Disseminated multiloculated peritoneal inclusion cysts: Bubble trouble in belly.

ABSTRACT: Multiloculated peritoneal inclusion cysts, usually arise from peritoneal mesothelium lining the serous cavity of the abdomen, pelvis and retroperitoneum. These lesions can be incidentally found on imaging or during surgery, and confirmation of the diagnosis is done by radiological imaging, histomorphology and immunohistochemical findings. Although fewer than 200 cases of solitary peritoneal inclusion cysts have been reported, their occurrence in a disseminated fashion has hardly ever been described in literature. Herein, we report a case of multiloculated peritoneal inclusion cysts that involved the whole abdominal and pelvic cavity and were successfully treated with surgery.

Multilocular cystic nephroma in an adult: a diagnostic quandary.

Multilocular cystic nephroma (MLCN) is an unusual, benign slow-growing renal cystic neoplasm which mimics other cystic renal lesions and has such clinical, radiological, and morphological features that causes diagnostic dilemma. MLCN lies in the spectrum of mixed epithelial and stromal tumor (MEST) family of kidney. According to World Health Organization (WHO 2016 classification), MEST encompasses spectrum of tumors ranging from predominantly cystic tumors, adult cystic nephroma (ACN) to tumors that are variably solid (MEST), thus creating diagnostic dilemma. Moreover, it has several benign and malignant differentials due to its several overlapping histomorphological features which when not cautiously dealt with may result in misdiagnosing it as malignant lesion. We hereby present a case of a woman in late twenties who presented with left flank swelling and pain since 6 months which was misdiagnosed as renal cell carcinoma on radiology which turned out to be ACN on histology and further verified on immunohistochemistry.

Standardizing Randomized Controlled Trials in Celiac Disease: An International Multidisciplinary Appropriateness Study.

BACKGROUND & AIMS: There is a need to develop safe and effective pharmacologic options for the treatment of celiac disease (CeD); however, consensus on the appropriate design and configuration of randomized controlled trials (RCTs) in this population is lacking. METHODS: A 2-round modified Research and Development/University of California Los Angeles Appropriateness Method study was conducted. Eighteen gastroenterologists (adult and pediatric) and gastrointestinal pathologists voted on statements pertaining to the configuration of CeD RCTs, inclusion and exclusion criteria, gluten challenge, and trial outcomes. Two RCT designs were considered, representing the following distinct clinical scenarios for which pharmacotherapy may be used: trials incorporating a gluten challenge to simulate exposure; and trials evaluating reversal of histologic changes, despite attempted adherence to a gluten-free diet. Each statement was rated as appropriate, uncertain, or inappropriate, using a 9-point Likert scale. RESULTS: For trials evaluating prevention of relapse after gluten challenge, participants adherent to a gluten-free diet for 12 months or more with normal or near-normal-sized villi should be enrolled. Gluten challenge should be FODMAPS (fermentable oligosaccharides, disaccharides, monosaccharides, and polyols) free, and efficacy evaluated using histology with a secondary patient-reported outcome measure. For trials evaluating reversal of villus atrophy, the panel voted it appropriate to enroll participants with a baseline villus height to crypt depth ratio ≤2 and measure efficacy using a primary histologic end point. Guidance for measuring histologic, endoscopic, and patient-reported outcomes in adult and pediatric patients with CeD are provided, along with recommendations regarding the merits and limitations of different end points. CONCLUSIONS: We developed standardized recommendations for clinical trial design, eligibility criteria, outcome measures, gluten challenge, and disease evaluations for RCTs in patients with CeD.

Incidental Identification of Osteoid Osteoma of Skull Bone on 68 Ga-PSMA PET/CT.

ABSTRACT: An osteoid osteoma (OO) is a benign bone neoplasm, characterized by significant nocturnal pain that usually responds to nonsteroidal anti-inflammatory drugs. It occurs most commonly in the lower extremities and vertebrae. Here, we present a case of carcinoma prostate, who was referred to our department for 68 Ga-PSMA PET/CT scan, and we incidentally found out PSMA-avid OO involving frontal bone of skull, which is a rare finding. To the best of our knowledge, this is the second case in which high PSMA uptake is found in the OO, suggesting a possible PSMA expression related to osteoblastic activity.

Transient Suppression of Bacterial Populations Associated with Gut Health is Critical in Success of Exclusive Enteral Nutrition for Children with Crohn's Disease.

