軟組織腫瘤的病理診斷之五

血管腫瘤第一部分

良性腫瘤滑膜血管瘤?

起自于滑膜表面的良性血管增生?

非常少見?

患者多為兒童和青少年?

男性多見部位和臨床表現(xiàn)?

膝，肘，手?

緩慢性生長，常伴有腫脹和關(guān)節(jié)滲液?

1/3的病例為無痛性?

MRI

有助于術(shù)前診斷大體形態(tài)?

口徑大小不等的血管，充血性?

周界清楚，或?yàn)閺浡越M織學(xué)?

海綿狀血管瘤為主?

少數(shù)為毛細(xì)血管瘤，或其他血管類型?

增生的血管位于滑膜下方，間質(zhì)呈黏液樣或纖維樣?

可以有較多的含鐵血黃素沉著預(yù)后?

小的病變可以完整性切除，一般不會復(fù)發(fā)?

病變較為廣泛者較難做到完整性切除，可復(fù)發(fā)血管內(nèi)乳頭狀血管內(nèi)皮增生Intravascular

Papillary

Endothelial

Hyperplasia，IPEHIPEH的三種類型(1)

發(fā)生于血管內(nèi);(2)

發(fā)生于一些前驅(qū)血管病變的基礎(chǔ)上，包括血管瘤（特別是深部的海綿狀血管瘤或肌內(nèi)血管瘤）、血管畸形、痣或曲張的靜脈;(3)發(fā)生于血管外，與血腫相關(guān)組織形態(tài)?

周界清楚?

可見擴(kuò)張的薄壁靜脈，常見血栓形成?

病變早期，增生的內(nèi)皮細(xì)胞沿血栓的輪廓表面生長，并形成以纖維素為軸心的粗乳頭狀結(jié)構(gòu)?

典型病例則以附著于血管壁向腔內(nèi)生長的無數(shù)纖細(xì)乳頭為特征?

乳頭表面襯覆單層肥胖或腫脹的內(nèi)皮細(xì)胞，其軸心為膠原化的纖維組織有點(diǎn)類似胎盤結(jié)構(gòu)反應(yīng)性血管內(nèi)皮瘤病reactive

angioendotheliomatosis反應(yīng)性血管內(nèi)皮增生?

是一種發(fā)生于皮膚的血管反應(yīng)性增生?

病因不明，但75%的患者與一些系統(tǒng)性疾病如亞急性細(xì)菌性心內(nèi)膜炎、結(jié)核、冷蛋白血癥、冷球蛋白血癥、淋巴造血系統(tǒng)疾患、肝腎功能衰竭、自身免疫性疾?。ㄈ顼L(fēng)濕性關(guān)節(jié)炎和系統(tǒng)性紅斑狼瘡）和免疫抑制（如肝腎移植后和骨髓移植后）等有密切的關(guān)系臨床表現(xiàn)?

可發(fā)生于任何年齡，但多見于中老年?

多累及肢體、面部和軀干，呈多灶性的紅斑、紫癜性丘疹、瘀斑和紫癲性斑塊?

臨床上類似卡波西肉瘤、血管肉瘤、移植后的排異反應(yīng)和壞疽性膿皮病等病

例介紹例1,男性,

83歲,因反復(fù)腹痛、腹瀉及血便1年,皮膚結(jié)節(jié)6個(gè)月入院。皮膚科檢查:雙側(cè)膝關(guān)節(jié)以下、左手有大小不等的高出于皮面的紫紅或黑褐色硬性結(jié)節(jié)狀斑塊,約150個(gè),斑塊直徑013～8

cm,邊緣呈浸潤狀,壓之不褪色。部分結(jié)節(jié)呈斑塊融合,有壓痛或潰破,表面形成黃白色痂殼例2女性,

37歲,遇冷后顏面及雙下肢起紅斑、淤斑,保暖后消退3年,復(fù)發(fā)加重4天入院。皮膚科檢查:雙側(cè)顏面、臀部及雙下肢可見暗紅色斑,捫之質(zhì)韌,壓痛,表面破潰實(shí)驗(yàn)室檢查例1

外周血流式細(xì)胞術(shù)T淋巴細(xì)胞分類計(jì)數(shù)示總T淋巴細(xì)胞相對比為01855,

CD4

+

/CD8

+為0.7

(正常值1.4～2.2)例2

紅細(xì)胞冷凝集試驗(yàn)效價(jià)為1∶80(正常值<

1

∶40)

。組織形態(tài)?

