Struma ovarii with synchronous ascites and elevated CA125 level: a retrospective cohort study.

BACKGROUND: Benign struma ovarii (SO) with synchronous ascites and elevated CA125 level is extremely rare that the incidence, clinical characteristics, and risk factors remain unclear. METHODS: We conducted a retrospective study of patients with SO treated in our hospital between 1980 and 2022. Logistic regression was used to identify potential risk factors for SO patients presenting with ascites and elevated CA125 levels. The receiver operating characteristic (ROC) curve was used to evaluate the predictive performance of the identified risk factors. RESULTS: A total of 21 patients with synchronous ascites and elevated CA125 levels were identified in 229 patients with SO, the crude incidence rate was 9.17%, and four patients (1.75%) had pseudo-Meigs' syndrome. Ascites were completely involuted within 1 month postoperatively and the serum CA125 level decreased to normal between 3 d and 6 weeks after surgery. Multivariate logistic regression showed that age ≥49 years (OR 3.71, 95% CI 1.29 - 10.64, p = 0.015), tumor size ≥10.0 cm (OR 8.79, 95% CI 3.05 - 25.35, p < 0.001), and proliferative SO (OR 11.16, 95% CI 3.01 - 41.47, p < 0.001) were the independent risk factors for patients presenting ascites and elevated CA 125 level. The ROC curve revealed that the predictive performance for age and tumor size was unsatisfactory with an area under the curve (AUC) was 0.646 and 0.682, respectively. Linear regression demonstrated that the serum CA125 level has a moderate positive correlation with the volume of ascites (log2CA125 = 0.6272*log2ascites + 2.099, p = 0.0001, R2 = 0.5576). CONCLUSIONS: Less than one-tenth of patients with SO would present ascites and elevated CA125 levels, while age ≥49 years, tumor sizes ≥10 cm, and the presence of proliferative SO were the risk factors.

Struma ovarii with massive ascites mimicking ovarian carcinoma treated with conservative laparoscopic surgery: a case report.

Struma ovarii is a rare taratoma that accounts for 0.5-1% of all ovarian tumors. It is sometimes difficult to differentiate struma ovarii from ovarian carcinoma. We encountered a case of struma ovarii that was suspected to be malignant due to the accumulation of massive ascites and an elevated CA125 level. It was successfully treated with laparoscopic surgery.A 37-year-old nulliparous woman consulted a local physician with a chief complaint of abdominal distention. Computed tomography (CT) of the abdomen revealed a pelvic tumor with a large amount of ascites. She was referred to our department. Contrast-enhanced magnetic resonance imaging (MRI) and CT showed bilateral ovarian tumors with multicystic and solid components. CA125 level was markedly elevated. Two cytological examinations of ascites showed no malignant cells. Preoperatively, malignancy was strongly suspected, but considering the possibility of a benign ovarian tumor, laparoscopic surgery was scheduled. During laparoscopic surgery, 4,850 mL of ascites were aspirated, and the left adnexa was removed. Intraoperative rapid pathology suggested struma ovarii with no evidence of malignancy. Postoperative pathology showed mature teratoma and struma ovarii.Although struma ovarii is benign in 90-95% of cases, there have been scattered case reports in which suspected malignancy led to unnecessary or excessive surgery. We propose that appropriate preoperative imaging and accurate intraoperative rapid pathology can prevent excessive surgery, conservative or laparoscopic excisions should be considered.

Deciphering the puzzle: a case report of Tjalma syndrome (pseudo-pseudo Meigs' syndrome) with profoundly elevated CA-125 and pleural effusion.

Elevated CA-125 levels, polyserous effusions (such as pleural effusion, ascites, etc.) in young women with systemic lupus erythematosus (SLE) may signal pseudo-pseudo Meigs' syndrome (PPMS), after excluding other causes. We describe a 32-year-old SLE patient with recurrent bilateral pleural effusions and unexplained hypercalcemia for 10 months. Extensive evaluations revealed no infections or tumors. Cytokine analysis showed elevated interleukin (IL) levels, especially IL-6 in pleural effusion. Treatment with immunosuppressive therapy resulted in reduced cancer antigen (CA) 125 levels and decreased effusion volume, demonstrating a positive response to intervention in this case of PPMS.

