Case report: Three cases of systemic lupus erythematosus presenting primarily with massive ascites and significantly elevated CA-125 levels and a review of pseudo-pseudo Meigs' syndrome in literature.

This article presents three detailed case reports and a brief review of the literature on a rare manifestation of systemic lupus erythematosus (SLE) known as Pseudo-Pseudo Meigs' Syndrome (PPMS). The patients' condition was characterized by elevated CA-125 levels, massive ascites andpleural effusion which is typically associated with ovarian malignancies but can also present in various non-malignant conditions, including SLE. A thorough literature review was conducted, summarizing similar cases and their clinical outcomes to provide a broader understanding of this uncommon syndrome. The findings emphasize the need for heightened awareness and consideration of pseudo-pseudo Meigs' syndrome in patients with SLE presenting with unexplained ascites and pleural effusion.

Características clínicas, diagnóstico e tratamento de mulher com síndrome de Meigs: relato de caso e revisão narrativa / Clinical characteristics, diagnosis and treatment of a woman with Meigs syndrome: case report and narrative review

O objetivo deste estudo é descrever o caso de mulher com síndrome de Meigs e apresentar a revisão narrativa sobre o tema. Paciente do sexo feminino, 30 anos, nulípara, encaminhada ao hospital por massa anexial e história prévia de drenagem de derrame pleural. Evoluiu com instabilidade hemodinâmica por derrame pleural hipertensivo à direita, sendo submetida a drenagem torácica, com citologia do líquido negativa. Após, foi submetida a laparotomia: realizada salpingo-ooforectomia esquerda. A congelação e a análise histopatológica diagnosticaram fibroma ovariano. A citologia ascítica foi negativa. CA-125 elevado, presença de derrames cavitários e exame de imagem suspeito podem mimetizar um cenário de neoplasia maligna de ovário em estágio avançado. Entretanto, na síndrome de Meigs clássica, o tratamento é cirúrgico, sendo o diagnóstico obtido por meio da análise histopatológica do tumor ovariano. O manejo da síndrome de Meigs clássica é cirúrgico e, após a remoção do tumor, o derrame pleural e a ascite desaparecem.

To describe a case of Meigs syndrome and present a narrative review of the condition. Female patient, 30 years old, nulliparous, referred to the hospital due to an adnexal mass and a previous drainage of pleural effusion. She developed hemodynamic instability due to a hypertensive right pleural effusion being submitted to chest drainage, with negative cytology of the fluid. She underwent laparotomy: Left salpingo-oophorectomy was performed and frozen section and histopathological analysis diagnosed an ovarian fibroma. Ascites cytology was negative. Elevated CA-125, presence of cavitary effusions, suspicious imaging exam can mimic a scenario of ovarian cancer at an advanced stage. However, in classical Meigs syndrome, treatment is surgical, and the diagnosis is obtained through histopathological analysis of the ovarian tumor. Classical Meigs syndrome' management is surgical. After tumor removal, pleural effusion and ascites resolve.

Pseudo-pseudo Meigs syndrome in systemic lupus erythematosus misdiagnosed as pseudo-Meigs' syndrome: A case report.

Symptoms of pelvic masses, elevated serum CA125 levels, massive ascites, and pleural effusion in female patients are usually associated with malignancy. Some benign ovarian tumors or other nonmalignant tumors may also produce similar symptoms, called Meigs syndrome or pseudo-Meigs' syndrome, which should be one of the differential diagnoses. However, there is an extremely rare form of SLE called pseudo-pseudo Meigs syndrome (PPMS), which may also present with the above symptoms, but is not associated with any of the tumors. In this paper, we report a case of a 47-year-old woman who presented with abdominal distention. The patient was found to have elevated serum CA125 levels to 182.9 U/mL before the operation. Her PET-CT suggested a large heterogeneous mass in the pelvis measuring 8.2 × 5.8 cm with a large amount of ascites. She was initially diagnosed with ovarian cancer and underwent exploratory laparotomy. Pathology of the surgical specimen revealed a uterine leiomyoma. Two months after discharge, the patient's ascites reappeared along with recurrent intestinal obstruction. After ascites and serological tests, she was eventually diagnosed with systemic lupus erythematosus and received systemic hormonal therapy.

