Document 20812

25(
INTERNATIONAL ACADEMY OF PATHOLOGY
-South African Division Short Course No.ll.
DISEASES OF KIDNEY BLADDER AND 'PROSTATE
Dr F Kash Mostofi
Cape Town, S. Africa
July 17, 1976
INTERNATIONAL ACADEMY OF PATHOLOGY
S OUTH AFRICAN DIVI SIO N
INTERNASIONALE AKAOEMIE VIR PATOLOGIE
SUI D -AFRIKAANSE A FDELI NG
SHORT OOIJRSE ID . 11 .
DISEASES OF KIDNEY, :Si.A.DDER AND PRO STATE.
l~oderator :
Dr. F. Xash h!ostofi ,
Armed Forces In stitute of Pathology,
Washington D. C.
17th J uly , 1976.
CASE !'ll. 1 :
(1431135)
50 ye ar old male witli symptoms of prostatism .
showed stony hard prostate .
CASE llO . 2 :
Rectal e xaminat ion
(1502469)
75 year old male with soft enlarged prostate .
CASE NO. 3:
(1501163)
67 year old male pr esente d >lith symptoms of obstruction.
clinical impression was benign prostatic hyperplasia,_
CASE liD . 4 :
The
( 1562257)
90 year old male with urinary retention ; clinically e nlarged
nodular prostate .
CASE liD . 5 :
( 1494471 )
72 year old female with bladder tumour.
CASE liD . 6 :
(155_4562)
46 year old female with two weeks history of haematuria; cys toscopy
revealed papillary lesion in done of bladder.
CASE NO , 7 :
( 1562629)
40 year old male with bladder tumour .
CASE NO . 8 :
( 1531813 )
37 year old male with longstanding history of renal s tones .
CASE llO. 9 :
( 1556386)
18 year old female who developed flank pain and haematuria during
pregnancy.
CASE llO. 10:
(Supplied by Dr. R. Kaschula)
An 11 month old infant weighing 7. 8 kilograms presented with progressive abdominal distensi on developing over s ome months . A large tumour
arising within the r ight renal capsule and together with t he kidney we ighing
1. 4 kilograms was resected but residual tumour was left adherent to the
inferior vena cava.
•
Dl'mRllA TIONAL
- South
SHORT COURSE NO , 11
Moderat or :
ACADEMY OF
African
PA'll!OLOGY.
Division -
Diseases of Kidney , Bladder and Prostate .
Dr. F. Kaah ldoatofi ,
Armed Forces Institute of Pathology,
SU.B!IIIT'J.ED
l~ashington
D. C.
DIAGNOSES:
1.
.... ' .................... ................................................ .
2.
......................... ... ........................ ...... ................
~
..................... .. ..... .. ... ....................... .......... ........
...................... .. . ................. ........ .......................
...... .......... .. . .. .. .. . . . . . . .. .................. .. .. ...................
6.
...................................................................... .. .
8.
........... ........ .................................. .............. ..........
.........-· .............................................................. .
9.
. ......... ......................... ...................................• "• .
10.
. ............. .......................................................... .
1.
N,B, Each participant should complete and return this sheet anonymously to
the Secretary (Dr. R. Kaschula.) at Red Cross. Chil(lren• s Hospi tal ,
~
Ronde bos ch "('lOO to roach him rrot later than 2.00 p . m. en ~!onday 12th
Jul y .
IN'IERNATIONAL
- South
African
SHORT
KIDNEY,
LIST
ACADEMY
OOURSE
BLADDER
OF
OF
PATHOLOGY
Division -
NO .
AND
11
PROSTATE.
DIAGNOSES
and
DISCUSSIOJi OF CASES
DR . F . K. MOS'IOFI
Armed Forces Institute of Pathology,
Washin gton , D. C.
Ca pe Town
17th
Jul y,
1976.
LIST OF
DIAGNOSES.
Page No .
1.
GRANUI.OMA'fOUS PROSTATITIS
,
2.
!IIALIGNANT LYidPHOMA OF THE PROSTATE
2
BASAL CELL HYPERPLASI A (FETALIZATION) OF 'ffiE
PROSTATE
3
INVER'IED PAPILI.OIU
4
BLADDER, CARCI!m!IIA TRANSITIONAL - AND SPINDLE
CELL, GRADE II , PAPILLARY AND INFILTRATING.
5
6.
NEPHROGENIC ADENOI11A OF URINARY BLADDER.
6
7-
BLADDER - PARAGA.NGLIOI•IA.
7
8.
XAlf'IHOGRAliUUJ~IA'TOUS
8
4·
PYEI.ONEPRRITIS.
