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A study of stromal endometriosis
.A STUDY OF STROMAL
ENDOMETRIOSIS* HOWARD
(From
the
University
of
Oregon
Medical
School,
C. STEARNS, Department
M.D.,
of Obstetrics
PORTLAND,
and
ORE.
Gynecology)
s
TROMAL endometriosis is a primary uterine neoplasm essentially benign in its clinical and histological manifestations and yet often revealing malignant attributes in that it may invade adjacent structures, metastasize to distant areas, and, if not controlled, destroy its host. It need not display cytological characteristics of malignancy to accomplish this end, though it sometimes does. Originating usually as a pure growth of stromal cells from the endometrium, the process tends to invade the uterine musculature, to protrude as polypoid masses into the endometrial cavity, and, with time, to extend int,o the broad ligaments, involve the ureters, often the bladder and rectosigmoid, and to infiltrate the lateral pelvic walls. Once beyond the confines of the uterus the growth is likely to be difficult to eradicate surgically. Doran and Lockyerl in 1908 appear to have first reported this neoplasm, though, according to Ober and JasonZ3 Virchow definitely described stromal endometriosis in both its diffuse and polypoid forms in 1864. Casler14 in 1920 reported the first such growth recognized in the United States. Robertson, Hunter, and associatesl” in 1942 reported a series of 6 cases of stromal endometriosis, emphasizing its pathological and clinical manifestations and stressing their belief that stromal endometriosis and adenomyosis are variants of the same process. Hunter 2~3 has followed with other valuable contributions on the’ same subject, and recently has uncovered an excellent example of a uterine tumor composed of both adenomyosis and stromal endometriosis.32 The stromal elements, the spearhead of the process of adenomyosis, became a relatively pure growth dominating the pathological picture. Goodal121 in 1940 reported 14 To Goodall and Hunter, well-documented cases of stromal mdometriosis. working independently, must go considerable credit for laying the groundwork for our present concept of this disease. In addition, the studies of Novak,33 Frank I5 Miller and Tennant,18 Henderson6 Park and Tennent,2g and Symmonds, Docke&,y, and PratFO deserve recognition. The chief obstacle to the recognition of such growths and to the spread of knowledge concerning them lies in a confusing nomenclature; and, secondarily, there is disagreement by some regarding histogenesis. Such terms as endometrial sarcoma, endometrial sarcoid, endolymphatic stromal myosis, stromatous endometriosis, endometrioma interstitiale, and stromatoid mural sarcoma have been cians
at the Sixty-eighth Annual Gynecologists, Hot Springs, Va.,
*Presented
and
Meeting of the American Sept. 5 to 7, 1957.
Association
of Obstetri-
\Jolume Numlw
75 3
STUDY
OF
STROMAL
FNT)OMETRIOSTS
665
One adenomyosis, stromal endometriosis can be made out even more plainly. sees in freshly cut surfaces of fixed Cssue, very discretely outlined, rounded and st.reak-like structures standing out with great distinctness, both on account of their sharp definition and faint yellow color which contrasts with the white of the myomctrium. (2) I n anothei* group apparently often combined with ( I), numerous polyps of peculiar form cxt,end into the carum . . . the features that distinguish t,hese from ordinary endometrial polyps are (a) the large number, I(b) yellow color, (c) perfectly smooth surfaces, (d) tendency to have either a broad pedicle with a pointed end or a thin stalk with a bulbous extremity, (e) eompletc absence of cysts [later retracted], and (f) a diffusely soft and homogeneous structure. “A third variant of the above types is that in which the stromal cells have Even in these situations the yellow reached the adnesa or even the peritoneum. color of the t,issue and the tendency to form smooth-surfaced ball-like masses over surfaces is characteristic. ’ ’ Hunter, in a recent article,? adds, “it would have been appropriate had we added that even the masses extending beyond the uterus have the resiliency of rubber bands. In a few instances, too, as we now know, cysts may be found in any part of stromal cndometriosis and may be of grossly variable size. “Macroscopically the sarcomatous form is a soft, sometimes sizable tumor which tends to be white rather than yellow and which conforms to the fish-flesh characteristics of many sarcomas.” Microscopically the cells may varv considerably in morphology, even from arca to area in the same specimen. They are commonly of a spindle-cell form, often round. In the benign form the cells are usually uniform in shape, size, a-nd staining qualities. Additionallyi, blood vessels with definite, often thick, w.alls are seen throughout the growth. Multinucleat,ion is not seen and mitoses are scants or absent. mhen one seessuch a picture he should next make reticulum stains, for the normal basket-weave reticulum of endometrial stroma encircling individual cells is retained in neoplasms arising from such stromal cells. The gross picture suggests the diagnosis; the demonstration of stromal cells individually encased in the basketlike reticulum confirms the suspicion. The reader is direct.ed to a paper by Symmonds, Doekerty, and Pratt30 for their discussion on the histology of these growths.
Clinical Aspects of Stromal Endometriosis A perusal of reliably reported casesof stromal neoplasms, together with our personal experience, presents a good outline of age incidence, growth habits, signs and symptoms, and response to therapeutic efforts. The incidence of reported casesis low (about 100 in the English literature). The facts, however, that Symmortds, Dockerty, and Pratt,“O in a review of 56 cases previously diagnosed as endometrial sarcoma, concluded that 16 (29 per cent) of them were definitely stromal-cell neoplasms of a relatively benign character and that all but 3 patients were living and well without demonstrable recurrence from one to fourteen years after operation suggest t,hat other such reviews studied in the light of present-day knowledge would certainly uncover a large number of similar casesimproperly diagnosed. Schiffer, Mackles, and Wolfe31 reported a similar experience in a review of 49 casesof uterine sarcoma. The youngest patient, aged 22 years, was reported upon by Miller and Tennantls and the oldest, aged 68 years, by Symmonds, Dockerty, and Pratt.3” The reporting of at least 8 definitely postmenopausal cases strengthens the belief that the growth is not dependent upon ovarian function. The vast majority occur between the ages of 30 and 50 years,
Slcllomet.i~or~l~n?;i:l,
~‘oStll~~~l~(~~,irll~i~
/
I~ltwling,
pt.li :t’ lmE1lt (1) sittill,
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may bc prominent . Of pain, and er~laiqmwi. reported casesprofuse and piulongctl 1til~~~ing OCCII rt~l. SJmltontls. Jhcl
to
several
childrci~.
