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CASE REPORT
Year : 2015  |  Volume : 18  |  Issue : 5  |  Page : 16-19

Choriocarcinoma with cerebral metastasis presenting as a stroke-like lesion


Department of Obstetrics and Gynaecology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, Nigeria

Date of Web Publication19-Jan-2015

Correspondence Address:
C Nnadi Daniel
Department of Obstetrics and Gynaecology, Usmanu Danfodiyo University Teaching Hospital, Sokoto PMB 2370, Sokoto
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1118-8561.149498

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  Abstract 

Choriocarcinoma is a very aggressive malignant variant of gestational trophoblastic disease characterized by malignant proliferation of trophoblastic tissues and absence of chorionic villi. We present a rare case of metastatic choriocarcinoma presenting with a stroke-like lesion. A 40-year old multiparous woman that presented with bleeding per vaginam, right-sided facial nerve palsy and hemiparesis following eight weeks of amenorrhea. Serum β-hCG was elevated and urine pregnancy test was positive in neat and in serial dilution. She was managed as a case of metastatic choriocarcinoma and had five courses of the EMA-CO drug regimen. She made rapid neurological recovery and was discharged in good condition. We conclude that metastatic choriocarcinoma may present in bizarre forms. It should be suspected in any woman presenting with abnormal vaginal bleeding, unusual systemic physical features and raised serum β-hCG. We conclude that combination chemotherapy is usually effective even in metastatic choriocarcinoma.

Keywords: Choriocarcinoma, cerebral metastasis, chemotherapy


How to cite this article:
Daniel C N, Ango I G, Nwobodo E I. Choriocarcinoma with cerebral metastasis presenting as a stroke-like lesion. Sahel Med J 2015;18, Suppl S1:16-9

How to cite this URL:
Daniel C N, Ango I G, Nwobodo E I. Choriocarcinoma with cerebral metastasis presenting as a stroke-like lesion. Sahel Med J [serial online] 2015 [cited 2024 Mar 28];18, Suppl S1:16-9. Available from: https://www.smjonline.org/text.asp?2015/18/5/16/149498


  Introduction Top


Choriocarcinoma is a very aggressive malignant variant of gestational trophoblastic disease characterized by malignant proliferation of trophoblastic tissues and absence of chorionic villi. [1] It may follow a molar pregnancy (50%), term pregnancy (20-30%), abortion (20%), and ectopic pregnancy (2%). Some cases of Choriocarcinoma have been known to arise de novo as a germ cell tumour. [2],[3] The incidence varies from 1 in 40,000 pregnancies in the USA to 0.87 and 0.8 per 1000 deliveries in Nigeria and Ghana respectively. [4] The aetiology is unknown; however there are associated risk factors. These include extremes of age, previous molar pregnancy, abortion or term pregnancy. It is also associated with low socio-economic class, deficiency in carotene, protein and animal fat. [5],[6] They are characterized by early and widespread metastases via the blood stream.

Common sites of metastases include the vagina, lungs, liver and distant organs like the brain, but metastasis has been described in virtually every part of the body like the eyes, fingers and in the gastro-intestinal tract.

We present a case of metastatic choriocarcinoma presenting with vaginal bleeding, and left sided hemispheric metastasis. The patient was managed with the EMA-CO drug regimen and had marked improvement.


  Case Report Top


Mrs. MI was a 40-year old P8+1 6alive house wife who presented to the gynaecological out-patient clinic on 19/10/10 with a 2-month history of recurrent bleeding per vaginam, following eight weeks of amenorrhoea. The bleeding was moderate in quantity and dark red in colour. There was no passage of vesicles or fleshy materials. It was associated with lower abdominal pain. There was no vomiting, easy fatigability, headache or dizziness. She did not complain of post-coital bleeding or dyspareunia. There were no syncopal attacks, gastro-intestinal or respiratory symptoms. She had presented earlier at the Specialist hospital, where she had uterine evacuation for suspected incomplete abortion. The vaginal bleeding however persisted necessitating a repeat evacuation of the uterus. A post- evacuation histological report revealed Choriocarcinoma. She was subsequently referred to our hospital Usmanu Danfodiyo University Teaching hospital. While on admission, the patient developed slurred speech and right-sided facial nerve palsy of the lower motor neuron type with associated weakness of the right upper limb. There was no loss of consciousness. The patient was not a known diabetic or hypertensive patient. She had uterine evacuation for complete Hydatidiform mole at the Usmanu Dan-fodio University Teaching Hospital, Sokoto in 2008. She defaulted in post-evacuation follow-up appointments.

