Multi-center prospective survey of hepatocellular carcinoma in Kerala: More than 1,200 cases.

BACKGROUND: Hepatocellular carcinoma (HCC) is considered uncommon in India. The aim of this study was to document the demographic characteristics and clinical aspects of HCC in Kerala, India. METHODS: A survey of HCC in Kerala was performed. All gastroenterologists in the region were invited. From May 2018 to April 2020, data was collected in a standardized questionnaire. RESULTS: Forty-three doctors from 15 centers contributed the data. Total 1217 patients were analyzed. This is the largest state-wide survey of HCC in India. HCC was more common in men (90%) than in women (p < 0.01). The etiology of liver disease was hepatitis B virus (7%), hepatitis C virus (4%) and alcohol (40%). Diabetes mellitus was present in 64%, hypercholesterolemia in 17% and hypertension in 38%. Obesity was present in 33% and 15% were overweight. Non-alcoholic fatty liver disease (NAFLD) with or without metabolic syndrome was present in 44%. Serum alpha-fetoprotein was > 400 ng/mL in 24%, total tumor diameter was > 5 cm in 59%, portal vein invasion was seen in 35% and distant metastasis was seen in 15%. Specific therapy was given to 52%. Treatments given included liver transplantation (n = 24), liver resection (n = 39) and transarterial chemoembolization (TACE, n = 184). Although the study was not designed to compare survival, patients who had liver transplantation had longer survival (median 69 months) compared to matched patients given only TACE (median 18 months) (p = 0.03). CONCLUSION: HCC is common in Kerala, India. NAFLD has a predominant association with HCC in Kerala. Most of the patients report late when curative treatment is not possible.

Spectrum of drug-induced liver injury in a tertiary hospital in southern India.

Background Anti-tuberculosis drugs are thought to account for about 50% of drugs that cause liver injury in India. We show that the spectrum of drugs is much wider than previously reported. Methods We evaluated all patients with unexplained acute liver injury presenting during 2006-2016 using a structured proforma for drug-induced liver injury (DILI). The Roussel Uclaf Causality Assessment Method was used to assess causality. Results DILI was found in 143 of 2534 patients with acute liver injury. Nineteen patients had probable ayurvedic DILI. The other common causes of DILI were statins (16 patients) and anti-tuberculosis drugs (11 patients). Eight patients had DILI post-liver transplant. Fluconazole was the most common cause of post-liver transplant DILI. Chronic DILI (abnormal liver function test after 12 months of stopping the suspected drug) was found in 2 patients. Conclusion In otherwise unexplained acute liver injury, DILI due to ayurvedic drugs should be sought. DILI should be considered in post-liver transplant patients. Patients with DILI should be monitored for at least 12 months to exclude progression to chronic DILI.

Radiological Features of Zollinger-Ellison Syndrome: A Report of Two Cases.

Hypersecretion of gastrin from duodenal or pancreatic gastrinomas results in a rare clinical entity called Zollinger-Ellison syndrome (ZES). It mostly presents with abdominal pain and diarrhea. ZES may be sporadic or occur in association with multiple endocrine neoplasia type 1. Usually, ZES manifests between 20 and 50 years. We report two cases of patients who presented with abdominal pain, vomiting, watery diarrhea, and significant weight loss.

A rare case of collision tumour of the ovary complicated by torsion.

Collision tumour is the coexistence of two adjacent, but histologically distinct tumours without histologic admixture. Collision tumours are rare in the ovary. It is mostly a histopathological diagnosis often missed in preoperative imaging. The radiologist, gynaecologists and pathologists should be aware of such a combination of tumours to avoid misdiagnosis. We describe the finding of a rare collision tumour, mature cystic teratoma and ovarian fibroma complicated by torsion.

18F-Fluorodeoxyglucose Positron-Emission Tomography/Computed Tomography Detected Inguinal Lymph Nodal Metastasis from Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) usually metastasizes to the regional abdominal lymph node, lungs, and bones. Nonregional lymph node involvement by HCC in the absence of regional lymph nodes is rare. We describe the 18F-fluorodeoxyglucose positron-emission tomography-computed tomography findings of an HCC patient with histopathologically proven inguinal lymph nodal metastasis in the absence of regional lymph nodal metastases.

Repurposing Pirfenidone for Nonalcoholic Steatohepatitis-related Cirrhosis: A Case Series.

We repurposed the antifibrotic drug pirfenidone-which is approved for treatment of idiopathic lung fibrosis-in a series of patients with nonalcoholic steatohepatitis-related cirrhosis. Our report demonstrates the observed improvements in necroinflammation and regression of cirrhosis with pirfenidone use for 12-weeks, associated with classical hepatic repair complex features on follow-up liver biopsies. This novel work could help stimulate further randomized trials of pirfenidone in patients with nonalcoholic steatohepatitis-related liver fibrosis or cirrhosis, for whom no recommended drug treatments exists currently.

