Pseudomyxoma peritonei

PSEUDOMYXOMA REPORT

PERITONEI

OF CASE PRESENTING

J.

DUFFY HARRY

BRONCHIAL

FISTULA

HANCOCK, M.D., F.A.C.S. AND M. WEETER, PH.D., M.D. LOUISVILLE,

KY.

INTRODUCTION

P

SEUDOMYXOMA peritonei,

sometimes caIIed “mucous ascites,” may be brieff y defined as a disease characterized by the presence of geIatinous pseudomucin in IocaIized masses or diffuse Iayers within the peritonea1 cavity foIIowing rupture of a pseudomucinous cyst of the ovary or a mucoceIe of the appendix, the masses or Iayers being attached to some intraperitonea1 structure or the parieta1 peritoneum. It was first described in 1884 by Werth who cIearIy estabIished the reIationship between “myxomatous change” of the peritoneum and pesudomucinous cysts of the ovary. WhiIe the condition is not one of the rarities of surgery it is, however, unusuaI and aIways of some interest. None of the reports to which we have had access describes an extension of the disease beyond the abdomina1 cavity. The cIosest approach are a case reported by Masson and Hamrich in which the diaphragm was “ practicaIIy destroyed ” after invasion of the diaphragmatic peritoneum and one mentioned by WiIson where a probabk metastatic area was found in the right Iung at autopsy some vears after the primary operation for the remova of a proIif” erating ovarian cyst. Our case, observed for severa years through two operations showed direct extension through the and an autopsy, diaphragm into the Iung with the estabIishment of a bronchia fistuIa. If this is not unique, it is at Ieast rare enough to justify a review of the disease and report of this particuk case. 731

732

J. DUFFY

HANCOCK

AND

HARRY

M. WEETER

INCIDENCE

Since the disease may foIIow the rupture of a mucoceIe of the appendix as we11 as a pseudomucinous cyst of the ovary, it may occur in either sex, the incidence of course being much higher in the femaIe. It is most common in the fifth and sixth decades of Iife and is very unIikeIy to occur before the age of thirty. SingIe women are rareIy affected. SYMPTOMS

The symptomatoIogy is rather indefinite. Perhaps the most usua1 compIaint is a rather rapid enIargement of the abdomen. WhiIe a sudden sharp pain may occur earIy, probabIy as a resuIt of the rupture of the cyst, there is usuaIIy onIy some degree of uneasiness or discomfort resuIting from pressure and causing a bearing down feeIing in the peIvis, urinary frequency, or shortness of breath. Gain in weight is occasionaIIy observed but some degree of emaciation is the ruIe. The menstrua1 habit is usuaIIy unaffected. Fever is not to be expected unIess there is some degree of acute peritonitis as occurs when a fistuIa deveIops between the cystic mass and some portion of the intestina1 tract. PHYSICAL

SIGNS

The outstanding physica sign is abdomina1 enIargement. This distention is usuaIIy symmetrica but occasionaIIy severa different masses may be identified. The consistency is IikeIy to be softer and more eIastic than that noted in an abdomen distended with thin fluid. The impuIse paIpated when the abdomen is struck with the examiner’s fmger has been described as a thriI1 rather than a wave. Vagina1 examination shows a rather fixed mass extending in a11 directions and the fundus does not float as it appears to in ordinary ascites. The skin usuaIIy shows some paIIor attributabIe to secondary anemia. Aspiration reIeases a thick semi-geIatinous fluid which may be coIorIess or tinged orange to pink.

