South African flag sign to a giant coronary artery aneurysm.

Background: Coronary arteritis leading to aneurysm is one of the unusual presentations of IgG4-related disease. Acute myocardial infarction as a complication of IgG4-related giant coronary artery aneurysm is even rarer. Case summary: We describe the case of a 56-year-old gentleman who presented to our institute with Canadian Cardiovascular Society (CCS) class III angina. His symptoms were persistent even with high-dose antianginal medications. He had an acute coronary syndrome two weeks back for which he was treated conservatively in a peripheral health centre. His 12-lead electrocardiogram at the time of the event was suggestive of high lateral ST-segment elevation myocardial infarction (South African flag sign). His transthoracic echocardiography showed mild left ventricular dysfunction and a large echogenic mass lateral to the left ventricle. Coronary angiography followed by cardiac computed tomography revealed a giant pseudoaneurysm of the proximal and mid-left anterior descending coronary artery. FDG-PET scan showed significant metabolic activity in the aneurysm wall and mediastinal lymph nodes suggesting active inflammation. IgG4-related coronary arteritis was suspected, and the patient underwent aneurysmectomy and coronary artery bypass (CABG) surgery. The histopathology of the resected segment showed diffuse IgG4-secreting plasma cells confirming the diagnosis. Discussion: Atherosclerosis is the most common cause of coronary aneurysms in adults. However, cardiologists should be aware of atypical causes like IgG4-related disease that can even present with acute coronary syndrome. Although multimodality imaging is beneficial during early evaluation, histopathological analysis is the cornerstone for the diagnosis of IgG4-related disease. The management involves both immunosuppressive medication and endovascular or surgical repair.

Trichilemmal carcinoma metastatic to parotid gland: First report of fine needle aspiration cytology features.

Trichilemmal carcinoma is a rare malignant cutaneous adnexal neoplasm arising from the outer root sheath of the hair follicle. Majority of cases occur on sun-exposed sites such as the face, scalp and neck, making them easily amenable to being biopsied and subjected to histological examination for definitive diagnosis. Thus, cytological features of trichilemmal carcinoma have not been described till date. Trichilemmal carcinoma is a low-grade malignancy, albeit with potential to metastasize to regional lymph nodes and distant sites. We report the case of trichilemmal carcinoma of scalp that metastasized to cervical lymph nodes and parotid gland and underwent fine-needle aspiration cytology (FNAC) from the parotid lesion. The aspirate showed tightly cohesive cell clusters with sharp borders. Tumour cells ranged from basaloid with scant cytoplasm to those with abundant cytoplasm. Nuclei were vesicular, with inconspicuous to prominent nuclei. Intercellular bridges, masses of keratin, and fragments of desmoplastic stroma were present, closely recapitulating histological features of trichilemmal carcinoma, which enabled diagnosis as metastasis. Cell block showed similar tumour fragments with evidence of differentiation towards outer root sheath. FNAC is the first-line investigation to obtain a tissue diagnosis of masses in the head and neck region. Although rarely encountered, the lack of knowledge of cytological features of trichilemmal carcinoma may hamper its FNAC diagnosis at metastatic sites. When intraparotid metastases occur, they may be mistaken as primary salivary gland carcinoma. Thus, awareness of the cytological features of this tumour must be raised among cytopathologists to enable accurate diagnosis and further management.

A large cardiac tuberculoma masquerading a malignant mass in a 15-year-old boy.

We hereby present a case of large cardiac tuberculoma which was thought to be a malignant mass on cardiac magnetic resonance imaging (MRI). The present case highlights that high index of suspicion is necessary to diagnose this rare entity especially in tuberculosis-endemic areas, or in those who have relevant past history of this condition.

Cutaneous Adnexal Tumours: A Study of 395 Cases from a Tertiary Care Hospital.

Background: Cutaneous adnexal tumours (CATs) are one of the commonest clinical presentations in dermatology outpatients. They constitute a subset of skin tumours with a common clinical presentation and variable histological findings. Almost all of them clinically present as a subcutaneous nodule. Depending upon the site and distribution, the clinician can suspect the diagnosis. However, histopathological examination is the gold standard for the definitive diagnosis and proper subtyping of CATs. Aims: The present study is conducted to see the overall spectrum, incidence and distribution of CATs in a large cohort at the tertiary care centre with their clinical profile. Methods: This was a retrospective study in which all the CATs diagnosed over a period of 5 years (2015 to 2019) in a tertiary care hospital were studied. Clinical data were recorded from the histopathology requisition forms. Results: Three hundred and ninety-five cases of CATs were retrieved. Approximately 90% of cases were benign and 10% were malignant. The age of presentation ranged from 8 months to 81 years with male preponderance in all the histological subtypes. The most common site was the head and neck followed by the extremities. Morphologically, the maximum cases showed a differentiation towards sweat glands (44%), followed by sebaceous (29%), follicular (26.5%) and apocrine differentiation (5.3%). Malignant tumours were common in the elderly age group with sebaceous carcinoma being the commonest. Conclusion: CATs comprise of a wide spectrum of tumours occurring in all age groups. Malignant CATs are rare and common in older age groups. Histopathological examination is the gold standard in distinguishing between the different subtypes.

