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Malignant ovarian tumor with ascites and hydrothorax simulating Meigs' syndrome
Malignant Ovarian Tumor with Ascites and Hydrothorax Simulating Meigs’ Syndrome WARREN P. KNUTH, M.D.,Fort Harrison, Montana, AND BENEDICT R. WALSKE, M.D., Omaba, Nebraska
T hydrothorax HE triad
of ovarian fibroma, ascites and as described by Meigs and Cass [I] in 1937 has re-emphasized the significance of this uncommon but highIy important syndrome. It is of historica interest that a number of authors, principaIIy Demons, CulIingworth and SpiegeIberg, had previousIy described the occurrence of pIeura1 and peritonea cavity fluid with ovarian tumors. It remained for Meigs and Cass, however, to demonstrate the importance of surgica1 expIoration in a11 suspected cases and consequently, this syndrome has been named in honor of the senior author. Much emphasis has been given Meigs’ syndrome in the Iiterature during the past twenty years and consequentIy, it is becoming increasingIy common to find this syndrome incIuded in the differentia1 diagnosis of femaIe patients with pIeura1 effusion and ascites. In the face of this awareness, there remain an appreciabIe number of women who are denied the chance for an early and compIete cure by faiIure to appreciate this syndrome. Beresford [2] has noted that these patients are frequentIy assigned to medica wards with such diagnosis as cirrhosis of the Iiver or congestive heart faiIure. Perhaps, just as often, they are considered as having a mabgnant ovarian tumor with metastases and are given up as hopeless. In recent years, Meigs [3] has stated that onIy those tumors which are soIid, benign and “fibroma-Iike” shouId be incIuded in this syndrome. Thecomas and granuIosa ceI1 tumors are fibroma-Iike and for this reason have been incIuded. UnfortunateIy, for purposes of classification a certain number of the Iatter are, in fact, maIignant and therefore the concept of benignity as a prerequisite for incIusion needs revision. Kimbrough [4] has stated that the syndrome shouId in&de either ovarian fiAmerican
Journal
of Surgery,
Volume
~9, March.
IQ58
480
bromas only, or else all ovarian tumors both cystic and soIid with ascites and hydrothorax provided there is no evidence of metastasis in the peritonea1 and pIeura1 cavities. Inasmuch as the importance of recognition of the syndrome is in its therapeutic impIications rather than in uncovering medica curiosities, we are in agreement with the last proposa1. If this were to become generaIIy accepted, al1 patients with adnexa1 masses associated with the other two features of the syndrome wouId be considered in this category and additiona patients might be saIvaged. The foIIowing case report is an exampIe of this situation. CASE
REPORT
H. Y., a fifty-five year oId white female schooIteacher, unmarried, was admitted with the complaint of a 20 pound weight Ioss accompanied by an enlarging abdomina1 mass in the Iower portion of the abdomen. These symptoms were noted to develop during the tweIve months prior to admission. The menopause was uneventfu1 and there had been no vagina1 bIeeding for two years. The patient denied having pain in the abdomen and attributed her weight Ioss to dieting over the past year. FamiIy history reveaIed that the patient’s mother underwent a mastectomy for cancer of the breast twenty-four years ago and was presentIy Iiving and weI1. The past history was noncontributory. On physica examination, the temperature was 98.4%; puIse IOO and bIood pressure 130/7o mm. Hg. The patient was 67 inches talI, weighed 135 pounds and was a we11 deveIoped, we11 nourished white woman with no apparent evidence of weight 10s~. Head and neck were negative for significant abnormaIities. AuscuItation of the chest reveaIed a duI1 friction rub at the Ieft base anteriorIy; the remainder of the Iung fieIds were cIear.