BACKGROUND AND AIMS: Exclusive enteral nutrition [EEN] is a dietary intervention to induce clinical remission in children with active luminal Crohn's disease [CD]. While changes in the gut microbial communities have been implicated in achieving this remission, a precise understanding of the role of microbial ecology in the restoration of gut homeostasis is lacking. METHODS: Here we reconstructed genomes from the gut metagenomes of 12 paediatric subjects who were sampled before, during and after EEN. We then classified each microbial population into distinct 'phenotypes' or patterns of response based on changes in their relative abundances throughout the therapy on a per-individual basis. RESULTS: Our data show that children achieving clinical remission during therapy were enriched with microbial populations that were either suppressed or that demonstrated a transient bloom as a function of EEN. In contrast, this ecosystem-level response was not observed in cases of EEN failure. Further analysis revealed that populations that were suppressed during EEN were significantly more prevalent in healthy children and adults across the globe compared with those that bloomed ephemerally during the therapy. CONCLUSIONS: These observations taken together suggest that successful outcomes of EEN are marked by a temporary emergence of microbial populations that are rare in healthy individuals, and a concomitant reduction in microbes that are commonly associated with gut homeostasis. Our work is a first attempt to highlight individual-specific, complex environmental factors that influence microbial response in EEN. This model offers a novel, alternative viewpoint to traditional taxonomic strategies used to characterize associations with health and disease states.

Isolated Urinary Bladder Metastasis in Renal Cell Carcinoma Detected on 18F-FDG PET/CT Scan.

ABSTRACT: Renal cell carcinoma (RCC) is an aggressive carcinoma with hematogenous spread commonly to lungs, liver, and bones. However, few cases of isolated urinary bladder metastasis have also been reported. Here we report a case of 63-year-old man, a known case of left RCC (clear cell type), post left nephrectomy, who was on regular clinicoradiological follow-up. He presented with complaints of painless hematuria; on further evaluation, 18F-FDG PET/CT revealed few FDG-avid intramural nodular lesions along the walls of urinary bladder. He underwent TURBT, and the tissue was sent for histopathological examination, which was diagnostic of metastatic RCC.

Primary Renal Well-Differentiated Neuroendocrine Tumors: Analyis of Six Cases from a Tertiary Care Center in North India with Review of Literature.

Well-differentiated renal neuroendocrine tumors are rare tumors. As their biologic behavior is not fully known, there is a need to know more about these cases. We performed a retrospective chart review of all the cases diagnosed with renal neuroendocrine tumors from January 2016 to December 2020 (five years) in order to understand their clinical features, morphological characteristics and outcome. We included six cases with mean age of 46.2 years (4 males) in our study. All patients underwent radical nephrectomy. Histologically all showed tumor disposed in nests and trabeculae and majority of the tumors belonged to well-differentiated neuroendocrine tumor Grade 1 (WHO criteria of gastoroenteropancreatic neuroendocrine neoplasms). Lymph node metastasis was seen in two cases at the time of clinical presentation. All the tumors were diffusely positive for neuroendocrine tumor markers (synaptophysin, chromogranin, NSE, CD56). Follow-up data was available in all cases with an average follow-up of two years and neither has shown evidence of metastasis or relapse till last follow-up. Role of morphological patterns and immunohistochemical markers is highlighted with the importance of including Ki-67 index in grading them to better understand their outcome.

Histopathological spectrum of duodenal polyps in a retrospective ten-year study.

INTRODUCTION AND AIMS:  Duodenal polyps are rare in patients undergoing upper gastrointestinal endoscopy. The present study is an experience of the histopathological spectrum of the duodenal polyps and its correlation with the clinical and endoscopic findings in a tertiary care centre. MATERIALS AND METHODS: The present study is a 10-year retrospective study from the year 2011 to 2020. All the relevant clinical, endoscopic and radiologic findings were retrieved from the hospital medical records. Old histopathology slides were restained, and wherever required, special stains and immunohistochemistry (IHC) were performed. All the cases were reviewed. The present study mainly included descriptive statistics with categorical and continuous variables. RESULTS: Total 81 cases of duodenal polyps were studied in the period of 10 years. The median age was 48 years. Male: female ratio was 2.2:1. The most common presenting system was abdominal pain. We experienced both solitary and multiple polyps. The majority of the duodenal polyps were non-neoplastic, with unremarkable mucosa or inflammatory type. Unlike previous studies the most common site for the hyperplastic polyp in the present study was the first part of the duodenum. Among the neoplastic polyps, adenomatous polyp was the most common type. Contrary to the previous studies, our study showed the first part of the duodenum as the most common site for the sporadic nonampullary adenomatous duodenal polyps. Of the rare entities, we encountered a single case each of lipomatous polyp and gangliocytic paraganglioma. Among the syndromes we encountered two cases of Peutz-Jeghers syndrome and one case of familial adenomatous polyp in our study population.CONCLUSION Duodenal polyps are a rare finding on endoscopic examinations, though most of them are non-neoplastic in nature, vigilant examination under the microcope is required to rule out any neoplastic pathology and identify the risk of malignancy.