AlfGIOI4YOLIPOIU Win! PREIXliUNE!lT LEIOMYOMA'fOUS
ElEMENTS.
10.
MESOBLASTIC NEPHROMA - LEIOMYOMA'I'OUS HAIW!'IDMA
9
10
GRANULOMATOUS
CASE NO . 1 :
PRO STATITIS .
Contribute d by Memorial Hospita l ,
Orlando , Florida.
(1431135)
50
year old male with symptoms of prostatism.
hard prostate .
Rectal examination
sho~~ed
stony
1he archi tecture is markedly a ltered by many cellular are as which have a nodular
appe arance . The nodules are fairl y we ll de fined but t hey are soJOO t i me s confluent ,
Al most invariabl y e i t her the remnants of a duct or de gene r ating corpora anyl acia
are seen some>Ihere in the nodule but usually in the cent e r . '!here is i nfiltrat ion with lymphocytes and plasma cells and macrophages .
l4a.ny of these cells have vacuolated cytoplasm but the cells occur in sheets and
tbere is no acinar formation. Occasional polymorphonuclear l e ukocytes are seen
and soJOOtiJOOs t he se occur in duct al lumina and at other times they form sma ll
abscesses.
Residual e lement s of a cinar e pit helium are see n and s o100 of t he se are s omewhat
exuberant .
DIAGNOSIS :
Granu l omatous prostatiti s
'lbe first quest i on is whether we are dealing with an inflammatory or neoplas t i c
process. Many of the·se are sent in because ( 1) clinically the prostate is stony
hard , leading the urological surgeon to feel that he is dea ling with a carcinoma
and (2) the pathologist , seeing the clear cells, is misle ad to diagnosis c arcinoma or seeing the pleomorphic cell population, suspects a Hodgkins .
This case repre se nts one pattern of r eaction. In other i nstances sheet s of
cl e ar ce lla are see n Nith small ve s icular nucle i. In s t i ll othe rs the cells
are large Nit h granular eosino phi li c cytoplasm. Occas ional e ntrapped a c i ni may
be seen which may be s mall . Several f eat ures are helpful i n t he correct diag-nosis. There i s usually an admixture of cells, rarely , i f e ver , true acinar
formation , invar i able relationship of the lesion to a duct ;lith partial or
complete loss of epit~lium. Often there are fraeroonts of degenerating corpora
amylacea and multinucleated giant cells.
It should be emphasized that granulomatous prostatitis is not malignant , it s
presence does not necessarily r ule out a carcinoma. 1\'e have seen seve r al
i netancea of c arc i noma - occasionally intermingled with granul omatous prost atitis and s ome t i me s indepe nde nt ly of i t ,
REFERENCES.
4:
1.
Malatinsky, E. et al .
179-81 1 1972,
2.
Mostofi 1 F. K. Tumors of the Male Genital System (Fascic l e 8) Atlas of
Tumor Pathology, 2nd Series , AFIP, Hashington , D. C. (Wi th E . B. Pri ce , Jr . )
1973.
Granulomatous prostatitis.
Int. Urol .
l~ phrol .
Schmi dt , K. Proceedings : Morphology of histiocytic granul omatous
prostat i tis . Verh. Dtsch. Qe s . Pat hol. 57 : 464 , 1973.
'lhybo 1 E. 1 e t al .
111-4 , 1973.
Granulomatous pr ostatitis.
- 1 -
Scand. J , Urol . Naphrol . 7 :
MALIGNANT LY111PHOMA OF THE PROSTATE.
CA·SE liD. 2:
75
"Yf'B:r
(1 502469)
old male
1~i th
Contributed by Dr.W.~I. Christman,
Sunbury Community Hospital
Sunbury, Pennsylvania
soft ·enlarged prostate,
There is .massive infiltr ation of both prostate and the bladder neck ~rith
uniform small cells. The nuclei are quite irregular ranging from round
to truncated. They are dark staining and except "for occasional vacuoles,
are nondescript. Al tho a fe•,r ce lls have vacuolated cytoplasm, for the
most part the nuclei are almost naked. There is no distinct pattern but
occasionally they occur in clumps separated f rom each other by less
.cellular areas . 'llie cells are .diffuse ly infiltrating the prostate and
bladder neck with compression and necrosis of muscle bundl:es. 'Ihere is
infil tration of vessel walls ,
DIAGNOSIS:
Nalignant lymphoma of the prostate
The differential diagnoses are be}~reen l ymphoma , severe chronic prostatitis
and undifferentiated small ce 11 carcinoma of the prostate,
In chronic prostatitfs there is almost invariab l y involvement of ducts ~rith
either l oss of integrity oi' the duct or squamous metaplasia, the infiltrate
is rare ·l y as massive but if so there may be follicle formation. There is
no compression and degeneration of muscle bundl es and infiltration of vesse l
~~alls .
