Article Type : Case Report
Authors : Daniyah E
Keywords : Benign ovarian neoplasms; Benign cystadenofibroma; Peritoneal implants
Serous
cystadenofibroma is a benign epithelial adnexal tumour. It is a rare tumour originates from the ovary
due to unknown etiology. It is composed of cysts, glandular elements, and
varying components of fibrous stroma. It is usually seen in adult women of
reproductive age. Cystadenofibroma is mostly asymptomatic and often seen as
incidental mass. This tumour may resemble malignant neoplasms on imaging.
However, specific tumour characteristics on imaging distinguish it from
malignant ovarian tumours. Therefore,
MRI is the imaging modality of choice. Peritoneal implants may be seen
intraoperatively making malignant neoplasm highly likely. Thus, intraoperative
frozen section biopsy is the gold standard for cystadenofibroma diagnosis as it
reduces unnecessary aggressive surgery. Complete surgical resection is the
treatment of choice. We report a 20-year-old female diagnosed with ovarian
serous cystadenofibroma with scattered lesions in the peritoneum resembling
malignant dissemination.
In 2020, breast cancer, cervical cancer, ovarian
cancer, and uterine cancer earned positions in the top 10 most common female
malignancies.1 Ovarian cancer is one of the most lethal gynecologic cancers
with less than one-half of patients surviving over five years after diagnosis.2
The strongest risk factors for ovarian cancer include family history and
advanced age.2 Ovarian tumors may be benign or malignant, and determining their
etiology accurately and timely is essential to proper treatment. This can be especially
difficult when benign tumours display features of malignancy [1]. Serous
cystadenofibroma is a benign epithelial adnexal tumour. It is a rare tumour originates from the ovary
and less likely the fallopian tubes due to unknown etiology. It is seen in
adult women of reproductive age. Serous
cystadenofibroma is composed of cysts, glandular elements, and varying
components of fibrous stroma [2]. Cystadenofibroma is mostly asymptomatic and
often seen as an intraoperative incidental mass.
An 18-year-old nulligravid female presented with
abdominal distention, secondary amenorrhea, and abnormal vaginal bleeding. Her
menstrual cycle started to be irregular 3 years prior to the presentation.
Vitals were within the normal range. Body mass index was 36.8 %. Physical exam
showed distended, tense, non-tender abdomen. A large cystic mass was noted
extending from the pelvis to the upper abdomen resembling a 30-week gravid
uterus. Thinning hair over the vertex was noted. No acne, facial hair, or
excessive body hair was noted. Complete blood count and serum biochemistry were
unremarkable. Urine pregnancy test was negative. CA-125 was not performed.
Pelvic CT scan with oral contrast demonstrated bilateral pelvic adnexal lesions
suggesting a possible ovarian origin with multiple cystic masses measuring 8 cm
on the left and 5.2 cm on the right. Another 1.9 cm hypodense lesion was noted
in the right pelvic cul-de-sac adjacent to the rectum but separate from the
right ovary.
Exploratory laparotomy was performed to confirm the diagnosis. Intraoperatively, a large cystic mass was visible of 7x10x11 cm in the abdomen. On further inspection, there was another large cystic mass filling the entire pelvis and abdomen measuring 10x18x21 cm. bilateral ovaries have multiple small cysts. The right ovary has abnormal appearing growths suspicious of neoplasm. No lymph node enlargement or regional metastasis was noted. The masses were drained and the fluid was sent out for pathology which turned out to be negative for malignancy. Cystic masses were resected sparing bilateral fallopian tubes. The fallopian tubes were reconstructed. The intraoperative frozen section of the right ovarian mass showed papillary serous cystadenofibroma.
The post-operative course was uneventful. On a
follow-up appointment, she was diagnosed with polycystic ovarian syndrome and
started on metformin and a combined oral contraceptive. Postoperatively, she
reported constant abdominal distention, pelvic discomfort, occasional nausea,
and vomiting suggestive of intermittent ovarian torsion and possibly intestinal
obstruction. No abnormal vaginal bleeding, hematuria, hematochezia, and melena
were reported. Pelvic ultrasound demonstrated no evidence of ovarian torsion. A
Pelvic CT scan showed enlarged residual cystic mass filling the pelvis and
abdomen likely originating from one of the ovaries. Although open surgery for
ovarian reconstruction was the best approach, preserving fertility was a
priority given the benign nature of the tumour and patient’s age. The decision
was made to perform mini-laparotomy procedure for the drainage of the pelvic
cystic masses, resection of the ovarian cyst, and construction of both ovaries.
