Mixed epithelial and stromal tumor (MEST) in a young adult male: A case report and literature review.

INTRODUCTION AND IMPORTANCE: A MEST is a rare renal tumor, with stromal as well as epithelial components. It is predominantly benign and local recurrence is not very common. In the majority of the cases, it occurs in females. Its occurrence in a young male makes it a rarity. CASE PRESENTATION: A 24 years old male presented at SIUT with the complaint of left flank pain on and off for one month. CT scan showed soft tissue density mass in left renal pelvis extending from mid-pole calyces to pelviuretric junction, leading to obstruction and ultimately mild uropathy. We found a partially obstructing staghorn calculus with asymmetrical cortical thinning. Left Robot-Assisted Nephro-ureterectomy plus excision of bladder cuff was planned in which 3 × 4 cm mass involving the left renal pelvis was excised. To date, there is no radiologic evidence of disease recurrence. CLINICAL DISCUSSION: MEST in young adults is an extremely rare tumor. They have been referred to by many alternate synonyms including 'adult mesoblastic nephroma' and 'cystic nephroma' with 'ovarian' or 'cellular' type stroma. Majority of patients with MEST present, with hematuria, abdominal pain, palpable flank mass, recurrent urinary tract infections. Similarly, our patient presented initially with nonspecific pain in the left flank region. Majority of cases in the literature presented with the tumor in benign stage, with localized spread, and without recurrence. CONCLUSION: Mixed epithelial and stromal tumors (MEST) of the kidney are distinct entities of benign kidney tumors. MEST in young males is a very rare entity, and a small number of cases exist. Histopathology plays a very cardinal role in diagnosis, and overall the disease has a promising outcome with conservative surgery.

Sepsis Following Liver Biopsy in a Liver Transplant Recipient: Case Report and Review of Literature.

Percutaneous liver biopsy is a relatively safe procedure with low complication rates. Infections following liver biopsy are uncommon and can lead to a poor outcome. There are limited data on liver biopsy-related infections among liver transplant (LT) recipients. Also, there is a paucity of data regarding the use of prophylactic antibiotics in LT patients undergoing percutaneous liver biopsy. We report a case of systemic sepsis following percutaneous liver biopsy in a LT recipient with choledochojejunal anastomosis. This was followed by severe rejection and deterioration of liver function and recurrence of primary sclerosing cholangitis (PSC) to the extent that he has been listed for retransplantation. This case report emphasizes the potential risk of sepsis in LT recipients with bilioenteric anastomosis undergoing percutaneous liver biopsy. This increased risk may warrant periprocedural broad spectrum antibiotic prophylaxis, in this subgroup of patients.

Unusual Presentation of Systemic Lupus Erythematosus in a Young Male: A Case Report.

Hepatic involvement in systemic lupus erythematosus (SLE) is common but described infrequently. Liver is usually never the primary organ to be affected in lupus. Again hepatic involvement probably does not carry much prognostic importance though it may correlate with lupus activity. We here report a case of 21-year-old man with no prior comorbidity or addiction who presented to us with acute hepatic illness with jaundice. He also had malar rash and arthralgia. Viral markers were negative. Antinuclear antibody and anti-double-stranded DNA (dsDNA) were strongly positive. Liver biopsy was consistent with autoimmune hepatitis, whereas skin biopsy was suggestive of SLE. He had a brisk and complete recovery with prompt use of immunosuppressive agents (corticosteroids and azathioprine). Cyclophosphamide was started latter in view of lupus nephritis. This is probably the fourth reported case of SLE presenting as acute hepatic illness with jaundice.

Survival and Outcome in Patients Receiving Drug-Eluting Beads Transarterial Chemoembolization for Large Hepatocellular Carcinoma (>5 cm).

BACKGROUND/AIMS: This study aimed to study the outcome and survival of patients with large hepatocellular carcinoma (HCC) receiving drug-eluting beads (DEBs) transarterial chemoembolization (TACE). In addition, tumor morphologies were correlated with the response and survival to analyze the association of morphology with the outcome. METHODS: Patients with large HCC (>5 cm) who underwent DEB-TACE for palliation were analyzed retrospectively. Patients were assessed for objective response (OR) and overall survival (OS), which was calculated from the first session of DEB-TACE to the last follow-up/death. OR and OS were calculated for the entire study group and were compared among the subgroups consisting of solitary versus multifocal HCC, unilobar versus bilobar disease, well-defined versus ill-defined HCC, and HCC with homogeneous enhancement versus HCC with heterogeneous enhancement. RESULTS: Sixty-seven DEB-TACE procedures were performed in 25 patients (average: 2.7 ± 1.4 sessions per patient). The mean lesion size was 9.9 ± 4.5 cm. Of 25 patients, 13 (52%) had multifocal HCC. Unilobar disease was seen in 15 patients (60%). The mean duration of follow-up was 24.4 months. OR at 6 and 12 months were 56% and 48%, respectively, with well-defined lesions showing better OR. The median OS was 28 months (95% confidence interval, 12.3-43.6). OS rate at 12 and 24 months was 92% and 57%, respectively. OS was seen to be superior in well-defined HCC and unilobar disease. CONCLUSION: In this study, DEB-TACE has shown to have a good response in patients having large/multifocal HCC with preserved liver functions. Well-defined HCC and unilobar disease have a better response and survival.

