Ovarian choriocarcinoma is an extremely rare form of ovarian cancer.[1] It can be broadly classified into two variants, gestational ovarian choriocarcinoma (GOC) and non-gestational ovarian choriocarcinoma (NGOC). NGOC are further subdivided into mixed, which contain other germ cell components, and pure subtypes, which contain only choriocarcinoma. The incidence of GOC is 1:369,000,000 whereas the incidence of NGOC accounts for just 0.6% of malignant ovarian germ cell tumors. [2-6]

This study aimed to describe a case of mixed non-gestational ovarian choriocarcinoma.

Case Report:

A 7year old prepubertal amenorrhoeic girl presented in March 2023 at our OPD with a history of lower abdominal pain and swelling for past 3 months, which were progressive in nature. She was admitted in the emergencies with a history of Acute Abdomen. CECT of Whole Abdomen done on 20/04/2023, it shows 14x12x9 cm large Heterogeneous enhancing solid lesion in mid, lower abdomen and pelvis compressing the bowel loops with proximal small bowel obstruction. Blood reports on 16/04/2023, AFP- 213, LDH- 2194, BetaHCG- 4145, CA-125: 96.7, CEA- 0.71, CA 19-9: 21.2

Figure 1: CECT scan of Pelvis (preoperative) showing a pelvic SOL compressing Bowel loops (arrow).

She was first managed conservatively and then underwent laparotomy with Cytoreductive intention with Excision of Left Ovarian Mass with Left Salpingo- oophorectomy, Metastatectomy (Excision of visible peritoneal mets, parts of mesentery, Omentum, Rectovaginal pouch, Fallopian Tube (Left), part of Sigmoid colon) on 21/04/2023 and all were sent for histopathological examination.

The post operative period was uneventful and she recovered well.

Grossly the tumor was hemorrhagic and necrotic. Histopathological examination revealed a thin rim of ovarian parenchyma with a tumor revealing wide areas of hemorrhage, necrosis with intervening solid sheets as well as peripheral riming of tumor cells around hemorrhagic and necrotic foci. The malignant cells were of four components: Yolk sac tumor (60%), immature teratoma (20%), dysgerminoma (18%) and choriocarcinoma (2%).

Figure 2: Histopathology of the tumour showing (A) Yolk sac, (B) Immature Teratoma, (C) Dysgerminoma and (D) Choriocarcinoma

Immunohistological Report (26/04/2023) from the FFPE block showed SALL4 and CK Positive for Yolk Sac Component; OCT3/4, D240 and C-KIT weak positive in Dysgerminoma component.

Figure 3: MRI brain (sagittal view) showing calverial metastasis

On Post operative evaluation, CECT THORAX on 21/5/23 did not reveal any obvious lesion. CECT Brain on 9/5/23, showed an Extra calvarial nonenhancing mild soft tissue swelling in right occipital (16X8mm) and mild bony erosion adjacent to soft tissue swelling. CECT whole Abdomen on 21/5/23 showed a Left paraaortic nodal mass(30x22mm) with Multiple vertebral and pelvic bony metastatic lesions, mild right hydronephrosis and bowel distension Considering a metastatic disease, she was then planned for multiagent chemotherapy. Unfortunately, the patient was lost to follow up for around 2 months.

Figure 4: CECT whole abdomen (axial view), Postoperative (after few months of defaulter) showing huge intraabdominal SOL almost occupying whole abdomen.

Then she again presented with huge abdominal distension and was unable to walk. She was again evaluated for the disease status. The Blood parameters, as on 01/08/2023, were: BetaHCG -> 96120 mIU/ml, CA-125 –> 207.5 U/ml, Sr LDH -> 4180 U/L, AFP –> more than 1000 ng/ml. CECT scan Thorax and Abdomen on 07.08.2023 showed, Large heterogenous enhancing SOL of 8.1 x 6.3 x 6.1 CM in POD, invading and incaving the ano-rectum region with bilateral pleural effusion.

Figure 5: CECT whole abdomen (sagittal view), Postoperative (after few months of defaulter) showing huge intraabdominal SOL almost occupying whole abdomen along with multiple vertebral metastases

Patient was planned for Chemotherapy as per decision made in the previous Tumor Board with EP regimen with Inj. Etoposide (90mg) D1, D2, D3 and Inj Carboplatin (300mg) in D1 IV

Chemotherapy was started on 08.08.2023, but was inadvertently stopped after an infusion of only 20 ml of INJ ETOPOSIDE (Inj Etoposide 90mg was dissolved in 500ml NS) due to sudden onset severe respiratory distress and hypertension. She was then shifted to Pediatric Intensive care unit (PICU). She was managed effectively with the help of Pulmonologists and CardioThoracic surgeons. After stabilization 1^st cycle of the planned regimen of the Chemotherapy was administered under PICU setup on 11.08.2023 with a very high risk informed consent. She was then stepped down to HDU and then to general ward with improving vitals.

Figure 6: Presentation of the said patient initially after few months of defaulter (bottom) and after inital 2cycles of Chemotherapy with improved symptoms (top)

She had completed another 2 cycles of Etoposide and Carboplatin in due course till October 2023. She was improving gradually both symptomatically as well as biochemically, though the AFP level was persistently high.

Figure 7: Chronological order of Tumour markers

During this period of time there is reduction of body weight about 9 kg which was mainly due to regression of the tumour and ascitic fluids. Her ECOG PS also had improved along with blood level markers changes as:

But after the 3^rd cycle, on follow up she developed recurrent infection, scar line tenderness, on examination a large pedunculated mass was found to be coming out per vagina.

Figure 8: Disease progression with per vaginum