Meigs' syndrome mimicking heart failure with preserved ejection fraction: a case report.

BACKGROUND: Meigs' syndrome is a rare disease characterized by a triad of presentations, including benign ovarian tumor, ascites, and pleural effusion. However, a clinical diagnosis of Meigs' syndrome remains challenging because pleural and ascitic effusions can be common findings in a variety of underlying conditions. Furthermore, these findings can often be misdiagnosed as pleural and peritoneal dissemination caused by potentially malignant tumors, leading to the administration of improper treatment. CASE PRESENTATION: We described a case of an 85-year-old postmenopausal female patient with atypical Meigs' syndrome presenting with right-sided pleural effusion, notable leg edema, and trivial ascites, which was initially mistaken as heart failure with preserved ejection fraction. However, pleural effusion was totally ineffective against diuretic therapy. Subsequently, thoracentesis yielded serosanguineous exudative effusion. Moreover, refractory pleural effusions and abdominal/pelvic computed tomography and magnetic resonance imaging findings strongly suggested bilateral malignant ovarian tumors with pleural dissemination. Repetitive negative cytological results allowed the patient to undergo bilateral salpingo-oophorectomy. Finally, a definitive diagnosis of Meigs' syndrome was made by confirming the presence of a benign mitotically active cellular fibroma of the ovary by pathology and that pleural effusion resolved following tumor resection. CONCLUSIONS: Our case highlights the clinical importance of assessing Meigs' syndrome in the diagnostic workup of pleural effusion in postmenopausal female patients. Given the favorable prognosis of Meigs' syndrome, clinicians should consider surgical resection, even with potentially malignant ovarian tumors with accompanying pleural effusion, ascites, or both.

A case of a 20-kg fibrothecoma.

A case report of a 50-cm diameter and 20-kg mass of benign ovarian tumor. Total abdominal hysterectomy with a bilateral salpingooophorectomy was performed with full patient recovery. Fibrothecomas can remain long asymptomatic and can grow to giant sizes.

Síndrome de Meigs y peritonitis esclerosante: una asociación poco frecuente / Meigs’ syndrome and sclerosing peritonitis: an uncommon association

Los tecomas ováricos son tumores estromales relativamente frecuentes que ocasionalmente pueden asociarse con derrame pleural. Los tecomas pueden presentar focos de luteinización y en estos casos se pueden asociar a peritonitis esclerosante. Esta entidad es muy poco frecuente, presenta características histopatológicas propias y clínica heterogénea. Se presenta el caso de una mujer posmenopáusica con dificultad respiratoria y un tumor anexial con sospecha clínica de cáncer ovárico metastásico cuyo estudio anatomopatológico evidenció un tecoma luteinizante con peritonitis esclerosante. Debido a la asociación de una masa ovárica junto a las manifestaciones clínicas que causaba la peritonitis esclerosante es relativamente usual que el diagnóstico de presunción sea de tumor maligno. Todo ello hace importante conocer la entidad, cuya fisiopatología y tratamiento no están consensuados (AU)

Ovarian thecomas are relatively frequent stromal tumors that can occasionally be associated with pleural effusion. Thecomas may contain luteinized foci, and in these cases, can be associated with sclerosing peritonitis. This is a rare, clinically diverse, entity, with its own histopathologic features. We report the case of a postmenopausal woman with respiratory symptoms, bilateral adnexal tumors and suspected metastasizing ovarian cancer, which histopathological study revealed to be a luteinized thecoma associated with sclerosing peritonitis. Due to the clinical manifestations derived from the sclerosing peritonitis and the abdominal mass, a presumptive diagnosis of malignant tumor is quite common. Familiarity with this entity is therefore important. There is no consensus on its physiopathology and treatment (AU)

Silent event-related fMRI reveals deficient motor and enhanced somatosensory activation in orofacial dystonia.

Previous studies showed cortical dysfunction and impaired sensorimotor integration in primary generalized and focal hand dystonia. We used a whistling task and silent event-related fMRI to investigate functional changes in patients with blepharospasm and patients with a combination of blepharospasm and oromandibular dystonia (Meige's syndrome). Whistling served as a model for a skilful orofacial movement with a high demand on sensorimotor integration. It allowed us to study the oromandibular motor system that is clinically affected in Meige's syndrome but not in isolated blepharospasm. In Meige's syndrome, functional MRI revealed deficient activation of the primary motor and ventral premotor cortex within the mouth representation area during whistling. Compared with healthy controls, both forms of orofacial dystonia had increased activation of bilateral somatosensory areas and the caudal supplementary motor area (SMA) in common. While overactivity of somatosensory areas and caudal SMA in Meige patients was partly reversed by botulinum toxin treatment, impaired motor activation was not. We conclude that impaired motor activation appears to be specific for the clinically affected oromandibular motor system in Meige's syndrome while enhanced somatosensory activation is a common abnormality in both forms of orofacial dystonia independent of the affected motor system. Somatosensory overactivity indicates an altered somatosensory representation in orofacial dystonia while impaired motor activation may be a functional correlate of reduced cortical inhibition during oromandibular motor execution in Meige's syndrome.

