Mediastinal tumours are not uncommon in paediatric population and often pose a diagnostic challenge. They include a variety of entities including developmental, inflammatory, infectious and neoplastic; most are malignant. These lesions can be classified based on imaging according to the specific compartment (anterior, middle and posterior), generating a focused differential diagnosis. This combined with a rational, clinically oriented approach based on patient’s history, focused physical examination, age, gender, symptoms, signs, anatomic localization, imaging characteristics and laboratory investigations including tumor markers paves way to a presumptive diagnosis guiding additional and prudent investigations. For example, a suspicion of lymphoma should be kept in a child presenting with a neck mass and superior vena cava syndrome. Neuroblastoma should be suspected among children younger than 5 years old with a posterior mediastinal mass. Such a structured approach along with histopathology will lead to an exact diagnosis. Surgery remains the mainstay of treatment of most benign and malignant non-lymphoid tumours. For optimal management, a combined modality of treatment incorporating chemotherapy and radiotherapy is often required in malignant tumours and is associated with high survival rates in these patients. In the present article, we review the approach to evaluation of mediastinal masses in childhood from a clinical perspective.
Herlyn-Werner-Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients usually present after menarche with pelvic pain, dysmenorrhea, mass, and rarely with primary infertility in later years. We report a case of a multiparous female who presented to the hospital with pain in lower abdomen for the past 2 months and acute retention of urine for 1 day. This is an atypical presentation in a multiparous female that has been described in a single case report so far. Intravenous pyelogram and Magnetic Resonance Imaging of the patient revealed uterine didelphys, obstructed right hemivagina causing hematohemicolpos and right renal agenesis. Thorough knowledge of imaging features can enable a radiologist to make a correct diagnosis even in an atypical presentation.
Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. Clinically, the malignancy is often mistaken for symptoms of respiratory tract infection or pneumothorax. The neoplasm is histologically characterized by primitive blastema and a malignant mesenchymal stroma that demonstrates multidirectional differentiation. The patients with PPB are managed by multimodal therapy. We present a report of 3 cases of histopathologically diagnosed pleuropulmonary blastoma. The patients presented with chief complaints of difficulty in breathing, cough, fever and chest pain. Radiographs of the patients showed partial to complete opacification of hemithorax. Contrast enhanced computed tomography scans revealed large well defined heterogenously enhancing solid mass lesions in the hemithorax. Knowledge of types, imaging findings, staging and association with other tumors is crucial for correct diagnosis of pleuropulmonary blastoma and subsequent adequate management.
A criss-cross heart is an uncommon congenital rotational anomaly. It accounts for less than 0.1% of all congenital heart defects. The anomaly is characterized by crossing of the atrioventricular connections caused by rotation of the heart about its long axis. It is commonly associated with diverse cardiac defects. Cardiac CT imaging of criss-cross heart is sparse. We present a case of 1-year-old child with chief complaints of bluish discoloration of the body and fast breathing. Cardiac CT revealed atrial situs solitus, criss-cross-atrioventricular connections, atrioventricular discordance, double outlet right ventricle and dextro-malposed great arteries (Van Praagh S,D,D).
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