Journal of Obstetrics and Gynaecology, 2015; Early Online: 1–2 © 2015 Informa UK, Ltd. ISSN 0144-3615 print/ISSN 1364-6893 online
CASE REPORT
Squamous cell carcinoma arising in a mature cystic teratoma exposed through a colo-ovarian fistula K.-W. Min1, S.-J. Lee2, H. Y. Jung3, H. S. Han3, S.-Y. Lee3, M.-K. Seong4, I.-K. Sung5 & W. Y. Kim3
J Obstet Gynaecol Downloaded from informahealthcare.com by Kungliga Tekniska Hogskolan on 08/11/15 For personal use only.
1Department of Pathology, Hallym University Sacred Heart Hospital,
Hallym University College of Medicine, Anyang, Gyeonggi-do, Republic of Korea, 2Department of Obstetrics and Gynecology, KonKuk University School of Medicine, Seoul, Republic of Korea, 3Department of Pathology, KonKuk University School of Medicine, Seoul, Republic of Korea, 4Department of Colorectal Surgery, KonKuk University School of Medicine, Seoul, Republic of Korea, and 5Department of Internal Medicine, KonKuk University School of Medicine, Seoul, Republic of Korea DOI: 10.3109/01443615.2015.1007339 Correspondence: Wook Youn Kim, Department of Pathology, Konkuk University School of Medicine, 120 Neungdong-ro (Hwayang-dong), Gwangjin-gu, Seoul 143-701, Republic of Korea. Tel: ⫹ 82-2-2030-5646. Fax: ⫹ 82-2-2030-5629. E-mail: [email protected]
Introduction Ovarian mature cystic teratoma (MCT) is composed of mature cells, usually from all three germ layers and may be associated with various complications such as torsion, rupture, infection, haemolytic anaemia and the development of malignancy. The rupture of MCT, leaking contents into the peritoneal cavity or adjacent organs has been reported as a complication with an incidence rate of less
than 1% (Peterson et al. 1955). Malignant transformation occurs in approximately 2% of cases, most commonly those of an epithelial origin, such as squamous cell carcinoma (SqCC), adenocarcinoma and small cell carcinoma (Dos Santos et al. 2007). This complication is usually observed in postmenopausal women (Kikkawa et al. 1997). Additionally, rapid growth, pain and weight loss may be evidence of a malignant transformation.
Case report A 67-year-old woman was admitted with complaints of haematochezia for the previous 2 weeks. On physical examination, she had tenderness in the left lower quadrant. Laboratory tests were consistent with normocytic anaemia. A computerised tomography (CT) scan showed a low enhancing mass within the sigmoid colon that abutted the left ovary (Figure 1a). A colonoscopy revealed a huge fungating mass (Figure 1b) through a large colonic fistula; thus, a biopsy was performed. SqCC was diagnosed histologically. A wholebody positron emission tomography showed diffuse high-level 18F-fluorodeoxyglucose (FDG) uptake by the mass. Additionally, multiple foci with marked 18F-FDG uptake were observed in the left para-aortic lymph nodes. The patient underwent an en bloc resection of the sigmoid colon and left ovary. The dissection of left para aortic and pelvic lymph nodes, grossly unremarkable in the operation, was not performed. The ovary revealed a unilocular cyst with a smooth external surface, measuring 9.8 cm, which was connected by a large fistula to a segment of the sigmoid colon, measuring 16.5 cm in length and 4.8 cm in circumference. The cyst was filled with a translucent fluid and brownish debris. A 5.3-cm-sized mural mass was found within the cyst (Figure 1c). Microscopically, the mural mass showed welldifferentiated SqCC invading deeper into the ovarian parenchyma (Figure 1d). No lymphovascular invasion was identified. Mesenteric lymph nodes were free of tumours as well.
Figure 1. (a) Axial pelvic contrast-enhanced CT scan shows a 4-cm-sized low-enhancing fungating mass (white asterisk) within the cystic lumen without significant enhancement in the left ovary. (b) Colonoscopical examination shows a huge fungating mass (white asterisk) exposed through a large colo-ovarian fistula (white arrow) and the true colonic lumen (white arrowhead). (c) A large fistula (black arrow) was detected between the colonic wall and ovarian unilocular cyst. A brownish mural mass (black asterisk) was found within the ovarian cyst. (d) Microscopical findings show a well-differentiated SqCC arising in the cystic cavity lined by stratified squamous epithelium (haematoxylin and eosin [H&E] ⫻ 100). Colo-ovarian fistula revealed a squamous epithelial lining with normal colonic mucosa (inset, H&E, ⫻ 100).
