Late Onset of Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report

Abstract

Lungs are one of most metastatic areas for primitive neuroectodermal tumor (PNET), however primary pulmonary PNET is extremely rare. Here we present a case of a 58-year-old male patient with a tumor in the right lung that originated from the lung but not from chest wall. Patient was diagnosed with PNET following histological and immunohistochemical examination of CT-guided percutaneous tru-cut needle biopsy and no distant metastasis were detected in PET-CT scan. As advised recently in published literature, surgical resection following neoadjuvant chemotherapy protocol is preferred in the treatment of our patient as it has better success of complete resection leading to higher 5-year survival rates. Although primary pulmonary PNET is uncommon, it should be taken into account and complete surgical resection should be aimed as treatment to achieve higher survival rates.

Outcome of multidisciplinary treatment of peripheral primitive neuroectodermal tumor

“…Further analysis showed that main parameters leading to higher survival included: more complete surgical resection, more than 10 cycles of chemotherapy and VDC/IE regimen. In the literature, the role of extensive and complete or near complete surgical excision has been advocated as critical for local tumor control and prolonged survival 5,21,22 , which was corroborated by our results. Retrospective analysis of several large groups gives the impression of maximal local control when surgery is feasible 23 .…”

“…This tumor, which belongs to the Ewing’s sarcoma family, mainly arises from primary neuroepithelia and possesses multidirectional differentiation potential 3 . Because of its embryonic origin, PNET may arise in any organ 5 . Under light microscopy, PNET exhibits diffuse sheets, lobules or focal nests of small round cells with deeply stained round, oval, or irregular nuclei.…”

Rare Primary Pulmonary Primitive Neuroectodermal Tumor: A Case Report and Literature Review

“…Upon searching all available literature published in English journals and reports on primary pulmonary PNETs without thoracic wall involvement, we retrieved 21 studies describing a total of 28 cases ( Table 1 ). 6–26 Among 28 cases, the proportion of male patients was slightly higher than that of female patients (17:11). Of all patients, 87% were younger than 35 years old at disease onset (range, 8–67 years old), which is in accordance with the common characteristics of PNET.…”

18F-FDG PET/CT Findings in a Patient With Primary Primitive Neuroectodermal Tumor of the Lung

“…Primitive neuroectodermal tumor is a highly malignant neoplasm that usually arises in the bone or soft tissue in children and adolescents with a slight male preponderance. [1][2][3][4] Primary PNET of the lung is very rare with a poor prognosis. [1][2][3][4][5][6] The most common clinical symptoms of pulmonary PNET are cough, hemoptysis, dyspnea, or chest pain.…”