vascular tumor

Vascular tumors make up one of the classifications of vascular anomalies. The other grouping is vascular malformations. Vascular tumors can be further subclassified as being benign, borderline or aggressive, and malignant. Vascular tumors are described as proliferative, and vascular malformations as nonproliferative.{{cite journal |last1=Steiner |first1=JE |last2=Drolet |first2=BA |title=Classification of Vascular Anomalies: An Update. |journal=Seminars in Interventional Radiology |date=September 2017 |volume=34 |issue=3 |pages=225–232 |doi=10.1055/s-0037-1604295 |pmid=28955111|pmc=5615389 }}

File:Pyogenic Granuloma Thumb 1.JPG

A vascular tumor typically grows quickly by the proliferation of endothelial cells. Most are not birth defects.

The most common type of benign vascular tumors are hemangiomas, most commonly infantile hemangiomas, and less commonly congenital hemangiomas.

{{Main|Infantile hemangioma}}

Infantile hemangiomas are the most common type of vascular tumor to affect babies, accounting for 90% of hemangiomas.{{cite journal |last1=Sadick |first1=M |last2=Müller-Wille |first2=R |last3=Wildgruber |first3=M |last4=Wohlgemuth |first4=WA |title=Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. |journal=RöFo: Fortschritte auf dem Gebiete der Röntgenstrahlen und der Nuklearmedizin |date=September 2018 |volume=190 |issue=9 |pages=825–835 |doi=10.1055/a-0620-8925 |pmid=29874693|doi-access=free }} They are characterised by the abnormal proliferation of endothelial cells and of deviant blood vessel formation or architecture.{{cite journal |last1=Darrow |first1=DH |last2=Greene |first2=AK |last3=Mancini |first3=AJ |last4=Nopper |first4=AJ |last5=SECTION ON DERMATOLOGY, SECTION ON OTOLARYNGOLOGY–HEAD AND NECK SURGERY, and SECTION ON PLASTIC |first5=SURGERY. |title=Diagnosis and Management of Infantile Hemangioma. |journal=Pediatrics |date=October 2015 |volume=136 |issue=4 |pages=e1060-104 |doi=10.1542/peds.2015-2485 |pmid=26416931|doi-access=free }} Hypoxic stress seems to be a major trigger for this.{{cite journal |last1=Wildgruber |first1=M |last2=Sadick |first2=M |last3=Müller-Wille |first3=R |last4=Wohlgemuth |first4=WA |title=Vascular tumors in infants and adolescents. |journal=Insights into Imaging |date=13 March 2019 |volume=10 |issue=1 |pages=30 |doi=10.1186/s13244-019-0718-6 |pmid=30868300|pmc=6419671 |doi-access=free }} Infantile hemangiomas are easily diagnosed, and little if any aggressive treatment is needed. They are characterised by rapid growth in the first few months, followed by spontaneous regression in early childhood.

Congenital hemangiomas are present and fully formed at birth, and only account for 2% of the hemangiomas. They do not have the postnatal phase of proliferation common to infantile hemangiomas. There are two main variants of congenital hemangioma: non-involuting, and rapidly involuting (beginning in the first year of life). A third variant is also recognised as partially involuting.{{cite web |title=Congenital haemangioma |url=https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/congenital-haemangioma |website=www.gosh.nhs.uk |language=en}} Congenital hemangiomas can also be distinguished from infantile hemangiomas in that neither variant of congenital hemangioma expresses the glucose transporter GLUT 1.{{cite journal |last1=van Vugt |first1=LJ |last2=van der Vleuten |first2=CJM |last3=Flucke |first3=U |last4=Blokx |first4=WAM |title=The utility of GLUT1 as a diagnostic marker in cutaneous vascular anomalies: A review of literature and recommendations for daily practice. |journal=Pathology, Research and Practice |date=June 2017 |volume=213 |issue=6 |pages=591–597 |doi=10.1016/j.prp.2017.04.023 |pmid=28552538}}

Some cases have been associated with a mild form of thrombocytopenia. Rare cases have been associated with heart failure.

{{Main|Hemangioblastoma}}

Hemangioblastomas are vascular tumors of the central nervous system.

A range of benign vascular tumors are described as reactive proliferative lesions that grow in response to a stimulus, such as trauma, or a local thrombosis. They can also form infrequently during pregnancy as a hormonal reaction affecting the gums.{{cite book |last1=Kumar |first1=V |last2=Abbas |first2=A |last3=Aster |first3=J |title=Robbins basic pathology |date=2018 |isbn=9780323353175 |pages=392–396 |publisher=Elsevier |edition=Tenth}}

The most common type of reactive proliferative tumors are pyogenic granulomas also known as lobular capillary hemangiomas, that are more often found in children and young adults. These granulomas are well defined growths of less than a centimetre across. They are bright red due to being highly vascularised, and bleed and ulcerate easily. Their colouring fades with age.

