vascular lesions – dr komala pillay - papsa 2012
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Vascular lesionsKomala Pillay
MBChB, FCPath (SA), FRCPath(UK), MMed Anat Path (UCT)
Histopathologist National Health Laboratory Services and University of Cape Town
Red Cross Children's Hospital
Vascular anomalies of skin and soft tissue
ISSVA WHOISSVA
• TUMOURS
WHO
Benign
• Haemangioma• Haemangioendothelioma
• Haemangioma (benign)Capillary, cavernous, AV, venous
• Lymphangiomag• Angiosarsoma
Lymphangioma• Angiomatosis
I t di t• MALFORMATIONS• Simple (slow flow) C,V,L
Intermediate• Haemangioendothelioma• Kaposi sarcomaSimple (slow flow) C,V,L
• Simple (fast flow) A• Combined, AVM,C-V,C-L,
Malignant• EpithelioidCombined, AVM,C V,C L,
L-V• Epithelioid
haemangioendothelioma• Angiosarcoma
Syndromes characterised by vascular malformations / haemangiomas
Bl bb bl b D S i C hi• Blue rubber bleb nevus syndrome
• Gorham syndrome
• De Santis Cacchione• Bloom syndrome • Ataxia telangiectasiay
• Mafucci syndrome• Proteus syndrome
Ataxia telangiectasia• Goltz syndrome• Xeroderma pigmentosa
• Sturge Weber• Kassabach-Merrit
• Turner• Rothman Thompson
O l W b R d HHT• Osler-Weber-Rendu HHT• Von Hippel Lindau• Bannayan syndromeBannayan syndrome• Riley-Smith
BENIGN
Arteriovenous malformation (arteriovenous haemangioma)
Cli i l f HiClinical features• Fast flow cutaneous
malformations
Histo• distorted arteries and veins• Irregularly thickened
• Present at birth, but only 30% visible at birthD t
Irregularly thickened fibromuscular walls and discontinuous elastic networkSt l fib i• Do not regress
• Purple colour or mass• Mainly head and neck
• Stromal fibrosis• Variable admixture of
capillariesMainly head and neck• Arterial embolisation – may
alter histology
p
http://ades.tmu.edu.tw/english/pcare/course/Hemangioma/small/Hemangioma.html
Venous malformation (venous haemangioma
M t l Histo• Most common vascular malformation
• Low flow
Histo• Poorly circumscribed• Complex network of ectatic
thin walled channels with• Localised or extensive• Head and neck
thin-walled channels with intermixed capillaries
• Thrombi may have recanalised• Calcification frequent• At birth but evident later
• Mafucci syndrome
• Calcification frequent
Clinical• Blue, may swell, expand
slowly phlebolithsslowly, phleboliths• Intralesional sclerotherapy and
Sx
Pathology NHLS,RXH
Blue rubber bleb Differential of ‘blueberry muffin
nevus syndromeS di
Differential of ‘blueberry muffin baby’
Dermal extramedullary• Sporadic• Skin and GIT mainly• Dome shaped, painful at
haemopoiesis• Congenital infection – rubella,
CMV Toxop ppuberty, recurrent GIT bleeding
• Histo – irregular widely dilated
CMV, Toxo• Haemolytic dx of the newborn• Twin transfusion syndromeg y
thin-walled venous channels extending from subcutaneous fat into deep dermis to
• Hereditary spherocytosis
Bl bb bl b dp
immediately beneath thinned epidermis
• May be recanalised thrombi
Blue rubber bleb nevus syndrome
Neoplastic infiltrationsy Neoplastic infiltrations neuroblastoma,leukaemia, RMS, chorioca.
