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Figure 1. Contrast-enhanced CT scan of the patient’s abdomen showing a heterogeneous mass extending from the uterus to the right pelvic side wall; massive ascites could also be detected.

the abdomen showed a 10 ⫻ 9 cm solid mass, together with a large amount of ascites. CA125 was found to be high at 939.70 U/ml (normal: ⬍ 35 U/ml). Chest X-ray did not show a pleural effusion. An abdominal CT scan showed that a heterogenic solid and cystic mass was present in the right lower quadrant of the abdomen and that massive ascites could also be detected (Figure 1). Paracentesis was done and resulted in the release of 2.3 litres of cloudy, greenish-yellow fluid. The ascites contained mesothelial cells and histocytes. Bacterial and fungal infections were absent. An exploratory laparotomy was done. During the operation, 4 litres of yellowish ascites were drained. A right-sided pelvic mass consisting of whitish firm tissue was noted. The mass was 12 ⫻ 11 ⫻ 7.8 cm in size, with a stalk connecting it to the uterus, together with tight adhesion to the right pelvic wall, to the distal appendix and to the mesentery of the small bowel. A parasitised blood supply from the adjacent tissues and organs could be seen. The tumour was excised. Both ovaries and fallopian tubes were grossly normal. The postoperative course was uneventful. No ascites were noted after the operation. The pathological report indicated a leiomyoma with areas of myxoid degeneration.

Discussion Pedunculated subserosal leiomyoma may present with various different symptoms and complications. In the previous literature, lower gastrointestinal haemorrhage has been found to be caused by parasitic leiomyoma adhering to the distal ileum (Rader et al. 1990). In another case, massive ascites and left pleural effusion resulting from a pedunculated leiomyoma (Kebapci et al. 2002) were reported. Meigs’ syndrome is an uncommon condition that was first report by Meigs and Cass in 1937. The definition of this syndrome is limited to the existence of benign and solid ovarian tumours, especially fibromas that are accompanied by ascites and hydrothorax. PseudoMeigs’ syndrome is defined as being similar to Meigs’ syndrome, except that the tumour may be associated with the ovaries, the fallopian tubes, the uterus, round ligaments or the colon. In our case, the patient presented with leiomyoma, a large amount of ascites (6.3 litres) and no hydrothorax, which is extremely rare and not often seen in the literature. This variant in terms of presentation has been called atypical pseudo-Meigs’ syndrome (Ricci et al. 2009). Several theories have been put forward to explain the presence of massive ascites in Meigs’ or pseudo-Meigs’ syndromes; these include twisting of the pedicles of the tumours, the pressure of the tumour on vessels and lymphatics and an inflammatory reaction due to the presence of