PSEUDOMYXOMA LABORATORY

PERITONEI

733

FINDING

The bIood count verifies the presence of a secondary anemia, and the number of white ceIIs wiII be variabIe depending somewhat upon the degree of peritonea1 irritation caused by the presence of the cystic materia1. There are no significant urinary changes. The geIatinous materia1 obtained by aspiration or at Iaparotomy can be identified as pseudomucin rather than mucin by the facts that it is aIkaIine and does not precipitate on boiIing or upon the addition of acetic acid. A reducing substances, gIucosamin, has been described. PATHOLOGY

The mere presence of geIatinous materia1 in the peritonea1 cavity does not per se constitute pseudomyxoma peritonei. That disease cannot be said to exist unless masses or Iayers of this materia1 be fixed or firmIy attached to the peritonea1 waI1 or some of the intraperitonea1 organs. The origina source of the substance is usuaIIy the ovary and occasionaIIy the appendix. It is not impossibIe that other organs might be primary foci. An appendix which is the site of a mucoceIe has generaIIy undergone previous inff ammation and rather compIete obIiteration of its canal, thus accounting for the fact that onIy in a smaI1 percentage of cases does infection foIIow rupture. The ovarian Iesion giving rise to mucous ascites is a muItiIocuIar pesudomucinous cystadenoma. WhiIe many Iarge ovarian cysts wiI1 present firm geIatinous contents in the IocuIi at the base of the tumor, the distinguishing characteristic of a pseudomucinous cyst is that its contents remain semi-soIid even in the Iargest IocuIi, instead of becoming more Iiquid as the size of the IocuIi increases. Pseudomucinous cystadenomas of the ovary are usualIy uniIatcra1 (80 per cent) and in onIy 4 per cent of the cases are they complicated by rupture and the deveIopment of pseudomysoma peritonci. WrhiIe the gross appearance of the ruptured cysts is usualI>- malignant, Masson

734

J. DUFFY

HANCOCK

AND

HARRY

M. WEETER

and Hamrich report in a series of 30 cases onIy 43.3 per cent definiteIy mahgnant. When the geIatinous materia1 is discharged, the pertionea1 response may be rather passive or quite active, the Iatter resuIting in genera1 peritonea1 thickening, ceIIuIar infiItration, formation of connective tissue by actua1 proIiferation of secondary tumors, formation of cysts, or a combination of any of these reactions. The peritoneum may become opaque, veIvety, or granuIomatous in appearance. The attachment of the impIants represents a miId infiItration resembIing absorption rather than invasion. When the peritonea1 reaction is such that the proIiferating cysts are choked at the base they are stiI1 IikeIy to rupture and cause other independent growths. The increase in the amount of geIatinous materia1 is probabIy not due to any myxomatous degeneration of the peritoneum but due to a proIiferation of the epitheIia1 eIements which are present in the contents of the ruptured cyst and which become attached to the peritoneum and by their proIiferation reproduce the origina type of cyst. The omentum is a frequent site of invoIvement and may become quite board-Iike in consistency. As much as 26 Iiters of the materia1 have been removed from the peritonea1 cavity. The cherry-Iike coIor sometimes present is due to capiIIary oozing. MicroscopicaIIy, there are numerous minute papiIIary growths usuaIIy covered by singIe Iayered cyIindrica1 epitheIium with eIongated nucIei situated near the base. Due to pressure, however, the ceIIs may be flattened or entireIy atrophied. The cytopIasm is reIativeIy cIear. DIAGNOSIS

A correct preoperative diagnosis is unusua1 unIess some of the aspirated materia1 has been identified. Remembering the possibiIity of the disease in those cases of abdomina1 enIargement where the ffuid wave is atypica1 and the peIvis rather fixed wiI1 probabIy Iead to more frequent accurate diagnoses.

PSEUDOMYXOMA

PERITONEI

735

TREATMENT

The first step in the treatment of the disease is surgica1. When the peritonea1 cavity is opened, presenting masses of the geIatinous materia1 shouId be evacuated and the primary focus sought. If ovarian in origin, and the patient is at or past the menopause, a biIatera1 oophorectomy and appendectomy are indicated; if the patient is much under that age and onIy one ovary is invoIved there is some disagreement as to whether or not the unaffected ovary should be removed. If onIy the appendix is invoIved, uniIatera1 or biIatera1 oophorectomy seems rather radica1. Hysterectomy, partiaI or compIete excision of the omentum, and even resection of some portion of the intestina1 tract may be indicated when these structures show much invasion. Adhesions shouId be freed and a11 accessibIe pseudomyxomatous materia1 removed. This is usuaIIy done manuaIIy, but saIine fIushing has been recommended on the beIief that saIine causes the jeIIy-Iike masses to sweI1 and become Ioosened from their peritonea1 attachment. The abdomina1 wound shouId be cIosed without drainage to minimize the possibiIity of infection. The estabIishment of a permanent fistuIa for continuous drainage does not appear to be a good practice. Postoperative treatment by x-ray or radium is IikeIy to be of service in deIaying or preventing recurrence in some instances. PROGNOSIS

