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.

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.

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.

[A Case of Pseudo-Meigs' Syndrome Caused by Ovarian Metastasis from Colon Cancer].

This case pertains to a female patient in her 60s who was diagnosed with carcinoma in the cecum with lung, ovarian, and peritoneal metastases. She complained of abdominal distension and poor feeding because her ascites and ovarian metastasis worsened 18 months after chemotherapy initiation. Repeated cytologic examination of the ascitic fluid revealed no malignant cells. Therefore, Pseudo-Meigs' syndrome was suspected. Bilateral salpingo-oophorectomy was performed as palliative surgery because of the patient's reduced capacity to perform activities of daily living(ADL)due to ascites. After palliative surgery, her ascites disappeared, and she was able to better perform ADL. Further, chemotherapy was resumed. The patient remains well 10 months after surgery. This case highlights the importance of considering Pseudo-Meigs' syndrome in patients with massive ascites and ovarian metastasis, because surgical resection can improve their quality of life.

Massive Ascites of Unknown Origin: A Case Report.

Ascites is the excessive accumulation of fluid in the peritoneal cavity and predominantly caused by liver cirrhosis, cancers, or heart failure. In this study, a 31-year-old woman with chronic renal failure of unknown etiology treated with hemodialysis and peritoneal dialysis was often hospitalized because of ascites, which appeared 4 years after the second kidney transplantation. The patient was regularly (every 2-3 weeks) treated with paracentesis. Peritoneal fluid tested negative for bacterial (including atypical) and fungal infections and tuberculosis. Doppler ultrasound and liver FibroScan did not show any irregularities. Computed tomography (CT) revealed an enlarged left ovary. A high level of CA 125 was found. The second diagnostic laparoscopy revealed no changes in the ovaries, and there were no tumor cells. Diagnostics were extended, but no deviations were revealed. Suspecting drug etiology, mycophenolic acid was discontinued, bringing no improvement. Diagnostic tests caused suspicion of Meigs' syndrome; therefore, oophorectomy of left ovary was conducted, revealing numerous small cysts filled with serous fluid, without tumor cells in the ovary or peritoneal fluid. Despite the procedure performed, ascites was recurrent. Five month later, ascites spontaneously stopped growing. Paracentesis to decompress ascites was no longer required. There were 9 paracenteses performed from oophorectomy (the latest on May 23, 2019). The need for repetitive paracentesis, significantly reducing the patient's quality of life, required diagnosis for casuistic diseases. The described case is atypical because of the confusing etiology of ascites and its spontaneous cessation. Despite numerous examinations and recession of ascites, the cause of the problem is not entirely clear.

Pseudo-Meigs Syndrome Caused by a Giant Uterine Leiomyoma with Cystic Degeneration: A Case Report.

Pseudo-Meigs syndrome is defined as secondary accumulation of ascites and hydrothorax associated with a pelvic tumor other than benign ovarian tumors such as fibroma, which usually resolve after surgical removal of the tumor. Here we report a case of pseudo-Meigs syndrome caused by a giant uterine leiomyoma, which was initially suspected to be ovarian cancer. A 37-year-old nulliparous woman presented with a 5-month history of abdominal distension and anorexia. Abdominal ultrasonography revealed a giant cystic lesion and solid mass in the peritoneal cavity, along with plentiful ascites. Chest X-ray images showed a small pleural effusion on the right side. The patient was referred to our hospital for treatment of suspected ovarian cancer and peritonitis carcinomatosis. Although serum CA125 level was elevated (up to 331.8 U/mL), magnetic resonance imaging showed a giant sub-serosal uterine leiomyoma with cystic degeneration (27 × 15 × 13 cm). A small dermoid cyst was also detected in the right ovary. Ascites was drained and the patient underwent myomectomy and ovarian cystectomy. The patient had a degenerated leiomyoma with no pathological evidence of malignancy. Because symptoms disappeared postoperatively and serum CA125 returned to normal, without recurrence of ascites, pseudo-Meigs syndrome was diagnosed.

Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil / Tjalma syndrome (pseudo-pseudo Meigs') as initial manifestation of juvenile-onset systemic lupus erythematosus

El síndrome de Tjalma o pseudo-pseudo Meigs es una entidad clínica que se presenta con derrame pleural, ascitis y elevación de CA-125 sin asociación a tumor ovárico benigno o maligno en un paciente con lupus eritematoso sistémico (LES). Tjalma describió el primer caso de un paciente con LES, ascitis, derrame pleural y elevación de CA-125. Presentamos el primer caso en una paciente pediátrica de 14 años, que se presentó con ascitis y derrame pleural refractarios a tratamiento con elevación de CA-125, sin encontrar tumor ovárico, que ameritó manejo agresivo

Tjalma syndrome or pseudo-pseudo Meigs' syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management

[Pseudo-Meigs syndrome secondary to ovarian serous cystadenoma]. / Pseudo-syndrome de Demons Meigs secondaire à un cystadénome séreux de l'ovaire: cas clinique.

