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PubMedDate not supplied

[Meigs' syndrome: its clinical picture and treatment].

Meigs' syndrome is a rare clinical entity characterised with ovarian benign tumour, ascites and hydrothorax. Between January '94-September '98 we observed three patients with: ovarian neoformation, light (1 patient), moderate (2 patients) monolateral pleural effusion, moderate (2 patients) and considerable (1 patient) ascites. In all patients the preoperative evaluation (sero-haematologic routine, Ca 125 and other oncologic markers, chest X-ray, abdominal and pelvic ultrasonography, total-body Tc, cytological analysis of pleural and abdominal effusion) was suggestive for malignancy but not confirmed it. So an explorative laparotomy with histological extemporary analysis was performed. The results were: 1 fibrothecomas, 1 fibroma, 1 ovarian inflammation with cystic luteinization areas and fibromatosis uterine. All three patients had a good postoperative course. The symptomatology and the effusions disappeared 7-10 days after operation. The first two cases were diagnosed as classic Meigs' syndrome, the third one, instead, as a pseudo-Meigs's syndrome. In conclusion the A.A., according to literature and their experiences, underline: 1) an ovarian mass with pleural and abdominal effusion not always represents an advanced malignancy; 2) even if elevated Ca 125 value is usually associated to a ovarian malignancy, there are some benign lesions in which we observed elevated level of this marker 3) the removal of the ovarian mass is the only resolutive treatment for these patients.

Aged↗
PubMed2000

Meigs' syndrome: the history of the eponym.

Meigs' syndrome is defined as presence of ascites with hydrothorax in association with benign ovarian tumor. The syndrome is named after Joe Vincent Meigs (1892-1963), a Harvard Medical School Professor of Gynecology. However, it was described by several authors in the 19th and the beginning of 20th centuries. Meigs and Cass deserve the credit for awakening the medical profession to the importance of the syndrome in 1937. At the end, the syndrome was coined Meigs' syndrome by Rhodes and Terrell in 1937.

Ascites↗
PubMedDate not supplied

Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature.

Meigs' syndrome is the association of ovarian fibroma, pleural effusion, and ascites. Meigs' syndrome with marked elevation of CA125 is an unusual clinical condition reported in 27 cases in the literature. The patient was a 46-year-old woman with right pleural effusion, ascites, ovarian tumor, and CA125 level of 1808 U/mL. Tomography revealed ascites and bilobate pelvic tumor of approximately 25 cm. The diagnosis of advanced epithelial ovarian cancer was considered, and the patient was treated with chemotherapy. Three chemotherapy schemes were applied due to the total lack of response in tumor volume; however, CA125 decreased to 90 U/mL. Thus, surgery was performed with resection of 25 cm of the left ovarian tumor, with intact capsule and without implants; the result of histopathologic analysis was fibroma. Postoperative CA125 was 11 U/mL. Patients with elevated CA125 and ascites cytology positive for malignancy must be cautiously treated due to the possibility of false positives, even if the probability is low. Therefore, minimally invasive surgery for biopsy collection must be considered. Although the association between ovarian tumor, pleural effusion, ascites, and marked elevation of CA125 is highly indicative of epithelial ovarian cancer, Meigs' syndrome must be considered in the differential diagnosis.

Adenocarcinoma↗
PubMed2005

Meige syndrome and pallidal deep brain stimulation.

The cause of primary Meige syndrome is unknown, and although gender and age predilections are different from idiopathic torsion dystonia, most investigators consider Meige syndrome a variant of that disorder. Interest in the use of stereotactic brain surgery for refractory forms of dystonia is thus increasing. There is little experience with the use of deep brain stimulation (DBS) in focal dystonias, and reports of its use in Meige syndrome are very rare. We report on a case of Meige syndrome successfully treated with bilateral pallidal DBS.

Adult↗
PubMed1988

Meige syndrome: neuropathology of a case.

Primary Meige syndrome is a form of cranial dystonia of unknown cause. Only three postmortem studies have been reported, and the results of these studies have not been consistent. We have examined the brain of a 72-year-old man with typical primary Meige syndrome and found mild to moderate cell loss in the zona compacta of the substantia nigra, locus ceruleus, midbrain tectum, and dentate nucleus of the cerebellum. Also frequent Lewy bodies were present in pigmented nuclei of the brainstem. No abnormalities were detected elsewhere. These pathological findings support the notion that brainstem pathology is important in the pathophysiology of cranial dystonia.

Aged↗
PubMedDate not supplied

[Pseudo-Meigs syndrome, a rare variant].