Capillary malformations / Macular stainshaemangioma
• Localised or extensive
Macular stains• Port wine stain: Pink –
bluish red to purple –• Localised or extensive• Face, trunk or limbs• Facial – trigeminal nerve area
Gro s in proportion to child
p pdarker irregular thickened and nodular
• Naevus flammeus• Grows in proportion to child• Bright red at birth• Evenly thickened skin, purple
nodules and pyogenic
• Naevus flammeus –resolves with time – may remain and become li htnodules and pyogenic
granuloma develop by adolescence
• Sturge weber – ipsilateral
lighter
• Sturge weber – ipsilateral ocular, pial and facial vascular anomalies
• Histo – marked dilatation of sto a ed d atat o ocapillaries and venules in superficial dermis
http://emedicine.medscape.com/article/459842-overview
Angiokeratoma• Localised or widespread• Dark red to black papules• Many covered by scales• Widespread AK – Fabry’s,
fucosidosis and sialidosisfucosidosis and sialidosis• No systemic findings are
associated with localised i k t
http://ades.tmu.edu.tw/english/pcare/course/Hemangioma/small/H i ht langiokeratoma
Clinical• Verrucous dark red to black
Hemangioma.html
Pathology NHLS,RXHVerrucous dark red to black• Minute – Mibelli type• Large – angiokeratoma
circumscriptumHisto –• Widely thin walled vascular• Widely thin-walled vascular
channels in upper dermis• Closely apposed to epidermis
Epithelioid haemangioma• Angiolymphoid hyperplasia
with eosinophilia
Pathology NHLS,RXH
• Polygonal / epithelioid endothelial cells lining capillary sized vasculature withsized vasculature with lymphocytes and/or endothelial cells
Pathology NHLS,RXH
http://ades.tmu.edu.tw/english/pcare/course/Hemangioma/small/Hemangioma.html
Lobular capillary haemangioma (pyogenic granuloma
Si l l id d l h• Single polypoid nodule that bleeds easily
• Face and lip – common sitesp• Also appears at sites of trauma
and infectionL b l f lif ti• Lobules of proliferating capillaries and fibroblasts
• Epithelial collarette
http://drkevinbrenner.com/wp-content/uploads/2011/04/DSCN0016.jpg
Pathology NHLS,RXHp
• at the base of the nodule
Congenital HaemangiomaSKIN and SOFT TISSUE
Infantile / juvenile haemangioma
Rapidly involuting haemangioma ( C )
Non-involuting haemangioma ( C )
Congenital Haemangioma(RICH) (NICH)
Growth and involution
Rapid postnatal growth,
Fully grown at birth; regresses by 12-18m
Fully grown at birth; fails to involuteinvolution growth,
stabilisation, followed by slow involution
regresses by 12 18m fails to involute
Appearance Strawberry like bright red colour at birth
Protruberant, hemispherical, violaceous and firm with central scar /
Only slightly raised, pink-purple
with central scar / ulcer
Sex F:M 3-5:1 1:1 1:1
The pathogenesis of RICH and NICH has not been elucidated, whereas IH are postulated to arise from embolized placental cells or angioblasts that differentiate p gtoward a placental microvascular phenotype
3. Bruckner A, Frieden I. Infantile hemangiomas. J Am Acad Dermatol 2006; 55:671
SKIN and SOFT TISSUE
Infantile / juvenile haemangioma
Rapidly involuting haemangioma (RICH)
Non-involuting haemangioma (NICH)( ) ( )
Radiology Homogenous signal; uniform parenchymal
MRI – larger flow voids, inhomogeneity
Homogenous signal; uniform parenchymalparenchymal
enhancementinhomogeneityAngiography –arterial aneurysms and direct A-V shunts
parenchymal enhancementArteriography –dilated arterial feeders and drainingshunts feeders and draining veins, like AVM but without early venous filling
Pathology Fat in involution Thrombi, infarction, haemosiderin, calcification, cysts, aneursyms, EMH in
Thrombi
involutionArchitecture, lobules, endothelium,
overlap overlap overlap
endothelium, capillary bm and extralobar vasculatureGLUT 1 P iti N ti N tiGLUT 1 Positive Negative Negative
May be variations of a single entity Pediatr Dev Pathol. 2003 Nov-Dec;6(6):495-510.
http://www.saintluc.be/en/services/vascular-anomalies/vascular-tumors.php
http://www.pedorthpath.com/infantile_hemangioma_gross_new.jpg
Pathology NHLS RXH
Pathology NHLS,RXH
Pathology NHLS,RXH
Pathology NHLS,RXH Pathology NHLS,RXH
Glut 1
Rapidly involuting congenital hemangioma: clinical and histopathologic features.Pediatr Dev Pathol. 2003 Nov-Dec;6(6):495-510.Berenguer B Mulliken JB Enjolras O Boon LM Wassef M Josset PBerenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich HP.
