Journal of Case Reports in Oncology and Therapy

Low grade endometrial stromal sarcoma: A case report

Rohi Wani1, Nazia Bhat1, Junaid Nazier2, Humaira Bashir1, Maajid Jahangeer3,
Ambreen Beigh1, Naila Nazier1
1
Department of Pathology, Government Medical College Srinagar, India
2
Department of Radiodiagnosis, Skims Medical College Srinagar, India
3
Departent of Surgery, Jammu and Kashmir Health Service Srinagar, India

Received May 03, 2015; Revised July 01, 2015; Accepted September 18, 2015; Published Online March 10, 2016

Case Report
Abstract
Low grade endometrial stromal sarcoma is a rare uterine mesenchymal tumor comprising 0.2% of all uterine
malignancies. We present here a case of low grade endometrial stromal sarcoma presenting clinically as fibroid uterus.

Keywords: Endometrial Stromal Sarcoma; Fibroid; Menorrhagia

Introduction
Low grade Endometrial stromal sarcomas (ESS) are rare histopathological examination uterus with cervix
malignant tumors that make up less than 10% of all measured 10 × 7 × 5 cms. On cutting open large
uterine sarcomas and only around 0.2% of all uterine multinodular growth measuring 8 × 7 cms involving
malignancies.1 Its clinical recognition may be difficult whole of the myometrium was identified. The growth
and most often a preoperative diagnosis of a uterine was was extending upto serosa. C/S through growth was
fibroid is made.2 Low grade ESS resembles endometrial tan/yellow (Figure 1).
stromal cells in the proliferative phase. It affects younger
women and the mean age is 42 to 58 years.3 ESS is an
indolent tumor with local recurrences and distant
metastasis and can occur even many years after initial
diagnosis.3

Case presentation
A 47-Year-old woman P3 presented with menorrhagia of
6 months duration. Her previous menstrual history was
normal. On examination she was severally anemic Hb
was 6 gm%, her general physical examination was
normal. On per abdominal examination a lump was
palpable corresponding to 16 weeks gestation. On per
vaginal examination uterus was uniformly enlarged to Figure 1: Low-grade endometrial stromal sarcoma showing
16weeks size. USG showed a isoechoeic and hypoechoic diffuse permeation of the myometrium in the form of small
mass of 80 mm by 70 mm size in the uterus suggestive of nodules bulging on the cut surface.
fibroid uterus with cystic degeneration. Bilateral ovaries
and fallopian tube were normal, so clinical diagnosis of Microscopic examination showed highly cellular tumor
fibroid uterus with cystic degeneration was made. Since composed of uniform cells with oblong/spindle shaped
the patient had intractable menorrhagia and the mass nuclei and scant cytoplasm arranged in sheets. The
was quite big, so a total abdominal hysterectomy with tumor cells showed characteristic infiltration in the form
bilateral salphingo-oopherectomy was performed. She of irregularly shaped tongues/islands placed randomly
was transfused 4 units of blood prior to surgery. between bundles of smooth muscle cells. Vascular
Intraoperative findings were those of an enlarged uterus invasion was also seen at places. Many mitosis (6/10
of 16 weeks with smooth surface. hpf) were seen An arborising vascular pattern was also
seen at places. Final diagnosis of low grade endometrial
Her postoperative period was uneventful and the patient stromal sarcoma was made {(Figure 2) and (Figure 3)}.
was discharged 5 days after surgery. On

Corresponding Author: Nazia Bhat; Department of Pathology, Government Medical College Srinagar, India.

Cite this article as: Wani R, Bhat N, Nazier J, Bashir H, Jahangeer M, Beigh A, Nazier N. Low grade endometrial stromal sarcoma: A
case report. J Case Rep Onc Ther. 2016; 2(1):214.

© Wani et al.
2 Wani et al.: Low grade endometrial stromal sarcoma
Figure 2: Low-grade endometrial stromal sarcoma. Broad
bands of tumor cells diffusely invading the myometrium in
LGESS (medium power view) with tumor cells in vascular
space.
Figure 3: Low-grade endometrial stromal sarcoma. The
tumor cells resemble the cells of normal proliferative phase
endometrial stroma. Many mitosis also seen in this picture
(high power view).
Discussion
Endometrial stromal tumors are among the least
common neoplasms of the uterine corpus, with an
annual incidence of about 2 per million women. 4 They
comprise 0.2% of all female genital tract malignancies.3
They usually occur at an earlier age (42-58 yrs) in
comparison to other uterine malignancies with about
10-25% patients occurring in the premenopausal age
group.5 The usual clinical presentation of ESS is
abnormal uterine bleeding, uterine enlargement or
pelvic pain.4,5
Most tumors grow through the intramural sections of
the uterus rather than intracavitary, hence making it
difficult for preoperative histopathology diagnosis. 6
These tumors have an indolent growth with a tendency
for late recurrence.3 Metastasis are rarely detected
before the diagnosis of the primary lesion. 1 The latest
2014 WHO classification scheme now incorporates
recent molecular findings into the classification, dividing
ESTs into endometrial stromal nodule(ESN), low-grade
endometrial stromal sarcoma (LGESS), high-grade
endometrial stromal sarcoma (HGESS) and
undifferentiated uterine sarcoma (UUS).7
© Wani et al.
The ESN and LGESS fall in the lower end of the spectrum
of this group of tumors. Both are typically composed of a
diffuse growth of small blue cells with scant cytoplasm,
and oval to spindle nuclei that resemble the endometrial
stromal cells of the proliferative endometrium.3 The
‘tongue-like’ patterns of myometrial and lymphovas-
cular invasion are classical histological features that are
important in the distinction from ESN.8 High grade
endometrial stromal sarcoma is characterized by a
monomorphic proliferation of round cells in a vaguely
nested or pseudoglandular pattern. About half of the
tumours are biphasic, where the round-cell component
is admixed with an LGESS-like component that is usually
fibrous or fibromyxoid.7,9 Undiffrentiated uterine
sarcoma represents a high-grade sarcoma that lacks
specific differentiation and bears no histological
resemblance to endometrial stroma.3
Diagnosis of low grade endometrial stromal sarcoma is
often made post operatively in most instances by
histopathological examination since in majority of cases
a preoperative clinical diagnosis of fibroid uterus is
made.2,6,10
Immunohistochemistry may be needed when there is a
problem in distinguishing these tumors from histologic
mimics like cellular leiomyoma cellular endometrial
polyp and adenomyosis.11 A panel of immunostains that
include CD10 and two smooth muscle markers desmin
caldesmon should be used, as there is no marker
specific for ESS.7,11
The treatment of choice is total abdominal hysterectomy
with bilateral salpingo oophorectomy and pelvic and
periaortic selective lymphadenectomy. 2,3 Due to the high
recurrence risk even with localized tumors adjuvant
treatment either with progestins, radiation therapy or
even aromatose inhibitors may be given in order to
suppress the tumor growth.12
Conclusion
LGESS may not be familiar to the gynaecologists because
of the rarity of the tumor. The usual preoperative
diagnosis is fibroid and diagnosis is usually made post
operatively after histopathological examination. In our
case also clinical diagnosis of fibroid uterus was made.
Final diagnosis of low grade endometrial stromal
sarcoma was made on histopathological examination
alone.
Conflict of interest
The authors declare that they have no conflicts of
interest. The authors alone are responsible for the
content and writing of the paper.
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