. . . a Vascular Anomalies Compendium that inclusively describes the subset that is us, the rare who are born with, those with congenital vascular malformations of different types and rarity within type.
NEOPLASTIC VASCULAR TUMORS (ISSVA: Vascular Tumors)
[1] ◈ [NVT]
Vascular tumors — neoplastic proliferations (benign, borderline, malignant).
These may be congenital, perinatal, or acquired in adulthood, depending on subtype.
Endothelial cells divide abnormally, creating new tissue — the definition of neoplastic.
Benign *
[1.1] ◈ [NVT—BE]
Infantile hemangioma
[1.1.01] ◈ [NVT-BE-IH]
Congenital hemangioma
[1.1.02] ◈ [NVT-BE-CH]
Tufted angioma
[1.1.03] ◈ [NVT-BE-TA]
Cherry angioma
[1.1.04] ◈ [NVT-BE-CA]
Epithelioid hemangioma
[1.1.05] ◈ [NVT-BE-EH]
Cutaneous epithelioid angiomatous nodule
[1.1.06] ◈ [NVT-BE-CN]
Pyogenic granuloma — Lobular Capillary Hemangioma
[1.1.07] ◈ [NVT-BE-PG]
Spindle-cell hemangioma
[1.1.08] ◈ [NVT-BE-SH]
Hobnail hemangioma
[1.1.09] ◈ [NVT-BE-HH]
Microvenular hemangioma
[1.1.10] ◈ [NVT-BE-MH]
Anastomosing hemangioma
[1.1.11] ◈ [NVT-BE-AH]
Glomeruloid hemangioma
[1.1.12] ◈ [NVT-BE-GH]
Papillary hemangioma
[1.1.13] ◈ [NVT-BE-PH]
Acquired elastotic hemangioma
[1.1.14] ◈ [NVT-BE-AE]
Intravascular papillary endothelial hyperplasia — Masson tumor
[1.1.15] ◈ [NVT-BE-IP]
Littoral cell hemangioma of the spleen
[1.1.16] ◈ [NVT-BE-LH]
Placental chorioangioma
[1.1.17] ◈ [NVT-BE-PL]
Eccrine angiomatous hamartoma
[1.1.18] ◈ [NVT-BE-EC]
Reactive angioendotheliomatosis
[1.1.19] ◈ [NVT-BE-RA]
Bacillary angiomatosis
[1.1.20] ◈ [NVT-BE-BA]
* Reactive proliferative vascular lesions are listed with benign vascular tumors.
Borderline
[1.2] ◈ [NVT—BO]
Kaposiform hemangioendothelioma
[1.2.01] ◈ [NVT—BO—KH]
Retiform hemangioendothelioma
[1.2.02] ◈ [NVT—BO—RH]
Papillary intralymphatic angioendothelioma (PILA), Dabska Tumor
[1.2.03] ◈ [NVT—BO—PI]
Pseudomyogenic hemangioendothelioma
[1.2.04] ◈ [NVT—BO—PS]
Polymorphous hemangioendothelioma
[1.2.05] ◈ [NVT—BO—PO]
Kaposi’s sarcoma
[1.2.06] ◈ [NVT—BO—KS]
Composite hemangioendothelioma
[1.2.07] ◈ [NVT—BO—CO]
Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT)
[1.2.08] ◈ [NVT—BO—ML]
Malignant
[1.3] ◈ [NVT—MA]
Angiosarcoma
[1.3.01] ◈ [NVT—MA—AN]
Epithelioid hemangioendothelioma
[1.3.02] ◈ [NVT—MA—EP]
STRUCTURAL VASCULAR MALFORMATIONS (ISSVA: Vascular Malformations)
[2] ◈ [SVM]
Vascular malformations involve abnormal vascular architecture — vessels formed incorrectly
during development. The structure is wrong; the endothelial cells are not proliferating abnormally.
ISSVA: “errors of vascular morphogenesis” — structural, not proliferative.
