PAT’IIiIiARY CYSTADENONA LYXPtIOhlATC)SU?\[:
A IZARIC
TERATOID
OF TFIE PAROTID
REGION
ALDRED SCOTT WARTHlN
( P r o f e s s o r of Plctliology atad Djreetor of the Patilological Lriborutorifs of the
University of ~ ~ i d t i g n t Ann
i,
Arbor)
T n a diagnostic mtiterial of scrcrnl liundred thousand cases
examined by me since 1895, there have been over seven hundred mixed tumors of the parotid gland, and over five h m clrccl brttiicliial epsts of the cervical region, and two cases only
of ti form of tumor of the parotid region which I must bclicve
to bc of vcry great rarity, not only because of its extremely
low iricidciice in my material, but also because of the silence
in tlie literature regarding it. These cases were as follows:
Case I . No. l43l-L-AD.
Mr. J. McD., age about 45. Tumor
from lower pole of parotid, about half as large as a hen’s egq. Slowly
growing for many years. Pathologic Diagnosis : Papilliferous eystadenoma, the papillae filled with lymphoid tissue with many germ
centers. The epithelium of tlie cyst spaces is a stratified columnar
epithelium of respiratory type. It is undoubtedly a congenital clisturbance of development, a teratoici cystadenoma. It may represent
ail accessory auditory structure. Not malignant.
Case ZZ. No. 4887-T~-AF. Mr. C., age 60. A completely encapsulated tumor 2 ems. in diameter, removed from beneath the lower
pole of the left parotid gland. Tumor has been there 20-30 ytws,
but has increased in size more rapidly recently. Pathologic Diagnosis:
A congenital papillary cystadenoma, lined with respiratory epithelium, the papillae containing much lyniphoid tissue, with germ centers.
A congenital disturhance of development ; may represent accessory ear
structure (Eustachiau tube niucosa) . Not malignant, and should not
recur if a11 removed.
These two tumors a r e practically identical in structure, so
that they may be described as one. They were wholly encnpd a t e d , elastic, oval tumors, with little or n o snrronnding in116
PAPILI,..IRY
CYSTADENOMA LYMPHOMATOSUM
117
flammatory reaction, and no evidences of infiltration. They
were regarded as enlargcd lymphnodes. On section they
wcre found t o be cystic, the cyst-spaces filled with papillae.
The fluid content was small, serous rather than mucoid.
Througliont the papillae whitish nodules, corresponding t o
1. CASE r. P.'IPII,LIFEROIJS CYSTADRNOMA WITH LYMPIIOIDFOLLICLES
GEnx CENTERS I N T H E I'APILLAE, WHICH BKE COVERED WITH A STRAT1FI):D
COLUMNARE P I ~ w E L I m r , I'AI~TLY
CILIA'I'ED A N D PARTLY
SHOWIKG
A CUTICULAI~
FIG.
AND
ROIIDRR.
t h germ Centers, could be seen. ~~icrosc.oy~ically,
tlie strnctiure of tlie two tumors is practically identical. Inside the
capsule is a narrow zone of lymphoid tissue coii taining germ
ceiiters, Init no cvidenccs of lymph sinuses, or a division into
cortical ;znd medullary portions. This lymphoid tissue is
arranged in many-branched papillae covered with stratified
columniir epi tlielium, haviiig a well-defined cuticular border
showing cilia distinctly o ~ e thc
r greater part. In no place
was the epithelium of a squamous cell type. The uppermost
118
ALDRED SCOTT WARTHIN
layer of epithelium 717as of a tall columnar type, with dceplystaining nuclei at tlie outer edge of tlie cells. Beneath the
columnar cell layer there were 2-3 layers of polygoiial or cuboidal cells liaving more lightly staining nuclei and a smaller
amowit of cytoplasm. There was no evidence of muciii-
formation in the cwlumiiar layer, but clear vacuoles appeared
here and there in tlic rather coarsely graiiular cytoplasm of
tlie basal layers. F e w mitoses were s e a l in the epithelium.
