Primary Hepatic Neuroendocrine Carcinoma: A Rare Entity.

Neuroendocrine tumors are typically low-malignancy growths arising from neuroectodermal cells of neural crest origin. Neuroendocrine carcinoma, on the other hand, represents a high-malignancy form of these tumors. While rare in the liver, they often indicate metastasis when present. We present a unique case of incidentally discovered primary hepatic neuroendocrine carcinoma. Initially, the patient's management was based on misleading radiological findings. However, histopathology confirmed the diagnosis, with subsequent imaging ruling out an extrahepatic source. Despite this, the patient opted against surgical intervention, resulting in a fatal outcome. This case underscores the critical importance of prompt diagnosis and intervention to avert adverse outcomes.

Tumor-like conditions that mimic liver tumors.

Non-neoplastic tumor-like conditions of the liver can appear similar to hepatic neoplasms. In many cases, a biopsy is required to confirm the pathology. However, several tumor-like conditions can be correctly diagnosed or suggested prospectively, thus saving patients from unnecessary anxiety and expense. In this image-focused review, we present the ultrasound, computed tomography, magnetic resonance imaging, and positron emission tomography scan features of eight such entities. Clues that indicate the correct pathology are discussed, and the usual clinical setting is described. Many of these lesions are treated differently from true neoplasms, and the current treatment plan is discussed in many of the cases presented. After reviewing this article, the reader will have a better understanding of these lesions and the situations in which they should be included in the differential diagnosis.

PIVKA-II combined with alpha-fetoprotein for the diagnostic value of hepatic tumors in children: a multicenter, prospective observational study.

BACKGROUND: To investigate whether protein induced by vitamin K antagonist-II (PIVKA-II) combined with alpha-fetoprotein (AFP) can improve the diagnostic and differential diagnostic accuracy of childhood hepatic tumors. METHODS: A multi-center prospective observational study was performed at nine regional institutions around China. Children with hepatic mass (Group T) were divided into hepatoblastoma group (Group THB) and hemangioendothelioma group (Group THE), children with extrahepatic abdominal mass (Group C). Peripheral blood was collected from each patient prior to surgery or chemotherapy. The area under the curve (AUROC) was used to evaluate the diagnostic efficiency of PIVKA-II and the combined tumor markers with AFP. RESULTS: The mean levels of PIVKA-II and AFP were both significantly higher in Group T than Group C (p = 0.001, p < 0.001), in Group THB than Group THE (p = 0.018, p = 0.013) and in advanced HB than non-advanced HB (p = 0.001, p = 0.021). For the diagnosis of childhood hepatic tumors, AUROC of PIVKA-II (cut-off value 32.6 mAU/mL) and AFP (cut-off value 120 ng/mL) was 0.867 and 0.857. The differential diagnostic value of PIVKA-II and AFP in hepatoblastoma from hemangioendothelioma was further assessed, AUROC of PIVKA-II (cut-off value 47.1mAU/mL) and AFP (cut-off value 560 ng/mL) was 0.876 and 0.743. The combined markers showed higher AUROC (0.891, 0.895 respectively) than PIVKA-II or AFP alone. CONCLUSIONS: The serum level of PIVKA-II was significantly higher in children with hepatic tumors, especially those with malignant tumors. The combination of PIVKA-II with AFP further increased the diagnostic performance. TRIAL REGISTRATION: Clinical Trials, NCT03645655. Registered 20 August 2018, https://www. CLINICALTRIALS: gov/ct2/show/NCT03645655 .

A case of intrahepatic mass-forming portal biliopathy mimicking intrahepatic cholangiocarcinoma.

A 75-year-old man was referred to our department because of an enlarging intrahepatic mass detected on magnetic resonance imaging (MRI) follow-up for another disease. MRI showed hypointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging in liver segment 4. Abdominal plain computed tomography (CT) indicated a low-density lesion with an unclear boundary, measuring approximately 4 cm × 3 cm in liver segment 4. Dynamic CT showed early rim enhancement and gradual central enhancement. Contrast-enhanced CT also showed occlusion of the portal vein in segment 4. As the possibility of intrahepatic cholangiocarcinoma could not be excluded on imaging studies, we performed laparoscopic left medial sectionectomy. Histologically, the lesion showed diminished numbers of hepatocytes with increased collagen fibers compared with normal, with no patent portal vein. We considered this lesion a reactive lesion caused by collapse of the liver parenchyma owing to localized obstruction and loss of the portal vein. This lesion was pathologically diagnosed as portal biliopathy. We experienced an extremely rare case of intrahepatic mass-forming portal biliopathy that mimicked a hepatic tumor, which was diagnosed by laparoscopic resection. Portal biliopathy rarely forms intrahepatic mass lesions and must be distinguished from a malignant hepatic tumor.

