Thoracic neurogenic tumors: A clinicopathologic evaluation of 42 cases

Abstract

Background & Objective: Neurogenic tumors typically originate from the peripheral nerves, paraganglionic nerves, or the autonomic nervous system. Tumors arising from peripheral nerves are classified as schwannoma, neurofibroma, and malignant peripheral nerve sheath tumors while tumors arising from the sympathetic chain ganglion cells are classified as ganglioneuroma, ganglioneuroblastoma, and neuroblastoma. Tumors arising from the parasympathetic chain ganglion cells are classified as paraganglioma. Neurogenic tumors of the thorax are uncommon and originate from large airways, lungs, the mediastinum, or the chest wall. In this study, we report the clinical and histopathological features of 42 patients diagnosed with neurogenic tumors of the thorax. Methods: A retrospective review of the medical records of 42 patients diagnosed with intrathoracic neurogenic tumors and treated in Uludag University between 2002 and 2012 was conducted. All pathology specimens were examined by a pathologist experienced in the examination of soft tissue tumors. The patients were evaluated according to age, gender, location and histological characteristics of the tumor. Results: The study group included 42 patients diagnosed thoracic neurogenic tumors, including 31 female (74%) and 11 male (26%). The sex ratio was 2.8 (female/male) and the mean age of the study population was 38.52 years. The age of patients ranged from 3 to 73 years. The neurogenic tumor was located in the posterior mediastinum in 31(74%) patients, anterior mediastinum in 2 (5%) patients, and in the chest wall in 9 (21%) patients. The origin of the neurogenic tumor was the peripheral nerve sheath in 31 (74%) patients, and the ganglion cells in 10 (24%) patients, and the paraganglion system in 1 (2%) patient. The study group also included 20 (48%) patients diagnosed with schwannoma, 6 (14%) patients diagnosed with malignant peripheral nerve sheath tumor, 5 (12%) patients diagnosed with neurofibroma, 5 (12%) patients diagnosed with ganglioneuroma, 4 (10%) patients diagnosed with neuroblastoma, 1 (2%) patient diagnosed with ganglioneuroblastoma and 1 (2%) patient diagnosed with paraganglioma. Our study group comprised 36 adults and 6 children. Of the adult patients, 20 (55%) had schwannomas, 6 (17%) malignant peripheral nerve sheath tumor, 5 (14%) neurofibroma, 4 (11%) ganglioneuromas, and 1 (3%) paraganglioma. Four of the six children (66%) included in our study group were diagnosed with neuroblastoma, 1 (17%) child was diagnosed with ganglioneuroma, and 1 (17%) child was diagnosed with ganglioneuroblastoma. The malignancy rate was 83% in children and 17% in adults.Conclusion: Age is an important clinical parameter in terms of histological type and malignancy rate. In our study group, malignancy rate in children was much higher than adults. The most common thoracic neurogenic tumor in adults and children was schwannoma and neuroblastoma, respectively.