Acute exacerbated severe form of nonalcoholic steatohepatitis leading to acute-on-chronic liver failure: A case series.

We present three patients with uncontrolled metabolic syndrome and acute-on-chronic liver failure in whom, after meticulous evaluation, the acute event was found to be a severe and exacerbated form of nonalcoholic steatohepatitis (NASH), a novel entry in the natural history of non-alcoholic fatty liver disease. This novel disease entity was described partially, 17 years before by Caldwell et al. We discuss current literature, pertinent features, and outcomes associated with a severe form of NASH, which is associated with high mortality.

Hemochromatosis in India: First Report of Whole Exome Sequencing With Review of the Literature.

BACKGROUND: Primary hemochromatosis is unusual in India. The homeostatic iron regulator (HFE) gene C282Y mutation, a common cause for hemochromatosis in Europe, is considered almost nonexistent in India. We are reporting a case of hemochromatosis with the HFE gene C282Y mutation and two other adult cases with a novel hemojuvelin (HJV) mutation from Kerala. METHODS: Of 434 cases with chronic liver disease, 3 cases were identified with the serum ferritin level of more than 1000 ng/mL and primary hemochromatosis after excluding secondary causes. Whole exome sequencing, including genes HFE, HJV, SLC40A1, TFR2, FTH1, HAMP, SKIV2L, TTC37, and BMP2, was performed for blood samples in all 3 cases. RESULTS: One patient with hemochromatosis had a homozygous HFE gene C282Y mutation, and two other adult cases had a novel homozygous HJV D355Y mutation. This is the first report of hemochromatosis associated with the HFE C282Y mutation from Kerala and the second report in India. This is the second report of hemochromatosis associated with an HJV mutation from India. CONCLUSION: HJV mutations may explain some of the adult onset primary hemochromatosis in India.

18F-Fluorodeoxyglucose Positron Emission Tomography-Computed Tomography in Malignant Transformation of Paget's Disease of Bone.

Malignant transformation Paget's disease of bone to sarcoma is relatively rare, occurring in approximately 1% of these patients. Although few clinical and imaging findings may help in prediction of malignant transformation, most of them are nonspecific. We describe a case of carcinoma vocal cord and Paget's disease of bone with sarcomatous transformation, where 18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography was helpful in accurate diagnosis by demonstrating differential intense FDG uptake in transformed areas of pagetic bone and also FDG-avid osteoblastic pulmonary metastasis.

Severe alcoholic hepatitis: current perspectives.

Severe acute alcoholic hepatitis (AH) is a catastrophic disease in the natural history of alcoholic liver disease with a very high 180-day mortality. It can present as acute on chronic liver failure with worse prognosis in the presence of infections and higher grades of liver disease severity. The clinical scenario involves a patient with a recent history of heavy alcohol consumption within three months of presentation with jaundice and characteristic liver enzyme elevation pattern with coagulopathy, hepatic encephalopathy, variceal bleeding and sepsis that results in extrahepatic organ failures. Several liver disease severities and therapy response indicators are in clinical use. Even though not approved, the only recommended treatment option for patients with severe AH is corticosteroids, which is without long term survival benefit. Novel efficacious treatment options awaiting high-quality multi-center studies include liver transplantation (involves strict selection criteria), growth factor therapy and fecal microbiota transplantation. In this exhaustive review, we discuss the definitions, disease severity, histopathology, and treatment options - past, present, and future, in patients with severe alcoholic hepatitis.

18F-FDG PET/CT in Lymphomatoid Papulosis Mimicking Primary Cutaneous Anaplastic Large Cell Lymphoma.

Lymphomatoid papulosis is a benign self-healing condition, presenting as papulonodular skin eruptions and mimicking malignant cutaneous lymphomas histopathologically. F-FDG PET/CT findings in this benign condition have not been described in detail in the literature. We present a case of lymphomatoid papulosis mimicking primary cutaneous anaplastic large cell lymphoma histopathologically and demonstrating intensely FDG-avid cutaneous lesions on F-FDG PET/CT, which disappear spontaneously in the follow-up scan.

Adamantinoma-Like Ewing's Family Tumor of the Sino Nasal Region: A Case Report and a Brief Review of Literature.

