软组织肿瘤的病理诊断之五

血管肿瘤第一部分

良性肿瘤滑膜血管瘤•

起自于滑膜表面的良性血管增生•

非常少见•

患者多为儿童和青少年•

男性多见部位和临床表现•

膝，肘，手•

缓慢性生长，常伴有肿胀和关节渗液•

1/3的病例为无痛性•

MRI

有助于术前诊断大体形态•

口径大小不等的血管，充血性•

周界清楚，或为弥漫性组织学•

海绵状血管瘤为主•

少数为毛细血管瘤，或其他血管类型•

增生的血管位于滑膜下方，间质呈黏液样或纤维样•

可以有较多的含铁血黄素沉着预后•

小的病变可以完整性切除，一般不会复发•

病变较为广泛者较难做到完整性切除，可复发血管内乳头状血管内皮增生Intravascular

Papillary

Endothelial

Hyperplasia，IPEHIPEH的三种类型(1)

发生于血管内;(2)

发生于一些前驱血管病变的基础上，包括血管瘤（特别是深部的海绵状血管瘤或肌内血管瘤）、血管畸形、痣或曲张的静脉;(3)发生于血管外，与血肿相关组织形态•

周界清楚•

可见扩张的薄壁静脉，常见血栓形成•

病变早期，增生的内皮细胞沿血栓的轮廓表面生长，并形成以纤维素为轴心的粗乳头状结构•

典型病例则以附着于血管壁向腔内生长的无数纤细乳头为特征•

乳头表面衬覆单层肥胖或肿胀的内皮细胞，其轴心为胶原化的纤维组织有点类似胎盘结构反应性血管内皮瘤病reactive

angioendotheliomatosis反应性血管内皮增生•

是一种发生于皮肤的血管反应性增生•

病因不明，但75%的患者与一些系统性疾病如亚急性细菌性心内膜炎、结核、冷蛋白血症、冷球蛋白血症、淋巴造血系统疾患、肝肾功能衰竭、自身免疫性疾病（如风湿性关节炎和系统性红斑狼疮）和免疫抑制（如肝肾移植后和骨髓移植后）等有密切的关系临床表现•

可发生于任何年龄，但多见于中老年•

多累及肢体、面部和躯干，呈多灶性的红斑、紫癜性丘疹、瘀斑和紫癫性斑块•

临床上类似卡波西肉瘤、血管肉瘤、移植后的排异反应和坏疽性脓皮病等病

例介绍例1,男性,

83岁,因反复腹痛、腹泻及血便1年,皮肤结节6个月入院。皮肤科检查:双侧膝关节以下、左手有大小不等的高出于皮面的紫红或黑褐色硬性结节状斑块,约150个,斑块直径013～8

cm,边缘呈浸润状,压之不褪色。部分结节呈斑块融合,有压痛或溃破,表面形成黄白色痂壳例2女性,

37岁,遇冷后颜面及双下肢起红斑、淤斑,保暖后消退3年,复发加重4天入院。皮肤科检查:双侧颜面、臀部及双下肢可见暗红色斑,扪之质韧,压痛,表面破溃实验室检查例1

外周血流式细胞术T淋巴细胞分类计数示总T淋巴细胞相对比为01855,

CD4

+

/CD8

+为0.7

(正常值1.4～2.2)例2

红细胞冷凝集试验效价为1∶80(正常值<

1

∶40)