位于真皮內(nèi)，少數(shù)病例可累及至皮下?

邊界不清，由多個(gè)簇狀增生的毛細(xì)血管組成?

按生長方式大致可分為彌漫型、小葉狀和彌漫－小葉混合型三種?

少數(shù)病例中可見擴(kuò)張的海綿狀血管，另在一部分病例中，可見血管分割膠原纖維的現(xiàn)象，類似皮膚血管肉瘤鑒別診斷?Kaposi肉瘤?血管肉瘤?獲得性簇狀血管瘤治療和預(yù)后?

支持治療?

局部切除?

屬于一種自限性疾病，多數(shù)病例預(yù)后良好，一部分病例可復(fù)發(fā)桿菌性血管瘤病Bacillary

Angiomatosis桿菌性血管瘤病?

與革蘭陰性桿菌-巴爾通體（Bartonella

）感染相關(guān)的反應(yīng)性血管增生?

幾乎均發(fā)生于HIV/AIDS

或免疫抑制（肝腎移植，激素和白血病等）的患者臨床表現(xiàn)?

多為30~60歲間的成年人?

多表現(xiàn)為皮下多發(fā)性結(jié)節(jié)，常見于軀干、手臂和頭面部;?

可累及淋巴結(jié)、臟器黏膜、肝（肝紫癜癥）和脾?

采用紅霉素和其他的抗生素治療組織形態(tài)?

由分葉狀的毛細(xì)血管型血管組成，類似分葉狀毛細(xì)血管瘤?

特征性形態(tài)之一為血管之間的間質(zhì)內(nèi)含有顆粒狀粉紅色或紫色物質(zhì)

Low-power

examination

of

bacillary

angiomatosis

(BA)

demonstrates

asuperficial

dermal

proliferation

of

blood

vessels

in

a

lobular

configurationIntermediate

magnification

of

BA

shows

a

proliferation

of

small

blood

vessels

arranged

in

a

vaguely

lobular

configuration

with

prominent

stromal

edema,

mild

fibrosis,

and

mixed

inflammatory

infiltrate

High

magnification

of

bacillary

angiomatosis

demonstrates

a

proliferation

of

small

blood

vessels

with

swollen

endothelial

cellssurrounded

by

edema

and

acute

inflammation

with

leukocytoclasia特殊檢查?

Warthin-Starry染色顯示為桿菌?

桿菌三層細(xì)胞壁結(jié)構(gòu)鑒別診斷?

化膿性肉芽腫：息肉樣，由分葉狀排列的毛細(xì)血管組成?

表皮可有潰瘍，或呈衣領(lǐng)狀?

HE

染色或Warthin-Starry染色無桿菌先天性血管瘤Infantile

Hemangioma先天性血管瘤的兩種類型?

快速消退型Rapidly

involuting

congenital

hemangioma

(RICH)?

不消退型Noninvoluting

congenital

hemangioma

(NICH)臨床表現(xiàn)發(fā)生率?

0.3%?

RICH

比NICH多見?

出生時(shí)既有，可在產(chǎn)前超聲發(fā)現(xiàn)?

無性別差異發(fā)生部位?

頭頸部，肢體（鄰近關(guān)節(jié)）?

孤立性

外生性病變?

RICH

可顯示肝臟±

皮膚病變RICH?

真皮內(nèi)，大小不等的分葉狀，毛細(xì)血管?

小葉中央有薄壁引流血管?

小葉間致密的纖維組織?

局部含鐵血黃素沉著，髓外造血?

可以有核分裂象?

消退改變起始于病變中央?

小葉減少，間質(zhì)纖維化，囊性區(qū)域，血管內(nèi)血栓?

消退后無纖維脂肪殘留（與嬰幼兒血管瘤不同）RICH

is

composed

of

well-defined,

variably

sized

lobules

ofcapillaries

that

are

separated

by

dense

fibrous

tissue

.

Theinterlobular

vascular

component

is

often

times

less

prominentthan

that

seen

in

NICH.RICHAlthough

they

can

be

seen

in

both

subtypes

of

congenitalhemangioma,

the

lobular

vessels

within

RICH

morefrequently

contain

organizing

fibrin

thrombi

with

evidenceof

recanalizationRICHNICH?