Most of the gro\vths are hisfologic~;~ll-\-IAgn. some definil,cly niaiign:rkll.. In any event, wit,11adequate extirpation, life cspectnnc~ is good in most instanrps. Burch’s” patient was living and w(~ll 2,~ ytws aftrr the first 0pe:rntion despite t’he fact that 22 years later rcTur'1'cIIl lllilSS('S of strir1nlal growth \\ClY’ resncted from t.llc omcntum. mesrntcrv of the il4snm,an(l the sigmoid colon into the wall of which it, had invaded ~how~~l~c~c~lion 1 A Jjatient reported upon 1)~ Hunter and associates,.’who had a hr\ !st~~t~?eiomp if1 194S for enlargement of 111~ uterus arid bleeding, was Fount1in 192 lo ha\-r numerous Irtalignnnt-appeari~i~ nodules in t.lie lung, S-ray studies r~k~p~~ntccl in 1953 mid April. 1954, showctil no change, nor XVPTC’ chest symptoms pr’esc~n1. Thy patient, tllected opcralion, so in ,Junc, 1954. snm~ of ills q’ovlh~ in one lung vc-rc shcllcd oul, lye 1)~. William Conklin, ‘l’hc;\- were I’irst Y~~pirttxl to lJ(: sarcomatous. Siibsec4uwt studies by Hunter and Lidbeck ~shc!~cil 1hfm tu lw of strom al-ccl1 origin identical with the uterine growfh. Hiniilar nnrc~nowd noduks iti the Jung appear rocnt,rrcnolopicall~ fo show sonIt’ I3'!:i'c~5ilii)ll ant1 1111: ptivnt rvmnins c~linically WCll. Of interest, also is another ca:;c~rvportcd by Huni~r” of a patient, operated upon in December, 1954. for a large fixed abdominal tumor which extended as an indurated mass into the left p(4vic wall. The tumor appeared to be inoperable and the abdomen was (:lost& She was readmitted in April, 1955, and a panhvstercctomy was snccessf~~ll~ performed by another surgeon with apparent extirpation of the entire grvwth. The gross picture was likn lhat of adenomyosis, but Dr. Emmanuel Bit aI*. p:lthologist7 predicted that. it was and proved it to be stromal endometriosis. AI communication j (1st received from Dr. Bitar states that t,he patient is iu good health without evidence of persisting growt.11. A pathologist not cognizant. of this stronlal neoplasm would probably term it sarcoma and offer a poor prognosis. Stromal endometriosis seemsto Ike rcllativcly vulnerable to s-ray, t,hough experience shows considerahlr differc~nce in Ihe response of individual tumors. Corscaden’” reported 2 cases whcxreilt rcsponsc to irradiation was esccpt.ionally good. Most reports, and our own cspericnce rcporled in Case 1: suggest, a temporary regression of the growth only. It maJ-, however, hc valuable in loosening a fixed inoperable growth to the point of operabilit,y. A review of the literature discloses the fact that subtotal hysterectomy is not adequak, for living stromal extensions into the ccks haye serrcd as a nidus for continued growtl1. 1 have been privileged in recc’n~.Yeats to see 6 bona fide casesof str0ma.l cndometriosis. Four of these have 1~~1 plvvionsly ycportcd by Hunter.?, 3 The other two are presented here fo10the first, time. 1.-i&s. G. F. J., aged 47 yeaw, was n white married womau, gravida ii! pars ii. This patient was first seen in July, 1950, with 1116*complaint of semr0 menstrual bleeding during the previous 6 months. So metvorrhagi:r hntl occurred. [Jrinary frequency CASE
\‘olume
75
Piumhcr
3
STUDY
OF
STROMAl,
667
ENI>OME’lRIOSlS
which had been present for several months was increasing. Pelvic examination showed a moderate cystourethrocele, a small rectocele. a cervix which came to the vaginal introitus on straining, a.nd a corpus enlarged by a 4 cm. tumor easily palpable in the fundal area. The c.avity was 4 cm. deeper than average and bleeding readily occurred mith sounding. h tentative diagnosis of a uterine myoma, together with uteriue descensus and vaginal relaxation, was made. Vaginal hysterectomy and repair wvprc advised and refused. She was next seen in November, 1956, with complaints of hcnvy menstrual flow anil (‘something Examirmtion disclosed a noclular uterus twice uornml size, falling from the vagina.” mobile, and p:-olapsed so the c.rrris protruded bcyontl the introitus. The relaxation of thcs vaginal wall was more marked.
w;:.
l.-case 1. Complcte uterus ren~~ved ~,vlien a nryomectomy was attemptccl. Both
vaginally.
myomas
Note
the
anal stromal
“worms”
cndometriosis
which
popped are present.
out
At operation curettage showed no evidence of malignancy. In order to effect delivery of the corpus a myomectomy was performed. With delivery of the smooth, discrete myoma, a “long white worm” popped out. Incision over another small myoma produced another “ \vorm,” discrete, white, and projecting 3 cm. beyond the serosal surface. A third “worm” issued from the left side of the corpus above the level of the uterine artery and extended into the left broad ligament. Because the lesions observed at this stage of the operation pointed t,o the presence not only of myomas but also of stromal endometriosis, care was taken to watch for other such projections of tissue into the broad ligament spaces. No other one was ssen. The distorted
uterus, contour
(Fig. 1) weighed of the corpus.
286 grams The exposed
and was 16 cm. long. Numerous myometrium, where the myoma
myomas had been
Volume
STUDY
75
Number 3
stromal endometriosie, and necessitated an additional remains well. CASE
2:-Mrs.
OF
STROMAL
the extension operation to
M. H. W.,
aged
of “worms” remove the
49 years,
669
ENDOMETRIOSIS
was a white
into
the
adnexa.
married
broad ligament To this date
woman,
gravida
structures the
patient
ii, para
ii.
I was priviIeged to see this patient with my associate, Dr. Melvin W. Breese, and to vim the pelvis at operation. In 1953 a subtotal hysterectomy was performed in another city and the tissue was reviewed by Dr. William Lidbeek of Salem, Oregon. Hysterectomy was performed because of profuse, irregular bleeding from an enlarged uterus. The diagnosis of degenerating myomas was returned. Vaginal bleeding continued; hence, the cervical Biopsy of the remaining cervical tissue was stump was removed partially in February, 1955. At this last study Dr. Lidbeck recognized performed twice again, in March and July, 1955. the neoplasm as stromal endometriosis. Re-esamination of all tissue previously removrd revealed the same picture. Following removal of a mass in the vagina1 vault in July, 1955, continuous vaginal %leeding occurred until she visited Dr. Breeee in March, 1956. At this visit the entire anterior vaginal wall to within 1 cm. of the introitus was involved in a clinically malignantappearing process. It was believed also to involve the upper rectovaginal septum and cardinal ligament areas. She entered the hospital, where intravenous pyelography indicated a nonfunctioning right kidney. The condition seemed inoperable, hence deep x-ray therapy through 3 pelvic ports to the extent of 2,850 I’ was delivered to the midplane of the pelvis over a period of 8 weeks. Clinical improvement and cessation of bleeding followed, and the mass became much smaller and more mobile. Pyelograms revealed normal function of both ureters. By May, 1957, right pelvic pain became a problem. Because of moderate mobility of the growth and recession in size, operation was decided upon. Kidney function was normal, the chest x-ray negative. With the abdomen opened, a tumor mass was found to involve the right tube, to envelop the right ureter, and to extend around the right hypogastric a.rtery laterally and posteriorly to its union with the common iliac artery. A block dissection of the entire mass was tediously performed, the upper half of the vagina included. Convalescence was uneventful and at this time the pelvis appears free of rrgrowth and her condition is good. Conzment.-Dr. Lidbeck, pathologist of Salem, was able finally to conclude the diagnosis brcause of experience recently gained in other cases of stromal endometriosis. The gross picture aroused suspicion, routine microscopic slides strengthened the suspicion, and silver reticulum stains revealed the typical basket-weave reticulum surrounding individual stromal c~&I. It was an error in the first place to perform subtotal hysterectomy in view of the then extension to the adnsxa had habits of this neoplasm, though it is possible that even occurred. Notable is the fact that x-ray therapy reduced the size of the tumor and produced mobility that invited operation. The question arises, however, whether the last operation was extensive enough in view of the severe involvement of the anterior vaginal wall. If reapperance of the tumor occurs and if the bladder wall is involved, resection of the bladder, vagina, and any other involved area with anastomosis of the ureters into an isolated ileal pouch with cutaneous ileostomy may be considered. An additional c:~se is added to the literature.