The lesion was light blue violet in color with central coarse telangiectases and peripheral pallor
Noninvoluting congenital hemangioma Jennifer A Stein MD PhD, Noushin Heidary MD, Melissa Pulitzer MD, Julie V Schaffer MD, Paula North MD PhDDermatology Online Journal 14 (5): 7
The lesion was light blue-violet in color, with central coarse telangiectases and peripheral pallor.
http://dermaamin.com/site/atlas-of-dermatology/13-n/1017-non-involuting-congenital-hemangioma-.html
Angiomatosisg• Diffuse neonatal angiomatosis – occurs in the first year of life• Widespread capillary haemangiomas involving skin and viscera esp.
GIT, brain, liver, lung, and with occasional associatedplacental chorangioma
Pathology NHLS,RXH
• Soft tissue angiomatosis – occurs in newborns and infants, lower extremities and buttocks, haphazard proliferation of vessels of varying i i ll l i l i ith l t i f ill i dsize, especially large irregular veins with clustering of capillary sized
vessels in venous walls or adjacent soft tissue, large amounts of adipose tissue, tendency for local recurrence
• Association with proteus syndrome
http://www.pedorthpath.com/page04.html
• Skeletal-extraskeletal angiomatosis – medullary cavity of bone +1 other siteC di l l h i• Can display cavernous lymphangioma, cavernous haemangioma and arteriovenous haemangioma pattern
• Vascuar tumours of bone (haemangiomas and lymphangiomas) may be associated with extensivelymphangiomas) may be associated with extensive absorption of bone or osteolysis – Gorham syndrome
• Can cross joints and result in loss of entire region of theCan cross joints and result in loss of entire region of the skeleton.
Vascular lesions of the liverVascular lesions of the liver
• Confusing terminology – infantile haemangioendothelioma, haemangioma g , g(cellular, capillary and cavernous) and haemangiomatosis frequently used tohaemangiomatosis frequently used to describe similar lesions.
Infantile haemangioendothelioma of theliver
• Type 1: proliferation of well-formed capillary spaces lined by a single layer of plump endothelial cellsShares histologic features with haemangiomasg gat other sites – early life, multicentricity, rapid early growth followed by involution
• Type 2: metastases and death; believed to represent low grade angiosarcomasrepresent low grade angiosarcomas
Hepatic haemangiomasHepatic haemangiomas• Single masses or more often multiple nodules
• Dark reddish brown masses composed of blood vessels filled with blood with or without areas of fibrosis, calcification and haemorrhageg
• Many cases = juvenile capillary haemangioma, GLUT 1 positive, as mptomatic hepatomegal incidental finding in the first fe eeksasymptomatic hepatomegaly, incidental finding in the first few weeks or months, multiple small nodules
Hepatic haemangiomasHepatic haemangiomas• Other cases featuring large abnormal vessels and arteriovenous
malformation like areas in association with a GLUT 1 negativemalformation-like areas in association with a GLUT 1 negative capillary proliferation – more likely represent vascular malformation, commonly associated with infarction, haemorrhage
d l ifi ti ( i il t RICH) ll t ti t bi thand calcification (similar to RICH), usually symptomatic at birth with severe oedema and congestive heart failure
Pathology NHLS,RXH
• Cavernous haemangioma –mainly in adults
• Associated findings – Kasabach Merrit syndrome and anaemia
• Large unresectable or multiple lesions can be treated by hepatic artery ligation, transarterial y p y gembolisation or transplantation
Skeletal muscle haemangiomaSkeletal muscle haemangioma
• Small vessel, large vessel or mixed types• Can occur during first yearCan occur during first year• More frequent- 2nd to 4th decade• Small cell variant – cellular pattern similar
to infantile haemangiomato a t e ae a g o a• May be impossible to distinguish
histologicall from angiomatosis clinicalhistologically from angiomatosis – clinical correlation essential.