the tumour (Amr and Hassan 1994). Here, the pedunculated leiomyoma was tightly adhering to the peripheral tissues. There was no opportunity for the pedicle to rotate or even twist. The appearance of massive ascites might be due to compression and subsequent congestion of the lymphatics and blood vessels because of the presence of the large pedunculated leiomyoma. Interstitial fluid secretion from a fibroid surface is also supposed to cause the development of ascites (Timmerman et al. 1995). In our case, the removal of the fibroid surface from the abdomen is one possible explanation for the disappearance of the ascites after resection of the tumour. CA125 is used as a tumour maker and is associated with ovarian cancer. However, other conditions such as endometriosis, pelvic inflammatory disease, pregnancy, benign ovarian tumours or even uterine leiomyomas are known to cause an elevation in CA125 levels. The level of CA125 is also increased when there is peritoneal, pleural or pericardial inflammation, as well as when there is peritoneal irritation due to a large pelvic mass. Peritoneal mesothelial cells are able to express CA125 (Ricci et al. 2009; Brown et al. 1988; Timmerman et al. 1995). In our case, the present of reactive mesothelial cells in the ascetic fluid and the persistent irritation to the peritoneum caused by the large pedunculated leiomyoma, are both potential sources of CA125. However, it has been found previously that there is no correlation between the tumour weight, amount of ascites and the CA125 level of the patient (Schmitt et al. 2005). In conclusion, it is important to remember that, when a patient has a pelvic mass, massive ascites and an elevated level of CA125, there can be a variety of causes other than ovarian cancer or another type of pelvic benign solid ovarian tumour. Fibroma, but also pedunculated leiomyoma, may be considered in the differential diagnosis in these circumstances. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Amr SS, Hassan AA. 1994. Struma ovarii with pseudo-Meigs’ syndrome: report of a case with review of the literature. European Journal of Obstetrics, Gynecology, and Reproductive Biology 55:205–208. Brown RS, Marley JL, Cassoni AM. 1988. Pseudo-Meigs’ syndrome due to broad ligament leiomyoma: a mimic of metastatic ovarian carcinoma. Clinical Oncology 10:198–201. Fasih N, Prasad Shanbhogue AK et al. 2008. Leiomyomas beyond the uterus: unusual locations, rare manifestations. Radiographics 28:1931–1948. Kebapci M, Aslan O, Kaya T et al. 2002. Pedunculated uterine leiomyoma associated with pseudo-Meigs’ syndrome and elevated CA-125 level: CT features. European Radiology 12:S12–S129. Meigs JV, Cass JW. 1937. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. American Journal of Obstetrics and Gynecology 33:249–267. Parker WH. 2012. Uterine fibroids. In: Berek JS, editor. Berek and Novak’s gynecology. 15th ed. Philadelphia: Lippincott Williams and Wilkins. p 439–445. Rader JS, Binette SP, Brandt TD et al. 1990. Ileal hemorrhage caused by a parasitic uterine leiomyoma. Obstetrics and Gynecology 76:531–534. Ricci G, Inglese S, Candiotto A et al. 2009. Ascites in puerperium: a rare case of atypical pseudo-Meigs’ syndrome complicating the puerperium. Archives of Gynecology and Obstetrics 280:1033–1037. Schmitt R, Weichert W, Schneider W et al. 2005. Pseudo-pseudo Meigs’ syndrome. Lancet 366:1672. Timmerman D, Moerman P, Vergote I. 1995. Meig’s syndrome with elevated serum CA125 levels: two case reports and review of the literature. 1995. Gynecologic Oncology 59:405–408.

Pseudo-Meigs’ syndrome secondary to endodermal sinus tumour T. Song1, M. K. Kim1, M.-L. Kim1, B. S. Yoon1, S. J. Seong1 & J.-Y. Kim2 Departments of 1Obstetrics and Gynaecology and 2Pathology, CHA Gangnam Medical Centre, CHA University, Seoul, Korea DOI: 10.3109/01443615.2013.832738

Gynaecology Case Reports 109 Correspondence: S. J. Seong, Department of Obstetrics and Gynaecology, CHA Gangnam Medical Centre, CHA University, 650–9 Yeoksam-1 Dong, Gangnam-gu, Seoul 135–081, Korea. E-mail: [email protected]

Introduction Meigs’ syndrome is characterised by ascites and hydrothorax, in association with an ovarian fibroma, which resolves after removal of the ovarian tumour (Meigs and Cass 1937). The same characteristic signs in association with another type of benign or malignant, pelvic tumour constitute pseudo-Meigs’ syndrome (Lurie 2000). Malignancy from ovarian germ cell tumour, including endodermal sinus tumour, is a rare cause of this syndrome and only a few cases have been reported (Bridgewater and Rustin 1997; Wolff et al. 2005). We report our clinical experience, review previously reported cases and discuss the diagnosis and treatment of pseudo-Meigs’ syndrome caused by endodermal sinus tumour.

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Case report A previously healthy 24-year-old woman was referred with exertional dyspnoea and a rapidly enlarging abdominal mass. She complained of a 2-week history of distended abdomen. The medical and gynaecological histories were otherwise uneventful. On pelvic and abdominal examinations, a large, well-delineated, solid mass extending to 1 cm above the umbilicus was palpable. Transvaginal ultrasound examination demonstrated a uterus of normal size and contours, moderate ascites and a huge solid ovarian tumour. Chest X-ray showed a mild right pleural effusion (Figure 1A). An abdomino-pelvic computed tomography (CT) scan showed a 16 cm, mainly cystic mass with enhancing solid lesion and multiple septa in the pelvic cavity (Figure 1B). Levels of α-fetoprotein and CA125 were elevated at 18,124 ng/ml (reference range 0–8 ng/ml) and 234 U/ml (reference range 0–35 U/ml), respectively, Serology for β-human chorionic gonadotropin, carcinoembryonic antigen and CA19–9 were all normal. Exploratory laparotomy was done, which revealed 500 ml of serous ascites. After aspiration of the ascitic fluid, an encapsulated pink-tan to gray-blue tumour of the right ovary measuring 15.8 cm ⫻ 14.3 cm ⫻ 8.3 cm, with a smooth lobulated surface was found (Figure 2A). The ovarian tumour disintegrated on mobilisation and peritoneal spillage was assumed. Intraoperative frozen section of the right adnexal mass showed an endodermal sinus tumour. The uterus and left adnexa appeared normal. Given the presence of a mass in the pouch of Douglas and a small cyst in the omentum, excision of the mass and infracolic omentectomy were done. There was no palpable pelvic or para-aortic lymphadenopathy; the liver, diaphragm and bowel were grossly free of disease. On postoperative

