Primary Ovarian Choriocarcinoma: Rare Entity

Abstract

Background. Primary pure ovarian choriocarcinoma is a rare aggressive tumor which can be nongestational arising from germ cells or gestational origin. Preoperative diagnosis of extrauterine choriocarcinoma is challenging due to nonspecific clinical presentation. Case Presentation. This article reports primary ovarian choriocarcinoma, likely gestational in a 25-year-old para 2 woman presenting with lower abdominal pain and swelling of two-week duration. Diagnosis was suspected by serum beta-human chorionic gonadotropin and confirmed histologically after surgery. Postoperatively, she was managed with multiple courses of chemotherapy using a bleomycin, etoposide, and cisplatin regimen, and the treatment was effective. Conclusion. In patients with adnexal mass presenting with nonspecific symptoms especially with high Doppler blood flow of the mass on ultrasound evaluation, serum beta-human chorionic gonadotropin determination is recommended before laparotomy. In setups where the genomic test is not available, histological and clinical effort to differentiate gestational versus nongestational choriocarcinoma is useful for specific management decision.

Non-Gestational Ovarian Choriocarcinoma: A Rare Ovarian Cancer Subtype

“…In the largest case review done by Lui et al, 39 case reports were reviewed with the peak age of onset ranging 12 to 25 years [13]. The most common signs and symptoms are vaginal bleeding, abdominal pain, adnexal mass on ultrasound, and a positive pregnancy test [6,11,13,14,22]. In children, initial presentation may also include precocious puberty [23].…”

“…At beta-HCG levels seen in NGOC cases, the Hook Effect becomes relevant [32]. When using point of care urine pregnancy tests, the Hook Effect occurs in sandwich immunoassays when the antigen concentration is high enough to saturate both the migratory phase and fixed detection antibodies independently, rather than binding occurring to subunits of the same molecule [14,15]. Thus, a falsely negative test could potentially lead to a delay in diagnosis and allow for progression of disease [33].…”

“…This can be overcome by diluting the sample, either 1:10 or 1:100, to allow for dilution of the beta-HCG [34]. However, serum-based beta-HCG tests are not subject to the Hook Effect and will reveal the true elevation of beta-HCG, which has been reported to be as high as 1 million [14].…”

“…There are two types of NGOC: the pure type and the mixed type. The pure type, which is extremely rare, only contains choriocarcinoma [14]; the absence of other germ cell elements is demonstrated by the lack of immunohistochemical staining for CD30, PLAP, and AFP [15]. The mixed type contains other germ cell tumors, including immature teratomas, endodermal sinus tumors, embryonal carcinomas, and dysgerminomas [13].…”

“…Histologically, both GOC and NGOCs have identical presentation with abnormal trophoblastic hyperplasia and anaplasia, absence of chorionic villi, high proliferative index, and the presence of hemorrhage and necrosis within the tumor tissue [2,16]. The histologic similarities between GOC and NGOC contribute to the diagnostic conundrum, see Table 1 [2,14,17]. One study compared the genetic molecular biology of GOC and NGOC, and found mutations involving DNAJB9, a negative feedback regulator of p53 and NGOC cells showing aberrant expression of p53 [18].…”