Lymphatic malformation / lymphangioma
L h i i i t
http://www.huidziekten.nl/afbeeldingen/lymphangiomacystichygroma.jpg
Lymphangioma circumscriptum• Plaque like lesion – skin and
submucosa• Vesicles• Recurrent cellulitis and
ecchymosesy
L h ti filli th d i- Lymphatics filling the dermis –• dilated, thin walled, may get
haemorrhageg
Pathology NHLS,RXH
http://www.huidziekten.nl/afbeeldingen/lymphan
Cystic hygroma / lymphangioma• Soft multilobular masses with slight bluish
discolouration usually involve large areas
http://www.huidziekten.nl/afbeeldingen/lymphangiomacystichygroma.jpg
discolouration, usually involve large areas• Limb - lymphoedema
– poorly circumscribed, numerous irregular lymphatic channels, with variable smooth muscle
Pathology NHLS,RXHPathology NHLS,RXH
and D2-40 esp. in small channels
Surgery,RXH
LymphangiomatosisLymphangiomatosis• Multiple• Diffuse involvement of bone, soft tissue and / or viscera
D2-40
CD34
Pathology NHLS,RXH
Intermediate
Infantile kaposiform haemangioendothelioma (KHE)
Ab h lf d bi h Diff f hildh d• About half – noted at birth or develop in early infancy
• Red or purple cutaneous patch
Differs from common childhood haemangioma
• Sexes affected equallyp p pthat rapidly enlarges, eventually becoming an indurated deep soft tissue
q y• Occurs more often on the
trunk, in the retroperitoneumand the proximal portion of theindurated deep soft tissue
mass• May be associated with
K b h M it d
and the proximal portion of the extremities
• Large and infiltrative Kasabach-Merrit syndrome
http://www.saintluc.be/en/services/vascular-anomalies/vascular-tumors.php
Histology KHE• Angiomatous – rounded or dilated vascular spaces lined by flattened
endothelial cellsendothelial cells• Kaposiform – infiltrating fascicles, sheets or nodules of spindled
cells lining compressed (slit-like) or crescentic vascular channels –peripheryperiphery
• Microthrombi, haemosiderin and hyaline globules• GLUT 1 negative• HHV-8 negativeRetroperitoneum – high mortality
Pathology NHLS,RXH
Pathology NHLS,RXHgy ,
Tufted angiomaTufted angiomaB i l l i Hi ll l b l• Benign vascular lesion
• <20% present at birth• Histo – small lobules or
vascular tufts scatterred in a multiple ‘cannon-ball’like’
• Slow growth, can cover large areas of the trunk and neck
distribution in the dermis and subcutis
• Overlap with KHE• Also head and extremities• May present with Kasabach-
Merritt syndrome
Overlap with KHE• Rapid expansion may require
surgical excisionMerritt syndrome
Tufted angioma. Schaffer, JV et al. Dermatology Online Journal. 2008 14 (10) 20Pathology NHLS,RXH
Endovascular papillary angioendothelioma
D b k• Dabska tumour• Intravascular proliferation• May be congenitalMay be congenital• Diffuse swelling or intradermal mass• Rare cases metastasizes to lymph nodes• Surgery is effective treatment
Retiform haemangioendotheliomaDabska-retiform haemangioendothelioma
Pathology NHLS,RXH
Composite haemangioendothelioma (CHE)• Heterogenous• Retiform HE epithelioid HE cavernousRetiform HE, epithelioid HE, cavernous
haemangioma, spindle cell haemangiomaR l it l• Rarely congenital
• Tendency for persistent growth with local e de cy o pe s ste t g o t t ocarecurrence and rare incidence of metastasismetastasis
Giant cell angioblastomaGiant cell angioblastomaR i l d i f il l• Rare congenital and infantile vascular tumour
• Head, neck and extremities• Concentric arrays of oval to spindle cells and multinucleate cells areConcentric arrays of oval to spindle cells and multinucleate cells are
