Author
Information
Kakade
AS*, Kulkarni YS*, Kumar P**.
(* Associate Professor,
**Second Year Resident. Department of Obstetrics & Gynecology, Bharati
Vidyapeeth Deemed University Medical College, Bharati Hospital & Research
Center, Pune, India.)
Abstract
Sclerosing stromal
tumors of ovary are rare benign tumors, which have distinctive clinical and
pathologic features. They are characterized by the pseudolobular pattern of the
cellular and hypocellular areas with marked vascularity and heterogeneity of
the cellular area. They tend to occur more commonly during second to third
decade of life and more than 80% of these tumors occur below the age group of
30 years. We present such rare case of sclerosing stromal tumor of the ovary in
a 38 years old patient. The ultrasound and gross findings were suggestive of a
malignant neoplasm which turned out to be sclerosing stromal tumor with a very
favorable outcome.
Introduction
Ovarian sex cord stromal
tumors represent approximately 8% of primary ovarian neoplasms.[1]
In 1973, Chalvardjain and Scully first described sclerosing stromal tumors
(SST) as a distinct disease entity in ovarian sex cord stromal tumors. SST is a
benign ovarian stromal tumor, which has distinctive clinical and histological
characteristics. It is prevalent in young age groups and has heterogeneous
pattern, which distinguishes it from the fibromas, thecomas and other types of
ovarian stromal tumors. [2] SST is a benign neoplasm and is distinguished
from other ovarian stromal by the production of collagen and a pseudolobular
pattern with cellular areas separated by edematous and collagenous areas.
[3]
Case Report
A 38 years old woman
came to the out-patient department with complaints of pain in lower abdomen for
10-12 days. The pain was cramping in nature, continuous, non-radiating and the
intensity of pain reduced in lying down and left lateral position. There were
no associated bowel or bladder complaints. She did not have any menstrual
complaints. Her last menstrual period was one month ago. Her previous cycles
were normal. She had three full term vaginal deliveries and underwent a
puerperal tubal ligation 14 years ago. She did not have any significant past
medical, surgical or personal history. She was given two doses of intravenous
iron sucrose by a general practitioner two days back for mild anemia (hemoglobin
8.7 g/dL).
She had a pulse rate of
90 beats per minutes, blood pressure of 120/70 mm Hg. She had mild pallor. Lymph
nodes were not palpable. Her cardiovascular and respiratory system did not
reveal any abnormality. Her breast and thyroid were normal. The abdomen was
soft on palpation and there was a mildly tender suprapubic mass of 9x5 cm,
which was firm and with reduced mobility. There was no clinical evidence of
free fluid in the abdomen. Per Speculum examination revealed cervical erosion
and visual inspection with acetic acid was negative and Pap smear was taken.
Per vaginal examination revealed a 9x5x5 cm cystic and solid mass with lobular
surface in the right and anterior fornix. It was tender and was felt separately
from the uterus. Mobility was reduced and left fornix appeared free.
She was admitted with a
suspicion of tubo-ovarian mass undergoing torsion. Her repeat hemoglobin was
8.6 g/dL and CA-125 value was 53.7 U/ml. Her ultrasound revealed a
heterogeneous predominantly hypoechoic lobulated mass measuring 10.1x10.0x 6.7
cm which is pushing the uterus caudally. It showed increased perfusion with resistance
index of 0.34 (tumoral vascularity). Few areas of necrosis were noted within
it. Left ovary was not seen separately from the mass. The right ovary was
normal. There was minimal free fluid in the pelvis and Morrison’s pouch. There
was no other abnormality in the abdomen. In the settings of raised CA 125 a
suspicion of malignancy was kept in mind.
She received one unit of
packed cell volume and underwent a staging laparotomy with total abdominal
hysterectomy with bilateral salpingo-oophorectomy.
Intraoperative a large
lobulated mass of 10 x 10 x 8 cm arising from the left ovary with single twist
torsion of the pedicle was seen.
Figure 1. Large
lobulated mass arising from the left ovary.
There were cystic and
solid areas with edematous ovarian tissue. There were no para-aortic lymph node
enlargement and her liver and other organs did not reveal any palpable tumor.
The peritoneal fluid was negative for malignant cells.
The cut section of the
tumor was yellowish, homogenous, and lobulated and showed a glistening, translucent
surface. Histopathological examination showed a benign stromal tumor composed
of many lobules of polygonal to spindle cells with uniform round to oval nuclei
and eosinophilic cytoplasm (figure 2).
Figure 2.
Histopathological appearance of the tumor.
The islands of tumor
cells were scattered in an edematous ovarian stroma with many dilated lymphatic
and vascular channels within it. The superficial ovarian cortex was relatively
spared. The findings were suggestive of sclerosing stromal tumor of ovary with
massive edema due to torsion.
She received one unit of
packed cells post operatively and had an uneventful recovery. She was
discharged after suture removal on day 8 of surgery. She did not have any
symptoms on follow up after one month of the surgery.
Discussion
SST is a benign subtype
of ovarian stromal tumor, described as a distinct entity in 1973 by
Chalvardjian and Scully. Sex cord stromal tumors have an approximate occurrence
in 8% of ovarian neoplasms and SST compromises less than 5% of these.[1]
It differs from fibromas, thecomas, and lipid cell tumors in clinical
presentation and histopathological features. SST usually occurs in the second
and third decades of life, younger than the mean age of patients with other
types of stromal cell tumors. The symptoms associated with these tumors are
altered menstrual patterns and lower abdominal pain. They may have some
functional activity and very rarely may have associated ascites.[2, 4, 5]
Histopatholgical
examination of these tumors reveals a pseudolobular pattern in the cellular and
hypo cellular areas. There is a prominent vasculature with
hemangiopericytomatous pattern. There are areas of cellular heterogeneity in
the luteinized theca like cells and there are many spindle shaped, fibroblast
like cells seen.[6, 7]
The term ‘sclerosing
stromal tumor’ was attributed because the cellular areas of this tumor revealed
collagenous sclerosis. Immunohistochemistry plays an important role in
distinguishing these tumors from massive ovarian edema and Krukenberg’s tumor. [3]
The luteinized theca
like cells may secrete vascular permeability factor and vascular endothelial
growth factor causing vasodilatation leading to hypervascularity,
hyperpermeability and edema.[7] This zonal edema is a distinctive
feature of SST. The edematous stroma of these tumors may show some vacuolated and
signet ring cells which can be confused with signets cells of Krukenberg tumor
of ovary.[8] Blood markers like calcitonin, inhibin, CD34, and α
–glutathione S-transferase positivity have been found to be useful to differentiate
SST from thecomas, fibromas and other sex cord stromal tumors.[9]
The etiology of SST
remains largely unknown. By virtue of their structural morphology they are
proposed to arise from the immature pluripotent stromal cells of the ovary.[10]
An alternative origin from the muscle specific actin positive elements of the
theca cells called as perifollicular myoid stromal cells has also been
reported.[7]
The reported case was a
rare occurrence of SST in fourth decade with symptoms and signs suggestive of
torsion, and a malignant neoplasm which turned out to be benign on
histopathology. The massive edema seen in the ovary of this patient can be
because of the torsion and the vascular hyperpermeability induced by VPF &
VEGF.
In conclusion SST should
be considered in women when clinical findings suggest a solid and cystic
adnexal mass with multiple lobulations, with an ultrasound showing low PI and
RI values and no ascites. The ultrasound features of SST may mimic those of
malignant ovarian tumors, and hence color Doppler ultrasound is advisable to
reveal the prominent peripheral vascularity. [11]
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Citation