Identifying erroneously used terms for vascular anomalies: A review of the English literature


Hippokratia 2022, 26(4): 126-130

Boulogeorgou K1, Avramidou E2, Koletsa T1
1Department of Pathology, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece
2School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, Greece


Background: The classification of vascular anomalies includes terms of nomenclature that are not based on histogenesis resulting in confusion among health professionals of different specialties. Ongoing efforts to classify them properly have taken place. This literature review aimed to identify erroneous nomenclature of vascular anomalies and to investigate their continued use over the past four years after the last International Society for the Study of Vascular Anomalies (ISSVA) update.

Methods: Literature research was based on pertinent classifications (ISSVA, WHO) and books related to vascular anomalies and soft tissue pathology. After identifying twelve entities with confusing terminology, new research in the Pubmed database was conducted to verify their continued use in the last four years.

Results: The literature review highlighted terms referring to vascular malformations as neoplasms. In addition, terms used as equivalents represent entirely different entities. On the other hand, different terms to characterize the same entity were also recorded. Furthermore, regardless of the last ISSVA update in 2018, terms that are only descriptive or do not correspond to vascular anomaly histogenesis are consistently used.

Conclusion:  Despite intensive efforts in the last decades for correct terminology and classification of vascular anomalies, modifications are still required. A common and broadly accepted scientific terminology should be applied, accurately representing histogenesis or pathogenesis, to obtain a common language among medical specialists, given that a multidisciplinary approach is crucial for managing vascular anomalies. HIPPOKRATIA 2022, 26 (4):126-130. 

Keywords: Vascular tumors, vascular malformations, vascular anomaly, terminology in vascular anomalies, pathology of vascular anomalies

Corresponding author: Triantafyllia Koletsa, MD, Associate Professor, Department of Pathology, School of Medicine, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece, tel: +302310999245, fax: +302310999229, e-mail:


Vascular anomalies are frequently encountered clinical entities that mainly affect neonates and pediatric patients1. Their interpretation and classification have been historically a puzzle, often inducing disagreements and conflicts among the medical community2,3.

Until the mid-twentieth century, vascular anomalies were considered a consequence of complication during labor or a divinely sent curse and were “treated” accordingly. Mulliken and Glowacki, in 19824, were the first who tried to unravel the hank of yarn and proposed the division of vascular anomalies into vascular tumor/hemangioma (the suffix -oma for “tumor or mass”4) and malformation categories based on their clinical findings and histopathological features5. Fourteen years later (in 1996), in a meeting in Rome6, the International Society for the Study of Vascular Anomalies (ISSVA) adopted their proposal for distinguishing and classifying vascular anomalies and additionally defined the particular characteristics of each category7. Specifically, vascular or vasoproliferative neoplasms derive from abnormal, active cell proliferation and may be classified as benign, borderline, or malignant8-10. On the other hand, vascular malformations represent inborn defects in vascular morphogenesis. They are generally characterized by abnormally formed channels within a vascular apparatus, lined by normal (in number and size) endothelial cells2,8-11. This classification provided the initial framework for great strides in research and treatment in the field7,12-15.

Since then, various corrections and revisions have been made, resulting in the expansion of the classification system in the 2014 ISSVA workshop in Melbourne7 as well as the recent updates (ISSVA, 201816 and 202217). The latest updates divide vascular anomalies again into tumors (benign, locally aggressive, malignant) and malformations (simple, combined, of major vessels, and associated with other anomalies)18 but also incorporate considerably more information, including newly named entities and identified genes7. However, even this system does not always take into consideration the pathogenesis and the biological behavior of the anomalies, which is confusing when it comes to treatment and prognosis. In this literature review, we aimed to identify objectionable terms in the nomenclature of vascular anomalies to emphasize the need for a common language among healthcare professionals of different specialties.

Methods and Materials

Search strategy

This particular literature review was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PRISMA Group. Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement)19.

Studying the nomenclature of vascular anomalies in the current literature   [classifications of ISSVA16,17, World Health Organization (WHO)20-23 and related books: Soft Tissue Tumors by Enzinger & Weiss24,25, Vascular Anomalies by Mulliken & Young5, and Surgical Pathology by Rosai & Ackerman26], we identified twelve entities whose nomenclature did not take into consideration histogenesis or biological behavior, causing profound confusion among the specialists. A search in the PubMed electronic database was conducted to look into the continued use of this confusing nomenclature after the most recent ISSVA classification update by using the following keywords: “cavernous hemangioma”, “lymphangioma”, “hobnail hemangioma”, “port-wine stain”, “Kimura disease and angiolymphoid hyperplasia with eosinophilia”, “Kaposiform hemangioendothelioma and tufted angioma”, “Verrucous hemangioma and angiokeratoma”, and “Dabska type tumors and retiform hemangioendothelioma”.

