Intimal Sarcoma of the Arteria Femoralis Superficialis: A Case-report

Article Outline

• Abstract
• Case Report
• Discussion
• Summary
• Acknowledgement
• Literature
• Copyright

A 76-year-old male presented with recurrent claudication. A duplex scan showed an occluded superficial femoral artery of the left leg. MRI revealed an inhomogeneously structured tumor, directly related to the femoral artery. The tumor was resected and histology showed an intimal sarcoma. The patient has survived now without further evidence of local recurrence or systemic metastatic disease.

Intimal sarcomas of the arteries other than arising from the aorta or pulmonary artery are rare. An intimal sarcoma of the superficial femoral artery has not been described previously.

Keywords: Intimal sarcoma, Arteria femoralis superficialis

Intimal sarcomas of arteries other than arising from the aorta or pulmonary artery are extremely rare. We report a case of intimal sarcoma of the superficial femoral artery.

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Case Report 

A 76-year-old male was admitted to our hospital with progressive pain in the left lower leg. In 2002 he presented to our vascular outpatient clinic with claudication. A duplex scan showed an occluded superficial femoral artery, without an aneurysm or other abnormality. He was treated conservatively and his claudication resolved.

In April 2005 he visited our outpatient clinic again with rest pain in his left leg. No peripheral pulses were palpable on examination as 3 years previously. However, now there was a 6cm swelling palpable in his left upper leg. A duplex scan showed no arterial flow within the femoral artery. MRI (Fig. 1) revealed an inhomogeneously structured tumor, directly related to the femoral artery and vein. A trucut biopsy was performed and showed a malignant histiocytoma. CT examination of thorax and abdomen showed no metastasis. The tumor was resected. The patient received adjuvant local radiotherapy, because of the small resection margins, in order to reduce the risk of local recurrence. He received 60 Gray, in fractions of 2 Gray up to 5 times a week. The first 50 Gray was directed to the whole adductor compartment, the last 10 Gray only to the operation area. At a 12 months follow up the patient was well without further evidence of local recurrence or systemic metastatic disease.

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• Fig. 1 

  MRI, tumor in the adductor loge of the left leg.

The operation specimen showed a sharply defined tumor surrounding the vascular stalk as a spindled cuff with a diameter of 2.8cm. Microscopy showed a tumor, located in the superficial femoral artery, penetrating through the arterial wall into the surrounding fatty tissue and even the superficial femoral vein with occlusion of the venal lumen (Fig. 2). The intraluminal extension of the tumor exceeded the extraluminal extension (as proven on snap frozen section). Therefore it can be assumed the tumor originates from the intima of the superficial femoral artery.

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• Fig. 2 

  Tumor extending from intima of femoral artery; the arrow indicates the intima; (lumen of the vessel is the central structure).

The tumor consists of spindle cells with elongated polymorphous hyperchromatic nuclei; mitotic and atypical mitotic figures are encountered frequently, up to 25 per 2mm². The stroma shows extensive hyalinization with calcification and iron depositions. The tumor was completely excised.

Immunohistochemistry: Vimentin (+), HHF 35 (+), Pan Cytokeratin (−), S-100 (−). All these findings are consistent with intimal sarcoma of the superficial femoral artery.

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Discussion 

Sarcomas of the arterial wall can be divided into mural sarcomas and intimal sarcomas. Intimal sarcomas are characterised by predominant intraluminal growth with destruction of the vessel lumen and the seeding of tumor emboli to peripheral organs.

Intimal sarcomas are extremely rare tumors, occurring mainly in the pulmonary artery and the aorta. The diagnosis of intimal sarcoma is in many cases found after autopsy. The clinical symptoms are mostly non specific, but sometimes related to arterial embolism. The tumorcells are from fibroblastic or myofibroblastic origin, as expressed by the immunohistochemistry like Vimentin and HHF 35.

In literature, approximately 120 pulmonary artery sarcomas are described, between 50 and 100 in origin from the aorta. Sarcomas of the other arteries are rare. An intimal sarcoma of the superficial artery has not been described previously.

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Summary 

In the case-report a 76 year old male is described with pain in his leg due to a tumor. Resection: histological examination showed an intimal sarcoma arose from the superficial femoral artery, invading the surroundings tissue, including the femoral vein.

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Acknowledgement 

Special thanks to prof. dr. J. Rauwerda, vascular surgeon VU Amsterdam, for his advise and corrections.

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Literature 

1. Akiyama K, Nakata K, Negishi N, Henmi A. Intimal sarcoma of the thoracic aorta; clinical course and autopsy finding. Ann Thorac Cardiovasc Surgery. 2005;2:135–138
2. Osei-Agyemang T, Geks J, Wagner HJ, Feek U, Gerdes B. Hochmalignes intimasarkom in einem aneurysma der infrarenalen bauchaorta. Der chirurg. 2004;8:823–827
3. 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(3):548–553
4. Sebenik M, Ricci A, DiPasquale B, Mody K, Pytel P, Jee KJ, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol. 2005 Sep;29(9):1184–1193
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