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Year : 2013  |  Volume : 56  |  Issue : 1  |  Page : 47-50
Pulmonary arterial intimal sarcoma with retrograde extension: Report of a case and review of literature

Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Mumbai, Maharashtra, India

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Date of Web Publication6-Aug-2013


Most of the pulmonary arterial sarcomas arise from multi-potential mesenchymal intimal cells and are designated as intimal sarcomas. These tumors grow in the direction of blood flow into peripheral arteries producing clinical features mimicking pulmonary thromboembolism. Retrograde extension is rare. We report one such case of intimal sarcoma that had a retrograde extension into the right ventricular outflow tract, and review such a presentation in the last ten years.

Keywords: Intimal sarcoma, pulmonary artery, retrograde extension, vascular sarcoma

How to cite this article:
Vaideeswar P, Pillai R. Pulmonary arterial intimal sarcoma with retrograde extension: Report of a case and review of literature. Indian J Pathol Microbiol 2013;56:47-50

How to cite this URL:
Vaideeswar P, Pillai R. Pulmonary arterial intimal sarcoma with retrograde extension: Report of a case and review of literature. Indian J Pathol Microbiol [serial online] 2013 [cited 2021 Dec 4];56:47-50. Available from: https://www.ijpmonline.org/text.asp?2013/56/1/47/116149

   Introduction Top

Sarcomas of the great vessels, in contrast to their soft tissue counterparts, are uncommon and affect inferior vena cava (IVC), pulmonary artery (PA) and aorta, in that order. The pathological classification of vascular sarcomas is basically made on the location of the tumors in relation to their walls, i.e., intraluminal and mural. [1] Most sarcomas of IVC are mural leiomyosarcomas, while PA and aortic tumors are intraluminal, intimal sarcomas. [2] In general, intimal sarcomas are thought to arise from multi-potential mesenchymal intimal cells, capable of diverse morphological differentiation. They are characterized by multi-focal gelatinous, polypoidal nodules or plaques, composed in most instances, of spindle or epithelioid cells with marked nuclear atypia. The incidence of pulmonary arterial intimal sarcomas (PAIS) is almost twice that of aortic sarcomas and the tumor grows insidiously in the direction of blood flow into peripheral arteries producing vascular obstruction; this clinically and radiologically mimics acute or chronic pulmonary thromboembolism (PTE). [1],[2] Less commonly, they grow in a retrograde manner to involve the right ventricular outflow tract. [3] We report a PAIS with extension to the pulmonary valve and formation of large sub-pulmonic nodule, and review such a presentation in the last ten years.

   Case Report Top

A 55-year-old woman was admitted with progressive shortness of breath, fever off and on, and mild intermittent chest pain for ten days. There had also been a history of orthopnea and paroxysmal nocturnal dyspnea. She had undergone an abdominal hysterectomy for uterine prolapse ten years ago, and was detected to be a diabetic (fasting blood glucose 231 mg%) two months back. On examination, she was afebrile with a pulse rate of 72 per minute, systolic blood pressure of 100 mm Hg, raised jugular venous pressure, pallor and clubbing. The clinical diagnosis was PTE. However, her dyspnea worsened and she succumbed before further investigations could be performed.

At autopsy, the heart (260 gm) showed a broad-based roughly pyramidal firm and friable 4 × 2.7 × 2.1 cm mass in the right ventricular outflow tract that protruded into pulmonary valvular orifice [Figure 1]. All the pulmonary valvular cusps were shortened, markedly thickened with rolled margins; the intima of the pulmonary trunk showed a leathery diffuse white thickening and could be peeled off at places [Figure 1]. This thickening (plaque-like at places) extended into the right and left branches, which were occluded by reddish-brown thrombi; these were also seen in most of the intrapulmonary arterial branches [Figure 2]. The gross impression had been of a mural infective endocarditis of possible fungal etiology with pulmonary thrombo-embolism. The histology was a surprise as it showed pleomorphic spindle cells in varying patterns [Figure 3], amidst abundant fibrinous material, organized and recanalized thrombi, clusters of hemosiderophages and inflammatory cells [Figure 4]. The spindle cells were positive only for vimentin [Figure 4]; desmin, smooth muscle actin and CD 34 were negative. A diagnosis of pulmonary intimal sarcoma with a retrograde extension was made.
Figure 1: The image in the center shows large broad-based polypoid mass M in right ventricular RV outflow tract. Note the peeling off (arrow) of markedly thickened inti ma of pulmonary trunk PT. Secti ons stained by hematoxylin and eosin of areas a (left image) and b (right image) shows thickening and shortening of the pulmonary valve cusps (arrows), fi lling of their sinuses by tumor, supravalvular and sub-pulmonic tumor masses M (IVS interventricular septum)

