Primary bone leiomyosarcoma of distal femur: case report and literature review

We present the case of a 58-year-old patient presented with a spontaneous right supracondylar fracture. The initial bone biopsy, highlighted the defining histopathological (HP) elements for a leiomyosarcoma (LMS), initially considered a metastasis. The complex imaging examinations did not reveal ano...

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Bibliographic Details
Published in:	Romanian journal of morphology and embryology Vol. 63; no. 3; pp. 569 - 574
Main Authors:	Georgeanu, Vlad Alexandru, Pletosu, Raluca Ioana, Vlădescu, Teodora Camelia, Bondari, Simona, Crăciunescu, Andrea, Russu, Octav Marius
Format:	Journal Article
Language:	English
Published:	Romania Academy of Medical Sciences, Romanian Academy Publishing House, Bucharest 01-07-2022
Subjects:	Bone Neoplasms - pathology Case Report Femur - pathology Humans Leiomyosarcoma - pathology Middle Aged Soft Tissue Neoplasms - pathology
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Summary:	We present the case of a 58-year-old patient presented with a spontaneous right supracondylar fracture. The initial bone biopsy, highlighted the defining histopathological (HP) elements for a leiomyosarcoma (LMS), initially considered a metastasis. The complex imaging examinations did not reveal another tumor, so the final diagnosis was primary bone LMS. Final treatment was a wide tumor resection and reconstruction with a knee tumor prosthesis, preceded and followed by three cytostatic cycles (Doxorubicin 75 mg∕m²). The HP examination has confirmed the previous diagnosis. The key microscopic features for the diagnosis of bone LMS was: malignant mesenchymal proliferation composed of intersecting fascicles of cells with eosinophilic, fibrillary cytoplasm and pleomorphic, elongated, blunt-ended, cigar-shaped nuclei of variable sizes; variable mitotic count; presence of tumor necrosis and stroma with changes that include hyalinization, myxoid change, with absence of chondroid or osteoid matrix; diffuse positivity for smooth muscle immunohistochemical markers: smooth muscle actin, desmin, h-caldesmon. At 12 months after the tumor resection, the patient is in good condition without any sign of local recurrence or metastatic disease. LMS represents a type of soft tissue sarcoma (STS), a variant of the spindle cell sarcomas, accounting for about 7% to 10% of all STS. Bone LMS can be primary or secondary; the primary variant is very rare, representing a very small percentage (around 0.7%) of all primary malignant bone tumors, according to the literature data. Very few cases are presented in the literature; the management of this kind of tumor is controversial, especially regarding the chemo- and radiotherapy.
ISSN:	1220-0522 2066-8279
DOI:	10.47162/RJME.63.3.12