day 2, drainage of the pleural effusion was done to alleviate persisting dyspnoea. A total of 400 ml of serous fluid without malignant cells on cytological examination was drained. Definitive pathological examination of the right ovary confirmed the frozen section diagnosis of endodermal sinus tumour. Microscopically, the tumour showed the typical perivascular formation (Schiller-Duval bodies) of the endodermal sinus tumour (Figure 2B). Some parts of the tumour also showed glandular, myxomatous and solid patterns. Round hyaline globules that were PAS-positive and AFP-positive were also frequently noted (Figure 2C,D). Omentum and ascitic fluid were free of tumour. The histopathological diagnosis of the mass in the pouch of Douglas was endometriosis. A final diagnosis of stage Ic endodermal sinus tumour associated with non-malignant ascites and pleural effusion was made. The postoperative course was uneventful and ascites and pleural effusion subsided. The patient was given chemotherapy with etoposide (100 mg/m2 per day for 3 days), bleomycin (15 mg/m2 per day for 3 days) and cisplatin (20 mg/m2 per day for 5 days), for four cycles separated by 3 weeks. At 6 months after the surgery, tumour markers were within the normal range and an abdomino-pelvic CT showed no sign of tumour growth or recurrent fluid retention either in the pleural or abdominal cavity.

Discussion The presence of an ovarian tumour, massive ascites, pleural effusion and elevated CA125 almost inevitably indicates a malignant condition, most commonly secondary to epithelial ovarian carcinoma. However, benign, non-epithelial malignant and metastatic ovarian tumours can display a similar clinical picture in the form of Meigs’ or pseudo-Meigs’ syndrome (Lurie 2000; Meigs and Cass 1937). While a definitive diagnosis of Meigs’ or pseudo-Meigs’ syndrome can only be made in retrospect following the resolution of ascitic and pleural fluid after removal of the ovarian mass, considering these syndromes as differential diagnoses in the preoperative period is of clinical significance. These may be particularly relevant in the preoperative counselling of women who desire fertility. Endodermal sinus tumours are one of the most common types of ovarian malignancy in these women. Endodermal sinus tumours typically demonstrate a very good response to chemotherapy and thereby, permit fertilitysparing surgery. A MEDLINE search of the English language literature using the terms: ‘endodermal sinus tumour’, ‘yolk sac tumour’, ‘germ cell tumour’, ‘pseudo-Meigs’ syndrome’ and ‘Meigs’ syndrome’ produced three reports of germ cell tumour associated with pseudo-Meigs’ syndrome (Table I) (Bridgewater and Rustin 1997; Gücer et al. 2005; Wolff et al. 2005). The histological types reported have been endo-

Figure 1. Radiographic findings. (A) Chest X-ray showing right pleural effusion. (B) Abdomino-pelvic CT scan showing a large pelvic mass of about 16 cm and moderate amount of ascites in the perihepatic, perisplenic, and pelvic cavity.

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Figure 2. Pathology findings. (A) Macroscopic findings. Grossly, the removed ovarian tumour had a smooth lobulated surface and revealed a grey-yellow, solid, soft to gelatinous cut section with some necrotic and haemorrhagic foci. (B–D) Microscopic findings. (B) H&E stain, ⫻ 40; (C) PAS stain, ⫻ 400; (D) AFP stain, ⫻ 400. The tumour showed the classical perivascular formation (Schiller–Duval bodies) of the endodermal sinus tumour (arrows in B). Round hyaline globules (PAS-positive and AFP-positive) are noted (arrowhead in C and arrows in D).