One year later and due to persistent symptoms, the patient underwent
robotic-assisted lysis of adhesions and drainage of multiple ovarian cysts
which measure about 8 to 10 cm in size each. Bilateral oophoropexy was pursued
for the treatment and prevention of ovarian torsion. Pelvic MRI without contrast
showed T2-weighted hypointense innumerable cystic changes of varying sizes in
both ovaries favouring bilateral ovarian cystadenofibroma. Evaluation for solid
components was limited due to lack of intravenous contrast.
While ovarian cystadenofibromas are benign tumours,
their symptomatology and presentation may be virulent and result in significant
patient morbidity. Cystadenofibromas are rare, benign ovarian tumours composed
of both epithelial and fibrous stromal components that typically present in the
fourth to fifth decade of life. They account for less than 2% of benign ovarian
tumours. On imaging, these tumours display typical characteristics of
malignancy with cystic, complex cystic, or solid components separated by
irregular, thick septae with papillary projections. Even on direct
visualization during surgery, these tumours grossly appear virulent due to
their heterogeneous characteristics. The tumour’s fibrous components may allow
it to be differentiated from malignant tumours on MRI. Unlike cyst
adenofibroma, serous cystadenoma has minimal connective tissue components with
the cysts being the major feature [3]. Preoperative imaging is inadequate to
differentiate between benign and malignant tumours. Moreover, the gross
appearance of these tumours intraoperatively may lead to misdiagnosis as
malignant neoplasms due to peritoneal implants, extensive dissemination, or
projections as seen in this patient [4,5]. However, specific tumour
characteristics on imaging distinguish it from malignant ovarian tumours. Therefore, MRI is the imaging modality of
choice [6]. Given the fibrous component of these benign adnexal tumours, they
demonstrate low signal intensity in T2 weighted magnetic resonance images [7].
In contrast, serous cystadenocarcinoma demonstrates a strongly enhanced solid
component. Cystadenofibroma most frequently demonstrates cystic mass with
multiple septae and variable amounts of solid components [8]. However, in some
cases, it can demonstrate bilocular or unilocular cystic mass with partially
thickened walls on MR images. In some rare cases, it demonstrates unilocular or
multilocular mass with cystic components and absent connective tissue structure
similar to serous tumours. MRI is the gold standard modality to further
investigate the nature of the tumour. These morphological patterns will be
enhanced after administration of intravenous contrast. Knowledge of these
criteria help distinguish cystadenofibroma from other malignant ovarian
tumours. Frozen section biopsy is the gold standard cyst adenofibroma diagnosis
as it prevents aggressive surgery. Complete surgical resection is the treatment
of choice. The prognosis is favourable.
To relive pelvic discomfort while preserving fertility, cyst aspiration may be
pursued instead of aggressive surgical approach. Careful observation will be
practiced because of the high risk of ovarian torsion. Patient education and
counselling for the benign nature of cystadenofibroma alleviate the
psychological burden of the disease and may prevent unnecessary surgery.
Serous cystadenofibroma is a benign ovarian tumour. It
resembles malignant neoplasms on imaging and morphological appearance. MRI is
the imaging modality of choice for pre-operative diagnosis. Frozen section
biopsy is the gold standard for cystadenofibroma diagnosis. The prognosis is
excellent after complete surgical resection. Patient age and fertility are
taken into consideration when choosing a treatment approach.
· Ovarian serous
cystadenofibromas are rare benign epithelial ovarian tumours. These tumours may cause acute abdomen due to
cyst rupture and ovarian torsion.
· Benign ovarian tumours
may mimic malignant neoplasms on imaging.
· MRI is the modality of
choice for further characterization of the tumour.
· Frozen sections should be
performed intraoperatively to guide and prevent unnecessary aggressive surgical
approach.
· Surgical resection of the
tumour is a treatment of choice considering fertility in young patients.