%0 Journal Article
%T Thoracic Primitive Neuroectodermal Tumor: An Unusual Case and Literature Review
%A Kubra Erol Kalkan
%A Ahmet Bilici
%A Fatih Selcukbiricik
%A Nurcan Unver
%A Mahmut Yuksel
%J Case Reports in Pulmonology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/326871
%X We describe herein a rare case of a primary primitive neuroectodermal tumor (PNET) in the mediastinum of a 75-year-old man. Grossly, the tumor was located in the left upper anterior mediastinum. Transcutaneous fine-needle biopsy (TCNB) revealed small round-cell proliferation. The expression immunohistochemical analysis was confirmed the diagnosis of PNET. He was successfully treated with chemotherapy and is alive with no sign of recurrence for 17 months after the diagnosis. 1. Introduction PNET represents a family of tumors which shows varying degrees of neuronal differentiation most often presenting as a bone or soft tissue mass in the trunk or axial skeleton in adolescents and young adults [1]. Infrequently, PNETs have been described in other organs, such as the kidney, gonads, pancreas, and myocardium. Intrapulmonary PNET is very rare, PNET is a highly malignant neoplasm and it is composed of small, round, uniform cells [2]. Diagnosis of the tumor is confirmed using various immunohistochemical studies and detecting the presence of a translocation, t(11;22) through fluorescent in situ hybridization (FISH) [3]. PNET can be treated with various combinations of radical surgical resection, neoadjuvant and adjuvant chemotherapy, and irradiation. The chemotherapy of choice for these tumors consists of combinations of doxorubicin, ifosfamide, cyclophosphamide, and vincristine [4]. 2. Case Report A 75-year-old man was admitted to our Department of Medical Oncology because of dry cough which started 1 month ago in July 2011. Chest X-ray showed a left paramediastinal mass. Computed tomography (CT) of the chest demonstrated a 30 ¡Á 90£¿mm in diameter in the upper anterior mediastinal mass with pleural effusion around the left lower lobe. A diagnostic work up was started because of possible primary lung malignancy. FDG-PET-CT scan revealed intense and homogenous hypermetabolic activity at the upper anterior paramediastinal region (standardized uptake value (SUV): 7). Furthermore, there was hypermetabolic lesions at the left basal pleura which was compatible with metastases (Figure 1). Thereafter, a percutaneous needle biopsy was performed. Histopathologic examination of the biopsy specimen indicated malignant, small, round-cell tumor. Some of the cells had irregularly vacuolated cytoplasm secondary to glycogen deposition, which was positive for Periodic Acid Schiff (PAS) stain. In addition, the glycoprotein p30/32 (CD99), which is encoded by the MIC2 gene, is strongly expressed on the surface of the tumor cells (Figure 2). The chromosome rearrangement
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