The BCOR-rearranged sarcoma involving the lung: Diagnosis with clinical outcome and literature review.

ABSTRACT: BCOR-rearranged sarcomas (BRS) constitute relatively newly described sarcomas, which, within the musculoskeletal sites, usually occur in the bones, followed by soft tissues. Primary BRS involving the visceral organs is very rare, and only a single case is reported in the lung. These tumors share overlapping morphological and immunohistochemical (IHC) features with other neoplasms, such as synovial sarcoma, Ewing sarcoma, as well as carcinosarcoma, the latter especially when occurring in the visceral organs. BCOR immunostaining is useful in triaging a tumor for molecular diagnosis, which constitutes the "essential" diagnostic criterion for these tumors. To report an extremely rare case of a BRS, confirmed by BCOR-rearrangement by fluorescence in situ hybridization (FISH), primarily occurring in the lung, emphasizing the diagnostic approach and management, along with review of literature. An18-year-old boy presented with complaints of left-sided chest pain, along with cough, fever, loss of appetite, and weight. On radio imaging, there was a complete collapse of the left lower lobe of lung with moderate pleural effusion. The biopsy showed a biphasic tumor comprising primitive round cells admixed with spindle cells. Immunohistochemically, the tumor cells were positive for BCOR, TLE1, and p53. FISH showed BCOR gene rearrangement. A diagnosis of primary BRS of lung was offered. The patient had a favorable response to the chemotherapy regime. BRS is an ultra-rare tumor, which rarely involves visceral organs. The lung is an exceptionally rare site, with only single reported case previously. An exact confirmation by molecular testing has treatment-associated implications. A review of similar reported cases is presented herewith.

Role of textural and radiomic analysis parameters in predicting histopathological parameters of the tumor in breast cancer patients.

INTRODUCTION: Texture and radiomic analysis characterizes the tumor's phenotype and evaluates its microenvironment in quantitative terms. This study aims to investigate the role of textural and radiomic analysis parameters in predicting histopathological factors in breast cancer patients. MATERIALS AND METHODS: Two hundred and twelve primary breast cancer patients underwent 18F-FDG PET/computed tomography for staging. The images were processed in a commercially available textural analysis software. ROI was drawn over the primary tumor with a 40% threshold and was processed further to derive textural and radiomic parameters. These parameters were then compared with histopathological factors of tumor. Receiver-operating characteristic analysis was performed with a P-value <0.05 for statistical significance. The significant parameters were subsequently utilized in various machine learning models to assess their predictive accuracy. RESULTS: A retrospective study of 212 primary breast cancer patients was done. Among all the significant parameters, SUVmin, SUVmean, SUVstd, SUVmax, discretized HISTO_Entropy, and gray level co-occurrence matrix_Contrast were found to be significantly associated with ductal carcinoma type. Four parameters (SUVmin, SUVmean, SUVstd, and SUVmax) were significant in differentiating the luminal subtypes of the tumor. Five parameters (SUVmin, SUVmean, SUVstd, SUVmax, and SUV kurtosis) were significant in predicting the grade of the tumor. These parameters showcased robust capabilities in predicting multiple histopathological parameters when tested using machine learning algorithms. CONCLUSION: Though textural analysis could not predict hormonal receptor status, lymphovascular invasion status, perineural invasion status, microcalcification status of tumor, and all the molecular subtypes of the tumor, it could predict the tumor's histologic type, triple-negative subtype, and score of the tumor noninvasively.

Nivolumab and Cabozantinib Immunotherapy Induced Thyroid Dysfunction Detected on 18 F-FDG PET/CT in Renal Cell Carcinoma.

