Pleural-based granular cell tumor Chibuzo Odigwe, MD, Robert Bradley, MD, and Rohan Ranjit Arya, MD
Granular cell tumors are rare tumors of Schwann cell origin that arise from tissues of neural crest and mesenchymal origin. We report the clinical, radiographic, and pathological features of a pleural-based granular cell tumor in a 60-year-old African American man.
G
ranular cell tumors are mostly benign tumors of Schwann cell origin and occur mostly in the skin, subcutis, and along mucosal surfaces and occasionally within skeletal muscle, the gastrointestinal tract, and the respiratory tract. A granular cell tumor was originally reported by Abrikossoff in 1926 (1). Clinical manifestations depend on the involved body part and whether the tumor is benign or malignant. These tumors exhibit a characteristic histologic appearance on routine hematoxylin and eosin–stained sections, and the diagnosis can be confirmed by immunohistochemical coexpression of S100 and CD68 by the tumor cells, among other markers (2). CASE PRESENTATION A 60-year-old black man with a past medical history of schizophrenia, hepatitis B, and tobacco abuse presented to our clinic for evaluation of a right lung mass. He had presented earlier to his primary care physician with complaints of a chronic cough and chest congestion prompting a computed tomography (CT) scan of his chest for further evaluation. Cough was intermittent, present for about 1 to 2 years, and worsening in the prior 3 to 4 months. There was no hemoptysis, but there was production of yellow-green sputum. He denied any dyspnea, fevers, or chills. He smoked two packs of cigarettes daily for over 30 years and still smoked about 1 pack daily. He had no occupational exposures and no family history of lung disease or malignancy. He was on clozapine, benztropine, and fluphenazine. His physical examination was remarkable for finger clubbing. CT scan of the chest showed a 2.6 cm smooth right paraspinal pleural-based mass and radiologic evidence of centrilobular emphysema with microblebs predominating in the upper lobes bilaterally (Figure 1). Pulmonary function tests showed a forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio of 71%; FEV1 of 2.6 L, or 112% of predicted; FVC Proc (Bayl Univ Med Cent) 2017;30(3):319–321
Figure 1. CT of the chest showing a 2.6 cm smooth right paraspinal pleuralbased mass and radiologic evidence of centrilobular emphysematous disease.
of 3.67 L, or 121% of predicted; slow vital capacity (SVC) of 4.67 L; residual volume of 5.56 L, or 297% of predicted; and total lung capacity of 10.23 L, or 183% of predicted. These findings were consistent with possible obstructive ventilatory defect with significant hyperinflation and air trapping. F18fluorodeoxyglucose positron emission tomography scan showed an increase in metabolic activity for the pleural-based mass at 3.8 SUV. There was a destructive lesion at the left aspect of the T4 vertebral body, which showed increased metabolic activity with a maximum SUV of 5.7. The patient underwent CT-guided fine needle aspiration (FNA) and core biopsies of the pleural-based mass (Figure 2). The FNA cytology was positive for a neoplasm composed of benign-appearing cells with abundant granular cytoplasm, and the hematoxylin and eosin–stained FNA cell block and core biopsy sections demonstrated closely packed and uniformly bland polygonal cells with similarly From the Division of Pulmonary, Critical Care, and Sleep Medicine, University of South Carolina School of Medicine, Columbia, South Carolina (Odigwe, Arya); and Professional Pathology Services, Columbia, South Carolina (Bradley). Corresponding author: Chibuzo Clement Odigwe, MD, Division of Pulmonary, Critical Care, and Sleep Medicine, University of South Carolina School of Medicine, One Richland Medical Park, Suite 300, Columbia, SC 29203 (e-mail: [email protected]). 319
a
b
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Figure 2. (a) Hematoxylin and eosin stain of the fine needle aspiration cell block. (b) S100 stain of the core biopsy. (c) CD68 stain of the core biopsy.
abundant eosinophilic cytoplasm. Immunohistochemical stains performed on the core biopsy showed that the tumor cells were negative for pan keratin, smooth muscle actin, HMB-45, and MART-1. The tumor cells were positive for S100, CD68, vimentin, inhibin, and SOX 10, with a low proliferative rate (2 mitoses/10 high-power fields at 200× magnification), a high nuclear-to-cytoplasmic ratio, and pleomorphism (4). In the chest and respiratory tract, Kutuya and Akiduki described an asymptomatic young woman who had an illcircumscribed mass with peripheral infiltration in the medial right upper lung field on chest radiograph (5). CT showed an intrabronchial mass that was found to be benign with the granules positive for S100. A right upper lobectomy and lymphadenectomy were done. In a 10-year review of tracheobronchial granular cell tumors in the Dutch Network and National Database for Pathology, 31 tumors were registered in 30 patients. About half of the patients were asymptomatic. All the tumors described were benign. Eighteen of the 30 patients received no specific treatment. The other patients received either surgery, electrocautery, or Nd-YAG laser treatment. The surgical treatments offered were tracheal resection and subsequent reconstruction (for tracheal tumors) and pneumonectomy or lobectomy for bronchial tumors. The patients who had surgery had remission for up to 10 years, and those with residual disease remained stable (6). Multiple reports have described tumors at myriad other locations (7–10). 1. 2.
