Pseudomyxoma peritonei

PSEUUOMYXOMA WITH

HESRY (Prom

the Depnrtmrnt

TV.

o,f

A

PERITONEI*

REPORT OF

ERVINC;, Gynrcoloy~~,

B.A.. PC

THRRK

M.D., t.:fr.sil#

(‘ASE:S ~'ITTSBURGH,

(I/' Pit

t.ubw,c/h,

PA.

School

of

.llrtli~t

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SEUDOIEYXOMA peritonei is an interesting pathologic entity. This condition is characterized by the a?c~umuIation of masses of gelatinous pseudomucinous or mutinous material free in the abdominal cavit,y, and distrihut,ed over the 1)eritoneum either as a homopenous layer or in the form of multiple cyst.ic massc’s. IJcspite removal of the source and the gelatinous material, rcpcated rcaccumulations may occur. necessitating secondary operations. Ries states, and quotes Wcrth as stating, that the mat,erial must have bccomc part of some organ in the abdomen to be called pseutlom~xoma pe&onei. It is it r&ativelF rare condition (30 cases in 18 years at. the Mayo (.%nic*, 3 casesin 20 years at the Elizabeth Steele Magee IIospit;ll), seldom seen in patients before the agt of 40. and occurring in hot h males anal females, although more yornmonly in the latter sex. P&n in 1871 r~ferrcd 1.03 case uf *’ mysomatous &generation of the peritoneum” but Worth in 1884 is (,redited with giving the first description of pseudomyxoma pcritonci. Ilntil 1901 it was considered a diseaseof the female alone, being attributed to rupture of a pseudomucinous ovarian cyst. Then Fraenkel report,ed a cast in a. male with the appendix serving as the original focus. Other SOU~CCY have been claimed ; namely, gall bladder, divertieulum of the cecum, umbilical t,umor developing from a persistent portion of the omphalomesentcric duct, and dermoid cysts; hut in general, the two chief origins are eonsidered to be the ovaries and the appendix. A few caseshave been rc’ported in which both a ruptured ovarian cyst and a perforated caolloid cyst of the appendix were found. Krivsky is convinced that ii’ both organs are involved, the diseasehas two distinct, and simultaneous places of origin; the one is not a result of the other. Xxperimentall?-, psendomyxoma peritonei has been produced in rahbit,s both by- ligation of t,hth appendix, exclusive of the blood supply, and after irrigation of fh~ 111. men, and by the in,jection of unfiltered pseudomucinous material from a typical case. This paper deals only with pseudomysoma peritonci in which the ovaries are the point. of origin. Krivsky thinks the prognosis is wors(~ in this form. When a pseudomucinous cyst of the ovary rupt.ures, thcb reception of the contents by the pcrit~onenm may be passive with absorption of the contents, or there may occur generalized peritoneal thickening, cellular infiltration, formation of adhesions, proliferation o-f secondary tumors and formation of multiple eyst.s; or a combination of *Presented 1942.

at n meeting

of the

Pittsburgh

Obstetrical

and

Gynecological

Suc’iety.

ERVING

:

I’SETJDOMYXOMA

I’KRITONEI

493

a11 these. There may be a different reaction in different parts of the peritoneum. Large amounts of gelatinous material may form, colored in various shades of red, yellow, brown, or grayish white, depending on the amount of hemorrhage, fatty material, cholesterol, and cellular debris. The mechanism of the continued formation of pseudomucinous material before or after the removal of the primary source is still a moot question. Possibly there has been alternate rupture and closure of the ovarian tumor. Or does direct implantation of epithelial cells from the lining of the original cyst occur? Only occasionally can epithelial elements be found in the colloid masses, but perhaps the epithelial cells originally implanted are crowded out. and disappear after having given rise to mutinous material. Can the peritoneal epithelium assume the function of producing mucus? Or is it a metastasis via the peritoneal lymph or blood stream as is undoubtedly the case in secondary cysts found in the liver and portal vein’! Undoubtedly the character of the myxomatous change in the peritoneal walls will vary with that of the primary new growth. For practical purposes, therefore, there may he considered to he two types of secondary pscudomucinous growth : (1) pseudomyxoma peritonei simplex, a benign passive deposit of the pscudomucinous material in the abdominal cavity ; (2) pseudomyxoma peritonei malignum, a transplantation of epithelial cells to the peritoneal surfaces, cells which originally may be malignant or take on malignant characterist,ics. The distinction between malignancy and nonmalignancy depends upon t,hc degree of recurrence. Thus, some cases can be considered definitely malignant with recurrences in as short a period as a few months, others relatively benign, with no recurrence for pears; and still other cases benign when there is no recurrence. Chemically the gelatinous material is alkaline in react,ion. Whether t,he material is acid when the appendix is the focus and alkaline when an ovarian cyst is the focus as Trotter states, is open to question. Also whether the material is pseudomucin or t,ruc mucin as based on its staining reaction with mucicarmine or reduction tests is a valueless distinction ; all gradations of st,ainin, p reactions occur, and reduction tests may be obtained with t,rue mucin. Clinically pseudomyxoma peritonei presents no inconvenience to the patient until enlargement of the abdomen occurs, as simple rupture of t,he ovarian tumor does not, usually produce any noticeable symptoms. Most patients have s>-mptoms for many months before consulting a physician. The chief complaints are fullness of the abdomen, weakness, tlyspnea, frequency of urination, loss of weight, anorexia and often abdominal pain. There may be a history of a previolls operation for removal of the appendix or an ovarian cyst. An interval of twenty-two years after the primary operation before the appearance of pseudo-

