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Obstetric Case Reports 535 chance for a successful pregnancy, being better than other uterine anomalies (Heinonen 2000). The surgical treatment, which consists in marsupialisation of the blind hemivagina, must be performed as early as possible to avoid important complications and to improve the reproductive outcome (Heinonen 2000; Patton et al. 2004; Beer and Carstairs 2013). Heinonen (2000) published a relevant study about the reproductive performance of women with a didelphic uterus and possible associated obstetric complications. The fertility of women with a didelphic uterus was comparatively good: 94% of the women who wanted to conceive had at least one pregnancy and 89% of them had at least one living infant. The incidence of primary infertility was not significantly increased in women with a didelphic uterus. The spontaneous abortion rate (21%) was not significantly different from the rate observed in the general population (15–20%). Preterm delivery occurred in 24% of all deliveries. This rate is higher than that found in the general population (9–10%). Breech presentation represents the first indication for caesarean section in these women (as in our case). The high frequency of caesarean section (84%) reflects the high incidence (51%) of fetal breech presentation among patients with a didelphic uterus. From a review of the literature, we noted that pregnancies in the side of previous obstruction are not frequent: Altchek and Paciuc (2009) counted nine women who had pregnancies in the obstructed uterus after surgical treatment. In two of the nine cases, including the case described in their study, there was a second pregnancy in the affected side. In our case, the woman had a pregnancy in the affected side after a previous pregnancy occurred in the other uterus. As we observed in the literature and in our case, fertility in women with a didelphic uterus is not significantly impaired. The prognosis of pregnancies is comparatively good, with a high fetal survival rate, while an increased risk of preterm delivery, premature rupture of membranes (as described in the present case), fetal growth retardation and malpresentation indicates the need of meticulous prenatal care (Heinonen 2000; Chen and Ng 2006). Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Acién P. 1997. Incidence of Müllerian defects in fertile and infertile women. Human Reproduction 12:1372–1376. Altchek A, Paciuc J. 2009. Successful pregnancy following surgery in the obstructed uterus in a uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: case report and literature review. Journal of Pediatric and Adolescent Gynecology 22:e159–e162. Beer WM, Carstairs SD. 2013. Herlyn–Werner–Wunderlich syndrome: an unusual presentation of acute vaginal pain.Journal of Emergency Medicine 45: 541–543. Chen FP, Ng KK. 2006. Term pregnancy at the site of atresia following vaginal canalization in a case of uterus didelphys with hemivaginal atresia and ipsilateral renal agenesis. Taiwanese Journal of Obstetrics and Gynecology 45:366–368. Fedele L, Motta F, Frontino G et al. 2013. Double uterus with obstructed hemivagina and ipsilateral renal agenesis: pelvic anatomic variants in 87 cases. Human Reproduction 28:1580–1583. Heinonen PK. 1982. Longitudinal vaginal septum. European Journal of Obstetrics, Gynecology, and Reproductive Biology 13:253–258. Heinonen PK. 2000. Clinical implications of the didelphic uterus: long-term follow-up of 49 cases. European Journal of Obstetrics, Gynecology, and Reproductive Biology 91:183–190. Madureira AJ, Mariz CM, Bernardes JC et al. 2006. Uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis. Radiology 239:602–606. Orazi C, Lucchetti MC, Schingo Paolo MS et al. 2007. Herlyn–Werner– Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases. Pediatric Radiology 37:657–665. Patton PE, Novy MJ, Lee DM et al. 2004. The diagnosis and reproductive outcome after surgical treatment of the complete septate uterus, duplicated cervix and vaginal septum. American Journal of Obstetrics and Gynecology 190:1669–1675.