Conclusion Stromal endometriosis, a neoplasm until recently poorly understood and thought to be rare, is now recognized to be not uncommon. Formerly most often reported as endometrial sarcoma it, is now recognized to be relatively benign, with exceptions. Ability of the pathologist to recognize the gross differences between stromal endometriosis, adenomyosis, and true uterine sarcoma, then to
STEARNS
670
Am. J. Obst. & Gym. March. 195s
proceed with proper histological studies is essential to proper diagnosis. The wrll-trained clinician, once made aware of the gross appearance of this lesion can alert the pathologist to the likelihood oi’ its presence. There are no signs or symptoms peculiar to stromal endometriosis, though menometrorrhagia, pelvic pain, and enlargement of the uterus arc most common. It is possible for the pathologist to diagnose some casesfrom curettings. \17hile the diseaseis most often benign, it map be histologically malignant,. In rithcr cvcnt it is a dangerous growth and, once beyond the confinen of the uterus, tliI’ficult to eradicate. It ma;- invade norma tissue and metastasize to distant parts. Jt is not dcprlldcllt upon ovarian ;lctivity. The growth may be dcpressed but u2unll-y not. erndicatcd by x-ray. Its cure lies chiefly in comgltte surgical extirpation, the cnrlicr the Ix4Icr. 1n young women with histologically hcnign stromnl cndomctriosis apparent Is confined to the uterus, total hysterc+ iunly with rctcntio3 of ovaries seemsI~cnsonnhle. Tn-o new easesof stl~omal enclorncxtriosishave bern nddctl to 111clitcrat ure. References 1. Doran, 2. Hunter, 3. Hunter, 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31.
Alban H. G., and Lockycr, Cuthbert: Proc. Roy. Roe. Med. 2: 24, 1908. Warren C.: Surgery 34: 258, 1X3. Warren C., Nohlgren, Joseph E., a::d I,ancefield, Stuart M.: ;l?r. J. OBST. & GE’NEC. 72: 1072, 1956. Park, W. Wallace: J. Oljst. 8 Gynacc. Brit. Emp. 56: 755, 1949. A Study of Endometriosis, Endosalpingitis, I%xloeervieosis a11d Goodall, James R.: Peritoneo-ovarian Sclerosis, Philadelphi:~, 1!#3, J. I3. T,ippincott Compnny, chaps. 2, 8, and IO. Henderson, D. Nelson: AM. J. OBST. & G~NEC. 52: 1000, 3946. DeCarle, Donald W.: West. J. Surg. 53: 48, 1945. Embrey, Mostyn D.: J. Ohst. & Ggnaec. Brit. Emp. 59: 846, 1952. Pedowitz, Paul, Felmue, T,aurence B., and Grayzel, David 11.: AM. J. ORST. R- GPX~X’. 69: 1309, 1955. Pedowitz. Paul. Felmus. Laurence IX.. and GravLel. David CT.: A&f. J. 0~~1’. 9 GTN~x’. . 67: ‘549, 1$54. ’ Youngs, P. A., Ingrrsoll, F. M., Mvigs, .T. V.. Wyma,l, Stanley, and Mallory, T. I:.: New England .J. Med. 236: 835, 1947. WheeIock. M. C.. and Strand. C. M.: Oljst. & C,ner. 2: 3%. 195.1. Corscadei, James A.: A&I. :T. OBST. RG GYNEC. 61: 743, l%i. Casler, Dewitt, B.: Surg., G.vnec. $ Ohst. 31: 150, 3920. Frank, Robert T.: Am. J. Cancer 16: 1326, l!Xi?. Brown, Chester R.: New York J. Med. 42: 161, 1942. Keettel, William C., Lee, James CT.. and Randall, .Tohn II.: .Tournal Lancet 69: 261, 1949. Miller, James R., and Tennant. Robert: A>I. J. (hST. 6: GYKEC. 47: 784, 1941. Robertson, Thomas, Hunter. Warren C., T,arson, P., and Snyder, George A. C.: Am. J. Clin. Path. 12: 1, 1942. Rymmonds, Richard I%. and Dockrarty, Malcolm R.: Hurg., Gyncc. & Obst. 100: 232, 1955. Goodall, James R.: J. Obst. & Gynaec. Brit. Emp. 47: 13, 1940. Arthur H.: Textbook of Gync~cologp. ed. 5, I’hiladc,lphia, 1916, W. B. Saundt,rn Curtis, Company, pp. 339-3-13. Oher, William R., and Jason, Rohmt ,S.: A. 34. A. hrclt. I'nth. 56: 301, 1953. Mayenr, M. H., and Alvxar~der, M. K.: .I. Obst. & Gpxw. Xrit. Emp. 58: 8X?, 1951. Vcsell, Morton: West. J. Surg. 57: 593, 1949. Mears, R. F.: Minnesota Med. 37: 812, 1954. DaCunha, F.: J. Obst. & Gynaec. Brit. Emp. 57: 633, 1930. T,ash, A. F., and Lash, R. R. : hr. J. OBST. 6. Gus-x>. 62: 1163, 1951. Park, W. Wallace, and Tennent, R. A.: J. O&t. & Gpnaec. Brit. Emp. 55: Q:i, 1948. Symmonds, Richard E., Dock&y, Mnleolrn F., an11 Pratt, J. H.: Aixr. J. OBST. & G\z~I:I:. 73: 1054, 1955. SchifTer, M. A., Macklcs, Abraham, and Wolfe, 6. Z4.: A&r. J. OBST. & GYNEC. 70: 551. 1955.
FNI’OMF’l’RlOSlS 1‘ 1
OF 32. Hunter, 3.3. Novak,
Warren C. : Personal communication, Emil: Gynecologic and Obstetric Saunders Company, pp. 222223.
1957. Pathology,
671
>
e11. 3, Philndelphin,
1933,
TV.
IX.
Discussion DR. EDWIN J. DE COSTA, Chicago, Ill.-Dr. Stearns has referred to the multiplicity I will confine my remarks largely to the consideration of of names applied to this tumor. nomenclature. There seem to be two causes for confusion in terminology. The first arises from the morphologic characteristics of the neoplasm which suggest benignancy. Microscopically the cells resemble those of the stroma; they are well differentiated and, although there arc The second cause many nuclei, they are usually neither anaplastic nor hyperchromatic. for confusion lies in the tendency to sIow and local spread m.ther than to d:stant metastases. Actually, neither fact should militate against the diagnosis of malignancy. By definition, a malignant growth is one which (‘tends or threatens to produce death. ” The observation that the tissue is not particularly bizarre, that growth is slow, and that it can be eradicated by adequate surgery does not cheek its inherent ability to kill. Hence it is malignant! To speak of borderline malignancy is playing with words; if it can cause death, it is malignant, whether the ultimate dem’se is brought about quickly or slowly. And since it is malignant and of mesotlelial origin, it must be called a sarcoma. Dr. Stearns’ own case reports amply bolster this contention. Permit me to illustrate further how easp it is to err unless careful observation has lleen mado over a long period of time. The litrraturc contains many refercnccs to recurrence after 18 or 20 years. But most illustrat,ive is the reference to stromal endometriosis in the lifth edition of Curtis’ Te;?;tbook of G,ywrology. In Case 2 the growth is described as a “richly cellular sarcoma-like tissue,” yet it is considered a “ henign growth, bordering on malignancy. ’ ’ And the patient was reported to be well 2 years after her operation. But what Curtis could not know at that time was that this same patient died several years later of generalized abdominal metastases of th:s same “ sarcomatoid’ ’ growtll. DR. W. NORMAN THORNTON, JR,., Charlottesville, Va.-Dr. Hunter probably deserves more credit than anyone else for popularizing knowledge of this lesion among his fellow pathologists and gynecologists. At the present time there are only about 100 of these lesions reported in the literature, including those reclassified by Symmonds, Dockerty, and Pratt. There remain some differences of opinion as to nomenclature, but the lesion is now recognized as a distinct neoplasm. The experience of the Mayo group, mentioned above, points out the difficulties encountered in making the diagnosis of stromatosis or stromal endometriosis. Their experience may also account for the wide range of reported 5 year survivals in sarcoma of the endometrium or the uterus. Examination of 56 neoplasms previously reported as sarcomas of the endometrial stroma resulted in 16 of them being reclassified, These authors have presented histologic criteria’for the diagnosis of sarcoma which they believe will avoid some of the difEcuIties in the differential diagnosis between sarcoma and stromal endometriosis, or sl romal hyperplasia. This difference is of considerable importance from a prognostic standpoint. When the varied manifestations of mixed mesodermal tumors of the uterus are recognized, it is not unreasonable to consider stromatosis or stromal endometriosis as a type of mesodtrmal tumor. Although the lesion is usually considered to be benign, it may, by local, endolymphatic or intravenous extension, act as a malignant neoplasm and destroy the host. There will remain some difficulty in distinguishing between stromal endometriosis and sarcoma. As a result of this difficulty, and the reported late recurrence of this lesion, the prognosis must remain guarded. DR. represent