Síndrome de Meigs: caso clínico y revisión de la literatura / Meigs syndrome: a case presentation and revision of the literature

El Síndrome de Meigs se define como la existencia de ascitis e hidrotórax en asociación con una tumoración ovárica benigna. Es una entidad clínica poco frecuente que se asocia sólo muy ocasionalmente con los de fibromas del ovario. Se presenta el caso de una paciente de 22 años con una historia de dolor abdominal de 3 días de evolución. El estudio clínico y ultrasonográfico revela presencia de masa hipogástrica compleja, ascitis severa, asociada a derrame pleural. El estudio de laboratorio muestra una elevación de CA 125. Es intervenida quirúrgicamente, realizándose extirpación tumoral y remoción de líquido ascítico (13 litros). Biopsia informa fibroma edematoso del ovario izquierdo de 17x14x cm. Evoluciona favorablemente con remisión de la ascitis y del derrame pleural.

[Leptin and its influence on the main gynecoobstetric diseases]. / Leptina y su influencia en los principales padecimientos ginecoobstétricos.

Leptin is a protein hormone synthesized and secreted by adipose tissue and also probably in other organs and systems in human body. It has multiple functions such as to regulate feed intake and energy balance, gonadal regulation, action in the hypothalamo-pituitary-gonadal axis, regulates the metabolism of the fetal-placental unit in the pregnancy, fertility and reproductive systems, actions in the endometrium, mammary gland with corresponding influences on important physiologic processes such as menstruation, pregnancy and lactation. In the gynecologic surgery the serum leptin concentration is also modified. The knowledge of serum leptin concentration in the oncological diseases is going-up. Leptin is modified in the choriocarcinoma, Meigs' syndrome and other tumors. A better understanding of regulatory mechanisms will have direct clinical significance, as leptin has been proposed to impact on those causes of human perinatal morbidity and mortality that are associated with abnormalities of fetal maturity and development, general concept growth, trophoblast endocrinology, and placental sufficiency. Further investigations in this area will be necessary to improve new knowledge and a better understanding of the actions about this hormone.

Chewing pattern in patients with Meige's syndrome.

Meige's syndrome presents with a combination of upper and lower facial motor dysfunction, including eye closing spasms and oromandibular dystonia. While the pathophysiology of eye closing spasms has been extensively studied using the blink reflex and other trigeminofacial reflexes, very few studies have been carried out with regard to the abnormal perioral movements. We hypothesized that action-related dystonic features could be revealed by the analysis of the semiautomatic rhythmic movements required for chewing and swallowing. A total of 7 patients with Meige's syndrome that complained of chewing problems and 7 age-matched healthy volunteers were studied. Subjects were instructed to munch and swallow a small muffin while surface electromyographic (EMG) activity of masseter (MAS) and orbicularis oris (OOr) of the dominant side was recorded. In healthy subjects, MAS and OOr showed a rhythmic alternating phasic EMG pattern during chewing, which changed to a tonic cocontraction of both muscles during swallowing. Mean duration of MAS and OOr EMG bursts was, respectively, 297 +/- 28 msec and 328 +/- 29 msec. Patients exhibited the following alterations: excess duration of muscle activity, frequent cocontraction, loss of rhythmicity during chewing, and abnormalities in the chewing to swallowing transition phase. These abnormalities, similar in type to those encountered in other forms of focal dystonia, may be the expression of an abnormal motor control of basal ganglia over mastication-related movement pattern generators of the brainstem.

[Demons-Meigs' syndrome. Report of a case with ovarian tumor of the granulosa]. / Le syndrome de Demons-Meigs. A propos d'un cas avec tumeur ovarienne de la granulosa.

We report an original and atypical case of Meigs' syndrome with bilateral pleural blood effusion and without ascites in a woman with a rare ovarian tumour involving the stratum granulosum. Excision of the tumour rapidly resulted in the pleural effusion drying up.