Abstract
#Primary leiomyosarcoma (LMS) of the bone is a very rare tumor with #smooth muscle differentiation on #immunohistochemical studies.
Radiologically, LMS appears as an osteolytic lesion with aggressive
features but no #pathognomonic radiological findings that helps making
the diagnosis. We report one case of primary LMS of the femur with
fluid-fluid levels (FFLs) on the MRI, the first case to our knowledge.
We consider we should include primary LMS of the bone in the
differential diagnosis of "FFL” lesions. LMS is a malignant tumor comprising of spindle-shaped cells with
distinctive smooth muscle cell features [1]. They are approximately 7%
to 10% of all soft tissue sarcoma [2]. Most of these tumors occur
frequently in uterus and soft-tissues and more rarely in bone [3-5]. The
first description of a primary bone LMS in a long bone was given in
1965 [1]. Since then, to the best of our knowledge, around 130 cases of
primary LMS of bone have been reported, mostly small case reports that
have focused on its #histological diagnosis [4]. The clinical behavior of
osseous LMS is generally aggressive. Most of the published studies of
LMS of the bone have reported poor prognosis with a 35% overall survival
rate [6]. LMS diagnosis is established by the presence of #morphologically typical spindle cells with smooth muscle differentiation and the positivity of a
smooth muscle actin and other muscle markers on tumor cells, such as
#desmin and h-caldesmon [7]. Furthermore, the specific characteristic of
primary bone LMS is a solitary osteolytic lesion with indistinct margins
and cortical destruction with the absence of either osteoid or
chondroid matrix [8,9]. Since its recognition as a distinct entity, to
our knowledge no sizeable series has examined its imaging features.
Furthermore, only a few studies about primary LMS of the bone with
emphasis on MRI findings have been published [10]. We present the
clinical features, radiological findings and pathological description of
a primary LMS originating from the distal femur in a middle-aged lady.
Best to our knowledge, this is the first case report of "FFLs” on MRI of
a primary LMS of the bone.
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