Physical examination revealed a fully conscious patient who was afebrile to touch and anicteric. She weighed 70 kg and was 1.60m tall. The pulse rate was 80 beats per minute and the blood pressure was 120/80 mmHg. She was well oriented in person place and time. There was a right sided facial nerve palsy of the lower motor neuron type. The muscle bulk was normal, but there was decreased tone on the right upper limb. The power was 4/5 with increased deep tendon reflexes on the affected side. The chest was clinically clear. The abdomen was full and moved with respiration and there were no areas of tenderness. The uterus was about 13 weeks size. Vaginal examination revealed a normal vulva and vagina and a normal cervix. The cervical Os was closed. The uterus was bulky and there were no palpable adnexal masses. The assessment was that of Choriocarcinoma with cerebral metastasis in a multiparous woman.

The patient and her relations were counseled. A urine pregnancy test was positive in the neat sample of urine and in serial dilution of 1 in 150. The serum β-hCG was >10,000 mIU/mL. The full blood count, serum electrolytes and urea including the liver function test were essentially within normal limits. The blood clotting profile and the fasting blood sugar were within the normal range. A chest radiograph showed multiple opacities within the lung fields. A computerized tomography (CT) scan done revealed acute multiple intra-cerebral hemorrhages and mottled areas of opacity as seen in [Figure 1], [Figure 2] and [Figure 3].
Figure 1: The radio-opaque area in the right upper quadrant shows the site of the lesion

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Figure 2: Mid-section view of the lesion

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Figure 3: The lower extent of the lesion

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She was commenced on Etoposide, Methotrexate, Actinomycin-D, Cyclophosphamide and Vincristine (EMA-CO) regimen. She had 5 courses of the regimen and made a rapid recovery. The serum β-hCG declined steadily and fell to 3.9 mIU/mL by the 4 th course of EMA-CO; thereafter she had 2 more courses administered. During the course of therapy however, she was transfused with 2 units of blood when the packed cell volume fell to 27%. The patient made rapid neurological recovery. She was counseled on contraceptives and she chose tubal ligation. She was subsequently discharged and was given a follow-up appointment at the out-patient clinic.


  Discussion Top


The risk factor for choriocarcinoma in the case presented was a previous molar pregnancy which the patient had in 2008. Choriocarcinoma is suspected when there is persistent or irregular uterine haemorrhage, following spontaneous abortion or evacuation of a normal pregnancy or a hydatidiform mole.

The clinical presentation depends on the stage of the disease. In early cases, the main presentation is vaginal bleeding, while metastatic Choriocarcinoma may present with symptoms related to the organ involved. Pulmonary embolization of the tumour may present with haemoptysis, while headache and neurological features suggest cerebral metastases. The diagnosis of Choriocarcinoma in a patient presenting with symptoms from distant metastases may be challenging.

About 4% of patients presenting with trophoblastic disease have cerebral metastasis at the time of diagnosis. However, in contrast with other malignancies related to cerebral metastases, trophoblastic disease patients with central nervous system (CNS) disease can routinely be cured of the disease. Metastatic choriocarcinoma presenting with intra-cerebral haemorrhage has been reported in the United States of America (USA) and Netherlands. [7],[8] In India, Choriocarcinoma presenting with brain metastases, raised intra-cranial pressure and small bowel perforation was seen in a 24-year old housewife. [9]

Our patient presented with vaginal bleeding, right-sided facial nerve palsy and hemiparesis involving the right upper limb.

The development of brain metastasis can be divided into 3 stages; [10]

Stage one, also referred to as the initial stage or stage of embolism is characterized by sudden appearance of neurologic symptoms such as headache, vomiting, sensory or motor, mental or visual disturbances. These symptoms disappear after a few minutes or hours or days. Stage two or progressive stage or stage of brain tumour formation, is due to invasion of brain parenchyma by tumour cells to form a real tumour in the brain. It is characterized by re-appearances of previous symptoms but of progressive nature with rapid deterioration of the condition of the patient. The terminal stage or stage 3 is also known as the stage of brain herniation. The patient at this stage usually dies suddenly of respiratory arrest. Our patient most likely presented in stage 1 and this partly explains the good outcome.

The diagnosis of choriocarcinoma may be based mainly on clinical presentation and elevated serum β-hCG levels with or without confirmatory histology.

Choriocarcinoma is highly sensitive to chemotherapy with a response rate ranging from 60-100% depending on the stage of the disease. Cerebral metastasis is considered as a poor prognostic factor and qualifies for combination chemotherapy. In low-risk patients with non-metastatic disease, single agent chemotherapy with Methrotrexate or Actinomycin-D is mainly used. For high-risk metastatic disease, combination chemotherapy with EMA-CO is advocated. Other alternatives including EMACE (Etoposide, Methotrexate, Cyclophosphamide Etoposide), EMA-EP (Etoposide, Methotrexate, Actinomycin D-Etoposide, cis-platinium) and VIP (vincristine, ifosfamide, cis-platinium) etc., may be used in resistant cases. The EMA-CO combination therapy was introduced in 1986, and became widely used thereafter because of its efficay. [11] It has remained the standard of care for the primary the treatment of high-risk gestational trophoblastic neoplasia (GTN) [12] and has replaced earlier regimens such as methotrexate, actinomycin-D, Cyclophosphamide (MAC) and other combinations. [12] Tumour resection and whole brain irradiation (WBRT) is occasionally done for drug resistant cases. [2]

