Pediatric Melanoma Arising in a Background of Melanocytic Nevi.

Malignant melanoma is an aggressive neoplasm primarily involving the skin. They may arise de novo or from a premalignant melanocytic lesion. Melanomas are primarily known to occur in adults. Pediatric melanomas (PM) are rare and predominantly occur de novo following ultraviolet deoxyribonucleic acid (DNA) damage. They may also be associated with the presence of congenital melanocytic nevi. We report a rare case of a 6-year-old child with multiple melanocytic nevi subsequently diagnosed with melanoma of the back and metastatic disease.

Rectal Bleed in a Child With Abernathy Malformation- Endovascular Management.

Abernathy malformations are congenital extrahepatic porto-systemic shunts which allow splanchnic circulation to bypass the metabolic screen of the liver and drain directly into the systemic circulation. The resulting metabolic abnormalities have a multitude of implications ranging from hyperammonaemia, hepatic encephalopathy, to pulmonary hypoxemia. The shunt also causes anatomical implications in the form of varices. Interventional radiology plays the central role in this era of minimal invasive surgeries from establishing diagnosis to therapeutic interventional management. The holistic approach provided through interventional radiology reduces intraprocedural time as well as hospital stay. We describe a very rare case of peripheral congenital porto-systemic shunt communicating Inferior mesenteric vein and internal iliac vein with rectal bleed with complete management at the department of interventional radiology.

Renal Thrombotic Microangiopathy: A Complication of Paediatric Systemic Lupus Erythematosus That Requires Attention.

Systemic lupus erythematosus (SLE) is a multi-system disorder with a variety of clinical presentations. A wide range of renal vascular lesions (VL) is described predominantly in adult patients. The exact prevalence of renal VL in the pediatric SLE (pSLE) population is yet to be determined. A 10-year-old female patient with lupus nephritis (LN) presented with deteriorating kidney function. An exhaustive array of clinical-biochemical and pathological evaluations resulted in a diagnosis of class IV LN with thrombotic microangiopathy (TMA) associated with malignant hypertension and hypocomplementemia. Renal VL is overlooked or underreported in SLE patients, as it is neither accorded much importance in the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification nor in the activity and chronicity scoring. The TMA lesions in LN patients can be managed following the recently devised PLASMIC score; hence, reporting such VL has therapeutic implications.

Idiopathic Chylous Pericardial Effusion Due to Lymphatic Malformation of Mediastinum.

Chylopericardium can be due to a variety of secondary causes like trauma, radiation, tumors, following cardiac surgery, etc., or may be idiopathic due to abnormal lymphatic system and mediastinal lymphangiectasia, which is a rare entity. Here, we present a case of a 34-year-old previously healthy male presenting with idiopathic chylopericardium. 2D echocardiography revealed massive pericardial effusion without features of cardiac tamponade. Following pericardiocentesis, a CT scan of the thorax and MR lymphangiogram were done to arrive at a diagnosis of idiopathic chylopericardium. In addition to medical management, surgical treatment included partial pericardiectomy and sclerotherapy of the mediastinal lymphatic sac. The patient had an uneventful post-operative period.

Clinical Profile of Pediatric Tuberculosis in a Tertiary Hospital in Northeast India: A Retrospective Analysis.