In undifferentiated carci noma of prostate t he cells usually have some cytoplasm, the nucle i are a l so de nse but t he cells occur in columns, rows a!'ld
almost invariably there is some gla~4ular differentiation.
Patient had had a liver biopsy •~hich had been diagnosed as lymphoma;
h01rever, ~rhen TUR ~ras done on him for relief of obst ruction, the qUestion
of an undi ffe·r e nt i ate d ca:rcinoma of prostate ~ras raise d.
1•
Cartagena, R., et al.
gland. Urology 5 (6)
Preliminary re ticulum cel l -sarcoma of pro state
815-6, 1975·
- 2 -
BASAL CELL HYPERPLASIA (FETALIZATION) OF '!HE PROST~
CASE 110 , 3 :
Contributed by Dr. L. P. Rosier
lee Memorial Hospital
Fort Myers , Florida.
( 1501163)
67 year old male presented with symptoms of obstruction.
impression was benign prostat ic hyperplasia.
'!be clinical
In addition to glandular hype rplasias of var ious types, typical atypical ,
oocondary a.nd cysti c, there are one large and 2 small nodules that are
quite cellular. Instead of the usual double layer of cells , here a large
number of acini <J.re seen lined by pile d u p ce lls at ti mes resembling basal
oells and at othe r ti me s transitional epithelial cells , each cell appearing
to rest on the basal layer . Occasionally the basal cells and the usual
acinar are seim side by side . All stages are present from essentially
normal acini to acini that are complete ly replaced by this epi the l ium. The
oells are fairly unif orm, no nucleol i, nuclear vacuolization or giant cells
are seen. At times there is a suggestion of squamous nrataplasia. 'lhe
glands range in size i'rom small to large 1 oftti~res 1~ith ramifications . 'lhey
are close ly packed but t he stroma i.s quite cellular ,
DIAGll>SIS :
Basal ce ll h vperplasia ( fetali zation) of the oro state .
The di fforential diagnosis is between metaplasi a , transitional cell carci noma
or a carci noma der ive d from basal ce lls . Transitional or squamous cell ~mta­
plas i a is a common finding i n prostates most often as a result of infarction,
less i'requently secondary to inflammatory infiltration. Metaplasia secondary
to infarction is usually seen adjace nt to an area of infarction or fibrosis
i n a hyperplastic prostate . Metaplas ia secondary to inflammation is usually
associated ;lith a prostatic duct and there is inflammatory cell infiltration
which involves the acini secondarily. Neither situation pre vail s here .
Transitional cell carcinoma of the pro state is usually secondary to a tumor
in urethra or bladder oock. Primary transitional cell carcinomas of the
prostate almost invariably arise from ducts and the oells usually show considerable anaplasia . Jfo anaplasia is seen in tho ce ll(! a.nd t he involvement
see ms to be principally that of acini and not t he ducts. Secondarily, the
oells do not sho•t any evidence of mal ignancy.
we had considerabl e discussion about whether thi s waa benign or malignant .
Several of the WHO consul ta.'lts revie;ted the caee and a ll agreed it >tas
benign. The pat i ent io living and well 2 ye ars late r . The case resembles
the picture described by Obendorfer in 1931 and more recently used in our
depart100 nt as fetalization of the prostate , '!be lesion i s believed to be
benign.
- 3-
INVERTED
CASE NO . 4:
PAPILLOMA.
(1562257)
Contributed by Dr. Nichols
VJlli , Port land , Oregon.
90
year old male tdth urinary retention ; clinically e':llarged nodular
prostate ·.
'l'he se ction showG a very cellular mass covered in areas by transi tiona!
e pithelium of variable thick.'1ess . llo•m>rard proliferat i on of epi the liwn
is frequently seen having the a ppearance of von Brunn ' s r.ests . The mass
itself cons ists of anas·~omo.sing cor ds and fronds, many of >rhich seem to be
prolongations of ~~perficia.l von Brunn's nests. They are supported by
delicate fibr ovascular stroma . The cords are fai rly uniform and consist
of multi-layered e l ongate d ce ll s arranged per pendicularly to the suppor ting
stroma . I n the center of the cords t he cells resemble t ra."'!si tional e pi thelium ~rhile at the peri pher y they have the appearance of basal cells. No
ce llular anaplasia is seen and only rare mitotic activity . Ther e is ;~horled
patter n i n the " center" of many of t he cords suggesting squamous meta plasia.
