IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Pulmonary Artery Intimal Sarcoma Kougaku Umezawa1, Kei Takamura1, Makoto Yamamoto1, and Keisuke Kikuchi2 1
First Department of Medicine and 2Department of Pathology, Obihiro Kosei General Hospital, Obihiro, Japan
Figure 1. On the chest computed tomography scan, upper mediastinal space was occupied with low-density mass, which overcrowded from the pulmonary trunk to peripheral sites with phymatoid enlargement.
An 84-year-old woman was transferred because of dyspnea. She had no past history or family history of sarcoma. Her respiratory sounds were decreased, hypotension was observed, and blood gas analysis showed hypoxemia. Both bilateral hilar lymphadenopathy and Figure 2. (A) Postmortem examination revealed a remarkable dilatation of pulmonary artery and dilated pulmonary artery were suspected mucous tumors (*) that originated from the pulmonary artery trunk. (B) Histologically, proliferation on chest X-ray. On chest computed of undifferentiated fusiform tumor cells with atypical cylindroid nucleus was observed in the tomography scan, the upper mediastinal lumen of the pulmonary artery. (C) Immunohistochemical examination was positive for vimentin, space was occupied with low-density a-SMA (smooth muscle actin), and HHF-35 (common muscle actin). mass, which spread from the pulmonary artery trunk to peripheral branches with phymatoid enlargement (Figure 1A). Although we treated the symptoms, the patient died of respiratory failure on the third hospital day. Postmortem examination revealed a remarkable dilatation of the pulmonary artery, and mucous tumors that originated from the pulmonary artery trunk were observed (Figure 2A). Histologically, proliferation of undifferentiated fusiform tumor cells with atypical cylindroid nucleus was observed in the lumen of the pulmonary artery (Figure 2B). Immunohistochemical examination was positive for vimentin, a-SMA (smooth muscle actin), and HHF-35 (common muscle actin) (Figure 2C). These findings revealed the tumor was a pulmonary artery intimal sarcoma.
Am J Respir Crit Care Med Vol 190, Iss 12, pp e67–e68, Dec 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201407-1311IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
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IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Pulmonary artery sarcoma is a rare tumor of the great vessels. The diagnosis is usually made postoperatively or at autopsy. The average age at presentation is 48 years, with a slight female predominance (1). The most common symptom is dyspnea, followed by chest or back pain, cough, hemoptysis, and malaise (1). Computed tomography findings include a heterogeneous tumoral attenuation resulting in a filling defect that primarily involves the entire luminal diameter of the proximal pulmonary trunk, with variable intravascular extension in one or both pulmonary arteries (2). The prognosis is poor, and mean survival is 12–18 months (3). Surgical resection has been reported to decrease clinical symptoms and lengthen survival (4). Recently, the effectiveness of chemotherapy and radiotherapy have been reported (5). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Burke A, Virami R. Tumors of the great vessels. In: Burke A, Virami R, editors. AFIP atlas of tumor pathology. Washington, DC: AFIP; 1996. pp. 221–227. 2. Timmers L, Bove´ T, De Pauw M. Intimal sarcoma of the pulmonary artery: a report of two cases. Acta Cardiol 2009;64:677–679. 3. Ozbek C, Emrecan B, Calli AO, Gurbuz A. Intimal sarcoma of the pulmonary artery with retrograde extension into the
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pulmonic valve and right ventricle. Tex Heart Inst J 2007;34: 119–121. 4. Mattoo A, Fedullo PF, Kapelanski D, Ilowite JS. Pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up. Chest 2002;122:745–747. 5. Hirose T, Ishikawa N, Hamada K, Inagaki T, Kusumoto S, Shirai T, Okuda K, Ohnishi T, Kadokura M, Adachi M. A case of intimal sarcoma of the pulmonary artery treated with chemoradiotherapy. Intern Med 2009; 48:245–249.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 12 | December 15 2014
First Department of Medicine and 2Department of Pathology, Obihiro Kosei General Hospital, Obihiro, Japan
Figure 1. On the chest computed tomography scan, upper mediastinal space was occupied with low-density mass, which overcrowded from the pulmonary trunk to peripheral sites with phymatoid enlargement.