Pseudo-Meigs syndrome combines a benign (all histological types are included) or malignant (primitive ovarian tumor or ovarian metastasis from another primitive tumor) ovarian tumor or a pelvic tumor (not necessarily ovarian or uterine, for example) with ascites and pleurisy (non-metastatic in the case of malignant tumor). These effusions disappear after tumor resection. A 37-year old female patient was admitted to our Department with dyspnoea and left intercostal pain. Radiological examinations showed left pleurisy of average abundance, ascites of low abundance and a pelvic mass. Surgical exploration showed ovarian tumor. After ablation, pleurisy solved spontaneously. Of particular interest, with regard to pneumology, is that this syndrome has occurred in a woman with pleurisy whose etiological assessment was negative and that abdominopelvic ultrasound allows diagnostic orientation.

Pseudo-pseudo Meigs' syndrome: a rare presentation of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic inflammatory multisystem autoimmune disease. Ascites when associated with pleural effusion and raised CA-125 levels in SLE patient, is known as pseudo-pseudo Meigs' syndrome (PPMS). This is the case of a 22-year-old lady who presented with complaints of abdominal distension for one month and had a history of spontaneous abortion in the past. Abdominal imaging did not reveal any tumor and after extensive workup a diagnosis of PPMS was made. She was successfully treated with steroids, hydroxychloroquine and cyclophosphamide.

Tjalma syndrome (pseudo-pseudo Meigs') as initial manifestation of juvenile-onset systemic lupus erythematosus. / Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil.

Tjalma syndrome or pseudo-pseudo Meigs' syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management.

A Challenging Case of Intracardiac Leiomyomatosis Accompanied by Pseudo-Meigs Syndrome Originating from Uterine Leiomyoma.

BACKGROUND: Pseudo-Meigs syndrome is an unusual condition involving pelvic tumors, excluding ovarian fibroma, and is associated with ascites and hydrothorax. Pseudo-Meigs syndrome originating from uterine leiomyoma is an even rarer condition. Intravenous leiomyomatosis originating from uterine tumors, which extend from the inferior vena cava to the right heart chambers, is also extremely rare. CASE REPORT: This report presents an extremely rare case of intravenous leiomyomatosis with intracardiac extension accompanied by Pseudo-Meigs syndrome caused by uterine leiomyoma. To the best of our knowledge, this is the first reported case of two different rare conditions originating simultaneously from one common pelvic benign tumor. We could only partially resect the tumor owing to its firm adherence to the vessel wall. It is crucial to remember that a pelvic neoplasm in a woman with various manifestations, such as hydrothorax, ascites, intravascular leiomyomatosis, intracardiac mass, and even elevated CA125 levels, does not always imply malignancy. All the accompanying symptoms can be resolved by simple surgical removal of the mass. CONCLUSIONS: Accurate preoperative assessment and detailed preparation for the surgery according to the nature of the tumor may yield successful results.

Long-term survival in pseudo-Meigs' syndrome caused by ovarian metastases from colon cancer.

BACKGROUND: Meigs' syndrome is defined as the co-existence of benign ovarian fibroma or fibroma-like tumor, ascites, and pleural effusion. In contrast, pseudo-Meigs' syndrome is defined as the co-existence of other ovarian or pelvic tumors, ascites, and pleural effusion. In Meigs' and pseudo-Meigs' syndromes, ascites and pleural effusion resolve promptly after the complete resection of the ovarian or pelvic tumor(s). Secondary ovarian tumors from colorectal gastrointestinal metastases rarely cause pseudo-Meigs' syndrome; only 11 cases of pseudo-Meigs' syndrome secondary to colorectal cancers have been reported in the literature. Therefore, the prognosis and etiology of pseudo-Meigs' syndrome caused by ovarian metastasis from colorectal cancers remain unclear. CASE PRESENTATION: We report here a rare case of pseudo-Meigs' syndrome caused by ovarian metastases from sigmoid colon cancer with long-term survival. A 47-year-old woman presented with abdominal distention of 1-month duration. She developed acute dyspnea 2 weeks after the initial presentation. Colonoscopy and computed tomography revealed sigmoid colon cancer with an ovarian metastasis, along with massive ascites and bilateral pleural effusion. Emergency operation, including bilateral oophorectomy and sigmoidectomy, was performed. Subsequently, ascites and bilateral pleural effusion resolved rapidly. Curative hepatic resection was performed for liver metastases 29 months after the first operation, and as of this writing, the patient is alive with no evidence of a disease 78 months after the first operation. In general, colorectal cancer with ovarian metastasis is hard to cure, and long-term survival in patients with colorectal cancer with pseudo-Meigs' syndrome is rare. Our experience suggests that curative resection for pseudo-Meigs' syndrome caused by ovarian metastasis from colorectal cancer may offer long-term survival. CONCLUSIONS: Our experience suggests that pseudo-Meigs' syndrome can occur in a patient with colorectal cancer after metastasis to the ovaries, causing massive ascites and pleural effusion. Aggressive treatment, including R0 resection, for this disease if allowed by the patient's general condition may offer long-term survival.

