- Email: [email protected]
Primary Pulmonary Meningioma Presenting with Hemoptysis on Exertion
References 1. Gamberi G, Serra M, Ragazzini P, et al. Identification of markers of possible prognostic value in 57 giant cell tumors of bone. Oncol Reports 2003;10:351– 6. 2. Zhen W, Yaotian H, Songjian L, et al. Giant cell tumor of bone. The long-term results of treatment by curettage and bone graft. J Bone Joint Surg Br 2004;86-B:212– 6. 3. Mendenhall W, Zlotecki R, Scarborough M, Parker Gibbs C, Mendenhall N. Giant cell tumor of bone. Am J Clin Oncol 2006;29:96 –9. 4. Ochsner A Jr, Lucas GL, Mc Garland GB Jr. Tumors of the thoracic skeleton: review of 134 cases. J Thorac Cardiovasc Surg 1966;52:311–21. 5. Athanassiadi K, Kalavrouziotis G, Rondogianni D, Loutsidis A, Hatzimichalis A, Bellenis I. Primary chest wall tumors: early and long-term results of surgical treatment. Eur J Cardiothorac Surg 2001;19:589 –93. 6. Sabanathan S, Salama FD, Morgan WE, Harvey JA. Primary chest wall tumors. Ann Thorac Surg 1985;39:4 –15.
Primary Pulmonary Meningioma Presenting with Hemoptysis on Exertion Nobuhiro Izumi, MD, Noritoshi Nishiyama, MD, Takashi Iwata, MD, Koshi Nagano, MD, Takuma Tsukioka, MD, Shoji Hanada, MD, and Shigefumi Suehiro, MD Department of Thoracic Surgery, Osaka City University Hospital, Osaka, Japan
An 18-year-old woman presented with hemoptysis during long-distant running. A chest computed tomographic scan revealed a well-demarcated, round-shaped, solitary mass (3.0 cm in diameter) in the left pulmonary hilum. The bleeding was getting more often and a left upper lobectomy was carried out to avoid critical airway bleeding. A solid proliferation of short spindle cells with a remarkable whorl pattern was demonstrated histologically. Immunohistochemical studies showed positive for vimentin and S-100, and focally positive for epithelial membrane antigen. Primary pulmonary meningioma was thus diagnosed. The patient is well without recurrent disease 15 months after surgery. (Ann Thorac Surg 2009;88:647– 8) © 2009 by The Society of Thoracic Surgeons
CASE REPORT IZUMI ET AL PULMONARY MENINGIOMA PRESENTING WITH HEMOPTYSIS
647
hemoptysis on exertion that was successfully treated by surgical resection. An 18-year-old woman presented with hemoptysis on exertion, especially during long-distant running. She was otherwise healthy and had no history of pulmonary or neurologic disturbances. A chest roentgenogram showed a solitary mass of 3.0 cm in diameter in the left pulmonary hilum. A chest computed tomographic scan revealed a well-demarcated, round-shaped, and homogenous lesion without showing any cavity lesions or densities of fatty or calcified contents, or both, in the left upper lobe adjacent to the pulmonary artery and vein (Fig 1). Brain and abdominal computed tomographic scans demonstrated no evidence of metastasis. Values of carcinoembryonic antigen and squamous cell carcinomaassociated antigen in serum were within normal ranges. Diagnostic bronchoscopy or computed tomographicguided needle biopsy of the tumor was refrained from because of possible massive bleeding from the hilar vessels or the hemorrhagic tumor itself during the procedures. Furthermore, the bleeding was getting easier and more frequent; therefore, pulmonary resection was scheduled to prevent further acute bleeding, without confirming definitive histologic diagnosis. Preoperative differential diagnoses were hamartoma, sclerosing hemangioma, or a metastatic tumor of unknown origin. A left upper lobectomy was carried out through a small axillary thoracotomy of 5 cm with video assistance. Diffuse telangiectasis was seen on the visceral pleura of the upper lobe. A resected specimen showed an elastic soft, well-demarcated tumor, measuring 33 mm in diameter. The cut surface was yellowish white, soft, and fragile. An intraoperative frozen section revealed a spindle-cell tumor partially containing atypical epithelial components. A definitive diagnosis of benign or malignant disease was not obtainable; therefore, mediastinal dissection was also undertaken in case of possible epithelial malignancy. Postoperative histologic examination showed the lesion consisted mainly of a solid proliferation of short-spindle
P
rimary pulmonary meningioma (PPM) is an extremely rare entity [1]. Since Erlandson described the first case in 1981, only 33 cases have been reported in the English literature [2]. Most of the cases were asymptomatic. We report herein a case of PPM presenting with
Accepted for publication Dec 15, 2008. Address correspondence to Dr Iwata, Department of Thoracic Surgery, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Chest computed tomographic scan shows a solitary, well-demarcated, round-shaped and homogenous 3.0 cm diameter tumor in the hilum of the left upper lobe, adjacent to the pulmonary artery and vein. No cavity lesion or any density of fatty or calcified contents are seen. 0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2008.12.058
FEATURE ARTICLES
Ann Thorac Surg 2009;88:647– 8
648
CASE REPORT IZUMI ET AL PULMONARY MENINGIOMA PRESENTING WITH HEMOPTYSIS
Fig 2. Pathologic findings of the resected pulmonary meningioma. The lesion mainly consists of a solid proliferation of short spindle cells, involving a remarkable whorl pattern with a slight degree of difference in the sizes of the nuclei. (Hematoxylin & eosin; ⫻200.)
