Disorders of the Mediastinum: Pleura and Chest Wall |

Primary Pleural Primitive Neuroendocrine Tumor: A Case Report FREE TO VIEW

Mayuri Johari, MBBS; Deepak Kumar Prajapat, DTCD; Arjun Khanna, DM; Deepak Talwar, DM
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Metro Center for Respiratory Diseases, Noida, India

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;149(4_S):A227. doi:10.1016/j.chest.2016.02.235
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SESSION TITLE: Pleura and Chest Wall

SESSION TYPE: Case Report Poster

PRESENTED ON: Sunday, April 17, 2016 at 11:45 AM - 12:45 PM

INTRODUCTION: Primitive neuro-ectodermal tumors (PNET), are rare lung tumors. Primary pleural PNET is an extremely rare entity.

CASE PRESENTATION: A 52 year old non smoker female presented with shortness of breath, dry cough and right sided pleuritic pain since one month. CT chest revealed a right effusion with pleural nodules and contralateral mediastinal shift. Pleural fluid aspiration revealed neutrophilic exudate with mesothelial cells. She then underwent medical thoracoscopy which revealed multiple lobulated pleural nodules of varying sizes without lung nodules. Pleural fluid cytospin showed few atypical cell clusters with hyperchromatic nuclei. Fibreoptic bronchoscopy showed normal anatomy. PET CT showed a heterogeneously enhancing lesion (2.8 X 2.0 cm) with increased FDG uptake (SUVmax-14.6) in right pleura with multiple enhancing nodular pleural lesions involving costal and diaphragmatic pleura and station 7 and bilateral 4R lymphadenopathy. Histopathology revealed small round cell proliferation with mitosis of 1-2/HPF. Immuno-histochemistry revealed positivity for NSE, CD56, CD99, S100 and Ki67 of 70 % consistent with PNET. Post thoracoscopy, pleurodesis with Talc was done and ICD removed after 2 days. Patient was advised chemotherpy which was refused, and the patient lost followup.

DISCUSSION: PNET are rare tumors occurring in the posterior sulcus or chest wall. Usually painful, these invasive thoracic tumors may develop on and invade chest wall, lung, or mediastinum1. The pathological differentials of lung PNET include small cell carcinoma and other small round-cell tumors, such as malignant lymphoma, Langerhans’cell histiocytosis, rhabdomyosarcoma, and synovial sarcoma with the characteristic pathology of nests of small cells and Homer-Wright pseudo-rosettes with an acidophilic positivity of neurofibrillar elements2. Our patient's biopsy showed infiltration with round and spindle shaped tumor cells. However, CK, Thyroid transcription factor (TTF1), P63, CALRETININ, HBME1 markers were negative on IHC. Further, Vimentin and CD-99, S100 and NSE were positive, with Ki-67 positivity of 70%; clinching the diagnosis of malignant neuro endocrine tumor3. As PET was negative for distant metastasis, the diagnosis of primary pleural PNET was considered.

CONCLUSIONS: PNET, a rare aggresive thoracic tumor, can originate from the pleura. The treatment of choice for these tumors depends on the primary site, functional status and the stage of the disease.

Reference #1: B. Askin et al. “Malignant small cell tumor of the thoracopulmonary region in childhood. A distinctive clinicopathologic entity of uncertain histogenesis,” Cancer, vol. 43, no. 6, pp. 2438-2451, 1979.

Reference #2: WD Zhang et al. Computed tomography imaging of anterior and middle mediastinal Ewing sarcoma/primitive neuroectodermal tumors. J Thorac Imaging. 2010 May;25(2):168-72.

Reference #3: S. Tsuji et al., “Peripheral primitive neuroectodermal tumour of the lung:report of two cases,” Histopathology, vol.33, no.4, pp. 369-374, 1998.

DISCLOSURE: The following authors have nothing to disclose: Mayuri Johari, Deepak Kumar Prajapat, Arjun Khanna, Deepak Talwar

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