富于脂肪軟組織腫瘤

富于脂肪的軟組織腫瘤總醫院病理科脂肪組織的種類成熟脂肪組織不成熟脂肪組織棕色脂肪組織其他伴隨組織的形態和結構血管、膠原纖維、軟骨、平滑肌造血組織良性梭形細胞惡性梭形細胞細胞小圓形惡性上皮細胞炎癥細胞WHO脂肪組織腫瘤脂肪瘤脂肪瘤病神經脂肪瘤病脂肪母細胞瘤/脂肪母細胞瘤病血管脂肪瘤平滑肌脂肪瘤軟骨樣脂肪瘤腎外血管平滑肌脂肪瘤梭形細胞/多形性脂肪瘤棕色瘤（hibernoma)良性惡性去分化脂肪肉瘤粘液型脂肪肉瘤圓細胞脂肪肉瘤多形性脂肪肉瘤混合型脂肪肉瘤未分類脂肪肉瘤（not

otherwise

specified)棕色脂肪冬眠瘤FIG.

1.

Age

and

gender

distribution

of

170

cases

of

hibernoma.From:

Furlong:

Am

J

Surg

Pathol,

Volume

25(6).June

2001.809-814Pale-staining

hibernoma

cells

with

less

eosinophilic

cytoplasm

areidentified

in

the

pale

cell

subtype

of

typical

hibernoma

togetherwith

some

white

fat

cells.The

eosinophilic

subtype

of

typical

hibernoma

iscomposedpredominantly

of

granular-appearing

brown

fat

cells

withprominent

nucleoli.The

myxoid

variant

has

hibernoma

cells

floating

in

aloosemyxoidmatrix.Only

rare

hibernoma

cells

are

identified

in

the

lipoma-likesubtype.不成熟幼稚脂肪組織脂肪空泡大小差別顯著可見很小的單個脂肪空泡多脂肪空泡脂肪空泡分散細胞核相對增大，染色質常常深染富于薄壁小血管各階段脂肪母細胞脂肪母細胞瘤脂肪母細胞瘤和脂肪母細胞瘤病非常少見，幾乎均發生于兒童，尤其是3歲以下兒童。脂肪母細胞瘤有包膜，病

限。而脂肪母細胞瘤病沒有包膜，病變彌漫?但兩者組織學形態相同。典型病例由大小不等的脂肪小葉構成，由成熟脂肪組織和各階段脂肪母細胞組成，之間為厚薄不一的纖維組織分割。多數病例纖維分割帶細胞稀疏，與脂肪纖維瘤病容易鑒別。Lipoblastomas

are

rare

benign

tumors

most

often

seen

in

infants

and

youngchildren.They

exhibit

a

wide

range

of

cellular

differentiation

and

maturation,fromlipoblasts

to

mature

adipocytes.Complete

resection

achieves

optimal

results,

but

recurrence

is

possibleandclose

follow-up

is

mandatory.脂肪母細胞瘤或脂肪母細胞瘤病非常重要。成人幼兒不要輕易該病需要慎重，而嬰脂肪肉瘤。梭形及多型性脂肪瘤梭形及多形性脂肪瘤相對少見，約占脂肪組織腫瘤的1.5％。遠少于脂肪瘤。梭形細胞脂肪瘤和多形性脂肪瘤

(SCPLs),在組織形態上有相似和連續性，具有相同的

學異常，被認為屬于同一種腫瘤類型。由成熟脂肪組織、良性但缺乏分化的梭形細胞以及多少不等的繩索狀嗜酸性膠原構成。伴有或不伴有少量的脂肪母細胞。多形性細胞、奇異細胞、梭形細胞以及花環狀巨細胞是多形性脂肪瘤的特點。免疫組化表達CD34,梭形細胞和多形性細胞不表達S-100Typical

example

of

intradermal

spindle

cell

lipoma

withpoorly

defined

margins.lipomaspindle

cell

lipomapleomorphic

lipomaintermediate

power(B)

had

characteristicfloret-likemultinucleate

giantcells.不典型脂肪瘤樣腫瘤/高分化脂肪肉瘤脂肪瘤樣型硬化型是局部侵襲性的腫瘤，腫瘤多數或部分由成熟脂肪組織構成，細胞大小有顯著差異，并且脂肪細胞和間質細胞至少有局灶異型性?？梢妴蝹€或多個境界清楚的胞漿內空泡，分布于增大的深染核周圍。根據腫瘤部位和能否再次切除決定是否應用不典型脂肪瘤性腫瘤名稱。多見于肢體深部軟組織，尤其是大腿。其次為腹膜后，旁區域以及縱隔。也可有發生于皮下，皮膚少見。多數為中年人，兒童非常少見。Typical

well-differentiated aofthelipoma-like

type(a)andthesclerosing

type

(b).

