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Primary endobronchial schwannoma
Journal of Pediatric Surgery (2010) 45, 2241–2243
www.elsevier.com/locate/jpedsurg
Primary endobronchial schwannoma Turkan Tansel a,⁎, Alper Toker a , Dilek Yilmazbayhan b , Mine Gullluoglu b , Enver Dayioglu a a
Istanbul University, Istanbul Medical Faculty, Department of Cardiovascular and Thoracic Surgery, Capa, 34390 Fatih, Istanbul, Turkey b Istanbul University, Istanbul Medical Faculty, Department of Pathology, Capa, 34390 Fatih, Istanbul, Turkey Received 14 April 2010; revised 17 June 2010; accepted 19 June 2010
Key words: Pulmonary; Bronchial; Neurogenic tumor; Schwannoma; Intrabronchial schwannoma; Intrabronchial neurilemmas
Abstract Primary intrabronchial neurilemmas, also called schwannomas, are extremely rare tumors that originate from Schwann cells. We report a case of intrabronchial schwannoma arising in the left main bronchus in an 8-year-old child. The diagnosis was made by bronchofiberscopic biopsy. Although the tumor was benign, the patient was subjected to left pneumonectomy because of destruction of the pulmonary parenchyma distal to the obstruction. The diagnosis of intrabronchial schwannoma was supported pathologically with characteristic findings on Antoni A and Antoni B areas. This case is unique in that it is the youngest case in the literature. © 2010 Elsevier Inc. All rights reserved.
Tracheobronchial tumors in children are rare (Table 1) [1]. Primary neurogenic tumors arising from the trachea and bronchus are extremely rare and often present late [2]. Depending on the location and the size of the tumor, the patient may present with cough, hemoptysis, and dyspnea, or features of partial or total lung collapse [3]. We present a case of intrabronchial schwannoma with proximal airway obstruction and total lung destruction in an 8-year-old child.
fever. During this period, she was repeatedly treated with antibiotics for pneumonia of the left lung. On physical examination, pulmonary sounds of the left lung were not audible. The chest radiograph showed decreased aeration of the left lung field and an emphysematous right lung (Fig. 1a). Pulmonary function tests showed normal expiratory volumes and expiratory capacity. Contrast computerized tomography (CT) revealed a 2 × 1–cm intrabronchial Table 1
1. Case report
Benign tumors
An 8-year-old girl was referred to our clinic with a 2year history of productive cough, purulent sputum, and Malignant tumors ⁎ Corresponding author. Tel.: +90 212 4142000x32424; fax: +90 212 534 22 32. E-mail addresses: [email protected], [email protected] (T. Tansel). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.06.036
Primary tracheobronchial tumors 1. Epithelial tumors—papilloma, polyps 2. Mucus gland adenoma 3. Salivary gland tumors 4. Neurogenic tumors 1. Malignant tracheal or bronchial adenoma, including carcinoid tumors 2. Fibrosarcoma 3. Bronchial squamous cell carcinoma 4. Adenocarcinoma
2242
T. Tansel et al.
Fig. 1 a, Chest radiograph shows decreased aeration of the left lung and an emphysematous right lung. b, Computed tomography revealed multiple cystic formations and decreased volume of the left lung, increased volume of the right lung, and fibrosis of the left lung. c, Imaginary bronchography showed a 1.5-cm mass with soft tissue density completely obstructing the lumen of the left main bronchus at 2 cm below the carina. Superior, lingular, and lower lobe bronchi are open.