Ewing's sarcoma family of tumors (EFTs) are malignant mesenchymal tumors with a predilection for bone and soft tissue. They are characterized by their monomorphic small blue round cell morphology. However rare morphologic variants of EFTs can also show overt epithelial differentiation in the form of squamoid differentiation along with strong cytokeratin expression. This particular subset of EFTs are known as adamantinoma-like EFTs which can be difficult to differentiate from epithelial head and neck malignancies. Here we report a case of sinonasal adamantinoma-like EFT in an 18-year-old male patient. The lesion differed from a typical EFT by means of overt squamoid differentiation which showed a basaloid appearance with peripheral palisading. The immunohistochemistry was positive for pan-cytokeratin, p40, p63, ERG, FLI1, and CK5/6. It was negative for actin, desmin, and WT-1. Initial diagnosis of a basaloid squamous cell carcinoma was made. Further molecular studies were also done due to the complex presentation of the tumor. EWSR testing with break-apart analysis confirmed EWSR1 and FLI1 rearrangements. Further confirmation was done with RT-PCR. The case was found to be positive for EWS-FLI-1 translocation. The revised immunohistochemistry panel showed CD99, ERG, FLI1, and synaptophysin positivity. The lesion was reclassified as an adamantinoma-like ES. Our case reinforces the fact that a subset of EFTs can show histomorphologic and immunohistochemical features of aberrant epithelial differentiation. These cases are difficult to differentiate from usual epithelial malignancies which occur in this region. This diagnostic pitfall can be avoided by the inclusion of CD99 and/or FLI1 in the immunohistochemical assessment of any round cell malignancy at any anatomic location. A strong and diffuse CD99 positivity should prompt molecular testing for the presence of EWSR1 gene rearrangements.

Peritoneal Lymphomatosis Mimicking Peritoneal Carcinomatosis from Ovarian Malignancy on F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

Peritoneal lymphomatosis is relatively uncommon cause of diffuse malignant peritoneal disease, and differentiating it from other causes of diffuse peritoneal disease such as peritoneal carcinomatosis is often difficult on imaging. Common findings observed in peritoneal lymphomatosis in contrast to other etiologies include frequent nodal involvement and splenomegaly. We present a case of diffuse peritoneal disease along with ovarian lesions in the absence of abdominal lymphadenopathy or splenomegaly on fluorodeoxyglucose positron emission tomography-computed tomography in the setting of elevated cancer antigen-125 levels, mimicking primary ovarian malignancy causing peritoneal carcinomatosis, which was finally proven to be lymphoma.

A Rare Case of Liver Metastasis from Prostate Cancer Mimicking Hepatocellular Carcinoma on Immunohistochemistry: Role of F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Diagnosis.

We present a case with space-occupying lesion in cirrhotic liver, diagnosed as hepatocellular carcinoma on immunohistochemistry, who underwent F-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) and showed FDG-avid lesions in liver as well as in the prostate. These findings guided in establishing the diagnosis of prostate cancer, metastasizing to liver by performing additional immunohistochemical markers. PET/CT was also useful in identifying coexisting non-Hodgkin's lymphoma.

Atypical Presentation of T-Cell Large Granular Lymphocytic Leukemia Mimicking Pleural Malignancy on 18F-FDG PET/CT.

T-cell large granular lymphocytic leukemia is a rare form of leukemia, caused by clonal proliferation of cytotoxic T-cells, characterized by modest lymphocytosis and cytopenias of other lineage with hepatosplenomegaly and relatively rare lymph nodal involvement. Involvement of other organs is extremely rare. It is predominantly an indolent disease and most of patients remain asymptomatic for a long period. We present a rare case of aggressive form (CD56 positive) of large granular lymphocytic leukemia with atypical presentations mimicking pleural malignancy on F-FDG PET/CT.

18F-FDG PET/CT of Internal Mammary Lymph Node Hepatocellular Carcinoma Metastases.

Hepatocellular carcinoma usually metastasizes to regional abdominal lymph nodes. Distant lymph nodal metastases are relatively rare with most common extra abdominal sites being mediastinum and juxtaphrenic regions. Metastasis to internal mammary lymph nodes is extremely rare, and we present a case of hepatocellular carcinoma with histopathologically proven internal mammary lymph nodal metastasis in the absence of regional abdominal lymph nodal metastases.

Branch Duct-type Intraductal Papillary Mucinous Neoplasm Presenting as Paraneoplastic Small Plaque Para-psoriasis.

To present and discuss a novel association between branch duct-type intraductal papillary mucinous neoplasm and paraneoplastic parapsoriasis. We present the case of a middle-aged male presenting with skin lesions that were suggestive of parapsoriasis, resistant to treatment, and in whom a diagnosis of branch-type intraductal papillary mucinous neoplasm of the pancreas was eventually made. A curative Whipple's surgery led to complete resolution of the skin lesions within 3 weeks. Paraneoplastic parapsoriasis in association with intraductal papillary mucinous pancreatic neoplasm has never been reported before.

A Rare Surprising Cause of Acute Severe Pancreatitis.

A 39-year-old woman is admitted with clinical, biochemical and imaging features suggestive of acute pancreatitis. Conservative and guideline based management failed to improve her condition. Repeate the imaging utilizing contrast revealed the progression of extra-pancreatic signs in the absence of pancreatic necrosis, the absence of intra-abdominal fluid collections, but enhancing omental and peritoneal deposits and obstructive pancreatitis. A biopsy and histopathological evaluation of the omentum revealed rare anaplastic large cell lymphoma causing obstructive pancreatitis. In patients who present with pancreatitis not responding to conventional treatment and with an atypical disease course, the diagnostic possibility of rare causes of pancreatitis such as obstructive/infiltrative pathology must be thought of.