FAST-FLOW
[2.1] ◈ [SVM—FF]
Isolated
[2.1.01] ◈ [SVM—FF—IS]
AVM
[2.1.01.01] ◈ [SVM—FF—IS—AM]
Intramuscular Fast-flow Vascular Anomaly
[2.1.01.02] ◈ [SVM—FF—IS—IN]
AVF
[2.1.01.03] ◈ [SVM—FF—IS—AF]
Multifocal
[2.1.02] ◈ [SVM—FF—MU]
CM-AVM 1 & 2
[2.1.02.01] ◈ [SVM—FF—MU—CA]
HHT 1 & 2 / JPHT
[2.1.02.02] ◈ [SVM—FF—MU—HJ]
PHTS
[2.1.02.03] ◈ [SVM—FF—MU—PH]
Syndromic
[2.1.03] ◈ [SVM—FF—SY]
PHOST (PHTS)
[2.1.03.01] ◈ [SVM—FF—SY—PH]
Parkes-Weber Syndrome
[2.1.03.02] ◈ [SVM—FF—SY—PW]
SAMS
[2.1.03.03] ◈ [SVM—FF—SY—SA]
CAMS
[2.1.03.04] ◈ [SVM—FF—SY—CA]
SLOW-FLOW
[2.2] ◈ [SVM—SF]
CAPILLARY
[2.2.01] ◈ [SVM—SF—CM]
Nevus simplex, Salmon Patch
[2.2.01.01] ◈ [SVM—SF—CM—NS]
Port Wine CM — Port Wine Birthmark, Nevus flammeus
[2.2.01.02] ◈ [SVM—SF—CM—PW]
Isolated (incl. Phacomatosis Pigmentovascularis)
[2.2.01.02.01] ◈ [SVM—SF—CM—PW—IS]
Syndromic
[2.2.01.02.02] ◈ [SVM—SF—CM—PW—SY]
With hypertrophy or extracutaneous disease
[2.2.01.02.02.01] ◈ [SVM—SF—CM—PW—SY—HYP]
Sturge-Weber Syndrome
[2.2.01.02.02.02] ◈ [SVM—SF—CM—PW—SY—SWS]
DCMO
[2.2.01.02.02.03] ◈ [SVM—SF—CM—PW—SY—DCM]
Reticulate / Telangiectatic CM
[2.2.01.03] ◈ [SVM—SF—CM—RT]
Isolated
[2.2.01.03.01] ◈ [SVM—SF—CM—RT—IS]
Syndromic
[2.2.01.03.02] ◈ [SVM—SF—CM—RT—SY]
M-CM
[2.2.01.03.02.1] ◈ [SVM—SF—CM—RT—SY—MC]
MIC-CAP
[2.2.01.03.02.2] ◈ [SVM—SF—CM—RT—SY—MI]
DCMO
[2.2.01.03.02.3] ◈ [SVM—SF—CM—RT—SY—DC]
Geographic Pattern CM
[2.2.01.04] ◈ [SVM—SF—CM—GP]
Isolated
[2.2.01.04.1] ◈ [SVM—SF—CM—GP—IS]
Syndromic
[2.2.01.04.2] ◈ [SVM—SF—CM—GP—SY]
Klippel-Trenaunay Syndrome (KTS)
[2.2.01.04.2.1] ◈ [SVM—SF—CM—GP—SY—KT]
Associated with CLOVES / Disorders of PROS
[2.2.01.04.2.2] ◈ [SVM—SF—CM—GP—SY—CL]
Low-resistance CM / CM with faster flow
[2.2.01.05] ◈ [SVM—SF—CM—LR]
Isolated
[2.2.01.05.1] ◈ [SVM—SF—CM—LR—IS]
Syndromic
[2.2.01.05.2] ◈ [SVM—SF—CM—LR—SY]
CM-AVM 1 and 2
[2.2.01.05.2.1] ◈ [SVM—SF—CM—LR—SY—AV]
Parkes-Weber Syndrome
[2.2.01.05.2.2] ◈ [SVM—SF—CM—LR—SY—PW]
Cutis Marmorata Telangiectatic Congenita
[2.2.01.06] ◈ [SVM—SF—CM—CC]
Telangiectasias and Spider Angiomas
[2.2.01.07] ◈ [SVM—SF—CM—TS]
Isolated
[2.2.01.07.1] ◈ [SVM—SF—CM—TS—IS]
Syndromic
[2.2.01.07.2] ◈ [SVM—SF—CM—TS—SY]
CM-AVM 1 and 2
[2.2.01.07.2.1] ◈ [SVM—SF—CM—TS—SY—AV]
HHT 1 & 2 and JPHT (see also AVM category)
[2.2.01.07.2.2] ◈ [SVM—SF—CM—TS—SY—HJ]
LYMPHATIC
[2.2.02] ◈ [SVM—SF—LM]
Isolated
[2.2.02.01] ◈ [SVM—SF—LM—IS]
LM (Discrete)
[2.2.02.01.1] ◈ [SVM—SF—LM—IS—LD]
Macrocystic
[2.2.02.01.1.1] ◈ [SVM—SF—LM—IS—LD—MA]
Microcystic
[2.2.02.01.1.2] ◈ [SVM—SF—LM—IS—LD—MI]
Mixed Macromicrocystic
[2.2.02.01.1.3] ◈ [SVM—SF—LM—IS—LD—MX]
Angiokeratoma
[2.2.02.01.2] ◈ [SVM—SF—LM—IS—AN]
Complex
[2.2.02.02] ◈ [SVM—SF—LM—CO]
GLA
[2.2.02.02.1] ◈ [SVM—SF—LM—CO—GL]
KLA
[2.2.02.02.2] ◈ [SVM—SF—LM—CO—KL]
GSD
[2.2.02.02.3] ◈ [SVM—SF—LM—CO—GS]
GLD
[2.2.02.02.4] ◈ [SVM—SF—LM—CO—GD]
CCLA
[2.2.02.02.5] ◈ [SVM—SF—LM—CO—CC]
Isolated
[2.2.02.02.5.1] ◈ [SVM—SF—LM—CO—CC—IS]
Syndromic (RASopathy)