ABSTRACT: Targeted immunotherapy became the most advanced approach for cancer treatment. Programmed death-1 (PD-1) expressed on activated T cells can reverse immune suppression and cause T-cell activation. Nivolumab, a PD-1 immune checkpoint inhibitor antibody that is a fully human immunoglobulin G4, blocks PD-1 and promotes antitumor immunity. Cabozantinib (tyrosine kinase inhibitor) inhibits the tyrosine kinase activity of vascular endothelial growth factor receptors 1, 2, and 3. As a result of enhancing immune response in normal tissues, immune-related adverse events can occur. Thyroid dysfunction is a common form of immune-related adverse event and seen on 18 F-FDG PET/CT scans post therapy.

Splenic Dysfunction in Children With Sickle Cell Disease: A Single Centre Experience From Central India.

OBJECTIVE: To assess the prevalence and predictors of splenic dysfunction in children with sickle cell disease (SCD). METHODS: A cross-sectional study was conducted between June 2019 and December 2020 where children aged 1 to 15 years of age with SCD were screened for splenic dysfunction. Children who were splenectomised, those with other diseases known to affect splenic function like congenital malformations, immunodeficiencies, and chronic diseases like tuberculosis, nephrotic syndrome, diabetes mellitus, chronic liver disease, celiac disease or malignancy were excluded. Splenic size was assessed by clinical examination and ultrasonography. Splenic dysfunction was assessed by Technetium-99m (99mTc) labeled autologous RBCs and by the presence of Howell Jolly bodies in the peripheral smear. Laboratory and clinical predictors of splenic dysfunction were assessed by multiple logistic regression. RESULTS: We evaluated 66 children with SCD with a mean (SD) age of 7.41 (3.3) years. Impaired and absent splenic function as assessed by 99mTc scintigraphy was found in 13 (19.7%), and 3 (4.6%) children, respectively. Howell Jolly bodies in peripheral smear were found in 5 (7.5%) children; 3 of them had abnormal uptake on scintigraphy; all five had splenomegaly. Age > 5 years, > 4 episodes of vaso-occlusive crisis (VOC), > 3 hospitalization events in the past, > 5 blood transfusions, children not receiving hydroxyurea, reticulocyte count > 4%, and HbS > 70% were independent predictors of splenic dysfunction. CONCLUSION: The prevalence of splenic dysfunction in children with SCD in Central India is lower than that reported from the West. The decision to start antibiotic prophylaxis can be individualized in these children.

Acute Airway Obstruction: An Unusual Presentation of Posttransplant Lymphoproliferative Disorder in a Renal Transplant Recipient.

Posttransplant lymphoproliferative disorder is a life-threatening complication after solid-organ transplants. In adults, recipients of heart transplants have the highest risk, whereas renal transplant recipients have the lowest risk among all solid-organ transplants. The most common site for posttransplant lymphoproliferative disorders are gastrointestinal tract followed by the graft itself. Airway involvement in posttransplant lymphoproliferative disorder is rarely encountered. We report a case of a 26-year-old renal allograft recipient who presented to the emergency room with airway obstruction necessitating an emergency tracheostomy. Imaging revealed a left tonsillar mass extending into the nasopharynx and retropharyngeal space causing complete oropharyngeal occlusion. Endoscopic biopsy from nasopharyngeal mass showed a diffuse large B-cell lymphoma and was Ebstein-Barr virus positive. Reduction in immunosuppression and treatment with posttransplant lymphoproliferative disorder-1 risk-stratified approach resulted in complete remission.

Role of 2-(fluorine-18) Fluoro-2-deoxy-D-glucose Positron Emission Tomography/Computed Tomography in Initial Staging and Bone Marrow Involvement Prediction in Patients with Newly Diagnosed Lymphoma in Correlation with Bone Marrow Study.

