High-grade pulmonary mucoepidermoid carcinoma supplied by the pulmonary artery and vein in a 6-year-old child: a case description and literature analysis
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PROVIDER: S-EPMC9816712 | biostudies-literature | 2022 Sep
REPOSITORIES: biostudies-literature
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Project description:Pulmonary mucoepidermoid carcinoma (PMEC) is the most common malignant salivary gland tumor in the lungs and accounts for 0.1-0.2% of all lung malignancies in adults. It has no specific epidemiological or clinical characteristics. Correct diagnosis requires the combined examinations of images, laboratories, pathology, and immunohistochemistry (IHC) as well as molecular characteristics. PMEC tumors are characterized by squamous, intermediate, and mucus-secreting cells. Currently, histological appearance, mitotic frequency, cellular atypia, and necrocytosis allow the classification of PMEC into low grade or high grade. Molecular changes are crucial to pathological diagnosis. The driver of PMEC seems to be the fusion protein MECT1-MAML2 that is generated from a genetic mutation in t (11; 19) (q21; p13), while other gene mutations are also reported. However, no treatment of PMEC exists so far; surgical excision is still the primary treatment, while the efficacies of chemotherapy or radiotherapy are undefined. Tyrosine kinase inhibitor (TKI) therapy and immunotherapy showed to have significant therapeutic effects but require more investigation and better understanding. This review focuses on the clinical characteristics, imaging and pathologic features, immunohistochemical examination, mutation analysis, differential diagnosis, prognosis, and treatment of PMEC.
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Project description:RationaleHemoptysis is an uncommon chief complaint but a distressing symptom in pediatric patients. Due to the recurrence and mortality in minor patients, an accurate diagnosis of the underlying cause is quite essential for treatment. The etiologies causing hemoptysis in children are similar to that in adults. Isolated unilateral pulmonary vein atresia (PVA), as an unusual cardiovascular anomaly, has rarely been reported to be an etiology of hemoptysis in children.Patient concernsA 2-year and 11-month-old boy was admitted into our hospital with a complaint of recurrent hemoptysis for 2 months and the symptom became more aggravated in recent 4 days before admission. Physical examination was only remarkable for slightly diminished breath sounds over the left lung field, pale face, and colorless lip. Series of targeted laboratory evaluation were negative expect for anemia. Due to the identification of asymmetrical transparency of bilateral lung, slight emphysema of right lung, less volume of left lung with ground-glass opacity and reticular opacity, and ipsilateral mediastinal shift on chest CT, and varices of submucosal vessels in the left bronchial tree on the fiber-optic bronchoscope.DiagnosesIt more likely indicated a congenital cardiovascular disease. The diagnosis of left isolated unilateral PVA was ultimately confirmed through chest CT angiography (CTA) with three-dimensional (3D) reconstruction.InterventionsSince the boy did not complain with hemoptysis after admission, respiratory tract infections seldom occurred and no pulmonary hypertension was detected, a conservative approach was chosen with periodic clinical follow-up after discussing with the cardiac surgeons and in accordance to his parents' own wishes.OutcomesFortunately, he was doing well after 3 months of clinical observation.LessonsWe firstly reported a rare case of hemoptysis in children secondary to isolated unilateral PVA with no associated congenital heart disease in Chinese population. It is significant to improve the recognition and prompt diagnosis of this rare condition for pediatric clinicians, and widen the etiology spectrum of hemoptysis in children. The diagnosis of unilateral PVA should be considered for a patient with recurrent hemoptysis and imaging findings that indicate hypoplastic lung, ipsilateral mediastinal shift, and smooth margins of left atrium without evidence of rudimentary pulmonary veins.
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Project description:A 46-year-old patient who had undergone right pneumonectomy for pulmonary artery intimal sarcoma presented with hypoxemia. The recurrent sarcoma in the mediastinum revealed external compression to the left pulmonary veins (PVs), leading to obstructive shock and cardiac arrest. Venous artery extracorporeal membrane oxygenation (VA-ECMO) was initiated; however, withdrawal was difficult, and the patient's survival seemed hopeless. However, the patient's condition improved with stenting for the compressed PV; therefore, VA-ECMO was discontinued, and he was discharged on foot. This is the first case report of obstructive shock due to critical PV stenosis caused by compression of a malignant tumor that responded to PV stenting.
