to have a poor prognosis and commonly found as advanced lesions. Histologically, AFPGCs have been described as having hepatoid and fetal enteric (enteroblastic) morphology and are associated with conventional adenocarcinomas. Prior studies reported a hepatoid component present only in invasive areas and hypothesized that AFPGCs may develop hepatoid features during the process of tumor invasion. We report three cases of AFP-producing early gastric cancer which had an intramucosal hepatoid component. Immunohistochemistry showed that the hepatoid component was diffusely immunoreactive for SALL4, AFP, arginase-1, and HepPar1, and focally for CDX2 and PDX1. An intramucosal transition between the hepatoid component and conventional intramucosal adenocarcinoma was identified. Two patients also had a coexistent fetal enteric component, which was admixed with a hepatoid component. Although at an early stage one patient subsequently developed liver metastasis and a second patient was suspected of having liver metastasis, these were not biopsy-proven. The latter patient had a previous history of hepatocellular carcinoma (HCC) and SALL4 was used on the HCC to distinguish metastatic/further HCC from a gastric metastatic primary with hepatoid differentiation.

BackgroundPrimary hepatoid adenocarcinoma of the gallbladder is a relatively rare type of extrahepatic adenocarcinoma. The genetic changes involved in this type of adenocarcinoma were unexplained so far. We reported a rare case of primary hepatoid adenocarcinoma of gallbladder with Mab-21 domain containing 2 (MB21D2), polypeptide N-acetylgalactosaminyltransferase 12 (GALNT12), and AT-rich interaction domain 2 (ARID2) mutations, which was confirmed after surgical resection pathologically.Case SummaryA 69-year-old female with distention of hypogastrium and constipation received enema treatment, but ineffectively. No abnormalities were found on relevant physical examination. Then, the CT and MRI demonstrated a 3.3–4-cm soft tissue mass shadow in the neck of the gallbladder. The primary lesions consisted of two components: high-grade intraepithelial neoplasia of glands and hepatoid glands microscopically after laparoscope cholecystectomy. Immunohistochemical staining showed the sameness and difference of the two areas. Furthermore, tumor mutational burden (TMB) shows that the MB21D2, GALNT12, and ARID2 genes were mutated.ConclusionThis is the first report of primary hepatoid adenocarcinoma of the gallbladder with MB21D2, GALNT12, and ARID2 mutations. This will provide a theoretical basis for genetic changes in rare tumors.

Jon Arne Søreide1,2 1Department of Gastrointestinal Surgery, Stavanger University Hospital, Stavanger, Norway; 2Department of Clinical Medicine, University of Bergen, Bergen, NorwayCorrespondence: Jon Arne SøreideDepartment of Gastrointestinal Surgery, Stavanger University Hospital, POB 8100, Stavanger N-4022, NorwayTel +47 905 31770Fax +47 51519919Email jonarne.soreide@uib.noAbstract: Hepatoid adenocarcinoma of the stomach (HAS) is a rare subgroup of gastric cancer (GC). Morphologically, this tumor exhibits both adenocarcinomatous and hepatocellular differentiation, and most tumors show immunohistochemical staining for alpha-fetoprotein (AFP) or elevated AFP serum levels. The diagnosis of HAS is frequently delayed, and at least half of patients have advanced disease at the time of diagnosis. Despite a lack of evidence, treatment approaches have mostly followed principles for the treatment of common gastric cancer (CGC), including radical surgery in eligible patients with curative intent. The indications for and the type of adjuvant systemic treatments remain unclear. Additionally, there is a lack of evidence allowing any firm conclusions to be drawn regarding the best treatment for patients with metastatic HAS (mHAS). Chemotherapy regimens, including cisplatin-based chemotherapy, are considered the most efficient first-line systemic treatment in advanced situations. Their combination with targeted therapy (i.e., trastuzumab) in HER2-positive tumors seems promising. The rarity of these patients and the scarce and heterogeneous literature on this particular subgroup of GC make it difficult to provide any robust evidence for the clinical management of patients with HAS.Keywords: hepatoid adenocarcinoma, stomach, gastric, alpha-fetoprotein, therapy, prognosis