The pseudo- Meigs syndrome is defined as a pelvic tumour, other than the ovarian fibroma complicated with ascites and hydrothorax that can be recovered after the tumour is surgically extirpated. The uterine leiomyoma is an extremely rare cause of this syndrome, only 24 cases have been recorded so far, most of them presenting hydropic degeneration or necrosis. The case exposed by us, a 50- year old obese,with nanism woman, presented clinical, biological and imaging characteristics of the syndrome; moreover, she had arterial high blood pressure for more than five years, fact that didn't need postoperative treatment. She was sent to the ER because she had severe respiratory insufficiency, arterial high blood pressure, tachycardia and, at the clinical examination, she presented massive right hydrothorax, ascites, and pelvic tumour. The biologic explorations (the benign cytology in the pleural liquid and ascites, CA-125 with ten times the normal value) and the imagery completed the picture of a Meigs/ pseudo-Meigs syndrome that implied the laparotomy. The H-P examination and the postoperative evolution confirmed the diagnosis. We presented this case in order to emphasize both its rarity and its real positive and differential diagnosis problems.

Ascites↗
PubMed1995

[Perioperative management of patients with Meigs syndrome].

We report our perioperative management of three cases of Meigs syndrome. The major pre-operative problems in Meigs syndrome are physical trouble caused by giant mass in peritoneal space, respiratory distress, and poor nutrition. These problems must be settled before the operation. The important points in the pre-operative management are 1) respiratory care employing the intermittent positive pressure breathing (IPPB) and the pleural effusion drainage, and 2) the correction of intravascular volume and the concentration of albumin and hemoglobin by transfusion of massive lactated Ringer solution and albumin solution and/or whole blood when they are necessary. During the operation, the epidural anesthesia under spontaneous breathing is the best method of anesthesia. According to circumstances, we adopt the intra-tracheal intubation with continuous positive airway pressure breathing (CPAP). We can generally deal with excessive bleeding by transfusion of lactated Ringer solution and plasma expander, during the first half of operation. By the end of the operation, however, the correction of the concentration of albumin and hemoglobin must be made by the fresh frozen plasma and blood transfusion. After the operation, we use epidural analgesia to control the postoperative pain. We have succeeded in the treatment of three cases of Meigs syndrome owing to our perioperative management as described above.

Analgesia, Epidural↗
PubMed2000

Conjunctival edema and alopecia of the external third of the eyebrows in a patient with Meige syndrome.

PURPOSE: To describe a patient with Meige syndrome in whom we observed the coexistence of hereditary lymphedema of the lower legs, conjunctival edema and alopecia of the lateral third of the eyebrows. METHODS: Case report. RESULTS: Histological examination of the conjunctival and skin specimens showed dermal edema and a slight reduction in the number of severely ectatic lymphatics in the reticular dermis. The vessel were identified as lymphatics on the basis of immunohistochemical evidence of discontinuity and/or absence of basement membrane. CONCLUSIONS: Clinical and histological findings suggest that the etiopathogenesis of the edema in Meige syndrome is related to a structural ectatic defect of lymphatics. This anomaly seems to involve both skin and other sites, such as conjunctival mucosa.

Adult↗
PubMed1998

Pseudo-Meigs' syndrome due to broad ligament leiomyoma: a mimic of metastatic ovarian carcinoma.

Pseudo-Meigs' syndrome is a rare complication of benign leiomyomas of the female genital tract. We report a patient with pseudo-Meigs' syndrome due to a large broad ligament leiomyoma, which also caused bilateral reversible hydronephrosis. This unusual combination of pseudo-Meigs' syndrome, broad ligament leiomyoma and hydronephrosis requiring ureteric stenting does not appear to have been reported previously. Features of the syndrome that led to a diagnostic problem, the mimicking of metastatic ovarian carcinoma, are presented. The little that is known of the pathogenesis of the pleural and ascitic fluids is discussed. An elevated level of serum CA 125 antigen is reported, we believe for the first time in association with pseudo-Meigs' syndrome.

Adult↗
PubMed2001

Cumulative dosage effect of a RAD51L1/HMGA2 fusion and RAD51L1 loss in a case of pseudo-Meigs' syndrome.