Effectiveness of Butorphanol in alleviating intra- and post-operative visceral pain following microwave ablation for hepatic tumor: a dual-central, randomized, controlled trial.

Many patients who underwent hepatic percutaneous microwave ablation (MWA) reported experiencing pain during the procedure. This study utilized a well-designed multicentral, randomized, and placebo-controlled format to investigate the effects of Butorphanol. Patients who underwent MWA were randomly assigned to either Butorphanol or normal saline group. The primary outcomes of the study were assessed by measuring the patients' intraoperative pain levels using a 10-point visual analog scale (VAS). Secondary outcomes included measuring postoperative pain levels at the 6-h mark (VAS) and evaluating comprehensive pain assessment outcomes. A total of 300 patients were divided between the control group (n = 100) and the experimental group (n = 200). Butorphanol showed statistically significant reductions in intraoperative pain levels compared to the placebo during surgery (5.00 ± 1.46 vs. 3.54 ± 1.67, P < 0.001). Significant differences were observed in postoperative pain levels at the 6-h mark and in the overall assessment of pain (1.39 + 1.21 vs. 0.65 + 0.81, P < 0.001). Butorphanol had a significant impact on reducing the heart rate of patients. The empirical evidence supports the effectiveness of Butorphanol in reducing the occurrence of visceral postoperative pain in patients undergoing microwave ablation for hepatic tumor. Furthermore, the study found no noticeable impact on circulatory and respiratory dynamics.

Mid- to long-term outcome of laparoscopic ultrasound-guided radiofrequency ablation for malignant hepatic tumors.

BACKGROUND: This study aimed to evaluate the long-term outcomes of fully laparoscopic ultrasound-guided radiofrequency ablation (LURFA) in malignant hepatic tumors that are difficult to curatively treat with the percutaneous approach or laparoscopic liver resection (LLR). METHODS: Between 2011 and 2021, 62 patients with malignant hepatic tumors (37 hepatocellular carcinomas [HCCs] and 25 metastatic colorectal cancers [mCRCs]), who were not feasible to be curatively treated by percutaneous radiofrequency ablation or LLR, were enrolled and treated only by LURFA. Patients who underwent concurrent surgical resection were excluded. The cumulative incidence rates of local recurrence (LR) and survival were analyzed. RESULTS: All 93 tumors with a median diameter of 22.0 mm (IQR, 8.0-50.0) and a median number of 1.5 tumors (IQR, 1.0-6.0) in 62 patients were successfully treated. According to the IWATE criteria for LLR, 33 of 62 patients (53.2%) had tumors in difficult locations (segments I, VII, VIII, and IVa). Over a median follow-up period of 92.4 months (IQR, 60.0-128.0), the 1-, 2-, 3-, 5-, 8-, and 10-year cumulative incidence rates of LR were 6.9%, 13.8%, 17.2%, 17.2%, 20.9%, and 20.9%, respectively. In patients with HCC, 1-, 3-, 5-, and 8-year survival rates were 97.2%, 80.6%, 55.6%, and 40.1%, respectively. In patients with mCRC, 1-, 3-, 5-, and 8-year survival rates were 100.0%, 36.4%, 27.3%, and 16.4%, respectively. Adverse events of grade 3 occurred in only 3 of 62 patients (4.8%). CONCLUSION: Full LURFA is a safe and effective treatment for malignant hepatic tumors, even in difficult percutaneous ablation or LLR areas.

Rational design of NIR-II molecule-engineered nanoplatform for preoperative downstaging and imaging-guided surgery of orthotopic hepatic tumor.

