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Introduction: Insulinomas are rare, usually sporadic, and typically benign pancreatic neuroendocrine tumours. Pre-operative localization is challenging and evidence on comparative analysis of anatomic and scintigraphic modalities for pre-operative tumour localization is limited, even in contemporary series. Methods: The current study was designed to study the clinical features and management challenges of insulinomas managed at a tertiary care centre. Clinical features, diagnosis, imaging techniques, surgical procedures, and outcomes details were collated. Pre-operative imaging techniques (CT/MRI, nuclear scintigraphy) were compared with intraoperative and histopathological findings to assess their accuracy of localization. Results: Thirty-seven patients (15 females [42%]; median age 36 years [IQR 28–49]) were included in the study. In four patients (10.8%), the tumour occurred in the setting of multiple endocrine neoplasia type 1 (MEN 1) while the remaining were sporadic. The sensitivity of pre-operative localization was 61.5% (multiphasic CT), 66.6% (multiphasic MRI), 100% (68Ga Exendin-4 PET-CT), and 91.6% (EUS). Three patients with normal multiphasic CT had localization on 68Ga Exendin-4 PET-CT. The positive predictive value (PPV) of both Exendin-PET-CT and EUS was similar at 91.6% and 91.6%, respectively. All patients (except one with nesidioblastosis), who underwent enucleation or partial pancreatic resection, were cured. Conclusion: 68Ga Exendin-4 PET-CT based is a non-invasive imaging modality that has high sensitivity and PPV and can be used as a first-line imaging modality. The overall prognosis of these tumours is good with high cure rates attained following surgical resection.

Post-liver transplant (LT) hepatic artery vasospasm is a vascular complication that is not well recognized and its incidence is not known. As a possible sequela to vasospasm, hepatic artery thrombosis is the second major cause of allograft failure after primary nonfunction and its reported incidence is 2.9% in adults and 8.3% in pediatric LT. Lacuna in knowledge regarding early hepatic artery vasospasm post-LT makes it a difficult condition to diagnose and treat, as the initial ischemic insult on graft can have devastating consequences. We report a case of pediatric progressive familial intrahepatic cholestasis type 3-related chronic liver disease who underwent cadaveric orthotopic LT and postoperatively developed fever, worsening hypotension, and elevated liver enzymes with an absence of arterial flow in intrahepatic branches on Doppler ultrasound. Suspecting early hepatic artery thrombosis, the patient was re-explored and the graft hepatic artery was found to be in a state of vasospasm. Following the infusion of intra-arterial papaverine, urokinase, and intravenous nicorandil, there was an improvement in blood flow. The patient responded well and was discharged on postoperative day 23 with normal liver enzymes.

Objectives: The objective is to decide the site of aortic clamping based on preoperative imaging and intraoperative findings and assess the outcomes on midterm follow-up in patients with juxtarenal aortoiliac occlusion (JRAIO) managed with aortic thromboendarterectomy and bypass grafting. Methods: Forty patients (39 males and one female) with JRAIO were managed with aortobifemoral bypass (ABFB). Two patients required concomitant superior mesenteric artery (SMA) bypass. The aortic clamping site was inter-renal, suprarenal, or supra-celiac, depending on the extent of thrombus relative to the renal artery and SMA ostia. We compared serum creatinine levels with renal ischemic time in the postoperative period. At the mean follow-up of 26.7 months, patients were assessed for graft patency, limb salvage, and survival rate. Results: The mean age of patients was 53.9 years, and 97.5% of patients were heavy smokers. Of the total patients, inter-renal clamping was done in 12.5%, suprarenal clamping in 70%, and supra-celiac clamping in 17.5%. Perioperative morbidity and mortality were 22.5% and 5%, respectively. Five patients with renal ischemic time ≥20 min developed acute kidney injury, showing a significant positive correlation (r = 0.465; P < 0.0001) with clamp time. Postoperative mean serum creatinine values also showed a significant difference (P = 0.0001) between

