Diverse facets of MDSC in different phases of chronic HBV infection: Impact on HBV-specific T-cell response and homing.

BACKGROUND AND AIMS: Chronic HBV infection (CHI) is associated with a diverse natural history that includes immune-tolerant (IT), HBeAg-positive chronic hepatitis B (CHB) (EP-CHB), inactive carrier, and HBeAg-negative CHB (EN-CHB) phases. A hallmark of CHI is impairment of HBV-specific T-cell response. Recently, myeloid-derived suppressor cells (MDSCs) have emerged as key regulator of T cells, and their properties are sculpted by their microenvironment. Here, we investigated the distinctive features of MDSCs during CHI, identified factors responsible for their functional discrepancies, and studied their impact on HBV-specific T-cell response and homing. Influence of antiviral therapy on MDSC profile and T-cell response was also assessed. APPROACH AND RESULTS: Flow cytometric analysis indicated that MDSCs in EP-CHB/EN-CHB patients had profound suppressive ability, expressing arginase 1 (Arg1)/inducible nitric oxide synthase (iNOS)/programmed death ligand 1 (PD-L1)/cytotoxic T lymphocyte-associated protein 4 (CTLA-4)/CD40 at significantly greater levels relative to healthy controls (HC). However, in IT, only Arg1+ MDSCs and in inactive carrier, iNOS+ and PD-L1+ MDSCs were higher than HC. In vitro assays demonstrated that high HBsAg titer in IT/CHB induced Arg1+ MDSC. Furthermore, elevated serum TNF-α and IL-4 in CHB potentiated Arg1/PD-L1/CD40/CTLA-4 expression, whereas increased IL-1ß in CHB/IC triggered the expansion of PD-L1+ MDSCs and iNOS+ MDSCs. MDSCs, sorted from CHB/IC, greatly attenuated IL-2/interferon gamma (IFN-γ) production by HBV-specific CD8+ /CD4+ T cells, the effect being more pronounced in CHB. However, MDSCs of IT minimally affected the cytokine production by T cells. Adding Arg1-/iNOS-inhibitor restored only IFN-γ production, while neutralizing PD-L1 recovered both IL-2 and IFN-γ secretion by T cells. Moreover, MDSCs from IT/CHB disrupted virus-specific T-cell trafficking by down-regulating chemokine receptor type 5 on them via TGF-ß signaling. One year of tenofovir therapy failed to normalize MDSC phenotype and HBV-specific T-cell response. CONCLUSIONS: Diversity of MDSCs during CHI affects HBV-specific T-cell response and homing. Hence, therapeutic targeting of MDSCs could boost anti-HBV immunity.

Immunohistochemistry as a Surrogate Marker of Underlying Molecular Derangements in Sporadic Colorectal Carcinoma in Children - A Series of Three Cases.

Background: Colorectal carcinomas (CRCs) are uncommon tumors in children. Here, we elucidate three cases of childhood CRCs with their underlying molecular derangements using immunohistochemistry (IHC) with emphasis on BRAF mutation. Case summary: All three CRCs were sporadic tumors involving the left colon with two of them having a mucinous phenotype. We performed IHC for BRAF, p53 and ß-catenin along with markers of microsatellite instability (MSI) in all three tumors. All the tumors had diffuse strong cytoplasmic BRAF positivity, with focal p53 positivity in two cases and cytoplasmic ß-catenin staining in one case. One case showed CpG island hypermethylation with isolated loss of PMS2 staining. None of the cases had any family history of CRC. Conclusions: IHC can be used as a surrogate marker for determining the underlying molecular derangements in CRC. Sporadic CRCs in children are a cumulative effect of multiple mutations, of which BRAF mutation is significant and critical for planning targeted therapy.

Utility of PAS and ß-catenin staining in histological categorisation and prediction of prognosis of hepatoblastomas.