In others there i s a small lumen.
DIAGIIDSIS:
I nverted pavi llorna.
The case ~~as se l e cted because many of these are sent i n as infiltrating
transitional ce l l carcinoma, grade II. Thi s is undoubtedly due to massive
cellulari ty and mi s i ·nterpretat ion of the i rt;Tard gro,,:th of papi llary f ronds
t~ich simul ate infiltrating carcinoma.
They Q-"9 distinguishable f r om infiltrating carcinoma by the 1"act that in the lat ter the cells are definit e l y
anaplastic and t he advanci ng bor der consisting either of broad front or
tentacular gr01~th is irregular , and the ce 11 clust e r s are of varying shapes
and size.s. In many i ns t anees there is so~re inflammatory ce 11 infilt r ati on.
'l'he !,>TOss appearance is also· different from carcinoma in that it consists
of a. peduncul ated or s ome tines sessile mass >ri t h smooth surface . The l esion
resemb l e s inve rted papi lloma of nasopharynx. Almost all r epor ted cases have
had a benign course 1 ho~rever , ~~ have seen instances i n Nhich t here is a ssociated papilloma and in sever a l cases ce llul ar anapl as i a . ~le hav-e therefore
at time s made the diagnosis of inverted pa pi lloma .rith mali gnant change .
REF'i'.:RElWES.
1.
Cummings , R. J .
225- 7'
Inve rted papilloma of the b l adder .
Pa t hol. 112:
1974·
2.
IeMeester , L.J. et a l . L"lverted papi llomas of the urinary bladder.
Cancer 36(2) 505- 13, 1975·
3,
Potts , I . F. and Hirst E . Inverte-d papilloma of the bladder .
Urology , 90 (2), 175-9 1 1963.
- 4-
J . of
BLADDER 1 CARCI:N'O!I!A
TRANSITIOI~AL
- AND SPINDLE CELL,
GRADE II, PAPILLARY Al'ffi INFILTRATI NG.
CASE liD.
72
~ar
5:
(1494471)
Contributed by Dr. Wa lland Hause
St. Mary's Hospital 1 l)jcatur 1
Illinois.
old female >lit h bladder tumor.
Soil& fragnents consist of definite papi llary and infiltrating transitional
cell carcinoma. Se:vc:tal fragnents, ho>rever 1 ·p resent a rather edematous stroma
.supporting sheets and groups of spindle oells >Ihi ch ·are somtimes interlacing.
One or t•10 small islands of d istinctly epithelial oells are seen a.nd in areas
t ransitions bet•,;een epithelial and spindle cells is quite apparent.
DIAGNOSIS:
Bl adder, carcinoma transitional -and s pi ndl e cell, grade II,
papillary and infiltrating.
'!he differential diagnosis here is between a carcinoma with spindle areas,
carcinoma 1~i th desmoplastic reaction of the stroma and carcinosarcoma. In
carcinosarcoma of b l adder we have insisted that the sarcomatous elements
consist of identifiable soft tissue · tumor, e.g. l eiomyosarcoma, rhabdomyosarcoma, osteochondrosarcoma and that thes.e e l ements be distinctly neoplasti c ,
This woul d be a collision twnor . No definite; sarcomatous eleJOOnts are seen
i n this instance .
In carcinoma 1~ith desmoplastic reaction the carcinoma is usually mor e
anaplastic, t he 2 components are intimately associated >Iith each but there
is a distinct line of demarcation between the two . In most instances the
desmoplastic reaction falls short of a definite malignant transformation;
hoNever, occasionally it i ·s definitely sarcoma similar to the picture seen
in uterus >~here both endometrial glands and stroma are mal ignant.
REFERENCES.
1.
Brinton, J . A., Ito, Y. , and Olsen B. S. Carcinosarcoma of the urinary
bladder. A case report and review of the lit e r ature . Cancer 25:
1183-6, 1970.
2.
Constance 1 T.J.
Localized myositis ossificans.
J. Pathol. 68: 381-5 1
1954·
),
Crane 1 A. R. and Tremblay, R. G. Primar;v- ost eogenic sarcoma of the
b l adder . Complete review of sarcomata of the bladder. Ann. Surg. 118:
887- 908, 1943·
- 5-
NEPHROGElTIG ADENDJI!A OF URTIWiY BLADDER.
CASE NO , 6:
Contributed by Dr. D.H. Dain,
St. Joseph's Hospital
Albuquerque 1 1'21~ 14exico.