A study on the morbid histopathological changes in COVID-19 patients with or without comorbidities using minimally invasive tissue sampling.

COVID-19 causes morbid pathological changes in different organs including lungs, kidneys, liver, and so on, especially in those who succumb. Though clinical outcomes in those with comorbidities are known to be different from those without-not much is known about the differences at the histopathological level. To compare the morbid histopathological changes in COVID-19 patients between those who were immunocompromised (Gr 1), had a malignancy (Gr 2), or had cardiometabolic conditions (hypertension, diabetes, or coronary artery disease) (Gr 3), postmortem tissue sampling (minimally invasive tissue sampling [MITS]) was done from the lungs, kidney, heart, and liver using a biopsy gun within 2 hours of death. Routine (hematoxylin and eosin) and special staining (acid fast bacilli, silver methanamine, periodic acid schiff) was done besides immunohistochemistry. A total of 100 patients underwent MITS and data of 92 patients were included (immunocompromised: 27, malignancy: 18, cardiometabolic conditions: 71). In lung histopathology, capillary congestion was more in those with malignancy, while others like diffuse alveolar damage, microthrombi, pneumocyte hyperplasia, and so on, were equally distributed. In liver histopathology, architectural distortion was significantly different in immunocompromised; while steatosis, portal inflammation, Kupffer cell hypertrophy, and confluent necrosis were equally distributed. There was a trend towards higher acute tubular injury in those with cardiometabolic conditions as compared to the other groups. No significant histopathological difference in the heart was discerned. Certain histopathological features were markedly different in different groups (Gr 1, 2, and 3) of COVID-19 patients with fatal outcomes.

Successful surgical correction of recurrent primary chylopericardium due to mediastinal lymphangiomyoma by total pericardiectomy and diaphragmatic fenestration.

We present a rare case of hemodynamically significant recurrent primary chylopericardium due to a rare lymphatic malformation, mediastinal lymphangiomyoma, treated by complete surgical resection with total pericardiectomy. Recurrent chylopericardium or chylothorax, a common complication following the primary intervention, was successfully treated by diaphragmatic fenestration technique which is as good as thoracic duct ligation.

A rare pediatric case of pedunculated cardiac hemangioma of the left ventricle.

A 12-year-old male child with atypical chest pain and fatigue since past 3 months underwent cardiac magnetic resonance (CMR) imaging for suspicious globular mobile mass detected in the left ventricle on a transthoracic echocardiography. CMR revealed peripheral vascular blush on the first pass perfusion images with centripetal progressive intensely bright enhancement on late gadolinium-enhanced images. Further confirmation and histological diagnosis of the tumor was made through transaortic aortic resection of the mass.

Contrast enhanced ultrasound for characterization of suspected soft tissue vascular anomalies.