Early diagnosis of Meigs syndrome in children A case report and a review of the literature.

Meigs syndrome is a rare disease defined by the coexistence of benign ovarian neoplasm, ascites and hydrothorax, which mainly affects women over the age of 30. This clinical condition refers only to cases in which the ovarian neoformation is a fibroid, a thecoma, a granulosa cell tumor or a Brenner tumor with disappearance of symptoms and effusions after removal of the neoplasm. Meigs syndrome is most frequently characterized by the presence of an ovarian fibroid, which in childhood is very rare and not commonly associated with the disease. In this article we report the case of an 11- year-old girl who came to our observation for a high fever for five days accompanied by cough and abdominal pain; imaging methods revealed bilateral hydrothorax, ascites, and a voluminous expansive right ovarian formation. On histological examination, the mass showed a cellular fibroid and the diagnosis of Meigs syndrome was made. Furthermore, we present a review of the literature aimed at detecting the state of knowledge on this disease in pediatric age, giving particular emphasis to the condition for which, in the presence of pleural effusion and ascites, an ovarian neoformation is not necessarily malignant. KEY WORDS: CT, Meigs syndrome, Pediatric, Pelvic mass, Ultrasounds.

Giant Ovarian Thecoma Associated with Meigs Syndrome: A Striking Case.

Introduction: Meigs syndrome consists of the presence of a benign ovarian tumor, ascites and pleural effusion, and the latter two subdued after surgical excision of the ovarian tumor. Elevated Ca-125 in this context is confusing and is reported only in a handful of cases. A rare but striking case with the above features is presented herein. Case Presentation: A 46-year-old woman with a giant pelvic/abdominal mass originating from her right adnexa, ascites and pleural effusion, with elevated Ca-125 (938 IU/mL) was treated with the presumptive diagnosis of stage IV ovarian cancer. Imaging modalities showed a 22 cm solid adnexal mass and the patient underwent total abdominal hysterectomy and bilateral salpigooophorectomy, omentectomy and drainage of 4L of ascetic fluid. Surprisingly, final histopathology was negative for malignancy, characterizing the primary tumor as ovarian thecoma. Ascites and pleural effusion resolved by the seventh postoperative day, setting the diagnosis of Meigs syndrome. Discussion: Meigs syndrome accounts for 1% of all ovarian tumors, however it should be considered in the differential diagnosis when clinicians come across the classic triad of the syndrome, even when Ca-125 is elevated. These patients have normal life expectancy with meticulous management, while pathophysiology of this condition remains uncertain in various points.

Obstetric and gynecologic causes of pleural effusions.

Obstetric and gynecologic pleural effusions may occur in the setting of different diseases and conditions, early and appropriate recognition of the different etiologies of these effusions will aid in appropriate treatment management. In this paper we will give an overview of the different pleural effusion etiologies that may be encountered including catamenial hemothorax, ovarian hyperstimulation syndrome, the different Meigs' syndromes and benign peripartum pleural effusion.

A case of Meigs' syndrome with preceding pericardial effusion in advance of pleural effusion.