Table I. Summary of reported cases of pseudo-Meigs’ syndrome with germ cell tumour. Author

Bridgewater and Rustin

Year Age (years) Histopathology

1997 18 Endodermal sinus tumour

Stage Tumour size (cm) Clinical symptom

Ic∗ 15 Abdominal pain and dyspnoea Not given 4,130 Normal Present Bilateral Surgery† and BEP CTx NED, 6 months

CA125 (U/ml) AFP (ng/ml) β-hCG (mIU/ml) Ascites Pleural effusion Treatment Current status and FU

Wolff et al. 2005 11 Mixed germ cell tumour Ic∗ 12 Fever, dyspnoea and cough Not given 36,690 Normal 1.5 l Left 1.5 l Surgery‡ and BEP CTx NED, 1 month

Gücer et al. 2005 25 Dysgerminoma Ia 22 Abdominal swelling 338 Normal 113 1.2 l Right, 1.5 l Surgery§ NED, 12 months

Present case 2012 24 Endodermal sinus tumour Ic∗ 16 Dyspnoea and abdominal distension 234 18,124 Normal 0.5 l Right, 0.4 l Surgery|| and BEP CTx NED, 6 months

AFP, α-fetoprotein; β-hCG, β-human chorionic gonadotropin; FU, follow-up period; BEP CTx; chemotherapy with bleomycin, etoposide, and cisplatin for 3–4 cycles; NED, no evidence of disease. ∗The ovarian tumours disintegrated on mobilisation and these tumours were upstaged to stage Ic. †The patient underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, subcolic omentectomy and resection of right common iliac nodes. ‡The patient underwent a right salpingo-oophorectomy. §The patient underwent a left salpingo-oophorectomy, wedge resection of right ovary, pelvic and para-aortic lymph node dissection and infracolic omentectomy. ||The patient underwent a right salpingo-oophorectomy and infracolic omentectomy.

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Gynaecology Case Reports 111 dermal sinus tumour (one case), mixed germ cell tumour (one case) and dysgerminoma (one case). We describe an additional case to the second reported in the literature with pseudo-Meigs’ syndrome caused by endodermal sinus tumour. Although the pathophysiology of these fluid collections is poorly understood, several theories about formation of ascitic fluid have been proposed: irritation of peritoneum from the tumour; obstruction of the lymphatics by the tumour; fluid secretion by the tumour from increased permeability of neovasculature; secondary to torsion of the pedicle of the tumour itself; low serum proteins and release of toxins and inflammatory products (Khan et al. 2005; Meigs and Cass 1937). The fluid in the chest in these patients originates from the fluid in the abdomen, as has been determined by injecting indian ink into the ascites, which appears in the pleural effusions after about 3 h (Amant et al. 2001). Most effusions in pseudo-Meigs’ syndrome are right-sided, owing to a greater frequency of developmental defects in the right hemi-diaphragm (Cotran et al. 1999). In pseudo-Meigs’ syndrome, pleural fluids are also negative for malignant cells, suggesting that this is not a sign of metastatic disease. In our review of the four cases of pseudo-Meigs’ syndrome secondary to germ cell tumour (Table I), serum CA125 was elevated in two cases (50%). The exact mechanism that accounts for the elevation of CA125 in pseudo-Meigs’ syndrome is still unknown; however a possible explanation is the irritation and subsequent inflammation of pleural and peritoneal surfaces produced by the presence of free fluid in these spaces. In conclusion, we present an unusual case of pseudo-Meigs’ syndrome associated with endodermal sinus tumour. Although the association between ovarian tumour, pleural effusion, ascites and elevation of CA125 is highly indicative of epithelial ovarian carcinoma, pseudoMeigs’ syndrome must be considered in the differential diagnosis.

