Author
Information
Kimaya Mali*. Shruti Panchbudhe*, Vibha More*, Meena
Satia**
(* Assistant
Professor, ** Professor. Department of Obstetrics and Gynecology, Seth G. S. Medical College & K.E.M. Hospital,
Mumbai, India.)
Abstract
We report a case of
endometrial stromal sarcoma which had a varied presentation. Initially patient
was diagnosed and managed as a case of
incomplete abortion, but in view of persistence of symptoms, patient was
subjected to further evaluation.
Histopathology along with imaging modalities like ultrasonography and
magnetic resonance imaging helped to reach a diagnosis of endometrial
carcinoma, which was managed with total hysterectomy with bilateral
salpingo-oophorectomy.
Introduction
Uterine Sarcoma are rare tumors of
mesodermal origin and constitute 2 to 6% of uterine malignancies.[1] Endometrial
stromal sarcoma is a rare type of uterine sarcomas comprising
approximately 0.2% of all uterine malignancies.[2] According
to the latest WHO classification, the term endometrial stromal tumor is applied
to neoplasms composed of cells that resemble endometrial stromal cells of the
proliferative endometrium.[3] Endometrial stromal sarcoma usually presents in
perimenopausal women between ages 45 and 50 years. The
main differential diagnosis of low-grade endometrial stromal sarcoma (ESS) includes endometrial
stromal nodule, cellular leiomyoma and cellular intravenous leiomyomatosis.
Case report
A 31 yrs old woman, second para married for 12 years, presented
to a private practitioner for heavy bleeding for 2 days.
On speculum examination fleshy mass
like structure was seen with bleeding and on vaginal examination
the
uterus was 10 weeks and cervical os was open. A probable diagnosis of
incomplete abortion was made and emergency curettage was done. She was
discharged the next day. The patient had similar complaints of heavy bleeding
with passage of blood clots two weeks after the curettage for
which she again visited the same private practitioner. On
examination her clinical findings of speculum and vaginal examination were
similar to the previous episode. In view of similar findings again a repeat
curettage was performed and the fleshy material obtained was sent for
histopathological examination. At this time after curettage her uterine size
had regressed to 6 weeks. Histopathological report was suggestive of atypical
cellular leiomyomatous polyp with clear
cell pattern, or possibly clear cell carcinoma. In view of this histopathology
report the patient was referred to tertiary care center.
On detailed history at
tertiary care center patient gave a history of
intermittent episodes of heavy bleeding for four months.
She attained menarche at the age of 14 years and her past menstrual cycles were
regular with average flows. She was married for 12 years and
had undergone two cesarean sections in the past in both her pregnancies for
obstetric indications. On general examination she had pallor. Her vital
parameters were normal. On abdominal examination she had
an infraumbilical vertical midline scar. Speculum
examination showed a 2x2 cm fleshy mass at the cervix. On vaginal examination
the
uterus was 10 to 12 weeks, anteverted and the fornices
were free and non tender. Her hemoglobin was 6 g % for which she was transfused
with two units of whole blood. Ultrasonography with color Doppler flows was
done which showed uterine size of 10x8x7 cm with an endometrial
thickness of 4 cm in the
center and
arterial peak systolic velocity of 0.35-0.2 per centimeter per second
and there was no evidence of arterial-venous malformation. Histopathology
slides were reviewed which were suggestive of “PEComa” (Perivascular
epitheliod cell tumour) as shown in figure 1. Immunohistochemisty
study was advised for confirmation. but was
deferred as patients was not affording. Magnetic resonance imaging was done. It was suggestive
of bulky uterus with thickened endometrium, with loss of endo-myometrial interface, with no lymphadenopathy and preservation of fat planes.
These findings
were
suggestive of neoplastic etiology.
In view of above
findings total abdominal hysterectomy with bilateral
salpingo-oophorectomy was performed. On cut
section the specimen showed a polypoidal growth which was occupying the entire
endometrial cavity and was encroaching on the
myometrium (figure 2). Her
histopathological report obtained from the uterine specimen was suggestive
of endometrial stromal tumor (figure 3). Her
postoperative course was uneventful and she was discharged on day 7. She
was referred to oncology center where her slides were
reviewed and in view of same histopathological diagnosis she did not require
any further management and was advised
regular follow up.
Figure1.
Histopathology
of Perivascular epitheliod cell showing spindle cell encircling vessels.
Figure
2. Cut section of the specimen.
Figure3. Histopathology of endometrial stromal sarcoma showing nests and spindle cells invading endometrial stroma and myometrium, having oval vesicular nuclei with endometrial glands with mild hyperplasia
Discussion
Uterine sarcomas are the
most malignant group of uterine tumors and differ from endometrial cancers related
to the
diagnosis, clinical behavior, pattern of spread, and management. The three most common histologic variants of
uterine sarcoma are endometrial stromal sarcoma (ESS), leiomyosarcoma, and
malignant mixed müllerian tumour (MMMT) of both homologous and heterologous
type.[4] In general, leiomyosarcoma and MMMT each make up
about 40% of tumors, followed by
endometrial stromal sarcoma (15%) and other sarcomas (5%). Endometrial stromal
tumors are a variety of homologous type of pure nonepithelial tumors and are
composed purely of cells resembling normal endometrial stroma. They are further
categorized into three types on the
basis of mitotic activity, vascular invasion, and prognosis.