Determinants of Outcomes in Autoimmune Hepatitis Presenting as Acute on Chronic Liver Failure Without Extrahepatic Organ Dysfunction upon Treatment With Steroids.

BACKGROUND AND AIMS: Autoimmune hepatitis presenting as acute on chronic liver failure (AIH-ACLF) is a novel entity with limited data on clinical course and management. We assessed outcomes in patients of AIH-ACLF with no extrahepatic organ dysfunction/failure when administered steroids. METHODS: In this retrospective analysis, clinical data, laboratory parameters, liver biopsy indices and prognostic scores such as model for end-stage liver disease (MELD) and Child-Turcotte-Pugh (CTP) scores at baseline were computed for patients with AIH-ACLF and compared across strata of incident infections and transplant-free survival. The primary outcome was 90-day transplant-free survival. Biochemical remission was assessed, and predictors of end points were identified. RESULTS: Twenty-nine patients of AIH-ACLF were included with a median follow-up of 4 months. The 90- and 180-day transplant-free survival rates of 55.2 [95% confidence interval (CI): 39.7-76.6]% and 30.2(95% CI: 16.7-54.6)%, respectively, were attained on steroids. Three patients (10.3%) underwent liver transplant while 16 (55.2%) deaths occurred. Infections developed in 12 patients (41.3%), leading to worsening prognostic scores, new onset organ dysfunction/failure and 11 deaths. Seven of ten patients (70%) in the transplant-free survivor group attained biochemical remission on follow-up. The MELD score<24 (sensitivity: 68.4%; specificity: 80%) and CTP<11 (sensitivity: 78.9%; specificity: 90%) had best predictive value for survival, in addition to decrease in the MELD score at 2 weeks (sensitivity: 78.9%; specificity: 70%). CONCLUSION: Patients with AIH-ACLF have a morbid disease course despite treatment with steroids. Patients with no extrahepatic organ failure with good baseline prognostic scores may be administered steroids with close monitoring for change in MELD over 2 weeks.

Relative Adrenal Insufficiency in Patients with Alcoholic Hepatitis.

BACKGROUND/AIMS: Alcoholic hepatitis (AH) is an acute hepatic inflammation associated with high morbidity and mortality. Treatment with steroids is known to decrease short-term mortality in severe AH patients. Hence, we hypothesize that adrenal insufficiency can be associated with severe AH and affects prognosis. The aim of this study was (1) to evaluate relative adrenal insufficiency (RAI) in patients with AH and (2) to Compare RAI with the severity of AH. METHODS: Newly diagnosed cases of AH hospitalized in SMS Medical College and Hospital, Department of Gastroenterology were, enrolled. All patients of AH were classified as mild and severe AH on the basis of Maddrey discriminant function (DF). After baseline serum cortisol, 25 IU ACTH (Adreno Corticotrophic Hormone) was injected intramuscularly and blood sample was collected after 1 h and assessed for serum cortisol. RAI was defined as <7 µg increase in the cortisol level from baseline. RAI was compared with severity of AH. RESULTS: Of 120 patients of AH, 58 patients fulfilled the inclusion criteria, in which 48 patients were diagnosed as severe AH and 10 patients were diagnosed as mild AH. In patients with severe AH, the baseline mean serum cortisol level was significantly high as compared with mild AH; 26 patients (54.16 %) of 48 patients with severe AH showed RAI (P ≤ 0.001).Whereas in patients with mild AH, none of patients showed RAI. RAI also showed negative correlation with DF. There was no difference in RAI with respect to acute kidney injury (AKI). CONCLUSION: RAI is a common entity in patients with severe AH, and it is related with the severity of disease.

INASL Guidelines on Management of Hepatitis B Virus Infection in Patients receiving Chemotherapy, Biologicals, Immunosupressants, or Corticosteroids.

Hepatitis B Virus (HBV) reactivation in patients receiving chemotherapy, biologicals, immunosupressants, or corticosteroids is emerging to be an important cause of morbidity and mortality in patients with current or prior exposure to HBV infection. These patients suffer a dual onslaught of illness: one from the primary disease for which they are receiving the culprit drug that led to HBV reactivation, and the other from HBV reactivation itself. The HBV reactivation not only leads to a compromised liver function, which may culminate into hepatic failure; it also adversely impacts the treatment outcome of the primary illness. Hence, identification of patients at risk of reactivation before starting these drugs, and starting treatment aimed at prevention of HBV reactivation is the best strategy of managing these patients. There are no Indian guidelines on management of HBV infection in patients receiving chemotherapy, biologicals, immunosupressants, or corticosteroids for the treatment of rheumatologic conditions, malignancies, inflammatory bowel disease, dermatologic conditions, or solid-organ or bone marrow transplantation. The Indian National Association for Study of the Liver (INASL) had set up a taskforce on HBV in 2016, with a mandate to develop consensus guidelines for management of various aspects of HBV infection, relevant to India. In 2017 the taskforce had published the first INASL guidelines on management of HBV infection in India. In the present guidelines, which are in continuation with the previous guidelines, the issues on management of HBV infection in patients receiving chemotherapy, biologicals, immunosupressants, or corticosteroids are addressed.