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K.-W. Min et al.
On a follow-up CT 6 months later, the left para-aortic lymph nodes were enlarged and a distant metastasis was seen at the psoas muscle, L3 vertebral body and ureter. The patient then received chemotherapy based on the first-line treatment, followed by second line, third line, fourth line and palliative radiotherapy, but developed progressive metastatic masses and subsequently died a year and half after surgery.
J Obstet Gynaecol Downloaded from informahealthcare.com by Kungliga Tekniska Hogskolan on 08/11/15 For personal use only.
Discussion A fistula usually forms due to leakage from a ruptured ovarian cyst that induces a dense adhesion between the cyst and the bowel. Leakage of cystic fluid is thought to be caused by torsion, trauma, malignant transformation or infection (Okada et al. 2005). Of the provoking factors, ovarian torsion is the most frequent complication of MCT, occurring in 16.1% of cases in one large case series (Peterson et al. 1955) and tends to predispose the MCT to rupture (Malkasian et al. 1965). The MCT may rupture into adjacent organs, most frequently, the bladder or the rectum. Malignant transformation may cause a fistula to form, but it is not an essential factor in fistula formation (Okada et al. 2005). Most studies have demonstrated that leakage of cystic fluid is the primary factor in fistula formation. Although formation of an entero-ovarian fistula due to a ruptured MCT is well documented (Conway and Hanna 2012), a preoperative colonoscopical assessment has not been reported. In our case, the malignant transformation of the MCT was detected by colonoscopy, whereas a previous radiological finding found that primary colon cancer was more likely to be detected, as opposed to a tumour arising from an ovarian cyst. It is thought that a large colo-ovarian fistula can be mistaken for the true colonic lumen on abdominal CT, as the radiological differential diagnosis between primary colon cancer and a fungating mass of MCT remains difficult. The pathophysiology of fistula formation is not well understood. It is hypothesised that a mostly sebaceous cyst’s contents slowly leak into the abdominal cavity through a small rupture site, leading to a chronic inflammatory response (von-Walter and Nelken 2012). However, the inflammatory reaction was minimal in our case, presenting as a colo-ovarian adhesion with a fistula. It is possible that the leakage
was due to small spontaneous ruptures which ultimately progressed towards a chronic event, such as a fistula. MCTs often exhibit malignant transformations of a constituent tissue, most frequently the squamous epithelium, followed by the formation of a typical SqCC. A previous study demonstrated various prognostic factors for MCT fistulas, including tumour stage, size, age and CA-125 (Chen et al. 2008). In summary, our case raises awareness that unusual clinical findings are important and require earlier consideration and pre-surgical planning in order to provide the best possible outcome.
Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References Chen RJ, Chen KY, Chang TC et al. 2008. Prognosis and treatment of squamous cell carcinoma from a mature cystic teratoma of the ovary. Journal of the Formosan Medical Association 107:857–868. Conway AZ, Hanna T. 2012. A dermoid cyst fistulating with the transverse colon. Annals of The Royal College of Surgeons of England 94:e230–231. Dos Santos L, Mok E, Iasonos A et al. 2007. Squamous cell carcinoma arising in mature cystic teratoma of the ovary: a case series and review of the literature. Gynecoloic Oncology 105:321–324. Kikkawa F, Ishikawa H, Tamakoshi K et al. 1997. Squamous cell carcinoma arising from mature cystic teratoma of the ovary: a clinicopathologic analysis. Obstetrics & Gynecology 89:1017–1022. Malkasian GD, Symmonds RE, Dockerty MB. 1965. Malignant ovarina teratomas. Report of 31 Cases. Obstetrics & Gynecology 25:810–814. Okada S, Ohaki Y, Inoue K et al. 2005. A case of dermoid cyst of the ovary with malignant transformation complicated with small intestinal fistula formation. Radiation Medicine 23:443–446. Peterson WF, Prevost EC, Edmunds FT et al. 1955. Benign cystic teratomas of the ovary; a clinico-statistical study of 1,007 cases with a review of the literature. American Journal of Obstetrics & Gynecology 70:368–382. von-Walter AR, Nelken RS. 2012. Benign cystic ovarian teratoma with a fistula into the small and large bowel. Obstetrics & Gynecology 119:434–436.