They
are (i) endometrial stromal nodule (less than 5 mitotic figures per 10 high-power
microscopic field ), (ii) endometrial stromal sarcoma ( mitotic rate of less than 10 MF/10 HPF) and (iii) high-grade or undifferentiated
sarcoma ( greater than 10 MF/10 HPF). The traditional classification of ESS into low-grade and high-grade
categories is now changed to endometrial stromal sarcoma for previous low grade
endometrial stromal tumors (LGESS) and undifferentiated endometrial sarcoma is
used for previous high-grade endometrial stromal sarcoma (HGESS).[5] The World Health Organization defines perivascular
epithelioid cell tumors (PEComas) as "mesenchymal tumors composed of histologically
and immunohistochemically distinctive perivascular epithelioid cells
(PECs)" which have a marked female preponderance, the uterus is the commonest site
and is often mistaken for
leiomyomatosis.[6]
The most frequent
symptom is abnormal uterine bleeding, abdominal pain and pressure caused by an
enlarging pelvic mass but 25% of them are asymptomatic. The usual preoperative
diagnosis is uterine leiomyoma, but the confirmatory diagnosis is established
after endometrial biopsy. On gross examination
there is nodular growth involving the endometrium, myometrium, or both, which is associated
with varying degrees of permeation of the myometrium, including worm-like elastic extensions of tumor into
myometrial veins and may extend into
parametrial veins and lymphatics. Microscopically there is only mild
nuclear atypia with mitotic activity of 5 MF/10 HPF and rarely there is
necrosis. Immunohistochemistry helps in the detection of tumor markers
specific for endometrial stromal tumor, which is CD10, which helps in distinguishing these tumors from
histologically mimicking tumors like cellular leiomyoma which are CD 10
negative.[7] However, the role of CD10 for a preoperative diagnosis
is not well established. Other immunomarkers such as oxytocin receptors,
h-caldesmon, desmin, CD10, and inhibin are useful in distinguish between
cellular leiomyoma and ESS. Leiomyoma expresses oxytocin receptors, h-caldesmon, desmin while ESS expresses CD10 and inhibin.
Immunohistological studies shows that ESS is almost always positive for both
estrogen and progesterone receptors and the standard surgical treatment
recommended is total hysterectomy, with bilateral salpingo-ophorectomy. Hormone
replacement therapy is contraindicated postoperatively.[8] The retained
adnexae increases the risk of tumor extension into the parametria, broad
ligaments, and adnexal structures due to the stimulating effect of
estrogens from retained ovaries on the
tumor cells. ESS usually behaves in an indolent clinical fashion; however
recurrences and distant metastases can occur. Radiation therapy is recommended
for inadequately excised or locally recurrent pelvic disease.[9]
Recurrent or metastatic lesions may also be treated by surgical excision. Prolonged
survival as well as cure is common
despite the development of recurrent or metastatic disease.
References
- Harlow BL, Weiss NS, Lofton S. The epidemiology of sarcomas of the uterus. J Natl Cancer Inst 1986; 76:399-402.
- Koss LG, Spiro RH. Brunschwing A. Endometrial stromal sarcoma. Surg Gynecol Obstet1965; 121: 531-7.
- World Health Organization classification of tumours. In: Tavassoli FA, Devilee P,editors. Pathology and genetics of tumours of the breast and female genital organs. Lyon: IARC Press; 2003.
- Kempson RL, Bari W. Uterine sarcomas: classification, diagnosis and prognosis. Hum Pathol 1970;1:331-349.
- Amant F, Vergote I, Moerman P. The classification of uterine sarcoma as ‘high grade endometrial stromal sarcoma’ should be abandoned. Gynecol Oncol 2004 ; 95:412–3.
- Folpe AL. Neoplasms with perivascular epithelioid cell differentiation (PEComas). In World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone. Edited by Fletcher CDM, Unni KK, Mertens F. Lyon: IARC Press; 2002:221-222.
- Chu PG, Arber DA, Weiss LM, Chang KL. Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: An immunohistochemical comparison of 34 cases. Mod Pathol 2001;14:465–71.
- Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas.Sarcoma. 2010;2010:506182.
- Weitmann HD, Kucera H, Knocke TH, Pötter R. Surgery and adjuvant radiation therapy of endometrial stromal sarcoma. Wien Klin Wochenschr 2002;114:44–9.
Citation
Mali
K, Panchbudhe S, More V, Satia M. Endometrial stromal sarcoma - A rare
presentation. JPGO Volume 1 Issue 3, March 2014, available at: http://www.jpgo.org/2014/03/endometrial-stromal-sarcoma-rare.html