Context Tuberculosis (TB) is India's major public health problem. The profile of childhood TB in the northeast region of India is still limited. Aim To analyze the clinical, radiological, and bacteriological profiles of children with TB at a tertiary health care facility. Materials and methods A three years retrospective descriptive analysis of children admitted to a tertiary centre with TB before the introduction of cartridge-based nucleic acid amplification test (CBNAAT) for testing. Children below 18 years who were admitted from 2012 to 2014 and were diagnosed with TB were included. Relevant data were extracted in a predesigned format and entered into a Microsoft Excel sheet. Descriptive statistic was used for analysis. The results of variables are given in proportions and means and a Chi-square test was done for the test of significance using Epi-info tools. The study was done after getting ethical approval from the institute. Results A total of 150 children were included in the analysis with a Male: Female ratio of 1.1:1. A majority of the cases were under five years (n=46) and 11 to 15 years old (n=45) with a mean age of 9.3 ± 4.4 years. Fever was a common presentation (70%). Disseminated TB was seen in 31.3%, isolated central nervous system (CNS) TB was found in 30.6%, and all CNS TB with dissemination was found in 46 cases (40.7%) making extra-pulmonary TB a common finding in our study (83.3%). Isolated pulmonary TB was seen in 16.7% and total pulmonary cases along with dissemination was seen in 60 cases (40%). A bacteriological diagnosis was made in 23%. Overall mortality was 9.3%, out of which mortality in CNS TB was 13% with a p-value of 0.004 as compared to mortality other than CNS TB which was significant and mortality in under-five years was significant with a p-value of 0.001. Conclusions Pulmonary and extra-pulmonary were both causes of admission in the pediatric age group. We found that extra-pulmonary TB was the most common cause of admission in children, with CNS manifestation and disseminated TB, being the most common presentations and significant mortality was seen in under-five years and in children diagnosed with CNS TB.

High-grade transformation of adenoid cystic carcinoma of parotid gland with isolated intratemporal facial nerve involvement: a case report and literature review.

BACKGROUND: High-grade transformation Adenoid cystic carcinoma (HGT-AdCC) of the parotid gland is a rare transformation noted in slow growing low grade AdCC. Perineural invasion and spread is an important feature of this tumor. Temporal bone involvement is rare. A total of only 10 cases of HGT-AdCC in parotid gland has been reported in literature so far predominantly in the elderly with peak incidence in 5th-6th decade. CASE PRESENTATION: We present a young lady of HGT-AdCC of right parotid gland with temporal bone involvement in the form of isolated perineural invasion (PNI) of facial nerve till the tympanic segment. She underwent right radical parotidectomy with modified radical neck dissection with modified lateral temporal bone resection and pectoralis major myocutaneous flap reconstruction. Histopathological examination revealed both low- and high-grade areas. Sections from facial nerve showed tumor invasion. CONCLUSION: The radiological features of isolated perineural spread in intratympanic part of facial nerve can be easily missed if not specifically looked for. Every attempt should be made preoperatively and intraoperatively to determine the complete extent of the tumor for adequate disease clearance. A combined clinico-radiological approach aided by histopathology examination helps in early detection of this carcinoma and in better patient management.

Rasmussen's aneurysm: A rare cause of haemoptysis.

Rasmussen's aneurysm is a rare and life-threatening cause of recurrent hemoptysis in post tuberculosis patients. Appropriate and early treatment can prevent complications like severe life-threatening hemoptysis due to aneurysm rupture.

Complete persistent remission of rheumatoid arthritis after COVID-19 infection - A rare case and literature review.

A 55-year-old man was admitted to a hospital in Northeast India with fever, cough and breathlessness and was diagnosed with severe COVID-19 pneumonia. He was a known case of seropositive, erosive rheumatoid arthritis (RA) and was taking disease-modifying anti-rheumatic drugs (DMARDs). The patient was treated with remdesivir, heparin and a short course of corticosteroids for COVID-19 pneumonia. With the improvement of COVID-19 pneumonia, the patient also noticed a marked improvement in his joint symptoms despite not taking any DMARDs for RA. The temporal relationship between the time of disappearance of all signs and symptoms of RA within a few days after COVID-19 pneumonia and maintenance of RA remission for over one year of follow up to date suggests that COVID-19 likely caused the remission of RA. This case highlights the need for larger studies to understand the COVID-19 effects on RA remission and their potential link if any. However, the evidence of worse outcome with COVID-19 in immunosuppression which is common in RA cannot be overlooked.

Diffusion tensor imaging analysis of rheumatoid arthritis patients with neuropsychiatric features to determine the alteration of white matter integrity due to vascular events.