相對比較大的小葉狀毛細(xì)血管?

小葉中央引流血管比較大，星狀，常呈擴(kuò)張狀?

內(nèi)皮細(xì)胞常呈釘突樣?

小葉間纖維血管間隔，含有大的發(fā)育不良的靜脈，和淋巴管樣血管The

lobular

endothelial

cells

within

NICH

are

often

small,hyperchromatic,

and

bulge

into

the

vascular

lumen,

impartinga

hobnailed

appearanceNICHThe

interlobular

stroma

of

NICH

frequently

contains

abundantlarge

and

irregularly

shaped

venous,

arterial,

and

lymphatic-likestructuresNICHMany

of

the

interlobular

vessels

in

NICH

are

distorted

in

shapeand

composition.

This

stellate-shaped,

venous-like

structurehas

poorly

developed

media

with

variable

areas

of

thicknessand

deficient

smooth

muscle

and

elastic

tissue.NICHLobular

endothelial

cells

of

NICH

may

contain

foci

ofintracytoplasmic

eosinophilic

globules,

which

tend

to

clusterNICH鑒別

診斷嬰幼兒血管瘤（Infantile

Hemangioma）?

大多數(shù)并不發(fā)生于出生時(shí)（發(fā)生于出生后幾周）?

出生后生長迅速（超過嬰兒本身的發(fā)育）?

GLUT1(+)?

與嬰幼兒血管瘤相比，先天性血管瘤含有較高的

FLT-1

(VEGFR-R1)表達(dá)和

IGF-2低表達(dá)嬰幼兒毛細(xì)血管瘤?

是一種主要由毛細(xì)血管型血管組成的血管瘤?

是嬰幼兒最常見的血管瘤?

發(fā)病率為1/100新生兒，約占所有血管腫瘤的32%~42%臨床表現(xiàn)?

多見于兒童，常發(fā)生于生后數(shù)周內(nèi)生后數(shù)月增生1~12歲開始可有消退?

可發(fā)生于任何部位，但好發(fā)于頭面部，尤以口唇及眼瞼部為多見

，其次見于頸部和軀干的皮膚?

病變隆起于皮膚，邊界清晰，鮮紅色

或紫紅色，直徑數(shù)毫米至2~3cm，加壓不褪色，也不縮小Infantile

hemangioma

is

characterized

by

a

dermal

&/orsubcutaneous

proliferation

of

tightly

packed

capillaries

growingThe

proliferative

phase

demonstrates

increased

cellularity

withdense,

solid-appearing

areas

containing

inconspicuous

vascularlumina

.

These

solid-appearing

areas

are

composed

of

plumpendothelial

cells

and

pericytes.During

the

proliferative

phase,

the

endothelial

cells

andpericytes

are

plump

with

bland

cytologic

featuresEarly

proliferative

lesions

may

be

mitotically

active

withmultiple

mitotic

figures

observed

per

high-powered

fieldThe

periphery

of

infantile

hemangiomas

involute

first

witheventual

involution

of

the

remainder

of

the

lesion.

As

such,within

the

same

specimen,

features

of

both

the

proliferativeand

involution

phases

may

be

evident.During

involution,

the

overall

cellularity

of

the

lesion

decreasessecondary

to

progressively

increasing

interstitial

fibrosisDuring

the

involution

phase

of

infantile

hemangioma,

thecapillaries

become

more

dilated

and

the

endothelial

cells

areflattenedLate

in

the

involution

phase,

the

remaining

vessels

oftendevelop

thickened

basement

membranes

with

subsequentToward

the

end

stage

of

involution,

the

vascular

component

ofinfantile

hemangiomas

are

progressively

replaced

by

fibrousand

fatty

tissue

.

A

few

small

lobules

of

capillaries

may

persist.免疫組化?

GLUT1,

IGF-2,

CD31,

ERG,

CD34,

F8,

WT1?

Pericytes

are

SMA(+)Infantile

hemangiomas

are

immunoreactive

with

GLUT1,

whichstains

the

cytoplasm

of

the

endothelial

cells

in

all

phases

ofdevelopment鑒別診斷Congenital

Hemangioma?

In

utero

growth

with

fully

developed

lesions

present

at

birth?

Rapidly

involuting

type

regresses

faster

than

infantile

hemangiomas?