Correspondence: R. Parsaei, Department of General Surgery, Imam Khomeini hospital, Keshavarz Boulevard, Tehran, Iran. E-mail: [email protected]

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Discussion

References Amant F, Gabriel C, Timmerman D et al. 2001. Pseudo-Meigs’ syndrome caused by a hydropic degenerating uterine leiomyoma with elevated CA 125. Gynecologic Oncology 83:153–157. Bridgewater JA, Rustin GJ. 1997. Pseudo-Meigs’ syndrome secondary to an ovarian germ cell tumor. Gynecologic Oncology 66:539–541. Cotran RS, Kumar V, Collins T. 1999. The female genital tract. In: Cotran RS, Kumar V, Collins T, editors. Robbins pathologic basis of disease. Philadelphia: WB Saunders. p 1035–1091. Gücer F, Oz-Puyan F, Mulayim N et al. 2005. Ovarian dysgerminoma associated with Pseudo-Meigs’ syndrome and functioning ovarian stroma: a case report. Gynecologic Oncology 97:681–684. Khan J, McClennan BL, Qureshi S et al. 2005. Meigs syndrome and gliomatosis peritonei: a case report and review of literature. Gynecologic Oncology 98:313–317. Lurie S. 2000. Meigs’ syndrome: the history of the eponym. European Journal of Obstetrics, Gynecology, and Reproductive Biology 92:199–204. Meigs JV, Cass JW. 1937. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. American Journal of Obstetrics and Gynecology 33:249–267. Wolff AL, Ladd AP, Kumar M et al. 2005. Pseudo-Meigs syndrome secondary to ovarian germ cell tumor. Journal of Pediatric Surgery 40:737–739.

Case report A 49-year-old woman presented to the outpatient clinic of our centre, with a 2-month history of vaginal bleeding. She had been menopausal since 4 years previously. Her history was otherwise unremarkable. A colposcopy was performed and a mass was found in the uterine cervix. A biopsy was taken and the histopathology reported infiltration of the cervix with neoplastic tissue composed of highly atypical polygonal cells with eosinophilic cytoplasm, clumped chromatin, conspicuous nucleoli and frequent mitotic activity and suggested diagnosis of poorly-differentiated carcinoma in favour of squamous cell carcinoma but recommended immunohistochemistry for definite diagnosis. Immunohistochemistry of the specimen was positive for: S-100, HMB45, Melan-A and negative for cytokeratin AE/E3 and cytokeratin 7; findings that were compatible with malignant melanoma. The patient was re-examined and the vulva and vagina were normal. The mass was confined to the cervix and its black stains were obvious. A thorough examination of the skin and mucus membranes showed no abnormality. Fundoscopy revealed no abnormal lesion and a chest and abdominopelvic CT-scan was negative for metastases and lymphadenopathy. The patient underwent a total hysterectomy and bilateral salpingooophorectomy, with dissection of para illiac and obturator lymph nodes. In a gross specimen, a 6 ⫻ 4 cm nodular black-stained lesion was seen in cervix (Figure 1). One of surgical margins was involved with tumour and all 17 resected lymph nodes were free of tumour. The tumour was stage IIA2, according to FIGO staging criteria. We referred the patient for chemoradiotherapy. Malignant melanoma arises from melanocytes and accounts for 3% of all cancers (Landis et al. 1998). It is primarily a malignancy of skin but also occurs less commonly in other sites, such as the mucus membranes of the oral cavity, oesophagus, anus, conjunctiva and female genital tract, that altogether consist 2% of all malignant melanomas (Patrick et al. 2007; McLaughlin et al. 2005). The vulva and vagina are usual origins of malignant melanoma in the female genital tract. Primary malignant melanoma of the uterine cervix is quite rare. In fact, the uterine cervix had been doubted as a possible origin for primary malignant melanoma, postulating that there are no melanocytes in cervix (Kristiansen et al. 1992) but since 1959, when Cid reported the presence of melanin in 3–5% of cervixes and with confirmation from additional research, the uterine cervix is now accepted as a possible, although rare, origin of malignant melanoma (Cid 1959; Goldman and Friedman 1967).

Primary malignant melanoma of uterine cervix R. Omranipour1, H. Mahmoodzadeh1, A. Jalaeefar1, A. Abdirad2, R. Parsaei1 & R. Ebrahimi1 Departments of 1Surgical Oncology and 2Pathology, Cancer Institute, Tehran University of Medical Sciences, Tehran, Iran DOI: 10.3109/01443615.2013.834297

Figure 1. Gross specimen showing nodular lesion with black spots in the cervix.

Pseudo-Meigs' syndrome secondary to endodermal sinus tumour.

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