AIMS: The aim of this study was to assess the usefulness of PAS, ß-catenin and Ki-67 in categorising hepatoblastomas (HBs) and their significance in predicting prognosis. In addition, we have also documented the various chemotherapy induced histologic changes in HBs. METHOD: In this retrospective observational study of 29 cases of hepatoblastomas, 27 cases were considered for statistical analysis, excluding two cases of diagnostic core biopsies. All clinicopathological parameters and follow up data were collected. All HB cases including the mixed epithelial and mesenchymal HBs were classified into two groups: fetal predominant and embryonal predominant type according to the predominant epithelial component. PAS, ß-catenin and Ki-67 staining were done and their correlation with histologic subtypes was assessed. Kaplan-Meier survival analysis was performed in relation to histology, PAS, ß-catenin and Ki-67 staining characteristics. RESULT: Diffuse nuclear staining of ß-catenin was significantly more common in embryonal predominant type (p = 0.036), whereas strong PAS positivity was significantly associated with fetal predominant type (p = 0.002). But no significant correlation was seen between histologic type and Ki-67 staining (p = 0.42). Survival analysis showed cases with diffuse PAS positivity, focal nuclear ß-catenin staining and low Ki-67 LI had better survival. CONCLUSION: PAS is a simple stain and can be utilised in histological categorisation of HB and also predicting its outcome. Nuclear ß-catenin staining which is significantly common in embryonal elements in HB predicts shorter survival.

INI Expressing Epithelioid Sarcoma with Osteoclastic Giant Cells in a Child: A Case Report with Summary of Prior Published Cases.

BACKGROUND: Epithelioid sarcoma is a heterogeneous tumor with 2 subtypes, classic and proximal. The proximal variant is more aggressive and occurs in proximal location in young adults. CASE REPORT: We present a proximal epithelioid sarcoma in the leg of an 8 year old girl with rhabdoid morphology and scattered osteoclastic giant cells. Nuclear INI-1 was retained. Despite wide local excision, local recurrence occurred at 8 months. Following re-excision, she developed a chest wall metastasis after 9 months. CONCLUSION: Epithelioid sarcoma, proximal type with osteoclastic giant cells in the pediatric age group has not been reported previously and should be considered in the differential diagnoses of tumors with epithelioid cell morphology and scattered osteoclastic giant cells. Retained INI expression helped to differentiate this tumor from malignant rhabdoid tumor.

Primary Renal Rhabdomyosarcoma: An Unusual Bone Metastasizing Tumor of Kidney.

Rhabdomyosarcoma (RMS) is one of the common malignant tumors in the pediatric age group. There is only a single case report of primary renal alveolar RMS. Fine needle aspiration (FNA) findings of primary renal RMS has not been reported so far. Hence we present an unusual case of primary alveolar RMS of the kidney. An 11 year old boy presented with an abdominal mass. On FNA a diagnosis of undifferentiated sarcoma and anaplastic Wilms tumor were considered. The tumor was resected and showed histopathological features of alveolar rhabdomyosarcoma. He developed multiple bony metastases and succumbed to the illness despite aggressive chemotherapy. RMS of the kidney should be considered in the differential diagnosis of children with a renal mass, and may have an aggressive clinical course with bone metastases.

Outcome of surgery for recurrent pyogenic cholangitis: a single center experience.

BACKGROUND: Although, recurrent pyogenic cholangitis (RPC) is prevalent in several parts of India, there is paucity of published literature from India. The aim of this study was to report on the short and long-term outcomes of surgery for RPC. METHODS: All the patients, who underwent surgery for RPC between August 2007 and February 2016 in the Department of Surgical gastroenterology, Institute of Postgraduate Medical Education and Research, Kolkata, India were retrospectively reviewed. RESULTS: Of the total 94 patients, 66 (70%) were women. The median age at presentation was 40 years. Left lobe was solely involved in 49% of patients. Left hepatectomy was performed in 23/44 (52%) patients who underwent liver resection. Thirty two postoperative complications developed in 27 patients (29%). Complete stone clearance was possible in 83% of patients after primary surgery. Over a median follow up of 36 months, seventy one of 87 patients (81%) remained free of stones. Recurrent cholangitis occurred 10 of 87 patients (11%). CONCLUSIONS: Surgery plays an important role in the management of RPC achieving excellent short and long-term outcomes.