CASE REPORT
Squamous cell carcinoma arising in a mature cystic teratoma exposed through a colo-ovarian fistula K.-W. Min1, S.-J. Lee2, H. Y. Jung3, H. S. Han3, S.-Y. Lee3, M.-K. Seong4, I.-K. Sung5 & W. Y. Kim3
J Obstet Gynaecol Downloaded from informahealthcare.com by Kungliga Tekniska Hogskolan on 08/11/15 For personal use only.
1Department of Pathology, Hallym University Sacred Heart Hospital,
Hallym University College of Medicine, Anyang, Gyeonggi-do, Republic of Korea, 2Department of Obstetrics and Gynecology, KonKuk University School of Medicine, Seoul, Republic of Korea, 3Department of Pathology, KonKuk University School of Medicine, Seoul, Republic of Korea, 4Department of Colorectal Surgery, KonKuk University School of Medicine, Seoul, Republic of Korea, and 5Department of Internal Medicine, KonKuk University School of Medicine, Seoul, Republic of Korea DOI: 10.3109/01443615.2015.1007339 Correspondence: Wook Youn Kim, Department of Pathology, Konkuk University School of Medicine, 120 Neungdong-ro (Hwayang-dong), Gwangjin-gu, Seoul 143-701, Republic of Korea. Tel: ⫹ 82-2-2030-5646. Fax: ⫹ 82-2-2030-5629. E-mail: [email protected]
Introduction Ovarian mature cystic teratoma (MCT) is composed of mature cells, usually from all three germ layers and may be associated with various complications such as torsion, rupture, infection, haemolytic anaemia and the development of malignancy. The rupture of MCT, leaking contents into the peritoneal cavity or adjacent organs has been reported as a complication with an incidence rate of less
than 1% (Peterson et al. 1955). Malignant transformation occurs in approximately 2% of cases, most commonly those of an epithelial origin, such as squamous cell carcinoma (SqCC), adenocarcinoma and small cell carcinoma (Dos Santos et al. 2007). This complication is usually observed in postmenopausal women (Kikkawa et al. 1997). Additionally, rapid growth, pain and weight loss may be evidence of a malignant transformation.
Case report A 67-year-old woman was admitted with complaints of haematochezia for the previous 2 weeks. On physical examination, she had tenderness in the left lower quadrant. Laboratory tests were consistent with normocytic anaemia. A computerised tomography (CT) scan showed a low enhancing mass within the sigmoid colon that abutted the left ovary (Figure 1a). A colonoscopy revealed a huge fungating mass (Figure 1b) through a large colonic fistula; thus, a biopsy was performed. SqCC was diagnosed histologically. A wholebody positron emission tomography showed diffuse high-level 18F-fluorodeoxyglucose (FDG) uptake by the mass. Additionally, multiple foci with marked 18F-FDG uptake were observed in the left para-aortic lymph nodes. The patient underwent an en bloc resection of the sigmoid colon and left ovary. The dissection of left para aortic and pelvic lymph nodes, grossly unremarkable in the operation, was not performed. The ovary revealed a unilocular cyst with a smooth external surface, measuring 9.8 cm, which was connected by a large fistula to a segment of the sigmoid colon, measuring 16.5 cm in length and 4.8 cm in circumference. The cyst was filled with a translucent fluid and brownish debris. A 5.3-cm-sized mural mass was found within the cyst (Figure 1c). Microscopically, the mural mass showed welldifferentiated SqCC invading deeper into the ovarian parenchyma (Figure 1d). No lymphovascular invasion was identified. Mesenteric lymph nodes were free of tumours as well.
Figure 1. (a) Axial pelvic contrast-enhanced CT scan shows a 4-cm-sized low-enhancing fungating mass (white asterisk) within the cystic lumen without significant enhancement in the left ovary. (b) Colonoscopical examination shows a huge fungating mass (white asterisk) exposed through a large colo-ovarian fistula (white arrow) and the true colonic lumen (white arrowhead). (c) A large fistula (black arrow) was detected between the colonic wall and ovarian unilocular cyst. A brownish mural mass (black asterisk) was found within the ovarian cyst. (d) Microscopical findings show a well-differentiated SqCC arising in the cystic cavity lined by stratified squamous epithelium (haematoxylin and eosin [H&E] ⫻ 100). Colo-ovarian fistula revealed a squamous epithelial lining with normal colonic mucosa (inset, H&E, ⫻ 100).