PurposeHepatoid adenocarcinoma of the stomach (HAS) is a highly malignant and aggressive tumor. The purpose of this study was to describe the clinical, computed tomography (CT), and prognostic features of HAS to increase the awareness of this entity and determine its distinguishing features from non-HAS tumors.MethodsThe CT features and clinical data of 47 patients in our hospital with pathologically documented HAS were retrospectively analyzed, and the relevant differences between pure HAS (pHAS) and mixed HAS (mHAS) were determined. In addition, 141 patients with non-HAS tumors in the same T stage in the same period were selected as the control group. The data were compared between the two groups, and factors affecting the prognosis of HAS were analyzed. In addition, we included 9 patients with HAS and 27 patients with non-HAS tumors from another center for external validation.ResultsThe patients in the HAS group were predominantly men (n = 33), and the tumor location was mostly the cardia or fundus (n = 27). Between the HAS and non-HAS groups, there were observed differences in terms of: sex, serum alpha-fetoprotein (AFP), carbohydrate antigen (CA)-125, and CA-724 levels; longest tumor diameter; degree of differentiation; vascular invasion; N stage, M stage, and tumor-node-metastasis (TNM) stage; thickest tumor diameter; plain CT attenuation; arterial-phase CT attenuation; CT attenuation between the venous and arterial phases; enhancement modes; and degrees of enhancement (all P < 0.05). In the data from another center for external validation, there were observed differences in terms of: age, degree of differentiation, vascular invasion, thickest tumor diameter, the ratio of arterial CT attenuation to CT attenuation of the abdominal aorta at the same level (RA), CT attenuation difference between the venous phase and arterial phase (HUv-a) (all P < 0.05). The results of the multivariate analysis revealed that the independent factors for differentiation were serum AFP level (P = 0.001), M stage (P = 0.038), and tumor enhancement on CT (P = 0.014). Among patients in the HAS group, 72.34% had pHAS and 27.66% had mHAS. The thickest tumor diameter and the longest short diameter of the metastatic lymph nodes of the mHAS group were on average 6.39 cm and 1.45 cm, respectively, which were larger than those in the pHAS group. The median progression-free survival time was 18.25 months in the HAS group, which was shorter than that in the non-HAS group (72.96 months; P = 0.001). The median overall survival time in the HAS group was 24.80 months, which was shorter than that in the non-HAS group (67.96 months; P = 0.001). The factors affecting the prognosis of HAS were M stage (P = 0.001), overall TNM stage (P = 0.048), presence of vascular cancer emboli (P = 0.040), and pHAS type (P = 0.046). Multifactorial analysis revealed that M stage (P = 0.027) and pHAS type (P = 0.009) were independent risk factors affecting the prognosis of HAS.ConclusionAlthough HAS is a rare clinical entity, it should be considered in the differential diagnosis of gastric tumors. Patients with HAS often have advanced-stage disease at presentation and a worse prognosis than patients with non-HAS tumors. CT findings, combined with laboratory results, can support the diagnosis of HAS. However, the final diagnosis needs to be confirmed with a histopathologic examination. If the postoperative pathologic findings reveal the mHAS type, a rapid clinical intervention and a detailed follow-up with CT are essential.