Intraductal papillary neoplasm of the bile duct: A case report of a rare tumor with a brief review of literature.

INTRODUCTION AND IMPORTANCE: Intraductal papillary neoplasm of the bile duct (IPNB) is a rare neoplasm, mostly found in patients from far Eastern areas where hepatolithiasis and clonorchiasis are endemic. Very few cases are reported from India. CASE PRESENTATION: We present a case of recurrent cholangitis in a 59-year-old male, initially suspected to have IPNB based on preoperative imaging. Confirmation occurred intraoperatively, with mucin-filled bile ducts and papillary lesions in the resected hepatic duct. Treatment included left hepatectomy, extrahepatic bile duct excision, and Roux-en-Y hepaticojejunostomy. Histopathology indicated invasive pancreatobiliary-type IPNB with clear margins. The patient experienced post-hepatectomy hepatic insufficiency and superficial incisional surgical site wound infection, managed conservatively. Discharge occurred on postoperative day 21, with satisfactory recovery at the 16-month follow-up. CLINICAL DISCUSSION: IPNB is recognized as the biliary equivalent of intraductal papillary mucinous neoplasm, as these two conditions exhibit multiple commonalities in terms of clinical and histopathological characteristics. The unique aspect of our case lies in the intricacies associated with its diagnosis. Initially, imaging modalities did not yield a definitive characterization of the lesion. Notably, the endoscopist misinterpreted mucin expression emanating from the papilla as purulent material, primarily due to the patient's concurrent cholangitis. Subsequent repetitions of both CT scan and MRI provided some valuable insights that contributed to the diagnostic clarity of the IPNB. CONCLUSION: In cases of symptoms like biliary obstruction with bile duct dilation, wall nodules, papillary/solid-cystic masses, and upstream-downstream dilation, IPNB should be considered. Striving for R0 resection is crucial for enhanced long-term patient survival.

Cytomorphological and morphometric analysis of 22 cases of Rosai-Dorfman disease: a large series from a tertiary care centre.

BACKGROUND: Rosai-Dorfman disease (RDD) is a rare benign idiopathic histiocytic proliferation. Most commonly reported cases are lymph nodal. Only 30-40% of cases occur in extranodal sites. Although the morphological features of RDD are well documented, there is limited information about the morphometric variations in the histiocytes of this entity. METHOD: Twenty-two cases of RDD diagnosed on fine-needle aspiration cytology (FNAC) were retrieved from the archives. Both Papanicolaou- and May-Grünwald-Giemsa-stained slides were available for evaluation in all cases. Nuclear area, diameter and histiocyte size were measured taking reactive histiocytes as controls. RESULTS: Among the 22 patients (male/female ratio 3:2; age range 5-55 years, mean 26 years), 3 cases were extranodal and 19 cases were nodal. The nodal sites included cervical, axillary, inguinal and submandibular lymph nodes. The extranodal sites were the retroperitoneum, mediastinum and skin. The most common clinical presentation was enlarged lymph nodes. Cytological features included numerous large benign histiocytes with emperipolesis. All the morphometric parameters were significantly (p < 0.01) higher in RDD histiocytes than in histiocytes in the reactive lymph node. CONCLUSIONS: In view of the rarity of the disease and the variable clinical presentation in RDD, accurate diagnosis is necessary. This is the first study to document the morphometric parameters of RDD histiocytes and their comparison with histiocytes in the reactive lymph node.

Primary hepatic leiomyosarcoma masquerading as focal nodular hyperplasia of liver: A wolf in sheep's clothing.