MaIignant
Ovarian
PaIpation of the abdomen showed a large mass in the Ieft lower quadrant which extended above the umbilicus. It was bulbous and appeared cystic. On petvic examination a movable non-tender cervix with a sIight bIuish hue was seen and the uterus was smaI1 and markedIy anteff exed. The mass appeared to be continuous with the Ieft adnexa. The right adnexal area appeared normaI. There was no peripheral edema noted. The remainder of the examination was essentiaIIy within normal limits. Laboratory studies revealed a hemogIobin of 14.6 gm. and 4.5 miIIion red blood ceIIs per cu. mm. Urinalysis was compIetely within norma1 limits and the seroIogy as determined by the VDRL method was negative. X-ray of the chest showed a pIeura1 effusion on the left side. (Fig. I.) ShortIy after admission, thoracentesis was performed and 20 cc. of bIood-tinged fluid was aspirated from the Ieft pIeura1 cavity. This fluid was centrifuged and examined microscopicaIIy. In addition to red celIs, there were numerous smaI1 round ceIIs and Iarge histiocytes, but no ceIIs were noted which were thought to be of maIignant type. SeveraI days Iater, the patient was taken to surgery and the peIvis was expIored through a midIine incision. ConsiderabIe ascitic Auid was present and a Iarge cystic tumor was encountered which measured 26 by 18 by 13 cm. and weighed 3,600 gm. The tumor was attached to the Ieft faIIopian tube and uterus and had the appearance of a cystadenocarcinoma of the ovary. Total hysterectomy with biIatera1 saIpingooophorectomy was carried out without diff& cuIty. The peritonea1 cavity was expIored for evidence of metastatic seeding, but no abnormalities were noted. The abdomen was cIosed and the patient was returned to her room in good condition. PostoperativeIy the course was uncompIicated. The patient was discharged from the hospita1 on her ninth postoperative day and re-examination at varying intervaIs over the past tweIve months revealed no evidence of recurrence. She is presentIy back at her work teaching schoo1; she has gained 15 pounds and Iooks and feeIs weI1. Repeated x-rays of the chest revea1 compIete resolution of the previously noted Ieft pleura1 effusion. The patient has no puImonary symptoms. Figure 2 shows the specimen opened and reveaIs the numerous cystic cavities within the tumor mass. The cysts contained a dark
Tumor
FIG. I. Roentgenogram of the chest showing left hvdrothorax. serosanguineous fIuid and in some areas, dark yeIIowish puruIent exudate was present. The tumor tissue was yeIIowish gray and the cysts were lined with smaI1 papiIIary tumor projections. HistopathoIogicaIIy, a typica papillary serous cystadenocarcinoma was found. (Fig. 3.) COMMENTS It is interesting to note that a variety of cystic and soIid lesions may produce ascites and hydrothorax. In addition to the ovarian neoplasms [5,8-IO], there are Ieiomyomas of the uterus, faIIopian tube papiIIomas, adenocarcinemas of the uterus, and cystic and neoplastic Iesions of the pancreas. In the majority of patients the pIeura1 effusion is Iimited to the right side, but it is not too uncommon to be left-sided or even biIatera1. Many theories have been suggested to expIain the unusua1 formation of ascitic and pleural fluid. Those more commonIy mentioned incIude edema of the tumor, pressure on abdominal Iymph channels and the presence of a hypersensitivity state (Selye’s aIarm reaction). None of these theories have been entirely satisfactory in that certain aspects are not aIways adequateIy expIained. It has been shown that the fluid migrates from the abdominal cavity to the chest in most 481
Knuth
and
WaIske
FIG. 2. Gross appearance of the tumor mass sectioned through its mid-portion.
the chest cIeared and the patient is we11 without recurrent pIeura1 effusion five years Iater. Meigs in 1953 had reported eighty-four cases of true Meigs’ syndrome, and if one considers a11 other cases such as cystadenocarcinomas, Brenner cell tumors, teratomas and Krukeaberg tumors, there wouId be approximateIy 200 case reports at this time. The case reported herein demonstrates the saIient features of the syndrome and varies from the origina definition of the Meigs’ syndrome only by the presence of a non-fibrous ovarian tumor. SUMMARY AND CONCLUSIONS
FIG. 3. Photomicrograph of the tumor showing a papillary serous cystadenocarcinoma.
