Histopathological reporting of paediatric cutaneous vascular anomalies in relation to proposed multidisciplinary classification system

Abstract

Background: The terminology applied to vascular anomalies has been variable in previously published literature making interpretation suboptimal. The International Society for the Study of Vascular Anomalies (ISSVA) has proposed a revised classification based on clinical features and histopathological findings. This classification is increasingly being accepted as clinically useful and a platform for future studies. Aims: To examine the extent to which the ISSVA classification can be practically applied to diagnostic histopathological specimens. Methods: Cutaneous vascular lesions received in a single paediatric pathology unit during a 2-year period (2004-5) were reviewed, including glucose transporter protein 1 (GLUT1) immunostaining where required, and lesions were reclassified according to the ISSVA classification. Results: 144 specimens were identified. Appropriate full clinical information was provided in only 17% of cases at submission. Infantile haemangiomas comprised 46% of cases, 18% of which were regressive type, initially inaccurately identified as vascular malformations before GLUT1 immunostaining. 30% of lymphatic malformations and all lymphovenous malformations were previously classified as vascular malformations, not otherwise specified. Conclusions: The ISSVA classification of vascular anomalies provides a useful framework for histopathologists to classify vascular anomalies. However, meaningful and appropriate use of such a system is dependent on the adequacy of clinical information provided and routine use of immunohistochemical markers.

Diffuse neonatal hemangiomatosis: An evidence-based review of case reports in the literature

“…The histopathologic diagnosis of cutaneous vascular anomalies has advanced in response to newer classification schema and with the advent of GLUT-1 as a specific immunohistochemical marker for IH. 18,19 When biopsy specimens are obtained from the skin or other sites, immunohistochemical staining with GLUT-1 and other relevant stains (D2-40 or LYVE-1) may be needed to distinguish IH from other vascular tumors or malformations. Consultation with a pathologist with expertise in vascular anomalies may be required for unusual cases.…”

Classification of Benign Vascular Lesions: History, Current Nomenclature, and Suggestions for Imagers

“…Unfortunately, such information is typically not available to radiologists and pathologists. In a study of 144 cutaneous vascular lesions, adequate clinical information was available in only 17% of surgical specimens [16]. Additionally, although tumors and malformations may be assumed to be distinct, overlapping features may be identified in some patients [17], and some hemangiomas may be noninvoluting (termed "noninvoluting congenital hemangioma") [19].…”

“…Hemangioma of infancy (juvenile hemangioma), for example, is the most common congenital or perinatal lesion, affecting up to 10% of children [13]. This proliferative lesion characteristically regresses spontaneously and is distinguished by showing a strong expression of glucose transporter protein 1 (GLUT1) from the endothelial cells [16,17]. GLUT1 is an immunohistochemical marker that is highly specific for this diagnosis and is seen in the early proliferative phase as well as in the late involutional phase of this lesion [17].…”

Life-threatening hemorrhage from a congenital hemangioma caused by birth trauma

“…Infantile hemangiomas and congenital hemangiomas are distinguished based on their clinical and histologic features and presence of GLUT-1 expression on the endothelial cells of an infantile hemangioma [2,3]. Congenital hemangiomas are further divided into rapidly involuting congenital hemangioma (RICH) and noninvoluting congenital hemangioma (NICH), and treatment depends on accurate classification [3].…”