Mucinous adenocarcinoma of the intestinal type arising from mature cystic teratoma of the ovary: a rare case report and review of the literature
© Takai et al.; licensee BioMed Central Ltd. 2012
Received: 30 October 2012
Accepted: 27 November 2012
Published: 5 December 2012
Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary. Malignant tranformation occurs in 1-2% of these neoplasms. Although most of the malignancies arising from MCTs are squamous cell carcinomas, adenocarcinoma of the gastrointestinal type is extremery rare. We herein present a case of adenocarcinoma of the intestinal type arising from a MCT.
A 49-year-old female underwent surgery for a left ovarian tumor. The histology of the cyst walls revealed a MCT with a few hair shafts and a squamous layer, while another part of the tumor showed adenocarcinoma of the intestinal type. Five years after surgery, she is alive without disease.
KeywordsMalignant transformation Mature cystic teratoma Adenocarcinoma Intestinal type
Mature cystic teratomas (MCTs) are the most common germ cell tumors of the ovary. MCTs comprise 18% of ovarian neoplasms, and malignant teranformation occurs in 1-2% of these neoplasms  . Most of the malignancies arising from MCTs are squamous call carcinomas, with adenocarcinomas comprising only 7% of the malignant tumors . Moreover, there have been few reports of adenocarcinoma of the gastrointestinal type in the ovary arising from a MCT [2–5]. We herein present a case of ovarian adenocarcinoma of the intestinal type arising from a MCT in a perimenopausal female, and provide a review of the literature.
An exploratory laparoscopy revealed an enlarged left ovary (80mm × 70mm). The surface of the tumor was smooth and well-circumscribed. There was no adherence to any other pelvic organs. A small amount of ascites was detected, and the cytology of ascites was negative for malignancy. A laparoscopic left salpingo-oophorectomy was performed, and the intraoperative diagnosis of frozen sections was suspicious of borderline malignancy. Therefore, we changed the procedure to a laparotomy and abdominal simple total hysterectomy, and a right salpingo-oophorectomy and partial omentectomy were carried out. The patient’s postoperative course was uneventful. The pathological findings revealed the tumor to be adenocarcinoma of the intestinal type arising from a MCT. The serum level of CEA was normalized 7 days after surgery. We explained to the patient that there was no evidence about the need for adjuvant therapy because the disease is extremely rare. She decided not to receive adjuvant therapy. The patient had no recurrence of the disease as of 5 years after the surgery.
Macroscopic and microscopic evaluations
The final diagnosis was left ovarian MCT with malignant transformation and mucinous adenocarcinoma, intestinal type, FIGO stage Ic (b).
Preveous reports of adenocarcinoma of the gastrointestinal type in the ovary arising from a MCT
Tumor size (cm)
G.Ueda et al. (1993)
Fishman A et al. (1998)
CEA: 40 ng/ml CA 125: 80 U/ml CA153: 60 U/ml
20 x 13 x 8.5
DOD 3 month after surgery
Levine DA et.al (2004)
CEA: 11.2 ng/ml CA 125,AFP, HCG: WNL
15 x 12 x 11
Unilateral SO +OMT +PLN+PAN
Kushima M (2004)
CA19-9: 109 U/ml SLX: 58.5 U/ml CA125: 36 U/ml CA72-4: 19 U/ml
6.4 x 4.8 x 2.8
KJ Min et.al (2006)
CA125: 72 U/ml
17 x 14 x 2
Gunney M et.al (2006)
CEA: WNL CA1 25: 99.1 U/ml CA19-9: >1000 U/ml
CEA: 6.9 ng/ml CA 125: 20 U/ml CA19-9: 3.8 U/ml SCC: 1.1 ng/ml
6.7 x 5.7
MCTs of the ovary represent the majority of benign ovarian neoplasms in females younger than 30 years of age , whereas malignant transformation of MCTs is usually found in postmenopausal females. The present case was perimenopausal and three of the six previous cases were premenopausal. Adenocarcinoma of the gastrointestinal type arising from MCTs may occur at a lower age than other histological types of malignant transformation of MCTs.