We present the 18F-Fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) findings in a 57-year-old woman with post-menopausal bleeding diagnosed with hepatoid adenocarcinoma (HAC) with a primary tumour in the uterine corpus and a highly elevated level of serum-α-fetoprotein (S-AFP) at presentation. HAC is a variant of adenocarcinoma with hepatic differentiation representing a heterogeneous group of neoplasms that morphologically and immunphenotypically resemble hepatocellular carcinoma (HCC) but are of extrahepatic origin. Microscopically, they are usually poorly differentiated adenocarcinomas proliferating in solid sheets or in a trabecular or cord-like arrangement. Primary uterine HAC is exceedingly rare with a general poor prognosis, and data is sparse and limited to case reports, making the clinical management challenging. Various primary anatomical sites have been reported in the literature, with the stomach being the most common primary site. 18F-FDG PET/CT plays an important role in staging and follow-up in many gynecological malignancies including uterine corpus cancer. To the best of our knowledge, this is the first report describing a primary uterine hepatoid adenocarcinoma with metastases to bone, vagina and lymph nodes on 18F-FDG PET/CT. By utilizing the ability of PET to detect early metabolic changes prior to visible structural changes on conventional imaging, this case illustrates a potential role of 18FDG-PET/CT in the staging of primary endometrial HAC by depicting distant metastasis that is not readily identifiable on CT alone.

Hepatoid adenocarcinoma of the lung (HAL) is a rare extrahepatic tumor characterized by histologic features of hepatocellular carcinoma. The standard treatment for nonresectable HAL has not been established, though traditionally, these tumors have been treated with platinum-based chemotherapy. Here, we report the use of combination chemotherapy and immunotherapy in a patient presenting with metastatic HAL and an elevated alpha-fetoprotein. The patient had an excellent clinical, radiographic, and biomarker response. This case supports the use of chemoimmunotherapy, which is now the standard of care first-line treatment in NSCLC, for HAL.

Abstract Background Gastric hepatoid adenocarcinoma (GHAC) is an atypical form of gastric cancer (GC) that has similar tissue morphology to hepatocellular carcinoma and frequently produces alpha-fetoprotein. We present an exceedingly rare case of GHAC resulting in a spontaneous gastric perforation. Case presentation A 61-year-old man presented at our institution complaining of abdominal and back pain. A computed tomography scan revealed a spontaneous gastric perforation with a solitary liver tumor and lymph node swelling. Following a diagnosis of advanced-stage GC with a gastric perforation, perforative peritonitis, multiple lymph node metastases, and a solitary metastasis of the lateral segment of the liver, the patient underwent distal gastrectomy. Histopathology of the resected specimen revealed that the tumor cells were arranged in a hepatoid pattern. On immunohistochemical staining, the tumor cells were positive for alpha-fetoprotein and Sal-like protein 4. Thus, the patient was diagnosed with GHAC. Hepatic resection of the solitary liver metastasis was performed. However, recurrence occurred and the patient achieved complete response following tegafur/gimeracil/oteracil-based chemotherapy. Conclusions GHAC is a highly malignant histological subtype of GC. We reported on an extremely rare case of GHAC resulting in a spontaneous gastric perforation and reviewed the literature, including epidemiological data, treatment regimens, and the association between GHAC and alpha-fetoprotein-producing GC.

Hepatoid adenocarcinoma (HAC) is a rare extrahepatic tumor distinguished by having both hepatoid and adenomatous features, which can make the diagnosis challenging. Although it mostly originates in the stomach, several other sites of origin have been reported. We report a case of HAC originating in the duodenum, a very unusual location. We also discuss an approach to the diagnosis of HAC using morphological and immunohistochemical features, and explore possible therapeutic options.

Purpose: To report a case of primary hepatoid adenocarcinoma of the orbit. Observations: An adult patient was referred for evaluation of an orbital mass. Histopathology of the orbital biopsy indicated a carcinoma with hepatoid features. Laboratory studies revealed normal liver function tests, elevated serum alpha-fetoprotein, and whole-body positron emission tomography/computed tomography scan showed no evidence of liver involvement or an alternative primary origin. Conclusions and importance: To the authors' knowledge, this is the first reported case of primary hepatoid adenocarcinoma of the orbit.