494

AMERICAS

.JOITRNAI,

OF

OBSTETRICS

A\t-XI

fiYKECOLO(iE’

myxoma peritonei has been noted. The menstrual hist,org may have been regular or irregular. Marital stains and parity Hart no bearing, for whereas all three of OII~ patients U’(JI*C sin~lc~ wonton, 3S of ih(* 30 1’~ tier& cited by Masson and HamricAk were nl:\rticd ;t nd 21 had had ch i Idren. Physically the ljaticnts usually havrb it mild secondary anemia, abdominal dist,ention, sallow c~omplcxion, and o(*~i~siona.11~ a cystic mass may be felt on abdominal or pelt-icd csan~ination. Treatment consists of two pnrls: (1 ‘, Retttoval of the source and (2) removal of the ps(~uclomncinolw material. ( )ViLl’iXl tumors, whet tl?l’ ruptured or not, should be extirpated, and also 1hc appendix sho~~ltl bc removed even if it is so imbedded in gelatinous material that tht, CPCI~I~ must be resected. If 1he uterus is invaded, a hysterectomy is indicated. If there is a nodule in the umbilicus, remove the umbilicus; and i-f the omentum is involvetl. it should also Ijc esc*isc~cl. The treatment 01‘ the Felatinous material loose in the abdomen and the masses on the parietal and visceral peritoneum is difficult. Washing out is insufficient and complete removal leaves great raw itrfws. (lt,ntlt: wiping out is 11siially resorted to and greater or smaller cluantitirs of material are neccssarily left behind. Isolated l,sc~~~domysomatol~s tumors should brb removed as completely as possible. 13~~~ions csperiencc dictates that, no drains should be left in the abdomen. Finally, s-ray therapy should be given, especially if the pathologist reports malignancy. Hn.t.zlc~r mentions a caase in which temporary imprort~rnc~nt was coincident, with the use of s-ray. lTnfortunatel,v, t hc value of s-ray is neither provt’d nor easy to evaluate. as the secondary lesions map disaypea L’ spanlaneouslv after rcnioYw1 of the source. It is difficult to be clogmatic about the prognosis of patients with psrudomyxoma peritonei. Ries slates that t hct results of operation run>be good at times even though entircx masses 01 gelatinous material ~~nild not be removed. He cites a scvrntr,t,ll-Ivrwr CUI’C’. Several c*i\ses hail been reported of extensive pseudomysoma ptlritonci becoming arrclsted spontaneously and even complet,elg ilbSOrbed. There is no fq>lanat ion for this. Masson and Hamrick bclicve that, it’ t,hc*re is no cvidrnw~ of cancer the ontlook is good for ~>erm>rnt~lrt curt’. Two or threcc opc~rat ions may bc necessary. However, with the peritoneum diffuselp involved, it is impossible t,o eradicate t,he growth, ant1 111;1ily pat ienl-s go downhill so that the condition beromes clinically malignant. To quotct Ihing. * * the evacuation of this material is sometimes followed by remission. rarely 1~~ (qrc, but usually thC condition Wf’llI’S ilild pfmisls PVtItl WllPIl very few tumor cells can be found. Iutert’crenc+c with intest inal l’iui~ tion by adhesions and strictures t.erttl inates thtl prolonged course oi’ iriany Novak, l>ou~lass and Faulkner, and KiIlWlf’I agree 011 this. It cases. ’ ’ is to be noted that a,11the latt,er arc patholoaisls. CI<1% 1 CASE

woman,

REPORTS

l.-Miss N. S., aged 42 years, small, frail, emaciat.ed whit,e was admitted Jan. 19, 1928, to the hospital with a complain1 ol

ERVIiKG

:

