Case Study: Non-gestational ovarian choriocarcinoma in a young female

By Dr. Moushumi Lodh, Dr. Abhishek Mukherjee and Dr. Ratnadeep Ganguly

Summary

We describe a case of non-gestational ovarian carcinoma with omental and central nervous system metastasis in a twenty-two year old woman.

Background

The symptoms of extra-uterine choriocarcinoma mimic common disorders of young women, such as an ovarian cyst, abscess, torsion, or ectopic pregnancy. It is imperative then, that it is considered a differential diagnosis when the patient presents with clinical findings of ectopic pregnancy, especially with elevated β human chorionic gonadotropin.

Case Presentation

Choriocarcinoma commonly metastasizes to the lungs, brain and liver. Non-gestational choriocarcinoma constitutes 0.6% or less of all ovarian neoplasms. (1) Ovarian choriocarcinoma is an extremely rare tumor arising from germ cells, accounting for fewer than 5% of all ovarian cancers diagnosed in Western countries. (2)

The diagnostic criteria for non-gestational choriocarcinoma of the ovary include exclusion of molar pregnancy, intrauterine pregnancy, intrauterine disease and pathological confirmation of the disease. (3) The central nervous system metastases of the choriocarcinoma are seen in 10-20% of cases, (4) and may present as arteriovenous malformation bleed, parenchymal or subarachnoid hemorrhage from rupture of neoplastic aneurysms, carotid cavernous fistula, infarct due to tumor embolus or a subdural hematoma. (5)

Our patient, a twenty-two-year-old was brought to the emergency department of our hospital with a history of unconsciousness and sudden fall. Her pupils were responding sluggishly, and she was not able to move her right limbs when she arrived in the emergency department, although her left limbs were showing spontaneous movements. There was no external injury and no history of convulsions. For the last four days, she had been complaining to her family of a persistent headache with intermittent vomiting. For the last four months, she had irregular periods and lower abdominal pain. The patient had delivered a healthy baby five years ago, by normal vaginal delivery.

No complaints of lethargy, poor appetite, or marked weight loss existed. There was no history of prolonged oral contraceptive use, or any prolonged medication or surgery for any other ailment. There was no family history of molar pregnancy, abortion or consanguineous marriage.

Her blood pressure was 120/60 mm Hg, heart rate was 68/minute, respiratory rate was 24/minute, and oxygen saturation in room air was 94%. Heart sounds were normally audible, and breath sounds were bilateral and vesicular. Her abdomen was soft but had a firm, mobile mass of about 10×7 cm, in the right adnexal region. The ultrasound confirmed the mass to be an 11×7 cm solid lesion, probably arising from the ovary. The other pelvic organs, including the uterus, were found to be normal, and there was no gestational sac or evidence of hydatidiform mole.

Investigations

Her laboratory tests were as follows: hemoglobin level 9.3 grams/dl, total WBC count 18,900/cu mm associated with [s2If !is_user_logged_in()]…

[/s2If][s2If is_user_logged_in()] neutrophilia. Coagulation profile, random plasma glucose, serum electrolyte, bicarbonate, blood urea nitrogen, serum creatinine, and liver enzymes were within the reference ranges. Serum lactate dehydrogenase was high, at 1,165 U/L. Tumor markers CA 19.9 and CA 15.3 were normal at 6 U/ml and 16.5 U/ml respectively. Alpha fetoprotein was 1.2 U/L and CA 125 was 24.56 U/L. Urine human chorionic gonadotropin was 191,000 IU/L and serum human chorionic gonadotropin (HCG) was 447,500 IU/L, estimated by chemiluminescence immunoassay (Siemens Centaur). The plasma/spinal fluid HCG ratio was 189. Chest X-ray was clear. Computed tomography scan of the brain revealed a left basal ganglia bleed 4×4.5 cm, with a midline shift of 7 mm. The patient then underwent temporoparietal craniotomy and evacuation of the bleed at 2 days after admission and then explorative laparotomy five days after admission. The right ovarian mass, omentum and endometrial curretings were removed and sent to the laboratory for histopathological evaluation. Post-surgery, HCG levels came down to 22,600 IU/L.