instances. However, it shouId be remembered that a smaI1 fibroma with Iittle or no ascitic fluid may be associated with hydrothorax of considerabIe degree. In the case reported by Annest [6,7] a very small fibrous tumor was present with no ascites aIthough there was a marked hydrothorax. FolIowing oophorectomy,
Meigs’ syndrome is a rather uncommon but important condition requiring abdomina1 expIoratory surgery and resection of the ovarian tumor. Many patients previousIy considered hopeIess from a variety of misdiagnosed conditions such as metastatic maIignant tumor, congestive heart faiIure, cirrhosis of the Iiver, etc., may be given a new Iease on life by resection of the ovarian tumor. A case demonstrating the various features of Meigs’ syndrome has been presented. The authors are in agreement with the proposal of Kimbrough for the incIusion of a11 ovarian tumors with associated ascites and hydrothorax providing there is absence of peritonea1 or pleura1 metastases. REFERENCES I. MEIGS, J. V. and CASS, J. W. Fibroma of ovary with ascites and hydrothorax with report of
Malignant seven cases. z. 3.
4. 5.
6.
Am.
J.
Obst.
C’P Gynec.,
Ovarian
33: 249,
1937. BERESFORD,0. D. and AIDIN, R. Meigs’ syndrome. Lancer, 2: 211-212, 1950. MEIGS, J. V. Fibroma of the ovary with ascites and hydrothorax-Meigs’ syndrome. Am. J. Obst. t? Gjmec., 67: 962-984, 1954. KIMBROUGH, R. A. Discussion of Meigs’ paper, vol. 67, PP. 985986, 1954. ~~ILLIAHD, G. W., MOORHEAD, C. and TREHERNE, C. A. hleigs’ syndrome; report of case. J. A. .M. A., 15 I : 738-739, 1953. ANXST, L., CLEVELAND, F. and BAKER, J. Report
7. 8.
g. 10.
Tumor of a case of Meigs’ syndrome and a case of malignant tumor of ovary simulating a Meigs’ syndrome. Bull. Mason Clin., g: g;-104, 1955. ANNEST, L. Personal communication. RHOADS, J. E. and TERRELL, A. W. Ovarian tibroma with ascites and hydrothorax (Meigs’ syndrome). J. A. M. A., log: 1684-1688, 1937. ENNS, P. Thecoma of the ovary producing Meigs’ syndrome. Canad. M. A. J., 72: 595.-596, 1955. NORA, E. D. and DAVISON, R. M. Pleurisy with effusion associated with pseudomucinous cystadenoma (Meigs’ syndrome). Dis. of Chest, 13: 423-435,
483
‘947.
T hydrothorax HE triad
of ovarian fibroma, ascites and as described by Meigs and Cass [I] in 1937 has re-emphasized the significance of this uncommon but highIy important syndrome. It is of historica interest that a number of authors, principaIIy Demons, CulIingworth and SpiegeIberg, had previousIy described the occurrence of pIeura1 and peritonea cavity fluid with ovarian tumors. It remained for Meigs and Cass, however, to demonstrate the importance of surgica1 expIoration in a11 suspected cases and consequently, this syndrome has been named in honor of the senior author. Much emphasis has been given Meigs’ syndrome in the Iiterature during the past twenty years and consequentIy, it is becoming increasingIy common to find this syndrome incIuded in the differentia1 diagnosis of femaIe patients with pIeura1 effusion and ascites. In the face of this awareness, there remain an appreciabIe number of women who are denied the chance for an early and compIete cure by faiIure to appreciate this syndrome. Beresford [2] has noted that these patients are frequentIy assigned to medica wards with such diagnosis as cirrhosis of the Iiver or congestive heart faiIure. Perhaps, just as often, they are considered as having a mabgnant ovarian tumor with metastases and are given up as hopeless. In recent years, Meigs [3] has stated that onIy those tumors which are soIid, benign and “fibroma-Iike” shouId be incIuded in this syndrome. Thecomas and granuIosa ceI1 tumors are fibroma-Iike and for this reason have been incIuded. UnfortunateIy, for purposes of classification a certain number of the Iatter are, in fact, maIignant and therefore the concept of benignity as a prerequisite for incIusion needs revision. Kimbrough [4] has stated that the syndrome shouId in&de either ovarian fiAmerican
Journal
of Surgery,
Volume
~9, March.
