A rare case of epithelioid angiosarcoma
DOI:
https://doi.org/10.48729/pjctvs.301Keywords:
Surgical Oncology, Vascular surgery, Diagnosis, Differential, AngiosarcomaAbstract
Epithelioid angiosarcoma is a rare high-grade vascular neoplasm with a poor prognosis.
We present an anticoagulated 77-year-old man, with a history of popliteal/soleal vein thrombosis in the previous month, complaining of ipsilateral persistent lower limb pain and claudication. Absent popliteal/distal pulses prompted an arterial doppler ultrasound (DUS), revealing thrombosis of the distal superficial femoral artery and a popliteal mass. As the arterial wall’s integrity could not be appropriately evaluated by DUS, adventitial cystic disease of the popliteal artery was suspected. Computed tomography angiography and magnetic resonance imaging findings were also suggestive. Due to refractory pain, he was submitted to a popliteal mass excision along with a femoral-posterior tibial bypass. Pathology revealed an epithelioid angiosarcoma. He was referred to a Sarcoma Center, requiring hospitalization for agitation and fever. A positron emission tomography (PET) scan revealed extensive lower limb disease persistence and distant metastases. He died on the 56th day after surgery.
To our knowledge, there are only 15 cases of angiosarcoma of the popliteal artery described in the literature. Ours stands out as the first one unrelated to a popliteal aneurysm.
Being a highly-aggressive tumor, an early diagnosis is challenging but essential to a successful treatment, warranting the need for suspicion of this neoplasm. An early core biopsy or surgical sample may expedite the diagnosis.
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References
Kogon B, Kabeer M, Sawchuk AP, Dalsing M, Billings S, “Angiosarcoma presenting as an occluded popliteal artery pseudo-aneurysm,” J Vasc Surg, 1998 May; 27(5):970-3. doi: 10.1016/ s0741-5214(98)70280-0.
Mitchell RN, Halushka MK. Blood Vessels. In: Kumar V, Abbas AK, Aster JC, editors, Robbins & Cotran Pathologic Basis of Disease, 10th ed. Philadelphia, PA: Elsevier; 2021. p. 485-525.
Arts L, Roesti A, Haller C, Danzer D, “Angiosarcoma after Popliteal Aneurysm Exclusion Surgery. A Systematic Review,” Ann Vasc Surg, 2022 Jul; S0890-5096(22)00331-4. doi: 10.1016/j. avsg.2022.06.012.
Hart J, Mandavilli S, “Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis,” Arch Pathol Lab Med, 2011; 135(2):268-72. doi: 10.5858/135.2.268.
Cristaudo A, Steffen C, “Not Just a Popliteal Aneurysm: A Case of Metastatic Epithelioid Angiosarcoma,” EJVES Extra, 2012 May; 23(5): e50-2. doi: 10.1016/j.ejvsextra.2012.03.002.
Werra UEM, Petzold PT, “Epithelioid Angiosarcoma of the Popliteal Artery-Primary or Secondary? A Rare Disease Entity Difficult to Diagnose and Easily Overlooked-Literature Research and Case report,” Ann Vasc Surg, 2021 Jul; 74:524.e1-7. doi: 10.1016/j.avsg.2021.02.010.
Ko JS, Billings SD, “Diagnostically Challenging Epithelioid Vascular Tumors,” Surg Pathol Clin, 2015 Sep; 8(3):331-51. doi: 10.1016/j.path.2015.05.001.
Chen TW, Burns J, Jones RL, Huang PH, “Optimal Clinical Management and the Molecular Biology of Angiosarcomas,” Cancers, 2020 Nov; 12(11): 3321. doi:10.3390/cancers12113321.
Morris R, Lee CJ, “Development of Angiosarcoma in a Saphenous Vein Graft after Femoral to Above-Knee Popliteal Artery Bypass,” Ann Vasc Surg, 2018 Jul; 50:299.e9-13. doi: 10.1016/j. avsg.2018.01.087.
Nocturne G, Sellam J, Miquel A, M'Bappé P, Berenbaum F, “Is sarcoma a complication of arterial femoro-popliteal bypass?,” Joint Bone Spine, 2010 Jul; 77(4):358-60. doi: 10.1016/j.jbspin.2010.03.018.
Catalán J, Martín I, Cobo M, “Popliteal Angiosarcoma after Bypass with Autologous Saphenous Vein,” Ann Vasc Surg, 2019 Feb; 55:308.e1-4. doi: 10.1016/j.avsg.2018.06.034.
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