FEATURE ARTICLES
cells with a remarkable whorl pattern (Fig 2). A slight degree of difference in sizes of the nuclei was observed, but no atypical cells and no mitotic figures were found. Psammoma bodies were not seen in the specimen. Immunohistochemical studies were positive for vimentin and S-100. Epithelial markers, such as CAM 5.2, AE1/ AE3, and Ki-67, were negative, whereas epithelial membrane antigen was focally positive. Mesenchymal markers, such as CD-34, desmin, and ␣-smooth muscle actin were not expressed at all. The ALK-1 and c-kit were also negative. The CD-68 stain was positive. Cerebral or spinal involvement was excluded by magnetic resonance imaging studies. Thus, the diagnosis was confirmed as a PPM without malignant characteristics. The patient is well without symptoms or recurrent disease 15 months after surgery.
Comment The cause of PPMs remains unknown. Most PPMs have been found incidentally on routine chest roentgenogram without any clinical symptoms, and presented as a single and well-circumscribed nodule on radiologic studies [1]. Only 2 patients, including our case, have been reported to be symptomatic, both presenting with hemoptysis [3]. No specific findings on radiographic or blood chemical studies have been reported. High metabolic activity on 18-fluorodeoxyglucose positron emission tomography was reported [4]. However, we did not perform a positron emission tomographic scan to rule out malignancy for our case because a lobectomy should have been carried out to avoid further bleeding, regardless of whether the tumor was malignant or benign. Invasive diagnostic procedures that would lead to massive airway bleeding, such as bronchoscopic or percutaneous computed tomographic-guided needle biopsy of the tumor, were also refrained from, because the tumor was bleeding easily
Ann Thorac Surg 2009;88:647– 8
and was surrounded by hilar vessels. Our case was thus problematic to obtain preoperative diagnosis. Mechanism of acute bleeding from the tumor is also unknown. In our case, histologic examination showed little vascularity in the tumor. However, we recognized diffuse telangiectasia over the whole visceral pleura during the operation, suggesting possible congestion of the left upper lobe. In addition, the tumor was soft and fragile, thus the stretching of such a congestive hilar anatomical structure and tumor tissue itself might have resulted in hemoptysis on exertion. The origin of PPMs is not fully understood. Ectopic embryonic remnants, meningothelial erroneous differentiation of the pluripotential stem cells, or so-called minute pulmonary meningothelial-like nodule have been considered as its origin [5]. Most PPMs are reported as benign conditions, except three malignant cases [2]. Benign ectopic, or extracranial meningiomas, including PPMs, share similar histologic characteristics with intracranial and intraspinal meningiomas having syncytial groups, fascicles, and whorled cellular arrangements, and most of them had varying number of psammoma bodies [1]. The immunologic profile of benign ectopic meningiomas is characterized by positive staining with antibodies to vimentin and epithelial membrane antigen, and there is a variable degree of expression of staining for S-100 and cytokeratin [1]. The histologic differential diagnosis includes pulmonary chemodectoma, solitary fibrous tumor, and neurinoma. Pulmonary chemodectomas are relatively common, small (⬍2 mm) and frequently multiple, and they are usually only found incidentally by microscope, and they occur in the vicinity of the veins in the interstitium [6]. Solitary fibrous tumors of the lung do not have a whorled meningiotheliomatous appearance, and they are vimentin and CD-34 positive but S-100 negative. Neurinomas demonstrate strongly positive S-100, but are negative. Surgical resection is the best treatment option and prognosis after complete surgical removal is excellent [1].
