CASE

Miiller-ian

“pt-i1n;irv” “t’rimq” tiliitlg

tu1110rs

01

miitlerkm

C'IIK'I.~C

of thr

~ht ov;trian

0rigin;de either fronl epitheliunl and stronu.”

arise

from

v;cgirialis

t);irri( surfke)

iil;uly

cwibrvonat

ntiilteri:un

“Seumdnry”

ct,irheliulr~,~~hich

Imiica

twl\-ic mrsotllr~liuln, t.ririg

tumors

tiut,I5.

tniillrrian

l'ron~

twu~lhelium

may

rniitlerian

nic31i’r that the IIIc.

testis

01' the

"src~o~~ia~~"

develops

testis

from

1.1.OIII

(in women. the

cells

tumors the

II-0111 the

meso~lirliur~~

coy-

1)~ mcfqdasia.

The first group umt)i-isrs c;ircinoiii;i of ihr appendix testis together wilti qstad~~1~01n;is drisi1ig fi-om wmimts of 1niilleriari epithcliuni wit bin ttic tcstic-uldi tissue itself. ‘l&r sec.ond group cwntains serous. IIILIC incw. 01 c~ntlometrioict cystic tumors of rhe ovarian type. Summing ;mlinarioii.

up

1tir prcsrril

0\2ri;iri

t~b~~,rt tratisilion

of

tkfinitety

primal-v

Furthw

tvpe,

or

diagnosk

1i(.113;11.at~d t);~rat~sti(~ular ;tptwldix I)v

testis,

;hIc.

Lo lhr

mic~rosuot)i(~ pic.(urv

mic rowopic

p;lttrrli

Brief Review of Testicular Common Epi~!helial r’pe

‘1’0 0111‘I~ilo~~trtlge. of d wrom

as

testis.

the

tes-

of the coultl

Llt~WIniid-

;mct the tlit+erent tumors.

of Mdlerian

cdsc is the ninth

rrstwclivcly. Origin,

in rtie literacurc

type, and tht fir51 scrokis tqGll;iry ( ~sladerio~arc,inoma of w&an type cte5~rihcd in ttir tvhlis. ‘I‘able 2 briefly summarizes thrse seven (.d\c’c. Ihe p~ienrs wew between i I and 60 years old: onI\ two

01

ttieni

I)elongecl

IO Ihc ;qe

of 0vari;in

group

uncler

’ “0 _ yrxrs.

and

cddcr

th;in -14 years. Clinical follow-iit) unwed ,I yImI of I IO I1 years withour eviclenc-e of recul-renq 01 tiw13s(;isc’s,. with ltic excrption of one patient who died front 1nc~r;islaric luilg wnwrf: no mrtaslases of thr lestic.utat~ (111nor No inforniation was ;~vail;ihlv in three \\?I t’ torlntl to ;lLlq’w.‘~’ ttw

0~tier.s

c vslic tumor

I are

‘I‘hese tumors

strurrures,

Tumors

orhe

c.;u-vinonu

course,

tww3ponding

our

01 rnuc~inoris

such

dinkrl

of thosc

of’the

tumors.

considered

01. wtr

regard

c‘ys-

with

xid

In our opinion. it c~anlicb( the (urnor belongs 10 the

tumors.

rpitiidymis,

rlllrd 0111with

papillary

malignancy

miitterian

scco~idarv

ex-

mic roscopy,

as a serous

borderline

wherhel-

tliff&n~i:d

pathologic

and electron

iiiro invasive cancer.

deter-n,incd

g~orip of

of- c.cmventional

rlitnor ~nay be classified

1;3dtwom;i, Iw

the rcsul~s

iilirniulotiisto~tieniistr~.

w-t.1 e

DESMOPLASTIC A DIAGNOSTIC I‘IIOMAS

I%. C:KOI

MALIGNANT DILEMMA 11’. \lR.

MK(:Pl,

MESOTHELIOMA THOMAS

V.

COLUY,

MASQUERADING MD, PF.IOX (:. (L\Y. VI).

AS SCLEROSING FCCP,

MEDIASTINITIS:

ANI) KICH.WD.J.