BACKGROUND: Evaluation of perfusion characteristics is the key to characterize vascular anomalies. Contrast-enhanced ultrasound (CEUS) is a radiation-free modality to evaluate this in real-time and can be coupled along with Doppler-ultrasound (US). OBJECTIVE: To evaluate the utility of CEUS in characterization of suspected soft-tissue vascular anomalies and compare its diagnostic accuracy with Doppler-US. MATERIALS AND METHODS: This was an ethically-approved cross-sectional study, done from November 2017- November 2019, involving 93 patients (55 M/38F; mean age 23.6 ± 11.9 years) in development cohort and 128 (66 M/62F; mean age 21.4 ± 11.1 years) in validation cohort. Patients suspected to have soft-tissue vascular anomalies on clinical evaluation and US were included. Clinical features, US, Doppler, subjective and quantitative CEUS features (from time-intensity curves) were evaluated. Composite gold standard employing MRI, phlebogram, DSA or biopsy was used to make the final diagnosis. The CEUS features found to be significantly different in the development cohort were prospectively validated in the validation cohort. P-value < 0.05 was considered significant, ROC curves were drawn and threshold values obtained for the various quantitative parameters. A prospective diagnosis was suggested in the validation group along with a degree of diagnostic confidence and accuracy was calculated. RESULTS: The spectrum of lesions included 77 venous malformations, 46 fibro-adipose vascular anomalies, 32 vascular tumors, 20 arteriovenous malformations and 20 lymphatic malformations. All lesions were found to have distinctive temporal and morphological subjective enhancement patterns. Quantitative parameters like rise-time, mean-transit-time, time-to-peak, peak-enhancement showed significant differences between the various groups (P value < 0.05). Addition of CEUS not only increased the diagnostic accuracy of Doppler-US from 76.5% (91/119) to 88.2% (105/119), but also the degree of diagnostic confidence in characterization of soft-tissue vascular anomalies. None of the patients showed any contrast-related adverse effects. CONCLUSION: Contrast-enhanced ultrasound is useful to characterize different types of soft-tissue vascular anomalies and increases the diagnostic accuracy and confidence over Doppler-ultrasound.

Detection of cardiac allograft vasculopathy on dual source computed tomography in heart transplant recipients: comparison with invasive coronary angiography.

OBJECTIVE: We aimed to evaluate the diagnostic accuracy (DA) of dual-source CT coronary angiography (DSCTCA) against invasive coronary angiography (ICA) in assessing stenotic cardiac allograft vasculopathy (CAV) in heart transplant (HTX) recipients. METHODS: Consecutive HTX recipients(n = 38) on annual surveillance, underwent DSCTCA prior to ICA on a 192-detector 384-slice DSCT scanner. Cases were classified as no CAV (no stenosis), any CAV (any degree of stenosis) or significant CAV (>50% stenosis). RESULTS: Mean age was 33.66 ± 11.45 years (M:F = 27:11, median time from HTX-23.5 months). Prevalence of any CAV on DSCTCA and ICA was 44.7%(n = 17) and 39.5%(n = 15), respectively and that of significant CAV was 21.1%(n = 8) and 15.8%(n = 6), respectively. 557 (96.7%) segments were interpretable on DSCTCA. Mean radiation dose was 4.24 ± 2.15 mSv. At patient-level, the sensitivity, specificity, positive-predictive value, negative-predictive value (NPV), and DA of DSCTCA for detection of any CAV and significant CAV were 100%, 91.3%, 88.2%, 100%, 94.73% and 100%, 94%, 75%, 100%, 95% respectively. The same on segment-based analysis were 96%, 97.6%, 80%, 99.6%, 97.5% and 100%, 99.6%,86.7%,100%, 99.6%, respectively. There was excellent agreement between the two modalities for detection of any CAV and significant CAV [κ = 0.892 and 0.826 (patient-level), 0.859 and 0.927 (segment-level)]. CAC score correlated significantly with the presence of any CAV on both modalities. A diagnosis of rejection on biopsy did not correlate with any/significant CAV on DSCTCA or ICA. CONCLUSION: High sensitivity and NPV of DSCTCA in the evaluation of stenotic CAV suggests that it can be an accurate and non-invasive alternative to ICA for routine surveillance of HTX recipients. ADVANCES IN KNOWLEDGE: DSCTCA detects the stenotic CAV non-invasively in transplant recipients with high sensitivity, specificity and NPV when compared with catheter coronary angiography, at lower radiation doses. There is excellent agreement between CT angiography and catheter coronary angiography for detection of any CAV and significant CAV. A diagnosis of rejection on biopsy does not correlate with any/significant CAV on CT angiography or catheter angiography.

Patient profile and comparison of three diagnostic criteria for cardiac sarcoidosis in a tuberculosis endemic population.