BACKGROUND: Meigs' syndrome is defined as the presence of a benign ovarian tumor with pleural effusion and ascites that resolve after removal of the tumor. The pathogenesis of the production of ascites and pleural effusion in this syndrome remains unknown. Aside from pleural effusion and ascites, pericardial effusion is rarely observed in Meigs' syndrome. Here, we report the first case of Meigs' syndrome with preceding pericardial effusion in advance of pleural effusion. CASE PRESENTATION: An 84-year-old Japanese non-smoking woman with a history of lung cancer, treated by surgery, was admitted due to gradual worsening of dyspnea that had occurred over the previous month. She had asymptomatic and unchanging pericardial effusion and a pelvic mass, which had been detected 3 and 11 years previously, respectively. The patient was radiologically followed-up without the need for treatment. Two months before admission, the patient underwent a right upper lobectomy for localized lung adenocarcinoma and intraoperative pericardial fenestration confirmed that the pericardial effusion was not malignant. However, she began to experience dyspnea on exertion leading to admission. A chest, abdomen, and pelvis computed tomography scan confirmed the presence of right-sided pleural and pericardial effusion and ascites with a left ovarian mass. Repeated thoracentesis produced cultures that were negative for any microorganism and no malignant cells were detected in the pleural effusions. Pleural fluid accumulation persisted despite a tube thoracostomy for pleural effusion drainage. With a suspicion of Meigs' syndrome, the patient underwent surgical resection of the ovarian mass and histopathological examination of the resected mass showed ovarian fibroma. Pleural and pericardial effusion as well as ascites resolved after tumor resection, confirming a diagnosis of Meigs' syndrome. This clinical course suggests a strong association between pericardial effusion and ovarian fibroma, as well as pleural and peritoneal fluid. CONCLUSIONS: In female patients with unexplained pericardial effusion and an ovarian tumor, clinicians should consider the possibility of Meigs' syndrome. Although a malignant disease should be suspected in all patients with undiagnosed pleural and/or pericardial effusion, Meigs' syndrome is curable by tumor resection and should be differentiated from malignancy.

Enormous ovarian fibroma with elevated Ca-125 associated with Meigs' syndrome. Presentation of a rare case.

In medicine, Meigs' syndrome is the triad of ascites, pleural effusion, and benign ovarian tumor (fibroma, Brenner tumour, and occasionally granulosa cell tumour). It resolves after the resection of the tumor. Because the transdiaphragmatic lymphatic channels are larger in diameter on the right, the pleural effusion is classically on the right side. The etiologies of the ascites and pleural effusion are poorly understood. Atypical Meigs' syndrome,characterized by a benign pelvic mass with right-sided pleural effusion but without ascites, can also occur. As in Meigs syndrome, pleural effusion resolves after removal of the pelvic mass. The authors would like to share their own experience of a case of Meigs' syndrome associated with an enormous ovarian fibroma and elevated Ca-125.

Meigs Syndrome Superimposed on Gorlin Syndrome in a 14-Year-Old Girl.

BACKGROUND: Meigs syndrome is a rare complication associated with ovarian fibromas. Although ovarian fibromas are rare in children, they are common in women with Gorlin syndrome after puberty. CASE: A 14-year-old girl with Gorlin syndrome was admitted to our hospital for ablation of basal cell carcinoma. A chest x-ray revealed pleural effusion. Ultrasonography revealed bilateral multinodular ovarian masses. Meigs syndrome associated with ovarian fibromas was considered. A laparotomy revealed bilateral ovarian masses, which were resected. Microscopically, the masses were composed of mitotically active fibroma and areas resembling hemangiopericytoma and luteinized thecoma. The pleural effusion disappeared soon after the surgery. SUMMARY AND CONCLUSION: Physicians should consider the possibility that pleural effusion might precede the diagnosis of ovarian fibroma in patients with Gorlin syndrome.

Pleural Effusion in Meigs' Syndrome-Transudate or Exudate?: Systematic Review of the Literature.