I’Is.\sI,

MD, FCCP

HUMAN PATHOLOGY

Volume 23, No. 1 (January

Malignant InesothelioInas typkilly involve the pleura 0I peritoneum in ;I diffuse nz~nner.‘~~ In~olverncnt of the medi;Istinuni hy diffuse pleural rrresotlieliot~ias does occur. hut usually when the tumor is ;It ai1 ;ldvancetl stage. It is rare foi a malignant mesothrlioma to present as a mediastinal mass without other pleuropulmon;~r~ involvement. We report an cxainple of ;I desnioplastic malignant mesothelioma of the mediastinmii thal was initially diagnosed as sclerosing mediastinitis because of its extensive &smc~plasia arid unusu;Il location.

1992)

MicI-oscopic ex;InIin~ttion of the biopsy samples t&en from the peric_ardiuIn and periaortic tissue showed dense collagenous tissue with sInal foci of tnildly :Ityikal spindle cells interpreted initi;illy ;Is fit~rohlasts (Fig I, left). A few cellular ;Ire;is were found iri which the cells were slightly more atypical ~lci h~l”r’lII.oIII~ItiI. in appear;Ince (Fig I, right). The tnediastinal lymph nodes demc~nstmted IIecrotiLing g_raIIul~~Inas COP taining yeast organisms rrIorphologicatty resembling Histoj/lIrrowing of the left nkn bronchus and the left pulmonary artery W;IS;tccompa~iiecl by extension p~renchyIu;I. A of the pathologic proi’css into the a(+ccnt small left-sided pleural effusion w;Is noted on one occasion but w:I> ;It)sent at subsequent examinations. A barium contrast study of the rsopllagus showed ;I localized extrinsic constriction in the posterior niecliastinunI. Pul~nona~-y arteriograph~ aintl e~lio~arcliog~i~)~~~ were “‘5 months after the onset of’ performed in lkcrniher 1989, _. synipton~s, These clemonstratecl almost cotnplete stenosis of the left pulIIioIIary artery, ;I moclrrate stenosis of the right l~11l111011iIry aI-tery ;it its origin, right centricular hypertrophy, and ;I snIall peric:IrdizIl effusion. A second thoracotomy was performed in March I !)!)O. and ;In :IttenIpt w;1s rnacle to bypass thr right pul~nona~~ artery stenosis using ;I Hmc~~k prosthesis coIIIIected to the right ventricle. Biopsy s;Iniptes were aS;iin taken from the sclerotic. :IrczIs iII the Inedlastinun~ ;Ind revealed ;I siInilar niicroscopic appearkince to the previous biopsy speciinens taken in .!une 1088. I’ostol’er~It Ivel~, the patietIt expcriericecl progressive I-espiratory difficulties :urcl required continued tnech;Inical rcspir:Itory stIpport Trr-Inin~Illy, she developed septic-t-rnia, and Ckrtdida ,g/n/““/n w;Is cultured froni I~lood, trache;Il secrctioiis,

A 1X-ye:ir-old woni;ui w;Ib referred to the M;Iyc) Clinic iti February 1988 for evaluation of 2 I-month history of leftsided chest discomfort, mild clyspnea. ;llIct left \~or;tl cord paralysis. These synlptorr~s were precedecl 2 months earlier by ;I flu-like illness. The patient lived in ~-In-al Iowa and had no history of significant asbestos exposure. Radiographic exaniIIation of the thor;Is showed ;I s~nall infiltrate in thr lateral left upper lobe and a calcilird ~‘_;~nulon~a in the right upper lobe. Histopl;IsnI:I coInpleInent fixation old antibody immunodiffusion tests. sputum culture for flmgi ancl acid-fast bacilli, ;mtl tuberculin skin testing were neg:Itive. A repeat chest r~Icliogr:iph 1 InoIiIh Liter showed almost ~~ornpletc resolution of the lung infiltrate. However, the patient’s symptoms persisted and she w;is rc-evaluated 3 months later. Chest N-l‘;Iyfilms at that tiIIIe showed a11 ill-definecl and irregular left upper lobe opiicity with rccluction of left uppeIlobe vc~tun~e. prominence of niediastinal soft lissues extending down to the lrft hilum. and clev;Ition of the left herniclial,hr;IgIII. No pleur;Il effusion W:IS seen. Flexible fiberoptic t,r-onchoscopy showed hyperemic ~nucos;~ iII the left upper lohe bronchus but no endotII.oIIc.hial lesion. Further investiPItions, including IIiaiiiniogI~~Il~hy :inci coniputeri7ecl toniography of the abdonwn and pelvk. were negative. A thorac.t)tom): ~‘;Is perforrnect in June 1988. ;UIC\ a dense sclerosing process W;ISnoted to involve the proxiinal segments of the pulInonary artery and aorta, the left phrenic and vagus nerves. and the pericarcliutn ;It the base of the he:Irt. Multiple biopsy speciIneIIs were taken fr-oni these sites; nIecliastinal lymph nodes were also san~pletl, ;Ind ;I wedge resection of the le1.t upper lolx wiis perfornied

