Tuberc Respir Dis.  2012 Feb;72(2):218-222.

A Case of Pulmonary Artery Intimal Sarcoma Masquerading as Pulmonary Embolism

Affiliations
  • 1Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea. LeeSS@paik.ac.kr
  • 2Department of Radiology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
  • 3Department of Pathology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
  • 4Department of Cardiosurgery, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
  • 5Department of Radiation Oncology, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.

Abstract

Pulmonary artery intimal sarcoma is a rare tumor with no characteristic symptoms. It is frequently misdiagnosed as pulmonary embolism. We report a case of pulmonary artery intimal sarcoma in a 48-year-old man with dyspnea, cough and blood-tinged sputum. He was initially suspected and treated as a pulmonary embolism. Computed tomography of the chest showed filling defects occupying the entire luminal diameter of the right and left pulmonary artery as well as extraluminal extension of the intraluminal mass. Surgical resection of the tumor confirmed pulmonary artery intimal sarcoma. After surgery, he received 8 cycles of combined chemotherapy consisting of doxorubicin and ifosfamide. After 8 cycles, Computed tomography of the chest showed interval regression of the residual tumor. Radiotherapy was done as total 6,000 cGy for 5 weeks, following the 8th chemotherapy. The patient's condition was successfully stabilized with chemotherapy and radiotherapy.

Keyword

Vascular Neoplasms; Sarcoma; Pulmonary Embolism; General Surgery; Chemotherapy, Adjuvant

MeSH Terms

Chemotherapy, Adjuvant
Cough
Doxorubicin
Dyspnea
Humans
Ifosfamide
Middle Aged
Neoplasm, Residual
Phenobarbital
Pulmonary Artery
Pulmonary Embolism
Sarcoma
Sputum
Thorax
Vascular Neoplasms
Doxorubicin
Ifosfamide
Phenobarbital

Figure

  • Figure 1 Chest radiograph on admission showed poorly-defined multiple nodular and mass-like opacities in both lower lung fields. Dilatation of both pulmonary arterial shadows were suspected.

  • Figure 2 Axial enhanced CT scans obtained with pulmonary embolism protocol showed multifocal low attenuating filling defects within the proximal left pulmonary artery (A), distal right and left pulmonary arteries, left lower lobar artery and right middle and lower lobar arteries (B, C). Main lesion was luminal expansile (arrow). Subpleural nodular consolidations were seen in the right middle lobe and left lower lobe (D). CT: computed tomography.

  • Figure 3 (A~C) Axial fusion images of FDG-PET/CT showed multifocal strong FDG uptake, corresponding to the filling defects on CT scan. (D) Maximum intensity projection image of FDG-PET scan showed increased uptake of FDG in the left pulmonary hilar lesion. FDG: fluorodeoxyglucose positron; PET: emission tomography; CT: computed tomography.

  • Figure 4 The anaplastic tumor cells appear storiform-patterned arrangement (H&E stain, ×200).


Reference

1. Delany SG, Doyle TC, Bunton RW, Hung NA, Joblin LU, Taylor DR. Pulmonary artery sarcoma mimicking pulmonary embolism. Chest. 1993. 103:1631–1633.
2. 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.
3. Head HD, Flam MS, John MJ, Lipnik SS, Slater DL, Stewart RD. Long-term palliation of pulmonary artery sarcoma by radical excision and adjuvant therapy. Ann Thorac Surg. 1992. 53:332–334.
4. Hirose T, Ishikawa N, Hamada K, Inagaki T, Kusumoto S, Shirai T, et al. A case of intimal sarcoma of the pulmonary artery treated with chemoradiotherapy. Intern Med. 2009. 48:245–249.
5. Genoni M, Biraima AM, Bode B, Shan AC, Wilkler MB, Turina MI. Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk. J Cardiovasc Surg (Torino). 2001. 42:829–833.
6. Blackmon SH, Rice DC, Correa AM, Mehran R, Putnam JB, Smythe WR, et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg. 2009. 87:977–984.
7. Xu Y, Wang K, Geng Y, Shao Y, Yin Y. A case of intimal sarcoma of the pulmonary artery successfully treated with chemotherapy. Int J Clin Oncol. 2011. 10. 27. [Epub]. DOI: 10.1007/s10147-011-0338-8.
8. Mandelstamm M. Über primäre neubildungen des herzens. Virchows Archiv. 1923. 245:43–54.
9. Furest I, Marn M, Escribano P, Gómez MA, Cortinac J, Blanquer R. Intimal sarcoma of the pulmonary artery: a rare cause of pulmonary hypertension. Arch Bronconeumol. 2006. 42:148–150.
10. Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg. 1995. 59:1487–1490.
11. Mayer E, Kriegsmann J, Gaumann A, Kauczor HU, Dahm M, Hake U, et al. Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg. 2001. 121:77–82.
12. Krüger I, Borowski A, Horst M, de Vivie ER, Theissen P, Gross-Fengels W. Symptoms, diagnosis, and therapy of primary sarcomas of the pulmonary artery. Thorac Cardiovasc Surg. 1990. 38:91–95.
13. Yi CA, Lee KS, Choe YH, Han D, Kwon OJ, Kim S. Computed tomography in pulmonary artery sarcoma: distinguishing features from pulmonary embolic disease. J Comput Assist Tomogr. 2004. 28:34–39.
14. Choi EY, Yoon YW, Kwon HM, Kim D, Park BE, Hong YS, et al. A case of pulmonary artery intimal sarcoma diagnosed with multislice CT scan with 3D reconstruction. Yonsei Med J. 2004. 45:547–551.
15. Tueller C, Fischer Biner R, Minder S, Gugger M, Stoupis C, Krause TM, et al. FDG-PET in diagnostic work-up of pulmonary artery sarcomas. Eur Respir J. 2010. 35:444–446.
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