Large cell neuroendocrine carcinoma arising in mature cystic teratoma: a case report and review of the literature

Background: Malignant transformation of ovarian mature cystic teratoma is rare, and occurs in approximately 1% of all cases. The most common histology arising in mature cystic teratoma is squamous cell carcinoma. Less frequently, malignant transformation is represented by an endocrine tumor. To date, only five cases of large cell neuroenclocrine carcinoma (LCNC) arising in a mature cystic teratoma of the ovary have been reported. Clinical case: A 69-year-old woman presented with a 15-cm left ovarian mass, and was diagnosed with Stage IV large cell carcinoma neuroendocrine carcinoma (LCNC) arising in mature cystic teratoma (MCT) of the left ovary. The patient received adjuvant chemotherapy with paclitaxel and carboplatin, however, residual tumors increased in size. Six months after the debulking surgery she succumbed to the disease. A literature review revealed LCNC of the ovary showed excessively aggressive phenotype in malignant transformation from ovarian mature cystic teratoma. Conclusion: The present case of LCNC arising in MCT had an exceedingly poor prognosis, which was suggested in the previous five cases reported.

Ovarian tumor; Mature cystic teratoma; Large cell neuroendocrine carcinoma; Paclitaxel; Carboplatin

[1] Hackethal A., Brueggmann D., Bohlmann M.K., Franke F.E., Tinneberg H.R., Münstedt K.: “Squamous-cell carcinoma in

mature cystic teratoma of the ovary: systematic review and analysis of published data”. Lancet Oncol., 2008, 12, 1173.

[2] Sakuma M., Otsuki T., Yoshinaga K., Utsunomiya H., Nagase S., Takano T. et al.: “Malignant transformation arising from mature cystic teratoma of the ovary: a retrospective study of 20 cases”. Int. J. Gynecol. Cancer, 2010, 20, 766.

[3] Hirasawa T.: “Ovarian neuroendocrine carcinoma associated with mucinous carcinoma and teratoma”. Nippon Rinsho, 2004, 62, 973.

[4] eras E., Deavers M.T., Silva E.G., Malpica A.: “Ovarian nonsmall cell neuroendocrine carcinoma: a clinicopathologic and immunohistochemical study of 11 cases”. Am. J. Surg. Pathol., 2007, 31, 774.

[5] Chênevert J., Bessette P., Plante M., Têtu B., Dubé V.: “Mixed ovarian large cell neuroendocrine carcinoma, mucinous adenocarcinoma, and teratoma: a report of two cases and review of the literature”. Pathol. Res. Pract., 2009, 205, 657.

[6] Chen K.T.: “Composite large-cell neuroendocrine carcinoma and surface epithelial-stromal neoplasm of the ovary”. Int. J. Surg. Pathol., 2000, 8, 169.

[7] Ohira S., Itoh K., Shiozawa T., Horiuchi A., Ono K., Takeuchi H. et al.: “Ovarian non-small cell neuroendocrine carcinoma with paraneoplastic parathyroid hormone-related hypercalcemia”. Int. J. Gynecol. Pathol., 2004, 23, 393.

[8] Collins R.J., Cheung A., Ngan H.Y., Wong L.C., Chan S.Y., Ma H.K.: “Primary mixed neuroendocrine and mucinous carcinoma of the ovary”. Arch. Gynecol. Obstet., 1991, 248, 139.

[9] Jones K., Diaz J.A , Donner L. R.: “Neuroendocrine carcinoma arising in an ovarian mucinous cystadenoma”. Int. J. Gynecol. Pathol., 1996, 15, 167.

[10] Eichhorn J.H., Lawrence W.D., Young R.H., Scully R.E.: “Ovarian neuroendocrine carcinomas of non-small-cell type associated with surface epithelial adenocarcinomas. A study of five cases and review of the literature”. Int. J. Gynecol. Pathol., 1996, 15, 303.

[11] Choi Y.D., Lee J.S., Choi C., Park C.S., Nam J.H.: “Ovarian neuroendocrine carcinoma, non-small cell type, associated with serous carcinoma”. Gynecol. Oncol., 2007, 104, 747.

[12] Ahmed Z., Aftab K., Kayani N.: “Ovarian primary neuroendocrine carcinoma of non-small cell type: report of an extremely rare neoplasm”. J. Pak. Med. Assoc., 2005, 55, 82.