Noninvoluting

type

does

not

regress

and

grows

commensurately

withthe

child?

Hemosiderin

and

extramedullary

hematopoiesis

commonly

present?

GLUT1(-)Lobular

Capillary

Hemangioma

(Pyogenic

Granuloma)?

Usually

older

children

and

adolescents

(mean

age:

6

years

in

pediatricpopulation)?

Polypoid

or

sessile

mass

±

ulceration?

Lobule(s)

of

capillaries

surrounded

by

an

epidermal/mucosalcollarette?

GLUT1(-)化膿性肉芽腫?

又稱肉芽組織型血管瘤?

是毛細(xì)血管瘤的一種特殊亞型?

病變位于皮膚或黏膜表面，呈息肉狀生長?

鏡下由小葉狀的增生性毛細(xì)血管型血管組成，常伴有表皮潰瘍和間質(zhì)水腫臨床表現(xiàn)?

患者多為20歲以上的成年人?

好發(fā)部位依次為牙齦、手指、唇、面部和舌?

多數(shù)病例發(fā)展較快，病程常在2個(gè)月之內(nèi)，1/3有輕微外傷史Intravascular

Pyogenic

granulomaintravenous

pyogenic

granuloma病例?

男，30歲左右?

丁丁頭上紅色小丘疹，痛?

取一個(gè)活檢AE1/AE3CD31CD31您的診斷?

毛細(xì)血管瘤?

上皮樣血管內(nèi)皮瘤?

上皮樣血管瘤?

上皮樣血管肉瘤CD31ERG您的診斷?

毛細(xì)血管瘤?

上皮樣血管內(nèi)皮瘤?

上皮樣血管瘤?

上皮樣血管肉瘤上皮樣血管瘤?

是一種內(nèi)皮細(xì)胞呈上皮樣的良性血管腫瘤?

20-40歲，女性略多見?

頭部（特別是前額），耳周，頭皮，肢體遠(yuǎn)端（指趾）?

少數(shù)病例可發(fā)生于陰莖、骨和深部軟組織臨床表現(xiàn)?

多數(shù)病例表現(xiàn)為局部腫塊?

?？稍谕唤馄什课煌瑫r(shí)有多個(gè)病灶?

多數(shù)病變位于皮下?

病程多<1年，偶可達(dá)15年大體形態(tài)?

05-2.0cm，極少超過5cm?

結(jié)節(jié)狀，可有出血?

一些病變周界清楚，類似淋巴結(jié)（周圍可有淋巴組織）組織學(xué)形態(tài)?

小的毛細(xì)血管型血管，或小靜脈?

內(nèi)襯胖上皮樣細(xì)胞，胞漿嗜伊紅色，可呈空泡狀?

部分病例間質(zhì)內(nèi)可含有大量的嗜酸性粒細(xì)胞和淋巴細(xì)胞?

部分病例可有實(shí)性成分At

low

power,

it

typically

shows

a

vaguely

lobular

growthpattern

and

may

be

associated

with

a

small

artery

or

veinThe

lesional

endothelial

cells

of

EH

are

usually

plump

andepithelioid

and

have

eosinophilic

or

amphophilic

cytoplasm.

Anassociated

brisk

chronic

inflammatory

infiltrate

is

typical免疫表型?

ERG，CD31，CD34?

可局灶性表到AE1/AE3?

周皮細(xì)胞表達(dá)a-SMACD31預(yù)后?

局部復(fù)發(fā)率

1/3鑒別診斷?

上皮樣血管肉瘤FOS-LMNA

gene

fusionDifferential

DiagnosisKimura

Disease?

Endemic

in

Asian

population?

Associated

with

lymphadenopathy,

peripheral

eosinophilia,

elevatedserum

IgE?

Prominent

eosinophils;

lacks

epithelioid

endothelial

cells皮膚上皮樣血管瘤樣結(jié)節(jié)Cutaneous

Epithelioid

Angiomatoid

Nodule，CEANCEAN?

由Brenn和Fletcher于2004年首先描述?

好發(fā)于中青年，年齡范圍為15~79歲?

多發(fā)生于軀干和四肢皮膚，少數(shù)病例位于面部或鼻黏膜，偶可發(fā)生于陰莖?

大多數(shù)病例表現(xiàn)為單個(gè)紅色或紅棕色丘疹樣至藍(lán)色皮膚結(jié)節(jié)組織學(xué)?