3.
4.
Abrikossoff A. Uber Myome, ausgehend von der quergestreiften Willkürlichen muskulatur. Virchows Arch Pathol Anat 1926;260(1):215–233. Rekhi B, Jambhekar NA. Morphologic spectrum, immunohistochemical analysis and clinical features of a series of granular cell tumors of soft tissues: a study from a tertiary referral cancer center. Ann Diagn Pathol 2010;14(3):162–167. Nath D, Gupta A, Arava S, Jain D, Madan K. Synchronous existence of granular cell tumor and small cell carcinoma of the lung: an unusual entity. Indian J Pathol Microbiol 2016;59(1):90–92. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22(7):779–794.
Baylor University Medical Center Proceedings
Volume 30, Number 3
5.
6.
7.
Kutuya N, Akiduki A. Radiologic appearance of a bronchial granular cell tumor with secondary obstructive changes Clin Imaging 2010;34(2):148– 151. Van der Maten J, Blaauwgeers JLG, Sutedja TG, Kwa HB, Postmus PE, Wagenaar SS. Granular cell tumors of the tracheobronchial tree. J Thorac Cardiovasc Surg 2003;126(3):740–743. Soh WM, Yeong ML, Wong KP. Malignant granular cell tumour of the mediastinum. Malays J Pathol 2014;36(2):149–151.
July 2017
8. Ihara K, Ito H, Nakajima Y, Fukaya E, Nakazawa H, Nozaki M. Granular cell tumor of the suprasternal space. J Dermatol 2010;37(10):900– 903. 9. Nakao M, Hishida T, Ishii G, Yoshida J, Nishimura M, Nagai K. Malignant granular cell tumor of the posterior mediastinum with dissemination. Asian Cardiovasc Thorac Ann 2012;20(1):71–73. 10. Mehrdad MB, Adams S, Catalano PW. A 65 year old woman with granular cell tumor of the lung. Tanaffos 2010;9(1):59–62.
Pleural-based granular cell tumor
321
Granular cell tumors are rare tumors of Schwann cell origin that arise from tissues of neural crest and mesenchymal origin. We report the clinical, radiographic, and pathological features of a pleural-based granular cell tumor in a 60-year-old African American man.
G
ranular cell tumors are mostly benign tumors of Schwann cell origin and occur mostly in the skin, subcutis, and along mucosal surfaces and occasionally within skeletal muscle, the gastrointestinal tract, and the respiratory tract. A granular cell tumor was originally reported by Abrikossoff in 1926 (1). Clinical manifestations depend on the involved body part and whether the tumor is benign or malignant. These tumors exhibit a characteristic histologic appearance on routine hematoxylin and eosin–stained sections, and the diagnosis can be confirmed by immunohistochemical coexpression of S100 and CD68 by the tumor cells, among other markers (2). CASE PRESENTATION A 60-year-old black man with a past medical history of schizophrenia, hepatitis B, and tobacco abuse presented to our clinic for evaluation of a right lung mass. He had presented earlier to his primary care physician with complaints of a chronic cough and chest congestion prompting a computed tomography (CT) scan of his chest for further evaluation. Cough was intermittent, present for about 1 to 2 years, and worsening in the prior 3 to 4 months. There was no hemoptysis, but there was production of yellow-green sputum. He denied any dyspnea, fevers, or chills. He smoked two packs of cigarettes daily for over 30 years and still smoked about 1 pack daily. He had no occupational exposures and no family history of lung disease or malignancy. He was on clozapine, benztropine, and fluphenazine. His physical examination was remarkable for finger clubbing. CT scan of the chest showed a 2.6 cm smooth right paraspinal pleural-based mass and radiologic evidence of centrilobular emphysema with microblebs predominating in the upper lobes bilaterally (Figure 1). Pulmonary function tests showed a forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) ratio of 71%; FEV1 of 2.6 L, or 112% of predicted; FVC Proc (Bayl Univ Med Cent) 2017;30(3):319–321
Figure 1. CT of the chest showing a 2.6 cm smooth right paraspinal pleuralbased mass and radiologic evidence of centrilobular emphysematous disease.
