Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 5  |  Issue : 2  |  Page : 111-114

Intimal sarcoma of the popliteal artery presenting as popliteal artery aneurysm: A rare case report


Department of Vascular and Endovascular Surgery, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication3-May-2018

Correspondence Address:
Dr. Mukesh Kumar Garg
Department of Vascular and Endovascular Surgery, Sir Ganga Ram Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_49_17

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  Abstract 


Intimal sarcoma is a highly aggressive and rare malignant tumor of the vascular system. We are reporting this unusual primary tumor of popliteal artery presenting as aneurysm in an infant. We report a case of an 8-month-old girl with the incidental observation of nontender and pulsatile swelling in the right popliteal fossa. An arterial duplex scan and computed tomography angiography revealed an intensely enhancing well-defined lesion (3.2 cm × 2.4 cm) of the popliteal artery suggestive of pseudoaneurysm/aneurysm. The patient underwent exploration of the popliteal artery with excision of aneurysmal sac. On histopathological examination and immunohistochemical staining, findings were suggestive of intimal sarcoma. Postoperative recovery was uneventful. At 1-year of follow-up, patient is clinically stable with no disability and any signs of recurrence or metastasis. Malignancy in peripheral arterial aneurysmal is extremely rare. More empirical evidence is yet required to make the diagnostic and therapeutic protocol to deal with this aggressive tumor of the vascular system.

Keywords: Aneurysm, intimal sarcoma, popliteal artery


How to cite this article:
Garg MK, Satwik A, Yadav A, Agarwal S, Bedi VS. Intimal sarcoma of the popliteal artery presenting as popliteal artery aneurysm: A rare case report. Indian J Vasc Endovasc Surg 2018;5:111-4

How to cite this URL:
Garg MK, Satwik A, Yadav A, Agarwal S, Bedi VS. Intimal sarcoma of the popliteal artery presenting as popliteal artery aneurysm: A rare case report. Indian J Vasc Endovasc Surg [serial online] 2018 [cited 2019 Jun 16];5:111-4. Available from: http://www.indjvascsurg.org/text.asp?2018/5/2/111/231848




  Introduction Top


Intimal sarcoma is a highly aggressive malignant tumor of the vascular system. Patients generally present with thrombotic or embolic complications of the tumor.[1] We are reporting this unusual primary tumor of the popliteal artery, in an infant, presenting as a popliteal artery aneurysm.


  Case Report Top


An 8-month-old girl was brought to our institution by her parents with incidentally observed swelling in the right popliteal fossa. There was no antecedent history of trauma. On examination, the swelling was nontender, pulsatile, and confined to the right popliteal fossa [Figure 1].
Figure 1: Clinical photograph of the child with bulge visible on right popliteal fossa

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An arterial duplex scan was performed which showed a heterogeneous pulsatile mass involving the right popliteal artery suggestive of a thick-walled aneurysm [Figure 2]. Distal arterial flows in the limb were biphasic with normal velocities (as compared to the opposite limb). Computed tomography (CT) angiography findings reveal an intensely enhancing well-defined lesion (3.2 cm × 2.4 cm) with hypodensity along its margin in relation to the distal popliteal artery with sudden cutoff of the popliteal artery suggestive of pseudoaneurysm with significantly attenuated tibioperoneal (TP) trunk, anterior tibial, posterior tibial, and peroneal arteries [Figure 3].
Figure 2: B mode, color and spectral wave form showing arterial flow velocity wave form in the aneurysm sac

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Figure 3: Computed tomography angiogram showed intensely enhancing lesion over the popliteal artery with normal distal flow

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The patient underwent exploration of the popliteal artery with excision of aneurysmal sac. As the patient had palpable pulses in spite of occlusion of the popliteal artery, it was decided not to revascularize the popliteal artery. No frozen section was performed during surgery [Figure 4]. Aneurysmal sac was sent for biopsy. Postoperative recovery was uneventful [Figure 5]. The patient had a palpable distal posterior tibial pulse.
Figure 4: Intraoperative photograph showed aneurysm wall being excised

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Figure 5: Postoperative clinical photograph of the patient showing healing suture line with no signs of limb ischemiachange

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On histopathological examination, the right popliteal arterial wall was congested and irregular approximately 5 cm × 2.5 cm in size with a wall thickness ranging from 0.2–0.5 mm. The cut surface was gray-white. Sections from the popliteal artery showed a spindle cell lesion within the intima. The intima was expanded and showed proliferation of spindle-shaped cells arranged in sheets. At places, prominent myxoid change was seen in the background. The spindle cells had mildly anisomorphic spindle-shaped nuclei. Few mitotic figures were seen. The tumor cells were seen infiltrating the muscular layer. The adventitia was free of tumor [Figure 6]. On immunohistochemical staining, the tumor cells were positive for vimentin and smooth muscle actin (SMA). Ki67 proliferation was 10%–15% [Figure 7]. Vimentin and SMA are the markers for soft tissue sarcoma. Ki67 is a marker of proliferation and indicates the proliferative activity of the tumor. This marker is present in all the stages of cell cycle except the resting phase (G0).
Figure 6: Histopathological examination showing proliferation of spindle cells in intima