WhiIe frequentIy neither the impIants nor the origina focus of the disease are definiteIy maIignant the outIook is In some instances, the peritonea1 growths not promising. have been reported as subsiding after the remova of the recurrence is the ruIe especiaIIy primary tumor. However, where the primary site is the ovary. The majority of cases terminate fataIIy aIthough death may be deferred some years. Recurrences have been reported as earIy as five months and

736

J. DUFFY

HANCOCK

as_Iate as twenty-two thk routes of exitus.

AND HARRY

M. WEETER

years. Sepsis and emboIism

CASE

are frequentIy

REPORT

The particuIar case that Ied us to present this subject foIIows : Miss A. S., a saIesIady twenty-nine years of age, was first seen at her residence on March I, 1930 in consuhation with her physician, Dr. F. S. CIark: She compIained of abdominal distention, and pain in the Iower right side of her chest. Her family history and previous personal history were of no significance with the exception of an attack of pIeurisy at the base of the right Iung some six months previousIy. Her menstruation had always been reguIar and normaI, but rather scant. She had been as we11 as usual unti1 the previous Christmas hoIidays when she noticed some vague indigestion which she attributed to overeating. In January, 1930, a rather severe pain was noticed in the region of the liver, resembling the attack of pleurisy severa months before. There was a moderate cough at first but this disappeared with the acute pain. Some soreness and tenderness, however, persisted. Two weeks after the onset of pain she observed some distention of the Iower abdomen which rapidly increased until the entire abdomen was quite distended. She became short of breath and while not nauseated, couId eat only smaI1 quantities of food at a time. She had been in bed for the past four weeks. PhysicaI examination showed marked pallor and emaciation, temperature 101.2OF., pulse 130, respiration 24, and bIood pressure I 12/88. The Iungs were cIear but the diaphragm seemed eIevated on both sides. Her heart was normal except for its increased rate. Both pupiIIary and pateIIar reflexes were normal. The abdomen was greatly enlarged and tightIy distended, the overlying skin gIistening. The expected fluid wave couId not be deIiniteIy obtained. No individua1 masses were paIpabIe. There was sIight tenderness in the region of the gal1 bIadder. The percussion note was fiat throughout. Vagina1 examination was difficuIt because of an intact hymen. The cervix was smooth but fuI1, enlarged, and pushed downwards. Pressure upwards on it caused considerabIe pain. The fundus was smaI1 and tightIy fixed in a firm mass that fiIIed the entire peIvis. Recta1 examination confirmed the vaginal findings and further showed the presence of a smaI1 noduIe in the cuI-de-sac. SeveraI days Iater, a drachm of gelatinous materia1 was obtained by paracentesis of the abdomen. The patient was removed to St. Joseph’s