An 84-year-old woman was transferred because of dyspnea. She had no past history or family history of sarcoma. Her respiratory sounds were decreased, hypotension was observed, and blood gas analysis showed hypoxemia. Both bilateral hilar lymphadenopathy and Figure 2. (A) Postmortem examination revealed a remarkable dilatation of pulmonary artery and dilated pulmonary artery were suspected mucous tumors (*) that originated from the pulmonary artery trunk. (B) Histologically, proliferation on chest X-ray. On chest computed of undifferentiated fusiform tumor cells with atypical cylindroid nucleus was observed in the tomography scan, the upper mediastinal lumen of the pulmonary artery. (C) Immunohistochemical examination was positive for vimentin, space was occupied with low-density a-SMA (smooth muscle actin), and HHF-35 (common muscle actin). mass, which spread from the pulmonary artery trunk to peripheral branches with phymatoid enlargement (Figure 1A). Although we treated the symptoms, the patient died of respiratory failure on the third hospital day. Postmortem examination revealed a remarkable dilatation of the pulmonary artery, and mucous tumors that originated from the pulmonary artery trunk were observed (Figure 2A). Histologically, proliferation of undifferentiated fusiform tumor cells with atypical cylindroid nucleus was observed in the lumen of the pulmonary artery (Figure 2B). Immunohistochemical examination was positive for vimentin, a-SMA (smooth muscle actin), and HHF-35 (common muscle actin) (Figure 2C). These findings revealed the tumor was a pulmonary artery intimal sarcoma.
Am J Respir Crit Care Med Vol 190, Iss 12, pp e67–e68, Dec 15, 2014 Copyright © 2014 by the American Thoracic Society DOI: 10.1164/rccm.201407-1311IM Internet address: www.atsjournals.org
Images in Pulmonary, Critical Care, Sleep Medicine and the Sciences
e67
IMAGES IN PULMONARY, CRITICAL CARE, SLEEP MEDICINE AND THE SCIENCES Pulmonary artery sarcoma is a rare tumor of the great vessels. The diagnosis is usually made postoperatively or at autopsy. The average age at presentation is 48 years, with a slight female predominance (1). The most common symptom is dyspnea, followed by chest or back pain, cough, hemoptysis, and malaise (1). Computed tomography findings include a heterogeneous tumoral attenuation resulting in a filling defect that primarily involves the entire luminal diameter of the proximal pulmonary trunk, with variable intravascular extension in one or both pulmonary arteries (2). The prognosis is poor, and mean survival is 12–18 months (3). Surgical resection has been reported to decrease clinical symptoms and lengthen survival (4). Recently, the effectiveness of chemotherapy and radiotherapy have been reported (5). n Author disclosures are available with the text of this article at www.atsjournals.org.
References 1. Burke A, Virami R. Tumors of the great vessels. In: Burke A, Virami R, editors. AFIP atlas of tumor pathology. Washington, DC: AFIP; 1996. pp. 221–227. 2. Timmers L, Bove´ T, De Pauw M. Intimal sarcoma of the pulmonary artery: a report of two cases. Acta Cardiol 2009;64:677–679. 3. Ozbek C, Emrecan B, Calli AO, Gurbuz A. Intimal sarcoma of the pulmonary artery with retrograde extension into the
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pulmonic valve and right ventricle. Tex Heart Inst J 2007;34: 119–121. 4. Mattoo A, Fedullo PF, Kapelanski D, Ilowite JS. Pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up. Chest 2002;122:745–747. 5. Hirose T, Ishikawa N, Hamada K, Inagaki T, Kusumoto S, Shirai T, Okuda K, Ohnishi T, Kadokura M, Adachi M. A case of intimal sarcoma of the pulmonary artery treated with chemoradiotherapy. Intern Med 2009; 48:245–249.
American Journal of Respiratory and Critical Care Medicine Volume 190 Number 12 | December 15 2014