Background: Lymphoma is a common malignant proliferative disease in which bone marrow infiltration will upstage the disease and thus affect prognosis of the disease. As of now bone marrow biopsy is considered as a reference standard to find out bone marrow involvement in lymphoma. Performing an invasive and painful intervention in all newly diagnosed lymphoma patients is controversial. PET-CT is a non-invasive technique that gives functional information about the cells using the glucose metabolism. It can detect early bone marrow and extra medullary organ involvement which can lead to restaging of the disease. These advantages make PET-CT a valuable adjunct in diagnosis of lymphoma. Aims and Objectives: Our study aims to evaluate the usefulness of 18 F-FDG PET-CT, a non-invasive, semi quantitative whole body imaging technique for detection of early bone marrow and extra medullary organ involvement in lymphoma patients which in turn can obviate the need for bone marrow study (BMS). The primary objective of study is to categorise FDG uptake in bone marrow as diffuse /unifocal /multifocal / no uptake and to correlate pattern of FDG uptake to bone marrow study. Our study also assesses the role of FDG PET/CT in staging of lymphoma. Materials and Methods: Thirty patients with newly diagnosed lymphoma in the age group 18 to 75 years of both sexes within 3 months of diagnosis and who have not been started on any treatment was included in the study. Marrow uptake on FDG PET/CT has been categorized as diffuse, unifocal, multifocal and no uptake. Agreement between bone marrow study and FDG PET/CT has been assessed by reliability analysis using Cohen's kappa. Sensitivity, specificity, PPV, NPV of PET/CT in detecting marrow involvement have been calculated. Results: The sensitivity, specificity, PPV, NPV and accuracy of 18 F-FDG PET-CT in detecting marrow involvement of lymphoma cases are 86.6%, 77.7%, 68.4%, 91.3% and 80.9% respectively. 18 F-FDG PET-CT detected bone marrow involvement in 86.6% (13 out of 15 total positive cases) cases of lymphoma which included both HL and NHL. Reliability analysis using Cohen's kappa is used to test the agreement between bone marrow study and 18F-FDG PET/CT. k value of 0.6 was obtained which showed a moderate agreement between bone marrow study and 18F-FDG PET/CT in marrow assessment. Conclusion: 18F-FDG PET/CT is a highly sensitive imaging modality which can pick up extra-nodal organ and BMI in patients with lymphoma and can upstage the disease and alter treatment strategies. PET-CT cannot completely replace the bone marrow study. However, being an invasive painful procedure, BMB can be avoided in cases with unifocal or multifocal marrow involvement on PET-CT.

Role of Textural Analysis of Pretreatment 18F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in Response Prediction in Esophageal Carcinoma Patients.

Introduction: Positron emission tomography/computed tomography (PET/CT) is routinely used for staging, response assessment, and surveillance in esophageal carcinoma patients. The aim of this study was to investigate whether textural features of pretreatment 18F-fluorodeoxyglucose (18F-FDG) PET/CT images can contribute to prognosis prediction in carcinoma oesophagus patients. Materials and Methods: This is a retrospective study of 30 diagnosed carcinoma esophagus patients. These patients underwent pretreatment 18F-FDG PET/CT for staging. The images were processed in a commercially available textural analysis software. Region of interest was drawn over primary tumor with a 40% threshold and was processed further to derive 92 textural and radiomic parameters. These parameters were then compared between progression group and nonprogression group. The original dataset was subject separately to receiver operating curve analysis. Receiver operating characteristic (ROC) curves were used to identify the cutoff values for textural features with a P < 0.05 for statistical significance. Feature selection was done with principal component analysis. The selected features of each evaluator were subject to 4 machine-learning algorithms. The highest area under the curve (AUC) values was selected for 10 features. Results: A retrospective study of 30 primary carcinoma esophagus patients was done. Patients were followed up after chemo-radiotherapy and they underwent follow-up PET/CT. On the basis of their response, patients were divided into progression group and nonprogression group. Among them, 15 patients showed disease progression and 15 patients were in the nonprogression group. Ten textural analysis parameters turned out to be significant in the prediction of disease progression. Cutoff values were calculated for these parameters according to the ROC curves, GLZLM_long zone emphasis (Gray Level Zone Length Matrix)_long zone emphasis (44.9), GLZLM_low gray level zone emphasis (0.006), GLZLM_short zone low gray level emphasis (0.0032), GLZLM_long zone low gray level emphasis (0.185), GLRLM_long run emphasis (Gray Level Run Length Matrix) (1.31), GLRLM_low gray level run emphasis (0.0058), GLRLM_short run low gray level emphasis (0.005496), GLRLM_long run low gray level emphasis (0.00727), NGLDM_Busyness (Neighborhood Gray Level Difference Matrix) (0.75), and gray level co-occurrence matrix_homogeneity (0.37). Feature selection by principal components analysis and feature classification by the K-nearest neighbor machine-learning model using independent training and test samples yielded the overall highest AUC. Conclusions: Textural analysis parameters could provide prognostic information in carcinoma esophagus patients. Larger multicenter studies are needed for better clinical prognostication of these parameters.