PSEUDOMYXOMA

PERITONEI

:3-

Infirmary. Urine examination there was not remarkable. The blood count showed 60 per cent hemogIobin, 3,620,ooo R.B.C. and 8600 W.B.C. Dr. Irvin Abe11 was caIIed in consultation, suggested the diagnosis of coIIoid carcinoma or pseudomyxoma peritonei, and advised expIoration. The operation scheduIed for March 8, 1930 had to be postponed for severaI days because of a rise in temperature to 105’~. and pulse to 135 with increased tightness in the abdomen. This acute condition rapidI!subsided and on March 12, 1930, exploration was done. The Ieft tube and ovary and the appendix were normal. The right ovary was incorporated in a Iarge pseudomucinous cyst which had ruptured. This cyst, including the right tube and ovary was excised. Seven liters of coIorIess or slightly pinkish geIatinous materia1 were removed from the peritoneal cavity. At no pIace was it attached firmIy to the peritoneum. Even two smaII masses in the cuI-de-sac were easiIy wiped free. The abdomina1 wound was cIosed without drainage. The excised cyst was Iost whiIe the geIatinous material was being measured. The Iatter was soIubIe in water, not precipitated b\ acetic acid, but precipitated by 94 per cent aIcoho1, the precipitate being soIubIe in water, thus presenting the characteristics of pseudomucin from cystadenoma of the ovary. The patient made a nice recovery and was discharged from the hospita1 on March 27, 1930. On ApriI 14, 1930 (two and one-haIf weeks Iater) she was readmitted to the Infirmary compIaining of pain of one week’s duration and a tender mass in the right Iower quadrant, nausea, no vomiting and no constipation. Her temperature was IOI’F., pulse 104, and respiration 22. She was quite dehydrated and the mass just above Poupart’s ligament on the right side was definiteIy paIpabIe and tender. A diagnosis of IocaIized abscess of undetermined origin was made. Enemas, douches, and local applications of ice gave complete reIief and when she was discharged two weeks later, the mass had disappeared, her temperature was normal, and she felt much better generaIIy. She remained symptom-free for eleven months. In February, 1931, she first observed a feeIing of pain, weight, and puIling downward in the Ieft Iower quadrant, and then the appearance there of a mass that graduaIIy enlarged to the right and upwards. Her previous menstrual period had been five or six days late and accompanied by slight nausea. She had Iost weight and experienced some shortness of breath especiaIIy at night. On March 3, 1931 she reentered the hospital. Her genera1 appearance was somewhat better than at the time of the first admission. The temperature was normal, puIse 90, respiration 18, and blood pressure 12gj8g. The chest was stiII essentiaIIy negative. Examination of the abdomen showed a Iarge mass entireIy fIIIing the Ieft side of the abdomen and extending we11 towards the right. Tenderness and noduIes

738

J. DUFFY

HANCOCK

AND HARRY

M. WEETER

were paIpabIe in the Ieft ffank. There was flatness to percussion everywhere except in the right Aank. The same type of indefinite ffuid wave, observed before, couId be detected. Vagina1 examination showed the cervix norma in size, the fundus couId not be paIpated and there was a genera1 feeling of fullness throughout the peIvis. A few R.B.C. were found in the urine. The bIood count showed 70 per cent hemoglobin, 3,720,ooo R.B.c., and I 1,300 W.B.C. with 77 per cent poIymorphonucIear Ieucocytes. A diagnosis of ruptured pseudomucinous cyst of the Ieft ovary was made and the patient’s abdomen was again expIored. The appendix and Ieft tube were normaI. The Ieft ovary showed a Iarge ruptured pseudomutinous cyst. The geIatinous pseudomucin was present in a11 parts of the peritonea1 cavity and in contrast to the findings at the previous operation was firmIy attached to the peritoneum in many pIaces, especiaIIy in the cul-de-sac and on the omentum, mesoappendix, mesosigmoid, and the peritonea1 reflection over the dome of the bIadder. The appendix, Ieft tube, Ieft ovary, some of the impIants, and a11 of the easiIy removabIe pseudomutinous substances were removed. It was, however, noted on the operative record that “the case appears hopeIess as sufficient implants remain to cause further recurrence.” The wound was cIosed without drainage. The pathoIogica1 diagnosis was chronic appendicitis, chronic saIpingitis, and pseudomucinous cyst of the ovary with no microscopic evidence of maIignancy, although the cIinica1 and physica findings were definiteIy to the contrary. Sections through various parts of the tumor masses showed a heavy fibrous stroma; many of the vacuoIes contained no epitheIia1 ceIIs, others showed lining ceIIs varying from coIumnar to fIattened ceIIs with no penetration of the basement membranes. The patient had a stormy convatescence. There was a profuse drainage from the wound, phIebitis in the Ieft thigh, and on one day, there were severa incontinent stooIs of geIatinous material. On April 17, Ig31,‘about six weeks after the operation, she was discharged from the hospita1. In June, 1931, a hard tumor mass appeared in the abdomina1 scar, but never ruptured nor attained much size. In February, 1932, she came to the of&e compIaining of a soft tumor on the right posterior chest waI1 at the IeveI of the ninth or tenth rib. Incision into this mass reIeased a Iarge amount of pseudomucinous material. In May, 1932, she began to expectorate materia1 which she described as simiIar to that which had drained from the tumor on the back. This I was abIe to verify Iater by having her cough some in my presence. The fluid coughed up on three separate occasions was submitted to the Iaboratory for examination. One specimen gave the characteristics of pseudomucin, the others contained numerous misceIIaneous organisms and pus ceIIs and appeared to contain mucus from the respiratory tract.