FIGURE 1. Biopsy samples from the mediastinum showing dense fibrous tissue with small fibroblast-like cells (left) and other more densely cellular areas with slight nuclear atypio (right).

80

CASE STUDIES

FIGURE 2. Caudal view of transverse section of lungs and mediastinum at the level of the aortic arch showing the mediastinal location of the neoplasm with extension into the left lung along bronchovoscular bundles.

27

lllotlttls

;lf‘(rT

the

otrse1

of

smrll

]‘trlttxl-t,;lsrtI

IitGotl

were

]‘lac]llt3 in the

li)tiiitl

hlicrosc.opic tliastirlunl I0

Illat

prcviolist\

gi-XIV

xai-c.otti;i

wc’rc

itlet~Litirtl

xtttl

dcscril~ed

;intl, ttxnsitiott

ittfilttxtetl

w;,s

the

Ivlt

]“~“‘]o~l~itl;ltltly 01

ttic

Iwtiotlic

prrsrtit

in it-cm-stained

kel-atin

01xn1~ ~m2s; hotti

This

ttclrtic

cwoplastilit

Itistcx

hotlie

wt’rt

ivitv

hlai~tl and

in

c-vtts

ttt~ilignati~

1101

\\a~

both

(tit,

bilhitt

ft-

I stains

I .c7-hl

of itlot-ptiologic pa1tei~iis

lttmic-aI

di;tat;tw-tli-

Itrtt~irttiost~iit~iii~

pail

ml’igrit

staitiiiig

fat- tlrstnoptastic~

I~~ttlcltcs u ~tti]~~t~d

.infl

lihwt’.

c~ctolcyic;ill~

coiistcltafiotl

itnt~iiiiiotiisto~t~etiii~~~l

was.

trrc~plasitt

ul;it

;tftc*t

A+cslos

01 luttg

~~it.citic,ettit,t-~~tti~~

negative.

idrtttifietl

ruins.

ttw

hov;t5(

and

tiectxbis

of.

‘I‘trc

tittt(~i~2rttiitt~

wrtions

;it~d

l;t(tt9..

h1onc

01‘ high-

~;itx~otit;itous

ttw

IlIt’-

sitiikir

fiht olic

J~lc~it-~~I J~l~iqiif~s wcw

uitti

htrotlg

cells

mtl

the

1oc.i lttc

1\txm

iii Iltc

was

acid-Scltiff

showed

~;~t~~otit;~lo~~b

VI-

rissuc.

tnucitl

tieoplasttt

lrorlt

;rtltlitioii.

:lt,lXlJ”.

tllr

‘l‘tir

of S;llTolll:ltollS

gt3tett. fi)i.

;tlotlg

J’]tU;lt

lit)rohis

I)ctuwti

sctcrotic~

wptx

itt~t~;~ccllttl:tr

st.iinitig

in

wa:, I)t.cxvtt

lung

,it~tl tlrc ititrrlot~ul;~tNo

ttir

invasion

t.tkett

oL‘(tcth(:

~l-ecpctltlv

in t)otti

IytnJAlatic

tissric.

