Introduction
The Soft tissue sarcomas (STS) are exceptionally rare
mesenchymal derived tumors accounting for only 1% of all malignancies.
Leiomyosarcoma (LMS) include 5-10% of STS cases that displays
heterogeneous subtype of malignant mesenchymal tumor originated from
smooth muscle tissues 1. The LMS Commonly diagnosed in
the fifth and sixth decades of life, and it can be detected every
anatomic site, including the uterus, retroperitoneum, extremities, and
vasculature 2. The two primary categories of LMS
contains uterine LMS (uLMS) and extrauterine (euLMS) types, the first is
commonest subtype of uterine sarcoma, while metastatic euLMS are not
well described in terms of treatment, outcomes and prognostic factors3. The treatment of this tumor is controversial.
However, regardless the site of origin, surgical resection is
cornerstone treatment for localized LMS 2,4. The
standard surgical procedure includes a complete excision with wide
negative margins that gives the best chance of cure 2.
Pelvic external beam radiation therapy with or without brachytherapy are
recommended for locally resected tumors, especially for advance
metastatic disease, chemotherapy considered as an option2,4. In the euLMS is not clear that the choice of
specific agent or sensitivity to first-line systemic therapy affects the
response rate and clinical outcomes 5.
The molecular heterogeneity of leiomyosarcoma is uknown also targeted
therapy not available now, detection of different molecular subtypes is
urgent to weigh novel therapeutic options. Two diagnostic
immunohistochemical markers newly detected in formalin-fixed,
paraffin-embedded tissues; are LMOD1 (Leiomodin 1) in subtype I and
ARL4C (ADP-ribosylation factor-like protein 4C) in subtype II
leiomyosarcoma 6,7. Acording leiomyosarcoma tissue
microarray and considering clinical outcome, specified that subtype I
leiomyosarcoma is associated with good outcome in extrauterine
leiomyosarcoma nonetheless, subtype II is related to poor prognosis in
uterine and extrauterine leiomyosarcoma 7.
Due to rarity and few confirmed cases reported in the literature, this
case report presents a young female patient with euLMS, invading the
ovary, uterus, small intestine, sigmoid colon, rectum, omentum and
pancreas, which were treated with radical surgery and adjuvant
chemotherapy.