{1554562)
A 46 yea:r old f emale with a. history of hematuria of tt~o treeks duration,
Cystoscopy sho~red a papi llary lesion of dome of bladder.
The tumor presents an unusual papi llary appearance in ~rhich the epi the li!l-1
cells seem to be forming large and small t ubul ar structures and acinar spaces.
'ftie cells vary from flattened to high cuboidal. Many of the cells have a hob
nail appearance. The cytopl asm varies from scanty t o abundant 'a nd from
amphophylic to vacuo l ate d . fuer a is some cellular anapl asia but no definite
evidence. of malignancy i s seen.
DIAGNOSI S:
Ne phrogenic adenoma of urinary b l adder .
The case ~ras selected for several reasons . It il lustrat es an adenoma i n
Hhich the cells form structure s suggesting renal tubul es. These are seen
mostly i n the trigone area. and have been designated as nephrogenic adenoma
because thi s is the area of the bladder ~rhich is mesonephri c in ori gin . The
present case 1 ho~rever 1 ,,,as not from t r i gone. 'l.he l ocation in the dome r aises
the possibilit y of a 'urachal ca:rcinoma.; ho,;ever, the tumor is entirely mucosal
and not intramural and the hi s t ology is not that of urachal carcinoma which is
us ually mucinous adenocarci noma, altho t ransi tional cell carci nomas have a l so
been seen,
In many of our patients Nith this lesion there is a history of trauma to the
bladder 4 - 6 years pr i or t o t he development of the lesion, The behavi or of
these tumors is of interest . They tend to recur. We have seen a number of
t hem progress to adenocarcinoma, mesonephric type. The di stinct i on bettreen
neplirogeni c adenoma and mesonephric adenocarcinoma is at times difficul t,
The most re liab le criterion is definite deep invasi on of the l<all . Some of
these ~rill shot~ areas of transitional and mucinous adenocarcinoma.
Our concept of the genesis of this lesion i s t hat it is a m~festation of
t he potent iality of vesi cal epi thelium ~1hich can becoJre squamous , glandular
or tubular. So altho 1;e do make a di agnosis of nephrogeilic adenoma, we alert
the urological surgeon to the malignant potential of the tumor .
REFERENCES.
1.
Friedman, li , B. and Kuhlenbeck, H. Adenomatoid t umors of the bladder
reproducing renal structures. (Ni!phrogenic adenoma) J, Urol, 64:
657- 670, 1950.
2.
Kaany, E. et al.
l~phrogenic adenoma of bladder,
Urology 4 (3)
343-5 t 1974·
3.
K.a.lloor, G.F. et al. Nephrogenic adenoma of the bladder.
Urol. 46: 91-5 1 1974.
- 6 -
Brit . J.
·'
BLADDER - PARAGANGLIO!o!A .
CASE .!«) . 7 :
40
(1562629)
Contributed by tlright- Patterson
AFB, Ohio
year old male ,,,i t h bladder tumor .
'!he surface epithelium sho~rs s oiD?l prol iferative change but no anaplas i a .
'!he wall of the bladder is heavily i nfiltr ated by a tumor which i s rather
cellular. !!he cells are large pol yhedral with abundant granular or vacuolated cytoplasm surrounding round or oval mostly vesicular nucle i l<ith
delicate peripheral distribution of chromatin. The cells vary considerably
in size , shape and staining. Occasional mitoses are found . A second ce 11
type is seen either under the surface epithel ium or in relationship to
vascular spaces. 'Jt>.ese are small , densely staining nuclei with little or
no cyto.plasm. Vie~red by themselves these cells s-uggest neuroblastoma or
an undifferentiated carcinoma. The characteristic feat ure of the tumor is
the r ich endocrine vascular pattern. Occasionally large sinusoidal spaces
are pre sent •
DIAGifOSIS :
Bladder - Paraganglioma.
'!he differentiated dia.gnos1a is bet1.een a carcinoma and paraga118lioma. In
paraganglioma the cystoscopic appearance is almost i nvariably that of a
sessile tumor vrith intact normal appearing overlying mucosa. Microscopically the gro1·rth pattern is the most important distinguishing feature of this
tumor from carcinoma. Helpful f indings are absence of any surface involveID?lnt and the pre sence of neuroblastoma like areas .
The se tumors occurs in all ages (14 t o 78 years} . The maj orit y have painless
hematuria. Hypertension is frequent espe cially during mictur ition. About
2o% recur and about 5% shovr metastasis. The diagnosis of malignancy is
oftentimes difficult , The follOI<ing criteria. are he lpful : vascular and lymphatic invasion , local spread deep in bladder wall or to adjacent organs with
destruction of muscle la.yer. !!he tumor may recur several years later.