Study selection and data collection process

The results of the electronic research were examined by two authors (KB, TK) independently and selected based on the predefined inclusion and exclusion criteria. Specifically, eligible studies were considered those i) oriented to the nomenclature of vascular malformations and vascular tumors or ii) to the historical conversion in the definition of the studied vascular entities, iii) written in the English language, and d) published as original articles, reviews, and case reports during 2019-2022. Studies regarding cavernous hemangioma, lymphangioma, hobnail hemangioma, and port-wine stain were excluded should they concerned with i) a mere report regarding differential diagnosis, ii) congenital abnormalities, vascular malformations of infants or children under ten years old, iii) surgical techniques, therapeutic methods, preoperative preparation or postoperative complications, and iv) experimental animal studies. For Kimura disease and angiolymphoid hyperplasia with eosinophilia, the search was focused on whether these two entities were considered identical. For Kaposiform hemangioendothelioma and tufted angioma, as well as Dabska-type tumors and retiform hemangioendothelioma, all the studies interpreted these “pair-entities” as completely different diseases were recorded. Abstracts were reviewed independently by each author, and generated a list of studies to retrieve for full-text review. The lists were compared, and any discrepancies were resolved by consensus.

After the selection of the agreed reviews, original studies, and case reports, we designed an Excel spreadsheet where we collected all the required information. The three authors performed the data extraction independently and subsequently verified between them. The following outcomes were extracted and assessed: year of publication, age of onset, pathophysiology and origin of each vascular anomaly, clinical presentation, histopathologic and immunohistochemical characteristics, biological behavior, and prognostic factors.


For “cavernous hemangioma”, “lymphangioma”, “hobnail hemangioma”, and “port-wine stain”, the search strategy retrieved 18,613 publications in total from the PubMed database. Out of these, 2,059 accounted for the period 2019-2022. Forty-nine (49) reports were excluded for being mere reports, usually in context to the differential diagnosis of other entities. Subsequently, 146 studies were ruled out as concerned with congenital or pediatric disease, while 557 were not included as they were focused on surgical techniques, therapeutic methods, and postoperative complications. Finally, eight studies were disregarded because they involved experiments on animals. Among the remainder of 1,299 studies (Figure 1), only four were interested in the nomenclature of vascular anomalies, two of which mentioned the erroneous use of the term lymphangioma and proposed using the term “lymphatic malformation” instead. The other two studies referred to “cavernous hemangioma” and its renaming to “cavernous malformation”. In 399 out of 1,299 studies, we came across the term “lymphangioma” without any report of the term “lymphatic malformation”. Among the 722 studies referring to “cavernous hemangioma”, we also encounter the term “cavernoma” in 22, the term cavernous angioma in 25, and only in one study the term “cavernous lymphangioma”. Concerning “hobnail hemangioma”, only one study was acceptable after the implementation of the exclusion criteria. In 177 out of the 1,299 studies, we came across the term “port wine stain”, either as a “single” disease or as part of various syndromes such as Klippel-Trenaunay syndrome or Sturge Weber syndrome. Indeed, among the last 177 studies, there were five that proposed the term “nevus flammeus” instead.

Figure 1: Flow chart of the search strategy and data collection process followed in this review that identified erroneously used terms for vascular anomalies.

Regarding the “pair entities”, the search in the PubMed database retrieved 730 publications in total. For the last four years, the publication number was decreased by 80. Only 15 studies out of 80 were about a comparative analysis among the two entities of each pair. Seven of 15 studies referred to “Kimura disease and angiolymphoid hyperplasia with eosinophilia”. In six of them, the entities mentioned above were interpreted as different anomalies and in one as the same. Five studies concerned “Kaposiform hemangioendothelioma and tufted angioma”, with four studies considering them to belong to the same spectrum and one study being different entities. Only one study referred to “verrucous hemangioma and angiokeratoma” where the authors declared that those entities are distinct based on the different locations of the lesions. Finally, two reports referred to “Dabska-type tumors and retiform hemangioendothelioma”, and both agreed that those entities are alike.


The literature search indicates that several medical terms are used incorrectly in everyday clinical practice without considering the pathogenesis, the origin, the immunophenotype, or the clinical behavior of the diseases. Typical instances are those of lymphangioma and cavernous hemangioma, the nomenclature of which refers to neoplasms, whereas they frankly represent malformations due to some kind of dysregulation in vascular development27,28. In detail, lymphangiomas result from the failure of lymphatics to communicate with the venous system26, while cavernous hemangiomas represent a kind of endothelial dysmorphogenesis from a lesion that is present at birth in the venous system24. Both lesions are benign and usually clinically present at birth or during the first few years of life26,29. It is also worth mentioning that both entities are characterized by the absence of endothelial hyperplasia5,9 .