Although abdominal aortic aneurysm (AAA) is common in the atherosclerotic aorta in the elderly, isolated AAA (IAAA) is a rare entity. Only few case reports of IAAA associated with concomitant dissection are reported in the literature. Here, we present a case of a middle-aged male who presented with abdominal pain with stable hemodynamics. Abdominal examination revealed a pulsatile mass in the left paraumbilical region. CT angiography of the abdomen revealed an isolated infrarenal AAA with dissection extending into the bilateral common iliac arteries and an eccentric partial thrombosis of the false lumen. The patient underwent successful open aneurysmorrhaphy with PTFE graft. Postoperative course was uneventful, and the patient was discharged to follow up in clinics.

Abstract Buerger's disease or thromboangiitis obliterans is a type of obstructive vascular diseases categorized as vasculitis and usually present in 95% of young smoker men. The main pathogenetic mechanism is interplay between immune system and inflammation. Earlier our phase II study has shown that Stempeucel is safe when injected at 2 million cells/kg body weight by virtue of its anti‐inflammatory, immunomodulatory, and angiogenetic properties. The present study was conducted to further assess the safety and efficacy of Stempeucel in critical limb ischemia due to Buerger's disease after obtaining approval from Indian FDA based on the data generated in the phase II study. This is an open label, multicenteric phase IV PMS study conducted across India with experienced vascular surgeons. Fifty patients of critical limb ischemia due to Buerger's disease with Rutherford III‐5 or III‐6 were included in the study and each individual received a dose of 2 million cells/kg body weight of Stempeucel in the calf muscles and around the ulcer. These patients were evaluated over 12 months from drug administration. The present study showed the continued long term efficacy over a period of 12 months follow up in these patients corroborating the result obtained in the previous phase II studies. There was significant improvement in rest pain, ankle systolic pressure, and ankle brachial pressure index with accelerated ulcer healing. In conclusion, the present study shows that the intramuscular administration of Stempeucel continues to be safe, tolerable, and effective alternative treatment in patients with Buerger's disease.

Purpose Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignant neoplasm, usually diagnosed in advanced stage. Role and efficacy of adjuvant radiotherapy has not been well defined. The objective of this study is to describe the various clinical characteristics and prognostic factors affecting the survival of ACC along with the role radiotherapy on overall survival and relapse free survival. Methods A retrospective analysis of 30 patients registered between 2007 and 2019 was carried out. The medical records containing clinical and treatment details were analysed. Data was analysed using SPSS 25.0. Survival curves were computed using Kaplan-Meier method. Univariate and multivariate analyses were performed to analyze the prognostic factors affecting the outcome. A p value of less than 0.05 was considered to be statistically significant. Results The median age of patients was 37.5 years (range, 5–72 years). 20 patients were females. Twenty-six patients had advanced stage (III/IV) disease while only four patients presented in early stage. Twenty-six patients underwent total adrenalectomy. Eighty three percent patients received adjuvant radiation therapy. The median follow up was 35.5 months (range, 7 monthss-132months). The estimated three- and 5-years overall survival (OS) was 67.2% and 23.3%, respectively. Capsular invasion and positive margins were the independent prognostic factors influencing both OS and relapse free survival (RFS). Out of 25 patients who received adjuvant radiation, only three patients had local relapse. Conclusion ACC is a rare and aggressive neoplasm with majority of patients presenting in advanced stage. Surgical resection with negative margins remains the mainstay of treatment. Capsular invasion and positive margins are independent prognostic factors for survival. Adjuvant radiation reduces the risk of local relapse and is well tolerated. Radiation can be used effectively in adjuvant and palliative settings in ACC.

