Vascular tumor: Difference between revisions

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__NOTOC__
{{CMG}}; {{AE}} {{HMHJ}}
==Overview==
'''Vascular tumor''' may mean:
'''Vascular tumor''' may mean:


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:* a '''highly vascularized tumor''' or '''poorly vascularized tumor''', referring to the degree of adequate or inadequate ([[ischemic]])  blood supply to  a tumor formed from any [[biological tissue]]
:* a '''highly vascularized tumor''' or '''poorly vascularized tumor''', referring to the degree of adequate or inadequate ([[ischemic]])  blood supply to  a tumor formed from any [[biological tissue]]


==Vascular Tumors==
==Classification==
===Benign vascular tumors 1===
===Benign vascular tumors===
====Infantile hemangioma / Hemangioma of infancy====
====Infantile hemangioma / Hemangioma of infancy====
{| class="wikitable"
{| class="wikitable"

Revision as of 20:12, 9 October 2018


Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Hannan Javed, M.D.[2]


Overview

Vascular tumor may mean:

Classification

Benign vascular tumors

Infantile hemangioma / Hemangioma of infancy

Pattern Different types
  • focal
  • multifocal
  • segmental
  • indeterminate
  • superficial
  • deep
  • mixed (superficial + deep)
  • reticular / abortive / minimal growth
  • others
Association with other lesions
PHACE association /

syndrome

Posterior fossa malformations, Hemangioma, Arterial

anomalies, Cardiovascular anomalies, Eye anomalies ,

sternal clefting and ⁄ or supraumbilical raphe

LUMBAR (SACRAL,

PELVIS) association /

syndrome

Lower body hemangioma, Urogenital anomalies,

Ulceration, Myelopathy, Bony deformities, Anorectal

malformations, Arterial anomalies, and Renal anomalies

Congenital hemangioma

  • Rare tumor that arises in utero and presents as fully developed lesion at birth. Following birth they can regress completely, partially or not at all. So they can be classified as Rapidly involuting (RICH), Non-involuting (NICH), Partially involuting (PICH).[16][17]
    1. Rapidly involuting (RICH)
    2. Non-involuting (NICH)
      • Fast flow tumor that presents as well defined, plaque like lesion with pink to purple color, telangiectasias and pale borders. Typically remains stable but there have been some reports of growth and expansion.[17]
    3. Partially involuting (PICH)
      • These lesions start involution as RICH but become stable over time and persist as NICH.[19]
  • Somatic mutations in GNAQ/GNA11 are thought to cause the congenital hemangioma. GNAQ and its paralogue GNA11 function in intracellular signaling pathways as Gq alpha subunit.[17][20]
  • Diagnosis is usually clinical but imaging techniques such as MRI, CT scan, contrast-enhanced ultrasound and later biopsy can be considered if required. Surgical excision should be considered in case of complications, NICH and PICH.[21][22]

Tufted angioma

Spindle-cell hemangioma

  • Rare benign tumor that manifests as solitary or multiple nodules confined to dermis and subcutaneous tissues in almost all of the cases. Histopathologically it appears as solid areas that are cellular and consist of spindle cells seen attached to vessel walls, and cavernous spaces that can be thrombosed. Size may increase over time and patient usually complains of swelling and pain. The nodules or masses can be mobile and elastic or can be firm and immobile.[35][36][37]
  • Somatic mutations in IDH1 and IDH2 have been found to be present in 70% of spindle-cell hemangiomas. IDH1 and IDH2 are important enzymes in cell energy cycles (α-ketoglutarate and NADPH generation).[38][39]
  • Diagnosis often requires biopsy and imaging studies such as MRI to ascertain the extent of the tumor. Local excision is the treatment modality of choice with excellent prognosis in majority of the cases although recurrence is very common.[35][36][37][40][41]

Epithelioid hemangioma

Pyogenic granuloma

Hobnail hemangioma

  • Benign tumor that typically presents as solitary growth with often, but not always, tagetoid appearance of a central papule and peripheral brown ring that may or may not disappear over time. Characteristic histopathological feature include plump endothelial cells in superficial dermis that line ectatic and irregular vessels, and project into lumina like hobnails. Deeper dermis shows vessels dissecting collagen fibers. Majority of the lesions are fund on trunk and extremities with head and neck, and oral cavity as uncommon locations. Patient may present with pain, or an asymptomatic growing lesion.[62][63][64]
  • Etiology is not well understood but trauma may play a key role in pathogenesis. Some studies have found congenital etiology in some lesions.[62][63][65][66]
  • Diagnosis is based on clinical features and histopathological studies. Treatment is usually by excision. Other modalities of treatment include intermittent triamcinolone intralesional injections and pulsed dye laser treatment.[62][63]

