engleski

PRIMITIVE NEUROECTODERMAL TUMOR OF THE LUNG IN A 29-YEAR-OLD WOMAN

Introduction: Primitive neuroectodermal tumor (PNET) is a rare, highly malignant neoplasm, usually found in children and adolescents, with low survival rate. Case Report: A 29-year-old female presented with pain in the right side of the thorax and progressive breathlessness of one month duration. Chest radiograph showed a large homogenous opacity in upper zone of right lung with ipsilateral pleural effusion. Multislice computed tomography (MSCT) of the chest revealed a right sided pleural effusion and a mass with lobulated margins, size of 92x84x92 mm in the upper right lobe. In pleural effusion malignant cells were found. Bronchoscopic examination revealed extrinsic compression of right upper lobe bronchus, and no malignant cells were found in biopsy specimens. Transthoracic MSCT guided biopsy of the mass histologically revealed small, round cells, with hyperchromatic nuclei, immunohistochemically positive for CD99, NSE and CD56, which confirmed the PNET. Positron emission tomography (PET) presented upper lobe of the right lung enhancement. Octreoscan showed pathological accumulation of radiopharmaceuticals in the right upper lung lobe and mediastinum, indicated a lesion with the expression of somatostatin receptor. The patient received two cycles of chemotherapy (cisplatin and etoposide), and octreotide acetate. Post-therapy PET/CT showed progression of the tumor. Palliative radiotherapy was administered. Despite all, tumor progressed rapidly and the patient died eleven months after the diagnosis. Conclusion: Primitive neuroectodermal tumor is aggressive, with bad prognosis, therefore should be considered in the differential diagnosis of thoracic tumors regardless of the age of patient.

primitive neuroectodermal tumor, pleural effusion, CD99, chemotherapy, malignant tumor

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