;I rnislurr

(1:;~ 3). ‘l‘tir ;ll’c:l4

S;II’~,Illl;ltolls

01

~howcd

;I 100-1111.

cwit\.

chcxt

txmination

at arilops,v

g xitlt

;lloll lefr

wert‘

fintliilgs c otisidel

ed

;ittd tli+-

Itt~sc~ttreliotlr;r.

I)IS(:ITSSION .I‘his

cast

n;is

u~tusual

usually

ttt~Sottieliotii~i~

Iittttirtt~ot~i~to~t~~t~ti~;il tiicttiartitiutti, ttctiwcl

have

front ,‘blOltlt’l’

\Yhite

;I

FIGURE 3. Tissue from mediostinum comatous differentiation.

showing

high-grade

sar-

Llitited

;itttl

St trrosirr~

ai

tticsc

COIIIWC tile

;I

do~nitietitetl

rx~~osut-e

ttiKusr

sJxif

hl~iligtiattt tlitfuxly.‘~’

t’

stuck\

01 “lodi7cd

tltxritxd

I)rfvr

in

tht

tutriot

5 art

tii~)t’v

tikrt~

tiwtc

c t4ls txtltrt.

tharl

ttiv

tiiost

Jxittiologi,rl(~ itluli;tStitiilis

where

is foutid

;bd)t.Stos Itir

\ attics this

cxposut’r.

‘I J~IXIL~J~ risk

niesotlrcliottt;~. histon

Stala.

tti;tligtimt

(‘kIW \(;I\, ttlC .lJ’]‘;““tll

01 Signiticxtit

is kndcwhtedly of

ntidwesterti

(liriical

ttial

tiistoty

exposuw

~~f‘;ishestos

I’ei-I~;IJV

ruc~titral tiavc

;lSJX’c‘l 01 t]liS

Jxitierit’s

dcwloptiicnt

with

ot‘tx~botrs.

~~lelli~;it

~dlh.‘~.’

ash~stos

l’oi- the

wilt3

ultrxt

iirclica~cd

illlctWtiIlg

in the

the

whic.11

sut-,tttesotheli:d

in~cothelial

;tl)hcnce

c;ibcs

arid

tttesotlirliotii;is.”

fibi-011s

i‘txm

for ;i tiutiih~i involve

1,1c 10,

pt~oportioti

c~oiisidrtA~l~.

pticnt

livrtl.

in ;iJ)J)t-ositii;~r~‘ll,

01 It1 ttw

3 tiistcq

.‘)o% of’Jxiticilts

itiesottirlioittas.” intrtmtittji rt~suttlkittc~ ia 3 txw

asps

I 01 ttrib

to sclc~rosittg c-cbtttliriott

Ilt;lt

c;lhc

w:b

tht

tiiecliastiriiti~. 1ltcM

0fttYt

HUMAN PATHOLOGY

Volume 23, No. 1 (January

occurs as a late sequela ofinfection with Hcc~psulntum. although the precise etiologic relationship between the two events remains unclear.“,7 The clinical features of this condition result from the excessive proliferation of fibrous tissue that permeates the normal structures of the mediastinum. The fibrosis is frequently organized in a concentric fashion around foci of raseation necrosis. Many of the symptoms that our patient experienced are typical presenting features of sclerosing mediastinitis: cough, dyspnea, hemoptysis, chest pain, vocal cord paralysis, dyspha@a. and weight loss. Obstruction of airways and pulmonary blood vessels, as seen in our patient, is characteristic of endstage mediastinitis. Radiographic features of sclerosing mediastinitis include mediastinal widening with obliteration of the soft tissue planes, and hilar masses. Parenchymal infiltrates and loss of lung volume are less commonly seen. Because the period of time between the infectious episode and onset of symptoms is usually quite long. the results of serologic tests for H cupsulatum are freyuently negative, even in cases such as ours, in which there was unequivocal histologic evidence of previous infection. While many cases of sclerosing mediastinitis resoIve spontaneously, some behave more aggressively and result in progressive impairment of respiratori function. In cases in which there is involvement of major airways and blood vessels, the mortality rate approaches 40%. and death may occur within 3 years after the onset of symptoms. Medical and surgical intervention has been largely unsuccessful in these cases7 Therefore, the clinical course followed hy our patient was similar in many respects to that seen in severe cases of sclerosing mediastinitis. The granulomas seen in this case bore witness to a previous infection consistent with H cup~ulatum. However, we believe these were an incidental finding. The fibrosing process seen in this case was probably unrelated to the previous infection as it had no direct relationship with the granulomas in the mediastinal lymph nodes. The histologic features of this lesion at autopsy were typical of a desmoplastic mesothelioma.” Typically, these tumors are composed of a mixture of dense fibrous tissue containing small numbers of cytologically bland spindle cells. and other more cellular areas with obvious cytologic, malignancy. The boundary between the fibrotic and cellular areas is often abrupt, as was evident in this esample. The differential diagnosis in this case included not only sclerosing mediastinitis but also a number of other neoplasms.