Microvenular hemangioma

  • Rare lesion that most often manifests as single asymptomatic nodule, plaque or papule with color varying from red to bluish-red. Majority of the lesions are located on trunk and limbs. Histologically, the tumor consists of irregular and branching venous structures with inconspicuous lumina. Endothelial cells display absence of atypia and mitotic figures. Some lesions may be painful and/or tender.[67][68][69][70]
  • Etiology and pathogenesis have not been well-understood but a recent study may associate progesterone with microvenular hemangioma.[71]
  • Diagnosis requires biopsy because of rarity of this tumor. Treatment is through surgical excision.[67][68][69][70]

Anastomosing hemangioma

Glomeruloid hemangioma

  • Characterized by red blood cells filled clumps of capillaries inside dilated vascular spaces. These collections of capillaries, lined by swollen endothelial cells, resemble renal glomeruli and stain positive for periodic acid-Schiff (PAS)-positive, diastase-resistant eosinophilic globules. Clinical presentation varies and are not discernible from other cutaneous lesions. Majority of the lesions manifest as multiple, asymptomatic pauples or nodules..[80][81]
  • Glomeruloid hemangioma is associated with POEMS syndrome in majority of the cases and rarely with Castleman's disease. Very few isolated cases of glomeruloid hemangioma have been reported.[80][81]
    • POEMS stands for peripheral neuropathy (P), organomegaly (O), endocrinopathy (E) monoclonal plasma-cells proliferative disorder (M) and skin changes (S) although diagnosis does not require presence of all of these symptoms. Other manifestations of POEMS syndrome may include sclerotic bone lesions, papilledema, edema, ascites, effusions, pulmonary hypertension, Castleman’ disease (CD), thrombocytosis and erythrocytosis, and increased serum VEGF.[82]
    • Castleman’s disease is characterized as lymphoproliferative disorder with inflammatory response involving multiple systems. Clinical presentation ranges from asymptomatic lymphadenopathy to severe systemic manifestations such as weight loss, fever and organomegaly.[83]
  • Etiology is not well-understood but some theories suggest role of vascular endothelial growth factor (VEGF), increased estrogen levels, human herpesvirus-8 and increased cytokines in its pathogenesis.[80][81]
  • Diagnosis relies on characteristic histology. Patients who present with glomeruloid hemangioma should undergo evaluation for POEMS syndrome and should be kept under follow-up because these lesions can precede full-blown POEMS syndrome in some cases.[80][81]

Papillary hemangioma

Intravascular papillary endothelial hyperplasia

  • Also called as Masson's tumor, this benign lesions is characterized by presence of intravascular papillary structures that are enveloped by proliferating endothelial cells. It is considered to be a reactive lesion associated with an organizing thrombus. Clinically it manifests as solitary painless mass in head-neck and the extremities especially the hand, that may grow rapidly in size and become painful and/or tender. Some lesions have been found intra-abdominallly such as in the liver that can bleed and present with anemia.[87][88][89][90]
  • This lesions appears to be associated with vascular trauma, and thrombus that may lead to chronic irritation and increased levels of fibroblast growth factor (FGF), hypoxia-inducible factor-1 (HIF-1α), and vascular endothelial growth factor (VEGF) stimulating endothelial cells proliferation.[88][89][91]
  • Histopathological studies are generally required for diagnosis and may also require immunohistochemical confirmation. Treatment is surgery with uncommon recurrence.[91][92]

Cutaneous epithelioid angiomatous nodule

Acquired elastotic hemangioma

Littoral cell hemangioma of the spleen

Related lesions

Locally aggressive or borderline vascular tumors

Kaposiform hemangioendothelioma

Retiform hemangioendothelioma

Papillary intralymphatic angioendothelioma (PILA), Dabska tumor

Composite hemangioendothelioma

Pseudomyogenic hemangioendothelioma

Polymorphous hemangioendothelioma

Kaposi sarcoma

  • An AIDS-associated vascular malignancy that usually presents as mucocutaneous lesions [147] but can also occur in viscera such as lungs. It can remain confined to skin but widespread visceral involvement may occur.
  • There are three known variants
    • One variant occurs spontaneously in Jewish and Italian males in Europe and the United States.
    • Another more aggressive variant is endemic in young children is endemic in Africa.
    • A third form occurs in about 0.04% of kidney transplant patients. There is also a high incidence in AIDS patients. HHV-8 is the suspected cause.[148]
  • To learn more about KS, click here.

Malignant vascular tumors

Angiosarcoma

Epithelioid hemangioendothelioma

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