R. R. GREENE, hemangiopericytomas.
Chicago,
Ill.-1 was once misquoted I am sure that is not true.
as believing that all of these I am sure they are an entirely
tliffwrnt I,~JII! (lf hi2011 :Lrl+iug From ~.III~IIII~.,! I I:{] rtrtrru:~. ‘I‘lwy i’:li’l’ S(IV,l ~l~~ri!lrtP lll:lli,~1:ihilf I”‘t,l’llt,i:llitit’s aI111 I do noI tlllrllc it m:ltlt.ri v vt~llirr y,il v:tll 'IlPlil ~~11d0111~'11.1:,, :h:ti'l'oill:,'. 2r, ~Sl~Oill:li~~Sis, 01’ isll:lt, il’ ycj11 n.:ilixc. 1I~:it t Ii:,\ !.3 lmsv pf~lc~11 iill/ t>:,. !‘41r 1,):~ i’..i~ cil:~lij~ri:ci~,~~. l)K!. Ims.uI) N. ITEPJDERHOS, ‘1~1il.lIlili, or1i. ,\I> L(%.,?!! Ior rtltt.riliC tliib. Ilii(-~ili+~(:il is tlmt 10 ywrs ago i\e wportr~(l :L 3:llricp 18fl 7 631 !Ilt:sc~ WY? i:nil>u:Ll tumr~w. :Illd \!ililC t11ca f0llOwu~~ is not cou,plctc 011 tllow p:lt.icul-: iSI LhiV, \,c IIHVI Ii\-,, ;,>r,, iufc~r~~Qtirl~ nlwcr\:t1 iiuw lo l)ring to your :Ittchntillll ucw. ‘1’111~first CilS(’ \Y(L c’lll~ouut~~1i’~ ! v :I:’ tll+. i‘iil>, ;ttl\-:%lIc~~rl t> ‘1’. *:.i:lt ihcsit \Lul-ullilit3 ~‘xl~~rlsiuiii of tumor throughout the I)rosd lig::unc~ui. and fstcntling up tltc! l~Et ovarian arlery as f’:lr :LP 011e rould palpate. An iworupl(+r opwatiorl was obviously performetl and that p:*ti17it has rcmnine~l alive and w:cll fol, 19 ywr~. ‘I’hc sWonc1 c:CV was that of :I youugcxr patient, 08 pp:trs of age, who had a right salpillgo-ooplrorceiom~ an11 supravnginal hysterectomy, Aftcr thr: remorai and examination OF’ tilts specimen, iI was decided that this girl had an ~7ldomctrial sarcoma. The patir:11t Was rroperated upon one month later and the left adnexa, whit;11 were infilt,rated by these fingerlike procesws oT tumor tissue, at111 Ihr, Cwvical stump were removed. That was ill 1943. That girl has remained in apparent good health to Jatc. However. in Xovemher of last year, a routine chest s-ray was taken in the plaw where she worked, and two isolateli tumor nodules wcrc discovered iu 111~ Iraft lung, ou(’ in i.hrz upper lobe, deep i ti tltu lung, and one supcrficixlly Pituatetl in ilr~, iwriphcry of tlw lowr lobe. These haw Iwen rcmowd locally and arc identical in appcuraw~c with the original tumor remowl 11 pwrs :1g0. ?‘hc Il:it,lent still rfmains in good health. I was pleased t 0 hear 1Il.. Gr~wi* say Ihat hr (lid not beliew that these ~<*I’c ali pcri~~tomas I~ceauso, whrw WC got. t.his sprcimcw from this patient’s lung, the pathologist, insihted that it was pcricytoma, so WP lookr~l up the olti scctions and I think we finally Conviucc~~l him that the tumors \v(wc idw~Kiw1 :rnd cert,ainlp wtrc not peric$onuts. \YP have had one case since 1Iif, wrlw 1~2s reported, agaiu with total I~ilutcral salpingo-oophorectomy ; the p:tt,ictnt remained alive and well for drvclopr~l estensiv+? abdominal rccurrcn(~e :tl~tl diet7 in the sixth year.
bysterectoniy 1 years and
and then
1 think that, while it is all righl to ~111 theso tumors sarcoma and they are, there is some virtue in having a separate nomenelaturc to isolate t.hem from the ordinary run of endometrial sarcomas. It ix my impression that where you have a histologic 98rcoma of the eadometrium the prognosis is ext.rrmely poor, whereas in ihis t,ype of lesion tha prognosis is unpredictable. DR. STEARNS (Closing) .-I wish to tleswihe one morn wse which was associated with In this particular case, au interesting metastases of this growth lo the pulmonary tissue. which v-as not under my care, the utcrinca tumor removed in 1949 was diagnosed correctly as stromal endometriosis by the ydthologist. The pelvis had been cleaned out so that no further persistence of growth is detectable at this time. In 1952 a routine Public Health checked x-ray showed smal1, circular lesions iu the Ieft lung. These were of golf-ball size. Nothing was done about them for 2 years at which time, at the prompting of her physician, they wwe removed, or at least a few were. Theso growths shelled out readily; some rather inaccassiblc ones were allowed to remain. The growths mere yellow, spheric@, and on microThe patient remains well in spite of the fact. scopic study proved to be of stromal origin. that two more such growths persist in the left lung and several in the right. Some of these None appears to ho advancing and no perceptible tumors have showed moderate regression. impairment of health is noticed. The question of naming this growth properly has not been settled. Some are true They mCalignancies ; some do not show the t.ypical histological attributes of malignancy. noed not show histological evidence of malignancy in order to metastasize, yet in spite of the often-ljcnign microscopic picture the growth may destroy the host.
ENDOMETRIOSIS* HOWARD
(From
the
University
of
Oregon
Medical
School,
C. STEARNS, Department
M.D.,
of Obstetrics
PORTLAND,
and
ORE.