Atypical Pseudo-Meigs' Syndrome without Pleural Effusion Due to Ovarian Metastasis from Sigmoid Colon Cancer.

We herein report a case of atypical pseudo-Meigs' syndrome without pleural effusion. A 46-year-old woman was diagnosed with an ovarian tumor and sigmoid colon cancer with massive ascites. She underwent surgical resection of the sigmoid colon and bilateral salpingo-oophorectomy. The pathological diagnosis was sigmoid colon cancer with ovarian metastasis. A few days after the operation, the massive ascites disappeared. Immunostaining for vascular endothelial growth factor (VEGF) suggested its overproduction was involved in the development of the ascites. Although cases of pseudo-Meigs' syndrome without pleural effusion are rare, reporting such cases will facilitate the choice of more appropriate treatment strategies in future.

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.

A case of death of patient with ovarian fibroma combined with Meigs Syndrome and literature review.

Ovarian fibroma is the most common benign pure stromal tumor. It has no specific clinical manifestation, most of which are pelvic or adnexal masses. 10-15% of cases with hydrothorax or ascites, after tumor resection, hydrothorax and ascites disappear, known as Meigs Syndrome. The elevated level of CA125 in a few patients was easily misdiagnosed as ovarian malignant tumor. A case of bilateral Ovarian fibroma associated with Meigs Syndrome is reported and the literature is reviewed in order to improve the understanding of the changes and avoid misdiagnosis.

Meigs syndrome.

BACKGROUND: Meigs syndrome is defined by the presence of a benign ovarian tumor, ascites, and pleural effusion (predominantly on the right side). A characteristic sign of Meigs syndrome is the complete disappearance of exudate after surgical resection of the ovarian tumor. CASE REPORT: We present a case report of a 58-year-old patient admitted for an advanced ovarian tumor with pleural effusion, ascites, and tumor marker elevation typical for ovarian cancer. Cytological examination of ascites and pleural effusion was repeatedly negative for malignancy. Histopathological examination of the bio-psied tissue was concluded as low-grade mesenchymal neoplasia. The second opinion of histopathological examination was concluded as low grade fibroblastic pelvic tumor without the possibility of exact specification. Dia-gnoses of desmoid fibromatosis and low-grade fibromyxiod sarcoma (less likely) were considered. Surgical resection was indicated, and a large tumor with numerous adhesions to the uterus, bladder, and thin loops with a noticeably thickened peritoneum were perioperatively described. Histologically, left ovarian fibroma with productive peritonitis and sanguine-induced ascites was dia-gnosed. Due to the clinical findings and the result of the histopathological examination, the case was classified as Meigs syndrome. Two months after the surgery, the ascites and pleural effusion disappeared, and the tumor marker levels normalized. CONCLUSION: The present case report documents that it is always necessary to consider diseases other than those most likely at the outset, as the treatment algorithm and prognosis of these rare diseases may differ significantly.

Rare cause of ascites and pleural effusion: The first case report and literature review of pseudo-pseudo Meig's syndrome in Taiwan.

Pseudo-pseudo Meigs' syndrome (PPMS) exhibits patients with ascites, pleural effusion, elevated CA-125, and diagnosed of systemic lupus erythematosus (SLE) eventually without evidence of ovarian or pelvic tumor. It's a rare diagnosis but it has a good response to treatment. We here present an 82-year-old female, who was found to have ascites, pleural effusion, and elevated CA-125. CT of abdomen revealed absence of pelvic tumor. However, a rapid decline in renal function and progressive proteinuria were also observed. We performed an autoimmune-associated investigation. A diagnosis of late-onset SLE was made due to meeting the criteria of serositis, hemolytic anemia, thrombocytopenia, renal disease, and positive anti-smith antibody. We gave this patient a regimen with steroids and hydroxychloroquine. Both ascites and pleural effusion resolved in one month. PPMS is an important differential diagnosis in female patients with ascites, pleural effusion, and elevated CA-125. A survey of the pelvic tumor should be done first to exclude Meigs' syndrome or pseudo-Meigs' syndrome. SLE flare-up should be kept in mind even in the elderly.

