软组织肿瘤的病理诊断之三

病例病理诊断隆突性皮纤维肉瘤

（DFSP）CD34KP-1Who

is

Who？2013

WHO软组织肿瘤分类•

脂肪肿瘤•

纤维母细胞/肌纤维母细胞肿瘤•

纤维组织细胞肿瘤•

平滑肌肿瘤•

血管周细胞肿瘤•

骨骼肌肿瘤•

血管肿瘤•

软骨-骨肿瘤•

胃肠道间质瘤•

神经鞘肿瘤•

分化不确定类肿瘤•

未分化/不能分类肿瘤纤维组织细胞性肿瘤皮肤纤维组织细胞瘤位于真皮内表皮可有增生表皮基底细胞可有色素沉着基底部相对清楚基底部也可伸入脂肪组织两侧边缘常穿插粗大胶原纤维常可见席纹状结构（非DFSP所特有）常可见泡沫样组织细胞常可见多核细胞（杜顿细胞等）常可见含铁血黄素沉着纤维组织细胞瘤的免疫组化•

无特异性标记物•

FXIIIa

-

非肿瘤性树突状细胞•

泡沫样组织细胞，巨噬细胞，杜顿巨细胞表达CD68（KP1，PGM1），CD163等组织细胞标记Intralesional

dendritic

cells

are

positive

for

FXIIIasiderphages

are

positive

for

KP-1multinucleated

giant

cells

are

positive

for

KP-1KP-1Touton-type

giant

cells

are

positive

for

KP-1易被误诊的几种情形•

席纹状排列，底部

深入脂肪，CD34部分阳性Vs.

DFSP•

含有大量含铁血黄素沉着

Vs.

恶性黑色素瘤•

动脉瘤样

Vs.

血管瘤样•

富于细胞性纤维组织细胞瘤

Vs.

低度恶性肿瘤•

非典型性纤维组织细胞瘤

Vs.

非典型性纤维黄色瘤BFHDFSP含铁血黄素沉着性纤维组织细胞瘤动脉瘤样

Vs.

血管瘤样

纤维组织细胞瘤血管瘤样纤维组织细胞瘤•

EMA•

CD99•

Desmin•

FISH：EWSR1EMADesminCD99EWSR1

apart

probe富于细胞性纤维组织细胞瘤SMA深部纤维组织细胞瘤深部纤维组织细胞瘤•

周界相对清楚•

类似皮肤富于细胞性纤维组织细胞瘤•

细胞多形性不明显•

泡沫样细胞/多核巨细胞多少不等•

40-50%

分支状血管（血管外皮瘤样）•

核分裂象易见•

可有灶性坏死•

偶可见畸形细胞•

40%的病例CD34/SMA阳性周界相对清楚类似皮肤富于细胞性纤维组织细胞瘤可见血管外皮瘤样结构非典型性纤维组织细胞瘤非典型性纤维组织细胞瘤

Vs.

非典型纤维黄色瘤Elizabeth

K

Satter

MD

MPHDermatology

Online

Journal

18

(9):

3AFH和AFX的鉴别诊断AFHAFX年龄部位

青少年四肢和躯体

老年人头颈部（日光暴晒）组织学

BFH

+

bizarre

cells多形性未分化肉瘤几种少见的纤维组织细胞瘤亚型上皮样纤维组织细胞瘤脂质化（踝型）纤维组织细胞瘤Lipidized

(ankle-type)BFH疤痕疙瘩样真皮纤维瘤Keloidal

dermatofibroma真皮肌纤维瘤（斑块样真皮纤维瘤病）Terminology•

Giant

cell

tumor

of

tendon

sheath,

nodular

tenosynovitis,

localizedpigmented

villonodular

synovitis

(PVNS)•

Benign

soft

tissue

tumor

of

synovial

origin•

Arises

from

tendon

sheath,

intraarticular

site,

or

bursaClinical

Issues•

2nd

most

common

tumor

of

hand•

Any

age;

peak:

3rd-4th

decade•

Digits

(85%)•

Around

large

joints

(10%)•

Benign

but

recurs

locally

(15-20%)•

Intraarticular

tumors

called

localized

PVNSMicroscopic•

Polymorphous

population

of

epithelioid

cells,

macrophages,

andosteoclast-like

giant

cells•

Well

demarcated•

Multinodular

with

fibrous

septa•

Stromal

fibrosis•

Mitotic

rate

1-20

per

10

high-power

fields

(average:

5

per

10

HPF)Localized-type

TGCT

is

composed

of

a

polymorphous

cellpopulation

including

large

epithelioid

cells

with

abundanteosinophilic

cytoplasm

and

eccentric

vesicular

nuclei

,mononuclear

stromal

cells

with

smaller

round

or

reniform

nuclei

,and

osteoclast-like

giant

cells

.

Note

the

hemosiderin

deposits

.Clusterin

ImmunostainStromal

FibrosisPseuodoglandular

PatternTop

Differential

Diagnoses•

Diffuse-type

tenosynovial

giant

cell

tumor

(PVNS)•

Giant

cell

tumor

of

soft

tissue•

Fibroma

of

tendon

sheath•

DermatofibromaTerminology•

Generally

benign

but

locally

aggressive

neoplastic

proliferation

ofsynovial

originIntra-

or

extraarticular•

Synonym:

Pigmented

villonodular

synovitis

(PVNS)丛状纤维组织细胞瘤•

Reported

in

1988

by

Enzinger

and

Zhang•

儿童和青少年，

70%

<20岁•

上肢

(50%),

下肢

(20%),

躯干

(15%)

和头颈部

(10%)Prof.