Uterine leiomyoma presenting with ascites and pleural fluid is referred to as pseudo-Meigs' syndrome. It is unclear whether common uterine leiomyomas and uterine leiomyomas causing pseudo-Meigs' syndrome are cytogenetically related or whether functionally different primary pathogenetic triggers are responsible for the differences in tumor phenotype. In this study, we investigated the possible involvement of RAD51LI and HMGA2 (formerly known as HMGIC) in initiation and/or progression of a huge uterine leiomyoma presenting as pseudo-Meigs' syndrome. The detailed cytogenetic and FISH analysis revealed the presence of two subclones with a complex karyotype, 46,XX,t(2;12)(q31;q21),ins(14;12)(q23-24;q15q21).ish del(12)(q15q15) (LL12NC01-142H1-,LL12NC01-27E12-),der(12)t(2;12)(LL12NC01-142H1+,LL12NC01-27E12-),der(14)ins(14;12)(q22;q15q15) (LL12NC01-142H1+,LL12NC01-27E12+,RAD51LI+), der(14)ins(14;12)(q23-q24;q15q21) (LL12NC01-142H1-, LL12NC01-27E12+) [20]/46,idem,del(14)(q21q24).ish(RAD51LI-) [6], indicating intragenic HMGA2 rearrangement and loss of one of the RAD51LI alleles in a derivative subclone with chromosome 14 deletion. Furthermore, RACE and RT-PCR analysis of the tumor cells did not reveal abnormal HMGA2 or RAD51LI transcripts. Additionally, the cellular subclone with intrachromosomal 14q21-q24/RAD51LI deletion showed an in vitro growth advantage over the subclone without the deletion. This observation supports a model in which accumulation of two independent mutations-a classical structural rearrangement involving HMGA2 and RAD51L1, in combination with a loss of the second RAD51L1 allele-might play a major role in the development of pseudo-Meigs' syndrome.

Adult↗
PubMed1998

Pseudo-Meigs' syndrome caused by paraovarian fibroma.

Meigs' syndrome includes an ovarian tumor, usually fibroma, associated with hydrothorax and ascites. It is accepted that uterine tumors, like fibromas, can also be associated with ascites and hydrothorax, but this is extremely rare. The mechanism of formation of peritoneal and pleural effusion is not well documented. The most likely pathogenesis ascribes the fluid formation to the filtration of interstitial fluid in the peritoneal through the tumor capsule, and the diffusion to the pleural space through the diaphragm lymphatic vessels at the foramen of Bochdalek. Paraovarian fibromas are also extremely rare neoplasms, probably of paramesonephric origin. It has been hypothesised that they can develop by proliferation of connective tissue cells around the Wolfian remnants. In this article, probably for the first time, a case of paraovarian fibroma with ascites and hydrothorax is presented.

Ascites↗
PubMed1998

Hydropic degenerating leiomyoma presenting as pseudo-Meigs syndrome with elevated CA 125.

BACKGROUND: Leiomyomas rarely cause pseudo-Meigs syndrome. Increased levels of CA 125 often are associated with some types of malignancy. No reported case of pseudo-Meigs syndrome presenting with hydropic degeneration of uterine leiomyoma and an elevated CA 125 level could be found on a MEDLINE search. CASE: A 46-year-old woman presented with a pleural effusion and a pelvic mass measuring 30 x 18 cm. Preoperative evaluation was remarkable for a CA 125 level of 254 U/mL. At laparotomy, the diagnosis was a benign leiomyoma with focal hyaline and extensive hydropic degeneration. Her pleural effusion resolved completely by 4 months postoperatively. CONCLUSION: Pseudo-Meigs syndrome can present with an elevated CA 125 level.

CA-125 Antigen↗
PubMed1997

Atypical Meigs' syndrome and bilateral ovarian stromal hyperplasia. A case report.

BACKGROUND: Atypical Meigs' syndrome has been observed in patients with dermoid tumors, struma ovarii, uterine leiomyomata and other benign pelvic tumors except for ovarian fibromas. Meigs' and atypical Meigs' syndrome present management decisions complicated by a high index of suspicion for malignancy. CASE: A 43-year-old woman, gravida 1, para 1, with ascites; a pleural effusion; radiologic evidence of enlarged, cystic adnexa; and a normal CA-125 level was found to have cortical stromal hyperplasia on bilateral ovarian pathologic evaluation. CONCLUSION: This is the first case of cortical stromal hyperplasia presenting with bilateral involvement of small ovaries, ascites and a pleural effusion. Meigs' syndrome and its variants develop with clinical pictures suggestive of malignancy. Thorough evaluation and individualized treatment are necessary.

Adult↗
PubMed2003

Meigs syndrome revisited.

The association of a benign ovarian tumor with ascites and hydrothorax that resolve after tumor resection is known as Meigs syndrome, and its importance was first emphasized by Meigs and Cass in 1937. The importance of Meigs syndrome is that the presence of ascites and pleural effusion does not necessarily indicate that a pelvic mass is malignant. The benign tumors in Meigs syndrome are usually fibromas or fibrothecomas and constitute 4% of all ovarian neoplasms. The authors present a case of Meigs syndrome with an ovarian fibroma. They focus on the evaluation of pleural fluid in the setting of an ovarian mass and then briefly discuss the imaging of ovarian fibromas and fibrothecomas.