Pseudo-pseudo Meig's syndrome presenting as an acute surgical abdomen: A rare entity and review of the literature.

AIM: Pseudo-pseudo Meigs' syndrome is a rare entity of systemic lupus erythematosus, which is defined with the combination of pleural effusion, elevated serum CA-125 levels, and ascites. It has similar clinical aspects with gynecological malignancies which may lead gynecologists to perform unnecessary surgeries and lab workouts. This review seeks to point out the importance of diagnosing pseudo-pseudo Meig's syndrome (PPMS) and endeavors to inform gynecologists about the differential diagnoses. METHODS: This article includes a review of the literature on different cases of PPMS. We searched the PubMed database using the search terms in various combinations "Pseudo-pseudo Meig's syndrome," "Tjalma syndrome," and "SLE." RESULTS: According to the current literature, a majority of internal medicine specialists recognized this clinical condition in the past few years and discussed PPMS as a new onset of systemic lupus erythematosus. PPMS is one of the disregarding entities where gynecologists do not consider it as a differential diagnosis in case of less awareness. When a gynecologist evaluates ascites manifesting as acute abdomen; ectopic pregnancy, ovarian hyperstimulation syndrome, cyst rupture, and malignancy come to mind first. Four-thirds of those patients evaluated by gynecologists were performed hysterectomy leading to unnecessary interventions and economic burden on the health system. CONCLUSION: Although the mechanism is unrevealed and remains unclear, PPMS has been noticed in the literature for the past few years. In order to prevent unnecessary interventions, this syndrome should be considered as a differential diagnosis.

A challenging case of pseudo Meigs syndrome: A case report.

Meigs Syndrome is a rare condition characterised by Ovarian fibroma, ascites and pleural effusion. Pseudo Meigs is called so because it mimics Meigs but occurs with tumours other than fibromas. The objective of this case report is to shed light on the diverse presentations of Ovarian carcinomas. We herein report a rare case of Pseudo Meigs syndrome in a 32-year-old female patient parity one and no miscarriage and who had right-sided ovarian mass, gross ascites and right-sided pleural effusion with cancer antigen 125 value of 518.5 IU/L. Clinical Diagnosis was that of Meigs Syndrome. The patient underwent laparotomy for surgical staging and large right-sided ovarian mass with draining of nine litres of ascitic fluid and total abdominal hysterectomy and bilateral salpingo-oophorectomy. The histopathology report showed that it was Endometroid Adenocarcinoma FIGO Grade 3. Definitive diagnosis was that of Pseudo Meigs Syndrome. The case was a diagnostic challenge and difficult to manage. The diverse presentation of ovarian carcinomas makes them difficult to diagnose and clinicians should have a high index of suspicion while managing such cases.

Meigs Syndrome Caused by Ovarian Granulosa Cell Tumor: A Case Report.

BACKGROUND: Ovarian granulosa cell tumor (GCT) is extremely rare in children and adolescents, especially along with Meigs syndrome (MS). CASE: We describe the case of a 12-year-old girl who was referred to our center for massive peritoneal effusions with evidence of a pelvic mass on ultrasonography and computed tomography. The patient was treated with laparoscopic surgery (fertility-sparing surgery) and postoperative chemotherapy. SUMMARY AND CONCLUSION: Our case highlights the clinical importance of assessing MS in the diagnosis of pediatric female patients with peritoneal effusion and ovarian mass and the importance of fertility-sparing surgery.

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.

Meigs' syndrome and adult-type granulosa cell tumor.