IQ58
480
bromas only, or else all ovarian tumors both cystic and soIid with ascites and hydrothorax provided there is no evidence of metastasis in the peritonea1 and pIeura1 cavities. Inasmuch as the importance of recognition of the syndrome is in its therapeutic impIications rather than in uncovering medica curiosities, we are in agreement with the last proposa1. If this were to become generaIIy accepted, al1 patients with adnexa1 masses associated with the other two features of the syndrome wouId be considered in this category and additiona patients might be saIvaged. The foIIowing case report is an exampIe of this situation. CASE
REPORT
H. Y., a fifty-five year oId white female schooIteacher, unmarried, was admitted with the complaint of a 20 pound weight Ioss accompanied by an enlarging abdomina1 mass in the Iower portion of the abdomen. These symptoms were noted to develop during the tweIve months prior to admission. The menopause was uneventfu1 and there had been no vagina1 bIeeding for two years. The patient denied having pain in the abdomen and attributed her weight Ioss to dieting over the past year. FamiIy history reveaIed that the patient’s mother underwent a mastectomy for cancer of the breast twenty-four years ago and was presentIy Iiving and weI1. The past history was noncontributory. On physica examination, the temperature was 98.4%; puIse IOO and bIood pressure 130/7o mm. Hg. The patient was 67 inches talI, weighed 135 pounds and was a we11 deveIoped, we11 nourished white woman with no apparent evidence of weight 10s~. Head and neck were negative for significant abnormaIities. AuscuItation of the chest reveaIed a duI1 friction rub at the Ieft base anteriorIy; the remainder of the Iung fieIds were cIear.
MaIignant
Ovarian
PaIpation of the abdomen showed a large mass in the Ieft lower quadrant which extended above the umbilicus. It was bulbous and appeared cystic. On petvic examination a movable non-tender cervix with a sIight bIuish hue was seen and the uterus was smaI1 and markedIy anteff exed. The mass appeared to be continuous with the Ieft adnexa. The right adnexal area appeared normaI. There was no peripheral edema noted. The remainder of the examination was essentiaIIy within normal limits. Laboratory studies revealed a hemogIobin of 14.6 gm. and 4.5 miIIion red blood ceIIs per cu. mm. Urinalysis was compIetely within norma1 limits and the seroIogy as determined by the VDRL method was negative. X-ray of the chest showed a pIeura1 effusion on the left side. (Fig. I.) ShortIy after admission, thoracentesis was performed and 20 cc. of bIood-tinged fluid was aspirated from the Ieft pIeura1 cavity. This fluid was centrifuged and examined microscopicaIIy. In addition to red celIs, there were numerous smaI1 round ceIIs and Iarge histiocytes, but no ceIIs were noted which were thought to be of maIignant type. SeveraI days Iater, the patient was taken to surgery and the peIvis was expIored through a midIine incision. ConsiderabIe ascitic Auid was present and a Iarge cystic tumor was encountered which measured 26 by 18 by 13 cm. and weighed 3,600 gm. The tumor was attached to the Ieft faIIopian tube and uterus and had the appearance of a cystadenocarcinoma of the ovary. Total hysterectomy with biIatera1 saIpingooophorectomy was carried out without diff& cuIty. The peritonea1 cavity was expIored for evidence of metastatic seeding, but no abnormalities were noted. The abdomen was cIosed and the patient was returned to her room in good condition. PostoperativeIy the course was uncompIicated. The patient was discharged from the hospita1 on her ninth postoperative day and re-examination at varying intervaIs over the past tweIve months revealed no evidence of recurrence. She is presentIy back at her work teaching schoo1; she has gained 15 pounds and Iooks and feeIs weI1. Repeated x-rays of the chest revea1 compIete resolution of the previously noted Ieft pleura1 effusion. The patient has no puImonary symptoms. Figure 2 shows the specimen opened and reveaIs the numerous cystic cavities within the tumor mass. The cysts contained a dark
Tumor
FIG. I. Roentgenogram of the chest showing left hvdrothorax. serosanguineous fIuid and in some areas, dark yeIIowish puruIent exudate was present. The tumor tissue was yeIIowish gray and the cysts were lined with smaI1 papiIIary tumor projections. HistopathoIogicaIIy, a typica papillary serous cystadenocarcinoma was found. (Fig. 3.) COMMENTS It is interesting to note that a variety of cystic and soIid lesions may produce ascites and hydrothorax. In addition to the ovarian neoplasms [5,8-IO], there are Ieiomyomas of the uterus, faIIopian tube papiIIomas, adenocarcinemas of the uterus, and cystic and neoplastic Iesions of the pancreas. In the majority of patients the pIeura1 effusion is Iimited to the right side, but it is not too uncommon to be left-sided or even biIatera1. Many theories have been suggested to expIain the unusua1 formation of ascitic and pleural fluid. Those more commonIy mentioned incIude edema of the tumor, pressure on abdominal Iymph channels and the presence of a hypersensitivity state (Selye’s aIarm reaction). None of these theories have been entirely satisfactory in that certain aspects are not aIways adequateIy expIained. It has been shown that the fluid migrates from the abdominal cavity to the chest in most 481
Knuth
and
WaIske
FIG. 2. Gross appearance of the tumor mass sectioned through its mid-portion.