位于真皮淺層?

周界清楚，結(jié)節(jié)狀?

表皮可伴有增生高倍鏡?

結(jié)節(jié)由實(shí)性片狀增生的多邊形上皮樣細(xì)胞組成?

胞質(zhì)豐富，透亮狀或嗜伊紅色，胞質(zhì)內(nèi)常含有空泡，核呈空泡狀，核仁明顯?

盡管血管腔隙并不明顯，但至少在局部可見有血管腔隙形成Composed

of

apredominantly

solid

andsheet-like

proliferation

oflarge

polygonal

epithelioidcells

containing

abundanteosinophilic

to

clearcytoplasm

and

vesicularnuclei

with

prominentnucleoli梭形細(xì)胞血管瘤?

是一種發(fā)生于淺表軟組織的血管腫瘤

，曾被稱為梭形細(xì)胞血管內(nèi)皮瘤（中間性），現(xiàn)認(rèn)為是一種良性血管瘤?

由海綿狀血管瘤樣區(qū)域和實(shí)性梭形細(xì)胞區(qū)域組成，海綿狀血管腔隙內(nèi)有時(shí)可見機(jī)化性血栓及靜脈石，梭形實(shí)質(zhì)內(nèi)偶含空泡狀上皮樣的內(nèi)皮細(xì)胞臨床表現(xiàn)?

好發(fā)于20~40歲間的青年人，男性略多見?

多發(fā)生于四肢的遠(yuǎn)端，部分病例也可位于軀干及頭頸部等處?

多表現(xiàn)為皮膚或皮下單個(gè)結(jié)節(jié)，無色或淺藍(lán)色，部分患者可伴有疼痛感?

約30%~40%的患者可為多發(fā)性，但病灶多分布于同一區(qū)域內(nèi)?

可伴有

Maffucci

syndrome

(multiple

enchondromas

+

spindle

cellhemangioma)大體形態(tài)?

呈單個(gè)或多個(gè)紅色或紫紅色小結(jié)節(jié)?

周界相對清楚，但無包膜，直徑在0.3~11cm，多不超過2cmMR

image

of

a

patient

with

Maffucci

syndrome

displayshyperintense

bone

lesions

consistent

with

enchondromas

inthe

calcaneus

and

distal

tibia.

The

hyperintense

plantarsubcutaneous

nodule

is

a

spindle

cell

hemangioma組織形態(tài)?

病變位于真皮或皮下?

由海綿狀血管瘤樣區(qū)域和實(shí)性梭形細(xì)胞區(qū)域兩種成分組成?

兩種成分在不同病例之間比例不等?

擴(kuò)張的血管內(nèi)可有血栓形成?

梭形細(xì)胞區(qū)域內(nèi)可見巢狀空泡狀上皮樣瘤細(xì)胞cavernous

vascular

spacescellular

zones

of

spindleSome

of

the

endothelial

cells

display

clear

cytoplasmic

vacuolesand

are

often

clustered

together

within

the

cellular

zonescreating

a

striking

resemblance

to

entrapped

groups

ofminiaturized

adipocytes.

This

is

a

characteristic

feature

of

SCHVacuolated

endothelial

cells

can

occasionally

be

quitenumerous

and

prominent

in

SCH,

as

depicted

in

this

image.AncillaryTests?

Expression

of

vascular

markers

(CD31,

CD34,

ERG)?

Often

have

IDH1

(or

IDH2)

mutationTop

Differential

Diagnoses?

Kaposi

sarcoma

(tumor

stage)?

Kaposiform

hemangioendothelioma?

Epithelioid

hemangioendothelioma?

Organizing

thrombus/intravascular

papillary

endothelial

hyperplasia?

Cavernous

hemangioma

(venous

malformation)Kaposi

Sarcoma

(Tumor

Stage)?

Usually

dermal-based

rather

than

subcutaneous?

Usually

in

either

HIV(+)

patients

or

in

elderly?

Generally

lacks

cavernous

areas?

Greater

cytologic

atypia

and

more

mitoses

than

SCH?

More

prominent

slit-like

or

sieve-like

vascular

channels

with

moreabundant

erythrocyte

extravasation?

Plasma

cells

often

present?

HHV-8

(LANA1)

(+)

by

immunohistochemistry肌內(nèi)血管瘤?