Macroscopically, the right ovarian mass, measuring 13x8x5 cm showed extensive areas of hemorrhage and necrosis with focal cystic areas. (See Figure 1.) No obvious nodularity was palpable in the overall specimen. The uterus had an unremarkable morphology without any evidence of gestation or hydatidiform mole.
Figure 1A .Macroscopic Picture of the tumor and 1B.Cut surface showing necrosis

Figure 1: A.) Macroscopic appearance of the tumor and B.) Cut surface showing necrosis

Microscopically, we found extensive areas of hemorrhage, necrosis, several nests of malignant trophoblastic cells of various sizes having bilaminar cytotrophoblastic and syncytiotrophoblastic proliferation, and showing significant nuclear pleomorphism, and coarse clumped chromatin with presence of binucleate and multinucleate tumor giant cells. (See Figure 2.) The omental tissue fragment received, measuring 12×7.5×1.5 cm, showed extensive areas of necrosis. Sections from omental tissue showed aggregates of tumor cells of the same morphology as described above, in a background of fibrofatty tissue with extensive areas of hemorrhage and necrosis. Sections from endometrial curretings showed late secretory phase changes of the endometrium. There was no decidualization. We confirmed its nongestational origin by DNA polymorphism analysis at 15 short tandem repeat loci.
FIG 2.giant cells

Figure 2: Tumor showing binucleate and multinucleate tumor giant cells

Treatment, Outcome and Follow Up

The patient was referred to a specialized oncology setup for treatment.

Discussion

Primary extra uterine choriocarcinoma can mimic other, more common conditions like ectopic pregnancy, hemorrhagic ovarian cyst, tubo-ovarian abscess, or ovarian torsion. (6)

Choriocarcinoma presents as a primary choriocarcinoma associated with ovarian pregnancy or as a metastatic choriocarcinoma from a primary gestational choriocarcinoma arising in the uterus or other parts of the genital tract. It may also arise as a germ cell tumor, differentiating in the direction of trophoblastic structures and admixed with other neoplastic germ cell elements. This is referred to as non-gestational choriocarcinoma. (7) 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. (8)

Learning Points/Take Home Messages

Although rare, choriocarcinoma is a differential diagnosis in cases resembling ectopic pregnancy.

About The Author

M LodhDr. Moushumi Lodh, M.B.B.S, M.D Biochemistry. Laboratory Services, The Mission Hospital, Durgapur.

 

 

References (click to show/hide)

  1. Park SH, Park A, Kim JY, Kwon JH, Koh SB. A case of non-gestational choriocarcinoma arising in the ovary of a postmenopausal woman. JGynecoOncol. 2009 ;20(3): 192-194.
  2. Çorakçi A, Özeren S, Özkan S, Gürbüz Y, Üstün H, Yücesoy I. Pure nongestational choriocarcinoma of ovary. Archives of Gynecology and Obstetrics 2005; 271: 176-177.
  3. Choi YJ, Chun KY, Kim YW, Ro DY. Pure nongestational choriocarcinoma of the ovary: a case report. World Journal of Surgical Oncology 2013; 11(7):1-3.
  4. Bhatia K, Vaid AK. Diagnostic Dilemma: Non-gestational or Gestational Choriocarcinoma of the Ovary. JAPI 2008;56: 1001-2.
  5. Shrestha P, Devkota UP, Panth R. Metastatic choriocarcinoma masquerading as an intracerebral arteriovenous malformation bleed. The Internet Journal of Neurosurgery 2010; 7 (2).
  6. Gon S, Majumdar B, Barui G, Karmakar R, Bhattacharya. A Pure primary nongestational ovarian choriocarcinoma: A diagnostic dilemma. Indian J Pathol Microbiol 2010; 53: 178-80.
  7. Yamamoto E, Ino K, Yamamoto T, Sumigama S, Nawa A, Nomura S, Kikkawa F. A pure nongestational choriocarcinoma of the ovary diagnosed with short tandem repeat analysis: case report and review of the literature. Int J Gynecol Cancer. 2007;17(1):254-8.
  8. Hirata Y, Yanaihara N, Yanagida S, Fukui K, Iwadate K, Kiyokawa T, Tanaka T. Molecular genetic analysis of nongestational choriocarcinoma in a postmenopausal woman: a case report and literature review. Int J Gynecol Pathol. 2012 Jul; 31(4):364-8.


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