OBJECTIVE: Adult-type granulosa cell tumors (GCT) are sex cord-stromal tumors and often accompanied with abdominal distention and hyperestrogenism-related symptoms. Adult-type GCT-presenting ascites and pleural effusion is extremely rare. CASE REPORT: A 56-year-old perimenopausal woman presented with abdominal distention and abnormal vaginal spotting. Ultrasound and abdominal computed tomography showed a complex cystic mass in the left ovary accompanied with bilateral pleural effusion and ascites. The patient underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, left pelvic lymph node dissection, omentectomy and appendectomy. Final histopathological diagnosis was adult-type GCT. The patient had postoperative hormone and anti-angiogenesis agent therapy with free of disease. CONCLUSION: Ovarian cystic complex mass accompanied with ascites and pleural effusion often results from malignant ovarian tumors or benign ovarian fibroma. Based on the aforementioned report, the rare types of ovarian tumors, such as adult-type granulosa cell tumor of the ovary should be taken into consideration.

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.

Ovarian dysgerminoma with pseudo-Meigs syndrome: A case report.

RATIONALE: Dysgerminoma is a rare malignant tumor of the ovary, more frequently occurring in young women. The main signs of pseudo-Meigs syndrome (PMS) are ascites and hydrothorax accompanying benign or malignant ovarian tumors (no fibroma or fibroma-like tumor). PATIENT CONCERNS: A 19-year-old woman with fever and chest tightness for 2 days. DIAGNOSES: Pectoral-abdominal computed tomography (CT) scan and contrast-enhanced magnetic resonance imaging revealed a large amount of right pleural effusion, a small amount of ascites, and a huge abdominopelvic mass measuring about 29.2cm × 11.8cm × 8.4 cm in the left ovary. The result of hydrothorax examination was consistent with the diagnosis of exudative pleural effusion. In addition, Rivalta-test showed a positive result and lactate dehydrogenase was elevated. The histopathological diagnosis was a giant germ cell tumor, which was consistent with dysgerminoma in terms of both morphology and immunophenotype. Based on these findings, a diagnosis of malignant ovarian neoplasm with PMS was made. INTERVENTIONS: Surgical resection of the tumor was performed. OUTCOMES: The patient recovered well after operation, and the pleural effusion and abdominal ascites vanished. No recurrence was observed during the 1-year follow-up period. LESSONS: Ovarian dysgerminoma with PMS is a rare malignant tumor of the ovary, which often occurs in young women. It should be considered in differential diagnosis of patients with a pelvic mass, ascites and pleural effusion. Early diagnosis and surgical treatment are beneficial to prolonged survival.

Pseudo-pseudo Meigs' syndrome (PPMS) in chronic lupus peritonitis: a case report with review of literature.

Gastrointestinal involvement in systemic lupus erythematosus (SLE) usually occurs in the form of mesenteric vasculitis, protein-losing enteropathy, intestinal pseudo-obstruction, and pancreatitis. We describe a 23-year-old female, a known case of SLE presented with significant ascites and pleural effusion. Further evaluation showed elevated CA-125 levels without evidence of malignancy. The patient was treated with corticosteroids, hydroxychloroquine, and azathioprine resulting in the resolution of ascites in 2 weeks. The triad of ascites, pleural effusion, and increased CA-125 is known as pseudo-pseudo Meigs' syndrome, which is rarely reported in the literature. Clinicians should be aware of this entity while evaluating an SLE patient with low serum-ascites albumin gradient (SAAG) ascites.

Meigs syndrome presenting with severely elevated CA-125 level.

A 64-year-old woman referred to Gynaecological Oncology secondary to the finding of pelvic mass and ascites. Imaging showed multiple pelvic masses, with the largest mass measuring 20 cm in diameter, as well as bilateral pleural effusions and abdominal ascites, suspicious for ovarian carcinoma. Laboratory findings included elevated cancer antigen 125 (CA-125) of 2124 units/mL. The patient underwent an exploratory laparotomy, total abdominal hysterectomy and bilateral salpingo-oophorectomy for pathological evaluation. Postoperatively, the patient had resolution of ascites and pleural effusion. Surgical pathology revealed a 26 cm right ovarian fibroma, confirming the diagnosis of Meigs syndrome. Despite the high suspicion for ovarian carcinoma in patients presenting with elevated CA-125 level, pelvic mass, ascites and pleural effusion, the diagnosis of Meigs syndrome cannot be excluded without pathological evaluation of mass.