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Figure 7: Immunohistochemical staining showing Ki 67 proliferative index

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  Discussion Top


Vascular tumors of the childhood are usually benign with infantile hemangioma being the most common.[2],[3],[4] Malignant primary vascular tumors in childhood are extremely rare. Secondary involvement of the vessel due to contiguous or metastatic spread from carcinoma or sarcoma is a common occurrence. Most of the primary vascular sarcomas involve large vessels (pulmonary artery, aorta, inferior vena cava, iliofemoral vessels, etc.).[5] Primary malignant tumors of the arteries are classified on the basis of origin from the vessel wall layer (adventitial, mural, or intimal). Intimal sarcomas can be further differentiated on the basis of their cell of origin into angiosarcomas (endothelial origin) and myofibroblastic sarcomas (mesenchymal origin).[6]

Intimal tumors present mostly as intraluminal polypoidal mass causing thrombosis or distal tumor embolisation.[6] Intimal sarcomatous degeneration leading to aneurysm formation is not a common clinic-pathological occurrence. So far, no case of spontaneous intimal sarcoma causing aneurysmal degeneration of popliteal artery in an infant has been reported in the literature.

As there is no case published in the literature of a popliteal artery aneurysm as intimal sarcoma and pre- and intra-operative work up could not predict the malignant potential of the lesion. On the basis of radiological diagnosis of popliteal artery aneurysm, exclusion of the aneurysm by open or endovascular means is the standard of care. Considering the age (infancy) and anticipated growth of the child, no stent graft can be deployed in popliteal artery.

There are no standard guidelines for the management of intimal sarcomas. Surgery with the intent of curative R0 resection with adjuvant therapy is considered to be the best possible management in the absence of metastasis.[1],[6],[7] In this patient, popliteal artery exploration with resection of aneurysm sac was performed. No vessels suitable for distal anastomosis could be appreciated. Considering the young age with on-going robust angiogenesis, excision of the aneurysm with ligation of the proximal TP trunk was performed. The aneurysm sac was sent for biopsy. Histopathological examination and immunohistochemical staining proved it to be the intimal sarcoma.

The role of adjuvant chemotherapy or radiotherapy is not very clear due to the rarity of the condition.[7],[8],[9] Chemotherapy or radiotherapy is generally preferred in the patients with the metastatic, embolic or unresectable disease. In our patient, complete curative resection of the aneurysm was performed. Hence, adjuvant therapy was not considered. At 1 year of follow-up, the patient is clinically stable with no disability and any signs of recurrence or metastasis. The patient is being planned for positron emission tomography-CT scan for assessment of any recurrent lesion.


  Conclusion Top


Peripheral arterial aneurysms should be watched carefully. Although malignancy is extremely rare in aneurysmal presentation it should be kept as a differential diagnosis. In suspicious cases of malignancy intraoperative frozen section should be performed to exclude the possibility of malignancy. More empirical evidence is yet required to make the diagnostic and therapeutic protocol to deal with this aggressive tumor of the vascular system.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the parents of the patient have given their consent for her images and other clinical information to be reported in the journal. The parents of the patient understand that her name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Choi SY, Min SK, Kim KI, Kim HY. Intimal angiosarcoma presenting with common femoral artery aneurysm. J Vasc Surg 2012;56:819-21.  Back to cited text no. 1
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2.
Trenor CC 3rd, Greene AK. Vascular tumors of childhood. Rutherford's Vascular Surgery. 8th ed. Vol. 1. Philadelphia:Elsevier; 2014. p. 1268-83.  Back to cited text no. 2
    
3.
Greene AK, Liu AS, Mulliken JB, Chalache K, Fishman SJ. Vascular anomalies in 5,621 patients: Guidelines for referral. J Pediatr Surg 2011;46:1784-9.  Back to cited text no. 3
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4.
Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-22.  Back to cited text no. 4
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5.
Ebaugh JL, Yuan M, Hu J, Chen A, Raffetto JD. Intimal sarcoma of the superficial femoral artery with osteosarcomatous differentiation. J Vasc Surg 2011;53:1394-7.  Back to cited text no. 5
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6.
Thalheimer A, Fein M, Geissinger E, Franke S. Intimal angiosarcoma of the aorta: Report of a case and review of the literature. J Vasc Surg 2004;40:548-53.  Back to cited text no. 6
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7.
Naughton PA, Wandling M, Phade S, Garcia-Toca M, Carr JC, Rodriguez HE, et al. Intimal angiosarcoma causing abdominal aortic rupture. J Vasc Surg 2011;53:818-21.  Back to cited text no. 7
    
8.
Ramjee V, Ellozy S. Aortic angiosarcoma masquerading as a thoracic aortic aneurysm. J Vasc Surg 2009;50:1477-80.  Back to cited text no. 8
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9.
Rosenberg SA, Tepper J, Glatstein E, Costa J, Baker A, Brennan M, et al. The treatment of soft-tissue sarcomas of the extremities: Prospective randomized evaluations of (1) limb-sparing surgery plus radiation therapy compared with amputation and (2) the role of adjuvant chemotherapy. Ann Surg 1982;196:305-15.  Back to cited text no. 9
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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