EnzingerProf.

Zhang

（张仁元）•

位于真皮深层和皮下交界处•

丛状或多个结节状•

单核组织细胞样细胞+破骨样巨细胞（可不明显）•

结节周围肌纤维母细胞丛状纤维组织细胞瘤免疫组化•

单核组织细胞样细胞和破骨巨细胞

CD68+，CD163+•

肌纤维母细胞SMA+•

MiTF通常阴性鉴别诊断•

Cellular

neurothekeoma•

慢性肉芽肿Cellular

NeurothekeomaCellular

Neurothekeoma•

组织分化不明，与神经鞘黏液瘤无相关性•

位于真皮内，由上皮样组织细胞样组成，形成多结节状，结节之间为纤维性

间隔，部分病例结节间质可伴有黏液样变性（30%）•

部分病例核可显示有不典型性（25%）•

儿童和青少年•

头颈部和上肢（面部和

肩部）•

良性

(即便镜下显示有不典型性)Subcutaneous

Involvement

Subcutaneous

involvementCellular

neurothekeoma

often

features

nests

of

varying

sizeand

shape

delineated

by

thin

fibrous

bands.

In

many

cases,

thecells

lack

nuclear

pleomorphism.Sheet-Like

GrowthHematoxylin

and

eosin

shows

cellular

neurothekeoma

with

confluentdermal

nests

forming

irregularly

shaped

cellular

aggregatesMyxoid

change

is

present

in

30%

of

cellular

neurothekeomaCellular

Neurothekeoma的免疫组化•

NKI/C3•

CD10•

MiTF•

S100(-)Neurothekeoma的免疫组化Neurothekeoma≠

Nerve

Sheath

MyxomaNerve

Sheath

Myxoma软组织巨细胞瘤giant

cell

tumor

of

soft

tissue与软组织相关的组织细胞病变•

黄色瘤•

朗格汉斯细胞组织细胞增生症•

幼年性黄色肉芽肿•

网状组织细胞瘤•

Rosai-Dorfman病•

结晶储积性组织细胞增生•

类风湿性结节•

树突细胞肿瘤Unlike

the

other

forms

of

xanthoma,

tuberous

(and

tendinous)xanthomas

usually

have

secondary

changes,

includingcollections

of

extracellular

cholesterol

with

cholesterol

cleftformation

and

associated

giant

cellsOther

secondary

changes

in

tuberous

(and

tendinous)xanthomas

include

varying

degrees

of

fibrosis

and

foci

ofnecrosis

containing

amorphous

eosinophilic

debris

withcholesterol

clefts

and

a

surrounding

rim

of

foamy

macrophages

.Tendinous

xanthomas

are

histologically

identical

to

tuberousxanthomas

except

that

they

involve

deeper

structures,

such

astendons

and

ligaments.Plexiform

XanthomaA

multinodular

proliferation

with

a

plexiform

architecture

fillingthe

dermis

is

typical

of

plexiform

xanthoma.

The

deep

nodulestend

to

be

more

well

developed

than

the

superficial

ones.朗格汉斯细胞组织细胞增生症临床表现•

多发生于儿童和青年人•

男性多见，3.5：1•

可为孤立性，单系统多脏器性，或多系统性•

骨，软组织，皮肤，肺，肝，外阴幼年性黄色肉芽肿网状组织细胞瘤Rosai-Dorfman病晶体储积性组织细胞增生Crystal-Storing

Histiocytosis•

淋巴组织增生性或浆细胞性疾病淋巴浆细胞性淋巴瘤，MALToma，结外边缘区淋巴瘤，MGUS，骨髓瘤，浆细胞瘤•

慢性感染性疾病或使用氯苯吩嗪•

结晶：免疫球蛋白深部环状肉芽肿deep

granuloma

annulare同义词•

皮下环状肉芽肿•

假风湿性结节临床表现•

儿童和青年人•

女性多见（2:1）•

皮下无痛性结节•

下肢，面部，头皮，臀部，手类风湿性结节rheumatoid

nodule临床表现•

无痛性皮下结节•

孤立性或多个•

直径mm-5cm•

发生于20-30%的风湿病患者•

肘部

，前臂，手，足，头皮，臀部，肺，心•

类风湿性关节炎或血管炎•

血清：类风湿因子Rheumatoid

nodules

are

palisading

granulomas

centered

in

thesubcutis

but

often

with

involvement

of

the

overlying

dermis.The

distinctive

red

granuloma

appearance

is

due

to

abundantcentral

fibrin

deposition.Histiocytes

form

a

distinct

palisading

rim

around

the

centralzone

of

necrobiosis

and

bright

red

fibrin.Fibrosis

and

mixed

inflammation

are

commonly

seen

adjacentto

rheumatoid

nodules

.

Lymphocytic

aggregates

andprominent

plas