Phaeohyphomycosis is a term used for a rare opportunistic infection caused by a group of dematiaceous fungi which contains melanin in their cell walls. In 1974, the term phaeohyphomycosis was first coined by Ajello for an entity caused by pigmented fungi.1 Four clinical forms of phaeohyphomycosis exist: i) cutaneous, ii) subcutaneous, iii) systemic, and iv) cerebral. Among these, the subcutaneous form (phaeohyphomycotic cyst) is the most common subtype which usually presents as nodular swelling mainly over the distal extremities, which may be misdiagnosed as epidermal inclusion cyst, ganglion or lipoma. These fungi are present in the soil, where they infect mostly farmers and persons working in fields and farms. It was also highlighted that these infections are seen mostly in immunocompromised individuals and are byproducts of antimicrobial, steroid, and immunosuppressive therapy for various illnesses, including cancer, autoimmune diseases, and transplant cases.1 The pigment giving the characteristic brownish-black appearance to the fungi is melanin, which prevents phagocytosis and hence acts as a virulence factor.2 This group has more than 120 species and 70 genera.3 They have a broad spectrum of clinical manifestations, including superficial and deep fungal infections, sinus involvement, and disseminated forms, including lung and brain abscesses. The incidence ranges from 1-3.1 per 100,000 patients.4 The gold standard method for diagnosis is histopathological examination and culture. Fontana-Masson stain is of immense help in identifying these dematiaceous fungi in tissue as it highlights the melanin pigment in the cell walls. Figure 1 refers to a 59-year-old farmer man who presented with nodular swelling over the dorsum of the left hand for the last 9 months. Initially, the swelling was of peanut size, which gradually progressed to the present size of 5x3x2cm. The lesion was painless, well-defined, and freely mobile. The joint was not affected. He is on medication for type II diabetes mellitus and hypertension. He underwent Whipple's surgery for periampullary carcinoma. He was treated for proximal sensory-motor axonal neuropathy and tuberculosis three years back. Because of isoniazid-induced hepatitis, the patient received a modified anti-tuberculous regime for 9 months, after which he developed gastric ulceration with hematemesis and melena. On endoscopic biopsy, he was found to have chronic active gastritis with Helicobacter pylori infection. In addition, the patient had severe iron deficiency and hypoalbuminemia due to malabsorption. After treatment, he completely recovered at the time of hospital discharge. Figure 1 Phaeohyphomycotic cyst. A - A well-encapsulated and unilocular cyst measuring 4.5x2.2x1.8cm in size (scale bar = 2.5cm); The cyst lumen contains homogenous and translucent gelatinous soft material; B - Multinucleated giant cells showing fungal profile with septate, branching and globose swelling (H&E; x200); C - Periodic Acid-Schiff stain showing bright magenta positivity (PAS stain; x200); D - Fontana Masson stain giving brownish black color due to melanin in the fungal cell walls (x200).: Thus, the nodular swelling was wholly excised and sent for histopathological examination. Grossly, a well-encapsulated mass measuring 4.5x2.2x1.8 cm was submitted for histological analysis (Figure 1A). The external aspect appeared intact, yellowish-white, and congested. A unilocular cyst was identified on serial slicing with a capsular thickness of 0.1-0.2cm. The cut surface was soft in consistency with homogenous and gelatinous translucent material within the lumen. On light microscopy, an outer thick fibrous capsule layer was identified. Just beneath this capsular layer was the vascularized granulation tissue, proliferating fibroblasts, numerous multinucleated foreign and Langhan’s giant cells, and variable lymphoplasmacytic cell infiltrate. Also, abundant basophilic mucoid material with a background of many degenerated cells. These giant cells engulfed pigmented fungal profiles that depicted branching, septate and globose swelling (Figure 1B). Periodic Schiff-Acid stain gave bright magenta color to these fungi (Figure 1C), whereas Fontana Masson stain gave brownish black color due to melanin in the fungal cell walls (Figure 1D). Given the morphology, a diagnosis of a phaeohyphomycotic cyst was rendered. On follow-up, the patient is doing well, and has not received any antifungal agent. The leading treatment choice in non-invasive subcutaneous phaeohyphomycosis is local excision.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological entity characterized by subcortical vasogenic edema presenting with acute neurological symptoms. Common precipitating causes include renal failure, pre-eclampsia/eclampsia, post-organ transplant, and cytotoxic drugs. Hypercalcemia is a rare cause of PRES; most cases occur in the setting of severe hypercalcemia secondary to malignancy or iatrogenic vitamin D/calcium overdose. Primary hyperparathyroidism (PHPT), as a cause of PRES, is an oddity. We report two cases of adolescent PHPT presenting with generalized tonic-clonic seizures and altered sensorium. On evaluation, both had hypertension, severe hypercalcemia (serum calcium 14.1 mg/dL and 14.5 mg/dL, respectively) and elevated parathyroid hormone levels. Magnetic resonance imaging (MRI) revealed T2/fluid-attenuated inversion recovery hyperintensities located predominantly in the parieto-occipital regions, suggestive of PRES. Identification and excision of parathyroid adenoma led to the restoration of normocalcemia. Neurological symptoms and MRI changes improved subsequently. An extensive literature search revealed only four cases of PHPTassociated PRES; none of them being in the pediatric/adolescent age group. The predominant clinical manifestations were seizures and altered sensorium. All had severe hypercalcemia; three had hypertension at presentation, while one was normotensive. Parathyroid adenomectomy led to normalization of serum calcium and resolution of neurological symptoms and radiological changes. Thus, severe hypercalcemia, although rare in PHPT, can lead to hypercalcemic crisis precipitating acute hypertension that can result in cerebral endothelial dysfunction with the breakdown of the blood-brain barrier, culminating in PRES. We therefore recommend that serum calcium levels should be checked in all patients with PRES and that PHPT be regarded as a differential diagnosis in those with underlying hypercalcemia.