INTRODUCTION: The pathophysiology of neuropsychiatric manifestations in rheumatoid arthritis is not well known. The magnetic resonance imaging of the brain in rheumatoid arthritis demonstrates non-specific findings, and in the majority of cases, magnetic resonance imaging fails to detect an abnormality, even in rheumatoid arthritis patients with neuropsychiatric manifestations. Therefore, we aimed to assess microstructural integrity changes of white matter in patients with rheumatoid arthritis by using different diffusion tensor imaging parameters. METHODS: Eighteen rheumatoid arthritis patients (10 with neuropsychiatric symptoms and 8 without any neuropsychiatric symptoms) and 14 controls were included. The volume of the T2 hyperintense lesions was assessed. Different diffusion tensor imaging parameters such as fractional anisotropy, apparent diffusion coefficient, trace, axial diffusivity, and radial diffusivity were obtained from six different regions of white matter. Inter group significant difference was determined by one-way analysis of variance followed by Tukey's post hoc test. The accuracy of diffusion tensor imaging matrices was evaluated from the receiver operating characteristic curve. RESULTS: No significant difference in lesions' volume was detected between rheumatoid arthritis patients with or without neuropsychiatric symptoms. There was an increased apparent diffusion coefficient and radial diffusivity (p < 0.05) as well as decreased fractional anisotropy and axial diffusivity (p < 0.5) in rheumatoid arthritis patients with neuropsychiatric symptoms compared to controls. Moreover, the apparent diffusion coefficient (p < .05) was increased in both positive and negative MRI of patients with neuropsychiatric features compared to the control group. The sensitivity and specificity of the apparent diffusion coefficient parameters was 73% and 72%, respectively. CONCLUSIONS: The various anisotropic metrics were altered in rheumatoid arthritis patients with neuropsychiatric symptoms by using diffusion tensor imaging analysis, representing that central nervous system vasculitis leads to tissue hypoxia resulting in vasogenic edema. This may lead to axonal and myelin degeneration of white matter fibers and neuronal cell disruption. Key Points • Our study confirms that neurovascular events are not uncommon in RA patients with NP features. Diffusion tensor imaging (DTI) is superior to conventional MRI scan for RA patients with NP features because it distinguishes between gray and white matter structures. • RA patients with NP features are more likely to have microstructural changes detected by DTI than by DWI, and it can provide comprehensive anatomical layouts describing regional disparities in neurodegeneration. • DTI's quantitative association of NP symptoms in a large patient cohort is an important study scope.

A Modified Manual Compression Technique for the Treatment of Traumatic Pseudoaneurysm of Superficial Temporal Artery.

Traumatic Pseudoaneurysm of Superficial temporal artery (STA) is an uncommon entity. Surgical management is the treatment of choice, and endovascular management is also equally feasible particularly for the proximal STA aneurysm. The manual compression for the treatment of STA aneurysm is also described, but it has a high chance of failure. We report a case of traumatic pseudoaneurysm of STA in a pregnant female, successfully treated with a modified manual compression technique.

Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma.

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

Papillary cystadenocarcinoma of the parotid gland: a rare sub-variant of salivary gland adenocarcinoma

Papillary cystadenocarcinoma of the salivary gland is a very rare malignant neoplasm accounting for only 2% of all salivary gland lesions. In 1991 it was first included as a separate entity in the World Health Organization (WHO) classification of salivary gland tumors and in 2017 WHO Classification, the tumor was clubbed as a sub-variant of adenocarcinoma, not otherwise specified. It most commonly occurs in the major salivary glands. Herein we report a case of salivary papillary cystadenocarcinoma in a 54-year-old female, who presented with rapid enlargement of the right parotid swelling. Based on radiology and fine-needle aspiration cytology, a working diagnosis of the malignant tumor involving the superficial lobe of the right parotid gland was made. In view of the malignant nature of the swelling, superficial parotidectomy was done. The histopathology and immunohistochemistry of the mass confirmed the diagnosis of papillary cystadenocarcinoma of the right parotid. With the revised 2017 WHO classification of salivary gland tumors, it is important to report all rare subtypes in order to understand their biology and behavior.