Although Meigs' syndrome is regarded as a well-defined entity, contradictory data on pleural fluid characteristics have been presented, with some papers classifying it as a transudate, whereas others stating that it is an exudate.The aims of the study were: (1) to evaluate pleural fluid characteristics in patients with Meigs' syndrome and (2) to analyze the prevalence of transudative and exudative pleural effusion in relation to the applied definition of the syndrome.We performed a search through medical databases (MEDLINE, EMBASE, SCOPUS, and GOOGLE SCHOLAR) to identify papers on Meigs' syndrome published between 1940 and 2013. Two authors independently reviewed each paper searching for prespecified data: (1) signs and symptoms, (2) tumor characteristics, (3) clinical and laboratory data on ascites, (4) clinical, radiological, and laboratory data on pleural fluid, (5) clinical course after tumor removal. All case reports were reclassified according to a new unequivocal classification of Meigs' syndrome-related entities.A total of 653 papers were initially identified, and 454 articles reporting 541 patients were included in the final analysis. After reclassification according to our case definitions, there were 196, 113, and 108 patients defined as classic Meigs' syndrome, nonclassic Meigs' syndrome, and pseudo-Meigs' syndrome, respectively. Significantly more patients presented with right-sided than left-sided and bilateral pleural effusions (P < 0.001). Median volume of withdrawn pleural fluid was 2950 (1500-6000) mL. The classification of pleural effusion with the use of Light's criteria was possible in only 7 patients. In 6 of these patients pleural effusion met the criteria for an exudate. When the protein concentration > 3.0 g/dL was applied as a criterion of pleural exudate, 88.8% (80/90) of effusions were classified as exudates. Increasing the cut-off level to 3.5 g/dL resulted in only a modest decrease in the percentage of exudative effusions (81%, 73/90).Surprisingly few reports on Meigs' syndrome present data reliably defining the character of pleural effusion. The available data indicate, however, that the majority of pleural effusions in patients with this entity are exudates. This finding may be a prerequisite for the verification of some earlier presented concepts.

METASTATIC RADIOIODINE AVID STRUMA OVARII ASSOCIATED WITH PSEUDO-MEIGS' SYNDROME.

We report a case of 21 years old lady who presented with ascites, left adnexal mass and elevated CA-125. With suspicion of ovarian malignancy, she underwent left salpingo-oophorectomy with omental biopsy. Histopathology revealed: 'follicular variant of papillary thyroid carcinoma arising in struma ovarii' with metastatic papillary thyroid carcinoma in omental and peritoneal nodules. Patient underwent total thyroidectomy followed by radioactive iodine therapy for metastatic omental and peritoneal disease. Post-therapy whole body scan, revealed extensive I-131 avid disease metastatic disease involving the chest, abdomen, pelvis and the musculoskeletal system. Patient was treated with multiple doses of high dose radioactive iodine. She became symptom free on supra-physiologic doses of oral thyroxin however her high thyroglobulin levels and residual radioiodine avid metastatic disease required further treatment. In literature a few cases of struma ovarii have been reported with elevated CA-125 and associated pseudo-Meigs' syndrome. The treatment for this rare disease is still not standardized and poses a therapeutic challenge. Our case emphasizes the need for a multidisciplinary approach for managing struma ovarii.

Large twisted ovarian fibroma associated with Meigs' syndrome, abdominal pain and severe anemia treated by laparoscopic surgery.

BACKGROUND: The Meigs' syndrome is a rare but well-known syndrome defined as the triad of benign solid ovarian tumor, ascites, and pleural effusion. Meigs' syndrome always requires surgical treatment. However, the optimal approach for its management has not been sufficiently investigated. CASE PRESENTATION: We report a patient with a large twisted ovarian fibroma associated with Meigs' syndrome, abdominal pain and severe hemolytic anemia that was treated by laparoscopic surgery. This case highlights the difficulties that may be encountered in the management of patients with Meigs' syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach and the adverse impact that Meigs' syndrome can have on the patient's condition, especially if it is associated with acute pain and severe anemia. Considering the patient's serious clinical condition and assuming that she had Meigs' syndrome with a twisted large ovarian mass and possible hemolytic anemia, we first concentrated on effective medical management of our patient and chose the most appropriate surgical treatment after laparoscopic examination. The main aim of our initial approach was preoperative management of the anemia. Blood transfusions and glucocorticoid therapy resulted in stabilization of the hemoglobin level and normalization of the bilirubin levels, which confirmed the appropriateness of this approach. Laparoscopic surgery 4 days after admission enabled definitive diagnosis of the tumor, confirmed torsion and removed the bulky ovarian fibroma, resulting in timely resolution of symptoms, short hospitalization, relatively low morbidity and a rapid return to her social and professional life. CONCLUSIONS: This case highlights the difficulties that may be encountered in the management of patients with Meigs' syndrome, including potential misdiagnosis of the tumor as a malignant ovarian neoplasm that may influence the medical and surgical approach, and the adverse impact that Meigs' syndrome can have on the patient's condition, especially if it is associated with acute pain and severe anemia. The present case suggests that laparoscopic surgery for potentially large malignant tumors is feasible and safe, but requires an appropriate medical and gynecological oncology expertise.