PSEUDOMYXOMA

PERITONEI

Chest examination, in&ding ftuoroscopy, was indefinite in regard to findings. In August, 1932, a mass appeared in the right upper quadrant of the abdomen, uIcerated through the overIying skin and discharged principaIIy pseudomucinous material Later, there was a smaI1 amount of feca1 drainage. She graduaIIy became weaker and could not Ieave the house after October, 1932. In November she deveIoped an edema of the feet and Iegs. On December 20, she became acuteIy iI with pneumonia which terminated fataIIy on December 23, 1932 approximateIy three years from the beginning of her illness and two years and nine months after the first operations, one year and nine months after the second. W’e were able to secure an autopsy and the findings foIIow. “The body is that of a taI1, poorIy deveIoped emaciated white femaIe about thirty years of age. With the exception of the abdomen and back, the body presents nothing abnormal on externa1 examination except the emaciation. In the mid-line scar between the umbiIicus and symphysis, there are several firm nodules of varying size. In the right upper quadrant at the anterior axiIIary Iine, there is an indurated uIcerating area apparently the externa1 opening of a feca1 IistuIa. On the right side of the back at about the IeveI of the ninth and tenth ribs, there is a soft tumor mass discharging geIatinous materia1. “Upon opening the abdomen, it seems that the masses in the oId scar are implants, as they do not communicate with the peritonea1 cavity. The cavity contains many areas of geIationous materia1 wideIy dispersed with generaIized impIants on the bIadder waI1, fundus, broad Iigaments, large and smaI1 intestines and under-surface of liver. “The appendix, both tubes and both ovaries are absent. “The sinus on the anterior abdomina1 wall communicates with the colon at the hepatic Aexure and the IistuIa is apparentIy due to invasion by the gelatinous impIant. The sinus on the back communicates with the mass of materia1 at the under-surface of the liver. This Iatter mass invades the substance of the Iiver and penetrates through the diaphragm. “Upon opening the chest, both pleura1 cavities are found to be free of fluid and adhesions, save at the right base. The heart is normaI. The left lung presents the usua1 signs of a bronchopneumonia. The right lung is IirmIy fixed to the upper surface of the diaphragm. Upon incising the right lung, there is found in the Iower Iobe a fibrous cavernous growth containing gelatinous material, communicating above with one of the Iarger bronchi (into which it has ulcerated). This is a direct extension of the process that had penetrated the Iiver and diaphragm.

740

J. DUFFY

HANCOCK

AND HARRY

M. WEETER

“Specimens of this mucoid materia1 were preserved and Iater examination showed it to be a pseudomucin Iike that found in the peritoneal cavity and that which had been expectorated a few weeks before death. “Microscopic sections of the tumor mass show an aIveoIar type of growth rather than the papiIIary growth noted at the time of remova of the Ieft ovarian tumor. This distinction, however, is not considered fundamenta1. The Iining epitheIium of these aIveoIi is severa Iayers thick and irreguIarIy flattened so that the individua1 Iining ceIIs are somewhat polyhedraI. SUMMARY