of 3.67 L, or 121% of predicted; slow vital capacity (SVC) of 4.67 L; residual volume of 5.56 L, or 297% of predicted; and total lung capacity of 10.23 L, or 183% of predicted. These findings were consistent with possible obstructive ventilatory defect with significant hyperinflation and air trapping. F18fluorodeoxyglucose positron emission tomography scan showed an increase in metabolic activity for the pleural-based mass at 3.8 SUV. There was a destructive lesion at the left aspect of the T4 vertebral body, which showed increased metabolic activity with a maximum SUV of 5.7. The patient underwent CT-guided fine needle aspiration (FNA) and core biopsies of the pleural-based mass (Figure 2). The FNA cytology was positive for a neoplasm composed of benign-appearing cells with abundant granular cytoplasm, and the hematoxylin and eosin–stained FNA cell block and core biopsy sections demonstrated closely packed and uniformly bland polygonal cells with similarly From the Division of Pulmonary, Critical Care, and Sleep Medicine, University of South Carolina School of Medicine, Columbia, South Carolina (Odigwe, Arya); and Professional Pathology Services, Columbia, South Carolina (Bradley). Corresponding author: Chibuzo Clement Odigwe, MD, Division of Pulmonary, Critical Care, and Sleep Medicine, University of South Carolina School of Medicine, One Richland Medical Park, Suite 300, Columbia, SC 29203 (e-mail: [email protected]). 319
a
b
c
Figure 2. (a) Hematoxylin and eosin stain of the fine needle aspiration cell block. (b) S100 stain of the core biopsy. (c) CD68 stain of the core biopsy.
abundant eosinophilic cytoplasm. Immunohistochemical stains performed on the core biopsy showed that the tumor cells were negative for pan keratin, smooth muscle actin, HMB-45, and MART-1. The tumor cells were positive for S100, CD68, vimentin, inhibin, and SOX 10, with a low proliferative rate (2 mitoses/10 high-power fields at 200× magnification), a high nuclear-to-cytoplasmic ratio, and pleomorphism (4). In the chest and respiratory tract, Kutuya and Akiduki described an asymptomatic young woman who had an illcircumscribed mass with peripheral infiltration in the medial right upper lung field on chest radiograph (5). CT showed an intrabronchial mass that was found to be benign with the granules positive for S100. A right upper lobectomy and lymphadenectomy were done. In a 10-year review of tracheobronchial granular cell tumors in the Dutch Network and National Database for Pathology, 31 tumors were registered in 30 patients. About half of the patients were asymptomatic. All the tumors described were benign. Eighteen of the 30 patients received no specific treatment. The other patients received either surgery, electrocautery, or Nd-YAG laser treatment. The surgical treatments offered were tracheal resection and subsequent reconstruction (for tracheal tumors) and pneumonectomy or lobectomy for bronchial tumors. The patients who had surgery had remission for up to 10 years, and those with residual disease remained stable (6). Multiple reports have described tumors at myriad other locations (7–10). 1. 2.
3.
4.
Abrikossoff A. Uber Myome, ausgehend von der quergestreiften Willkürlichen muskulatur. Virchows Arch Pathol Anat 1926;260(1):215–233. Rekhi B, Jambhekar NA. Morphologic spectrum, immunohistochemical analysis and clinical features of a series of granular cell tumors of soft tissues: a study from a tertiary referral cancer center. Ann Diagn Pathol 2010;14(3):162–167. Nath D, Gupta A, Arava S, Jain D, Madan K. Synchronous existence of granular cell tumor and small cell carcinoma of the lung: an unusual entity. Indian J Pathol Microbiol 2016;59(1):90–92. Fanburg-Smith JC, Meis-Kindblom JM, Fante R, Kindblom LG. Malignant granular cell tumor of soft tissue: diagnostic criteria and clinicopathologic correlation. Am J Surg Pathol 1998;22(7):779–794.
Baylor University Medical Center Proceedings
Volume 30, Number 3
5.
6.
7.
Kutuya N, Akiduki A. Radiologic appearance of a bronchial granular cell tumor with secondary obstructive changes Clin Imaging 2010;34(2):148– 151. Van der Maten J, Blaauwgeers JLG, Sutedja TG, Kwa HB, Postmus PE, Wagenaar SS. Granular cell tumors of the tracheobronchial tree. J Thorac Cardiovasc Surg 2003;126(3):740–743. Soh WM, Yeong ML, Wong KP. Malignant granular cell tumour of the mediastinum. Malays J Pathol 2014;36(2):149–151.
July 2017
8. Ihara K, Ito H, Nakajima Y, Fukaya E, Nakazawa H, Nozaki M. Granular cell tumor of the suprasternal space. J Dermatol 2010;37(10):900– 903. 9. Nakao M, Hishida T, Ishii G, Yoshida J, Nishimura M, Nagai K. Malignant granular cell tumor of the posterior mediastinum with dissemination. Asian Cardiovasc Thorac Ann 2012;20(1):71–73. 10. Mehrdad MB, Adams S, Catalano PW. A 65 year old woman with granular cell tumor of the lung. Tanaffos 2010;9(1):59–62.
Pleural-based granular cell tumor
321