Gynecology)
s
TROMAL endometriosis is a primary uterine neoplasm essentially benign in its clinical and histological manifestations and yet often revealing malignant attributes in that it may invade adjacent structures, metastasize to distant areas, and, if not controlled, destroy its host. It need not display cytological characteristics of malignancy to accomplish this end, though it sometimes does. Originating usually as a pure growth of stromal cells from the endometrium, the process tends to invade the uterine musculature, to protrude as polypoid masses into the endometrial cavity, and, with time, to extend int,o the broad ligaments, involve the ureters, often the bladder and rectosigmoid, and to infiltrate the lateral pelvic walls. Once beyond the confines of the uterus the growth is likely to be difficult to eradicate surgically. Doran and Lockyerl in 1908 appear to have first reported this neoplasm, though, according to Ober and JasonZ3 Virchow definitely described stromal endometriosis in both its diffuse and polypoid forms in 1864. Casler14 in 1920 reported the first such growth recognized in the United States. Robertson, Hunter, and associatesl” in 1942 reported a series of 6 cases of stromal endometriosis, emphasizing its pathological and clinical manifestations and stressing their belief that stromal endometriosis and adenomyosis are variants of the same process. Hunter 2~3 has followed with other valuable contributions on the’ same subject, and recently has uncovered an excellent example of a uterine tumor composed of both adenomyosis and stromal endometriosis.32 The stromal elements, the spearhead of the process of adenomyosis, became a relatively pure growth dominating the pathological picture. Goodal121 in 1940 reported 14 To Goodall and Hunter, well-documented cases of stromal mdometriosis. working independently, must go considerable credit for laying the groundwork for our present concept of this disease. In addition, the studies of Novak,33 Frank I5 Miller and Tennant,18 Henderson6 Park and Tennent,2g and Symmonds, Docke&,y, and PratFO deserve recognition. The chief obstacle to the recognition of such growths and to the spread of knowledge concerning them lies in a confusing nomenclature; and, secondarily, there is disagreement by some regarding histogenesis. Such terms as endometrial sarcoma, endometrial sarcoid, endolymphatic stromal myosis, stromatous endometriosis, endometrioma interstitiale, and stromatoid mural sarcoma have been cians
at the Sixty-eighth Annual Gynecologists, Hot Springs, Va.,
*Presented
and
Meeting of the American Sept. 5 to 7, 1957.
Association
of Obstetri-
\Jolume Numlw
75 3
STUDY
OF
STROMAL
FNT)OMETRIOSTS
665
One adenomyosis, stromal endometriosis can be made out even more plainly. sees in freshly cut surfaces of fixed Cssue, very discretely outlined, rounded and st.reak-like structures standing out with great distinctness, both on account of their sharp definition and faint yellow color which contrasts with the white of the myomctrium. (2) I n anothei* group apparently often combined with ( I), numerous polyps of peculiar form cxt,end into the carum . . . the features that distinguish t,hese from ordinary endometrial polyps are (a) the large number, I(b) yellow color, (c) perfectly smooth surfaces, (d) tendency to have either a broad pedicle with a pointed end or a thin stalk with a bulbous extremity, (e) eompletc absence of cysts [later retracted], and (f) a diffusely soft and homogeneous structure. “A third variant of the above types is that in which the stromal cells have Even in these situations the yellow reached the adnesa or even the peritoneum. color of the t,issue and the tendency to form smooth-surfaced ball-like masses over surfaces is characteristic. ’ ’ Hunter, in a recent article,? adds, “it would have been appropriate had we added that even the masses extending beyond the uterus have the resiliency of rubber bands. In a few instances, too, as we now know, cysts may be found in any part of stromal cndometriosis and may be of grossly variable size. “Macroscopically the sarcomatous form is a soft, sometimes sizable tumor which tends to be white rather than yellow and which conforms to the fish-flesh characteristics of many sarcomas.” Microscopically the cells may varv considerably in morphology, even from arca to area in the same specimen. They are commonly of a spindle-cell form, often round. In the benign form the cells are usually uniform in shape, size, a-nd staining qualities. Additionallyi, blood vessels with definite, often thick, w.alls are seen throughout the growth. Multinucleat,ion is not seen and mitoses are scants or absent. mhen one seessuch a picture he should next make reticulum stains, for the normal basket-weave reticulum of endometrial stroma encircling individual cells is retained in neoplasms arising from such stromal cells. The gross picture suggests the diagnosis; the demonstration of stromal cells individually encased in the basketlike reticulum confirms the suspicion. The reader is direct.ed to a paper by Symmonds, Doekerty, and Pratt30 for their discussion on the histology of these growths.
Clinical Aspects of Stromal Endometriosis A perusal of reliably reported casesof stromal neoplasms, together with our personal experience, presents a good outline of age incidence, growth habits, signs and symptoms, and response to therapeutic efforts. The incidence of reported casesis low (about 100 in the English literature). The facts, however, that Symmortds, Dockerty, and Pratt,“O in a review of 56 cases previously diagnosed as endometrial sarcoma, concluded that 16 (29 per cent) of them were definitely stromal-cell neoplasms of a relatively benign character and that all but 3 patients were living and well without demonstrable recurrence from one to fourteen years after operation suggest t,hat other such reviews studied in the light of present-day knowledge would certainly uncover a large number of similar casesimproperly diagnosed. Schiffer, Mackles, and Wolfe31 reported a similar experience in a review of 49 casesof uterine sarcoma. The youngest patient, aged 22 years, was reported upon by Miller and Tennantls and the oldest, aged 68 years, by Symmonds, Dockerty, and Pratt.3” The reporting of at least 8 definitely postmenopausal cases strengthens the belief that the growth is not dependent upon ovarian function. The vast majority occur between the ages of 30 and 50 years,
Slcllomet.i~or~l~n?;i:l,
~‘oStll~~~l~(~~,irll~i~
/
I~ltwling,
pt.li :t’ lmE1lt (1) sittill,
oliTnl.iii.
Eaiiguc~.v:lgiir;!l <{iS(>ll;rrgc, c’rl]iii’~(‘lrli’llt 01 {jl(l ill)(~(Jlll,‘Li, \veiglit loss, and lxnvc~l ohs1 ructioli 1);)i Ij I)lY~Il l’qml’tr’~l
IWmalwk, lllC1~~
canstipatiw,
of
IllC
ilJ~parc?lt-I)-
ulc1xs
is common
IlGl~~
tll,~llll;ll~. II;. ~wt?ropt!j 01' 1117~ f+('n is t~Sl~CUClS I~('\.lltld tllil LllC:Pl~S. Ji,KiIli(rrl ill1 l-11(, S~lll;)l~~lil5 :lll(l sign’. ‘3 ~~~(~~~(mel~~()~rh;rg:in. pivica oi’ ill(, ltt(‘1’lls ill? 1llOS~l P~1llllill1Il. Ill illi OE I’;ll’liS’ *i
imtl,
lw
~IitillW~
(Ii'
i I’ 1hc ~Ytk:Vtll
may bc prominent . Of pain, and er~laiqmwi. reported casesprofuse and piulongctl 1til~~~ing OCCII rt~l. SJmltontls. Jhcl
to
several
childrci~.
