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53
NJOG / VOL 7 / NO. 1 / ISSUE 13/ Jan-June, 2012 | 53
CORRESPONDENCE
Dr Ajay Agrawal
Department of Obstetrics and Gynaecology
Assistant Professor, BPKIHS
Phone: 9852049451
Email: drajayagrawal@yahoo.com
Ovarian Dysgerminoma: Unusual Presentation in Pregnancy
with Acute Abdomen due to Internal Hemorrhage
Agrawal A,1 Rijal P,1 Pokharel H,1 Karki S,2 Basnet P,1 Dahal P1
1Dept of Obs/Gyn, 2Dept of Pathology, BPKIHS
Dysgerminoma tends to occur in young women although its incidence in pregnancy is low. Torsion and
incarceration occurs in nearly half of the cases. Early diagnosis and appropriate treatment offers the best
prognosis to the patient presenting with dysgerminoma. However because of enlarging abdominal size,
pregnancy may mask the rapidly growing dysgerminoma often leading to late presentation.
This is a case report of a female 30 years of age with stage 3C dysgerminoma which lead to acute surgical
abdomen at 33weeks of pregnancy.
Keywords : Acute abdomen, dysgerminoma, pregnancy with ovarian mass
INTRODUCTION
Dysgerminoma, which accounts for 1-5% of all ovarian
cancers, is the most commonly occurring malignant germ
cell tumor contribu ng to 28% of it.1 It tends to occur in
young women, though the incidence of pregnancy with
dysgerminoma is extremely low. The rate of malignant
tumors in the total number of ovarian tumors associated
with pregnancy was reported from 1.3% to 7.9%.2 Torsion
and incarcera on are common complica ons among
these rapidly enlarging tumors. Obstetrical complica ons
occurred in nearly half and fetal demise occurred in one
quarter of the reviewed cases.3
The 5-year survival rate is 96% if the tumor is confi ned
to the ovary and 63% if extension occurs beyond the
ovaries. Pregnancy does not alter the prognosis of most
ovarian malignancies, but complica ons such as torsion
and rupture may increase the incidence of spontaneous
abor on or preterm delivery.4
CASE
A 30 years lady at 31 weeks and 5 days period of gesta on
referred from a primary health centre of Morang district
with ultrasound diagnosis of bilateral adnexal mass with
pregnancy was admi ed on 25th June 2011. She was
unbooked immunized G2 P1 L1 with previous uneven ul
pregnancy with normal vaginal delivery. She gave history of
dull aching lower abdominal pain and gradually increasing
abdominal distension since two weeks. She also had
occasional burning micturi on with increased frequency
and fever. She did not have history of loss of weight and
appe te. There was no family history of gynecologic
malignancy. There was no signifi cant past medical or
surgical history. On examina on she was thin built and
looked pale. She had fever of 100˚F. Her respiratory and
cardiovascular systems were unremarkable. Abdomen was
grossly distended with fullness in bilateral fl anks. Uterus
was 34 weeks size with single live fetus in longitudinal
lie and cephalic presenta on. There was huge mass of
variegated consistency of around 15× 20cm with ill defi ned
margin, restricted mobility, nontender on right side of
gravid uterus occupying whole of right hypochondrium
and similar but smaller mass of 10×8 cm in le fl ank. There
was no renal angle tenderness or palpable kidneys.
On inves ga on her hemoglobin was 7.4mg/dl,
urine culture had growth of E.Coli sensi ve to sulbactam+
cefoperazone, with which she responded and her fever
NJOG 2012 Jan-June; 7(3): 53-55
CASE REPORT
54
54 | NJOG / VOL 7 / NO. 1 / ISSUE 13/ Jan-June, 2012
subsided and other rou ne inves ga ons were within
normal limit. However her abdominal pain did not
subside. She was transfused with 3 units of whole blood.
She was given steroid coverage for fetal lung maturity.
An ultrasonography done on 1st July 2011 revealed large
abdominopelvic mass mostly solid in nature, 23×14 cm
in right side of abdomen and another solid mass 13×8.3
cm in pouch of Douglas with minimal ascites sugges ve
of bilateral adnexal mass with a single live fetus of 31-33
weeks of gesta on without any malforma ons. Addi onal
evalua on for serum CA125, AFP and βHCG was normal.
On 5th July, at 33 weeks of pregnancy she complained of
increasing abdominal pain which was con nuous and
generalized to whole abdomen not relieved with analgesic.
So with a diagnosis of twisted or ruptured ovarian mass
she was planned for lower segment caesarean sec on with
removal of ovarian mass and surgical staging. A er general
anesthesia, midline laparotomy incision was made. There
was hemoperitoneum of 2 liters with a superfi cial tumoral
vessel ac vely bleeding. A lower segment caesarean
sec on was fi rst performed.
Bosselated huge mass of 25×23×20cm was found
origina ng from right ovary and twisted around its pedicle
with mostly solid component extending from pouch of
Douglas to undersurface of liver. There were mul ple
metasta c deposits over parietal abdominal peritoneum
and omentum with dense adhesion to serosa of sigmoid
colon. No deposits were noted on surface of liver. ( Figure
1). Mass was removed a er haemosta c division of the
a achments and pedicle. The le ovary (4×1.5×1cm),
fallopian tube and uterus were grossly normal in contrast
to sonography diagnosis of bilateral ovarian mass.