BACKGROUND: Cardiac sarcoidosis (CS) is an underdiagnosed and life-threatening condition. Histopathological diagnosis is difficult due to the risks and variable diagnostic yield of endomyocardial biopsy. OBJECTIVES: To study the clinical profile and compare the diagnostic criteria of CS in a cohort of sarcoidosis. METHODS: A retrospective review of the Sarcoidosis database (375 patients) was performed to identify patients with CS. Demographic and clinical details were retrieved. We applied the available diagnostic criteria for the diagnosis of CS: The World Association of Sarcoidosis and Other Granulomatous Diseases (WASOG), Heart Rhythm Society (HRS), and Japanese Ministry of Health and Welfare (JMHW) criteria. RESULTS: Out of the 375 patients, 15 (4%) were identified with CS. The median age was 41 years, and 53% were female. The most common symptoms were breathlessness, palpitation, and fatigue in 80%, 53.3%, and 46.6% of patients, respectively. Tuberculin positivity (≥ 10mm induration) was seen in 26.6%. 80% and 53.3% of the patients had abnormal ECG and 2D echocardiography findings, respectively. Six patients had a history of Ventricular tachycardia (40%). LV Ejection fraction was reduced in 12 subjects (80%). Cardiac-MRI showed late gadolinium enhancement in 53.3%. A definitive histopathological diagnosis for sarcoidosis was established in 86.6% (13/15) patients. Of the 15, all satisfied JMHW criteria and WASOG criteria (12 (80%) at least probable category, 3 (20%) possible CS), and 13 (86.6%) met HRS criteria for a diagnosis of CS. CONCLUSION: In a cohort of 375 patients with sarcoidosis in a tuberculosis endemic setting, 4% were diagnosed with cardiac sarcoidosis. Histopathological diagnosis may be obtained by sampling from extracardiac sites. JMHW and WASOG criteria perform equally well in TB endemic settings.

Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features.

Kimura disease is a rare chronic inflammatory disorder mostly reported in males in second to third decades from south-east Asia. Head and neck is the most commonly involved region. The usual presentation is painless facial swelling with salivary gland involvement and regional lymphadenopathy. The diagnosis can be suspected on imaging but needs to be proven on histopathology. We describe three histopathology proven cases of Kimura disease and emphasize the specific clinic-radiological findings which can help in early identification of the condition and its differentiation from other diseases with similar presentation. Gray scale Ultrasound, contrast-enhanced ultrasound, and contrast-enhanced Magnetic Resonance Imaging (CE-MRI) were done in all patients and Computed Tomography (CT) was done in one. The salient imaging features were ill-defined solid mass in the subcutaneous location of the facial region which was heteroechoic on Gray scale Ultrasound, heterogeneously hyperintense on T2W MRI and without much diffusion restriction. There was involvement of salivary glands with ipsilateral cervical adenopathy. The lesion showed avid contrast enhancement on contrast-enhanced ultrasound and CE-MRI. We also present a systematic literature review of clinico-radiological features in Kimura disease in the head and neck. The articles with histopathologically proven cases and satisfactory description of imaging findings were included and we have compared the findings in our cases with published literature. In addition, we have included examples of radiological mimics of this disease. Certain imaging findings are highly suggestive and can aid in early diagnosis of Kimura disease which helps in early initiation of treatment with better patient prognosis.

Predicting Histology of Tracheobronchial Neoplasms: A CT Based Differentiation Model.

BACKGROUND: Tracheobronchial (TB) tumors follow same pathological classification as lung neoplasms; however, some entities are known to favor airways. Distinction of pathological types is necessary for suggesting appropriate management strategy. PURPOSE: To evaluate utility of multidetector CT (MDCT) in differentiation of primary TB tumors; and assess validity of a scoring system based on imaging biomarkers to differentiate tumor types. METHODS: MDCT features of 45 patients were analyzed for location, shape, calcification, attenuation, parenchymal changes, bronchoceles, extraluminal extension, lymphadenopathy, metastases. The two largest groups were compared with each other and remaining entities using Chi square tests. Six-point scoring system combining the differentiating features was devised and receiver operating characteristic curve analysis performed. RESULTS: The most frequent type was neuroendocrine tumors (NET) (51.1%), followed by salivary gland tumors (SGT) (20%); including adenoid cystic carcinoma (ACC) (13.3%) and mucoepidermoid carcinoma (MEC) (6.7%). Comparing NETs with other entities as a whole, and independently with SGTs, significant difference was found among location (p = 0.05 and 0.001 respectively), shape (p < 0.001), calcification (p = 0.038 and 0.041 respectively), attenuation (p = 0.001 and 0.019 respectively), bronchoceles (p = 0.013 and 0.010 respectively). Significant difference was found among ACC and MEC in location (p = 0.01) and morphology (p < 0.001). On receiver operating characteristic (ROC) curve analysis of the score, areas under curve for NET, SGT and ACC were 0.913, 0.872 and 0.962 respectively. Suggested cut-off values were >3.5 for carcinoid (sensitivity 70%, specificity 91%), <2.5 for SGT (sensitivity 78%, specificity 75%), <1.5 for ACC (sensitivity 83%, specificity 92%). CONCLUSION: Use of a scoring system enables maximum diagnostic accuracy in MDCT differentiation of TB tumors.