<p align="left">Vanishing lung syndrome (VLS), also known as giant bullous emphysema (GBE) is a distinct clinical syndrome usually affecting young male smokers characterised by large bullae that involve at least one-third of one or both hemithoraces. We are reporting one such case in a 40-year-old male patient, who was a chronic smoker for past 20 years. He was diagnosed having chronic obstructive pulmonary disease (COPD) on the basis of his history and chest X-ray findings 5 years back and treated symptomatically. He was never suspected of having VLS until a computed tomography of thorax done in our department revealed characteristic findings of VLS with pneumothorax and subcutaneous emphysema. Bullectomy, either via video thoracoscopy or conventional thoracotomy, is the treatment of choice. The patient is now in follow-up with pulmonary medicine department. This case exemplifies role of HRCT thorax in timely diagnosis and planning appropriate treatment of VLS.</p>
Pulmonary alveolar microlithiasis (PAM) is a rare chronic lung disease characterized by presence of widespread intraalveolar accumulation of innumerable minute calculi called microliths. It is caused by inactivating mutations in the gene "solute carrier family 34 member 2", encoding a sodium-dependent phosphate co-transporter (SLC34A2) expressed primarily in alveolar epithelial type II cells. It is most frequently diagnosed from birth to 40 years of age with a mean age of 27-30 years at the time of diagnosis. Most of patients are asymptomatic or having mild symptoms and are usually diagnosed incidentally. Chest radiograph and high-resolution CT of thorax are nearly pathognomonic for diagnosing PAM and histopathological confirmation is required only in few cases. This disease has slow progressive course ultimately leading to death by causing pulmonary fibrosis and cor pulmonale. Currently, there is no medical or gene therapy capable of reducing disease progression. Lung transplantation remains the only possible treatment for end-stage disease. Herein,we report a case of PAM in a 60-year-old gentleman who presented with a 5-year history of shortness of breath on exertion and intermittent cough with expectoration. His sister had similar respiratory symptoms and died 10 years back of which no details are available. The rarity of this disease and late age of presentation prompted us to report this case.
At autopsy, there were numerous individual fatty tumors arising within the pleural cavity, omentum, mesentery, serosa of small and large intestine, along the aorta, surrounding both kidneys, in the soft tissues of the back, thigh and upper arms, the subepicardial fat and in the bone marrow. He interpreted this entity as a systemic malignant disease of the soft tissue and coined the term ABSTRACT Multifocal soft tissue sarcoma (STS) is a rare and controversial entity, accounting for about 1% of patients with extremity STS and 4.5% of patients with liposarcoma. Multifocal presentation can occur synchronously or metachronously and is defined as the presence of tumor at two or more anatomically separate sites before the manifestation of disease in sites where sarcomas usually metastasize (e.g., lungs, liver, bone). Myxoid liposarcoma is the predominant histological type in multifocal presentation. This matter is debated as to whether this entity represent an unusual pattern of metastasis or multiple separate primary tumors as the differentiation between second primary and metastatic lesions has major clinical consequences. Recent literatures based on molecular biologic analysis of tumor clonal heterogeneity suggest metastatic nature. Multifocal myxoid liposarcoma has an aggressive clinical course with frequent recurrences and poor prognosis. Surgery remains the mainstay of treatment with adjuvant chemo and radiotherapy. Herein we are reporting a case of metachronous multifocal myxoid liposarcoma with multiple tumor sites (bilateral breasts, anterior chest wall, anterior abdominal wall, right shoulder area, left thigh etc.) which developed after one year of lumpectomy of myxoid liposarcoma of left breast. A recent review of literature pertaining to its unusual metastatic character, imaging and pathologic features is made.
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