18F-Labeled Fluoro-2-Deoxyglucose Positron Emission Tomography and Computed Tomography Findings in a Case of Atypical Presentation of Adult Cutaneous T-cell Lymphoma.

Adult cutaneous T-cell lymphoma is an uncommon malignancy with poor prognosis and is usually seen in association with human T-cell lymphotropic virus type 1. We present the case of a 25-year-old female who gave a history of extensive whole-body polypoidal cutaneous and bilateral orbital and breast swellings. Biopsy was suggestive of cutaneous T-cell lymphoma and the patient was evaluated with 18F-labeled fluoro-2-deoxyglucose positron emission tomography and computed tomography for initial staging of the disease.

18F Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Findings in a Case of Metastatic Eccrine Porocarcinoma - An Extremely Rare Malignant Adnexal Tumor.

Porocarcinoma is a rare malignant neoplasm of eccrine sweat glands representing 0.005 to 0.1% of all cutaneous tumors. As eccrine porocarcinoma carries a high risk of recurrence and metastases, early diagnosis and management are crucial to lower mortality rate. We present the case of porocarcinoma in a 69-years-old woman who underwent 18F-fluorodeoxyglucose positron emission tomography/computed tomography (PET/CT) for staging the disease. PET/CT showed metabolically active multiple cutaneous lesions and also picked up lymph nodal and distant metastases to lungs and breast accurately. PET/CT is useful for accurate staging of the disease and for treatment planning.

Role of textural analysis parameters derived from FDG PET/CT in differentiating hepatocellular carcinoma and hepatic metastases.

INTRODUCTION: Texture and radiomic analysis characterize the tumor's phenotype and evaluate its microenvironment in quantitative terms. The aim of this study was to investigate the role of textural features of 18F-FDG PET/computed tomography (CT) images in differentiating hepatocellular carcinoma (HCC) and hepatic metastasis in patients with suspected liver tumors. METHODS: This is a retrospective, single-center study of 30 patients who underwent FDG PET/CT for the characterization of liver lesions or for staging a suspected liver tumor. The histological diagnosis of either primary or metastatic tumor was obtained from CT-guided biopsy, ultrasound-guided biopsy, or surgical removal of a liver lesion. The PET/CT images were then processed in commercially available textural analysis software. Region of interest was drawn over the primary tumor with a 40% threshold and was processed further to derive 42 textural and radiomic parameters. These parameters were then compared between HCC group and hepatic metastases group. Receiver-operating characteristic (ROC) curves were used to identify cutoff values for textural features with a P value <0.05 for statistical significance. RESULTS: A retrospective study of 30 patients with suspected liver tumors was done. After undergoing PET/CT, the histological diagnosis of these lesions was confirmed. Among these 30 patients, 15 patients had HCC, and 15 patients had hepatic metastases from various primary sites. Seven textural analysis parameters were significant in differentiating HCC from liver metastasis. Cutoff values were calculated for these parameters according to the ROC curves, standardized uptake value (SUV) Skewness (0.705), SUV Kurtosis (3.65), SUV Excess Kurtosis (0.653), gray-level zone length matrix_long zone emphasis (349.2), gray-level zone length matrix_long zone low gray-level emphasis (1.6), gray-level run length matrix_long run emphasis (1.38) and gray-level co-occurrence matrix_Homogeneity (0.406). CONCLUSION: Textural analysis parameters could successfully differentiate HCC and hepatic metastasis non-invasively. Larger multi-center studies are needed for better clinical prognostication of these parameters.