Primary retroperitoneal mucinous cystadenocarcinoma during pregnancy H. M. Hanhan1, K. Gungorduk2, İ. A. Ozdemir2, M. Gokcu2, M. Sanci2, D. Ayaz3 & M. Özeren1 1Department of Obstetrics and Gynecology, Tepecik Education

and Research Hospital, İzmir, Turkey, 2Department of Gynecologic Oncology, Tepecik Education and Research Hospital, İzmir, Turkey, and 3Department of Pathology, Tepecik Education and Research Hospital, İzmir, Turkey DOI: 10.3109/01443615.2014.910501 Correspondence: K. Gungorduk, Department of Gynecologic Oncology, İzmir Tepecik Training and ResearchHospital, İzmir, Turkey. E-mail: [email protected]

Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare tumour. This case report describes the treatment and prognosis of a patient with PRMC during pregnancy. This is the third case of PRMC in a pregnant woman, worldwide. The patient was a 37-year-old woman presenting with a left mid-abdominal and pelvic semisolid, cystic mass at 29 weeks’ gestation. At 30 weeks’ gestation, she underwent an exploratory laparotomy, which revealed a solid tumour (22 ⴛ 13 ⴛ 11 cm) with an intact capsule extending from the inferior pole of the left kidney to the pelvic inlet in the left retroperitoneal area. The tumour had adhesions with the surrounding connective tissue and could be excised with its capsule intact. In conclusion, based on the limited information available, a PRMC with no visible dissemination excised with an intact capsule appears to have a good prognosis. Tumour excision may be adequate for treatment of PRMCs in the extragenital space and with no dissemination. Keywords: Fertility-sparing surgery, pregnancy, retroperitoneal mucinous cystadenocarcinoma

Introduction Mucinous tumours are a common histological type of gynaecological neoplasm that occur especially in the ovaries. However, primary retroperitoneal mucinous tumours are extremely rare and can be further subdivided into benign, borderline and cystadenocarcinomatous (Roma and Malpica 2009; Sonntag et al. 2005). They are usually seen in women of reproductive age, but have been described in patients of a wide age range (Sonntag et al. 2005). To date, only 37 patients with primary retroperitoneal mucinous cystadenocarcinoma (PRMC) have been reported in the English-language literature, two of whom were pregnant patients (Sonntag et al. 2005; Kashima et al. 2008). Because of the rarity of this neoplasm, its pathogenesis, biological behaviour and staging system remain ambiguous. Preoperative diagnosis can often be confusing. Surgeons must be aware of this type of tumour and include it in the differential diagnosis. Owing to its rarity, the optimal treatment, survival and exact prognosis remain uncertain. Here, we report the third pregnant patient with PRMC worldwide and with the longest survival to date. She underwent fertility-sparing surgery during pregnancy, with a good perinatal outcome.

Case report A 37-year-old gravida 5, para 5 woman was referred to our clinic for evaluation of an asymptomatic, semisolid cystic mass in the left midabdomen and pelvis. The mass was found during a routine ultrasound examination at 29 weeks’ gestation. An ultrasound examination showed a single fetus of 29–30 weeks’ gestational age and a normal amniotic fluid volume. Magnetic resonance imaging (MRI) showed a single intrauterine fetus and a cystic mass (22 ⫻ 13 ⫻ 11 cm) in the left mid-abdomen; the mass contained mural nodules and had a smooth, thin border (Figure 1a, b). No ascites were detected, and the

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Figure 1. (a, b) Magnetic resonance imaging demonstrates a cystic tumour with mural nodules. The pregnant uterus is marked (asterisk). (c) On macroscopic inspection, the resected tumour appears to have an intact capsule. (d) Gross appearance of the formalin-fixed tumour. The arrows indicate mural nodules. (e) A microphotograph of the tumour with haematoxylin and eosin (H&E) staining shows a mucinous cystadenocarcinoma (⫻ 40). Immunohistochemical staining: (f) diffuse immunoreaction for cytokeratin 7; (g) diffuse immunoreaction for cytokeratin 20; (h) diffuse immunoreaction for cytokeratin for CEA.

levels of the tumour markers CA125, CA15-3 and alpha-fetoprotein were in the upper ranges of normal. Only the level of CA19-9 was high (630 U/ml; normal, ⬍ 0 to 33 U/ml). An exploratory laparotomy was performed at 30 weeks’ gestation, and no tumour dissemination was found. There were also no ascites, and a peritoneal wash sample was obtained. A left retroperitoneal mass of about 32 ⫻ 18 ⫻ 16 cm was found. It was fixed at the left side of the posterior abdomen, and the left kidney was pushed under the spleen. The tumour was removed without rupture of the capsule (Figure 1c, d). The ovaries, fallopian tubes, appendix, colon, kidneys, omentum and other intra-abdominal organs were normal in appearance. There were no large lymph nodes in the pelvic region or para-aortic space. Lymphadenectomy was not performed because of advanced gestation. A macroscopic examination revealed that the tumour was 23 ⫻ 13 ⫻ 10 cm, well encapsulated with a smooth border, and surrounded by