References 1. Lockett L, Chiang V, Scully N. Primary pulmonary meningioma: report of a case and review of the literature. Am J Surg Pathol 1997;21:453– 60. 2. van der Meij JJ, Boomars KA, van den Bosch JM, van Boven WJ, de Bruin PC, Seldenrijk CA. Primary pulmonary malignant meningioma. Ann Thorac Surg 2005;80:1523–5. 3. Zhang FL, Cheng XR, Zhang YS, Ding JA. Lung ectopic meningioma: a case report. Chin Med J (Engl) 1983;96:309 –11. 4. Meirelles GS, Ravizzini G, Moreira AL, Akhurst T. Primary pulmonary meningioma manifesting as a solitary pulmonary nodule with a false-positive PET scan. J Thorac Imaging 2006;21:225–7. 5. Rowsell C, Sirbovan J, Rosenblum MK, Perez-Ordonez B. Primary chordoid meningioma of lung. Virchows Arch 2005; 446:333–7. 6. Kodama K, Doi O, Higashiyama M, Horai T, Tateishi R, Nakagawa H. Primary and metastatic pulmonary meningioma. Cancer 1991;67:1412–7.
Primary Pulmonary Meningioma Presenting with Hemoptysis on Exertion Nobuhiro Izumi, MD, Noritoshi Nishiyama, MD, Takashi Iwata, MD, Koshi Nagano, MD, Takuma Tsukioka, MD, Shoji Hanada, MD, and Shigefumi Suehiro, MD Department of Thoracic Surgery, Osaka City University Hospital, Osaka, Japan
An 18-year-old woman presented with hemoptysis during long-distant running. A chest computed tomographic scan revealed a well-demarcated, round-shaped, solitary mass (3.0 cm in diameter) in the left pulmonary hilum. The bleeding was getting more often and a left upper lobectomy was carried out to avoid critical airway bleeding. A solid proliferation of short spindle cells with a remarkable whorl pattern was demonstrated histologically. Immunohistochemical studies showed positive for vimentin and S-100, and focally positive for epithelial membrane antigen. Primary pulmonary meningioma was thus diagnosed. The patient is well without recurrent disease 15 months after surgery. (Ann Thorac Surg 2009;88:647– 8) © 2009 by The Society of Thoracic Surgeons
CASE REPORT IZUMI ET AL PULMONARY MENINGIOMA PRESENTING WITH HEMOPTYSIS
647
hemoptysis on exertion that was successfully treated by surgical resection. An 18-year-old woman presented with hemoptysis on exertion, especially during long-distant running. She was otherwise healthy and had no history of pulmonary or neurologic disturbances. A chest roentgenogram showed a solitary mass of 3.0 cm in diameter in the left pulmonary hilum. A chest computed tomographic scan revealed a well-demarcated, round-shaped, and homogenous lesion without showing any cavity lesions or densities of fatty or calcified contents, or both, in the left upper lobe adjacent to the pulmonary artery and vein (Fig 1). Brain and abdominal computed tomographic scans demonstrated no evidence of metastasis. Values of carcinoembryonic antigen and squamous cell carcinomaassociated antigen in serum were within normal ranges. Diagnostic bronchoscopy or computed tomographicguided needle biopsy of the tumor was refrained from because of possible massive bleeding from the hilar vessels or the hemorrhagic tumor itself during the procedures. Furthermore, the bleeding was getting easier and more frequent; therefore, pulmonary resection was scheduled to prevent further acute bleeding, without confirming definitive histologic diagnosis. Preoperative differential diagnoses were hamartoma, sclerosing hemangioma, or a metastatic tumor of unknown origin. A left upper lobectomy was carried out through a small axillary thoracotomy of 5 cm with video assistance. Diffuse telangiectasis was seen on the visceral pleura of the upper lobe. A resected specimen showed an elastic soft, well-demarcated tumor, measuring 33 mm in diameter. The cut surface was yellowish white, soft, and fragile. An intraoperative frozen section revealed a spindle-cell tumor partially containing atypical epithelial components. A definitive diagnosis of benign or malignant disease was not obtainable; therefore, mediastinal dissection was also undertaken in case of possible epithelial malignancy. Postoperative histologic examination showed the lesion consisted mainly of a solid proliferation of short-spindle
P
rimary pulmonary meningioma (PPM) is an extremely rare entity [1]. Since Erlandson described the first case in 1981, only 33 cases have been reported in the English literature [2]. Most of the cases were asymptomatic. We report herein a case of PPM presenting with
Accepted for publication Dec 15, 2008. Address correspondence to Dr Iwata, Department of Thoracic Surgery, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka, 545-8585, Japan; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. Chest computed tomographic scan shows a solitary, well-demarcated, round-shaped and homogenous 3.0 cm diameter tumor in the hilum of the left upper lobe, adjacent to the pulmonary artery and vein. No cavity lesion or any density of fatty or calcified contents are seen. 0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2008.12.058
FEATURE ARTICLES
Ann Thorac Surg 2009;88:647– 8
648
CASE REPORT IZUMI ET AL PULMONARY MENINGIOMA PRESENTING WITH HEMOPTYSIS
Fig 2. Pathologic findings of the resected pulmonary meningioma. The lesion mainly consists of a solid proliferation of short spindle cells, involving a remarkable whorl pattern with a slight degree of difference in the sizes of the nuclei. (Hematoxylin & eosin; ⫻200.)
