Uterine Leiomyosarcoma (LMS), the rare, aggressive tumours carrying poor prognosis are mostly diagnosed incidentally at time of myomectomy/hysterectomy done for a presumed benign condition.
The incidence of LMS is1 in 10,000 general population. The incidence of leiomyosarcoma in uterine leiomyomas is between 0.13 to 0.29%
LMS generally arise from a solitary lesion and are a result of genetic instability (errors in p16, p53, Ki67) with aggressive biology and chemotherapy resistance.
LMS is characterized by early dissemination with an overall survival of less than 50% at 2 years.
The modern diagnosis of LMS is based on the work of Bell et al and includes a combination of features including
Diffuse moderate to severe cellular atypia
Mitotic count >10 mitotic figures/10 HPF
Coagulative tumor necrosis
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Early Prediction, Proper Planning can lead to Prevention of Disastrous Progression of Uterine Leiomyosarcoma
1. Early Prediction, Proper Planning can lead to Prevention of
Disastrous Progression of Uterine Leiomyosarcoma
Dr.Indraneel Jadhav*, Dr. Ashwini AP**, Dr. Shashikala Bhat***
(*Resident **Senior Resident ***Professor )
Dr. TMA Pai Hospital, Udupi, MMMC, Manipal University
Purpose ManagementPreoperative investigationsINTRODUCTIONINTRODUCTION
Patient profile
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LOGO
• Uterine Leiomyosarcoma (LMS),
the rare, aggressive tumours
carrying poor prognosis are
mostly diagnosed incidentally at
time of
myomectomy/hysterectomy
done for a presumed benign
condition.
• The incidence of LMS is1 in
10,000 general population. The
incidence of leiomyosarcoma in
uterine leiomyomas is between
0.13 to 0.29%
• LMS generally arise from a
solitary lesion and are a result of
genetic instability (errors in p16,
p53, Ki67) with aggressive
biology and chemotherapy
resistance.
• LMS is characterized by early
dissemination with an overall
survival of less than 50% at 2
years.
• The modern diagnosis of LMS is
based on the work of Bell et al
and includes a combination of
features including
• Diffuse moderate to severe
cellular atypia
• Mitotic count >10 mitotic
figures/10 HPF
• Coagulative tumor necrosis.
Pap smear was negative for intraepithelial
lesion or malignancy. Endometrial
sampling was suggestive of
leiomyosarcoma (LMS) of uterus. Blood
parameters were within normal range.
Chest X- ray was normal. Pre-operative
workup was done and operative
intervention was planned.
A 66 years old parous lady came with
complaints of white discharge per
vaginum and spotting since 2 days. She
was postmenopausal since 15 years and
had no previous episodes of post
menopausal bleeding. She was married
since 43 years, had one male child of
38yrs and one MTP for contraceptive
failure. Tubal ligation was done post
MTP. She was a well controlled
hypertensive since 1year and was on
treatment with Cilnidipine 10mg once
daily.
Her father had gall bladder cancer with
secondaries in the liver and mother had
small cell carcinoma of lung with bone
metastasis.
Her general examination and systemic
examination was normal. Abdominal
examination was unremarkable.
Speculum examination showed healthy
cervix and vagina with minimal white
discharge. Bimanual pelvic examination
revealed a retroverted, bulky and mobile
uterus, with free fornices.
1. Gockley AA, Rauh-Hain JA, del Carmen MG. Uterine
leiomyosarcoma: a review article. International Journal
of Gynecological Cancer. 2014 Nov 1;24(9):1538-42.
Here we present case of leiomyosarcoma
of uterus detected in early stage due to
high degree of suspicion when patient
approached the hospital with trivial
symptoms
Discussion
Though endometrial samplingThough endometrial sampling
correctly identifies only 25-50%correctly identifies only 25-50%
of LMS pre-operatively (partlyof LMS pre-operatively (partly
because of their myometrialbecause of their myometrial
origin), every post menopausalorigin), every post menopausal
woman should be offered thewoman should be offered the
same as a part of routinesame as a part of routine
screening procedure whenscreening procedure when
symptomatic. Effective treatmentsymptomatic. Effective treatment
to prevent disastrous diseaseto prevent disastrous disease
progression can then be plannedprogression can then be planned
CONCLUSION
Specialized investigations
Ultrasonography with Doppler:
Vascular mixed echogenic lesion (3cm)
with few cystic spaces within the uterine
cavity.
PET–CT:
Relatively well defined metabolically active
enhancing soft tissue lesion (5.1 x 3.7 x
3.3 cm) in the endometrial cavity –
neoplasm
Enlarged left proximal external iliac lymph
node (15 x11 mm) likely metastatic .
Abdominal panhysterectomy with pelvic
lymphadenectomy was performed
Histopathology revealed SUBMUCOSAL
LEIOMYOSARCOMA and lymph node
negative for malignancy
Chemotherapy deferred as FIGO staging
IB post surgery
On follow up, clinically and with
ultrasound abdomen and CECT thorax,
no evidence of metastasis was found till
date
Adjuvant treatment with chemotherapy
(Doxorubicin/Docetaxel and gemcitabine)
or radiotherapy can be considered in
advanced disease as these have limited
role in early-stage disease
Histopathology