little fat tissue (Figure 1c). The peritoneal wash cytology was negative for malignant cells. The inner surface of the tumour contained a uniloculated cyst, brown mucoid liquid and papillary projecting lesions; the largest lesion was 5 ⫻ 3 ⫻ 2 cm and dirty white in colour (Figure 1d). The capsule thickness was 2–4 mm. A microscopic examination of the specimens taken from a solid area of the cyst capsule revealed cuboidal and columnar mucinous cells, complex glands and papillary lesions with a cribriform pattern. Minimal to mid-level atypical nuclei and focal necrotic spaces were also present. There was no capsule invasion. The tumour cells stained strongly for cytokeratin 7 and 20 and weakly for carcinoembryonic antigen (CEA) (Figure 1e–h). A pathological examination revealed a mucinous cystadenocarcinoma with an intact capsule (Figure 1c). The postoperative recovery was uneventful, and the patient was discharged from the hospital at 32 weeks’ gestation. She was followed for her pregnancy as an outpatient at our hospital. A

normal vaginal delivery occurred at 38 weeks’ gestation, and the infant was a normal, healthy, 3,450 g female. No adjuvant therapy was administrated. The patient has been followed regularly at our hospital and is presently doing well with no recurrence at 24 months after the surgery.

12 months NED 13 months NED 24 months NED


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Healthy term infant

Healthy term infant

Healthy term infant

None Left 22 630 Normal Normal AS 37 Present case


NA 17 Normal Normal Normal AS 26 28 Kashima et al. (2008)

AS, asymptomatic; GA, gestational age; TR, tumour resection; NED, no evidence of disease; CS, caesarean section; VD, vaginal delivery; NA, not available.




CS 38 weeks VD 38 weeks VD 38 weeks None TR

During C/S 38 weeks’ gestation Laparotomy 31 weeks’ gestation Laparotomy 30 weeks’ gestation Left 5 409.2 Normal Normal AS 35 30


Sonntag et al. (2005)

Labour Adjuvant treatment Treatment Operation time Location Size (cm) CA19-9 (U/ml) AFP (ng/ml) CA125 (IU/ml) GA at diagnosis (weeks) Symptoms Patient age (years)

Table I. Reported cases of primary retroperitoneal cystadenocarcinoma during pregnancy.