Abstract Duplication of the inferior vena cava is a rare congenital anomaly, with an incidence of 0.2-3%. Despite being asymptomatic, anomalies of IVC are important in aortoiliac and retroperitoneal surgeries. Preoperative CT imaging is essential to identify any IVC anomaly and to prevent unexpected hemorrhage during surgery. Here, we report a case of a juxtarenal abdominal aortic aneurysm in which we encountered a type I IVC duplication anomaly intraoperatively while performing transperitoneal aneurysmorrhaphy and took precautions to avoid any iatrogenic injuries to either of the two trunks or the pre-aortic trunk of the anomalous duplicate IVC.

Abstract Cyclin D1, a G1-S phase regulator, is upregulated in parathyroid adenomas. Since cyclin-dependent kinase (CDK) inhibitors, CDKN2A and CDKN2B, and RASSF1A (Ras-association domain family 1, isoform A) are involved in G1-S phase arrest and act as potential tumor suppressor genes, we aimed to study potential methylation-mediated inactivation of these genes in parathyroid adenomas. Gene expressions of cyclin D1 (CCND1) and regulatory molecules (CDKN2A, CDKN2B and RASSF1A) was analysed in parathyroid adenoma tissues (n = 30). DNA promoter methylation of cyclin D1 regulators were assessed and correlated with clinicopathological features of the patients. Gene expression analysis showed a relative fold reductions of 0.35 for CDKN2A (p = 0.01), 0.45 for CDKN2B (P = 0.02), and 0.39 for RASSF1A (p

ABSTRACT: Objective: Present a case of an atypical parathyroid adenoma in the mediastinum as a part of multiple endocrine neoplasia type 1 (MEN1).Methods: Clinical, laboratory, imaging, and histolopathogic analyses were performed.Results: A 50-year-old man who was diagnosed with chronic kidney disease secondary to bilateral nephrolithiasis was referred for evaluation of incidentally detected hypercalcemia. He had an acromegaloid facies and lipomas over the forehead. Laboratory evaluation revealed hypercalcemia (3.33 mmol/L), hyperphosphatemia, and elevated intact parathyroid hormone level (215 pmol/L). Serum insulin-like growth factor 1 was elevated and growth hormone was non-suppressible on oral glucose load. Contrast-enhanced magnetic resonance imaging of the sella showed a pituitary microadenoma. A scan using 99mTc-Sestamibi and combined positron emission and computed tomography using fluorocholine F-18 revealed a mediastinal ectopic parathyroid adenoma, thereby attributing hypercalcemia to primary hyperparathyroidism. A clinical diagnosis of MEN1 was made. Genetic analysis revealed an A>T substitution at cDNA 253 in exon 2 of the MEN1 gene. He underwent median sternotomy and a 3 × 3-cm mass was excised, weighing 14 g. Histopathology showed parathyroid tissue with moderate pleomorphism, pushing margins, fibrous trabeculae, atypical mitoses, and low Ki-67 index, suggestive of atypical parathyroid neoplasm. After surgery the patient became normocalcemic. On follow-up, he is asymptomatic without any evidence of recurrence of primary hyperparathyroidism.Conclusion: Parathyroid involvement in MEN1 is almost exclusively in the form of benign hyperplasia. Atypical parathyroid adenomas and parathyroid carcinomas in MEN1 are very rare, limited to only a few anecdotal case reports to which ours contributes a new case.Abbreviations: CKD = chronic kidney disease;iPTH = intact parathyroid hormone;MEN1 = multiple endocrine neoplasia type 1