A unique case of anaplastic thyroid carcinoma comprising of two distinct patterns osteoclastic variant and squamous cell carcinoma in separate lobe imitating collision tumor: A case report.

Anaplastic thyroid carcinoma with two distinct morphologies in two lobes with no intermixing is very rare and not reported earlier. Furthermore, osteoclastic variant of anaplastic thyroid carcinoma as well as primary squamous cell carcinoma without any differentiated components are also very rare with only few reported cases describing their cytological features. Herein, we are reporting a unique and first case of anaplastic thyroid carcinoma comprising of two distinct patterns osteoclastic variant and squamous cell carcinoma in different lobes in a 55-years-old lady with review of literature.

Umbilical nodule - A not always innocuous abdominal finding.

ABSTRACT: Sister Mary Joseph's nodule is an umbilical nodule found in patients with metastatic abdominal cancers. It is quite rare finding and needs a high index of suspicion for diagnosis. Detection of Sister Mary Joseph's nodule should warrant widespread search for abdominal carcinoma and it universally carries quite poor clinical prognosis. Here, we describe the case of a 28-year-old patient who presented with Sister Mary Joseph's Nodule and was found to have metastatic signet ring cell gastric carcinoma on investigation. A brief discussion about this rare condition is also presented.

An Unusual Presentation of Supraglottic Low-Grade B-Cell Non-Hodgkin's Lymphoma with Tracheostomal Myiasis.

60-year-old male patient presented with dysphagia and a change in voice for eight months. It was established after Direct laryngoscopy surgery and biopsy, that it was a low-grade B cell non-Hodgkin lymphoma. The primary lesion is resolved with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone regimen. Four months later, patient presented with a discharge and maggots at the tracheostomy site. Ifosfamide, Etoposide, Carboplatin was started after a secondary recurrence of disease progression. Hereby we infer this is an unusual case presentation, myiasis with lymphoma recurrence and tough exacting to the otolaryngologist as there are more chances of misdiagnosing as squamous cell carcinoma..

Role of Ga-68 DOTANOC Positron Emission Tomography/ Computed Tomography Scan in Clinical Management of Patients with Neuroendocrine Tumors and its Correlation with Conventional Imaging- Experience in a Tertiary Care Center in India.

Purpose of Study: Aim of the study was to evaluate the role of 68Gallium-DOTANOC positron emission tomography/computed tomography (68Ga-DOTANOC PET/CT), a pan somatostatin receptor (SSTR) analog in the clinical management of patients with neuroendocrine tumors (NETs) and its correlation with conventional imaging. Materials and Methods: We retrospectively evaluated 69 patients of known/suspected NETs who underwent 68Ga-DOTANOC PET/CT scan for tumor localization (n = 15), stage modification (primary staging, n = 26 and restaging, n = 25) and therapy monitoring (n = 3). We also compared PET scan with conventional imaging as reference standard and evaluated the impact of PET/CT in the clinical management of patients. Results: The concordant findings on 68Ga-DOTANOC PET/CT and conventional imaging seen in 33 and discordant in 36 patients. Among discordant group, disease was upstaged in 32 patients; down staged in 3 patients; no stage change in one patient. PET/CT localized primary tumor in 4 patients. Among patients with raised tumor markers (39/69), PET was positive in 29 and negative in 10 patients. Patients were followed for mean duration of 27 months to assess management. We found strong agreement between positive PET and raised tumor markers (Kappa value = 0.8). Sensitivity and specificity of PET/CT for primary tumor localization, stage modification, and therapy monitoring was >90% (P < 0.05). Conclusions: Study shows that DOTANOC, a broad spectrum SSTRs binding peptide labeled with Ga-68 in PET/CT scan is an excellent modality in the management of NETs patients.