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Fetal outcome after surgery

Discussion The routine performance of prenatal ultrasonography has raised the awareness, and thus the reported incidence, of adnexal masses during pregnancy. Adnexal masses are diagnosed in 2.3–8.8% of all pregnancies (Bernhard et al. 1999). The majority comprise corpus luteum or other functional cysts, and they usually resolve spontaneously by 16 weeks’ gestation (Bernhard et al. 1999). However, persistent adnexal masses are associated with risks for torsion, rupture, obstruction of vaginal delivery, and most importantly malignancy, which is estimated to occur in 1–8% of cases (Hoover and Jenkins 2011). On the other hand, surgical management during pregnancy may also be associated with critical complications, such as miscarriage, pre-term labour and perinatal death. Therefore, proper management of adnexal masses during pregnancy is essential for both maternal and fetal wellbeing. The histological diagnosis of adnexal masses in pregnancy is reportedly dermoid cysts in 25% of cases, serous cystadenomas in 14%, mucinous cystadenomas in 11%, functional cysts in 17%, and endometrioma in 11% (Hoover and Jenkins 2011). PRMC is an extremely rare occurrence in pregnancy, and only two cases have been described in the literature (Sonntag et al. 2005; Kashima et al. 2008; see Table I). The present case involves the first patient with PRMC and mural nodules during pregnancy. PRMC can occur at any age from 14 to 85 years, with a mean age of 39 years. Both cysts previously reported in association with pregnancy occurred in women in their late twenties/early thirties; our patient was 37 years old. This type of retroperitoneal tumour is usually asymptomatic. However, the most common symptoms are abdominal mass-associated distention, discomfort or pain. Our patient, and the previous two patients, were diagnosed with an asymptomatic abdominal swelling in the second, third and second trimesters, respectively. Because of its rarity, the aetiology and biological behaviour of PRMC remain ambiguous. In an attempt to explain its origin, four hypotheses have been postulated. The first hypothesis suggests that PRMCs arise from a retroperitoneal monodermal teratoma with predominantly columnar epithelium (Papadogiannakis et al. 1997). Another hypothesis is that of coelomic metaplasia (Thorbeck et al. 1984). Because these tumours resemble ovarian mucinous cystic neoplasms. The third explanation is that they develop from ectopic ovarian tissue; however, no ovarian tissue has ever been found within a PRMC (Kessler et al. 2002). The fourth possibility is that the tumours are remnants of the embryonal urogenital apparatus, in which the cysts develop from specialised mesothelial cells of the urogenital ridge (Green et al. 2007). The most widely accepted theory is that PRMCs develop from invaginations of the peritoneal epithelium during embryonic growth and subsequently undergo metaplasia (Subramony et al. 2001). Preoperatively, PRMC was not correctly diagnosed from imaging studies, such us ultrasound, computed tomography (CT) or MRI. Although previous cases have reported increased levels of CEA or CA19-9, tumour markers are not helpful in differentiating the lesion (Tapper et al. 2010). Similar to our case, Sonntag et al. (2005) reported that the levels of serum tumour markers were consistently within normal limits throughout pregnancy, with the exception of an elevated CA19-9. Conversely, Kashima et al. (2008) reported that tumour markers, including CA125, CA19-9 and CEA, were all within normal limits. There is currently disagreement among authors regarding the best management for adnexal masses in pregnancy, with some investigators recommending observation and others recommending surgical management (Hoover and Jenkins 2011). Whenever possible, surgery should be postponed until after delivery; if the surgery is necessary because of a strong suspicion of malignancy, the most ideal time for surgery is early in the second trimester. Organogenesis is complete in the second trimester, and the risk for spontaneous pregnancy loss

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Obstetric Case Reports

is substantially lower than in the first trimester, thus minimising the potential impact of surgery on the pregnancy outcome (Hoover and Jenkins 2011). However, in the management of the present patient, surgical invention was performed at 30 weeks’ gestational age, based on the following considerations: (a) The size of the tumour was 32 ⫻ 18 ⫻ 16 cm. It has been recommended that masses larger than 5–6 cm in diameter that persist past 16 weeks’ gestation and have indeterminate sonographic findings, should be surgically removed (Hoover and Jenkins 2011). (b) More recently, Koo et al. (2011) reported that the risk for malignancy associated with masses having a diameter ⬎ 15 cm was approximately 12-fold that of masses with a diameter ⬍ 6 cm. MRI revealed a cyst with solid mural nodules in our case. Some studies have shown that mural nodules are suggestive of malignancy (Hoover and Jenkins 2011; Mikami et al. 2003). Management protocols for PRMC are not well established. Most authors have advised comprehensive surgery that includes total hysterectomy, bilateral salpingo-oophorectomy and lymphadenectomy (Mikami et al. 2003; Tenti et al. 1994; Lee et al. 1996; Tangjitgamol et al. 2002; Law et al. 2006). On the other hand, these same authors have shown that if the mass has an intact capsule and the ovaries and uterus appear to be normal, PRMC can be treated by tumour excision alone in patients who wish to preserve fertility (Kashima et al. 2008; Kessler et al. 2002; Law et al. 2006). Chemotherapy should be limited to tumours that rupture during surgery or when invasion to adjacent structures is evident (Sonntag et al. 2005; Kashima et al. 2008). In our case, the tumour was removed with an intact capsule at 30 gestational weeks. Following tumour removal, the patient continued to carry the baby to full term. Because the tumour was completely resected with an intact capsule, adjuvant chemotherapy was not planned either during pregnancy or after delivery. The patient has shown no evidence of recurrence for 24 months after the surgery. In conclusion, PRMC is extremely rare in pregnancy. For young patients with PRMC who wish to preserve their fertility, tumour excision alone may be a feasible treatment if the tumour has an intact capsule and no dissemination to adjacent structures is detected. Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

References Bernhard LM, Klebba PK, Gray DL, Mutch DG. 1999. Predictors of persistence of adnexal masses in pregnancy. Obstetrics and Gynecology 93:585–589.