Recently, laparoscopic surgery for benign ovarian cystic tumors has been performed rather than laparotomy. However, there have been concerns about artificial tumor rupture due to chemical peritonitis and the spread of tumor cells. Hackethal et al.  reviewed and analyzed the published data about squamous cell carcinoma arising from MCTs of the ovary. According to their data, unlike common ovarian carcinomas, rupture of the tumor capsule has no adverse prognostic effects. In the present case, we performed a laparoscopic salpingo-oophorectomy and could not avoid the artificial rupture of the tumor. Fortunately, our case had no recurrence in the abdominal cavity. However artificial rupture of the tumor should be avoided in order to prevent recurrence . Furthermore, the previous analysis revealed that while hysterectomy, bilateral salpingo-oophorectomy and lymphadenectomy were associated with a better outcome, omentectomy did not affect the overall survival. This may suggest that the spreading pattern of malignant transformation of MCTs differs from that of epithelial ovarian cancer.
Several reports suggested that the SCC antigen, either alone or in combination with other markers, such as macrophage-colony stimulating factor (M-CSF) and carcinoembryonic antigen (CEA), should be considered useful markers for squamous cell carcinoma arising from MCTs for making a preoperative diagnosis [10–12]. On the other hand, there has been no data regarding adenocarcinoma arising from MCTs because of the small number of cases. However, as shown in Table 1, an elevation of CEA was seen in three of the 7 cases, including our present case. Therefore, tumors markers may be useful for making a preoperative diagnosis.
MCTs might occur as a result of failure of the first meiotic division, but it is unclear how the malignant transformation of MCTs occurs. Rim et al.  studied 11 cases of malignant transformation of MCTs and elucidated that 80% of MCTs are diagnosed in subjects who are of reproductive age. Based on that finding, Amanjit et al.  suggested that the malignant transformation of MCTs could be related to the long-term presence of non-removed MCTs. However, the pathogenesis is still unknown, and it is unclear whether malignant transformation occurs separately or co-exists with carcinoma.
It is difficult to make a diagnosis of malignant transformation of a MCT preoperatively. MRI findings may be helpful to distinguish malignant transformation from benign neoplasms. Several reports have revealed that an important feature of the malignant transformation of MCTs was the existence of an enhanced solid component in MCTs [15–18]. In our case, MRI was performed without gadolinium enhancement, therefore, it was difficult to determine whether the lesion was malignant.
When malignant transformation of MCTs is suspected preoperatively, enhanced MRI, intraoperative consultation and a postoperative histological diagnosis are very important.
Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Peterson WF: Malignant degeneration of benign cystic teratomas of the overy; a collective review of the literature. Obstet Gynecol Surv 1957, 12: 793–830. 10.1097/00006254-195712000-00001PubMedView ArticleGoogle Scholar
- Ueda G, Fujita M, Ogawa H, Sawada M, Inoue M, Tanizawa O: Adenocarcinoma in a benign cystic teratoma of the ovary: report of a case with a long survival period. Gynecol Oncol 1993, 48: 259–263. 10.1006/gyno.1993.1044PubMedView ArticleGoogle Scholar
- Min KJ, Jee BC, Lee HS, Kim YB: Intestinal adenocarcinoma arising in a mature cystic teratoma of the ovary: a case report. Pathol Res Pract 2006, 202: 531–535. 10.1016/j.prp.2006.03.005PubMedView ArticleGoogle Scholar