一種發(fā)生于肌肉組織內(nèi)的良性血管增生，常伴有多少不等的成熟脂肪組織?

發(fā)病年齡較廣，但青少年和青年人多見?

病程多為數(shù)年?

無性別差異部位和臨床表現(xiàn)?

多發(fā)生于下肢（特別是大腿）；其次為頭頸部，上肢和軀干?

少數(shù)病例發(fā)生于縱隔和腹膜后，偶可位于心肌?

緩慢性生長的腫塊，常有痛感，特別是運(yùn)動以后?

MRI術(shù)前可作出診斷大體形態(tài)?

如為毛細(xì)血管型多呈實(shí)性的灰白或灰黃色腫塊，可有小的血管樣腔隙?

如為大的血管，則可見明顯的血管性腔隙，常可見出血或血栓?

含有較多的脂肪組織時(shí)可呈黃色?

最大徑可超過10～15cm組織學(xué)?

毛細(xì)血管型?

毛細(xì)血管－靜脈型?

海綿狀血管型?

混合型（動脈、靜脈、毛細(xì)血管），加上脂肪組織可呈錯構(gòu)瘤樣預(yù)后?

局部復(fù)發(fā)率

30-50%?

取決于病變的大小和切緣情況Senile

hemangioma?

Cherry

hemangioma?

Ruby

spots?

Campbell

de

Morgan

spotsVerrucous

Hemangioma?

Usually

present

at

birth?

Well-circumscribed

hyperkeratotic

linear

vascular

plaques鞋釘樣血管瘤?

又稱靶樣含鐵血黃素沉著性血管瘤

（

Targeted

hemosiderotic

hemangioma）?

過去認(rèn)為病變在肉眼上呈孤立性圓形，中央呈紫色，向外依次為透明和瘀斑狀空暈，似靶樣，鏡下間質(zhì)內(nèi)多有含鐵血黃素沉著，故命名為靶樣含鐵血黃素沉著性血管瘤a

halo

(“targetoid”

appearance)臨床表現(xiàn)?

好發(fā)于青年人，平均年齡為30歲?

多發(fā)生在四肢、軀干、臀部和頭頸部的皮膚?

呈血管瘤樣或帶有色素的外生性腫塊Microscopic?

Vascular

proliferation

with

wedge-shaped

appearance?

Superficial

vessels

are

dilated

and

thin

walled?

Deeper

vessels

are

progressively

smaller?

Vessels

are

lined

by

small,

bland-appearing

endothelial

cells

withhobnail

appearance?

Focal

papillary

projections

with

fibrous

cores

may

be

present?

Hemorrhage

and

hemosiderin

deposition

are

typically

prominent?

Inflammation

is

usually

minimalHigher

magnification

of

the

superficial

portion

of

the

tumorshows

small

endothelial

cells

protruding

into

the

dilatedvascular

spacesHistologic

examination

of

the

deeper

aspect

of

the

lesionshows

smaller

blood

vessels

and

prominent

hemosiderindeposition

in

the

stromaDifferential

DiagnosisProgressive

Lymphangioma?

Thin-walled,

dilated

superficial

vascular

spaces

with

narrower

deepervessels?

Lacks

hobnail

endothelial

cell

morphology

and

hemosiderin

deposition

ofHHRetiform

Hemangioendothelioma?

Dermal

and

subcutaneous

tumor

characterized

by

proliferation

ofarborizing

vessels

lined

by

hobnail

endothelial

cells?

Typically

prominent

lymphoid

infiltrate,

which

is

lacking

in

HH?

HH

is

usually

more

superficial,

and

deeper

vessels

are

smaller

and

compact獲得性簇狀血管瘤?

又稱中川血管母細(xì)胞瘤獲得性進(jìn)展性毛細(xì)血管瘤?

是一種在真皮內(nèi)生長，由不規(guī)則的毛細(xì)血管型血管結(jié)節(jié)組成的良性腫瘤

，血管結(jié)節(jié)呈炮彈頭樣向位于周邊的新月形血管腔內(nèi)突出，形成血管內(nèi)的“簇狀”結(jié)構(gòu)臨床表現(xiàn)?

兒童和青少年?

頸部，肩部，軀干上部?

緩慢性

生長的紅斑或斑塊?