Hepatoid adenocarcinoma of the stomach (HAS) is a rare aggressive tumor with hepatocellular differentiation. HAS often produces alpha fetoprotein (AFP) and metastasizes to the lymph nodes and the liver. Molecular studies revealed Her2 amplification and overexpression, association with p53 mutations, but no association with KRAS mutations. EGFR and BRAF mutations have not yet been evaluated in hepatoid carcinoma of the stomach so far. Hereby, we present a case of a 41-year-old female patient with HAS with high AFP level and liver metastases. Molecular analysis revealed Her2 overexpression by immunohistochemistry (IHC), but no EGFR, KRAS, or BRAF mutations were detected. The patient underwent chemotherapy type DCX (docetaxel, cisplatinum, and capecitabine) every 3 weeks with partial response after two cycles, maintained for eight cycles, and then was on maintenance therapy with trastuzumab for 7 months before relapsing and dying 18 months from the day of diagnosis. Conclusively, HAS may be misdiagnosed as hepatocellular carcinoma; therefore, it should be considered in the differential diagnosis of multiple hepatic nodules with high AFP and no history of hepatitis, liver fibrosis or cirrhosis.

Hepatoid adenocarcinoma of the stomach is a rare form of gastric carcinoma with specific clinicopathological features and extremely poor prognosis. Here, we report a case of a 60-year-old male patient who presented in the outpatient department with pain abdomen and constitutional symptoms. Radiological examination revealed a growth involving the antrum of the stomach while the liver and spleen were normal. The serum alpha fetoproteins were 6590 ng/ml. The patient underwent a partial gastrectomy and microscopic examination of the growth showed two types of histological patterns. Majority of the tumor displayed hepatoid differentiation with adenocarcinomatous foci. Periodic acid Schiff positive intracytoplasmic hyaline globules were seen. The tumor was immunohistochemically positive for alpha fetoprotein. The patient underwent radical gastrectomy, received chemotherapy and has been on follow-up for the past 12 months. Though known to be an aggressive neoplasm early diagnosis of hepatoid adenocarcinoma can help improve the prognosis of the disease.

Hepatoid adenocarcinoma (HAC) is a rare type of extrahepatic carcinoma whose morphology is similar to that of hepatocellular carcinoma (HCC). Metachronous HCC and HAC in the same patient is extremely rare. The case of a 68-year-old man with chronic hepatitis B infection who had both HCC and HAC of the stomach is reported herein. Nine years previously this patient had been diagnosed with HCC and received a right lobectomy. HCC that recurred at the caudate lobe at 6 months after the operation was successfully treated with transarterial chemoembolization. The patient was followed up regularly thereafter without evidence of tumor recurrence for 9 years. In July 2010 his serum alpha-fetoprotein (AFP) level elevated from 6.5 ng/mL to 625.4 ng/mL, and he developed a probable single metastatic lymph node around the hepatic artery without intrahepatic lesions. Subsequent evaluation with upper endoscopy revealed a 4-cm ulcerative lesion on the antrum of the stomach. Subtotal gastrectomy was performed with lymph-node dissection. Histologic examination revealed a special type of extrahepatic AFP-producing adenocarcinoma-HAC with lymph-node metastasis-which indicates that HAC can be a cause of elevated AFP even in patients with HCC. HAC should be considered if a patient with stable HCC exhibits unusual elevation of AFP.