- Fishman A, Edelstein E, Altaras M, Beyth Y, Bernheim J: Adenocarcinoma arising from the gastrointestinal epithelium in benign cystic teratoma of the ovary. Gynecol Oncol 1998, 70: 418–420. 10.1006/gyno.1998.5123PubMedView ArticleGoogle Scholar
- Levine DA, Villella JA, Poynor EA, Soslow RA: Gastrointestinal adenocarcinoma arising in a mature cystic teratoma of the ovary. Gynecol Oncol 2004, 94: 597–599. 10.1016/j.ygyno.2004.05.045PubMedView ArticleGoogle Scholar
- Kushima M: Adenocarcinoma arising from mature cystic teratoma of the ovary. Pathol Int 2004, 54: 139–143. 10.1111/j.1440-1827.2004.01599.xPubMedView ArticleGoogle Scholar
- Guney M, Oral B, Demir F, Ozsoy M, Kapucuoglu N: Mucinous adenocarcinoma arising from the gastrointestinal epithelium in benign cystic teratoma of the ovary–case report. Eur J Gynaecol Oncol 2006, 27: 304–306.PubMedGoogle Scholar
- Koonings PP, Campbell K, Mishell DR, Grimes DA: Relative frequency of primary ovarian neoplasms: a 10-year review. Obstet Gynecol 1989, 74: 921–926.PubMedGoogle Scholar
- Hackethal A, Brueggmann D, Bohlmann MK, Franke FE, Tinneberg HR, Münstedt K: Squamous-cell carcinoma in mature cystic teratoma of the ovary: systematic review and analysis of published data. Lancet Oncol. 2008, 9: 1173–1180. 10.1016/S1470-2045(08)70306-1PubMedView ArticleGoogle Scholar
- Suzuki M, Tamura N, Kobayashi H, Ohwada M, Terao T, Sato I: Clinical significance of combined use of macrophage colony-stimulating factor and squamous cell carcinoma antigen as a selective diagnostic marker for squamous cell carcinoma arising in mature cystic teratoma of the ovary. Gynecol Oncol 2000, 77: 405–409. 10.1006/gyno.2000.5784PubMedView ArticleGoogle Scholar
- Kikkawa F, Nawa A, Tamakoshi K, Ishikawa H, Kuzuya K, Suganuma N, Hattori S, Furui K, Kawai M, Arii Y: Diagnosis of squamous cell carcinoma arising from mature cystic teratoma of the ovary. Cancer 1998, 82: 2249–2255. 10.1002/(SICI)1097-0142(19980601)82:11<2249::AID-CNCR21>3.0.CO;2-TPubMedView ArticleGoogle Scholar
- Miyazaki K, Tokunaga T, Katabuchi H, Ohba T, Tashiro H, Okamura H: Clinical usefulness of serum squamous cell carcinoma antigen for early detection of squamous cell carcinoma arising in mature cystic teratoma of the ovary. Obstet Gynecol 1991, 78: 562–566.PubMedGoogle Scholar
- Rim SY, Kim SM, Choi HS: Malignant transformation of ovarian mature cystic teratoma. Int J Gynecol Cancer. 2006, 16: 140–144. 10.1111/j.1525-1438.2006.00285.xPubMedView ArticleGoogle Scholar
- Bal A, Mohan H, Singh SB, Sehgal A: Malignant transformation in mature cystic teratoma of the ovary: report of five cases and review of the literature. Arch Gynecol Obstet 2007, 275: 179–182. 10.1007/s00404-006-0244-xPubMedView ArticleGoogle Scholar
- Kido A, Togashi K, Konishi I, Kataoka ML, Koyama T, Ueda H, Fujii S, Konishi J: Dermoid cysts of the ovary with malignant transformation: MR appearance. AJR Am J Roentgenol 1999, 172: 445–449.PubMedView ArticleGoogle Scholar
- Takemori M, Nishimura R: MRI findings of an ovarian dermoid cyst with malignant transformation. Magn Reson Med Sci. 2003, 2: 105–108. 10.2463/mrms.2.105PubMedView ArticleGoogle Scholar
- Park SB, Kim JK, Kim KR, Cho KS: Preoperative diagnosis of mature cystic teratoma with malignant transformation: analysis of imaging findings and clinical and laboratory data. Arch Gynecol Obstet 2007, 275: 25–31.PubMedView ArticleGoogle Scholar
- Yahata T, Kawasaki T, Serikawa T, Suzuki M, Tanaka K: Adenocarcinoma arising from respiratory ciliated epithelium in benign cystic teratoma of the ovary: a case report with analyzes of the CT, MRI, and pathological findings. J Obstet Gynaecol Res 2008, 34: 408–412. 10.1111/j.1447-0756.2008.00784.xPubMedView ArticleGoogle Scholar
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