Most of the gro\vths are hisfologic~;~ll-\-IAgn. some definil,cly niaiign:rkll.. In any event, wit,11adequate extirpation, life cspectnnc~ is good in most instanrps. Burch’s” patient was living and w(~ll 2,~ ytws aftrr the first 0pe:rntion despite t’he fact that 22 years later rcTur'1'cIIl lllilSS('S of strir1nlal growth \\ClY’ resncted from t.llc omcntum. mesrntcrv of the il4snm,an(l the sigmoid colon into the wall of which it, had invaded ~how~~l~c~c~lion 1 A Jjatient reported upon 1)~ Hunter and associates,.’who had a hr\ !st~~t~?eiomp if1 194S for enlargement of 111~ uterus arid bleeding, was Fount1in 192 lo ha\-r numerous Irtalignnnt-appeari~i~ nodules in t.lie lung, S-ray studies r~k~p~~ntccl in 1953 mid April. 1954, showctil no change, nor XVPTC’ chest symptoms pr’esc~n1. Thy patient, tllected opcralion, so in ,Junc, 1954. snm~ of ills q’ovlh~ in one lung vc-rc shcllcd oul, lye 1)~. William Conklin, ‘l’hc;\- were I’irst Y~~pirttxl to lJ(: sarcomatous. Siibsec4uwt studies by Hunter and Lidbeck ~shc!~cil 1hfm tu lw of strom al-ccl1 origin identical with the uterine growfh. Hiniilar nnrc~nowd noduks iti the Jung appear rocnt,rrcnolopicall~ fo show sonIt’ I3'!:i'c~5ilii)ll ant1 1111: ptivnt rvmnins c~linically WCll. Of interest, also is another ca:;c~rvportcd by Huni~r” of a patient, operated upon in December, 1954. for a large fixed abdominal tumor which extended as an indurated mass into the left p(4vic wall. The tumor appeared to be inoperable and the abdomen was (:lost& She was readmitted in April, 1955, and a panhvstercctomy was snccessf~~ll~ performed by another surgeon with apparent extirpation of the entire grvwth. The gross picture was likn lhat of adenomyosis, but Dr. Emmanuel Bit aI*. p:lthologist7 predicted that. it was and proved it to be stromal endometriosis. AI communication j (1st received from Dr. Bitar states that t,he patient is iu good health without evidence of persisting growt.11. A pathologist not cognizant. of this stronlal neoplasm would probably term it sarcoma and offer a poor prognosis. Stromal endometriosis seemsto Ike rcllativcly vulnerable to s-ray, t,hough experience shows considerahlr differc~nce in Ihe response of individual tumors. Corscaden’” reported 2 cases whcxreilt rcsponsc to irradiation was esccpt.ionally good. Most reports, and our own cspericnce rcporled in Case 1: suggest, a temporary regression of the growth only. It maJ-, however, hc valuable in loosening a fixed inoperable growth to the point of operabilit,y. A review of the literature discloses the fact that subtotal hysterectomy is not adequak, for living stromal extensions into the ccks haye serrcd as a nidus for continued growtl1. 1 have been privileged in recc’n~.Yeats to see 6 bona fide casesof str0ma.l cndometriosis. Four of these have 1~~1 plvvionsly ycportcd by Hunter.?, 3 The other two are presented here fo10the first, time. 1.-i&s. G. F. J., aged 47 yeaw, was n white married womau, gravida ii! pars ii. This patient was first seen in July, 1950, with 1116*complaint of semr0 menstrual bleeding during the previous 6 months. So metvorrhagi:r hntl occurred. [Jrinary frequency CASE
\‘olume
75
Piumhcr
3
STUDY
OF
STROMAl,
667
ENI>OME’lRIOSlS
which had been present for several months was increasing. Pelvic examination showed a moderate cystourethrocele, a small rectocele. a cervix which came to the vaginal introitus on straining, a.nd a corpus enlarged by a 4 cm. tumor easily palpable in the fundal area. The c.avity was 4 cm. deeper than average and bleeding readily occurred mith sounding. h tentative diagnosis of a uterine myoma, together with uteriue descensus and vaginal relaxation, was made. Vaginal hysterectomy and repair wvprc advised and refused. She was next seen in November, 1956, with complaints of hcnvy menstrual flow anil (‘something Examirmtion disclosed a noclular uterus twice uornml size, falling from the vagina.” mobile, and p:-olapsed so the c.rrris protruded bcyontl the introitus. The relaxation of thcs vaginal wall was more marked.
w;:.
l.-case 1. Complcte uterus ren~~ved ~,vlien a nryomectomy was attemptccl. Both
vaginally.
myomas
Note
the
anal stromal
“worms”
cndometriosis
which
popped are present.
out
At operation curettage showed no evidence of malignancy. In order to effect delivery of the corpus a myomectomy was performed. With delivery of the smooth, discrete myoma, a “long white worm” popped out. Incision over another small myoma produced another “ \vorm,” discrete, white, and projecting 3 cm. beyond the serosal surface. A third “worm” issued from the left side of the corpus above the level of the uterine artery and extended into the left broad ligament. Because the lesions observed at this stage of the operation pointed t,o the presence not only of myomas but also of stromal endometriosis, care was taken to watch for other such projections of tissue into the broad ligament spaces. No other one was ssen. The distorted
uterus, contour
(Fig. 1) weighed of the corpus.
286 grams The exposed
and was 16 cm. long. Numerous myometrium, where the myoma
myomas had been
Volume
STUDY
75
Number 3
stromal endometriosie, and necessitated an additional remains well. CASE
2:-Mrs.
OF
STROMAL
the extension operation to
M. H. W.,
aged
of “worms” remove the
49 years,
669
ENDOMETRIOSIS
was a white
into
the
adnexa.
married
broad ligament To this date
woman,
gravida
structures the
patient
ii, para
ii.
I was priviIeged to see this patient with my associate, Dr. Melvin W. Breese, and to vim the pelvis at operation. In 1953 a subtotal hysterectomy was performed in another city and the tissue was reviewed by Dr. William Lidbeek of Salem, Oregon. Hysterectomy was performed because of profuse, irregular bleeding from an enlarged uterus. The diagnosis of degenerating myomas was returned. Vaginal bleeding continued; hence, the cervical Biopsy of the remaining cervical tissue was stump was removed partially in February, 1955. At this last study Dr. Lidbeck recognized performed twice again, in March and July, 1955. the neoplasm as stromal endometriosis. Re-esamination of all tissue previously removrd revealed the same picture. Following removal of a mass in the vagina1 vault in July, 1955, continuous vaginal %leeding occurred until she visited Dr. Breeee in March, 1956. At this visit the entire anterior vaginal wall to within 1 cm. of the introitus was involved in a clinically malignantappearing process. It was believed also to involve the upper rectovaginal septum and cardinal ligament areas. She entered the hospital, where intravenous pyelography indicated a nonfunctioning right kidney. The condition seemed inoperable, hence deep x-ray therapy through 3 pelvic ports to the extent of 2,850 I’ was delivered to the midplane of the pelvis over a period of 8 weeks. Clinical improvement and cessation of bleeding followed, and the mass became much smaller and more mobile. Pyelograms revealed normal function of both ureters. By May, 1957, right pelvic pain became a problem. Because of moderate mobility of the growth and recession in size, operation was decided upon. Kidney function was normal, the chest x-ray negative. With the abdomen opened, a tumor mass was found to involve the right tube, to envelop the right ureter, and to extend around the right hypogastric a.rtery laterally and posteriorly to its union with the common iliac artery. A block dissection of the entire mass was tediously performed, the upper half of the vagina included. Convalescence was uneventful and at this time the pelvis appears free of rrgrowth and her condition is good. Conzment.-Dr. Lidbeck, pathologist of Salem, was able finally to conclude the diagnosis brcause of experience recently gained in other cases of stromal endometriosis. The gross picture aroused suspicion, routine microscopic slides strengthened the suspicion, and silver reticulum stains revealed the typical basket-weave reticulum surrounding individual stromal c~&I. It was an error in the first place to perform subtotal hysterectomy in view of the then extension to the adnsxa had habits of this neoplasm, though it is possible that even occurred. Notable is the fact that x-ray therapy reduced the size of the tumor and produced mobility that invited operation. The question arises, however, whether the last operation was extensive enough in view of the severe involvement of the anterior vaginal wall. If reapperance of the tumor occurs and if the bladder wall is involved, resection of the bladder, vagina, and any other involved area with anastomosis of the ureters into an isolated ileal pouch with cutaneous ileostomy may be considered. An additional c:~se is added to the literature.