Quite intriguing is also the fact that different terms are used for the same histomorphology based on the location of the lesion. In particular, tufted angioma and Kaposiform hemangioendothelioma appear to have common histological features (lobules of varying size comprising capillary-sized vessels with oval to spindled cells in the dermis or subcutaneous tissue-they can both present with lymphangioma-like areas) and an identical immunophenotype [Podoplanin (D2-40), Cluster of Differentiation (CD) 34, CD31 positivity, but Glucose transporter isoform 1 (GLUT1) negativity] while their differences concern mainly the location (superficial vs deep infiltrative lesion) and the biological behavior (benign vs aggressive) of each lesion1,5,11,21,26,30. The same applies to retiform hemangioendothelioma and Dabska-type lesions, the age of onset of which constitutes the main discrimination point (adults-children). However, the similar macroscopic (ill-defined, plaque-like lesions) and microscopic features (well-formed vessels lined by hobnail endothelial cells and surrounded by a dense fibrous-hyaline stroma with prominent lymphocytes), the common immunophenotypic profile with an intense expression of CD34 and D2-40 (a reason why Fanburg-Smith et al suggested the term papillary intralymphatic angioendothelioma for Dabska type hemangioendothelioma25), the exact location (usually distal extremities), and equal prognosis lend further support to their grouping as hobnail hemangioendothelioma21,22,25,26,31.

On the other hand, some of the terms used as equivalents represent entirely different entities. Typical examples are Kimura disease and angiolymphoid hyperplasia with eosinophilia, which used to be considered identical entities in the literature due to their similar histological features, while in essence, they differ in location (lymph nodes vs subcutaneous tissue of the head and neck), epidemiology (Asians vs Europeans), laboratory findings (elevated serum immunoglobulin IgE levels vs normal serum immunoglobulin levels), and treatment (conservative management in asymptomatic cases and surgery followed by glucocorticosteroids, cyclophosphamide, and radiotherapy in advanced cases vs complete surgical excision in every case)5,20,26,32-35.

In addition, there have been several reported cases in the literature where the lesion’s origin is not taken into account when entitled. Such is the case of hobnail hemangioma, whose immunophenotypic characteristics suggest lymphatic origin, while its nomenclature indicates a tumor of blood vessels26,36.

An issue that has emerged ever since the first meeting of ISSVA was using the terms angioma and hemangioma as synonyms (e.g., venous angioma but cavernous hemangioma). In fact, the former represents an umbrella term that encloses a broad spectrum of entities originating from blood (hemangiomas) or lymphatic vessels (lymphangiomas). Accordingly, it may be more prudent to maintain the term angioma exclusively for entities whose origin has not been established yet. The terms hemangioma and lymphangioma should be applied to entities of vascular and lymphatic origin, respectively. Terms like cystic hygroma and lymphangioma that were widely used in the past have already been revised in the current literature to perpetuate concerns among clinicians29,37,38.

At the other end of the spectrum, descriptive expressions, which are currently used among clinicians, should be replaced by terms that are based on histopathologic and biological features. For instance, the clinical term “port wine stain” corresponds to a common indolent venocapillary dysplasia11,39 and should be named as so.

A particular allusion should be made for verrucous venous malformation/hemangioma (VH) and angiokeratoma (AK), two entities yet not classified, prompting diagnostic confusion among pathologists40. Concerning VH, classification is still unclear because it exhibits clinical (favorable prognosis, rare regression) and histopathological features (absence of endothelial hyperplasia) similar to those seen in vascular malformations but expresses an immunoprofile [GLUT1+, Wilms’ tumor 1 (WT1) +] similar to vascular neoplasms24,40-43. This is probably why in the WHO skin classification of 2018, this entity appertains to the category of hemangiomas20. The same also applies to angiokeratoma20, albeit its superficial depth of invasion, indolent behavior, and lack of GLUT1/WT1 positivity, raising doubts among specialists. The equivalent occurs with synovial and intramuscular hemangiomas, which in the WHO soft tissue classification of 2020 are categorized as vascular tumors (hemangiomas) while, in fact, they both represent benign proliferations44 and ISSVA characterized them as provisional entities45. Among provisionally unclassified entities by ISSVA are also identified hepatic cavernomas or hepatic hemangiomas, or sinusoidal hemangiomas27.


Vascular anomalies cover a wide spectrum of lesions that may predominantly affect children but sometimes lead to serious, lifelong sequelae; with this into consideration, it is crucial to approach the diagnosis and treatment of these patients optimally and punctually, a fact that requires the appropriate definition and classification of the above entities3. “The investigation of the meaning of words is the beginning of wisdom” (“Αρχὴ σοφίας ἡ τῶν ὀνομάτων ἐπίσκεψις”) orated Antisthenes in the 4th century before Christ (BC), meaning that in order to solve a problem, you should initially confer to it an appropriate and representative name.

A considerable number of medical terms concerning vascular anomalies are still used incorrectly in everyday clinical practice. In all respects, the nomenclature of vascular anomalies should consider the pathogenesis, the histological features, and the clinical behavior of the entities and not be limited to their location or clinical manifestations. Also, it is necessary to reconsider terms given before the initial classification of vascular anomalies into tumors and malformations, and are preserved to this day for historical reasons (such as cavernous hemangioma, a term that Rudolf Virchow gave in 1863)5. Furthermore, the supersession of descriptive clinical terms based on subjective criteria is important to prevent any differential diagnostic considerations or pitfalls.

Since managing vascular anomalies requires cooperation among several medical specialists in the context of a multidisciplinary approach, adopting a common communication channel in this field is crucial. Therefore, a common scientific terminology should be decided and applied.

Conflict of interest

Authors declare no conflicts of interest. 


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