[2.2.02.02.5.2] ◈ [SVM—SF—LM—CO—CC—SY]
Lymphedemas
[2.2.02.03] ◈ [SVM—SF—LM—LY]
Primary
[2.2.02.03.1] ◈ [SVM—SF—LM—LY—PR]
Isolated
[2.2.02.03.1.1] ◈ [SVM—SF—LM—LY—PR—IS]
Syndromic
[2.2.02.03.1.2] ◈ [SVM—SF—LM—LY—PR—SY]
Secondary
[2.2.02.03.2] ◈ [SVM—SF—LM—LY—SE]
VENOUS
[2.2.03] ◈ [SVM—SF—VM]
Isolated
[2.2.03.01] ◈ [SVM—SF—VM—IS]
VM (Discrete)
[2.2.03.01.1] ◈ [SVM—SF—VM—IS—VD]
Phlebectatic
[2.2.03.01.2] ◈ [SVM—SF—VM—IS—PH]
Spongiform
[2.2.03.01.3] ◈ [SVM—SF—VM—IS—SP]
VVM
[2.2.03.01.4] ◈ [SVM—SF—VM—IS—VV]
FAVA
[2.2.03.01.5] ◈ [SVM—SF—VM—IS—FA]
Multifocal
[2.2.03.02] ◈ [SVM—SF—VM—MU]
VMCM
[2.2.03.02.1] ◈ [SVM—SF—VM—MU—CM]
MSVM
[2.2.03.02.2] ◈ [SVM—SF—VM—MU—MS]
BRBNS
[2.2.03.02.3] ◈ [SVM—SF—VM—MU—BR]
GVM
[2.2.03.02.4] ◈ [SVM—SF—VM—MU—GV]
HCCVM / CCM
[2.2.03.02.5] ◈ [SVM—SF—VM—MU—HC]
VMOS
[2.2.03.02.6] ◈ [SVM—SF—VM—MU—VO]
Syndromic
[2.2.03.03] ◈ [SVM—SF—VM—SY]
PHTS
[2.2.03.03.1] ◈ [SVM—SF—VM—SY—PH]
CLOVES
[2.2.03.03.2] ◈ [SVM—SF—VM—SY—CL]
Mafucci Syndrome
[2.2.03.03.3] ◈ [SVM—SF—VM—SY—MS]
Sinus Pericranii
[2.2.03.03.4] ◈ [SVM—SF—VM—SY—SP]
COMBINED
[2.2.04] ◈ [SVM—SF—CO]
Isolated
[2.2.04.01] ◈ [SVM—SF—CO—IS]
CLVM
[2.2.04.01.1] ◈ [SVM—SF—CO—IS—CLV]
LVM
[2.2.04.01.2] ◈ [SVM—SF—CO—IS—LVM]
CLM
[2.2.04.01.3] ◈ [SVM—SF—CO—IS—CLM]
CVM
[2.2.04.01.4] ◈ [SVM—SF—CO—IS—CVM]
HCCVM / VVM
[2.2.04.01.5] ◈ [SVM—SF—CO—IS—HCC]
Syndromic
[2.2.04.02] ◈ [SVM—SF—CO—SY]
PROS
[2.2.04.02.01] ◈ [SVM—SF—CO—SY—PR]
KTS (CLVM with hypertrophy)
[2.2.04.02.02] ◈ [SVM—SF—CO—SY—KT]
CLOVES
[2.2.04.02.03] ◈ [SVM—SF—CO—SY—CV]
CLAPO
[2.2.04.02.04] ◈ [SVM—SF—CO—SY—CL]
Proteus Syndrome
[2.2.04.02.05] ◈ [SVM—SF—CO—SY—PS]
DEVELOPMENTAL ANOMALIES OF NAMED VESSELS
[2.3] ◈ [SVM—NV]
Vena Cava
[2.3.01] ◈ [SVM—NV—VC]
Aorta
[2.3.02] ◈ [SVM—NV—AO]
Vein of Galen
[2.3.03] ◈ [SVM—NV—VG]
Others
[2.3.04] ◈ [SVM—NV—OT]
POTENTIALLY UNIQUE VASCULAR ANOMALIES
[3] ◈ [PUV]
As new or unique vascular anomalies are identified and confirmed, they will be documented here.
Updated: 2026.06
Note: This compendium is an adaptation by the Care4-Rare Project.
The ISSVA Classification & Glossary for Vascular Anomalies 2025 is owned and copyrighted by ISSVA.
Non-commercial use and reproduction are permitted without prior approval.
This is for personal reference and patient advocacy use only.
Diagnosis is the realm of qualified medical professionals.
Color key: NVT — Neoplastic Vascular Tumors | SVM — Structural Vascular Malformations | PUV — Potentially Unique Vascular Anomalies
◈ Navigation: The ◈ symbol between each ISSVA number and Catalog ID is a link to that entry’s dedicated page. Pages are added as content is developed.
Color key: NVT — Neoplastic Vascular Tumors | SVM — Structural Vascular Malformations | PUV — Potentially Unique Vascular Anomalies
◈ Navigation: The ◈ symbol between each ISSVA number and Catalog ID is a link to that entry’s dedicated page. Pages are added as content is developed.
Care 4 - Rare 