Post-CMV Organizing Pneumonia - An Unusual Presentation 10 years after Kidney Transplantation.

A 45-year-old gentleman underwent kidney transplantation in March 2010. He remained apparently healthy for the next 10 years when he developed anorexia and weight loss. Diagnostic workup revealed cytomegalovirus (CMV) pneumonia. While viremia resolved within 3 weeks of initiation of valganciclovir, he developed progressive breathlessness and hypoxia on exertion. Imaging of thorax revealed central peri-bronchovascular consolidation and fine reticulations with peripheral sparing. Computed tomography (CT)-guided percutaneous lung biopsy revealed organizing intra-alveolar exudates, suggestive of organizing pneumonia, with no evidence of active infection on biopsy as well as bronchoalveolar lavage (BAL) cytology. This atypical pattern of central distribution of opacities is not typical of organizing pneumonia where peripheral subpleural distribution is more common. Patient responded dramatically following escalation of steroids, with complete resolution of infiltrates on follow-up imaging.

Obstacles for T-lymphocytes in the tumour microenvironment: Therapeutic challenges, advances and opportunities beyond immune checkpoint.

The tumour microenvironment (TME) imposes a major obstacle to infiltrating T-lymphocytes and suppresses their function. Several immune checkpoint proteins that interfere with ligand/receptor interactions and impede T-cell anti-tumour responses have been identified. Immunotherapies that block immune checkpoints have revolutionized the treatment paradigm for many patients with advanced-stage tumours. However, metabolic constraints and soluble factors that exist within the TME exacerbate the functional exhaustion of tumour-infiltrating T-cells. Here we review these multifactorial constraints and mechanisms - elevated immunosuppressive metabolites and enzymes, nutrient insufficiency, hypoxia, increased acidity, immense amounts of extracellular ATP and adenosine, dysregulated bioenergetic and purinergic signalling, and ionic imbalance - that operate in the TME and collectively suppress T-cell function. We discuss how scientific advances could help overcome the complex TME obstacles for tumour-infiltrating T-lymphocytes, aiming to stimulate further research for developing new therapeutic strategies by harnessing the full potential of the immune system in combating cancer.

The prevalence and clinical characteristics of anti-HMGCR (anti-3-hydroxy-3-methyl-glutaryl-coenzyme A reductase) antibodies in idiopathic inflammatory myopathy: an analysis from the MyoCite registry.

This study aimed to determine the prevalence and clinical characteristics of anti-HMGCR antibodies in idiopathic inflammatory myositis (IIM) at a tertiary care centre in northern India. Data (adult and children) were retrieved from the MyoCite dataset, identifying patients with polymyositis, dermatomyositis, and antibody-negative IIM whilst fulfilling the ACR/EULAR criteria. SLE, sarcoidosis, and systemic sclerosis were included for comparison as disease controls. The baseline clinical profile, laboratory tests, and muscle biopsies were retrieved and analysed. Descriptive statistics and non-parametric statistics were used for comparison. Among 128 IIM (112 adults, 16 children, M:F 1:2.8) of age 37 (24-47) years and 6 (3-17) months disease duration, 4 (3.6%) young adults tested positive for anti-HMGCR antibodies. All children and disease control tested negative for the antibody. Anti-HMGCR + IIM exhibited higher muscle enzymes [AST (367 vs 104 IU/L, p = 0.045), ALT (502 vs 78 IU/L, p = 0.004), and CPK (12,242 vs 699 IU/L, p = 0.001] except lactate dehydrogenase with less frequent systemic features such as fatigue than antibody-negative IIM. One young girl presented with a Limb-girdle muscular dystrophy (LGMD) with chronic pattern. None of the patients exhibited rashes, statin exposure, or cancer, though one had anti-Ro52 and mild disease. Our observations depict a younger population while affirming previous literature, including NM-like presentation, and chronic LGMD-like pattern of weakness in one case. Although a small number of children were included, ours is one of the few paediatric studies that evaluated HMGCR antibodies thus far. Further investigations in a larger Indian cohort are warranted to substantiate our findings.