mass in the left main bronchus. The volume of the left lung was decreased owing to multiple cavities and fibrosis (Fig. 1b). Perfusion scintigraphy showed total lack of perfusion at the left lung. On echocardiography, intracardiac structures were normal. Virtual bronchography (software reconstruction from magnetic resonance images) showed a 1.5-cm mass with soft tissue density completely obstructing the lumen of the left main bronchus 2 cm below the carina (Fig. 1c). Three-dimensional CT angiography showed normal main pulmonary and right pulmonary arteries, but the left pulmonary artery was narrowed 2 cm beyond its origin. Bronchoscopy was performed, and biopsy was taken from the mass obstructing the bronchus. Frozen section revealed a mesenchymal tumor with the histomorphologic features of schwannoma. Left pneumonectomy was performed via standard posterolateral thoracotomy. The lung was found to be completely destroyed with extensive areas of abscess formation and fibrosis. On macroscopic evaluation, the intrabronchial mass obstructing the left main bronchus was well circumscribed, firm, and gray-yellow on the cut surface (Fig. 2a). The microscopic examination revealed a mass composed of nodules. Nodules were formed by spindle cells, arranged in fascicles, with their nuclei showing palisading that is a
characteristic finding on Antoni A areas of schwannomas. Less densely cellular Antoni B areas were also observed (Fig. 2b). The cells displayed S100 protein and vimentin immunoreactivity, whereas results of desmin and smooth muscle actin histochemistries were negative. The postoperative course was uneventful, and the patient was discharged on the fifth postoperative day. She has been well for 4 years postoperatively without evidence of recurrence.
2. Discussion Schwannomas can occur in any region of the thoracic cavity and progress into both intraluminal and/or extraluminal spaces and are almost always associated with neurofibromatosis [2,4]. However, primary schwannoma of the lungs without associated neurofibromatosis can occur and are predominantly benign with minimal tendency to recur [2]. Because these tumors generally present late, most patients are asymptomatic; and the tumors are usually detected during routine chest radiography examinations. The clinical course of the patient depends on the localization and
Fig. 2 a, The tumor nodule was observed in the left bronchus in the pneumonectomy specimen. b, Well-circumscribed nodule composed of spindle cells arranged in palisading fashion adjacent to the bronchial cartilage (right lower) (hematoxylin and eosin, original magnification 100×).
Primary endobronchial schwannoma the size of the tumor and degree of bronchial obstruction. Symptoms are usually nonspecific, mainly dry or productive cough, fever, hemoptysis, dyspnea, and postobstructive pneumonia [3]. Radiologically, the tumor appears as a round, ovoid, or lobulated homogenous mass with sharp borders and sometimes has calcifications. If a large bronchus is obstructed, atelectasis of the lung may be the only radiologic sign. Because imaging methods, including plain radiographs, CT, or magnetic resonance imaging studies, are not always diagnostic, the definitive diagnosis is based on histopathologic examination of the tumor. Detection of typical Antoni A formation on hematoxylin-eosin stain and positive S100 protein on immunoperoxidase stain are characteristic findings and confirm the diagnosis [4,5]. Treatment approaches for benign schwannomas vary. In the case of tumors with intra- and extraluminal involvement, complete surgical resection with surrounding pulmonary parenchyma may be indicated. Although malignant transformation of schwannomas is uncommon, inadequate resection of the tumor mass may lead to local recurrence [4]. Treatment
2243 can be managed by bronchoscopy with electrosurgical snaring or yttrium aluminum garnet laser resection, or surgically by lobectomy or pneumonectomy [6].
References [1] Parikh DH. Chapter 20; lung tumors. In: Parikh DH, Crabbe D, Auldist A, Rothenberg S, editors. Pediatric thoracic surgery. Springer-Verlag London Limited; 2009. p. 251-9. [2] Feldhaus RJ, Anene C, Bogard P. A rare endobronchial neurilemmoma (Schwannoma). Chest 1989;95(2):461-2. [3] Oxner CR, Shinners MJ, Godin DA, et al. Obstructing tracheobronchial schwannoma. J La State Med Soc 2005;157(3):159-61. [4] Kasahara K, Fukuoka K, Konishi M, et al. Two cases of endobronchial neurilemmoma and review of the literature in Japan. Intern Med 2003;42(12):1215-8. [5] Bosch X, Ramirez J, Font J, et al. Primary intrapulmonary benign schwannoma. A case with ultrastructural and immunohistochemical confirmation. Eur Respir J 1990;3(2):234-7. [6] Suzuki H, Sekine Y, Motohashi S, et al. Endobronchial neurogenic tumors treated by transbronchial electrical snaring and Nd-YAG laser abrasion: report of three cases. Surg Today 2005;35(3):243-6.