Orthotopic advanced hepatic tumor resection without precise location and preoperative downstaging may cause clinical postoperative recurrence and metastasis. Early accurate monitoring and tumor size reduction based on the multifunctional diagnostic-therapeutic integration platform could improve real-time imaging-guided resection efficacy. Here, a Near-Infrared II/Photoacoustic Imaging/Magnetic Resonance Imaging (NIR-II/PAI/MRI) organic nanoplatform IRFEP-FA-DOTA-Gd (IFDG) is developed for integrated diagnosis and treatment of orthotopic hepatic tumor. The IFDG is designed rationally based on the core "S-D-A-D-S" NIR-II probe IRFEP modified with folic acid (FA) for active tumor targeting and Gd-DOTA agent for MR imaging. The IFDG exhibits several advantages, including efficient tumor tissue accumulation, good tumor margin imaging effect, and excellent photothermal conversion effect. Therefore, the IFDG could realize accurate long-term monitoring and photothermal therapy non-invasively of the hepatic tumor to reduce its size. Next, the complete resection of the hepatic tumor in situ lesions could be realized by the intraoperative real-time NIR-II imaging guidance. Notably, the preoperative downstaging strategy is confirmed to lower the postoperative recurrence rate of the liver cancer patients under middle and advanced stage effectively with fewer side effects. Overall, the designed nanoplatform demonstrates great potential as a diagnostic-therapeutic integration platform for precise imaging-guided surgical navigation of orthotopic hepatic tumors with a low recurrence rate after surgery, providing a paradigm for diagnosing and treating the advanced tumors in the future clinical translation application.

Fibrolamellar hepatocellular carcinoma: a case report and gene analysis.

BACKGROUND: Fibrolamellar hepatocellular carcinoma (HCC) (FL-HCC) is rare in Japan. FL-HCC develops in young patients with no history of cirrhosis and tends to manifest lymphatic metastasis with clinical features similar to those of HCC. We present a case of FL-HCC in a young male patient. CASE PRESENTATION: A 14-year-old male patient underwent abdominal computed tomography (CT) to diagnose appendicitis, wherein a hepatic tumor was detected. Dynamic enhanced CT revealed a 35-mm solid tumor, which contrasted at the early phase of dynamic enhanced study of the right hepatic segments, with occlusion of the right portal vein. We performed right hepatectomy for these lesions. The patient experienced a single lymphatic recurrence on the hepatoduodenal ligament 12 months after the initial surgery. We performed lymphadenectomy for the recurrent tumor. We performed RNA sequencing (RNA-seq) and targeted DNA sequencing of the resected specimens (primary tumor, lymphatic metastasis, and normal liver). RNA-seq detected DNAJB1-PRKACA in both primary and metastatic lesions as previously reported. Furthermore, The Cancer Genome Atlas (TCGA) database was used to compare other gene expressions in this case with those of previously reported cases of FL-HCC and HCC in young patients. Principal component analysis of differentially expressed genes in the top 10% revealed that the gene expression in our case was similar to that of previous FL-HCC cases but was a different cluster from that in HCC cases in young patients. Mutational analysis did not detect any somatic mutations associated with carcinogenesis, including previously reported mutations (Kastenhuber et al. in Proc Natl Acad Sci USA 114: 13076-84, 2017). CONCLUSION: We encountered a case of FL-HCC, a rare hepatic tumor in an adolescent patient, and evaluated the genetic background. Our findings could contribute to the elucidation of the mechanisms underlying carcinogenesis and progression in patients with FL-HCC and thereby contribute to the development of new therapeutic strategies in the future that may improve patient prognosis.

Pedunculated Focal Nodular Hyperplasia: When in Doubt, Should We Cut It Out?

Focal nodular hyperplasia (FNH) is the second most common benign hepatic tumor and can rarely present as an exophytic solitary mass attached to the liver by a stalk. Most FNH cases are usually detected as incidental findings during surgery, imaging or physical examination and have a high female predominance. However, the pedunculated forms of FNH are particularly rare and commonly associated with severe complications and diagnostic challenges. Hence, our study aims to provide a comprehensive summary of the available data on the pedunculated FNH cases among adults and children. Furthermore, we will highlight the role of different therapeutic options in treating this clinical entity. The use of imaging techniques is considered a significant addition to the diagnostic toolbox. Regarding the optimal treatment strategy, the main indications for surgery were the presence of symptoms, diagnostic uncertainty and increased risk of complications, based on the current literature. Herein, we also propose a management algorithm for patients with suspected FNH lesions. Therefore, a high index of suspicion and awareness of this pathology and its life-threatening complications, as an uncommon etiology of acute abdomen, is of utmost importance in order to achieve better clinical outcomes.