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Figure 2: (a) The pulmonary trunk PT, and its right RPA and left LPA branches show thickening of their walls and are occluded by luminal reddish brown thromboti c material with peripheral grayish-white areas seen in some cross secti ons (arrows); The lumina of the RPA; (b) At the lung hilumand; (c) Its branches within the lung are obliterated by light brown to golden brownthrombi

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Figure 3: The tumor was composed mainly of pleomorphic spindle-shaped cells. (a) Paucicellular areas with large areas of collagenizati on; (b) Mild cellularity; (c) Hypercellularity; (d) Spindle cell are barely seen due to
extensive lymphocyti c infi ltrate. (Hematoxylin and eosin ×250)

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Figure 4: (a) Deposits of fibrin are present between tumor and inflammatory cells (Hematoxylin and eosinƒn×250); (b) Large numbers of hemosiderin-laden macrophages scattered amidst tumor cells (Hematoxylin and eosinƒn ×250); (c) Pleomorphic spindle cells showing positi vity for vimenti n (Hematoxylin and eosinƒn×400)

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   Discussion Top

PAIS is an uncommon malignant neoplasm affecting the cardiovascular system and since its first description at autopsy by Mandelstamm in 1923, only a few hundred cases have been reported in literature. [2] The incidence is said to be 0.001%, but it is likely that the true prevalence is underestimated because of its difficult diagnosis. [1],[2] A roughly equal gender distribution is now reported, though previously slight female predominance was said to occur. [2] The average age at diagnosis was 49.3 years with a wide age range of 13 to 81 years. [2] Our patient was a woman aged 52 years. The natural course of PAIS is largely determined by the extent of luminal extension into intra-parenchymal arteries; they have little predisposition to metastasize. Hence patients can have an asymptomatic period, before developing symptoms such as dyspnea, pleuritic chest pain, cough and hemoptysis, which is almost always misinterpreted as chronic PTE. [4] Moreover, this logical initial diagnosis leads to inappropriate anticoagulant or thrombolytic therapy.

The clinical diagnosis in the index case was also PTE as the tumor was found to be entirely confined to pulmonary trunk, its extrapulmonary and intrapulmonary branches. An added feature was involvement of pulmonary valve and formation of a large right ventricular outflow tract nodule. Such a retrograde extension, seen in about 10% of patients, is an added feature of PAIS. In the last ten years (2002 to 2011, [Table 1]), there have been reports of 12 patients with such a phenomenon. [1],[2],[3],[4],[5],[6],[7],[8],[9],[10] The mean age was 51.3 years; gender of three patients has not been mentioned in one report. [10] More than half of the patients had been diagnosed and treated as chronic PTE with pulmonary valvular extension and/or right ventricular extension. One had serpentine tumor extending even into superior vena cava; valve was not involved. [9] Recurrent tumors were present in one patient. [3] Unusual feature noted in the index case was that the tumor outside and within the lungs did not have the characteristic gelatinous appearance and was uniformly admixed with fresh and organizing thrombi with clusters of hemosiderophages. This can sometimes occur and may explain partial response to anticoagulants and/or thrombolytic therapy. [2] A glistening and rugose appearance was noted over the pulmonary trunk and the valve. On histology, the tumor was composed of moderately cellular spindled cells, which were only vimentin positive. Most of the intimal sarcomas generally show myofibroblastic differentiation; endothelial markers are negative; however, foci of specific mesenchymal differentiation can sometimes be seen. [1],[7] Low-grade tumors can mimic cellular foci of organizing thrombi.
Table 1: Pulmonary arterial inti mal sarcomas with retrograde extension: 10-year review

