A Rare Case of Multicentric Primary Pulmonary Artery Sarcoma: Eliminating the Masquerade with Multimodality Imaging.

A 68-year-old male presented with a short history of exertional dyspnea and a provisional diagnosis of pulmonary thromboembolism was made. However, chest radiograph and further investigations in the form of computed tomography pulmonary angiogram, magnetic resonance imaging of thorax, and whole body fluorodeoxyglucose (FDG) positron emission tomography-computed tomography revealed a large mass arising from the distal left pulmonary artery extending into adjacent lung and another lesion near the root of the main pulmonary artery, both of which showed post-contrast enhancement and intense FDG uptake. Tissue sampling by transthoracic computed tomography-guided biopsy and immunohistochemistry confirmed the diagnosis of pulmonary artery angiosarcoma. Here, we present such a case of very rare occurrence which, in view of multicentricity and substantial extension into adjacent lung, is the first of its kind to be reported, to the best of our knowledge.

Coronary artery air embolism complicating a CT-guided percutaneous lung biopsy.

Coronary arterial air embolism is an extremely rare but readily recognizable condition on computed tomography (CT) that may complicate a lung biopsy. We present an incidence of symptomatic air embolism into the right coronary artery during a percutaneous CT-guided lung biopsy that was successfully recognized during the procedure and managed accordingly. An active search for this complication should be made when the patient deteriorates on table and the usual complications (pneumothorax, vasovagal shock, etc.) are ruled out, as immediate resuscitative measures could be life-saving.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome: important differential for juvenile idiopathic arthritis.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is an inherited disorder characterized by congenital or early-onset flexion camptodactyly, childhood-onset of non-inflammatory arthropathy, often associated with non-inflammatory pericarditis or pericardial effusion and progressive coxa vara. The causative gene is located on chromosome band 1q25-31. This gene encodes for "proteoglycan-4" (PRG-4), which is a surface lubricant for joints and tendons. This syndrome has distinct radiological and histological features, which are important to recognize since it may clinically mimic juvenile idiopathic arthritis and mutation studies may not be easily available. We describe a case of a 3-year 3-month-old female with features of CACP syndrome.

Menkes kinky hair syndrome: a rare neurodegenerative disease.

Menkes kinky hair disease is a rare X-linked recessive disease nearly exclusively affecting males who present at 2-3 months of age due to abnormal functioning of copper-dependent enzymes due to deficiency of copper. Here, we describe a completely worked-up case of a 4-month-old male infant with very typical history and radiological features confirmed by biochemical and trichoanalysis. The initially seen asymmetric cortical and subcortical T2 hyperintensities in cerebral and cerebellar hemispheres converted into symmetrical diffuse cerebral and predominantly cerebellar atrophy with uniform loss of both white and grey matter on follow-up MRI. Also, subdural hemorrhages of various sizes and different stages and tortuosity of larger proximal intracranial vessels with distal narrowing were identified. Ours is a completely worked-up proven case of Menkes kinky hair disease (MKHD) with history, electroencephalography, biochemical, trichoanalysis, and MRI findings. This is a good teaching case and shows importance of clinical examination and biochemistry as complimentary to MRI. Tortuous intracranial arteries with blocked major vessels are found only in this disease, thus stressing the value of MR Angiography in these patients.