Background: Gastric cancer is the fourth most commonly diagnosed cancer and the second most common cause of cancer related death worldwide. It is the common cause of cancer related death in Nepal. Helicobacter Pylori has been classified as a definite carcinogen along with other factors. The aim of this study was to fi nd the incidence of gastric cancer among the patients undergoing upper gastroscopy, its various subtypes and association with Helicobacter Pylori. Materials and Methods: This is a retrospective and prospective study carried out at GRP Polyclinic and Kist Medical College Teaching Hospital. All the patients undergoing upper gastrointestinal endoscopy were included in this study. Data of all the gastric endoscopic biopsies done from June 2011 to January 2013 were collected and analyzed. All the biopsy specimens were processed routinely in histopathology laboratory. Specimens showing carcinoma were enrolled in this study and all the relevant demographic data were collected. Results: Out of 3395 biopsy cases; 49 cases (1.44%) were diagnosed as adenocarcinoma stomach. The overall mean age for carcinoma was 47.6 years with a mild male preponderance. Thirty cases (61.2%) were of intestinal type, (n=11; 22.4%) were of diffuse type and (n=8; 16.3%) were mixed type of adenocarcinoma. According to WHO classifi cation the most common subtype was tubular adenocarcinoma (n=35; 71.5%) followed by signet ring type (11 cases; 22.4%). Out of 49 cases of adenocarcinoma stomach 39 cases (79.5%) were Helicobacter Pylori positive. Conclusion: This study shows that gastric carcinoma is a male predominant neoplasm usually of old age but can occur at younger ages. It predominantly occurs in Helicobacter Pylori infected patients and H. Pylori eradication will help to decrease the incidence rate and mortality of stomach cancer. Journal of Pathology of Nepal (2013) Vol. 3, No.1, Issue 5, 403-407 DOI: http://dx.doi.org/10.3126/jpn.v3i5.7869

We report two cases of uterine carcinosarcoma associated with alpha-fetoprotein (AFP)-producing hepatoid adenocarcinoma. Samples were obtained from two women aged 63 and 82 years. Serum AFP levels of the two samples were 10,131 and 401 ng/ml, respectively. Histologically, in both cases the tumor cells were composed of hepatoid adenocarcinoma component and sarcoma component including rhabdomyosarcoma. Immunohistochemical analyses revealed that AFP was expressed in the cytoplasm of the carcinomatous component. After surgery, the patients received six courses of carboplatin/paclitaxel chemotherapy, and the serum levels of AFP decreased to normal range. The first patient is alive and well at the 2-year follow-up, while the second patient died of disease 1 year after initial operative treatment. This is, to our knowledge, the second report of carcinosarcoma of the uterine corpus with AFP-producing hepatoid adenocarcinoma, as proven by immunohistochemical analyses.

A 50-year-old male patient presented with pain abdomen of 6 months duration. Computed tomography scan revealed a large mass in the stomach occluding the lumen. Histopathology revealed a triple composite tumor comprising of tubular adenocarcinoma arising on a background of high-grade dysplasia, hepatoid adenocarcinoma (positive for Hep Par-1 and alpha fetoprotein) and large cell neuroendocrine carcinoma (positive for synaptophysin and chromogranin) with nodal metastasis.Triple composite tumors are distinctly rare with few reports in literature.

Hepatoid adenocarcinoma (HAC) is a rare type of extrahepatic cancer, whose pathologic features are indistinguishable from those of hepatocellular carcinoma. About thirty cases, nearly half of which occurring in the lung, have been described in patients with a normal liver. No imaging features are typical enough to allow a correct diagnosis.A localization of HAC in the soft tissues of the shoulder with invasion of the scapula in a woman without other symptoms is reported in this paper; soft-tissue HACs have never been described to date. An associate pulmonary HAC was eventually found in this patient.

Abstract Introduction Adenocarcinomas represent less than 2 percent of all urothelial neoplasms. Hepatoid adenocarcinoma is rare in the urinary bladder. Pathological diagnosis is based on a combination of histological features resembling hepatocellular carcinoma and positive immunostaining for α-fetoprotein. Case presentation We report a case of hepatoid adenocarcinoma of the urinary bladder. A 49-year-old Japanese woman underwent a total mastectomy, and post-operatively abdominal computed tomography revealed a tumor of the urinary bladder. Trans-urethral resection of the bladder tumor was performed, and pathological examination revealed a hepatoid adenocarcinoma of the urinary bladder. Our patient has had no evidence of recurrence 20 months after surgery to remove the tumor. Conclusions Hepatoid adenocarcinoma seems to be an aggressive malignant neoplasm that is rare in the urinary bladder. This case report is only the ninth case of hepatoid adenocarcinoma in the urinary bladder to appear in the literature. It is important to be aware of atypical cancer localizations in order to reach a correct diagnosis.