www.elsevier.com/locate/jpedsurg
Primary endobronchial schwannoma Turkan Tansel a,⁎, Alper Toker a , Dilek Yilmazbayhan b , Mine Gullluoglu b , Enver Dayioglu a a
Istanbul University, Istanbul Medical Faculty, Department of Cardiovascular and Thoracic Surgery, Capa, 34390 Fatih, Istanbul, Turkey b Istanbul University, Istanbul Medical Faculty, Department of Pathology, Capa, 34390 Fatih, Istanbul, Turkey Received 14 April 2010; revised 17 June 2010; accepted 19 June 2010
Key words: Pulmonary; Bronchial; Neurogenic tumor; Schwannoma; Intrabronchial schwannoma; Intrabronchial neurilemmas
Abstract Primary intrabronchial neurilemmas, also called schwannomas, are extremely rare tumors that originate from Schwann cells. We report a case of intrabronchial schwannoma arising in the left main bronchus in an 8-year-old child. The diagnosis was made by bronchofiberscopic biopsy. Although the tumor was benign, the patient was subjected to left pneumonectomy because of destruction of the pulmonary parenchyma distal to the obstruction. The diagnosis of intrabronchial schwannoma was supported pathologically with characteristic findings on Antoni A and Antoni B areas. This case is unique in that it is the youngest case in the literature. © 2010 Elsevier Inc. All rights reserved.
Tracheobronchial tumors in children are rare (Table 1) [1]. Primary neurogenic tumors arising from the trachea and bronchus are extremely rare and often present late [2]. Depending on the location and the size of the tumor, the patient may present with cough, hemoptysis, and dyspnea, or features of partial or total lung collapse [3]. We present a case of intrabronchial schwannoma with proximal airway obstruction and total lung destruction in an 8-year-old child.
fever. During this period, she was repeatedly treated with antibiotics for pneumonia of the left lung. On physical examination, pulmonary sounds of the left lung were not audible. The chest radiograph showed decreased aeration of the left lung field and an emphysematous right lung (Fig. 1a). Pulmonary function tests showed normal expiratory volumes and expiratory capacity. Contrast computerized tomography (CT) revealed a 2 × 1–cm intrabronchial Table 1
1. Case report
Benign tumors
An 8-year-old girl was referred to our clinic with a 2year history of productive cough, purulent sputum, and Malignant tumors ⁎ Corresponding author. Tel.: +90 212 4142000x32424; fax: +90 212 534 22 32. E-mail addresses: [email protected], [email protected] (T. Tansel). 0022-3468/$ – see front matter © 2010 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2010.06.036
Primary tracheobronchial tumors 1. Epithelial tumors—papilloma, polyps 2. Mucus gland adenoma 3. Salivary gland tumors 4. Neurogenic tumors 1. Malignant tracheal or bronchial adenoma, including carcinoid tumors 2. Fibrosarcoma 3. Bronchial squamous cell carcinoma 4. Adenocarcinoma
2242
T. Tansel et al.
Fig. 1 a, Chest radiograph shows decreased aeration of the left lung and an emphysematous right lung. b, Computed tomography revealed multiple cystic formations and decreased volume of the left lung, increased volume of the right lung, and fibrosis of the left lung. c, Imaginary bronchography showed a 1.5-cm mass with soft tissue density completely obstructing the lumen of the left main bronchus at 2 cm below the carina. Superior, lingular, and lower lobe bronchi are open.