FEATURE ARTICLES
cells with a remarkable whorl pattern (Fig 2). A slight degree of difference in sizes of the nuclei was observed, but no atypical cells and no mitotic figures were found. Psammoma bodies were not seen in the specimen. Immunohistochemical studies were positive for vimentin and S-100. Epithelial markers, such as CAM 5.2, AE1/ AE3, and Ki-67, were negative, whereas epithelial membrane antigen was focally positive. Mesenchymal markers, such as CD-34, desmin, and ␣-smooth muscle actin were not expressed at all. The ALK-1 and c-kit were also negative. The CD-68 stain was positive. Cerebral or spinal involvement was excluded by magnetic resonance imaging studies. Thus, the diagnosis was confirmed as a PPM without malignant characteristics. The patient is well without symptoms or recurrent disease 15 months after surgery.
Comment The cause of PPMs remains unknown. Most PPMs have been found incidentally on routine chest roentgenogram without any clinical symptoms, and presented as a single and well-circumscribed nodule on radiologic studies [1]. Only 2 patients, including our case, have been reported to be symptomatic, both presenting with hemoptysis [3]. No specific findings on radiographic or blood chemical studies have been reported. High metabolic activity on 18-fluorodeoxyglucose positron emission tomography was reported [4]. However, we did not perform a positron emission tomographic scan to rule out malignancy for our case because a lobectomy should have been carried out to avoid further bleeding, regardless of whether the tumor was malignant or benign. Invasive diagnostic procedures that would lead to massive airway bleeding, such as bronchoscopic or percutaneous computed tomographic-guided needle biopsy of the tumor, were also refrained from, because the tumor was bleeding easily
Ann Thorac Surg 2009;88:647– 8
and was surrounded by hilar vessels. Our case was thus problematic to obtain preoperative diagnosis. Mechanism of acute bleeding from the tumor is also unknown. In our case, histologic examination showed little vascularity in the tumor. However, we recognized diffuse telangiectasia over the whole visceral pleura during the operation, suggesting possible congestion of the left upper lobe. In addition, the tumor was soft and fragile, thus the stretching of such a congestive hilar anatomical structure and tumor tissue itself might have resulted in hemoptysis on exertion. The origin of PPMs is not fully understood. Ectopic embryonic remnants, meningothelial erroneous differentiation of the pluripotential stem cells, or so-called minute pulmonary meningothelial-like nodule have been considered as its origin [5]. Most PPMs are reported as benign conditions, except three malignant cases [2]. Benign ectopic, or extracranial meningiomas, including PPMs, share similar histologic characteristics with intracranial and intraspinal meningiomas having syncytial groups, fascicles, and whorled cellular arrangements, and most of them had varying number of psammoma bodies [1]. The immunologic profile of benign ectopic meningiomas is characterized by positive staining with antibodies to vimentin and epithelial membrane antigen, and there is a variable degree of expression of staining for S-100 and cytokeratin [1]. The histologic differential diagnosis includes pulmonary chemodectoma, solitary fibrous tumor, and neurinoma. Pulmonary chemodectomas are relatively common, small (⬍2 mm) and frequently multiple, and they are usually only found incidentally by microscope, and they occur in the vicinity of the veins in the interstitium [6]. Solitary fibrous tumors of the lung do not have a whorled meningiotheliomatous appearance, and they are vimentin and CD-34 positive but S-100 negative. Neurinomas demonstrate strongly positive S-100, but are negative. Surgical resection is the best treatment option and prognosis after complete surgical removal is excellent [1].
References 1. Lockett L, Chiang V, Scully N. Primary pulmonary meningioma: report of a case and review of the literature. Am J Surg Pathol 1997;21:453– 60. 2. van der Meij JJ, Boomars KA, van den Bosch JM, van Boven WJ, de Bruin PC, Seldenrijk CA. Primary pulmonary malignant meningioma. Ann Thorac Surg 2005;80:1523–5. 3. Zhang FL, Cheng XR, Zhang YS, Ding JA. Lung ectopic meningioma: a case report. Chin Med J (Engl) 1983;96:309 –11. 4. Meirelles GS, Ravizzini G, Moreira AL, Akhurst T. Primary pulmonary meningioma manifesting as a solitary pulmonary nodule with a false-positive PET scan. J Thorac Imaging 2006;21:225–7. 5. Rowsell C, Sirbovan J, Rosenblum MK, Perez-Ordonez B. Primary chordoid meningioma of lung. Virchows Arch 2005; 446:333–7. 6. Kodama K, Doi O, Higashiyama M, Horai T, Tateishi R, Nakagawa H. Primary and metastatic pulmonary meningioma. Cancer 1991;67:1412–7.