A Rare Case of Symptomatic Ciliated Hepatic Foregut Cyst in a 17-Year-Old Female.

A ciliated hepatic foregut cyst (CHFC) is a very uncommon cystic hepatic lesion that arises from an embryonic remnant of the foregut epithelium. CHFC is predominantly asymptomatic and is found incidentally. However, it can show various clinical presentations such as pain and weight loss. We present the case of a 17-year-old female who came to our hospital with complaints of right subcostal pain and abdominal discomfort and was diagnosed with CHFC by biopsy. Successful laparoscopic resection was performed due to the possibility of malignant transformation. Even though the majority of the patients are asymptomatic and cysts are commonly found incidentally, when the lesion has concerning features, they may need follow-up or resection due to rare reported cases of malignant transformation.

Case Report: Early detection and intervention of congenital portosystemic shunts in children.

Congenital portosystemic shunts (CPSS) are rare vascular anomalies that cause abnormal communications between the portal and systemic venous systems and may be incidentally detected on imaging or via abnormal laboratory parameters due to the lack of specificity in the condition's clinical presentation. Ultrasound (US) is a common tool for examining abdominal solid organs and vessels and is the initial imaging modality for diagnosing CPSS. Here we report the case of an 8-year-old Chinese boy with CPSS diagnosed using color Doppler US. Doppler US first found intrahepatic tumor, then revealed that the left portal vein was directly communicating with the inferior vena cava, and the boy was finally diagnosed with intrahepatic portosystemic shunts. Interventional therapy was employed to occlude the shunt. During the follow-up, the intrahepatic tumor disappeared and no complications. Hence, to be able to differentiate such vascular anomalies, clinicians should be fairly acquainted with the normal ultrasonographic anatomical features in daily clinical work. Furthermore, increased disease awareness and advances in imaging equipment and technology are essential for CPSS diagnosis.

Unusual presentation of a case of fetal hepatic mass: a case report.

BACKGROUND: Giant hepatic hemangiomas are rare and can cause serious complications that contribute to a high risk of perinatal mortality. The purpose of this article is to review the prenatal imaging features, treatment, pathology, and prognosis of an atypical fetal giant hepatic hemangioma and to discuss the differential diagnosis of fetal hepatic masses. CASE PRESENTATION: A gravida 9, para 0 woman at 32 gestational weeks came to our institution for prenatal ultrasound diagnosis. A complex, heterogeneous hepatic mass measuring 5.2 × 4.1 × 3.7 cm was discovered in the fetus using conventional two-dimensional ultrasound. The mass was solid and had both a high peak systolic velocity (PSV) of the feeding artery and intratumoral venous flow. Fetal magnetic resonance imaging (MRI) revealed a clear, hypointense T1-W and hyperintense T2-W solid hepatic mass. Prenatal diagnosis was very difficult due to the overlap of benign and malignant imaging features on prenatal ultrasound and MRI. Even postnatally, neither contrast-enhanced MRI nor contrast-enhanced computed tomography (CT) was useful in accurately diagnosing this hepatic mass. Due to persistently elevated Alpha-fetoprotein (AFP), a laparotomy was performed. Histopathological examination of the mass showed atypical features such as hepatic sinus dilation, hyperemia, and hepatic chordal hyperplasia. The patient was ultimately diagnosed with a giant hemangioma, and the prognosis was satisfactory. CONCLUSIONS: When a hepatic vascular mass is found in a third trimester fetus a hemangioma should be considered as a possible diagnosis. However, prenatal diagnosis of fetal hepatic hemangiomas can be challenging due to atypical histopathological findings. Imaging and histopathological assays can provide useful information for the diagnosis and treatment of fetal hepatic masses.

Primary hepatic adenosquamous carcinoma: a case report and review of the literature.

