PAT’IIiIiARY CYSTADENONA LYXPtIOhlATC)SU?\[:

A IZARIC TERATOID OF TFIE PAROTID REGION

ALDRED SCOTT WARTHlN

(Pro fe s sor of Plctliology atad Djreetor of the Patilological Lriborutorifs of the University of ~ ~ i d t i g n t i , Ann Arbor)

T n a diagnostic mtiterial of scrcrnl liundred thousand cases examined by me since 1895, there have been over seven hun- dred mixed tumors of the parotid gland, and over five hm- 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 eyst- adenoma, 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 clis- turbance 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 encap- sulated 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 epithe- lium, 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 re practically identical in structure, so that they may be described as one. They were wholly encnp- d a t e d , elastic, oval tumors, with little or n o snrronnding in-

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PAPILI,..IRY CYSTADENOMA LYMPHOMATOSUM 117

flammatory reaction, and no evidences of infiltration. They were regarded as enlargcd lymphnodes. On section they wcre found to be cystic, the cyst-spaces filled with papillae. The fluid content was small, serous rather than mucoid. Througliont the papillae whitish nodules, corresponding to

FIG. 1. CASE r. P.'IPII,LIFEROIJS CYSTADRNOMA WITH LYMPIIOID FOLLICLES

COLUMNAR E P I ~ w E L I m r , I'AI~TLY CILIA'I'ED A N D PARTLY SHOWIKG A CUTICULAI~ ROIIDRR.

AND GEnx CENTERS I N THE I'APILLAE, WHICH BKE COVERED WITH A STRAT1FI):D

t h germ Centers, could be seen. ~~icrosc.oy~ically, tlie strnc- tiure 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 r thc 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 dceply- staining nuclei at tlie outer edge of tlie cells. Beneath the columnar cell layer there were 2-3 layers of polygoiial or cu- boidal 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 seal in the epithelium. The cilia were very distinct in many places ; in others the cells appeared as i f borclercd by a liyaline cnticular zone about $$-1/8 as thick as the whole cpithelial layer. Between the pa- pillae there- was a rather coarsely granular albuminous pre- cipitate coiiiaining a few lcultocytcs and desqnamatecl cells. This staincd red with eosin, but was not dcnse enough or suffi- ciently 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 ~ lose lp 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 the 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 d the veins not large a id were thin- walled. No lymphatic trunks were found picrciiig the cap- sule. No cvicleiicc of malignancy was present in either tu-

9

mor ; tlic growth was typical arid regular, st ratified ciliated columnar cpitlicllium upon a lymphadenoid siibrnucosr?, witli- out aiiy evidciicc of iiifilt ration. Tlicsc two cystic tumo1.s re- sembled braiichial cysts in every way, except for the charact er 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~iioicl tissue aiid germ centers. Wliilc maiiy of tlicm arc papillomatous tlicp clo not

as a rulc sliow such a clcgree of iiitrncystic papilliferous growth as in the CRSC of these two cysts. In both cases the parotid, sliowiiip some degree of pressure atrophy, wns en- tirely outside of the cyst will and wholly detached from it.

It is evident from the structure described that we are deal- ing Iicrc with a hoterotopiu of miicoiis membrane from the pliaryigeal cntodcrm, represeiitiiig either the upper rcspirn- tory tract or the I~iistachian tube. The type of mileom rcpre-

PAPILLATLY CYSTADENOMA LYMP HOMATOSU M 121

seiited in this tumor is precisely like that of par t of the Eu- stachian tube wliicli has a tall stratified ciliated columnar epi- thelium with many lymphoid follicles beiieath tlie epitlielinm. Iii 110 other par t of the respiratory tract is thew such a close

F I G . 5 . CASE I T . Low POWER VIEW OF I’APILLIFEKOIJS CYSTADENOMA, SHOWINQ LYMPHOID TISSUE IN PAPILLAE.

resemblaiice between the normal rnucosa and tlic miicosa lin- ing these cystic tumors. Hearing out this resemblaiice is the occurreiice in my material of a polypoid tumor of the Rusta- chiaii 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 cili- ated 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 to these two paraparotid cysts that has oc- curred 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 cyst adenomas with lymphoid stroma represent a derclopmciital disturbance of the ear-accessory Bhst achiaii tube aiiltige, wlii~li has 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. In the litcr-

ature I have found oiily oiic report of what a re 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 LYMPHOMATOSTJM 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 re- garded by Alhrc~clit aiid Arzt as lictcrotopias of pliaryngeal entodermal aiilape iiit o lymph nodes. Clinically and anstom- ically they presented the picture of lymphomas, microscop- ically they were interpreted as papillary cystadenomas en- calosecl within typical lymph iiotle tissne. I differ in my iii- tcrpretatioii 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 OF EPITHELIUM AND

~ i Y h L P I I O I D TISSUE I N THE RTRONA O F PAPILLAE.

of the misplaced tissue (Enstachian tube mucosa), aiid iiot a s representing a lymph node into whicli the epithelial ele- ments 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 co- lumnar cells mid lymphoid tissue), to t h o parotid region. This latter form is \w*y m i d i rarer tliitii the braiichial cyst; i t 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 pti~dlxry cystaclenoma lymphomatosum, its C‘XCCS-

sive rarily m a l w it slight. In t h e e of tlic known four cases llic cyst bcypn to show neoplastic tciideiicay a t the age of 45

PAPILLARY CYSTADENOMA LYMPHOMATOSUM 125

in 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 an adciiocarciiioma. Such an event has not bceii observed as pet, although it is possible that some of the adeiiocarcinomas devc~loping piaimarily in the parotid region map h a w had such a n origin.

In co~ic1iision, this paper presents descriptions of two cases of papil l i fwoi ts rystadcnomn ly~iiphonrnto.sir?n, a very rare teratoid of thc parotid glaiid region, representing a hctcro- topia 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.