ABSTRACT: Primary hepatic leiomyosarcoma is a rare hepatic malignancy which requires exclusion of other primary site of origin. Clinical presentation and imaging of this tumor is nonspecific and mimics many other hepatic neoplasms. A 62-year-old female patient presents here with right hepatic mass with insidious onset and radiological features favoring a benign solid lesion suggestive of focal nodular hyperplasia. On right hepatectomy, an encapsulated mass identified about 11 cm in maximum dimension with pushing margin and central scar-like area. Histopathological examination reveals a spindle cell tumor and panel of immunohistochemical markers is required to distinguish it from other morphological mimickers. Diagnosis of primary hepatic leiomyosarcoma requires histopathology along with immunohistochemical examination. It is thus advisable to do preoperative biopsy with immunohistochemistry in all patients having atypical imaging and clinical features.

Differentiating biliary atresia from other causes of infantile cholestasis: An appraisal of the histomorphological changes on liver biopsy.

Background: Cholestatic disorders are a significant cause of morbidity and mortality in infants. Characterization of these disorders and differentiating biliary atresia (BA) from other causes of intrahepatic cholestasis is an age-old problem. Objectives: To study the spectrum of different infantile cholestatic disorders in our population, to differentiate BA from other causes of neonatal cholestasis (NC) on a liver biopsy, and validation of the available scoring system for the characterization of these disorders. Materials and Methods: This is an observational cross-sectional study performed over a period of 3 years between 2018 and 2021, done on neonates and infants presenting with cholestatic jaundice. The changes on liver biopsy were evaluated by different histological parameters and available scoring systems to differentiate BA from non-BA causes. Correlation with clinical, biochemical, and imaging findings was done in all cases. Results: This study included 87 cases of NC, of which BA comprised 28 cases (32%), whereas idiopathic neonatal hepatitis (INH) comprised only 12 cases (14%). Portal neutrophilic inflammation (P = 0.000053), ductal cholestasis (P < 0.001), neoductular bile plugs (P < 0.001) and bile ductular proliferation (P < 0.0001) were significantly more in BA, whereas lobular lymphocytic inflammation (P = 0.001) and giant cell transformation of hepatocytes (P = 0.0024) were more frequent in the non-BA group. Using the Lee and Looi scoring system, a histologic score ≥7 was helpful in identifying BA with 85.7% sensitivity, 92.6% specificity, and 90.6% accuracy. Conclusion: BA is the commonest cause of NC in neonates, whereas the frequency of INH is declining. Detailed histomorphologic analysis of liver biopsy, aided with IHC, is the cornerstone for the diagnosis of these disorders.

Extrahepatic mucinous biliary cystadenoma: A rare cause of gastric outlet obstruction.

Extrahepatic mucinous biliary cystadenoma is an extremely rare clinical entity that can present with varieties of vague clinical signs and symptoms. Gastric outlet obstruction caused due to this has never been reported before. We highlighted the diagnostic dilemma we faced when radiological investigations could not suggest the accurate organ of origin.

Outcome of Whipple's procedure for Groove pancreatitis: A retrospective cross-sectional study.

Background: Groove pancreatitis (GP) is a rare form of chronic pancreatitis primarily affecting the pancreatoduodenal groove. Very few studies have been published from India. The aim of the present study is to report our experience with Whipple's procedure for GP. Methodology: In this cross-sectional study, data of all patients who underwent Whipple's procedure for GP between August 2007 and July 2021 were retrospectively reviewed. Results: Of the total 504 Whipple's procedures, histopathologically proven GP was identified in 9 patients. All of them were male. Mean age at presentation was 42.66 ± 4.35 years. All of them had history of alcohol abuse. Eight (88.8%) of them had history of smoking. Postprandial abdominal discomfort and pain (n = 9, 100%) was the most common presenting symptom. Three (33.3%) patients had solid variety and six (66.6%) patients had cystic dystrophy of the duodenal wall. Two (22.2%) patients had mass in the head of the pancreas which was thought to be malignant. None of the patients underwent prior endoscopic management (stenting). Duration of surgery and blood loss was 330 (range, 300-379) minutes, and 250 (range, 200-750) ml respectively. There was no postoperative mortality. Postoperative complications developed in 5 (55.5%) patients. All the complications were managed conservatively. Median postoperative hospital stay was 10 (range, 9-16) days. Over a median follow-up of 41 (range, 12-120) months, complete remission of symptoms was achieved in 7 (78%) patients. Conclusion: Whipple's procedure is safe with acceptable perioperative outcomes and good long-term symptom control.