arranged around small vessels
http://ades.tmu.edu.tw/english/pcare/course/Hemangioma/small/Hemangioma.html
Vascular anomalies of skin and soft tissue
ISSVA WHOISSVA
• TUMOURS
WHO
Benign
• Haemangioma• Haemangioendothelioma
• Haemangioma (benign)Capillary, cavernous, AV, venous
• Lymphangiomag• Angiosarsoma
Lymphangioma• Angiomatosis
I t di t• MALFORMATIONS• Simple (slow flow) C,V,L
Intermediate• Haemangioendothelioma• Kaposi sarcomaSimple (slow flow) C,V,L
• Simple (fast flow) A• Combined, AVM,C-V,C-L,
Malignant• EpithelioidCombined, AVM,C V,C L,
L-V• Epithelioid
haemangioendothelioma• Angiosarcoma
Take home messagesTake home messages
• Benign vascular tumours are very common and most frequently occur in the q yskin
• At all sites it is often difficult to determine• At all sites, it is often difficult to determine whether benign vascular lesions are
fmalformations, true neoplasms or, in some cases, reactive processesp
Take home messagesTake home messages
• Immunohistiochemistry in the context of vascular lesions:
• CD31 pan-vascular marker• CD34 > blood vessel endotheliumCD34 blood vessel endothelium• D2-40 (podoplanin) > lymphatic
endotheliumendothelium• Glut 1 glucose transporter protein - positive in
endothelia at sites of blood tissue barrier
Take home messagesTake home messages• Congenital infantile haemangioma (Glut 1 positive),
RICH and NICH (Glut 1 negative) have overlapping features and may be variations of a single entityfeatures and may be variations of a single entity
T pe I hepatic infantile haemangioendothelioma• Type I hepatic infantile haemangioendothelioma = haemangiomaType II hepatic infantile haemangioendothelioma =Type II hepatic infantile haemangioendothelioma = angiosarcoma
• Haemangioendothelioma – intermediate malignancy except epithelioid haemangioendotheliomaexcept epithelioid haemangioendothelioma
ReferencesReferencesDiagnostic paediatric s rgical patholog B Neil Sebire MB BS FRCPath Marian Malone MB BCh BAO• Diagnostic paediatric surgical pathology, By Neil Sebire, MB, BS, FRCPath, Marian Malone, MB, BCh, BAO, FRCPath, Michael Ashworth, MBBCh, FRCPath and Thomas S. Jacques, MA, PhD, MRCP, FRCPath. Churchill Livingstone, 2010
• Potter's Pathology of the Fetus Infant and ChildPotter s Pathology of the Fetus, Infant and Child• Enid Gilbert-Barness AO MD FRCPA FRCPath DSci(hc) MD(hc) (Editor), Raj P. Kapur MD PhD (Editor), Luc
Laurier Oligny MSc MD (Editor), Joseph R. Siebert PHD (Editor) , Elsevier Inc, 2007
• Stocker and Dehner's Pediatric PathologyStocker and Dehner s Pediatric Pathology • J. Thomas Stocker (Author), Louis Dehner (Author), Aliya Husain (Author) , Lippincott Williams & Wilkins, 2011
• Pathology and Genetics of Tumours of Soft Tissue and Bone World Health Organization Classification ofPathology and Genetics of Tumours of Soft Tissue and Bone. World Health Organization Classification of Tumours. Edited by Christopher D.M. Fletcher, K. Krishnan Unni, Fredrik Mertens
• IARC Press Lyon, 2002
Rapidly involuting congenital hemangioma: clinical and histopathologic features.Rapidly involuting congenital hemangioma: clinical and histopathologic features.• Pediatr Dev Pathol. 2003 Nov-Dec;6(6):495-510.• Berenguer B, Mulliken JB, Enjolras O, Boon LM, Wassef M, Josset P, Burrows PE, Perez-Atayde AR, Kozakewich
HP.
THANK YOUAcknowledgement: RXH NHLS Histopathology staffAcknowledgement: RXH NHLS Histopathology staff
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