Giant Coronary Aneurysm Causing Ostial Occlusion of Coronary Artery by Mass Effect: A Case Report.

Coronary atherosclerosis can rarely lead to complications like giant coronary aneurysm (GCA), and acute myocardial infarction (AMI) due to thrombosis in the GCA is even rarer. Multimodality imaging is preferred over relying solely on selective coronary angiogram in such cases due to the limitations of invasive coronary angiogram in visualizing thrombosed aneurysms. We report a rare case of a patient with ST-elevation myocardial infarction caused by ostial occlusion of a right coronary artery (RCA) due to mass effect created by thrombosis in a GCA, thereby highlighting a mechanism of AMI that has not been previously described in GCA. Multimodality imaging led to the correct diagnosis and detection of the underlying mechanism, which had been completely missed by invasive coronary angiography (ICA). We also discuss the utility of multimodality imaging in such cases.

Hepatocellular Carcinoma with Inferior Vena Cava and Right Atrium Tumor Thrombus.

Hepatocellular carcinoma (HCC) is one of the leading causes of cancer and cancer related deaths worldwide. Metastasis of HCC into the cardiac cavity is mostly caused by direct tumor thrombus invasion through the major hepatic veins and of vena cava inferior with continuous extension into the right cardiac cavity. Right heart metastasis without invasion of inferior vena cava (IVC), which may be caused by haematogenous spread of cancer cells, is rarely reported. We report a case of HCC with IVC and right atrium (RA) thrombus in a patient who presented to us with decompensated cardiac failure. Strikingly, the patient was young and with negative serum HBsAg, and anti-HCV results. Our case highlights a rare presentation of metastatic intracardiac tumor thrombus involving the RA in advanced HCC without any symptoms of cardiac failure, and henceforth, the role of screening echocardiography for all patients with advanced HCC especially with vena caval involvement to rule out intracardiac thrombus.

Tracheal Diverticulum in SARS-CoV-2 Patients on Non-Invasive Ventilation A not so "Spontaneous" Cause of Pneumomediastinum? An imaging Pictorial Presentation of Two Cases with Review of Literature.

Spontaneous pneumomediastinum is a rare but potentially life-threatening condition, the incidence of which has showed an increase in patients with SARS-CoV-2 pneumonia, especially when they are on positive pressure ventilation. None of the reported cases of covid related pneumomediastinum had an associated tracheal diverticulum. Also, to the best of our knowledge, tracheal diverticulum has not been reported in patients on NIV. We report 2 cases of COVID-19 pneumonia on NIV with pneumomediastinum, which also had associated tracheal diverticulum, one of which developed after NIV. Though the establishment of causality needs further research, early detection of a tracheal diverticulum, which might be a harbinger of pneumomediastinum, can be a timely alarm to prompt titration of the pressure settings and judicious use of NIV. The role of inverted grey scale CT images in mediastinal window is a simple, yet hardly utilised radiological tool to increase detection of 'mediastinal air', let it be free air or air within a diverticulum. Through this case report, we would like to highlight the role of conventional and inverted CT imaging of pneumomediastinum and tracheal diverticulum in general and in SARS-CoV-2 pneumonia in particular, and to call for more objective research to throw light on the plausible relationship between pneumomediastinum and tracheal diverticulum.

Endovascular Parent Artery Occlusion for Intracranial Aneurysms is a Viable, Cost-Effective Alternative: An Institutional Experience from Northeast India.