REFERENCES .
1.
Brown, l~.J . et a l. Ultrast ructural and bioche mical correlates of
adre nal and e xt ra adrenal pheochromocytoma . Cancer 28: 744-759 , 1972
2.
Qle nner , G. O. and Grimley , P.l•! . Twnora of the ext ra adrenal paraganglion
system including chemorece ptor s . Atlas of ~Umor Pathology. Second Series,
Fascicle 9, \olashington, D. C. AFIP, 1973.
3.
Leestma , J . E. and Price , E. B., Jr .
Cancer , 28: 1063- 1073 , 1971 .
4·
B9nchekroun , A. et al . Pheochromocytoma of the bladder.
recent case. J . Urol . Nephrol . (Pari s ) 80112 Pts .: 551 ,
Par38Mglioma of urinary bladder.
Apropos of a
1974.
Fuse lier , H. A., J r . Paraganglioma of the bladder: report of a. case,
J , Urol. 113 (1) 42- 4 , 1975·
- 7-
XAN'IHOGRANUl.Ol<IA'lOUS PYEtDliEPHRITIS.
CASE NO. 8:
(15318H)
Contributed by Dr . R. l,l. Costello
Hospital Association,
Greensburg, Pennsylvania.
''le stmore land
37 year old male with longstanding history of renal s tones .
~/e have l ittle i nformation except that there
yell01·1ish necrot ic tiasue .
>~as
a atone surrounded by a
Microscopically, "there are sheets of uniform cells with foam,y or eosinophilic
cytoplasm surroundi ng a small vesicular nucleus. Scattered throughout and
often f ormi ng absce sses are collections of polymorphonuclear leuco cytes.
'nlere is a pse udo capsu l e and the kidney eho1~s evidence of chronic pyelonephri1:is.
DIAGNOSIS:
Xanthogranulomatous pye lone phritis .
The pr e sence of foam ce lls on these cases often leads to misdiagno sis of
carcinoma but bot h the gross appearance and t he h ist ology are characteristic;
grossly, the lesion resembles a corpus lutoum. Microscopically, tr.e pale or
granular cells , some of which may .be derived from epithelial cells, occur in
sheets without any evide nce of tubular arrange lll3 nt . 'mere is litt le or no
st roma. The lesion progre sses to fibrosis .
Occasi onally, it is extreoe l y difficult to di fferentiate be t Nssn a xanthogranuloma and a carcinoma. 'lbe gross a ppearance Nith a wavy yellowish borde r
and central cavity iE: most helpful. Microscopically, absence of a fibrevascular stroma supporting t he foam cells in a distinct pattern as seen in
renal ce ll carci noma is charac·teristic for xanthogranul omatous pye lonephritis.
A wor d of caution is in or der in t hat rarely c arcinoma and xanthogranulomatous
pyelonephritis may be found in the same k i dney.
As far a s dist inction bet•~en xanthogranulomatous pyelonephritis and malakoplakia is concerne d , the cell a ma.y have more granular cytoplasm in malako plakia
but it i s the identification of Michae li e Outman bodies >rhich makes t he diagnosis
of mala.l coplakia .
In the kidney , xanthogranulomatous pyelonephritis and mala.lcoplakia are usually
associated >lith stones , infection •rith B proteus or e:. coli and t here is
usually some obstruction. The lesion has been repor ted in patients from 1 month
to 17 years, but they are mostly i n t he 4th - 6th decade .
REFERENCES.
1.
Hans, J . P., Backer , E. de , Gratton- Deaart , T. and Maldague - Pham, H. Q,
Xanthogranulomatous p~~lonephrit is in pyonephrosis . Acta Urol Belg 40:
753- 763 , 197 2.
2.
Merning J . H. et al . Xanthogranulomatous pyelonephritis - Unusual
clinical prese ntations . Urology 1: 338-42, 1973·
3.
Pa l ub i nskas, A. J ,
4•
Povysil , C and Konickova, L. Experimental xanthogranulomatous pyelonephritis . Invest Ura l 9: 313- 18, 1972.
5·
Scot t , C. B. and Quigley, P. J . Xanthogranulomatous p;ye l onephri ti s . A
comparison of the di sease in the cat and man Ni th special reference to
the origin of fat . J . Clin Path 25 : 397-400, 1972.
Xanthogranul omatouo pye l onephrit i s .
Semin Roentgenol .