。组织形态•

位于真皮内，少数病例可累及至皮下•

边界不清，由多个簇状增生的毛细血管组成•

按生长方式大致可分为弥漫型、小叶状和弥漫－小叶混合型三种•

少数病例中可见扩张的海绵状血管，另在一部分病例中，可见血管分割胶原纤维的现象，类似皮肤血管肉瘤鉴别诊断•Kaposi肉瘤•血管肉瘤•获得性簇状血管瘤治疗和预后•

支持治疗•

局部切除•

属于一种自限性疾病，多数病例预后良好，一部分病例可复发杆菌性血管瘤病Bacillary

Angiomatosis杆菌性血管瘤病•

与革兰阴性杆菌-巴尔通体（Bartonella

）感染相关的反应性血管增生•

几乎均发生于HIV/AIDS

或免疫抑制（肝肾移植，激素和白血病等）的患者临床表现•

多为30~60岁间的成年人•

多表现为皮下多发性结节，常见于躯干、手臂和头面部;•

可累及淋巴结、脏器黏膜、肝（肝紫癜症）和脾•

采用红霉素和其他的抗生素治疗组织形态•

由分叶状的毛细血管型血管组成，类似分叶状毛细血管瘤•

特征性形态之一为血管之间的间质内含有颗粒状粉红色或紫色物质

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特殊检查•

Warthin-Starry染色显示为杆菌•

杆菌三层细胞壁结构鉴别诊断•

化脓性肉芽肿：息肉样，由分叶状排列的毛细血管组成•

表皮可有溃疡，或呈衣领状•

HE

染色或Warthin-Starry染色无杆菌先天性血管瘤Infantile

Hemangioma先天性血管瘤的两种类型•

快速消退型Rapidly

involuting

congenital

hemangioma

(RICH)•

不消退型Noninvoluting

congenital

hemangioma

(NICH)临床表现发生率•

0.3%•

RICH

比NICH多见•

出生时既有，可在产前超声发现•

无性别差异发生部位•

头颈部，肢体（邻近关节）•

孤立性

外生性病变•

RICH

可显示肝脏±

皮肤病变RICH•

真皮内，大小不等的分叶状，毛细血管•

小叶中央有薄壁引流血管•

小叶间致密的纤维组织•

局部含铁血黄素沉着，髓外造血•

可以有核分裂象•

消退改变起始于病变中央•

小叶减少，间质纤维化，囊性区域，血管内血栓•

消退后无纤维脂肪残留（与婴幼儿血管瘤不同）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•

相对比较大的小叶状毛细血管•

小叶中央引流血管比较大，星状，常呈扩张状•

内皮细胞常呈钉突样•

小叶间纤维血管间隔，含有大的发育不良的静脉，和淋巴管样血管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鉴别

诊断婴幼儿血管瘤（Infantile

Hemangioma）•

大多数并不发生于出生时（发生于出生后几周）•

出生后生长迅速（超过婴儿本身的发育）•

GLUT1(+)•

与婴幼儿血管瘤相比，先天性血管瘤含有较高的

FLT-1

(VEGFR-R1)表达和

IGF-2低表达婴幼儿毛细血管瘤•

是一种主要由毛细血管型血管组成的血管瘤•

是婴幼儿最常见的血管瘤•

发病率为1/100新生儿，约占所有血管肿瘤的32%~42%临床表现•

多见于儿童，常发生于生后数周内生后数月增生1~12岁开始可有消退•

可发生于任何部位，但好发于头面部，尤以口唇及眼睑部为多见

，其次见于颈部和躯干的皮肤•

病变隆起于皮肤，边界清晰，鲜红色

或紫红色，直径数毫米至2~3cm，加压不褪色，也不缩小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.免疫组化•

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鉴别诊断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(-)化脓性肉芽肿•

又称肉芽组织型血管瘤•

是毛细血管瘤的一种特殊亚型•

病变位于皮肤或黏膜表面，呈息肉状生长•

镜下由小叶状的增生性毛细血管型血管组成，常伴有表皮溃疡和间质水肿临床表现•

患者多为20岁以上的成年人•

好发部位依次为牙龈、手指、唇、面部和舌•

多数病例发展较快，病程常在2个月之内，1/3有轻微外伤史Intravascular

Pyogenic

granulomaintravenous

pyogenic

granuloma病例•

男，30岁左右•

丁丁头上红色小丘疹，痛•

取一个活检AE1/AE3CD31CD31您的诊断•

毛细血管瘤•

上皮样血管内皮瘤•

上皮样血管瘤•

上皮样血管肉瘤CD31ERG您的诊断•

毛细血管瘤•

上皮样血管内皮瘤•

上皮样血管瘤•

上皮样血管肉瘤上皮样血管瘤•

是一种内皮细胞呈上皮样的良性血管肿瘤•

20-40岁，女性略多见•

头部（特别是前额），耳周，头皮，肢体远端（指趾）•

少数病例可发生于阴茎、骨和深部软组织临床表现•

多数病例表现为局部肿块•

常可在同一解剖部位同时有多个病灶•

多数病变位于皮下•

病程多<1年，偶可达15年大体形态•

05-2.0cm，极少超过5cm•

结节状，可有出血•

一些病变周界清楚，类似淋巴结（周围可有淋巴组织）组织学形态•

小的毛细血管型血管，或小静脉•

内衬胖上皮样细胞，胞浆嗜伊红色，可呈空泡状•

部分病例间质内可含有大量的嗜酸性粒细胞和淋巴细胞•

部分病例可有实性成分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•

周皮细胞表达a-SMACD31预后•

局部复发率

1/3鉴别诊断•

上皮样血管肉瘤FOS-LMNA

gene

fusionDifferential

DiagnosisKimura

Disease•

Endemic

in

Asian

population•

Associated

with

lymphadenopathy,

peripheral

eosinophilia,

elevatedserum

IgE•

Prominent

eosinophils;