The cilia were very distinct in many places ; in others the cells
appeared as if borclercd by a liyaline cnticular zone about
$$-1/8 as thick as the whole cpithelial layer. Between the papillae there- was a rather coarsely granular albuminous precipitate coiiiaining a few lcultocytcs and desqnamatecl cells.
This staincd red with eosin, but was n o t dcnse enough or sufficiently hyaliiie t o be regarded as colloid, and showed no con-
I'APIT~I~ARY CYSTADENOMA LYMPHOMATOSUM
119
cciil ric lamination. No trace of m u c h was foniid between the
braiichiiig aiid nnastomosiiig papillae. On the whole there
was very little space between tlic latter for flnid, the greater
part of the iiitracapsular space being filled with the ~ l o s e l p
packed papillae. The epithelium rested upon a very iia rrow,
almost imperceptible, basement meml-,raiie. The remaiiiclcr
of the stroma coiisisted of lymphoid tissue, a delicate stroma
packed with lymphocytes, and showing numerous large germ
centers. The latter were especially developed in the broadest
portions of t h e papillae, which were romiclish aiid plump.
The narrower papillae without germ centers were Tillus-like
or spatulate ill form. The tissue mas iiot very vascular; the
arterial supply cmall, ~ i i dthe veins not large a i d were thinwalled. No lymphatic trunks were found picrciiig the capsule. No cvicleiicc of malignancy was present i n either tu9
mor ; tlic growth was typical arid regular, st ratified ciliated
columnar cpitlicllium u p o n a lymphadenoid siibrnucosr?, witliout aiiy evidciicc of iiifilt ration. Tlicsc two cystic tumo1.s resembled braiichial cysts in every way, except for the charact e r
of the epithelium, ciliated stratified cohxmnar instead of n
stratified sqnamous cpithclial lining. Rraiicliial c y s t s have
the same subcpitholiiil layer of 1yrnpliudC~iioicltissue aiid germ
centers. Wliilc maiiy of tlicm arc papillomatous tlicp clo not
as a rulc sliow such a clcgree of iiitrncystic papilliferous
growth as i n t h e CRSC of these two cysts. In both cases the
parotid, sliowiiip some degree of pressure atrophy, w n s entirely outside of the cyst will and wholly detached from it.
It is evident from the structure described that we are dealing Iicrc with a hoterotopiu of miicoiis membrane from the
pliaryigeal cntodcrm, represeiitiiig either the upper rcspirntory tract or the I~iistachiantube. The type of mileom rcpre-
PAPILLATLY CYSTADENOMA LYMP HOMATOSU M
121
seiited in this tumor is precisely like that of p a r t of the Eustachian tube wliicli has a tall stratified ciliated columnar epithelium with many lymphoid follicles beiieath tlie epitlielinm.
Iii 110 other p a r t of the respiratory tract is t h e w such a close
FIG. 5.
CASE
IT.
Low POWER
VIEW OF I’APILLIFEKOIJS
CYSTADENOMA,
SHOWINQ
LYMPHOID
TISSUE I N PAPILLAE.
resemblaiice between the normal rnucosa and tlic miicosa lining these cystic tumors. Hearing out this resemblaiice is the
occurreiice in my material of a polypoid tumor of the Rustachiaii tube which had idciitically the same structure as these
two tumors except that it was not eiiclosed in a cyst wall, hiit
preseiitccl a papillomatom growth covered with stratified ciliated epithelium, the stroma of the papillae contaiiiiiig diffnse
lymphoid tissue with large aiid iiiimerous germ centers. This
polyp of tlie Eustachiaii tube is the oiily structnrally closely
related growth t o these two paraparotid cysts that has occurred in my service. No iiasal or nasopharyngeal polyp,
122
A1,T)HED SCOTT WARTI-IIN
out of the many esamiiicd in my service, has showii the same
close resrmblaiicc. 1 am, therefore, iiicliiiccl to believe that
these two papilliferons c y s t adenomas with lymphoid stroma
represent a derclopmciital disturbance of the ear-accessory
Bhst achiaii tube aiiltige, wlii~lihas grow11 slowly through thc
years, assuming rieoplastic tendeiicy rather late in life.