Since she had no desire for future fer lity which was
discussed with her before taking her for laparotomy, a
total hysterectomy and bilateral salpingo-oophorectomy,
infracolic omentectomy with biopsy from parietal wall
was then performed. She delivered a preterm baby of 2
kg with apgar score of 7/10, 8/10 at 1 and 5 minutes of
life. Abdomen was closed with a drain in the pelvis. Post
opera ve period was uneven ul. Sutures were removed
on 8th post op day and drain was removed on 10th post
opera ve day.
Histopathology from right ovary revealed features of
malignant germ cell tumor having islands of large malignant
cells separated by scanty fi brous stroma and infi ltrated
by lymphocytes, with areas of hemorrhages compa ble
with ovarian dysgerminoma. Uterus, Le ovary and both
fallopian tubes were histologically unremarkable. Sec ons
from omentum and abdominal wall also showed histologic
features of tumor deposits (Fig 2a,2b).
The pa ent was assigned FIGO Stage 3C ovarian malignancy
and referred to Bharatpur Cancer Hospital for pla num-
based chemotherapy.
COMMENT
Associa on of ovarian carcinoma with pregnancy is a
rare occurrence. It may also present as acute abdomen
because of torsion (tumor twis ng around its pedicle)
or intra-abdominal hemorrhage (from conges on and
rupture of superfi cial tumor vessels) as seen in this
pa ent.5 Early diagnosis and appropriate treatment off ers
the best prognosis to the pa ent. Bilateral tumors occur
in up to 10-15% of cases.6,7 Lee reported mean age of
23.8 years (range 4-63 years).8 Dysgerminoma is also very
sensi ve to radia on while other germ cell tumors are
not.9 Chemotherapy and/or radia on can be used to treat
pa ents with metasta c disease.5 For pa ents with early
Agrawal et al. Ovarian Dysgerminoma: Unusual Presentation in Pregnancy with acute Abdomen due to Internal Hemorrhage
Fig 1(a). Intraopera ve fi nding of huge ovarian mass with
the twisted pedicle. Fig 1 (b). Post partum uterus with ovarian dysgerminoma
with omentum
55
NJOG / VOL 7 / NO. 1 / ISSUE 13/ Jan-June, 2012 | 55
stage disease, cure rates approach 100%, while for those
with advanced-stage disease it is least 75%.9 Preopera ve
diagnosis depends mainly on age, abdominal symptoms,
size and consistency of the tumor, and elevated tumor
markers. Our pa ent had late presenta on as she was
misguided by the increase in abdominal size thinking it
to be due to pregnancy. The presenta on is usually with
chronic abdominal pain and palpable abdominal tumour.
Some may present with vaginal bleeding, amenorrhea or
precocious puberty.9
Elevated tumor markers may assist in determining the
diagnosis preopera vely.4 Hoei-Hansen et al reported the
expression of following markers in ovarian dysgerminomas:
placental alkaline phosphatase (PLAP) in 100% cases, stem
cell-related markers OCT-3/4 and KIT in 80% cases, and
NANOG and AP-2γ in 50% cases.4 Kawai et al reported
posi ve rate of AFP in 11.8% dysgerminoma, and CA 125
in over 50% cases; they also found a high posi ve rate
of LDH.10 In this case CA125, AFP and βHCG levels were
normal.
Surgery is the fi rst step of management followed by
adjuvant chemotherapy (depending on histopathology)
9. Appropriate surgical treatment for pa ents where
fer lity needs to be preserved consists of laparotomy
with unilateral salpingo-oophorectomy and resec on
of all visible disease.7,11 A total abdominal hysterectomy
is not mandatory, but may be performed concurrently if
pa ent does not desire future pregnancy as in this case.
The current standard adjuvant chemotherapy consists of 3
cycles of BEP.8,11 Four courses of BEP are recommended in
case of bulky residual tumor a er surgery.4,11 Recurrence
in contralateral ovary can develop over the next 2 years.7
Fer lity sparing treatments are considered safe in pa ents
with pure ovarian dysgerminomas.12
CONCLUSIONS
Ovarian cancer very rarely presents as an acute abdomen
in pregnancy. However, ovarian malignant dysgerminoma
may present as an acute abdomen because of torsion,
passive blood conges on, rupture of superfi cial tumoral
vessels and subsequent intra-abdominal hemorrhage.
Ovarian dysgerminoma should also be part of the
diff eren al diagnosis in a young pregnant woman with
acute surgical abdomen and a solid heterogeneous pelvic
mass detected by ultrasonographic scan.
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104-8.
Agrawal et al. Ovarian Dysgerminoma: Unusual Presentation in Pregnancy with acute Abdomen due to Internal Hemorrhage
Fig 2(a). Microscopically islands of large malignant cells
separated by scanty fi brous stroma
Fig 2(b). Infi ltrated by lymphocytes, with areas of
haemorrhage