Clinical profile and outcome of solid pseudopapillary neoplasm of the pancreas: A retrospective study on 28 patients.

BACKGROUND: Solid pseudopapillary neoplasms (SPN) of the pancreas are rare tumors accounting for 0.9-2.7% of all exocrine pancreatic tumors. Very few studies comprising of more than 10 patients have been published. The aim of the present study is to report on our experience with SPNs over a period of 14 years from a tertiary center of Eastern India. METHODOLOGY: Data of all patients whose histopathology reports of surgically resected specimen confirmed SPN were retrospectively reviewed in the present study. RESULTS: Twenty-eight patients had a pathologically confirmed diagnosis of SPN. Twenty-five (89.3%) patients were females with a median age of 26 (15-45) years. Abdominal pain (89.2%) was the most common presenting symptom. Abdominal mass was palpable in 12 (42.8%) patients. The mean size of the tumor was 9.03 cm (range, 4-25 cm). The most common location of the tumor was in the body and tail of pancreas (35.7%). The most commonly performed operation was distal pancreaticosplenectomy (n = 17, 60.7%), followed by Whipple's procedure (n = 8, 28.5%). Thirty postoperative complications developed in 23 (82.1%) patients. The operative mortality was 3.5% (n = 1). The median hospital stay was 10 (5-25) days. Over a median follow-up period of 36 months (range), no patient developed recurrence. CONCLUSION: Although the size of tumor was quite large at the time of initial presentation, complete surgical resection was possible in all the patients. In experienced hand, surgery can be performed with acceptable perioperative mortality and excellent long-term outcomes.

Surgical Outcome of Brunner's Gland Hamartoma: A Single-Centre Experience.

Introduction Brunner's gland hamartomas (BGH) are rare benign lesions with an incidence of <0.01%, accounting for 5 to 10% of all benign tumors of the duodenum. It requires expeditious management by a multidisciplinary team. The aim of the study is to report our experience with surgery for BGH. Methodology Data of all patients who underwent surgical intervention for duodenal polypoidal mass between August 2007 and March 2020 were retrieved from our prospectively maintained gastrointestinal (GI) surgery database. All patients whose histopathology report of the resected specimen confirmed BGH ( n = 9) were included in the present study. Other pathological diagnosis like duodenal lipoma ( n = 2), ganglioneuroma ( n = 1), adenoma ( n = 10), and adenocarcinoma ( n = 4) were excluded. Results Nine patients had confirmatory histopathological diagnosis of BGH and met our inclusion criteria. Three (33.3%) of them were men with a median age of 45 (range: 24-61) years. The median interval between onset of symptoms and diagnosis of duodenal polyp was 14 (range: 4-180) days. Five patients (55.5%) presented with upper GI hemorrhage. Three (33.3%) patients presented with abdominal pain, and one (11.1%) patient presented with episodes of bilious vomiting. Diagnostic endoscopy could detect the lesion in all (100%) patients. Contrast-enhanced computed tomography detected duodenal polypoidal lesion in five (55.5%) patients. The mean size of tumor was 4.78 ± 1.36 cm. These lesions were symptomatic in all the patients and warranted intervention. In view of failed endoscopic intervention ( n = 7, 77.7%), or extramural extension of the tumor ( n = 2, 22.2%), surgical intervention was considered. Most commonly performed operation was duodenal polypectomy ( n = 6, 66.6%). Three postoperative complications developed in two (22.2%) patients. There was no surgery-related mortality. After a median follow-up of 60 (12 -78) months, no patient developed GI bleed or intestinal obstruction. Conclusion In this study, the clinical profile of BGH was explored from the surgeon's point of view. Although endoscopic management is the first-line treatment, surgery plays an important role, particularly, if this fails or is not feasible. In experienced hand, surgery can be performed with acceptable perioperative morbidity and mortality and long-term satisfactory outcomes.