Diagnosis, Differential↗
PubMed1996

[Meigs syndrome presenting as diaphragmatic pleural nodules].

A 52-year-old woman was admitted to our hospital because of non-productive coughing and dyspnea on exertion. A chest X-ray film revealed a moderate pleural effusion. The effusion was persistent and progressive in spite of repeated thoracentesis. Because examinations of the effusion did not result in a specific diagnosis, thoracoscopy was done under local anesthesia. Two nodules were found in the surface of the diaphragmatic pleura. Examination of biopsy specimens revealed mesothelial hyperplasia and lymphatic duct proliferation without malignant or granulomatous lesions. Systemic examinations revealed a tumor in the right ovary. The tumor was removed, and examination revealed that it was a serous adenofibroma without malignant lesions. Meigs syndrome was diagnosed. The pleural effusion disappeared quickly, two weeks after the operation. We know of no previous report of diaphragmatic pleural nodules in a case of Meigs' syndrome. Meigs' syndrome should be considered in the differential diagnosis of pleural nodules.

Adenofibroma↗
PubMed2002

Struma ovarii associated with pseudo-Meigs' syndrome and elevated serum CA 125.

BACKGROUND: Struma ovarii, presenting as pseudo-Meigs' syndrome with an elevated CA 125 level, is a rare condition. CASE: A 65-year-old patient presented with ascites, hydrothorax, right ovarian mass, and elevated CA 125 level. These findings were suspicious for an ovarian malignancy. The mass was removed and revealed struma ovarii, a specialized ovarian teratoma composed predominantly of mature thyroid tissue. In the setting of ascites and hydrothorax, the condition is known as pseudo-Meigs' syndrome. This is the second reported case in the English language literature of pseudo-Meigs' syndrome with an elevated CA 125 level resulting from struma ovarii. CONCLUSION: Struma ovarii is a rare cause of ascites, hydrothorax, and an elevated CA 125 level.

Aged↗
PubMed1993

[Meigs syndrome with bilateral hydrothorax].

The co-existence of pelvic tumor, hydrothorax and ascites has been known since the last century. The features of this disease were described by Meigs and Cass in 1937; in the same year Roads named it Meigs syndrome. According to the original description this syndrome only included, as pelvic involvement, an ovarian neoplasm; at present it is accepted that hydrothorax and the ascites can also be associated with a uterine tumor, like a fibroma. The existence of either an ovarian or a uterine neoplasm distinguishes the typical Meigs syndrome from a pseudo-Meigs syndrome. The most likely pathogenesis of Meigs syndrome ascribes the formation of the peritoneal and pleural effusion to the filtration of interstitial fluid in the peritoneum through the tumor capsule, and the diffusion to the pleural space, generally at the right side, through the diaphragm lymphatic vessels and the foramen of Bochdalek. Dockerty reported that at least 40% of ovarian tumors had a diameter of more than 6 cm when associated with hydrothorax and ascites. The entity of pleural and peritoneal effusion can be moderate or massive. The effusions generally derive from a transudative process, but they can occasionally contain blood cells. The connection between the pelvic tumor and the effusion is demonstrated by the regression of the latter when the neoplasm is excised. When the pelvic tumor has an ovarian location it derives from the connective tissue of the hilus, it appears during fertile age and has a slow growth, the clinical signs becoming evident in elder age.

Aged↗
PubMed2005

Ascites, pleural effusion, and CA 125 elevation in an SLE patient, either a Tjalma syndrome or, due to the migrated Filshie clips, a pseudo-Meigs syndrome.

BACKGROUND: The combination ascites, pleural effusion, and elevated CA 125 are usually associated with a malignancy. CASE: A 38-year-old SLE patient consulted her physician for shortness of breath. On clinical examination, she had a tender abdomen and reduced breathing sounds. X-ray and computed tomography of the chest showed pleural effusion. An adjustment of her SLE maintenance therapy was performed. Vaginal ultrasound and computed tomography of the abdomen revealed massive ascites and an intracavitair myoma of 2 cm, but no obvious mass in the pelvis. CA 125 was 887 U/ml. A laparoscopy was performed showing ascites and 2 Filshie clips embedded in the peritoneum of the vesicouterine pouch, but no sign of malignancy. Both clips were removed. The cytology of the aspirated ascites showed sings of acute inflammation. Within 10 weeks, the pleural effusion was resolved and the CA 125 normalized. CONCLUSION: The combination of ascites, pleural effusion, CA 125 elevation, and no tumor in an SLE patient is either a Tjalma syndrome or due to the migrated Filshie clips a pseudo-Meigs syndrome.

Adult↗