Unusual presentation of choriocarcinoma
World Journal of Surgical Oncology
Unusual presentation of choriocarcinoma
PG Balagopal 2
Manoj Pandey 2
K Chandramohan 2
Thara Somanathan 1
Ashwin Kumar 0
0 Department of Radiation Oncology, Regional Cancer Centre , Thiruvananthapuram, Kerala , India
1 Department of Pathology, Regional Cancer Centre , Thiruvananthapuram, Kerala , India
2 Department of Surgical Oncology, Regional Cancer Centre , Thiruvananthapuram, Kerala , India
Background: Choriocarcinoma is an aggressive neoplasm arising in the body of the uterus. The disease normally spreads to lung and brain. Case Report: A case of malignant trophoblastic disease with brain metastasis, raised intra cranial pressure and small bowel metastasis presenting with acute abdomen is reported. Conclusions: Malignant transformation in a hydatidiform mole is rare event. Involvement of gastrointestinal tract is rarer even in presence of disseminated disease. Surgery is the treatment of choice for gastrointestinal complications.
Trophoblastic diseases comprise a variety of biologically
interrelated conditions which form a clinical spectrum
consisting of four distinct clinical pathological entities
like (1) molar pregnancy (2) invasive mole (3) placental
site trophoblastic tumours and (4) choriocarcinoma .
Incidence of hydatidiform mole ranges from 1 in 500 in
India to 1 in 3000 in USA. Like wise malignant potential
of the disease is also higher in Southeast Asia reaching as
high as 1015% compared to 24% in west. Some of the
hydatidiform moles erode the wall of uterus, burrow into
myometrium and may even burst though the uterus into
peritoneum and are called invasive mole. Even though
locally aggressive, invasive mole does not metastasise. On
the other hand choriocarcinoma is rare but one of the
most malignant neoplasms arising in the body of uterus.
choriocarcinoma metastasises readily. It is not
uncommon to find secondary nodules in the cervix or vagina.
The lungs, brain and liver are the distant organs more
often involved. Lymph nodes are rarely the site of metastasis
. We report here a case of hydatidiform mole
metastasising to brain and later presenting with intestinal
metastasis and perforation.
A twenty-four-year old housewife underwent dilatation
and curettage for vaginal bleeding in 1994. Pathological
examination of curettage specimen showed hydatidiform
mole. She later conceived and had two normal deliveries.
She remained asymptomatic for eight years. In January
2002 she was brought to medical causality with features
suggestive of raised intra cranial pressure. A computerised
tomographic scan of the brain was taken which revealed
brain metastasis (figure 1) and the patient was referred to
our centre. After admission she was treated with six cycles
of chemotherapy consisting of etoposide, methotrexate,
actinomycin-D, cyclophosphamide and Vincristine
(EMA-CO regime). Ten days after completion of
chemotherapy she developed abdomen pain and vomiting.
On examination she was febrile, with mild abdominal
distension and rebound tenderness was present. A plain
X-ray abdomen in left lateral decubitus posture showed
free gas above the liver (figure 2). X-ray chest was taken
which showed metastatic lesions in the lung. With
diagnosis of perforative peritonitis the patient was taken up
for emergency laparotomy.
At laparotomy the stomach and duodenum were found to
be normal. A 1 1 cm perforation was found in the
jejunum, 10 cm distal to the dudonojejunal junction.
Another area of impending perforation was found 5 cm distal to
the site of perforation. Multiple metastatic nodules were
noted on the serosal surface of the small intestine, colon,
and bowel mesentery. A 3 4 cm metastatic nodule was
present on the superior surface of right lobe of liver.
Resection of the perforated segment of jejunum and end to end
anastamosis was carried out.
On gross examination the resected small intestine
measured 14 cm in length. A small perforation measuring 1
0.8 cm was seen on the serosa 6 cm from one of the
resected ends. Adjacent to the perforation the serosa showed
two nodules, each measuring 1 1 cm. On opening the
intestine, multiple small mucosal nodules were noted.
The cut surface of the serosal nodules were haemorrhagic
and grey white. Similar nodules were seen involving the
full thickness of the intestinal wall.
Microscopic examination showed multiple nodules of a
neoplasm involving the mucosa, submucosa, muscle and
serosa of the small intestine (fig 3,4,5). Cytotrophoblasts
having eosinophilic cytoplasm and round, vesicular
nuclei with nucleoli were seen admixed with
syncytiotrophoblasts, in a haemorrhagic background (fig 6). The
mesenteric fat showed tumour infiltration with a vascular
tumour emboli. These findings were consistent with
Postoperative period was uneventful. Patient was
discharged on tenth day. As she was not responding to
EMACO, she was switched over to BEP regimen (Bleomycin,
Etopside, and Predinisolon). She received eight cycles of
BEP. Her HCG fell to 12000 IU but then started to rise
again to reach 20,000 IU, patient discontinued the
chemotherapy at this stage and was lost to follow-up.
Gestational trophoblastic disease most commonly
follows molar pregnancy and may also occur following
normal or ectopic pregnancies and spontaneous or
therapeutic abortions. Its incidence varies with figures as
high as 1 in 120 pregnancies in some areas of Asia and
South America, compared to 1 in 1200 in United States.
Metastatic disease occurs in 4% patients after local
management of Hydatidiform mole. The incidence of
choriocarcinoma after complete hydatiditorm mole is about
1000 times greater than after a normal pregnancy . It
may occur possibly ab initio . Choriocarcinoma is a rare
tumour. In western countries, the incidence is 1 in 45,000
pregnancies . Higher incidence is reported from, Africa,
Asia and South America . Majority of cases occur in
women aged less than 35 years of age .
Choriocarcinoma is suspected when there is persistent or
irregular uterine haemorrhage, following abortion or
hydatidiform mole. Rapid growth and haemorrhage make
the tumour a medical emergency. Metastasis may occur in
lung, pelvis and vagina. Rare sites include gastrointestinal
tract, spleen, and kidney. The central nervous system is
seldom involved in the absence of pulmonary metastasis
. Metastatic disease occurs in 4% of patients after local
management of hydatidiform mole and very rarely after
tem pregnancies or abortions . Some times they may be
combined with malignant germ cells components,
occasionally it may be difficult to differentiate a primary
tumour from a metastasis. For females in non reproductive
age group, the tumours may arise from ovarian germ cells
which is histologically similar to gestational uterine
Chemotherapy is highly effective for all forms of
gestational trophoblastic disease. For stage I disease
hysterectomy and single agent chemotherapy is effective. For
Malignant transformation in a hydatidiform mole is rare
event. There are reports describing metastasis to large
intestine from choriocarcinoma presenting as
pesudoobstruction  and metastasis to stomach . Metastasis to
gastrointestinal tract and presention as perforation of
small intestine is very rare. The case is being reported for
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