The presence of cerebral metastases placed the above patient in the high-risk category or according to the International Federation of Gynaecology and Obstetrics (FIGO) score ≥ 7. Such high-risk patients require combination chemotherapy. [13]

Patients with Choriocarcinoma are followed up by clinical assessment and laboratory evaluation. Post treatment, serum β-hCG is monitored weekly until it becomes negative, then three additional weekly evaluations, then monthly for life. During the period of follow-up, the patient should be on an effective contraceptive method for 2-years. Thereafter, she is allowed to conceive if she so desired. The pregnancy should be confirmed early by ultrasound to rule out recurrence. Post delivery, the placenta should be sent for histology, and at 6-weeks post-partum, the serum β-hCG is assessed. The index patient was commenced on barrier method of contraception while awaiting sterilization by bilateral tubal ligation.


  Conclusion Top


Metastatic choriocarcinoma may present in bizarre forms should be suspected in any woman presenting with abnormal vaginal bleeding, unusual systemic physical features with raised serum β-hCG. Combination chemotherapy is usually effective and is advocated as the primary treatment method.

 
  References Top

1.
Crum CP, Lester SC, Cotran RM. The Female Genital System and Breast: In: Kumar V, Cotran R, Robbins SL (Eds). Robbins Basic Pathology, 7 th Ed. Saunders (pub) 2003:703-4.  Back to cited text no. 1
    
2.
Osamor jo, Oluwsola AO, Adewole IF. A clinic-pathological study of complete and partial Hydatidiform moles in a Nigerian population. J Obstet Gynaecol 2002;22:423-5.  Back to cited text no. 2
    
3.
Acosta-Sison H. Ab initio Choriocarcinoma: Two unusual cases. Obstet Gynaecol N Y 1959;13:350-2.  Back to cited text no. 3
    
4.
Akosa AB, Ampadu FO, Gyasi RK. A review of complete moles in Ghana. Ghana Med J 2001;35:85-9.  Back to cited text no. 4
    
5.
Adeleye JA, Ilesanmi AO. Gestational trophoblastic diseases: In: Okonofua F, Odunsi K (Eds.). Contemporary Obstetrics and Gynaecology for developing Countries. WHARC (Pub) 2003:73-90.  Back to cited text no. 5
    
6.
Nkeyer K. Gestational Trophoblastic Disease: In: Kwawukume EY, Emuveyan EE (Eds.). Comprehensive Gynaecology in the Tropics. Graphic Packaging LTD 2005:488-511.  Back to cited text no. 6
    
7.
Giannakopoulus G, Nair S, Snider C and Amenta PS. Implications of the pathogenesis of aneurysm formation: Metastatic Choriocarcinoma presenting with spontaneous splenic rupture. Case report and a review. Surgical neurology, 1992;30(3):236-40.  Back to cited text no. 7
    
8.
Van den Doel EMH, van Merrienboer FJJM, Tulleken CAF. Frontal cerebral haemorrhage from unsuspected Choriocarcinoma. Clin Neurology and Neurosurgery, 1985;87(4):287-90.  Back to cited text no. 8
    
9.
Balagopal PG, Pandey M, Chandramohan K, Somanathan T and Kumar S. unusual presentation of Choriocarcinoma -a case report. World Surg Oncol, 2003.  Back to cited text no. 9
    
10.
Mikko L, Eero P, Pentii L, Arto Lemines. Epidemiology of Choriocarcinoma in Finland 1953-1999. Gynaecologic Oncology, 2004;92(1):252-5.  Back to cited text no. 10
    
11.
Song HZ, yang SY, Xiang. Forty-five years experience of treatment of Choriocarcinoma and invasive mole. Int. J. Gynaecol and Obstet 1998;60(1):S77-S83.  Back to cited text no. 11
    
12.
Sanju Cyriac, Rejiv Rajendranath, Veuswani Sridev, Tenali G. Sagar. Etoposide, Cisplatinum-Etoposide, Methotrexate, Actinomycin-D, as primary treatment for management of very-high-risk gestational trophoblastic neoplasia. Int J Gynaecology Obstet 2011;115:37-9.  Back to cited text no. 12
    
13.
Bhattacharyya SK, Saha SP, Mukherjee G, Samanta J. Metastatic vulvo-vaginal choriocarcinoma mimicking a Bartholin cyst and vulvar haematoma-two unusual presentations. J Turkish-German Gynaecol Assoc 2012;12:218-20.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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