2
K.-W. Min et al.
On a follow-up CT 6 months later, the left para-aortic lymph nodes were enlarged and a distant metastasis was seen at the psoas muscle, L3 vertebral body and ureter. The patient then received chemotherapy based on the first-line treatment, followed by second line, third line, fourth line and palliative radiotherapy, but developed progressive metastatic masses and subsequently died a year and half after surgery.
J Obstet Gynaecol Downloaded from informahealthcare.com by Kungliga Tekniska Hogskolan on 08/11/15 For personal use only.
Discussion A fistula usually forms due to leakage from a ruptured ovarian cyst that induces a dense adhesion between the cyst and the bowel. Leakage of cystic fluid is thought to be caused by torsion, trauma, malignant transformation or infection (Okada et al. 2005). Of the provoking factors, ovarian torsion is the most frequent complication of MCT, occurring in 16.1% of cases in one large case series (Peterson et al. 1955) and tends to predispose the MCT to rupture (Malkasian et al. 1965). The MCT may rupture into adjacent organs, most frequently, the bladder or the rectum. Malignant transformation may cause a fistula to form, but it is not an essential factor in fistula formation (Okada et al. 2005). Most studies have demonstrated that leakage of cystic fluid is the primary factor in fistula formation. Although formation of an entero-ovarian fistula due to a ruptured MCT is well documented (Conway and Hanna 2012), a preoperative colonoscopical assessment has not been reported. In our case, the malignant transformation of the MCT was detected by colonoscopy, whereas a previous radiological finding found that primary colon cancer was more likely to be detected, as opposed to a tumour arising from an ovarian cyst. It is thought that a large colo-ovarian fistula can be mistaken for the true colonic lumen on abdominal CT, as the radiological differential diagnosis between primary colon cancer and a fungating mass of MCT remains difficult. The pathophysiology of fistula formation is not well understood. It is hypothesised that a mostly sebaceous cyst’s contents slowly leak into the abdominal cavity through a small rupture site, leading to a chronic inflammatory response (von-Walter and Nelken 2012). However, the inflammatory reaction was minimal in our case, presenting as a colo-ovarian adhesion with a fistula. It is possible that the leakage
was due to small spontaneous ruptures which ultimately progressed towards a chronic event, such as a fistula. MCTs often exhibit malignant transformations of a constituent tissue, most frequently the squamous epithelium, followed by the formation of a typical SqCC. A previous study demonstrated various prognostic factors for MCT fistulas, including tumour stage, size, age and CA-125 (Chen et al. 2008). In summary, our case raises awareness that unusual clinical findings are important and require earlier consideration and pre-surgical planning in order to provide the best possible outcome.
Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.
References Chen RJ, Chen KY, Chang TC et al. 2008. Prognosis and treatment of squamous cell carcinoma from a mature cystic teratoma of the ovary. Journal of the Formosan Medical Association 107:857–868. Conway AZ, Hanna T. 2012. A dermoid cyst fistulating with the transverse colon. Annals of The Royal College of Surgeons of England 94:e230–231. Dos Santos L, Mok E, Iasonos A et al. 2007. Squamous cell carcinoma arising in mature cystic teratoma of the ovary: a case series and review of the literature. Gynecoloic Oncology 105:321–324. Kikkawa F, Ishikawa H, Tamakoshi K et al. 1997. Squamous cell carcinoma arising from mature cystic teratoma of the ovary: a clinicopathologic analysis. Obstetrics & Gynecology 89:1017–1022. Malkasian GD, Symmonds RE, Dockerty MB. 1965. Malignant ovarina teratomas. Report of 31 Cases. Obstetrics & Gynecology 25:810–814. Okada S, Ohaki Y, Inoue K et al. 2005. A case of dermoid cyst of the ovary with malignant transformation complicated with small intestinal fistula formation. Radiation Medicine 23:443–446. Peterson WF, Prevost EC, Edmunds FT et al. 1955. Benign cystic teratomas of the ovary; a clinico-statistical study of 1,007 cases with a review of the literature. American Journal of Obstetrics & Gynecology 70:368–382. von-Walter AR, Nelken RS. 2012. Benign cystic ovarian teratoma with a fistula into the small and large bowel. Obstetrics & Gynecology 119:434–436.
