Primitivni neuroektodermalni tumor pluća s pleuralnim izljevom - prikaz slučaja

Introduction: Primitive neuroectodermal tumor (PNET) is a group of rare, highly malignant neoplasms usually found in children and adolescents. The tumor tends to metastasize early, and consequently the survival rate is low. Case report: A 29-year-old female presented with pain in the right side of the thorax and progressive breathlessness of one-month duration. Multi-slice computed tomography (MSCT) of the chest revealed a large pleural effusion in the right hemithorax, and a large expansive formation in the right upper lobe. Thoracentesis was performed, and malignant cells were found in pleural effusion. Bronchoscopic examination revealed extrinsic compression on the bronchi, without malignant cells in the samples taken. Transthoracic MSCT guided biopsy of the mass histologically revealed small cells, with hyperchromatic nuclei and atypical mitosis. Immunohistochemically, the cells were positive for CD99, NSE and CD56. Positron emission tomography (PET) CT revealed intense accumulation of 18F-fluorodeoxyglucose in the right upper lobe and in the mediastinal lymph nodes. Octreoscan showed pathological accumulation of radiopharmaceuticals in the right upper lobe and mediastinum, indicating a lesion with the expression of somatostatin receptor. The patient received chemotherapy (cisplatin and etoposide), and octreotide acetate. PET/CT showed tumor progression. Palliative radiotherapy was administered. The disease progressed rapidly and the patient died subsequently eleven months after the diagnosis. Conclusion: PNET is an aggressive and lethal disease, therefore should be considered in the differential diagnosis of thoracic tumors regardless of the age of the patient.