Multifaceted Defects in Monocytes in Different Phases of Chronic Hepatitis B Virus Infection: Lack of Restoration after Antiviral Therapy.

Monocytes play an important role in the control of microbial infection, but monocyte biology during chronic hepatitis B virus (HBV) infection (CHI) remains inadequately studied. We investigated the frequency, phenotype, and functions of monocyte subsets in different phases of CHI, namely, immune tolerance (IT), hepatitis B early antigen (HBeAg)-positive/HBeAg-negative chronic hepatitis B (EP-/EN-CHB, respectively), and inactive carrier (IC), identified factors responsible for their functional alterations, and determined the impact of antiviral therapy on these cells. Flow cytometric analysis indicated that HLA-DR+ CD14++ CD16- classical monocytes were significantly reduced while HLA-DR+ CD14++ CD16+ intermediate and HLA-DR+ CD14+ CD16++ nonclassical monocytes were expanded in IT and EP-/EN-CHB compared with those in IC and healthy controls (HC). In comparison to IC/HC, monocytes in IT and CHB exhibited diminished expression of Toll-like receptor 2 (TLR-2)/TLR-4/TLR-9 and cytokines interleukin-12 (IL-12)/tumor necrosis factor alpha (TNF-α)/IL-6 but produced higher levels of IL-10/transforming growth factor ß (TGF-ß). Further, monocytes in CHB/IT showed impaired phagocytosis and oxidative response relative to those in IC/HC. In vitro assays indicated that high titers of hepatitis B surface antigen (HBsAg) present in IT/CHB and of IL-4 in CHB triggered the functional defects in monocytes via induction of ß-catenin. Additionally, monocyte-derived M1 macrophages of CHB/IT produced fewer proinflammatory and more anti-inflammatory cytokines than those of IC/HC, while in CHB/IT, the monocytes skewed the differentiation of CD4+ T cells more toward regulatory T cells and a Th2 phenotype. Moreover, monocytes in CHB and IT overexpressed chemokine receptor CCR2, which coincided with increased intrahepatic accumulation of ß-catenin+ CD14+ cells. One year of tenofovir therapy failed to normalize monocyte functions or reduce serum HBsAg/IL-4 levels. Taken together, monocytes are functionally perturbed mostly in IT and EP-/EN-CHB phases. Targeting intramonocytic ß-catenin or reducing HBsAg/IL-4 levels might restore monocyte function and facilitate viral clearance. IMPORTANCE Chronic HBV infection (CHI) is a major cause of end-stage liver disease for which pharmacological treatments currently available are inadequate. Chronically HBV-infected patients fail to mount an efficient immune response to the virus, impeding viral clearance and recovery from hepatitis. Monocytes represent a central part of innate immunity, but a comprehensive understanding on monocyte involvement in CHI is still lacking. We here report a multitude of defects in monocytes in chronically HBV-infected patients that include alteration in subset distribution, Toll-like receptor expression, cytokine production, phagocytic activity, oxidative response, migratory ability, polarization of monocyte-derived macrophages, and monocyte-T-cell interaction. We demonstrated that high levels of hepatitis B virus surface antigen and IL-4 potentiate these defects in monocytes via ß-catenin induction while therapy with the nucleotide analog tenofovir fails to restore monocyte function. Our findings add to the continuing effort to devise new immunotherapeutic strategies that could reverse the immune defects in CHI.

Isolated pancreatic and peripancreatic nodal tuberculosis: A single-centre experience.