Primary hepatic adenosquamous carcinoma is considered a rare subtype of intrahepatic cholangiocarcinoma, with fewer than 100 domestic and international cases reported. This malignancy exhibits a high degree of malignancy, strong invasiveness, and an unfavorable prognosis due to its propensity for early lymph node and intrahepatic metastasis. The etiology of this disease remains uncertain, and preoperative diagnosis is exceedingly challenging owing to the nonspecific clinical features and lack of specificity in imaging studies. Radical surgical resection is the most effective treatment for non-metastatic tumors, while targeted adjuvant therapy administered postoperatively can enhance therapeutic efficacy and delay tumor recurrence. This article documents the diagnostic and therapeutic course of a case of primary hepatic adenosquamous carcinoma treated at our medical institution, along with a comprehensive synthesis of the clinical characteristics and advances in the diagnosis and treatment of this disease, aiming to augment understanding and serve as a reference for future clinical endeavors.

Case report: Diagnosis of NUT carcinoma of hepatic origin by next-generation sequencing.

NUT carcinoma is a rare subcategory of squamous cell carcinoma. The latter is primarily characterized by the fusion of the coding sequence NUTM1 on chromosome 15q14 with BRD4 or BRD3, both of which are acetyl-histone binding bromodomains. This tumor is often misdiagnosed due to its rarity and its histological similarity with other squamous cell carcinomas. It typically presents as a poorly differentiated squamous cell carcinoma in the head, neck, and mediastinal region, and has no distinct clinical characteristics that set it apart from other malignancies. Although uncommon, other NUT carcinomas have been reported in the literature outside of the midline region. Through next-generation sequencing, we were able to correctly diagnose our patient with the first-documented case of NUT carcinoma of hepatic-only origin.

Hepatic pseudotumor associated with Strongyloides infection: A case report.

BACKGROUND: Strongyloides sterocoralis is a parasitic infection caused by a roundworm that is transmitted through soil contaminated with larvae. It can infrequently cause hepatic abscesses in immunocompromised patients and is rarely reported to form hepatic lesions in immunocompetent hosts. CASE SUMMARY: We present a case study of a 45-year-old female who presented with right upper quadrant abdominal pain and constitutional symptoms for several weeks. Cross-sectional imaging identified several malignant-appearing liver masses. Further investigation, including serological testing and histopathologic examination, revealed the presence of serum Strongyloides antibodies and hepatic granulomas with extensive necrosis. Following treatment with ivermectin for 2 wk, there was complete resolution of the liver lesions and associated symptoms. CONCLUSION: This case highlights the importance of considering parasitic infections, such as Strongyloides, in the differential diagnosis of hepatic masses. Early recognition and appropriate treatment can lead to a favorable outcome and prevent unnecessary invasive procedures. Increased awareness among clinicians is crucial to ensure the timely diagnosis and management of such cases.

Resección laparoscópica de tumor hepático / Laparoscopic Resection of Hepatic Tumor

Introducción: La cirugía laparoscópica desde su introducción en 1987 ha experimentado un enorme desarrollo. La cirugía hepática laparoscópica de los tumores hepáticos plantea diversas controversias: los cirujanos deben estar formados en cirugía hepática y cirugía laparoscópica, la técnica laparoscópica más adecuada no está bien definida (totalmente laparoscópica o asistida con la mano), el abordaje o disección de grandes vasos entraña riesgos importantes, los métodos de sección parenquimatosa están menos desarrollados que en la cirugía hepática abierta entre otros retos. A pesar de estos criterios existen situaciones en que la cirugía puede llevarse a cabo con seguridad, sobre todo en tumores benignos y malignos cuando las lesiones se localizan principalmente en segmentos anteriores del hígado. Objetivo: Presentar una paciente portadora de una lesión hepática intervenida quirúrgicamente por videolaparoscopía con buenos resultados y evolución excelente. Caso clínico: Paciente femenina de 42 años de edad; antecedentes de salud con presencia de dolor abdominal de tres meses de evolución en región de hipocondrio derecho y trastornos dispépticos esporádicos. Se confirmó imaginológicamente un tumor de 5 cm de diámetro en segmento III, de aspecto hemangiomatoso con componente mixto y sospecha de malignidad. Se realizó resección laparoscópica, el diagnóstico anatomopatológico fue nódulo regenerativo, tipo nódulo cirrótico con hemangioma cavernoso de localización periférica en hígado sano, sin evidencias de malignidad en los estudios realizados. Conclusiones: Las resecciones quirúrgicas de lesiones hepáticas abordables por vía laparoscópica son una opción terapéutica segura y muy válida en pacientes con indicaciones específicas(AU)