Diagnostic yield and safety of percutaneous CT-guided biopsy of retroperitoneal lesions and analysis of imaging features.

BACKGROUND: Computed tomography (CT)-guided biopsy is emerging as a preferred method for obtaining tissue samples from retroperitoneal lesions due to clear visualization of needle and vessels. PURPOSE: To assess diagnostic yield and safety of CT-guided biopsy of retroperitoneal lesions and compare CT findings in different disease categories. MATERIAL AND METHODS: This retrospective analytical study included 86 patients with retroperitoneal lesions who underwent CT-guided biopsy from December 2010 to March 2020. All procedures were performed with co-axial technique and multiple cores were obtained and subjected to histopathology. Additional tests like immunohistochemistry or microbiological analysis were done depending on clinical suspicion. Diagnostic yield calculation and comparison of imaging findings was done by one-way ANOVA, chi-square, and Fisher's exact tests. RESULTS: CT-guided biopsy was technically successful in all cases with a diagnostic yield of 91.9%. Minor complications in the form of small hematomas were seen in two patients. Major disease categories on final diagnosis were lymphoma, tuberculosis, and metastases. A variety of malignant and benign soft-tissue neoplasms were also noted less commonly. With help of immunohistochemistry, lymphoma subtype was established in 88.8% of cases. Addition of microbiological tests like the GeneXpert assay helped in the diagnosis of tuberculosis in some cases. A mass-like appearance and vascular encasement was common in metastatic group and lymphoma. CONCLUSION: Percutaneous CT-guided biopsy is a safe method for the sampling of retroperitoneal lesions with high diagnostic yield. Imaging findings are mostly overlapping; however, some features are more common in a particular disease condition.

Interdigitating dendritic cell sarcoma of pleura, diagnostic challenges of a rare disease.

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare subtype of dendritic cell tumour. The solitary lymph node disease is most commonly seen, but may occasionally present extranodally in skin, intestines, soft tissue, liver or spleen. Here we present a case of IDCS in pleura in 53-year-old man, who presented with loss of appetite and chest pain. The initial biopsy was inconclusive. The patient was started on anti-tubercular treatment considering the higher prevalence of the disease in this part of the world. However, the symptoms worsened after 2 months and repeat PET-CT scan revealed extensive FDG avid lesions in the multiple sites in the body. Repeat PET guided biopsy confirmed this rare IDCS neoplasm. Diagnostic challenges of this rare tumour are discussed.

Autoimmune Encephalitis versus Creutzfeldt-Jakob disease in a patient with typical Facio-brachial dystonic seizures: A case report with Diagnostic challenges.

BACKGROUND: Diagnosis of rapidly progressive dementia (RPD) is very challenging. There are many conditions that fall into category of RPD ranging from autoimmune causes to neurodegenerative causes. Autoimmune encephalitis should be readily diagnosed and treated because of its response to immunomodulators. However there is no treatment available for conditions like Creutzfeldt-Jakob disease (CJD). CASE PRESENTATION: Here we present a case of anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis who presented with only typical facio-brachial dystonic seizures at presentation. On follow up, patient had a rapid cognitive decline with development of myoclonic jerks, akinetic mute state and ultimately death. Neuroimaging showed presence of hyperintensities in two cortical regions namely parietal and occipital on fluid-attenuated inversion recovery (FLAIR) sequence. Electroencephalogram showed diffuse slowing with occasional periodic sharp wave complexes. Thus a diagnosis of probable CJD was made. CONCLUSION: Autoimmune encephalitis mimicking CJD or vice versa is not a very commonly encountered phenomenon. This case discusses the clinical overlap of these two conditions and its diagnostic dilemmas. This case presented with typical LGI1 encephalitis and in spite of therapy with immunomodulators had a rapid decline and ultimately turned out to be CJD. This has been rarely described in literature.

18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in the Evaluation of Anorectal Malignant Melanoma: A Case Series.