the chest cIeared and the patient is we11 without recurrent pIeura1 effusion five years Iater. Meigs in 1953 had reported eighty-four cases of true Meigs’ syndrome, and if one considers a11 other cases such as cystadenocarcinomas, Brenner cell tumors, teratomas and Krukeaberg tumors, there wouId be approximateIy 200 case reports at this time. The case reported herein demonstrates the saIient features of the syndrome and varies from the origina definition of the Meigs’ syndrome only by the presence of a non-fibrous ovarian tumor. SUMMARY AND CONCLUSIONS
FIG. 3. Photomicrograph of the tumor showing a papillary serous cystadenocarcinoma.
instances. However, it shouId be remembered that a smaI1 fibroma with Iittle or no ascitic fluid may be associated with hydrothorax of considerabIe degree. In the case reported by Annest [6,7] a very small fibrous tumor was present with no ascites aIthough there was a marked hydrothorax. FolIowing oophorectomy,
Meigs’ syndrome is a rather uncommon but important condition requiring abdomina1 expIoratory surgery and resection of the ovarian tumor. Many patients previousIy considered hopeIess from a variety of misdiagnosed conditions such as metastatic maIignant tumor, congestive heart faiIure, cirrhosis of the Iiver, etc., may be given a new Iease on life by resection of the ovarian tumor. A case demonstrating the various features of Meigs’ syndrome has been presented. The authors are in agreement with the proposal of Kimbrough for the incIusion of a11 ovarian tumors with associated ascites and hydrothorax providing there is absence of peritonea1 or pleura1 metastases. REFERENCES I. MEIGS, J. V. and CASS, J. W. Fibroma of ovary with ascites and hydrothorax with report of
Malignant seven cases. z. 3.
4. 5.
6.
Am.
J.
Obst.
C’P Gynec.,
Ovarian
33: 249,
1937. BERESFORD,0. D. and AIDIN, R. Meigs’ syndrome. Lancer, 2: 211-212, 1950. MEIGS, J. V. Fibroma of the ovary with ascites and hydrothorax-Meigs’ syndrome. Am. J. Obst. t? Gjmec., 67: 962-984, 1954. KIMBROUGH, R. A. Discussion of Meigs’ paper, vol. 67, PP. 985986, 1954. ~~ILLIAHD, G. W., MOORHEAD, C. and TREHERNE, C. A. hleigs’ syndrome; report of case. J. A. .M. A., 15 I : 738-739, 1953. ANXST, L., CLEVELAND, F. and BAKER, J. Report
7. 8.
g. 10.
Tumor of a case of Meigs’ syndrome and a case of malignant tumor of ovary simulating a Meigs’ syndrome. Bull. Mason Clin., g: g;-104, 1955. ANNEST, L. Personal communication. RHOADS, J. E. and TERRELL, A. W. Ovarian tibroma with ascites and hydrothorax (Meigs’ syndrome). J. A. M. A., log: 1684-1688, 1937. ENNS, P. Thecoma of the ovary producing Meigs’ syndrome. Canad. M. A. J., 72: 595.-596, 1955. NORA, E. D. and DAVISON, R. M. Pleurisy with effusion associated with pseudomucinous cystadenoma (Meigs’ syndrome). Dis. of Chest, 13: 423-435,
483
‘947.