MULTICENTRIC DEVELOPMENT PAPILLARY HYPERPLASIA

OF PANCREATIC

1992)

The strong keratin positivity excluded the possibility of a mediastinal sarcoma, also a rare lesion. The negative staining foi mucins, carcinoemblyonic antigen, and Leu-M 1 argued against a diagnosis of a spindle cell carcinoma. which cm sometimes be extremely difficult to differentiate from a Sal-comatous mesothelioma.” As previously indicated, localized fibrous mrsotheliomas, which sometimes involve the mediastinum and may behave aggressively, lack inin~unotiistoctiemical evidence of epithelial differentiation.“’ This case illustrates the diagnostic diticulty that can he caused by a desmopfastic mesothefioma with an atypical presentation. The lesion, both clinically and pathologlcallv. was thought to represent sclerosing mediastinitis, even after’s sec.ond thoracotomy. The positive reaction of the bland fibroblastlike cells for keratin antihodies suggests that the correct diagnosis could have been rendered at the time of the first biops! if a desmoplastic malignant mesothelioma had been considered in the differential diagnosis. However. many benign I-eacti\e pleural lesions have been shown to stain positively with ker-atin,“’ and the itnmunohistocher~~ic~~l profile in sclerosing IIW diastinitis has not, to our knowledge, been specifically esamined.

INTRADUCTAL

CARCINOMA

THROUGH

ATYPICAL

TAKESHI OBAIW, MD, YLISLIKESAITOH, MD, HIRCIY~IKIMAGLCHI, MD, HITOSHI URA, MD, SHLIN,JIKIT.~L.~w~~,MD, Y~_qrKOIKE, MD, KIYCISHI OKAML~RA, MD, ANI) MASAYOSHI NAMIKI, MD

f[~endoscopic retr(lgmde pnnrrmtogr~phy. which demorxtrutPd multi,ble dilated bmnrhes of thlr pnucreatic duct in the body atld tail of thr pancreas. Histologxc examinution on the resected pancreas .rhowed diffuse atypical papikry hyperplasin in multiple dilated ducts a.c.cociated with multiple intraductal carcinomas. Histo@ic jeotures NIQ dpscrihrd and multicentric carrinogenesis through atypical pnpillary hyperplasia is di.rcussed. HUM P.47.1101. 23:82-85. Copyright 0 1992 by W.R. &under’s Company

Fro111the Third Department of Internal Medicine. Asahikawa Medical (College. Asahikawa. Hokluido, Japan. Accepted for puhlitation April 15, 1991. Kq uwr~Lr: pancreatic canret-. intraductal carcinoma. atypical papillary hyperplasia, papillary hyperplasia. duct hyperplasia. Address cot-respondenw and reprint requests to Takeshi Obar-a. MD. Third Department of Intertlal Medicine. Asahikawa Medical College. J-.5 Nishikagura. Asahikawa. Hokkaido 078. Japan. Copyright Q 1092 bv W.R. Saunders Company 0046-8 I 77/92/2301-00 17$.5.00/O

Although recent advances in and widespread use of diagnostic modalities are remarkable, carcinoma of the pancreas is generally recognized at an advanced stage and its prognosis is poor.’ EfTorts havp been made to detect early cases and to

82