mass in the left main bronchus. The volume of the left lung was decreased owing to multiple cavities and fibrosis (Fig. 1b). Perfusion scintigraphy showed total lack of perfusion at the left lung. On echocardiography, intracardiac structures were normal. Virtual bronchography (software reconstruction from magnetic resonance images) showed a 1.5-cm mass with soft tissue density completely obstructing the lumen of the left main bronchus 2 cm below the carina (Fig. 1c). Three-dimensional CT angiography showed normal main pulmonary and right pulmonary arteries, but the left pulmonary artery was narrowed 2 cm beyond its origin. Bronchoscopy was performed, and biopsy was taken from the mass obstructing the bronchus. Frozen section revealed a mesenchymal tumor with the histomorphologic features of schwannoma. Left pneumonectomy was performed via standard posterolateral thoracotomy. The lung was found to be completely destroyed with extensive areas of abscess formation and fibrosis. On macroscopic evaluation, the intrabronchial mass obstructing the left main bronchus was well circumscribed, firm, and gray-yellow on the cut surface (Fig. 2a). The microscopic examination revealed a mass composed of nodules. Nodules were formed by spindle cells, arranged in fascicles, with their nuclei showing palisading that is a
characteristic finding on Antoni A areas of schwannomas. Less densely cellular Antoni B areas were also observed (Fig. 2b). The cells displayed S100 protein and vimentin immunoreactivity, whereas results of desmin and smooth muscle actin histochemistries were negative. The postoperative course was uneventful, and the patient was discharged on the fifth postoperative day. She has been well for 4 years postoperatively without evidence of recurrence.
2. Discussion Schwannomas can occur in any region of the thoracic cavity and progress into both intraluminal and/or extraluminal spaces and are almost always associated with neurofibromatosis [2,4]. However, primary schwannoma of the lungs without associated neurofibromatosis can occur and are predominantly benign with minimal tendency to recur [2]. Because these tumors generally present late, most patients are asymptomatic; and the tumors are usually detected during routine chest radiography examinations. The clinical course of the patient depends on the localization and
Fig. 2 a, The tumor nodule was observed in the left bronchus in the pneumonectomy specimen. b, Well-circumscribed nodule composed of spindle cells arranged in palisading fashion adjacent to the bronchial cartilage (right lower) (hematoxylin and eosin, original magnification 100×).
Primary endobronchial schwannoma the size of the tumor and degree of bronchial obstruction. Symptoms are usually nonspecific, mainly dry or productive cough, fever, hemoptysis, dyspnea, and postobstructive pneumonia [3]. Radiologically, the tumor appears as a round, ovoid, or lobulated homogenous mass with sharp borders and sometimes has calcifications. If a large bronchus is obstructed, atelectasis of the lung may be the only radiologic sign. Because imaging methods, including plain radiographs, CT, or magnetic resonance imaging studies, are not always diagnostic, the definitive diagnosis is based on histopathologic examination of the tumor. Detection of typical Antoni A formation on hematoxylin-eosin stain and positive S100 protein on immunoperoxidase stain are characteristic findings and confirm the diagnosis [4,5]. Treatment approaches for benign schwannomas vary. In the case of tumors with intra- and extraluminal involvement, complete surgical resection with surrounding pulmonary parenchyma may be indicated. Although malignant transformation of schwannomas is uncommon, inadequate resection of the tumor mass may lead to local recurrence [4]. Treatment
2243 can be managed by bronchoscopy with electrosurgical snaring or yttrium aluminum garnet laser resection, or surgically by lobectomy or pneumonectomy [6].
References [1] Parikh DH. Chapter 20; lung tumors. In: Parikh DH, Crabbe D, Auldist A, Rothenberg S, editors. Pediatric thoracic surgery. Springer-Verlag London Limited; 2009. p. 251-9. [2] Feldhaus RJ, Anene C, Bogard P. A rare endobronchial neurilemmoma (Schwannoma). Chest 1989;95(2):461-2. [3] Oxner CR, Shinners MJ, Godin DA, et al. Obstructing tracheobronchial schwannoma. J La State Med Soc 2005;157(3):159-61. [4] Kasahara K, Fukuoka K, Konishi M, et al. Two cases of endobronchial neurilemmoma and review of the literature in Japan. Intern Med 2003;42(12):1215-8. [5] Bosch X, Ramirez J, Font J, et al. Primary intrapulmonary benign schwannoma. A case with ultrastructural and immunohistochemical confirmation. Eur Respir J 1990;3(2):234-7. [6] Suzuki H, Sekine Y, Motohashi S, et al. Endobronchial neurogenic tumors treated by transbronchial electrical snaring and Nd-YAG laser abrasion: report of three cases. Surg Today 2005;35(3):243-6.