Conclusion Stromal endometriosis, a neoplasm until recently poorly understood and thought to be rare, is now recognized to be not uncommon. Formerly most often reported as endometrial sarcoma it, is now recognized to be relatively benign, with exceptions. Ability of the pathologist to recognize the gross differences between stromal endometriosis, adenomyosis, and true uterine sarcoma, then to
STEARNS
670
Am. J. Obst. & Gym. March. 195s
proceed with proper histological studies is essential to proper diagnosis. The wrll-trained clinician, once made aware of the gross appearance of this lesion can alert the pathologist to the likelihood oi’ its presence. There are no signs or symptoms peculiar to stromal endometriosis, though menometrorrhagia, pelvic pain, and enlargement of the uterus arc most common. It is possible for the pathologist to diagnose some casesfrom curettings. \17hile the diseaseis most often benign, it map be histologically malignant,. In rithcr cvcnt it is a dangerous growth and, once beyond the confinen of the uterus, tliI’ficult to eradicate. It ma;- invade norma tissue and metastasize to distant parts. Jt is not dcprlldcllt upon ovarian ;lctivity. The growth may be dcpressed but u2unll-y not. erndicatcd by x-ray. Its cure lies chiefly in comgltte surgical extirpation, the cnrlicr the Ix4Icr. 1n young women with histologically hcnign stromnl cndomctriosis apparent Is confined to the uterus, total hysterc+ iunly with rctcntio3 of ovaries seemsI~cnsonnhle. Tn-o new easesof stl~omal enclorncxtriosishave bern nddctl to 111clitcrat ure. References 1. Doran, 2. Hunter, 3. Hunter, 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31.
Alban H. G., and Lockycr, Cuthbert: Proc. Roy. Roe. Med. 2: 24, 1908. Warren C.: Surgery 34: 258, 1X3. Warren C., Nohlgren, Joseph E., a::d I,ancefield, Stuart M.: ;l?r. J. OBST. & GE’NEC. 72: 1072, 1956. Park, W. Wallace: J. Oljst. 8 Gynacc. Brit. Emp. 56: 755, 1949. A Study of Endometriosis, Endosalpingitis, I%xloeervieosis a11d Goodall, James R.: Peritoneo-ovarian Sclerosis, Philadelphi:~, 1!#3, J. I3. T,ippincott Compnny, chaps. 2, 8, and IO. Henderson, D. Nelson: AM. J. OBST. & G~NEC. 52: 1000, 3946. DeCarle, Donald W.: West. J. Surg. 53: 48, 1945. Embrey, Mostyn D.: J. Ohst. & Ggnaec. Brit. Emp. 59: 846, 1952. Pedowitz, Paul, Felmue, T,aurence B., and Grayzel, David 11.: AM. J. ORST. R- GPX~X’. 69: 1309, 1955. Pedowitz. Paul. Felmus. Laurence IX.. and GravLel. David CT.: A&f. J. 0~~1’. 9 GTN~x’. . 67: ‘549, 1$54. ’ Youngs, P. A., Ingrrsoll, F. M., Mvigs, .T. V.. Wyma,l, Stanley, and Mallory, T. I:.: New England .J. Med. 236: 835, 1947. WheeIock. M. C.. and Strand. C. M.: Oljst. & C,ner. 2: 3%. 195.1. Corscadei, James A.: A&I. :T. OBST. RG GYNEC. 61: 743, l%i. Casler, Dewitt, B.: Surg., G.vnec. $ Ohst. 31: 150, 3920. Frank, Robert T.: Am. J. Cancer 16: 1326, l!Xi?. Brown, Chester R.: New York J. Med. 42: 161, 1942. Keettel, William C., Lee, James CT.. and Randall, .Tohn II.: .Tournal Lancet 69: 261, 1949. Miller, James R., and Tennant. Robert: A>I. J. (hST. 6: GYKEC. 47: 784, 1941. Robertson, Thomas, Hunter. Warren C., T,arson, P., and Snyder, George A. C.: Am. J. Clin. Path. 12: 1, 1942. Rymmonds, Richard I%. and Dockrarty, Malcolm R.: Hurg., Gyncc. & Obst. 100: 232, 1955. Goodall, James R.: J. Obst. & Gynaec. Brit. Emp. 47: 13, 1940. Arthur H.: Textbook of Gync~cologp. ed. 5, I’hiladc,lphia, 1916, W. B. Saundt,rn Curtis, Company, pp. 339-3-13. Oher, William R., and Jason, Rohmt ,S.: A. 34. A. hrclt. I'nth. 56: 301, 1953. Mayenr, M. H., and Alvxar~der, M. K.: .I. Obst. & Gpxw. Xrit. Emp. 58: 8X?, 1951. Vcsell, Morton: West. J. Surg. 57: 593, 1949. Mears, R. F.: Minnesota Med. 37: 812, 1954. DaCunha, F.: J. Obst. & Gynaec. Brit. Emp. 57: 633, 1930. T,ash, A. F., and Lash, R. R. : hr. J. OBST. 6. Gus-x>. 62: 1163, 1951. Park, W. Wallace, and Tennent, R. A.: J. O&t. & Gpnaec. Brit. Emp. 55: Q:i, 1948. Symmonds, Richard E., Dock&y, Mnleolrn F., an11 Pratt, J. H.: Aixr. J. OBST. & G\z~I:I:. 73: 1054, 1955. SchifTer, M. A., Macklcs, Abraham, and Wolfe, 6. Z4.: A&r. J. OBST. & GYNEC. 70: 551. 1955.
FNI’OMF’l’RlOSlS 1‘ 1
OF 32. Hunter, 3.3. Novak,
Warren C. : Personal communication, Emil: Gynecologic and Obstetric Saunders Company, pp. 222223.
1957. Pathology,
671
>
e11. 3, Philndelphin,
1933,
TV.
IX.