Introduction: Laparoscopic surgery, since its introduction in 1987, has undergone enormous development. Laparoscopic hepatic surgery of liver tumors raises several controversies: surgeons must be trained in hepatic surgery and laparoscopic surgery, the most appropriate laparoscopic technique is not well defined (totally laparoscopic or hand-assisted), the approach to or dissection of large vessels involves significant risks, the methods for parenchymal section are less developed than in open hepatic surgery, among other challenges. Despite these criterions, there are situations in which the surgery can be performed safely, above all in benign and malignant tumors when the lesions are located mainly in anterior segments of the liver. Objective: To present the case of a patient with a hepatic lesion and operated on by videolaparoscopy with good outcomes and excellent evolution. Clinical case: A 42-year-old female patient with a family history of disease presented abdominal pain of three months of evolution in the right hypochondrium and sporadic dyspeptic disorders. Imaging confirmed a tumor of 5 cm of diameter in segment III, with hemangiomatous aspect, mixed component and suspicion of malignancy. Laparoscopic resection was performed; the anatomopathological diagnosis was regenerative nodule, similar to a cirrhotic nodule with cavernous hemangioma of peripheral location in healthy liver, with no evidence of malignancy in the performed studies. Conclusions: Surgical resections of hepatic lesions that can be laparoscopically approached are a safe and very valid therapeutic option in patients with specific indications(AU)

Nodular hepatic lesion mimicking gallbladder carcinoma in myeloma.

An 81-year-old man was diagnosed with nodular hepatic lesion of extramedullary plasmacytoma with the absence of FDG uptake. Doppler ultrasonography showed pulsations and abundant blood flow signals within the tumor. The blood flow was temporally reduced after daratumumab-based induction therapy; however, the tumor rapidly re-expanded with blood reflow.

Cystadenocarcinoma of the intrahepatic bile duct in a dog.

A 14-year-old spayed female Shih-Tzu was referred to the Veterinary Medical Teaching Hospital of Konkuk University for evaluation of an abdominal mass. In diagnostic imaging, two large cystic masses were identified. The affected liver lobes were surgically resected, and the specimens were submitted for histopathological evaluation and immunohistochemical staining. The two cystic lesions were diagnosed as biliary cystadenocarcinoma (BCAC). Recurrence and regional invasion were identified on ultrasonography 36 days postoperatively. The patient died on postoperative day 271. To the best of our knowledge, previously reported case studies of BCAC in dogs presented limited clinical information. In this report, we present a detailed picture comprising a range of clinical information and histopathological examination of BCAC in a dog.

Prenatal diagnosis and management of a giant intrahepatic arteriovenous malformation-Sonographic findings, clinical implications, and treatment.

Prenatal detection of complex giant hepatic arteriovenous malformation requires an examination of the affected fetal hemodynamic situation with emphasis on the affected arterial supply pattern. Early pediatric surgeon presentation is needed, as timely surgical intervention appears to be essential.

Hepatocellular Carcinoma in a Free-Ranging Three-Toed Sloth (Bradypus variegatus).

The increasing interest of tumors in wildlife is important for biodiversity conservation and for monitoring environmental agents and/or contaminants with potential impact on human health. Here we described the occurrence of hepatocellular carcinoma (HCC) in noncirrhotic liver of a free-ranging three-toed sloth (Bradypus variegatus) from the Atlantic Forest biome in Brazil. The HCC showed a moderate mononuclear inflammatory infiltrate within the tumor tissue but with no inflammation and fibrosis in the adjacent liver tissue. Upon immunohistochemistry, neoplastic cells were diffusely positive for HepPar-1 and glutamine-synthetase presenting an irregular and random immunostaining pattern; ß-catenin was positive in the cytoplasmic membrane of malignant hepatocytes; and cytokeratin 19 immunostaining was restricted to bile duct epithelial cells. The liver tissue was negative for HBV-like and HCV-like viruses assessed by molecular tests. The potential similarity of pathogenesis may reinforce the need for research on environmental and/or infectious agents associated with HCC that may contribute to the understanding of cancer in wildlife.