PSEUDOMYXOMA

PERITONEI

495

pain in left groin, epigastric fullness, loss of weight and strength. There was a history of feeling weak and tired, having trouble with hemorrhoids, and loss of 18 pounds during past year. There was a sense of fluid splashing about in abdomen. Menopause occurred 2 years previously. For several months epigastric fullness and anorexia were more pronounced. She had to stop work four weeks prior to admission due to weakness. Physical examination showed the abdomen to be rounded Fluid wave was present. and tense with superficial veins prominent. Red blood count was 3,900,OOO; Hg, 76 per cent; and white blood cells, 7,400. Urine, blood chemistry and serology were negative. Operation performed on Feb. 1, 1928, revealed a right ovarian multilocular cyst extending 4 cm. above the umbilicus. Several loops of bowel were adherent. The cyst cont.ained a large amount of amhercolored gelatinous material, and this material was also free in the abdominal cavity, even extending up under the diaphragm. The left ovary was small and atrophic. Gall bladder and appendix were not examined. Cyst and gelatinous material were removed. The incision was closed without drainage. Pathologic Dia.q~osis : Pseudomupinous papillary cystadenoma of ovary, chronic inflammation of cyst wall. Postoperative course was uneventful and when seen six weeks following operation, she was feeling well. There was no further follow-up. C;\SF: ‘L.-Miss Iv. H., aged 62 years, thin, frail, white woman, was admitted Sept. 30, 1941, to the hospital with complaint of increasing fullness of abdomen, loss of 15 pounds in past year, and weakness. There was a history of two previous hospital admissions elsewhere: First in 1931 when she had an appendectomy and a right oophorectomy. Pathologic diagnosis : Dermoid cyst of right ovary, multilocular pseudomutinous cyst of right ovary, and chronic appendicitis. Second in 1936 when there was an operation for evacuation of abdominal contents and removal of part of growth. Pathologic diagnosis: Papillary pseudomutinous cystadenocarcinoma of right ovary. Physical examination revealed a tense abdomen with evidence of fluid, and on vaginal examination there was a soft mass in the cul-de-sac and pelvis, or rat,her multiple small masses. Red blood count was 4,300,OOO; hemoglobin, 76 per cent ; and white blood count, 4,450. Urinalysis, serology, and blood chemistry were negative. Operation performed Jan. 2, 1941, revealed two previous laparotomy spars which were excised. Peritoneum was markedly thickened and on the right side of the abdomen there appeared to be a secondary abdominal cavity well walled-off and filled with a yellowish gelatinous material. Seven quarts of this material were removed, and a biopsy was taken of the peritoneum. Abdomen was closed without drainage. Pathologic diagnosis : Peritoneum and skeletal muscle without evidence of tumor, pseudomyxoma peritonei. Patient appears well four months Postoperative course uneventful. later. X-ray treatments have been given. CASE 3.-Miss B. S., aged 55 years, a well-developed, well-nourished, pale Jewish female, was admitted to the hospit’al on Sept. 25, 1928, with chief complaint of mass in abdomen of one years’ duration, and progressive weakness. She had been operated upon in another hospital five years previously for “flooding spells.” She had a dilatation and

curettage, bilateral salpingo-oophorcctomy, and appendectornJ,. i’alhologic diagnosis : Pseudomucinous ovarian oysf, chronic peri-oophoritis. Physical examination revealed a distended abdomen with a cystic I~SS on the left side. Red blood count was Y,MO,OOO; hemoglobin, 78 per ,cent ; and white blood count,, li),800. Urinalysis, serology, and blood chemistry were negative. She was given a transfusion, and operation was performed on Oct. 1: 1928. “Half a pns basin” of pseudomucinous material was removed, also a left cystic struct,ure considered to be part of left ovary and cystic mass of omentum was removed. Pathologic diagnosis : Psendornuclinons multilocular cystadenocarcinoma of ovary and omentum. Postopcratirct course was uneventful. She remained in hospital twenty-eight days. Stmmd Admission, April, 1931.-She fell well until prrviolls SWI mer. Since then there had been a gradual reaccumulation of fluid in tht abdomen, increasing weakness, fatigue, and pressure symptoms. lit