Purpose: Endovascular parent artery occlusion (PAO) may be an alternative approach for complex intracranial aneurysm with potentially life-threatening complications. Moreover, the long-term follow-up of the PAO for an intracranial aneurysm is reported sparingly, limited to the case series. It is therefore important to carry out more research on long-term follow-up of the implication of PAO of intracranial aneurysm. The aim of the study was to analyses our experience of PAO for intracranial aneurysms with emphasis on long-term follow-up. Materials and Methods: The data of patients treated with PAO for intracranial aneurysms were reviewed. The outcome was evaluated based on aneurysmal occlusion on immediate angiography, follow-up magnetic resonance angiography (MRA), and complications. The modified Rankin score (mRS) was used to evaluate the functional outcome during the last follow-up. The mean, range, and standard deviation were reported for other variables - the patient's age, number, and percentage. Results: Endovascular treatment was performed in 178 patients including PAO in 18 patients. Of these 18 (eighteen) patients, there were 13 dissecting aneurysms, 4 mycotic aneurysms, and one traumatic aneurysm.10 (ten) patients underwent PAO for proximal intracranial artery aneurysm and 8 (eight) patients for distal cerebral aneurysms. Complete occlusion of the aneurysm was achieved in 16patients (88.89%) and retrograde filling of the aneurysm was seen in 2 (11.11%) patients. One patient had intraprocedural coil migration resulting in a major infarct with an mRS of 2. Another patient (5.56%) had recanalization of the aneurysm and presented with rupture and intracranial hemorrhage with an mRS score of 4. The mRS of the other 16 patients (88.89%) was zero. Conclusions: Endovascular PAO for cerebral aneurysms was highly feasible and achieved complete occlusion. The morbidity and mortality rates were at the long-term follow-up also acceptable with negligible complications.

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Pediatric Granular Cell Tumor of the Breast: An uncommon neoplasm in an uncommon site and age group.

Granular cell tumor (GCT) is a rare soft tissue neoplasm of Schwann cell origin. Most cases occur in adults; however, the precise incidence is unknown in children. GCT is usually a slow-growing, painless tumor involving the skin and soft tissues that is mostly located in the head and neck region, especially the tongue. The breast is one of the least common sites involved by GCT. This paper presents a 3-year-old girl who presented with a soft to firm, ill-defined swelling on the right breast with painful ulceration of the overlying skin. Fine needle aspiration rendered an initial diagnosis of fibrocystic change accompanied by apocrine metaplasia. Histologic evaluation of the excised breast mass revealed a benign granular cell tumor. Although rare, GCT of the breast should be included in the differential diagnosis for breast masses in pediatric patients. Proper diagnosis and timely management of this tumor are essential because of its malignant potential (<2% of cases) and high rate of local recurrence if not properly excised.

Maternal and Fetal Tuberous Sclerosis: Do We Know Enough as an Obstetrician?

BACKGROUND: Tuberous sclerosis, also known as tuberous sclerosis complex (TSC), is a rare genetic condition that mainly causes hamartomas to develop in different parts of the body. TSC, an autosomal dominant trait with variable penetrance, can adversely affect maternal and fetal outcome. CASE PRESENTATION: In this paper, a case of maternal and fetal tuberous sclerosis having fetal cardiac rhabdomyoma detected in utero at 26 weeks was reported who subsequently had fetal demise at 31 weeks. CONCLUSION: Tuberous sclerosis is a rare genetic condition that mainly causes development of hamartomas. In tuberous sclerosis, a cardiac rhabdomyoma is the only sign that can be detected prenatally. In maternal tuberous sclerosis, fetal ECHO is advisable after 24 weeks. A pregnancy complicated by maternal or fetal tuberous sclerosis deserves careful observation and the fetus should undergo prenatal fetal Doppler echocardiography and if possible magnetic resonance imaging for evaluation of other fetal structures including brain and renal parenchyma, so that parents can be counseled regarding its future prognostic implications. Tuberous sclerosis can lead to poor fetal outcome including intrauterine fetal death; hence regular antenatal follow up is required. Genetic counseling is recommended for couples who have a family history of tuberous sclerosis and who want to have children. Prenatal diagnosis is available for families with a known gene mutation or history of this condition.

Lower urinary tract obstruction in male children - a report of three cases.

Urinary retention in young children and infancy is relatively rare. The commonest malignancy cause of bladder outlet obstruction in infancy is prostatic rhabdomyosarcoma; the commonest cause of congenital bladder outlet obstruction in a male infantis posterior urethral valve and the commonest cause of urethral obstruction in male children is urethral calculi. We present here a report on three cases of urethral obstruction in male children.