Background: Primary hyperparathyroidism (PHPT) is characterized by inappropriately elevated serum parathyroid hormone (PTH) level despite elevated serum calcium. Insulin resistant is the basic pathophysiology, behind the higher prevalence of diabetes mellitus in patients with PHPT. However, the improvement in insulin resistance (IR) after curative parathyroidectomy (CPTX) has not been established yet, as the study results are conflicting. Materials and Methods: In this prospective interventional study, ten patients with mild PHPT (Group 1) and another ten patients with moderate to severe PHPT (Group 2) were undergone CPTX. The IR was assessed by homeostasis model assessment-IR (HOMA-IR), quantitative insulin sensitivity check index (QUICKI), fasting plasma glucose (FPG), and fasting serum insulin (FSI), before and 3 months after CPTX. Results: There was no significant change of FPG and FSI, before and after CPTX in Group 1 (P = 0.179 and P = 0.104) and Group 2 (P = 0.376 and P = 0.488). Before surgery, HOMA-IR was higher, and QUICKI was significantly lower, in both Group 1 (P = 0.058 and P = 0.009) and Group 2 (P = 0.023 and P = 0.005) as compared to published normal reference mean, with no significant difference between the groups. Three months after surgery HOMA-IR increased further and QUICKI remained unchanged as compared to baseline, in both Group 1 (P = 0.072 and 0.082) and Group 2 (P = 0.54 and 0.56), but statistically insignificant. Conclusion: IR remained unchanged after CPTX in mild as well as moderate to severe PHPT. Asymptomatic PHPT with abnormal IR should not be used as criteria for parathyroidectomy.

Peripheral vascular disease is a rare feature of pheochromocytoma. This potentially catastrophic but curable tumor should be suspected in combination of distal necrosis with hypertension and palpable pulses. We report such an unusual case of pheochromocytoma presenting as toe necrosis.

Focal infection is an uncommon complication of Salmonella septicemia, particularly in immunocompetent patients. The localization of Salmonella infection to breast tissue is regarded as a rare event. We report a case of bilateral breast abscesses due to Salmonella enterica serotype Typhi in a nonlactating female and highlight the fact that Salmonella spp. should be included in differential diagnosis of abscesses in individuals coming from endemic areas with the history of recent typhoid fever and should be treated accordingly.

Leiomyosarcoma of the gallbladder is a rare entity, constituting about 1.4 per 1000 gallbladder malignancies. Literature review shows female preponderance in sixth decade of life, due to unknown reasons. We report one such rare case of a 50-year-old female admitted with pain in right upper abdomen. On examination, mass was felt in right hypochondrium. The ultrasound abdomen showed mass with loss of interface with liver and cholelithiasis. CECT abdomen showed polypoidal gallbladder malignancy with ill-defined interface with liver. She was operated upon with diagnosis of carcinoma gallbladder; extended cholecystectomy was done. Histopathological examination revealed spindle-cell proliferation and possibility of malignant tumor of mesenchymal origin was kept. This was later confirmed on immunohistochemistry.