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篇名
婦產科-梅格斯症候群,臨床醫學教室
重要考點
梅格斯症候群指卵巢纖維瘤伴發腹水與胸腔積液的患者,在腫瘤切除後,胸腔積液和腹水可以消失的一組症候群。
說明
(前言)

梅格斯症候群指卵巢纖維瘤(fibroma)伴發腹水與胸腔積液的患者,在腫瘤切除後,胸腔積液和腹水可以消失的一組症候群(Meigs syndrome is the triad of ascites, pleural effusion and benign ovarian fibroma. It resolves after the resection of the tumor)。卵巢纖維瘤伴梅格斯症候群者年齡多數在40~60歲高社經階層者(Ovarian tumors are more prevalent in upper socioeconomic groups)。根據研究,腫瘤的大小是出現腹水的最重要因素(Tumor size, rather than the specific histologic type, is thought to be the important factor in the formation of ascites),其超過10cm的纖維瘤約有10~15%會合併腹水(Samanth and Black studied ovarian tumors accompanied by ascites and found that only tumors larger than 10 cm in diameter with a myxoid component to the stroma are associated with ascites),胸腔積液約佔1%,有時會造成臨床上誤判成惡性腫瘤的可能,但這些症狀都會在移除腫瘤後消失。Meigs syndrome也可以在其他卵巢腫瘤發現,如卵泡腺細胞瘤、卵巢甲狀腺瘤、顆粒細胞瘤。

(歷史)
1879年 Cullingworth 首先報導
1934年 Salmon 描述本症是胸腔積液伴有盆腔內良性腫瘤(Salmon described the association of pleural effusion with benign pelvic tumors)
1937年 Meigs and Cass 詳細描述7 例卵巢纖維瘤伴有胸腔積液和腹水而被命名為梅格斯症候群(Meigs and Cass described 7 cases of ovarian fibromas associated with ascites and pleural effusion)
1954年 Meigs 正式定義梅格斯症候群(true Meigs syndrome:benign and solid ovarian tumors accompanied by ascites and pleural effusion, with the condition that removal of the tumor cures the patient without recurrence)

(註)Pseudo-Meigs syndrome:consists of pleural effusion, ascites, and benign tumors of the ovary other than fibromas. These benign tumors include those of the fallopian tube or uterus and mature teratomas, struma ovarii, and ovarian leiomyomas。

(病理)

卵巢纖維瘤(fibroma)是常見的卵巢性索間質腫瘤,佔所有卵巢腫瘤的4%,單側多見,好發於中年女性。腫瘤一般光滑,邊界清楚,是所有卵巢腫瘤中質地最為堅硬的腫瘤。切面外觀可有粘液樣變(myxoid change)或囊性變(cystic degeneration)。鏡下顯示,瘤體由排列緊密的梭形細胞(spindle stromal cell)組成,排列成排列呈編織狀(featherstitched 或stroiform pattern),瘤細胞會製造膠原纖維(collagen)。免疫染色vimentin (+),α-inhibin可(+)。

(病理生理)

症後群所出現腹水可能原因可能有下列幾項,一是因瘤體刺激腹膜,引起一些介質如histamines、 fibrin degradation products釋放,使毛細血管滲透性增強有關(Meigs suggested that irritation of the peritoneal surfaces by a hard, solid ovarian tumor could stimulate the production of peritoneal fluid.Development of ascites may be due to release of mediators from the tumor,leading to increased capillary permeability);二是由於右側膈上的淋巴同比左側豐富,且右膈圓頂的位置也較高,抽吸作用較強,腹水經由淋巴或橫隔至胸腔,故胸腔積液多見於右側(the pleural effusion is classically on the right side);另外,有可能是賀爾蒙刺激,或卵巢瘤蒂中的大靜脈發生部分栓塞,或蒂扭轉(tumor torsion)造成瘤體淋巴管或血管受壓(direct pressure on surrounding lymphatics or vessels),引起血液或淋巴淤滯,液體自腫瘤漏向腹腔有關。

(臨床表現)

患者可能有卵巢癌的家族史(Patients with Meigs syndrome may have a family history of ovarian cancer)。可出現乏力(Fatigue)、腹脹(Bloating and Increased abdominal girth)、咳嗽(Nonproductive cough)、氣促(Shortness of breath)、胸悶、下肢浮腫、尿失禁、體重減輕(Weight loss)、陰道不規則、出血(Menstrual irregularity)、無月經(Amenorrhea for premenopausal women)等。胸腹水多為漏出液,少數為滲出液。

(診斷)

根據症狀、體徵、婦科檢查發現卵巢腫瘤,合併腹水、胸腹腔積液時,則應考慮該症候群;手術證實腫瘤是纖維瘤,則可確診。

(實驗室檢查)
腹水生化檢查 多為漏出液,比重多在1.010~1.017,細胞數常少於400×106/L,蛋白量常少於0.3g/L。乳酸脫氫酶(LDH)及腹水-血清LDH比值(ASLR)、腺苷脫氨酶(ADA)
CBC 測量hemoglobin, hematocrit, and platelet levels,本病症可能會有缺鐵性貧血(A low hemoglobin count requires further workup, including reticulocyte count, total iron-binding capacity, and iron and ferritin levels. Anemia in patients with Meigs syndrome is most likely due to iron deficiency)
血液腫瘤標志物
(Tumor marker serum levels)
無異常或升高(CA-125 can be elevated in Meigs syndrome, but the degree of elevation does not correlate with malignancy)
腹水細胞學、
細胞染色體檢查
良性
腹部超音波檢查 中等或增強回聲區,質較均勻,中等大小,無明確囊壁,盆腔或腹腔,有液性暗區(Abdominal and pelvic ultrasound confirms the ovarian mass and ascites)
x-ray 纖維瘤也可見鈣化區,胸腔積液徵象(Chest radiography confirms pleural effusion)
CT scan confirms ascites and ovarian, uterine, fallopian tube, or broad ligament mass
腹腔鏡檢查 組織病理學檢查
Papanicolaou test 正常
(預後)

卵巢纖維瘤為良性,少數可發生惡變,惡變率為1%~3%。

(治療)
  1. thoracentesis and paracentesis:drain off the transudate。
  2. unilateral salpingo-oophorectomy or wedge resection:correct the underlying etiology。
相關考題
(問題來了)以下試題摘自考選部醫師專技考試題目

<101-1-33> 一位婦女因超音波顯示出現卵巢實質腫瘤而住院做進一步的檢查,在一系列的檢查後,發現此患者合併出現ascites及hydrothorax,臨床上判定為Meigs’ syndrome。此卵巢腫瘤最可能是下列何者?

  1. Thecoma
  2. Cystic teratoma
  3. Endometrioma
  4. Fibroma
解答:D
關鍵詞
梅格斯症候群、卵巢纖維瘤

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