Numerous

lipoblasts

are

evident

in

the

former

(a),

whereaslipoblasts

may

be

sparse

in

the

latter

(b).Conventional

atypical

lipomatous

tumor.

There

is

maturefat

with

occasional

enlarged,

atypical

nuclei.

B,Conventional

atypical

lipomatous

tumor

with

fatty

andfibrous

areas.

Most

of

the

cells

with

enlarged,

atypicalnuclei

arein

the

fibrous

component,

and

cellularity

islow.C,Predominantly

fibrousconventional

atypical

lipomatoustumor.

No owcellularity.From:

Evans:

Am

J

Surg

Pathol,

Volume

31(1).January2007.1-14A,Cellular

atypical

lipomatous

tumor.

A

modera y

cellularfibrousarea

ispresent

alongside

mature

fat.B,Modera y

cellular

fibrouszone

incellular

atypical

tumor.There

ismoderate

nuclear

pleomorphism

anda

storiformpattern.C,

Highly

cellular

fibrous

component

in

cellularatypicallipomatous

tumor.

This

was

not

considered

dedifferentiatedbecause

the al

mitotic

rate

(3mitotic

figuresper

10high-powerfields)wastoolow.A,

A

highly

cellular

but

nonpleomorphic

myxoid

area

incellularatypical

lipomatous

tumor,

with

somewhat

increased

vascularity.B,

Thismodera y

cellular

myxoid

zone

in

a

cellular

atypicallipomatous

tumor

demonstrates

marked

nuclear

pleomorphism

andaprominent

vascular

network.C,

A

modera y

cellular

lipogenic

region

in

a

cellular

atypicallipomatous

tumor.

The

fat

cells

are

of

considerably

smallerthanadultsize.主要鑒別脂肪瘤：細胞大小，間質細胞異型性，有無脂肪母細胞（單泡或多泡）脂肪母細胞瘤：，分葉結構，間質細胞異型性梭形細胞/多型性脂肪瘤：境界清楚，頸后及肩部皮下，很少見于下肢。成熟脂肪、繩索狀膠原、脂肪母細胞較少，間質細胞核深染，無明顯異型性。Atypical

lipomatous

tumor/well-differentiatedadeveloped

in

theretroperito ofa

60-year-old

man.

A,Histologic

aspect.

B,

Positivity

of

CDK4.

C,

Positivity

ofMDM2inseveral

tumor

cells

inthe

sclerosing

component.D,

MDM2-positive

cells

are

rare

and

may

be

difficult

toshow

in

the

adipocytic

component成熟脂肪組織脂肪瘤（?。┌橛衅渌夹猿煞郑貉苤玖觯w維脂肪瘤，軟骨脂肪瘤，髓脂肪瘤，平滑肌脂肪瘤，嬰兒纖維性錯構瘤其他良性梭形細胞病變累及脂肪：纖維母細胞/肌纖維母細胞性腫瘤，纖維組織細胞性腫瘤其他惡性梭形細胞腫瘤累及脂肪淋巴造血細胞腫瘤累及脂肪轉移癌累及皮下或者網膜組織各種脂膜炎脂肪瘤（?。┥窠浿玖霾≈窘M織累及神經使得神經增粗多見于正中神經及其手指分支，可伴有巨指。受累神經

內及周圍有脂肪組織和纖維組織浸潤，分隔神經束。血管脂肪瘤多見于前臂，其次為軀干和上臂表現為皮下多發小結節，小于2公分由成熟脂肪和分支毛細血管構成，血管內可見纖維素血栓，脂肪與血管比例不固定。平滑肌脂肪瘤由成熟平滑肌和成熟脂肪組織組成非常罕見，多數為成年人大部分位于腹腔、腹膜后和

