LETTER TO EDITOR
Year : 2010 | Volume
: 53 | Issue : 1 | Page : 178--180
Pure primary non-gestational ovarian choriocarcinoma: A diagnostic dilemma
Sonia Gon, Bipasa Majumdar, Gopinath Barui, Rupam Karmakar, Aparna Bhattacharya
Department of Pathology, R G Kar Medical College, Kolkata, India
Flat no 1B, 23, Park Side Road, Kolkata-700 026
|How to cite this article:|
Gon S, Majumdar B, Barui G, Karmakar R, Bhattacharya A. Pure primary non-gestational ovarian choriocarcinoma: A diagnostic dilemma.Indian J Pathol Microbiol 2010;53:178-180
|How to cite this URL:|
Gon S, Majumdar B, Barui G, Karmakar R, Bhattacharya A. Pure primary non-gestational ovarian choriocarcinoma: A diagnostic dilemma. Indian J Pathol Microbiol [serial online] 2010 [cited 2021 Jan 21 ];53:178-180
Available from: https://www.ijpmonline.org/text.asp?2010/53/1/178/59226
Germ cell tumors of the ovary include all neoplasm derived from primordial germ cells of the embryonal gonad. Five per cent of germ cell tumors are malignant, representing three to five per cent of all ovarian carcinomas of which pure primary non-gestational ovarian choriocarcinoma accounts for less than one per cent of ovarian tumors.  They pose diagnostic challenges in reproductive age group patients because of elevated human chorionic gonadotrophin (hCG). We report a case of pure primary non-gestational ovarian choriocarcinoma mistaken for a twisted ovarian cyst clinically, resulting in a delay in diagnosis.
A 21-year old unmarried female, presented with right lower quadrant pain and a palpable abdominal lump. She underwent a sonographic examination for a possible twisted ovarian cyst. It revealed a normal appearing uterus without evidence of an intrauterine pregnancy, a gestational sac, or decidual reaction. The right ovary was enlarged with heterogeneous hyperechoic mass measuring 149mm x 120mm x 80 mm. No fetal cardiac activity was identified, and the findings were believed to be most consistent with an ovarian tumor. The serum ß-HCG level was markedly elevated at 279,000 IU/L. Pre-operative LDH levels, CA-125 and alpha-fetoprotein were 1595U/ml, 67.15U/ml and 1.98 U/ml respectively. A right sided salpingo-ophorectomy was done and the specimen was sent for histopathological examination.
On gross examination, the ovarian mass was well circumscribed, well encapsulated and measured 15 cms in its greatest dimensions. The cut surface was predominantly solid with extensive areas of hemorrhage and degenerative changes [Figure 1]. Microscopically, atypical syncytio-cytotrophoblastic cells amidst areas of hemorrhage and necrosis were present. No evidence of fetal tissue or chorionic villi was present [Figure 2]. Further blocks failed to demonstrate presence of other neoplastic germ cell component.
Primary choriocarcinoma of ovary could be gestational or non-gestational in origin. The distinction between the two is difficult, but necessary, as the non-gestational type has bad prognosis.  Most non-gestational choriocarcinoma of ovary occur in admixture with teratoma, endodermal sinus tumor, embryonal carcinoma or dysgerminoma. The most common primary site of choriocarcinoma is intrauterine. Primary extra-uterine choriocarcinoma has been reported to occur in the fallopian tube, ovary, and elsewhere in the abdomen and pelvis.  The diagnosis of primary extra uterine choriocarcinoma is challenging because the clinical symptoms are often nonspecific and can mimic other, more common conditions that occur in young women, such as a hemorrhagic ovarian cyst, tubo-ovarian abscess, ovarian torsion, and ectopic pregnancy. 
Saito et al.  in 1963, first described the diagnostic criteria for non-gestational choriocarcinoma which include absence of disease in the uterine cavity, pathological confirmation of disease, exclusion of molar pregnancy and exclusion of coexistence of intrauterine pregnancy. All the criteria were fulfilled in this case.
Distinction of non-gestational choriocarcinoma from gestational choriocarcinoma is impossible on histomorphology unless an evidence of pregnancy or other germ cell neoplasms is encountered. There are no ultra structural or immunohistochemical features unique to either. DNA polymorphism analysis using two or three appropriate VNTR loci from the tumor and the patient for identification of paternal sequences establishes the diagnosis of gestational or non-gestational choriocarcinoma. It is important to distinguish the two since non-gestational choriocarcinomas have been found to be resistant to single agent chemotherapy, have a worse prognosis, and therefore require aggressive combination chemotherapy. 
Pure primary non-gestational choriocarcinoma of the ovary is extremely rare and a diagnostic dilemma. However, distinction between gestational and non-gestational choriocarcinoma is necessary in view of prognostic value and management.
|1||Scully RE. Tumors of the ovary and mal-developed gonads. In: Hartmann WH, ed. Atlas of tumor pathology. Washington, DC: Armed Forces Institute of Pathology; 1979. p. 243-5. |
|2||Crasta JA, Mishra SK. Primary Choriocarcinoma of the Ovary- A Case Report. Journal of clinical and diagnostic research 2008;2:1207-9.|
|3||Mohammad Ali Roghaei, Farimah Rezaei and Parvin Mahzuni. A pure nongestational choriocarcinoma of the ovary. J Res Med Sci 2007;12:269-70.|
|4||Gerson RF, Lee EY, Gorman E. Primary Extrauterine Ovarian Choriocarcinoma Mistaken for Ectopic Pregnancy: Sonographic Imaging Findings. AJR 2007;189:280-3.|
|5||Saito M, Azuma T, Nakamura K. On ectopic choriocarcinoma. World Obstet Gynecol 1963;17:459-84.|