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Project description:In the normal lung, the only communications between the systemic and pulmonary arterial systems are the connections between the bronchial and pulmonary arteries that occur at the respiratory bronchioles, where pulmonary and bronchial capillaries freely anastomose. Rarely, anomalous connections can occur between normal or aberrant systemic arteries and pulmonary vessels. We performed a comprehensive literature review of all available manuscripts on PubMed and Google Scholar that included a case report or case series with diagnosis of systemic artery to pulmonary venous fistulas who underwent percutaneous treatment. Furthermore, we report three cases of children diagnosed and treated in our Pediatric Cardiology Center.
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Project description:Equine cardiovascular structures and function are routinely assessed by transthoracic echocardiography. Recently, investigators have described the echocardiographic visualization of equine pulmonary vein ostia. In companion animals, the right pulmonary vein (RPV) to right pulmonary artery (RPA) ratio has been used as an index to estimate the severity of cardiac diseases resulting in left ventricular volume overload. We sought to assess the feasibility of measuring RPV and RPA dimensions, and sought to provide various previously examined RPV and RPA variables in clinically healthy horses that could be used to assess cardiopulmonary disease status. Echocardiographic examination was prospectively performed in 70 healthy horses. The RPV and RPA were visualized using a modified right parasternal long-axis view and maximum and minimum diameters of both vessels were measured from 2D guided M-mode traces. The aortic diameter (Ao) was measured from the right parasternal short-axis view in early diastole. These measurements were then used to produce various ratio indices. RPV and RPA were imaged in all 70 horses. Median of the minimum and maximum RPV/RPA was 0.51 and 0.60, respectively. Median fractional dimensional change of vessels was 33% for RPV and 22% for RPA. The medians of the minimum and maximum RPV/Ao and RPA/Ao were 0.18, 0.28, 0.35 and 0.46, respectively. No relationships between either bodyweight or heart rate and any of the vein or artery variables were identified (maximum r2 = 0.04). Inter- and intra-observer measurement variability was very good for all RPV and RPA measurements. Measuring of RPV and RPA diameters using M-mode transthoracic echocardiography is feasible in healthy horses. Further studies of these variables in horses with cardiac diseases are needed to determine the clinical applicability and utility.
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Project description:BackgroundDissection involving patent arterial duct (PDA) extending to main pulmonary artery (MPA) is an extremely rare condition.Case summaryWe report a case of complex dissection involving PDA extending to MPA, in a child with vein of Galen malformation. This was surgically corrected under deep hypothermic circulatory arrest, and we describe the pathophysiology and surgical rationale in this report.DiscussionManagement of intracardiac lesion in the presence of intracranial arterio-venous malformations such as vein of Galen is very difficult, and employing deep hypothermic circulatory arrest allows one to manage cerebral protection while offering the visualization of intracardiac anatomy more effectively.
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Project description:BackgroundA cervical arteriovenous fistula (AVF) in neurofibromatosis type I (NF-1) is uncommon, and it brings challenges and difficulty in treatment.Case presentationA 39-year-old woman was diagnosed with an NF-1-associated spontaneous vertebral artery-internal jugular vein-spinal vein fistula. The fistula was placed by coil embolization. Postoperative examination showed that the fistula closure was satisfied, and the patient's abnormal clinical manifestation disappeared without any complications after 24 months of interventional embolization. As per the literature, interventional embolization is currently the main treatment method, and it has the distinguishing features of less trauma, quick recovery, and a good prognosis.ConclusionNF-1 associated with a spontaneous arteriovenous fistula is rare in clinical practice, which carries significant challenges in treatment, but can be effectively treated using endovascular embolism. Endovascular embolism could be the potential choice of treatment in NF-1 associated with AVF.
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Project description:Primary pulmonary high-grade mucoepidermoid carcinoma (MEC) with a cystic airspace is uncommon, and early metastasis is extremely rare. In such cases, however, it is clinically important for clinicians to consider whether the tumor has spread to the lymph nodes through the cystic airspace. A 77-year-old man presented to our hospital with cough and hemoptysis. Chest computed tomography showed a 25-mm-diameter mass with a cystic airspace located in the upper lobe of the left lung. The possibility of malignancy was considered. Without a definitive preoperative diagnosis, left upper lobectomy and mediastinal lymphadenectomy were performed. Histopathological examination revealed the typical histological characteristics of high-grade MEC (stage IA) and no lymph node metastasis. However, lymph node metastasis was found 6 months after surgical resection, and radiochemotherapy was performed. The patient developed widespread metastatic disease 4 months following completion of radiochemotherapy and died 2 months later. Primary pulmonary MEC with a cystic airspace is a rare malignant disease with uncommon imaging findings. Complete surgical resection is the main treatment method for high-grade MEC. In this case, we hypothesize that early metastasis was caused by seeding of tumor cells through the cystic airspace.
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