Discussion DR. EDWIN J. DE COSTA, Chicago, Ill.-Dr. Stearns has referred to the multiplicity I will confine my remarks largely to the consideration of of names applied to this tumor. nomenclature. There seem to be two causes for confusion in terminology. The first arises from the morphologic characteristics of the neoplasm which suggest benignancy. Microscopically the cells resemble those of the stroma; they are well differentiated and, although there arc The second cause many nuclei, they are usually neither anaplastic nor hyperchromatic. for confusion lies in the tendency to sIow and local spread m.ther than to d:stant metastases. Actually, neither fact should militate against the diagnosis of malignancy. By definition, a malignant growth is one which (‘tends or threatens to produce death. ” The observation that the tissue is not particularly bizarre, that growth is slow, and that it can be eradicated by adequate surgery does not cheek its inherent ability to kill. Hence it is malignant! To speak of borderline malignancy is playing with words; if it can cause death, it is malignant, whether the ultimate dem’se is brought about quickly or slowly. And since it is malignant and of mesotlelial origin, it must be called a sarcoma. Dr. Stearns’ own case reports amply bolster this contention. Permit me to illustrate further how easp it is to err unless careful observation has lleen mado over a long period of time. The litrraturc contains many refercnccs to recurrence after 18 or 20 years. But most illustrat,ive is the reference to stromal endometriosis in the lifth edition of Curtis’ Te;?;tbook of G,ywrology. In Case 2 the growth is described as a “richly cellular sarcoma-like tissue,” yet it is considered a “ henign growth, bordering on malignancy. ’ ’ And the patient was reported to be well 2 years after her operation. But what Curtis could not know at that time was that this same patient died several years later of generalized abdominal metastases of th:s same “ sarcomatoid’ ’ growtll. DR. W. NORMAN THORNTON, JR,., Charlottesville, Va.-Dr. Hunter probably deserves more credit than anyone else for popularizing knowledge of this lesion among his fellow pathologists and gynecologists. At the present time there are only about 100 of these lesions reported in the literature, including those reclassified by Symmonds, Dockerty, and Pratt. There remain some differences of opinion as to nomenclature, but the lesion is now recognized as a distinct neoplasm. The experience of the Mayo group, mentioned above, points out the difficulties encountered in making the diagnosis of stromatosis or stromal endometriosis. Their experience may also account for the wide range of reported 5 year survivals in sarcoma of the endometrium or the uterus. Examination of 56 neoplasms previously reported as sarcomas of the endometrial stroma resulted in 16 of them being reclassified, These authors have presented histologic criteria’for the diagnosis of sarcoma which they believe will avoid some of the difEcuIties in the differential diagnosis between sarcoma and stromal endometriosis, or sl romal hyperplasia. This difference is of considerable importance from a prognostic standpoint. When the varied manifestations of mixed mesodermal tumors of the uterus are recognized, it is not unreasonable to consider stromatosis or stromal endometriosis as a type of mesodtrmal tumor. Although the lesion is usually considered to be benign, it may, by local, endolymphatic or intravenous extension, act as a malignant neoplasm and destroy the host. There will remain some difficulty in distinguishing between stromal endometriosis and sarcoma. As a result of this difficulty, and the reported late recurrence of this lesion, the prognosis must remain guarded. DR. represent
R. R. GREENE, hemangiopericytomas.
Chicago,
Ill.-1 was once misquoted I am sure that is not true.
as believing that all of these I am sure they are an entirely
tliffwrnt I,~JII! (lf hi2011 :Lrl+iug From ~.III~IIII~.,! I I:{] rtrtrru:~. ‘I‘lwy i’:li’l’ S(IV,l ~l~~ri!lrtP lll:lli,~1:ihilf I”‘t,l’llt,i:llitit’s aI111 I do noI tlllrllc it m:ltlt.ri v vt~llirr y,il v:tll 'IlPlil ~~11d0111~'11.1:,, :h:ti'l'oill:,'. 2r, ~Sl~Oill:li~~Sis, 01’ isll:lt, il’ ycj11 n.:ilixc. 1I~:it t Ii:,\ !.3 lmsv pf~lc~11 iill/ t>:,. !‘41r 1,):~ i’..i~ cil:~lij~ri:ci~,~~. l)K!. Ims.uI) N. ITEPJDERHOS, ‘1~1il.lIlili, or1i. ,\I> L(%.,?!! Ior rtltt.riliC tliib. Ilii(-~ili+~(:il is tlmt 10 ywrs ago i\e wportr~(l :L 3:llricp 18fl 7 631 !Ilt:sc~ WY? i:nil>u:Ll tumr~w. :Illd \!ililC t11ca f0llOwu~~ is not cou,plctc 011 tllow p:lt.icul-: iSI LhiV, \,c IIHVI Ii\-,, ;,>r,, iufc~r~~Qtirl~ nlwcr\:t1 iiuw lo l)ring to your :Ittchntillll ucw. ‘1’111~first CilS(’ \Y(L c’lll~ouut~~1i’~ ! v :I:’ tll+. i‘iil>, ;ttl\-:%lIc~~rl t> ‘1’. *:.i:lt ihcsit \Lul-ullilit3 ~‘xl~~rlsiuiii of tumor throughout the I)rosd lig::unc~ui. and fstcntling up tltc! l~Et ovarian arlery as f’:lr :LP 011e rould palpate. An iworupl(+r opwatiorl was obviously performetl and that p:*ti17it has rcmnine~l alive and w:cll fol, 19 ywr~. ‘I’hc sWonc1 c:CV was that of :I youugcxr patient, 08 pp:trs of age, who had a right salpillgo-ooplrorceiom~ an11 supravnginal hysterectomy, Aftcr thr: remorai and examination OF’ tilts specimen, iI was decided that this girl had an ~7ldomctrial sarcoma. The patir:11t Was rroperated upon one month later and the left adnexa, whit;11 were infilt,rated by these fingerlike procesws oT tumor tissue, at111 Ihr, Cwvical stump were removed. That was ill 1943. That girl has remained in apparent good health to Jatc. However. in Xovemher of last year, a routine chest s-ray was taken in the plaw where she worked, and two isolateli tumor nodules wcrc discovered iu 111~ Iraft lung, ou(’ in i.hrz upper lobe, deep i ti tltu lung, and one supcrficixlly Pituatetl in ilr~, iwriphcry of tlw lowr lobe. These haw Iwen rcmowd locally and arc identical in appcuraw~c with the original tumor remowl 11 pwrs :1g0. ?‘hc Il:it,lent still rfmains in good health. I was pleased t 0 hear 1Il.. Gr~wi* say Ihat hr (lid not beliew that these ~<*I’c ali pcri~~tomas I~ceauso, whrw WC got. t.his sprcimcw from this patient’s lung, the pathologist, insihted that it was pcricytoma, so WP lookr~l up the olti scctions and I think we finally Conviucc~~l him that the tumors \v(wc idw~Kiw1 :rnd cert,ainlp wtrc not peric$onuts. \YP have had one case since 1Iif, wrlw 1~2s reported, agaiu with total I~ilutcral salpingo-oophorectomy ; the p:tt,ictnt remained alive and well for drvclopr~l estensiv+? abdominal rccurrcn(~e :tl~tl diet7 in the sixth year.
bysterectoniy 1 years and
and then
1 think that, while it is all righl to ~111 theso tumors sarcoma and they are, there is some virtue in having a separate nomenelaturc to isolate t.hem from the ordinary run of endometrial sarcomas. It ix my impression that where you have a histologic 98rcoma of the eadometrium the prognosis is ext.rrmely poor, whereas in ihis t,ype of lesion tha prognosis is unpredictable. DR. STEARNS (Closing) .-I wish to tleswihe one morn wse which was associated with In this particular case, au interesting metastases of this growth lo the pulmonary tissue. which v-as not under my care, the utcrinca tumor removed in 1949 was diagnosed correctly as stromal endometriosis by the ydthologist. The pelvis had been cleaned out so that no further persistence of growth is detectable at this time. In 1952 a routine Public Health checked x-ray showed smal1, circular lesions iu the Ieft lung. These were of golf-ball size. Nothing was done about them for 2 years at which time, at the prompting of her physician, they wwe removed, or at least a few were. Theso growths shelled out readily; some rather inaccassiblc ones were allowed to remain. The growths mere yellow, spheric@, and on microThe patient remains well in spite of the fact. scopic study proved to be of stromal origin. that two more such growths persist in the left lung and several in the right. Some of these None appears to ho advancing and no perceptible tumors have showed moderate regression. impairment of health is noticed. The question of naming this growth properly has not been settled. Some are true They mCalignancies ; some do not show the t.ypical histological attributes of malignancy. noed not show histological evidence of malignancy in order to metastasize, yet in spite of the often-ljcnign microscopic picture the growth may destroy the host.