[Sclerosing stromal tumor of the ovary associated with a Meigs' syndrome and pregnancy: a case report]. / Tumor estromal esclerosante de ovário associado à síndrome de Meigs e gestação: relato de caso.

The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.

Tumor estromal esclerosante de ovário associado à síndrome de Meigs e gestação: relato de caso / Sclerosing stromal tumor of the ovary associated with a Meigs' syndrome and pregnancy: a case report

O tumor estromal esclerosante de ovário é uma neoplasia benigna extremamente rara, mais frequente em mulheres jovens e sem sintomas específicos na maioria dos casos. Menos de 150 casos foram descritos, dos quais 8 diagnosticados durante a gestação. Neste relato, documentamos a associação entre tumor estromal esclerosante de ovário, síndrome de Meigs e elevação dos níveis de CA-125 em gravidez a termo.

The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.

Borderline mucinous ovarian tumor presenting as pseudo-Meigs' syndrome.

Borderline ovarian tumor with the initial presentation of pseudo-Meigs' syndrome is rare. A 52-year-old postmenopausal woman presented with a large ovarian tumor, ascites, and right hydrothorax. We found elevated serum carcinoembryonic antigens (44.4 ng/mL), carbohydrate antigen (CA)-125 (269.8 U/mL), and CA-199 (7942 U/mL). The frozen section pathology revealed a mucinous borderline ovarian tumor, and a staging operation was performed. Final pathologic examination confirmed the diagnosis of intestinal type ovarian mucinous borderline tumor with non-invasive cul-de-sac implants. Her pleural effusion and ascites resolved after surgery, and she remained tumor-free after 3 years' follow up. Physicians should be cautious for the rare possibility of pseudo-Meigs' syndrome in patients with pelvic tumors having the features of advanced ovarian cancer.

Meigs' syndrome: a rare cause of recurrent pleural effusion in scleroderma.

Meigs' syndrome represents a triad of pleural effusion, ascites, and an ovarian tumor, usually benign, occurring together. We describe here a case of Meigs' syndrome in a patient with systemic sclerosis, the first such report to our knowledge, in systemic sclerosis. A 53-year-old woman with systemic sclerosis presented with recurrent right-sided pleural effusion, which led to symptoms of shortness of breath, chest tightness, and a non-productive cough. Physical examination revealed a palpable, mobile mass in the right lower quadrant, in addition to typical physical features of scleroderma. Thoracentesis yielded exudative pleural fluid with cytology negative for malignancy. Pleural biopsy was consistent with inflammatory changes, but negative for malignancy. CT scan of the chest, abdomen, and pelvis revealed a soft tissue mass in the pelvis, which appeared to arise from the left ovary. The patient's cancer antigen 125 (CA-125) level was elevated at 222 U/mL (normal range, 0-30 U/mL). The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histology of the left ovarian mass was consistent with an ovarian fibrothecoma, a benign tumor of the ovary. At her 1-month follow-up appointment, the patient had complete resolution of the right-sided pleural effusion. To date, at 10 months past the initial presentation, she has not had recurrence of pleural effusion. Although rare, Meigs' syndrome should be considered as a possible cause of recurrent serositis in women with rheumatologic diseases. Removal of the ovarian tumor leads to prompt resolution of the serositis.