18F-Labeled Fluoro-2-Deoxyglucose Positron Emission Tomography and Computed Tomography in a Large Pulmonary Sclerosing Pneumocytoma with Contralateral Lung Metastasis.

Pulmonary sclerosing pneumocytoma is an exceedingly rare neoplasm of the lung. These tumors are usually slow growing with a benign disease course but can easily be mistaken for carcinoid tumors or adenocarcinoma in cytology or histopathology specimens. Rare occurrences of metastases have been reported in the literature making 18F-labeled fluoro-2-deoxyglucose positron emission tomography and computed tomography useful for the evaluation of these tumors.

Primary Biliary Tuberculosis Masquerading Cholangiocarcinoma in 18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography.

Hepatobiliary involvement is a less common manifestation of abdominal tuberculosis. We present the case of a 42-year-old female who presented with fever, abdominal pain, and jaundice of 2 months duration. 18F-fluorodeoxyglucose positron emission tomography/computed tomography done for disease evaluation suggested the likely possibility of cholangiocarcinoma but excision biopsy from periportal lymph node later confirmed a granulomatous etiology and she was successfully treated with antitubercular therapy.

Orbital Plasmablastic Lymphoma: A Rare Variant of Diffuse Large B-Cell Lymphoma Staged and Followed Up by 18F-FDG PET/CT.

ABSTRACT: Plasmablastic lymphoma (PBL) is a rare and aggressive variant of diffuse large B-cell lymphoma which is associated with HIV infection. Recently, it has also been reported in immunocompetent and solid organ transplant patients. PBL commonly presents in extranodal regions such as oral cavity, digestive tract, and skin. Orbital involvement by PBL is extremely rare with only few reports in the literature. We present a case of PBL involving the bilateral orbits in an immunocompetent patient with 1-year follow-up on 18F-FDG PET/CT scan.

Incidental lung findings in a COVID-19 patient on 18F-FDG PET/CT done for preoperative evaluation of Marjolin's ulcer-first report from Chhattisgarh.

The aim of this case is to illustrate the18F-fluorodeoxyglucose (FDG) positron emission tomography-computed tomography findings of a patient who was admitted in AIIMS, Raipur, for the preoperative evaluation of Marjolin ulcer and was later diagnosed with COVID-19 infection. Apart from the primary lesion in the right foot and pelvic lymph nodes, the scan revealed mild FDG-avid basal ground-glass opacities in bilateral lung fields with mediastinal and hilar lymph nodal involvement, in an otherwise asymptomatic patient.

Pulmonary sclerosing pneumocytoma masquerading adenocarcinoma with co-existing BRAF V600E and PTEN mutation.

We report a case of a massive primary sclerosing pneumocytoma (PSP) involving the right lower lobe adhering esophagus with small synchronous PSP on the superior segment of the left lower lobe with concurrent mutation for B-RAF proto-oncogene, serine/threonine kinase (BRAF V600E), and phosphatase and tensin homolog (PTEN) gene in a young female. She underwent right lower lobectomy and mediastinal lymph node dissection under single lung ventilation with tumor-free margins on diagnosis-based findings of preoperative computed tomography-guided biopsy and positron emission tomography. Histopathology was suggestive of PSP-papillary variant with concurrent mutation of BRAF V600E and PTEN genes. Post-operative follow-up at four weeks was uneventful. She has to undergo wedge resection for the contralateral disease after six weeks following recovery from the first surgery.

Membranoproliferative glomerulonephritis: A rare presentation of occult filariasis.

Parasitic infections do not usually present with rapidly progressive renal failure but can provoke glomerular lesions which are mostly proliferative. In filarial infection, glomerular involvement is usually mild and transient, and presentation with renal failure is rare. We report occult filariasis presenting as rapidly progressive renal failure due to immune-complex mediated membranoproliferative glomerulonephritis. Our patient responded to treatment with diethylcarbamazine and a short course of steroid. This case highlights the importance of thorough workup to identify the cause and consideration of filariasis in an endemic area.