We have attempted to give a brief but fairly definite picture of the disease designated as pseudomyxoma peritonei and have presented a case showing many usual and some very unusua1 features. Among the former were the usua1 rapid enlargement of the abdomen, the initia1 pain thought to be pleurisy but probabIy due to rupture of the cyst, the questionable fluid wave bearing more resembIance to a thriI1, the deveIopment of fever and peritonitis probabIy as the result of invasion of the intestina1 tract, and the rather prompt recurrence of the disease. The unusua1 features included the youth of the patient, her unmarried status, the presence of skin implants, the discharge of geIatinous material in the stool and mvst interesting, the extension of the process through the diaphragm with the subsequent estabIishment of a bronchia f%tuIa. REFERENCES 1. HAMRICH, R. A. Pseudomyxoma peritonaei. PTOC. .%a$ Meet. Mayo Clin., 3: 239, 1928. 2. LEWIS, E. G. Pseudomyxoma of the peritoneum. Surg. Gynec. 06sr., xg: 757-760, ‘9’4. 3. MASSON, J. C., and HAMRICK, R. A. Pseudomyxoma peritonaei of ovarian origin; an analysis of thirty cases. Surg. Clin. N. America, IO: 61-75, 1930. 4. RIES, E. Pseudomyxoma peritonei. Surg. Gynec. Obst., .+ig-579 (Nov.) 1924. 5. MASSON, J. C., and HAMRICK, R. A. Pseudomyxoma peritonaei secondary to ovarian cystadenoma. Canad. M. A. J., 22: 508-512, 1930. 6. WILSON, T. GeIatinous glandular cysts of the ovary and the so-caIIed pseudomyxoma of the peritoneum. Proc. Roy. Sot. Med., Sec. Obst. and Gynec., 6: 9-42, 1913. 7. MASSON, J. D., and HAMRICK, R. A. Pseudomyxoma peritonaei originating from mucocele of the appendix. Surg. Gynec. Obst., 50: 1023-1029, 1930. 8. BOYD, W. SurgicaI Pathology. PhiIa., Saunders, 1915, pp. 333, 392, 520. 9. GRAHAM, E. A. SurgicaI Diagnosis. Phila., Saunders, 1930, 2: 622. DISCUSSION

DR. JOHN W. PRICE, JR., LouisviIIe, Ky.: Dr. Hancock’s case is of interest primariIy because of the extension of the disease through the diaphragm and into the lung and externaIIy. ProIiferated papiIIomatous

PSEUDOMYXOhlA cysts of the ovary tumors cent

are not unusuat.

or growths

proIiferated

serous

of these

growth

of the ovary, cysts

to be present

to rupture, in the

hospitaIs

in both ovaries.

Since

the

and in the medica

Hancock’s

papiIIomatous toms.

father time

factor

That

cyst,

extended

in this

duration.

To emphasize

in JuIy,

1924, in which

adhesions more

years ago having

patient

Kv.:

presented

Dr. Hancock

ovaries,

the

fact I wish to mention cysts

mutinous

materia1

in them,

the typica

symp-

coming

to

had an!,

cavity. has brought

out an

The Iast report

ago, when the doctor

and appendis

or attached

a case I operated

upon

which had ruptured,

growth in the appendix,

to be malignant.

two months

uterus

been caIIed

when he sa)-s it may be of very long

her said that she was stiI1 working on the farm. That and both

is

of his, with a ruptured

found these patients

I found one of these

was about

in the

is removed.

be,yond the peritoneal

condition,

that

and disabiIit\-

confidence

but I do not recal1 ever having

of which were shown microscopicaIIy patient

the

As a rule, the diagnosis

similar cyst in the other ovary-, and a secondary of that

is probabIy

of its tendenc!

on a patient

I have occasionaI1y

DR. M. J. HENRY, LouisviIIe, important

condition.

or twenty-three

the hospita1 with a ruptured case where the growth

I think, because

as a whole we do not see many cases

to operate

cyst of the ovary.

Since that

cysts

20 per the

is opened and the specimen

I recaI1 some twenty-two by Dr.

tumors,

has developed

in a ruptured

made after the abdomen

with the show

The tumor,

profession

associated

papillomatous

and to produce

pubIic

4 per cent of aII

about

in the same o\.ary. About

benign

to have transplants,

come to the hospital

represent

with proliferated

of all designated

patients.

They

741

and are frequently

which develop

patients

most disastrous

PEKITONEI

a all

I had

who referred

was over ten years ago

\vere said to ha\-e maIignant

to them.