Inconsistency in classifying vascular anomalies: What's in a name?

Abstract

Background Vascular anomalies are a heterogeneous group of disorders seen in children and adults. A standard nomenclature for classification has been offered by the International Society for the Study of Vascular Anomalies (ISSVA). Its application is important for communication among the multiple specialties involved in the care of patients and for planning treatment, as well as for research and billing. We hypothesized that terminology still is not uniformly applied, and that this could have an impact on treatment. Methods We retrospectively reviewed the medical records of patients with non-brain lesions from our institutional vascular anomalies database seen 2010–2016 for whom at least one clinic visit, radiologic imaging report, and pathology report were available, to compare diagnoses among and within disciplines, and treatment recommendations. Diagnoses and referral patterns by community healthcare providers also were reviewed. Results Of 400 patients seen during the targeted time interval, 35 had clinical, imaging, and pathology reports. Agreement in terminology from initial clinic notes with imaging and pathology reports was noted in only 3 cases (9%). “Hemangioma” was often misused; “lymphangioma” and “cystic hygroma” persist as diagnostic labels. Community healthcare providers referred vascular malformations with a diagnosis of “mass” or “hemangioma” in 17/18 cases where that information was available. Incomplete or mislabeling of vascular anomalies sometimes delayed referrals to appropriate clinics, though it did not have a major impact on treatment. Conclusions An understanding of vascular anomalies as tumors or malformations is not uniform. Ongoing education will be needed to promote consensus terminology and facilitate referrals.

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“…Both across specialties and within associated disciplines, it has been shown that there is inconsistency in the terminology used to describe vascular anomalies [39]. Obtaining an accurate diagnosis with appropriate terminology is important for properly managing patients.…”

Case Report: A Primary Right Ventricular Vascular Malformation Presenting as a Mass

“…RV, right ventricle; IVS, interventricular septum; LV, left ventricle. The ISSVA classification standards have been published; however, there is a lack of consensus regarding the understanding of whether vascular anomalies are tumors or malformations (16). Initially, they were classified according to factors such as vascular distortion and structure.…”

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“…1 A pesar de que esta clasificación tiene más de 20 años de vigencia, y de los diversos intentos enfocados a divulgarla, hasta la fecha se siguen utilizando, erróneamente, los términos "hemangioma", "hemolinfangioma" o "linfangioma" para describir tumores vasculares y malformaciones, aludiendo solo al componente vascular, sin diferenciar entre el carácter proliferativo o malformativo propio de cada una; esto deriva en una atención inadecuada del paciente. 5 De aquí la importancia de que el médico de primer contacto, el pediatra y los especialistas de las…”