Green JM, Bruner BC, Tang WW, Orihuela E. 2007. Retroperitoneal mucinous cystadenocarcinoma in a man: Case report and review of the literature. Urologic Oncology: Seminars and Original Investigations 25:53–55. Hoover K, Jenkins TR. 2011. Evaluation and management of adnexal mass in pregnancy. American Journal of Obstetrics and Gynecology 205:97–102. Kashima K, Yahata T, Fujita K, Tanaka K. 2008. Primary retroperitoneal mucinous cystadenocarcinoma associated with pregnancy. International Journal of Gynecological Cancer 18:908–912. Kessler TM, Kessler W, Neuweiler J, Nachbur BH. 2002. Treatment of a case of primary retroperitoneal mucinous cystadenocarcinoma: Is adjuvant hysterectomy and bilateral salpingo-oophorectomy justified? American Journal of Obstetrics and Gynecology 187:227–232. Koo YJ, Kim TJ, Lee JE, Kwon YS, Kim HJ, Lee IH et al. 2011. Risk of torsion and malignancy by adnexal mass size in pregnant women. Acta Obstetricia et Gynecologica Scandinavica 90:358–361. Law KS, Chang TM, Tung JN. 2006. Fertility-sparing treatment of a primary retroperitoneal mucinous cystadenocarcinoma. International Journal of Obstetrics and Gynaecology 113:612–614. Lee IW, Ching KC, Pang M, Ho TH. 1996. Two cases of primary retroperitoneal mucinous cystadenocarcinoma. Gynecologic Oncology 63:145–150. Mikami M, Tei C, Takehara K, Komiyama S, Suzuki A, Hirose T. 2003. Retroperitoneal primary mucinous adenocarcinoma with a mural nodule of anaplastic tumor: a case report and literature review. International Journal of Gynecological Pathology 22:205–208. Papadogiannakis N, Gad A, Ehliar B. 1997. Primary retroperitoneal mucinous tumor of low malignant potential: histogenetic aspects and review of the literature. Acta Pathologica, Microbiologica et Immunologica Scandinavica 105:483–486. Roma AA, Malpica A. 2009. Primary retroperitoneal mucinous tumors: a clinicopathologic study of 18 cases. American Journal of Surgical Pathology 33:526– 533. Sonntag B, Lelle RJ, Steinhard J, Brinkmann OA, Hungermann D, Kiesel L. 2005. Retroperitoneal mucinous adenocarcinoma occurring during pregnancy in a supernumerary ovary. Journal of Obstetrics and Gynaecology 25:515–516. Subramony C, Habibpour S, Hashimoto LA. 2001. Retroperitoneal mucinous cystadenoma. Archives of Pathology and Laboratory Medicine 125:691–694. Tangjitgamol S, Manusirivithaya S, Sheanakul C, Leelahakorn S, Thawaramara T, Kaewpila N. 2002. Retroperitoneal mucinous cystadenocarcinoma: a case report and review of literature. International Journal of Gynecological Cancer 12:403–408. Tapper EB, Shrewsberry AB, Oprea G, Majmudar B. 2010. A unique benign mucinous cystadenoma of the retroperitoneum: a case report and review of the literature. Archives of Gynecology and Obstetrics 281:167–169. Tenti P, Carnevali L, Tateo S, Durola R. 1994. Primary mucinous cystadenocarcinoma of the retroperitoneum: two cases. Gynecologic Oncology 55:308–312. Thorbeck VC, Gustein D, Salvi M, Plata J. 1984. (Retroperitoneal enteroid cystadenocarcinoma (possible intestinal origin)). Revista Espanola de las Enfermedades del Aparato Digestivo 66:329–334.

Primary retroperitoneal mucinous cystadenocarcinoma during pregnancy.

Primary retroperitoneal mucinous cystadenocarcinoma (PRMC) is an extremely rare tumour. This case report describes the treatment and prognosis of a pa...
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