Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy

Simas, Ângela; Matos, Catarina; Silva, Rodrigo Lopes da; Brotas, Vítor; Teófilo, Eugénio; Albino, José Pereira

doi:10.1159/000313990

Cited by 16 publications

(14 citation statements)

References 15 publications

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“…Sunitinib is a novel, potent multitargeted receptor tyrosine kinase inhibitor approved for treatment of metastatic renal cancer, imatinib-resistant gastrointestinal stromal tumor, and pancreatic neuroendocrine tumors (4). Recent reports indicate a potential therapeutic benefit of sunitinib in patients with angiosarcomas (5)(6)(7). Ours is the first report of a complete and durable response to sunitinib in a patient with chemoresistant angiosarcoma of the breast.…”

Section: Refractory Angiosarcoma Of the Breast With Vegfr2 Upregulatimentioning

confidence: 82%

Refractory Angiosarcoma of the Breast with VEGFR 2 Upregulation Successfully Treated with Sunitinib

et al. 2015

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Section: Refractory Angiosarcoma Of the Breast With Vegfr2 Upregulatimentioning

confidence: 82%

Refractory Angiosarcoma of the Breast with VEGFR 2 Upregulation Successfully Treated with Sunitinib

et al. 2015

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“…Other features that can be associated with Klippel-Trenaunay-Weber syndrome are polydactyly [13] , syndactyly, seizures and hemimegalencephaly [14] , spina bifida, lymphatic obstruction, chronic venous insufficiency, limb ulceration, and thromboembolism [15] . Association with tumours including angiosarcomas has been reported [16] .…”

Section: Discussionmentioning

confidence: 99%

Atypical presentation (or a variant) of Klippel-Trenaunay-Weber syndrome: a case report and literature review

Singh¹,

Pycock²

2013

Grand Rounds

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We report the case of a 65-year-old man who we thought was an atypical presentation or a variant of the Klippel-Trenaunay-Weber syndrome. Although it is primarily a disorder of infancy and childhood, it has been reported to present in adulthood also [1,2]. Our case report highlights the need to consider the differential diagnosis of Klippel-Trenaunay-Weber syndrome in patients presenting with suggestive symptoms and signs, irrespective of their age.

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“…13,18,41,44,45,48 PWS and high-output heart failure Fifteen (31.3%) cases had high-output heart failure due to significant AV shunting. 13,21,24,26,29,30,33,34,38,41,48,49 There was no significant gender predominance (female-male ratio: 1:1), whereas 10 (66.7%) cases 23 46/M Limb amputation with chemotherapy Two months after died from pulmonary and cerebral metastases Ismail et al 24 16/M Hip disarticulation under low-flow cardiopulmonary bypass…”

Section: Patients' Characteristicsmentioning

confidence: 98%

“…Twelve (25.0%) cases were presented with chronic venous ulcerations of the affected limb 13,22,24,28,30,32,33,35,44,46,49,50 and four (8.3%) manifested distal arterial ischemia. 37,38,43,50 PWS and spinal AVM…”

Section: Pws and Venous Ulcerations/distal Arterial Ischemiamentioning

confidence: 99%

Parkes Weber syndrome—Diagnostic and management paradigms: A systematic review

et al. 2016

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Objectives Parkes Weber syndrome is a congenital vascular malformation which consists of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation. Although Parkes Weber syndrome is a clinically distinctive entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome that consists of the triad capillary malformation, venous malformation, and lymphatic malformation. Methods We performed a systematic review investigating clinical, diagnostic, and treatment modalities of Parkes Weber syndrome (PubMed/MEDLINE, Embase, and Cochrane databases). Thirty-six publications (48 patients) fulfilled the eligibility criteria. Results The median age of patients was 23 years (IQR, 8-32), and 24 (50.0%) were males. Lower extremity was affected in 42 (87.5%) and upper extremity in 6 (12.5%) patients; 15 (31.3%) patients developed high-output heart failure; 12 (25.0%) patients had chronic venous ulcerations, whereas 4 (8.3%) manifested distal arterial ischemia. The spinal arteriovenous malformations were reported in six (12.5%) patients and coexistence of aneurysmatic disease in five (10.4%) patients. The most frequently utilized invasive treatments were embotherapy followed by amputation and surgical arteriovenous malformation resection, and occasionally stent-graft implantation. All modalities showed clinical improvement. However, long follow-up and outcome remained unclear. Conclusion A diagnosis of Parkes Weber syndrome should be made on the presence of capillary malformation, venous malformation, lymphatic malformation, and arteriovenous malformation (as main defect) in overgrowth extremity. Arteriovenous malformation presents the criterion for distinguishing Parkes Weber syndrome from Klippel-Trenaunay syndrome, which is substantial for treatment strategy. The primary management goal should be patient's quality of life improvement and complication reduction. Embolization alone/combined with surgical resection targeting occlusion or removal of arteriovenous malformation "nidus" reliably leads to clinical improvement.

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Epithelioid Angiosarcoma in a Patient with Klippel-Trénaunay-Weber Syndrome: An Unexpected Response to Therapy

Cited by 16 publications

References 15 publications

Refractory Angiosarcoma of the Breast with VEGFR 2 Upregulation Successfully Treated with Sunitinib

Refractory Angiosarcoma of the Breast with VEGFR 2 Upregulation Successfully Treated with Sunitinib

Atypical presentation (or a variant) of Klippel-Trenaunay-Weber syndrome: a case report and literature review

Parkes Weber syndrome—Diagnostic and management paradigms: A systematic review

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