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mutinous material and a piece of perit,oneum were removed. Pathologic diagnosis : J’eritoneunl with chronic inflammalory reaction. PostoperaGve course was unevent t’l11. She was in the hospital t’lJllrteen days. Third Admissions, September, 2931 .----Tire abdomen had begun to swell shortly after leaving the hospital in May. She was readmitted to drain abdomen. At operation on Sept 10, 1931, ~OLIT gallons of gelatinous mat,erial were removed. Biopsy of peritoncnm was made. Patholoqic diagnosis : Papillary cystadenoma malignum oU ovary. Postoperative course was unevcnlful. Shp was in the hospital twenl)‘11inc days. Fourth Admissio)~, Ja,nuary, 1933.-There was marked improvement following the last operation, and her state of health has been bettet than at any time in years. A cyst is still present in the left low-cr quadrant, t,he size of a grapefruit. She is so much improved following last opera.tion that she was admitted to attempt a removal of what was probably the primary growth. At oper;ation on Jan. 30, 1933, intestines were matted together and involved in a recurrence of the papillary c~stndenocarcinoma. Largcl quantity ot’ Ihick jelly-like, yellowish mat.crml was removed. Supravaginal hysterectomy was done. The tumor growth was removed from remnants of omcntiim, pcritoncum, colon, livc>r, ant1 mesentery of small intestine. Colon was perforated. Yatllologic &ia~,po.sis: Atrophic. c~ndomctrium, phrenic metritis with arteriosclerosis, adenomyoma of uterus, and papillary cystadenoma, seeComplication : fecal fistula. Remained in hospital twentyondary. eight days. Fifth Admissiore, December, 19&Y-Pat.ient felt well up until a few months, a.go. Since then there had been fullness and discomfort in the Physical examination abdomen, anorexia, weakness, and diarrhea. showed a very pale, well-developed, well-nourished, white female of 64 years. Abdomen was dome shaped. Irregular masswas palpable in the upper abdomen. Red blood count was 3,300,OOO; hemoglobin 67 per cent; and white blood count, 9,400. Blood chemistry was negative. Urinalysis showed 1+ albumin. At operation on Dec. 10, 1935, the intestines were found to be studded with small tumorous nodules ; spleen was enlarged and covered with

DOCKERTT

AND

CRAIG

:

497

CHoRIONF:PITIIF:I,IOMA

t,umorous growth; and Y&I/~pounds of gelatinous material were removed. Pathologic diagnosis : Pseudomyxoma peritonei. Postoperative course was uneventful. She was in the hospital twentyone days. This patient was admitted to another hospital about six months lat,er and died in October, 1936, of int.estinal complicat.ions resulting from the abdominal tumor. REFERENCES 1. Ries, Emil: 2. 3. 4. 5. ti.

Masson, Krivsky, Gradinsky, Masson, Curtis:

Rwg., Gynec. & Obst. 39: 569, 1934.

J. C., and Hamriek, R. A.: Canad. M. J. 22: 508, 1930. L. A., Jr.: J. Obst. & Gynaec. Brit. Emp. 28: 201, 1921. M., and Rubnitz, A. 8.: Hurg., Gynec. & Obst. 73: 345, 1941. America 10: 61, 1930. J. C., and Hamrick, R. A.: S. Clin. North Obstetrics and Gynecology, Philadelphia, 1933, W. B. Saunders Co. 2:

p. 1001.

7. Ewing: Neoplastie Diseases, Philadelphia, 1940? W. B. Saunders Co., p. 650. 8. Novak: Gynecologic and Obstetric, Pathology, Philadelphia, 1940, W. A. Saunders co., p. 291. 9. Douglass and Faulkner: Essentials of Obstetrical and Gynecological Pathology, Rt. Louis, 1938, The C. V. Mosby Co., p. 143. IO. Karsner: Human Pathology, Philadelphi:t, 1938, .J. B. Lippincott Company, D. 829. Il. He>tzler, A. E.: The Peritoneum, St. Louis, 1919, The C. 1’. Mosby Co. 2: p. 817. 12. T,ewis, E.: Surg. Gynec. & Obst. 19: 757, 1914.

CHORIONEPITHELIOMA* AN

UNUS~L

CASE IN WHICH CEREBRAL ~V~ETASTASIS YEARS AFTER HYSTERECTO~~~Y

B. DOCKERTY, M.D., AND WINCHELI,

MALCOLM

McB.

OCCURRED

FOVR

CRAIG, M.D.

ROCHESTER, MINN.

ITH considerably more than 2,000 records of chorionepithelioma in the medical literature, an introductory apology is perhaps necesW sary before we add another. The following unique features in our case would appear to justify inclusion of it among the more unusual and interesting examples 0P this rare form of malignant process: (1) a four-year period of latency between removal of the primary neoplasm and the appearance of secondary lesions, (2) cerebra.1symptoms as the first indication of metastasis, and (3) a negative reaction t,o the Friedman test. RF,PORT

OF

A

CASE

A woman, 29 years old, came to the clinic in July, 1941, complaining of headache, weakness, and double and blurred vision. Two and onehalf weeks previous to her examination, she had noticed rather rapid loss of vision, until only the gross form of objects could be seen. There was marked loss of balance with staggering, but the patient did not fall. Thereafter, blurring of vision recurred frequently. Two weeks prior *Submitted

for

publication.

March

30,

1943.