lacks

epithelioid

endothelial

cells皮肤上皮样血管瘤样结节Cutaneous

Epithelioid

Angiomatoid

Nodule，CEANCEAN•

由Brenn和Fletcher于2004年首先描述•

好发于中青年，年龄范围为15~79岁•

多发生于躯干和四肢皮肤，少数病例位于面部或鼻黏膜，偶可发生于阴茎•

大多数病例表现为单个红色或红棕色丘疹样至蓝色皮肤结节组织学•

位于真皮浅层•

周界清楚，结节状•

表皮可伴有增生高倍镜•

结节由实性片状增生的多边形上皮样细胞组成•

胞质丰富，透亮状或嗜伊红色，胞质内常含有空泡，核呈空泡状，核仁明显•

尽管血管腔隙并不明显，但至少在局部可见有血管腔隙形成Composed

of

apredominantly

solid

andsheet-like

proliferation

oflarge

polygonal

epithelioidcells

containing

abundanteosinophilic

to

clearcytoplasm

and

vesicularnuclei

with

prominentnucleoli梭形细胞血管瘤•

是一种发生于浅表软组织的血管肿瘤

，曾被称为梭形细胞血管内皮瘤（中间性），现认为是一种良性血管瘤•

由海绵状血管瘤样区域和实性梭形细胞区域组成，海绵状血管腔隙内有时可见机化性血栓及静脉石，梭形实质内偶含空泡状上皮样的内皮细胞临床表现•

好发于20~40岁间的青年人，男性略多见•

多发生于四肢的远端，部分病例也可位于躯干及头颈部等处•

多表现为皮肤或皮下单个结节，无色或浅蓝色，部分患者可伴有疼痛感•

约30%~40%的患者可为多发性，但病灶多分布于同一区域内•

可伴有

Maffucci

syndrome

(multiple

enchondromas

+

spindle

cellhemangioma)大体形态•

呈单个或多个红色或紫红色小结节•

周界相对清楚，但无包膜，直径在0.3~11cm，多不超过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组织形态•

病变位于真皮或皮下•

由海绵状血管瘤样区域和实性梭形细胞区域两种成分组成•

两种成分在不同病例之间比例不等•

扩张的血管内可有血栓形成•

梭形细胞区域内可见巢状空泡状上皮样瘤细胞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肌内血管瘤•

一种发生于肌肉组织内的良性血管增生，常伴有多少不等的成熟脂肪组织•

发病年龄较广，但青少年和青年人多见•

病程多为数年•

无性别差异部位和临床表现•

多发生于下肢（特别是大腿）；其次为头颈部，上肢和躯干•

少数病例发生于纵隔和腹膜后，偶可位于心肌•

缓慢性生长的肿块，常有痛感，特别是运动以后•

MRI术前可作出诊断大体形态•

如为毛细血管型多呈实性的灰白或灰黄色肿块，可有小的血管样腔隙•

如为大的血管，则可见明显的血管性腔隙，常可见出血或血栓•

含有较多的脂肪组织时可呈黄色•

最大径可超过10～15cm组织学•

毛细血管型•

毛细血管－静脉型•

海绵状血管型•

混合型（动脉、静脉、毛细血管），加上脂肪组织可呈错构瘤样预后•

局部复发率

30-50%•

取决于病变的大小和切缘情况Senile

hemangioma•

Cherry

hemangioma•

Ruby

spots•

Campbell

de

Morgan

spotsVerrucous

Hemangioma•

Usually

present

at

birth•

Well-circumscribed

hyperkeratotic

linear

vascular

plaques鞋钉样血管瘤•

又称靶样含铁血黄素沉着性血管瘤

（

Targeted

hemosiderotic

hemangioma）•

过去认为病变在肉眼上呈孤立性圆形，中央呈紫色，向外依次为透明和瘀斑状空晕，似靶样，镜下间质内多有含铁血黄素沉着，故命名为靶样含铁血黄素沉着性血管瘤a

halo

(“targetoid”

appearance)临床表现•

好发于青年人，平均年龄为30岁•

多发生在四肢、躯干、臀部和头颈部的皮肤•

呈血管瘤样或带有色素的外生性肿块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获得性簇状血管瘤•

又称中川血管母细胞瘤获得性进展性毛细血管瘤•

是一种在真皮内生长，由不规则的毛细血管型血管结节组成的良性肿瘤

，血管结节呈炮弹头样向位于周边的新月形血管腔内突出，形成血管内的“簇状”结构临床表现•

儿童和青少年•

颈部，肩部，躯干上部•

缓慢性

生长的红斑或斑块•

可伴有Kasabach-Merritt

综合症（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.肾小球样血管瘤•

是一种反应性的血管增生•

在扩张的血管腔内可见增生的毛细血管襻，类似肾小球，故而得名•

由Chan等于1990年首先报道Prof.