Tufted angiomas are hereditary hemangiomas found in infants from birth to five years of age, however they may occur in adults. They are found on the neck, shoulders, and trunk as rounded nodules.{{cite web |title=MeSH Browser |url=https://meshb.nlm.nih.gov/record/ui?ui=C536924 |website=meshb.nlm.nih.gov |access-date=22 November 2019}} Tufted angiomas are usually poorly defined lesions of purple colouration.{{cite journal |last1=Victoria Martínez |first1=AM |last2=Cubells Sánchez |first2=L |last3=Esteve Martínez |first3=A |last4=Estela Cubells |first4=JR |last5=Febrer Bosch |first5=I |last6=Alegre de Miquel |first6=V |last7=Oliver Martínez |first7=V |title=[Tufted angiomas in childhood: A series of 9 cases and a literature review]. |journal=Anales de Pediatria |date=September 2015 |volume=83 |issue=3 |pages=201–8 |doi=10.1016/j.anpedi.2014.10.018 |pmid=25468452|s2cid=196370382 }} The tumors are of tufts of capillary-sized vessels in lobules that are scattered in the skin, and that sometimes reach into the subcutaneous tissue, and have lymph vessels on the periphery. Their growth is slow to begin with, and progresses to a stable size. They show a high rate of spontaneous regression, particularly in congenital and early-onset cases. They typically have a deep nodular component sometimes extending into the subcutaneous tissue, fascia, and muscle, and can sometimes be painful. Tufted angiomas are associated with arteriovenous malformations.

The origin of tufted angiomas is not clear but markers on the cells suggest a possible derivation from the endothelial cells of lymph vessels. They are also associated with the local secretion of growth factors that affect angiogenesis and promote the development of vascular lobules.

{{Main|Hemangioendothelioma}}

File:Kaposiform hemangioendothelioma.JPG

Kaposiform hemangioendotheliomas (KHEs) are borderline, locally destructive vascular tumors. They are named after their resemblance to the lesions of Kaposi's sarcoma.{{cite journal |last1=Vivas-Colmenares |first1=GV |last2=Ramirez-Villar |first2=GL |last3=Bernabeu-Wittel |first3=J |last4=Matute de Cardenas |first4=JA |last5=Fernandez-Pineda |first5=I |title=The importance of early diagnosis and treatment of kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon. |journal=Dermatology Practical & Conceptual |date=January 2015 |volume=5 |issue=1 |pages=91–3 |doi= 10.5826/dpc.0501a18|pmid=25692091|pmc=4325701 }} KHEs are described as locally destructive because they can infiltrate underlying muscle and fat. They are often seen to overlap with tufted angiomas (TAs) but TAs may be a milder, benign counterpart.{{cite journal |last1=Putra |first1=J |last2=Gupta |first2=A |title=Kaposiform haemangioendothelioma: a review with emphasis on histological differential diagnosis. |journal=Pathology |date=June 2017 |volume=49 |issue=4 |pages=356–362 |doi=10.1016/j.pathol.2017.03.001 |pmid=28438388}}

KHEs show as a red or purple expanding mass of soft tissue, found mostly in infants. Under the microscope KHE is characterised by nodules of tumor-like spindled endothelial cells. Unlike infantile hemangiomas, KHEs have a high mortality rate. Both KHEs and TAs are unique in that they carry the risk of the development of Kasabach–Merritt syndrome.{{cite journal |last1=Croteau |first1=SE |last2=Gupta |first2=D |title=The clinical spectrum of kaposiform hemangioendothelioma and tufted angioma. |journal=Seminars in Cutaneous Medicine and Surgery |date=September 2016 |volume=35 |issue=3 |pages=147–52 |doi=10.12788/j.sder.2016.048 |pmid=27607323}}

Malignant vascular tumors are rare, and include angiosarcomas, and epithelioid hemangioendotheliomas. Other types are hemangiopericytomas,{{cite journal |last1=Ghose |first1=A |last2=Guha |first2=G |last3=Kundu |first3=R |last4=Tew |first4=J |last5=Chaudhary |first5=R |title=CNS Hemangiopericytoma: A Systematic Review of 523 Patients. |journal=American Journal of Clinical Oncology |date=June 2017 |volume=40 |issue=3 |pages=223–227 |doi=10.1097/COC.0000000000000146 |pmid=25350465|s2cid=12236073 }} and lymphangiosarcomas.

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{{Vascular tumors}}

Category:Vascular anomalies

Category:Neoplasms

Category:Anatomical pathology