可伴有Kasabach-Merritt

綜合癥（消耗性凝血病）Shows

a

diffuse

dermal

proliferation

of

small,

elongated

blood

vessels

withnarrow

to

collapsed

lumina

and

a

background

of

dermal

sclerosis.Clinical

Issues?

Typically

occurs

in

adult

females?

Painless

bluish

or

red

nodule?

Often

occurs

on

extremities,

trunk,

or

breast?

Complete

excision

is

curative,

but

not

necessary

given

benign

nature

oflesions?

Excellent

prognosis,

no

malignant

potentiala

cutaneous

sinusoidal

hemangioma

with

large,

dilated

vascular

spaces.腎小球樣血管瘤?

是一種反應(yīng)性的血管增生?

在擴(kuò)張的血管腔內(nèi)可見增生的毛細(xì)血管襻，類似腎小球，故而得名?

由Chan等于1990年首先報(bào)道Prof.

JKC

Chan臨床表現(xiàn)?

常發(fā)生于伴有多灶性Castleman’s

病和POEMS綜合征的患者?

POEMS：Polyneuropathy：運(yùn)動和感覺神經(jīng)Organomegaly：

肝脾腫大、淋巴結(jié)腫大Endocrinopathy：閉經(jīng)、男性乳房發(fā)育、甲狀腺功能低下、腎上腺機(jī)能不全、性無能M-protein：骨髓漿細(xì)胞增多、異常蛋白血癥Skin

disease：血管瘤、色素沉著、多毛癥、皮膚增厚組織形態(tài)?

位于真皮淺層內(nèi)，可見多個(gè)擴(kuò)張性的血管?

在擴(kuò)張的血管腔內(nèi)可見增生的毛細(xì)血管，類似腎小球毛細(xì)血管襻?

形成所謂的“血管在血管內(nèi)”圖像血管瘤病?

血管的彌漫性增生?

生長方式：垂直性（累及多個(gè)組織平面）或累及同一類型的組織（如多組肌肉）血管瘤病?

2/3的患者發(fā)生于20歲以內(nèi)?

女性略多見?

多發(fā)生于下肢，其次為胸壁，腹壁，上肢?

受累部位呈彌漫性腫脹，病變的大小會有變化，會受重體力作業(yè)影響?

如有明顯的動靜脈吻合支存在，則可有皮膚溫度上升，震顫，

悸動，受累部位肥大等大體形態(tài)?

界限不清的腫塊，數(shù)cm至十幾cm組織學(xué)形態(tài)?

大的動脈、靜脈和小的毛細(xì)血管混合組成?

彌漫性分布于組織內(nèi)?

靜脈外形不規(guī)則，可有成簇的小靜脈或毛細(xì)血管從靜脈出芽式生長Numerous

small

capillaries

are

distributed

within

and

adjacentto

this

thick-walled

artery

in

this

example

of

angiomatosis.A

large

cavernous

vessel

with

an

irregular

wall

is

surroundedby

numerous

small

to

medium-sized

capillariesNumerous

small

and

dilated

capillaries

are

shown

infiltrating

thefibrous

connective

tissue

and

extending

down

into

the

adjacentadipose

tissue.Scattered

vessels

of

varying

size

and

wall

thickness

are

seen

within

thefibrous

connective

tissue

and

lobules

of

mature

adipose

tissue,consisting

of

muscular

venous

,

cavernous

,

and

capillary-type

vessels預(yù)后?

盡管是良性病變，但90%術(shù)后仍有病變存在?

50%多次復(fù)發(fā)?

病變彌漫，切除不凈淋巴管瘤?

由擴(kuò)張的海綿狀或囊狀淋巴管組成?

多發(fā)生于出生時(shí)或1歲以內(nèi)?

頸部、腋下和腹股溝淋巴管瘤病?

好發(fā)于兒童，無性別差異?

可伴發(fā)血管瘤，成為Maffucci’s綜合征的一部分?

是一種非常少見的異常發(fā)育，病變彌漫累及實(shí)質(zhì)臟器（如肺、胃腸道、肝和脾等）、骨或軟組織硬化性血管瘤樣結(jié)節(jié)性轉(zhuǎn)化sclerosing

angiomatoid

nodular

transformationSANTSANT?

是一種發(fā)生于脾臟的非腫瘤性血管病變?

病變起自于紅髓?

好發(fā)于中青年?