Melanomas are the malignancies that can affect any anatomic region where melanocytes exist (such as the epidermis, eyes, nasal cavity, and anus). Anorectal melanoma is a rare mucosal melanocytic malignancy, comprising 0.8% of all anorectal malignancies. Here, we report a case series of three patients of anorectal melanoma and role of positron emission tomography/computed tomography in diagnosis and follow-up to evaluate the local recurrence and distant metastases. Of three patients, two presented with rectal bleeding and one with obstruction. One patient had recurrence after 10 months, other after 24 months, and third remained disease free post surgery till 9 months.

Metastatic Thyroid Cartilage Lesion from Prostatic Adenocarcinoma on 68Ga-Prostate-Specific Membrane Antigen Positron Emission Tomography-Computed Tomography Scan: Case Series.

Prostate cancer usually metastasizes to regional lymph nodes and bone. Laryngeal cartilage metastases are very rare and only few cases have been published so far describing thyroid cartilage metastatic lesions from prostate cancer. Here, we describe 5 cases of carcinoma prostate, 3 staging and 2 follow-up, where 68Ga-prostate-specific membrane antigen positron emission tomography/computed tomography (PET/CT) scan revealed multiple skeletal lesions along with thyroid cartilage metastasis. Initially, laryngeal cartilage metastases remain asymptomatic and in later stages patients present with symptoms. These metastatic lesions to thyroid cartilage are rare entities and can often easily be missed on conventional imaging. PET-CT imaging has overcome this diagnostic problem due to its ability to provide for both anatomical and functional imaging.

Etiological Spectrum and Clinical Features in 215 Patients of Crescentic Glomerulonephritis: Is it Different in India?

INTRODUCTION: Crescentic glomerulonephritis (CrGN) characterized by the presence of crescents in most (≥50%) glomeruli on renal histology clinically presents as rapidly progressive renal failure. It can occur due to diverse etiologies with varying course and renal outcomes. We studied the prognostic significance of its classification as pauci-immune, anti-GBM, and immune-complex mediated CrGN. MATERIALS AND METHODS: Renal biopsies diagnosed as CrGN over 9 years were included. Clinical, biochemical, serological, and histological features of various classes of CrGN were correlated with renal outcome. RESULTS: 215 biopsies were diagnosed as CrGN during this period. A majority (63%) were immune-complex mediated while 32% were pauci-immune, followed by anti-GBM disease (5%). 85.5% of pauci-immune CrGN were ANCA associated. The levels of proteinuria and serum creatinine were significantly higher in anti-GBM CrGN as compared to the other two classes. The various histological features including Bowman's capsule rupture, peri-glomerular granulomatous reaction, fibrinoid necrosis, and vasculitis were more common in anti-GBM disease and pauci-immune CrGN. The median renal survival was 6.3, 5.3, 2.1 months in immune-complex mediated, pauci-immune, and anti-GBM CrGN, respectively. CONCLUSION: Immune-complex mediated is the commonest etiology of CrGN in India. Anti-GBM disease has the worst prognosis followed by pauci-immune and immune-complex mediated CrGN. Raised serum creatinine levels (>5mg%) and the degree of glomerulosclerosis at diagnosis were predictors of poor renal survival. High index of suspicion and prompt diagnosis can improve the outcome in these patients.

Rare thyroid Cartilage Metastases Detected on 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan.

Metastatic lesions to thyroid cartilage are very rare and can be often missed on conventional imaging. Anatomical and functional fused positron emission tomography/computed tomography (PET/CT) imaging has higher sensitivity as compared to conventional CT imaging and can thus identify such atypical sites of metastasis. Here, we present four cases of thyroid cartilage metastases detected on 18fluorodeoxyglucose PET/CT scan from carcinoma lung, breast, stomach and urinary bladder.

Isolated Splenic Tuberculosis Masquerading as Disease Progression of Hodgkin's Lymphoma on Interim18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan.

18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) scan is the imaging modality of choice in the management of lymphoma. However, 18F-FDG is a nonspecific tracer for tumoral metabolic activity and infective pathology, thus posing a challenge in accurate response assessment. Here, we present a case of Hodgkin's lymphoma, referred for staging PET/CT scan which showed FDG-avid disease in the mediastinum, and on interim 18F-FDG PET/CT in addition to the mediastinal mass lesion, a FDG-avid lesion was also noted in the spleen suggestive of disease progression. The biopsy report of the lesion was tuberculosis, which was masquerading as disease progression on interim 18F-FDG PET/CT.