Isolated pancreatic and peripancreatic nodal tuberculosis may mimic pancreatic malignancy and may result in unnecessary surgery. Being a treatable disease, it is desirable to diagnose this condition without a laparotomy. Pancreatic tuberculosis should be considered in the differential diagnosis of a peripancreatic mass if the patient is young, residing in endemic tuberculosis regions, or has had a history of tuberculosis. We report our experience with pancreatic tuberculosis. Over a period of 12 years, we have managed 16 such patients, of whom 10 were diagnosed preoperatively. In six, the diagnosis was only made after extensive surgery. All 16 patients received antitubercular drugs and were well at a median follow-up of 52 months. Although pancreatic tuberculosis can be diagnosed by modern image-guided biopsy in a fair number of patients, if a high index of suspicion is maintained, a laparotomy may still be unavoidable in a subset of patients.

Refractory hypoglycaemia in a localised gastrointestinal stromal tumour: Case report.

INTRODUCTION: GIST and NICTH are mesenchymal in origin however there are very few reports of GIST associated with NICTH which is a para neoplastic syndrome, generally diagnosed when a tumour induced hypoglycaemia is noted. CASE PRESENTATION: A 46 years old female with prime complain of awareness of a mass in the upper abdomen was admitted for evaluation and further management. Detailed investigation revealed the mass to be gastrointestinal stromal tumour. On the day of admission patient was found to be hypoglycaemic which didn't resolve even after 10% glucose infusion. A growth hormone releasing peptide-2 (GHRP-2) assay was carried out which showed an excessive reaction of basal growth hormone however corticotropin releasing hormone (CRH) tests were within normal limits. She was suspected to be Non Islet cell tumour hypoglycaemia (NICTH) and hypoglycaemia resolved upon administering dexamethasone. Later she underwent chemotherapy and surgical resection after which her blood sugar levels were within normal limits. DISCUSSION: Expression of big IGF-II on the surface of GIST be it metastatic or nonmetastatic can cause refractory hypoglycaemia and can be fatal if left untreated. CONCLUSION: Clinicians should be aware of refractory hypoglycaemia in patients with large GIST's as glucocorticoid therapy may prove to be extremely useful and lifesaving even before considering any forms of definitive management of the tumour.

Primary tubercular granulomatous hepatitis presenting as fluctuating jaundice.

Granulomatous hepatitis is an uncommon presentation of tuberculosis (TB). It is even more peculiar to have TB confined to the liver alone with no pulmonary or a disseminated form. In either form, there is the usual presentation of nonprogressive cholestatic jaundice, but no documented case with fluctuating jaundice in the literature was found. In order to highlight this rare presentation aiding the right diagnosis, we present one such case of a 46-year-old woman with no known comorbidities, who complained of fluctuating and painless type of jaundice, associated with fatiguability, pruritus, and weight loss. Preliminary blood investigations showed anemia and cholestatic pattern of jaundice. Ultrasonography and computed tomography imaging showed hepatomegaly with heterogeneous texture. Magnetic resonance cholangiopancreatography further revealed features of cholecystitis with hepatic ducts near proximal common bile duct showing postinflammatory change. The periampullary region was normal. Sputum acid-fast staining and cartridge-based nucleic acid amplification test were negative. Eventually, liver biopsy was done which showed caseating granulomas with Langhans giant cells. The tissue was abundant in acid-fast bacilli. The patient was started on a 9-month course of first-line Antitubercular treatment (ATT) and responded well. Fluctuating jaundice is a rare and undocumented presentation of primary hepatic TB and can cause diagnostic dilemmas.

Duodenal Ganglioneuroma: A Rare Tumor Causing Upper Gastrointestinal Bleed.

Neuroblastic tumors (NTs) include neuroblastoma, ganglioneuroblastoma, and ganglioneuroma (GN). They are very rare in adults. The Surveillance, Epidemiology, and End Results identified 144 patients ≥20 years old at diagnosis (6.1%) from 1973 to 2002. GNs account for 14% of all localized NT. Since 1957, a total of four cases of GN of the duodenum have been reported. We report a novel case of GN of the periampullary region in the duodenum in a 41-year-old man presenting with chronic upper gastrointestinal bleed. Given the rarity of GNs in this age group and the nonspecificity of radiological features, this diagnosis is often missed until histopathology is done. This may negatively affect the prognosis of an otherwise well-prognosticated disease.