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Confirmation of PAIS by radiological investigations also poses a problem as these modalities often reveal non-specific abnormalities, all of which again point out to PTE. [6] Suspicious findings include heterogeneous soft tissue density on CT and magnetic resonance imaging, "to and fro" motion of an irregular pedunculated mass lesion on angiography. [6] The prognosis of PAIS is extremely poor, with a median survival time without therapy of only 1.5 months. The main stay of treatment is surgical resection which lengthens survival and the right type of surgical option would depend on the extent of tumor; adjuvant chemotherapy and radiotherapy may also be offered. [6],[10] Among the 12 patients with retrograde extension, endarterectomies had been performed in all with outflow tract reconstructions in seven. [6],[7],[8],[9],[10] Five [4],[5],[6],[8],[9] also received chemotherapy. A follow-up of more than 12 months was available in four patients [4],[6],[7],[9] with one death. [9] To conclude, improved survival can be achieved when diagnosis of PAIS is made early and this diagnosis should be kept in mind in cases of PTE with poor response to anticoagulation and/or formation of mass lesions in the right ventricular outflow tract. It would also be important to sample all the pulmonary endarterectomies completely.

   Acknowledgments Top

The authors are grateful to Dr. Sanjay Navani, LabSurg Path, Mumbai, India for the immunohistochemistry services.

   References Top

1.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;45:547-51.  Back to cited text no. 1
2.Alsoufi B, Slater M, Smith PP, Karamlou T, Mansoor A, Ravichandran P. Pulmonary artery sarcoma mimicking massive pulmonary embolus: A case report. Asian Cardiovasc Thorac Ann 2006;14:71-3.  Back to cited text no. 2
3.Ozbek C, Emrecan B, Calli AO, Gurbuz A. Intimal sarcoma of the pulmonary artery with retrograde extension to the pulmonary valve and right ventricle. Tex Heart Inst J 2007;34:119-21.  Back to cited text no. 3
4.Viana-Tejedor A, Marino-Enriquez A, Sanchez-Recalde A, Lopez-Sendona JL. Intimal sarcoma of the pulmonary artery: Diagnostic value of different imaging techniques. Rev EspCardiol 2008;61:1355-65.  Back to cited text no. 4
5.Bloomberg RD, Butany JW, Cusimano RJ, Leask RL. Primary cardiac sarcoma involving the pulmonary artery and valve. Can J Cardiol 2003;19:843-7.  Back to cited text no. 5
6.Fernández-Golfín C, Escribano P, Cortina J, Tello R, Hernández F, López-RiosF, et al. Management of primary pulmonary artery sarcoma: Experience of a single center. Angiology 2008;59:636-9.  Back to cited text no. 6
7.Hou Y, Shen Z, Gao W, Ye W. Pulmonary artery intimal sarcoma: Case report. J Card Surg 2010;25:29-31.  Back to cited text no. 7
8.Shah DK, Joyce LD, Grogan M, Aubry MC, Miller JA, Ding W, et al. Recurrent pulmonary intimal sarcoma involving the right ventricular outflow tract. Ann Thorac Surg 2011;91:e41-2.  Back to cited text no. 8
9.Portillo-Sanchez J, Hessein-Abdou Y, Puga-Alcalde E, Perez-Martinez MA, Jimenez-Meneses MD, Camacho-Pedrero A, et al. Primary pulmonary artery sarcoma extending retrograde into the superior vena cava. Tex Heart Inst J 2011;38:77-80.  Back to cited text no. 9
10.Hui X, Qing-yu W. Surgical treatment of primary pulmonary artery sarcoma. Chin Med J 2011;124:461-3.  Back to cited text no. 10

Correspondence Address:
Pradeep Vaideeswar
Department of Pathology (Cardiovascular and Thoracic Division), Seth GS Medical College, Parel, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.116149

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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