Primary Leiomyosarcoma of Bone: Review and Update

Abstract

Context.— Leiomyosarcoma of bone is a rare primary osseous sarcoma characterized by smooth muscle differentiation and absence of malignant osteoid formation. Leiomyosarcoma of bone is diagnostically challenging; this can be improved with greater awareness of this entity and the ability to differentiate it from its histologic mimics. Because of its rarity, only a small number of studies are available in the literature. These factors contribute to our limited understanding of its pathology, prognosis, and treatment. Objective.— To review the clinicopathologic features of leiomyosarcoma of bone and present the most up-to-date understanding of its behavior and management in accordance with the current literature. Data Sources.— Review of pertinent literature on the major features, current knowledge thereof, and the authors' experience in the diagnosis and management of leiomyosarcoma of bone. Conclusions.— Leiomyosarcoma of bone is a rare but well-recognized primary osseous sarcoma that may arise de novo or in association with radiation. Although it is diagnostically challenging, awareness of this rare sarcoma and knowledge of its key histomorphologic and immunohistochemical features allow for accurate diagnosis.

Comparison of clinicopathological features and outcomes in patients with primary leiomyosarcoma of bone and soft tissue

“…This study found high‐grade tumors, initial distant metastases, nodal metastases, and tumor size ≥5 cm were each negative predictors of survival for both PLB and SLMS when grouped together. These results seem to align with the current literature detailing the aggressive nature of leiomyosarcoma, namely SLMS 5,15,16,18,19 . However, very few data describe the tumor characteristics of PLB, nor do they identify very many prognostic factors as these studies are mostly limited to case reports and small series 4,5,14,17,20–25 .…”

“…Then in 1991, the immunohistochemical profile of PLB was further elucidated by Meyers et al, 3 who observed that PLB, which had often been misdiagnosed as malignant fibrous histiocytoma or fibrosarcoma, is a distinct clinicopathologic entity that may be more common than previously recognized. Since then, few studies have cited modern histopathological capabilities as a potential cause for an increased incidence of both PLB and SLMS 15 . Despite the reported increase in the incidence of these tumors over time, however, there is a lack of data informing the proper management of PLB, as well as SLMS.…”

Dedifferentiated soft tissue leiomyosarcoma with heterologous osteosarcoma component: case report and review of the literature

“…Moreover, sinonasal tract LMSs are exceptionally rare with no more than 100 cases reported in the English literature [4]. LMS can also occur in bone, as primary or secondary tumor localization from distant sites, although the former is rare, with about 0.7% incidence of all primary malignant bone tumors [5].…”

Case report and literature review: Primary leiomyosarcoma of the bone in the trochanteric region of the femur

“…No markers of myogenic tumors are observed in fibroblastic osteosarcoma lesions, distinguishing them from LMSB (7,27). And SATB2 is a relatively specific marker of osteosarcoma which is also helpful in differentiating between LMSB and osteosarcoma (7,27). Metastatic sarcomatoid carcinoma has a unique immunohistochemical feature as it expresses p63 and PAX8 and lacks the markers of myogenic tumors such as SMA and desmin.…”