[Meigs' syndrome caused by bilateral ovarian fibroma mimicking ovarian cancer]. / Síndrome de Meigs por fibroma ovárico bilateral parecido al cáncer de ovario.

CASE REPORT: We report the case of a 55-year-old patient who pre- sented a pelvic mass, ascites and elevated serum CA125. Suspecting a malignant process she underwent surgery and a total hysterectomy with bilateral salpigo-oforectomy was performed. Pathologic report revealed a bilateral ovarian fibroma and non-tumoral ascites. The presence of elevated serum CA125 levels in a postmenopausal woman with a pelvic mass and ascites suggest an ovarian malignant disease. However, in case of Meigs'syndrome, all symptoms will diappear after removal of the pelvic tumor, so a fast surgical management of the patients is mandatory.

Pseudo-Meigs' syndrome secondary to metachronous ovarian metastases from transverse colon cancer.

Pseudo-Meigs' syndrome associated with colorectal cancer is extremely rare. We report here a case of pseudo-Meigs' syndrome secondary to metachronous ovarian metastases from colon cancer. A 65-year-old female with a history of surgery for transverse colon cancer and peritoneal dissemination suffered from metachronous ovarian metastases during treatment with systemic chemotherapy. At first, neither ascites nor pleural effusion was observed, but she later complained of progressive abdominal distention and dyspnea caused by rapidly increasing ascites and pleural effusion and rapidly enlarging ovarian metastases. Abdominocenteses were repeated, and cytological examinations of the fluids were all negative for malignant cells. We suspected pseudo-Meigs' syndrome, and bilateral oophorectomies were performed after thorough informed consent. The patient's postoperative condition improved rapidly after surgery. We conclude that pseudo-Meigs' syndrome should be included in the differential diagnosis of massive or rapidly increasing ascites and pleural effusion associated with large or rapidly enlarging ovarian tumors.

Luteinized Thecomas ("Thecomatosis") with Sclerosing Peritonitis (LTSP): Report of 2 Cases and Review of an Enigmatic Syndrome Associated with a Peritoneal Proliferation of Specialized (vimentin+/keratin+/CD34+) Submesothelial Fibroblasts.

OBJECTIVE: To present the clinicopathologic features of two cases of luteinized thecomas with sclerosing peritonitis (LTSP), characterize the cellular proliferation in the sclerosing peritonitis (SP), and review the literature. METHODS: The clinical, laboratory, and imaging data, operative findings, and pathology materials were reviewed and summarized. Samples of the SP were stained with keratin AE1/AE3, vimentin, CD34, calretinin, smooth muscle actin, ER/PR, CD10 and desmin. A literature search was performed to identify cases of LTSP for comparison. RESULTS: A total of 43 cases of LTSP syndrome were identified. Frequent clinical features included ascites (74%), abdominal pain (35%), bowel obstruction (42%), and bilateral masses (84%). We isolated a distinct form of ovarian luteinized thecoma (thecomatosis) and peculiar sclerosing peritonitis (SP). IHC analysis shows a proliferation of specialized (vimentin+/keratin+/CD34+) submesothelial fibroblasts (SMF) with patchy expression of calretinin and hormone receptors. CONCLUSION: LTSP syndrome is a rare entity presenting with abdominal pain, bowel obstruction, ascites, ovarian masses, and SP containing specialized (vimentin+/keratin+/CD34+) SMF. LTSP must be distinguished from abdominal cocoon, isolated SP, Meigs' syndrome, and peritoneal carcinomatosis. The importance of recognizing the diagnosis is stressed, as failure to manage this disease conservatively leads to significant morbidity and mortality. The SP and bowel obstruction may persist for months, even after resection of the tumours, resulting in extended medical therapy. Based on the immunophenotype of the peritoneal lesions, strategies to elucidate 'targeted' pharmacologic agents that could inhibit the proliferation of specialized (vimentin+/keratin+/CD34+) SMF may be of benefit.

The pleural effusion in ovarian tumors: Often a diagnosis challenge.

Pleural effusions associated with ovarian tumors are not always malignant. Neoplastic etiology of pleural efussion needs histopathological confirmation. We present three cases that illustrate various etiologies for pleural effusions in patients with ovarian tumors: thromboembolism, malignancy and Meigs syndrome. For these patients, it is essential to establish the correct etiology of the pleurisy, since it may change the therapeutic approach. All the cases must be carefully assessed and all the efforts must be done by a multidisciplinary team in order to offer the best solution for each case.