6: 331-7 , 1971 .
- 8 -
AJ.lGIOlWLIPO~fA I~ITH
CASE HO . 9 :
PREOOJ.lD!E}fT LEIOJ.ffili!IA'IOUS ELEMENTS.
Contributed by Dr. J.H. NOrton , Jr . ,
Monmouth loledical Center 1
Long Branch 1 }levi Jersey.
(1556386)
18 year old female v1ho deve lo ped flank. pain and hematuria during pregnancy.
Grossly , the kidney ~-.~ighed 1080 grams and measured 14 x 15 x 10 em . 'lhe
tumor occupied the upper pole of the kidney and it measured 15 x 10 em in
size . '!be tumor was gray white and whorled in appearance, firm in consistency and it had cystic areas lined by thin blue membrane . 'lhere was a large
blood clot and hemorrh~ over the pelvis.
Histologically, the t umor consists almost entirely of crisscrossing bands of
smooth musc l e s . '! 'here is some variation in size 1 shape and staining of cells
and nuclei . Occasional large nuclei and mitoses were seen. Scattered throughout are nests of ma.ture fat cells and a fev1 large "-essels, some 11ith very cellular walls; others with hyaliniza.tion of the wall. In association with or:-e area
of hemorrh~ the twnor is very oe llular , but here a{;a.in both vascular and fat
cell elements are present.
DIAGilOSIS:
Angio.!!IY.oliporroa
wij;,!I_.P:£~domi~E.tJ.e iom;yo.!!!..a.j;ous
elemenj;..!!•
'!he case was se lecte d because such cases are often sent in viith the diagnosis
of leiomyosarcoma or liposarcoma.
'!he problems in the diagnosis of these lesions relate chiefly to the frequent
atypicality of the smooth Jl!Uscle oells or the fat cells. 'lllese not infrequently
are pleomorphic and hyperchromatic and occasionally shOI< some mitotic activity
as well as giant mononuclear and multinucleated for ms . Thus, the tumor not
infrequent ly is interpreted as sarcoma. l~cro sis is a frequent findi ng and
acute hemorrhage is a common feature leading to nephre ctomy. Also , the lesions
are often multiple and bilateral 1 giving the impx·ession of metastasis . ~/e have
seen a number of these with identical lesions in regional lymph nodes . However,
n'B are una1,Iare of a ..-all documanted example of malignant behavior with convincing metastasis, and most observers feel such cases represent multicentric
lesions . Patients Hith the tuberous scler osis complex usually have histologically ident ical lesions , but these are l ess likely to be symptomatic, i n spite of
the great er likelihood of the ir having mul tiple 1 bilateral les ions . There is
some evidence that renal angiomyolipoma per se may represent a forme fruste
of tuberous sclerosis.
'lhe a.ngiom,yolipoma or renal hamartoma predominates in females by over 2: 1 and
the average age is 40-42 years . If the lesion is untre ated and acute hemorrhage does not neoeasitate surgery, the course is protracted with patients
living as long as 18 years before the tumor eventual l y complete ly erodes or
de.stro:ys re nal function.
REFERENCES .
1.
Bernie, J . E.
Renal angiomyolipoma. in an adolescent : A case report .
J . Urol . 109 : 492- 94, 1973.
2.
Drablos , P. A. et al .
Renal a.ngiomyolipoma..
J . Oslo Hospital 25 (9}
149- 54, 1975 ·
3•
.Farro>< , G. M. , Harr i son , E. O. , Utz, D. c., and Jones , D. R.
angiomyol ipoma.
A cl1nicopathological study of 32 cases.
Renal
Cancer 22:
564- 5701 1968.
4.
Price, E . B. Jr . and Mostofi 1 F. K.
kidney. Ca.noer 18 : 761- 74, 1965.
Symptomatic a."'giom,yolipoma of the
Tal ib, v. H. et a l. Bilateral renal angiomyolipoma. Report of a case
t<ith review of the literature . Indi.a n J . Cance r 11(1) 69-76 , 1974•
- 9 -
·'
!liE:SOBLASTIC NEPHRO!I1A - LEIOirnlMA'IOUS HJUiiA.R'IOI,IA.
CASE llO . 10:
Contributed by llr. R. Kaschula,
Red Cross l~ar J1le morial Children ' s
Hospital, Cape Town, $outh Africa.
11 month old African male infant ,,>eighing 7 . 8 kilograms, having had progressive enlargement of abdoroon over a period of some months . The kidney and
the tumor >1eighed 1.412 Kg. Multiple cysts ~rere seen on cut surface .