區的深部軟組織，也可發生于胸部四肢皮下大小10－25，平均15cm。二者比例約2：1SMA

Desmin陽性主要鑒別：脂肪肉瘤血管平滑肌脂肪瘤平滑肌及其他梭形細胞腫瘤累及脂肪軟骨樣脂肪瘤含有脂肪母細胞、成熟脂肪、和軟骨樣基質，與粘液樣脂肪肉瘤和骨外粘液樣軟骨肉瘤相似罕見，好發于成人多見于肢體近端，也可見于軀干頭頸部組織學特點為大量單泡或多泡脂肪母細胞排列呈巢或索，分布于粘液樣或軟骨基質中，混雜有不等量的成熟脂肪。嬰兒纖維性錯構瘤發生于嬰幼兒的良性表淺軟組織腫瘤。境界不清，由致密纖維膠原組織、疏松的幼稚小圓形間質細胞以及成熟脂肪呈樣排列。多見于前后胸壁，腋窩，上肢以及肩部等部位，絕大多數見于2歲以內。Fibrous

hamartoma

of

infancy乳腺型肌纖維母細胞瘤良性間葉性腫瘤，由具有肌纖維母細胞特征的梭形細胞，透明變性的粗大膠原間質，豐富的肥大細胞以及成熟脂肪構成。與乳腺的肌纖維母細胞瘤相似。多見，壁等多見于成年

，以

/還可見于腹壁、臀部、背部、處。Desmin

CD34陽性Admixed

with

adipose

tissueResemble

mammarymyofibroblastomaMammary

typemyofibroblastomaMalepredomiantAdultpatientsMammary

typemyofibroblastomapositive

for

desminand

CD34鑒別

：血管肌纖維母細胞瘤富于細胞血管纖維瘤梭形細胞脂肪瘤脂肪瘤樣型血管外皮細胞瘤?脂肪多少、形態及血管多少、形態有助鑒別AngiomyofibroblastomaFemalepredomiantWell

circumscribedAdultsPelviperinealsubcutaneousPromiant

small

thinwalled

vesslesLoose

edematousstromaRound

to

spindle

cellsDesmin

actin

PR

ERpositiveMultinucleate

cells

andepithelioid

cluster

inangiomyofibroblastoma脂肪纖維瘤病發生于兒童的纖維脂肪性腫瘤曾稱為嬰兒纖維瘤病好發于四肢末端，大小1－3cm由纖維性梭形細胞和成熟脂肪組成CD34

BCL-2

S100

Actin

常有表達復發率高，但不轉移。lipofibromatosisLipofibromatosis,

c:

focal

SMA

positivity

indicate

fibro/myofibroblasticdifferentiation鑒別

：嬰兒纖維性錯構瘤平滑肌脂肪瘤纖維脂肪瘤乳腺型肌纖維母細胞瘤其他梭形細胞腫瘤累及脂肪Fibrous

hamartoma

of

infancyEarlychildhoodPoorlycircumscribedSolitary

and

rapidgrowingSuperficial

softtissueFeets

and

hands

rareMorphologicaly

threecomponents脂肪瘤性血管外周細胞瘤少見生長緩慢，不復發和轉移由成熟脂肪細胞和血管外周細胞瘤樣結構組成。細胞無異型性，多見于四肢和腹膜后軟組織CD99

CD34

BCL-2陽性LipomatoushemangiopericytomaLipomatous

hemangiopericytoma:

positive

for

CD34血管平滑肌脂肪瘤多見于腎臟、肝臟等部位屬于

A范疇由厚壁血管、平滑肌和脂肪組織組成免疫組化表達SMA,

HMB45等髓脂肪瘤多見于腎上腺由造血組織和成熟脂肪構成其他腫瘤累及脂肪脂肪多數位于腫瘤邊緣多見于淋巴瘤等良性腫瘤多數為纖維母/肌纖維母細胞性腫瘤以及纖維組織細胞性腫瘤梭形細胞腫瘤累及脂肪需要與纖維脂肪瘤、平滑肌脂肪瘤、梭形細胞脂肪瘤、嬰兒纖維性錯構瘤、脂肪瘤樣血管外皮細胞瘤、高分化脂肪肉瘤（硬化型）、去分化脂肪肉瘤以及脂肪肉瘤伴平滑肌等分化鑒別。還要與含有脂肪組織的纖維母/肌纖維母細胞瘤鑒別（如乳腺型肌纖維母細胞瘤、富于細胞血管纖維瘤等）含脂肪組織腫瘤

的要點（嬰兒多見的腫瘤有那些