JKC

Chan临床表现•

常发生于伴有多灶性Castleman’s

病和POEMS综合征的患者•

POEMS：Polyneuropathy：运动和感觉神经Organomegaly：

肝脾肿大、淋巴结肿大Endocrinopathy：闭经、男性乳房发育、甲状腺功能低下、肾上腺机能不全、性无能M-protein：骨髓浆细胞增多、异常蛋白血症Skin

disease：血管瘤、色素沉着、多毛症、皮肤增厚组织形态•

位于真皮浅层内，可见多个扩张性的血管•

在扩张的血管腔内可见增生的毛细血管，类似肾小球毛细血管襻•

形成所谓的“血管在血管内”图像血管瘤病•

血管的弥漫性增生•

生长方式：垂直性（累及多个组织平面）或累及同一类型的组织（如多组肌肉）血管瘤病•

2/3的患者发生于20岁以内•

女性略多见•

多发生于下肢，其次为胸壁，腹壁，上肢•

受累部位呈弥漫性肿胀，病变的大小会有变化，会受重体力作业影响•

如有明显的动静脉吻合支存在，则可有皮肤温度上升，震颤，

悸动，受累部位肥大等大体形态•

界限不清的肿块，数cm至十几cm组织学形态•

大的动脉、静脉和小的毛细血管混合组成•

弥漫性分布于组织内•

静脉外形不规则，可有成簇的小静脉或毛细血管从静脉出芽式生长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预后•

尽管是良性病变，但90%术后仍有病变存在•

50%多次复发•

病变弥漫，切除不净淋巴管瘤•

由扩张的海绵状或囊状淋巴管组成•

多发生于出生时或1岁以内•

颈部、腋下和腹股沟淋巴管瘤病•

好发于儿童，无性别差异•

可伴发血管瘤，成为Maffucci’s综合征的一部分•

是一种非常少见的异常发育，病变弥漫累及实质脏器（如肺、胃肠道、肝和脾等）、骨或软组织硬化性血管瘤样结节性转化sclerosing

angiomatoid

nodular

transformationSANTSANT•

是一种发生于脾脏的非肿瘤性血管病变•

病变起自于红髓•

好发于中青年•

多见于女性，女：男约为2：1•

约半数以上的病例为体检时偶然发现（包括影像学检查），或为其他原因剖腹探查时所发现•

16%的病例可表现为腹痛、上腹部或腰背部不适，少数病例因脾肿大或腹部肿块就诊临床病史•

34岁女性•

脾脏肿块一周•

CT

提示

“血管瘤”•

大体:

切面可见6cmX5cmX5cm灰白色肿块镜下形态•

病变主要位于红髓，由多个散在分布、境界清楚的圆形或卵圆形血管瘤样结节和纤维硬化的间质组成•

结节大小不一，可呈融合状，结节周围为同心圆状排列的纤维组织SANT的组织学•

小的结节周围有一层不完整的胶原纤维带•

高倍镜下，血管瘤样结节由蜂窝状的血管腔隙组成，含有红细胞、毛细血管和窦样血管•

血管样结节由含有淋巴细胞和浆细胞的间隔所分开周围血管母细胞瘤•

脊神经根•

胸腔•

腰骶•

颈部•

肾脏•

肠道•

部分病例伴有VHL（Von

Hippel-Lindau

syndrome）容易犯的错误一上皮样血管平滑肌脂肪瘤容易犯的错误二透明细胞肾细胞癌容易犯的错误三脂肪肉瘤容易犯的错误四血管瘤CD34S100NSEa-inhibin吻合状血管瘤Anastomosing

hemangioma吻合状血管瘤•

最初报道在泌尿生殖道•

新近肝和胃肠道也有报道吻合状血管瘤•

6例发生于泌尿生殖道者均发生于成年人，中位年

龄为59.5岁，年龄范围49~75岁。4例发生于肾脏，

2例发生于睾丸。临床上，2例发生于肾脏者对以

间隙性血尿就诊，1例腹部隐痛•

6例发生于肝和胃肠道者均发生于成年人，48-71岁组织学形态•

镜下呈疏松的小叶状结构，由交通状或吻合状的血管组成，内皮细胞可呈鞋钉样，无核分裂象•

病变内偶可见髓外造血Lymphangioma•

Lymphangioma

circumscriptum

(superficial

lymphangioma/LAC）•

Lymphangiomatosis•

Cystic

lymphangioma

(cystic

hygroma)•

Deep

lymphangioma

(cavernous

lymphangioma)淋巴管瘤•

由扩张的海绵状或囊状淋巴管组成•

多发生于出生时或1岁以内•

颈部、腋下和腹股沟淋巴管瘤病•

好发于儿童，无性别差异•

可伴发血管瘤，成为Maffucci’s综合征的一部分•

是一种非常少见的异常发育，病变弥漫累及实质脏器（如肺、胃肠道、肝和脾等）、骨或软组织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