I have heen unable to find descriptions of similar cystic
lymphoid papillary adenomas in any of the special works on
neoplasms. They are not mentioned hy Ewing. I n the litcr-
ature I have found oiily oiic report of what a r e apparciitly
jdeiitical tumors. Albrecht aiid Arzt (1) describe two cases
mliich, according to the description given aiid the illnstrations
given of their structure, are without aiiy doubt wliolly similar
to mine. One was a tnmor the size of a small apple from the
left parotid rcyioii of a mail 64 years of age, the secoiid from
PAI’ILLAI1Y
C’YSTA1)ICNOlLlA L Y M P H O M A T O S T J M
123
the submaxillary region of a girl of twelve years. Both were
papillaiy cystadenomas with st ratified columnar epithelium
with a cuticular border, and a lymphoid stroma containing
Iympli follicles aiid germ centers. These tumors were regarded by Alhrc~clitaiid Arzt as lictcrotopias of pliaryngeal
entodermal aiilape iiit o lymph nodes. Clinically and anstomically they presented the picture of lymphomas, microscopically they were interpreted as papillary cystadenomas encalosecl within typical lymph iiotle tissne. I differ in my iiitcrpretatioii of tlie I p q h o i d tissue, as bciiig an essential part
FIG. 7.
I~II:IIEII.POIVEIL
VIEW, SILOWING
CIIIKA(TEK O F EPITHELIUM
AND
~ i Y h L P I I O I DTISSUE I N T H E RTRONA O F PAPILLAE.
of the misplaced tissue (Enstachian tube mucosa), aiid iiot
a s representing a lymph node into whicli the epithelial elements alone liavc been misplaced. Further, Albrecht and
Arzt describe the epithelium coverjiig the papillae i n their
two tumors as iioii-ciliated but with a cnticular border. Their
124
ALDRED SCOTT WARTHIN
illustrations make it evident that a ciliated border mas
present.
As the brancliial cysts represent lieterotopias or dystopias
of pliaryiigcal miicosa ( squamous epithelium and lymphoid
tissue), so theso two papillary Cystadellomas represent a
dystopia of Eustachiaii tnbe mncosa (ciliated stratified columnar cells mid lymphoid tissue), t o t h o parotid region.
This latter form is \w*ym i d i rarer tliitii the braiichial cyst;
it is d s o much i * i w c ~tliaii tliyrcoglossal remains, wliicli are
found iiwr tlie miildlc of tlie iicck, Iiavc. a single layer of
cdumiiw cclls, witliout lymphoid tissnc and usually show
thyroid aciiii iii tlicl cyst wall. As to the clinical significaiice
of sucli ii p t i ~ d l x r ycystaclenoma lymphomatosum, its C‘XCCSsive rarily m a l w it slight. In t h e e of tlic known f o u r cases
llic cyst bcypn t o show neoplastic tciideiicay a t the age of 45
PAPILLARY CYSTADENOMA LYMPHOMATOSUM
125
i n oiic case, 60 in a second aiid 64 i n the third case; lieiicc
lhe growth is very slow. There is, of course, the possibility
of a maligiiaiit traiisformatioii of such heterotopic tissuc, and
the development of a n adciiocarciiioma. Such an event has
not bceii observed as pet, although it is possible that some
of the adeiiocarcinomas devc~lopingpiaimarily i n the parotid
region map h a w had such a n origin.
In co~ic1iision,this paper presents descriptions of t w o cases
of papillifwoits rystadcnomn ly~iiphonrnto.sir?n,a very rare
teratoid of thc parotid glaiid region, representing a hctcrotopia o r clystopia of pharyngeal eiitoderm, resembling in
siructare most closely the miicosa of the cartilaginous portioii
of the Eiistachiaii tube.
REFERENCE:
1. ALBRECYIT
AhL)
-4RZT:
Frankfurt. Ztschr. f. Path., 1010, iv, 47.