'!he slide shows an infant ile kidney in which two distinct l e s ions are seen;
one of these consists of interlacing bands of spindle- shaped cells which
are uniform. Il!itoses are infrequent . In areas ·the tissue is edematous in one a.rea it is hyalinized. Scattered throughout are remnants of primitive
renal t:uoules and occasional glomeruli , These areas have the appearance of
renal dysgenesis . A number of thick-1·1alled vessels are present. The lesion
invo lve s peripelvic f at and sh01~s interdigitation with renal parenchyma.
Some of these are associated l·lith fetal nephrons.
Adjacent to one of these interdigitat ions is a very cellular l esion. This,
too , consists of inteTlacing bands of spindle-shaped ce lls. The cells are
quite uniform but mitotic figures are numerous . There are no residual
nephrons here . Scattered througll.out are a number of thrombosed blood vessels
occasionally with necrosis of the· wall . Several foci of hematopoiesis and
some areas of hemorrhage are also see-n . Al t ho in one area the t1~o lesions
seem to blend, for the most part the cellular area is distinct l y outlined.
1liAGliDSIS:
!l'~esoblastic
neohroma - Laiomyomatous hamartoma.
In addition to the above terms the lesion has been de scribed as fetal mesenchymal hamartoma, l eiomyoma, and fetal hamartoma. Although the lesion has
been kno1m for over 30 years it· is not infrequently confused with Nilm1 s
tumor . Some years ago 1oJe repor ted on 20 cases - 17 of ~1hi ch had been sa nt
t o us as llilms' . Sixteen of these ,,>ere surgical specimens and t~1o of the
patients had died as a result of treatment . These tumors have a chru·acteristic gross appearance : t hey appear to be circumscr ibe d , grayish white to
yel101~ish and firm.
The cut ,. surface has a 1·1horled appearance. Areas of
necrosis and hemorrhage are absent. The tumors usually asswne a large s ize .
Microscopically, t oo, the picture is characteristi c: i nt erlacing bands of
spindle-shaped cells 1~ith little or no cellular anaplasi a and with varying
degrees of mitotic activity. There are usually islands of normal or dysgenetic nephrone. Scatt.e red .c~ts and islands of cartilage and foci of hematopoi-esis are f requent. Almost invariably the tumors originate from the
medullary portions and may involve the k idney segrrentally.
The genesis of the lesion is not established. It may represent a variant
of segmental d.)'Sge:nesis , it may represent a hamartoma, i t may represent a
benign neoplasm of metanephric blastoma and thus related to the malignant
counterpart of the tissue -namely , Hilms' tumor, or a maturing ~films' t umor.
Along Hith many others "~ have Harned against t reating these patients as if
t hey had Wilms' tumor, However, in the last three year s 1re have had three
insta.'lces in which elseNhere in the kidney t here was either a definite Hi lms'
tumor or a sarcoma -thus complete excision and adequate sampling· is absolut ely
esse!'ltial.
REFERE}TCES.
1.
Bennint;ton, J . L. and Becla~orth, J.B. Tumor s of t he kidney , renal pelvis
and ureters. (Fascicle 12) At l a s of Tumor Pathology, 2nd Series, Armed
Forces I nstitute of Pathology, Nashington , D~C. 1975.
2.
Bogdan, R. , Tayl or , D.E. , JiJostofi, F . K.
kidney. Ca.'lcer 31: 462- 467 , 1973.
3.
Bolande , R.P. , Brough, A.J. and ban·~, R.J. Congenital mesoblasti<::
nephroma of infancy. F\edia:trics 40: 272-78, 1967 .
Laiomyomatous hamartoma of the
.•
4·
Bolande 1 R. P. Congenital 100 sob last ic nephroma of infancy. Perspeot i ve
Pediat ric Pathol. 1: 227- 50, 1973.
5.
Cancer Seminar , Penrose Canosr Hospital , Colorado Springs , Col . Fall
6.
1971 , Vol . 4 , No . 5· Case No . 1. Fetal hamartoma of t he kidney
P• 178-181 .
Ibid. Case No . 3, Conaenital I!Bsoblastic nephroma. p . 184- 187.
7.
Fu, Y. S. a.nd Kay, s . Congenital me soblastic ne phroma and its
recurrenos . Arch . Path. 96 : 66-70 , 1973.
8.
Wigger , H.J.
9.
Ibid. Fetal mesenchymal hamartoma of the kidney.
100senchY!!e • Cancer 36 : 1002-8, 1975.
Feta l hamartoma of kidney.
A. J .c . P. 51 : 323- 37, 1969.
A tumor of secondary
`