多見于女性，女：男約為2：1?

約半數(shù)以上的病例為體檢時(shí)偶然發(fā)現(xiàn)（包括影像學(xué)檢查），或?yàn)槠渌蚱矢固讲闀r(shí)所發(fā)現(xiàn)?

16%的病例可表現(xiàn)為腹痛、上腹部或腰背部不適，少數(shù)病例因脾腫大或腹部腫塊就診臨床病史?

34歲女性?

脾臟腫塊一周?

CT

提示

“血管瘤”?

大體:

切面可見6cmX5cmX5cm灰白色腫塊鏡下形態(tài)?

病變主要位于紅髓，由多個(gè)散在分布、境界清楚的圓形或卵圓形血管瘤樣結(jié)節(jié)和纖維硬化的間質(zhì)組成?

結(jié)節(jié)大小不一，可呈融合狀，結(jié)節(jié)周圍為同心圓狀排列的纖維組織SANT的組織學(xué)?

小的結(jié)節(jié)周圍有一層不完整的膠原纖維帶?

高倍鏡下，血管瘤樣結(jié)節(jié)由蜂窩狀的血管腔隙組成，含有紅細(xì)胞、毛細(xì)血管和竇樣血管?

血管樣結(jié)節(jié)由含有淋巴細(xì)胞和漿細(xì)胞的間隔所分開周圍血管母細(xì)胞瘤?

脊神經(jīng)根?

胸腔?

腰骶?

頸部?

腎臟?

腸道?

部分病例伴有VHL（Von

Hippel-Lindau

syndrome）容易犯的錯誤一上皮樣血管平滑肌脂肪瘤容易犯的錯誤二透明細(xì)胞腎細(xì)胞癌容易犯的錯誤三脂肪肉瘤容易犯的錯誤四血管瘤CD34S100NSEa-inhibin吻合狀血管瘤Anastomosing

hemangioma吻合狀血管瘤?

最初報(bào)道在泌尿生殖道?

新近肝和胃腸道也有報(bào)道吻合狀血管瘤?

6例發(fā)生于泌尿生殖道者均發(fā)生于成年人，中位年

齡為59.5歲，年齡范圍49~75歲。4例發(fā)生于腎臟，

2例發(fā)生于睪丸。臨床上，2例發(fā)生于腎臟者對以

間隙性血尿就診，1例腹部隱痛?

6例發(fā)生于肝和胃腸道者均發(fā)生于成年人，48-71歲組織學(xué)形態(tài)?

鏡下呈疏松的小葉狀結(jié)構(gòu)，由交通狀或吻合狀的血管組成，內(nèi)皮細(xì)胞可呈鞋釘樣，無核分裂象?

病變內(nèi)偶可見髓外造血Lymphangioma?

Lymphangioma

circumscriptum

(superficial

lymphangioma/LAC）?

Lymphangiomatosis?

Cystic

lymphangioma

(cystic

hygroma)?

Deep

lymphangioma

(cavernous

lymphangioma)淋巴管瘤?

由擴(kuò)張的海綿狀或囊狀淋巴管組成?

多發(fā)生于出生時(shí)或1歲以內(nèi)?

頸部、腋下和腹股溝淋巴管瘤病?

好發(fā)于兒童，無性別差異?

可伴發(fā)血管瘤，成為Maffucci’s綜合征的一部分?

是一種非常少見的異常發(fā)育，病變彌漫累及實(shí)質(zhì)臟器（如肺、胃腸道、肝和脾等）、骨或軟組織Benign

Lymphangioendothelioma?

Present

as

slow-growing

brownish

erythematous

plaques

or

theyresemble

hematomaBenign

lymphangioendothelioma

on

the

posterior

aspect

of

the

right

armBenign

lymphangioendothelioma

on

the

anterior

abdominal

wallA:

At

low

magnification,

the

lesion

is

formed

byvascular

spaces

with

irregular

lumina

that

dissectdermal

collagen

bundles.

B:

Jagged

vascular

slitslined

by

endothelial

cells

between

collagen

bundlesin

the

dermis

The

same

case

immunohistochemically

studied

withD2-40/podoplanin.

F:

The

irregular

vascular

spacesare

lined

by

D2-40/podoplanin

positive

lymphaticendothelial

cells.Massive

Localized

Lymphedema?

Pseudoneoplastic

process

relat