Primary leiomyosarcoma of bone—a clinicopathologic study of 8 uncommon cases with immunohistochemical analysis and clinical outcomes

Rekhi, Bharat; Kaur, Amrit; Puri, Ajay; Desai, Saral; Jambhekar, Nirmala A.

doi:10.1016/j.anndiagpath.2010.11.006

Cited by 35 publications

(48 citation statements)

References 31 publications

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“…1,2,14 The median age at diagnosis is during the fifth decade, and males and females are equally affected. It is typically an aggressive malignancy with a 5-year overall survival rate of 59%, similar to conventional osteosarcoma.…”

Section: Discussionmentioning

confidence: 99%

“…LMS can rarely present as a primary tumor of bone with only 147 reported cases, where it is thought to arise from smooth muscle of intraosseous blood vessels, pleuripotent mesenchymal stem cells, or intermediate cellular forms such as myofibroblasts. 1,2 The differential diagnosis includes primary intraosseous leiomyoma, which is an exceedingly rare tumor with about two dozen cases reported. 3,4 …”

Section: Introductionmentioning

confidence: 99%

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Primary Intraosseous Smooth Muscle Tumor of Uncertain Malignant Potential: Original Report and Molecular Characterization

Kropp

Siegal

Frampton³

et al. 2016

Rare Tumors

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We report the first case of primary intraosseous smooth muscle tumor of uncertain malignant potential (STUMP) which is analogous to borderline malignant uterine smooth muscle tumors so designated. The tumor presented in the femur of an otherwise healthy 30-year-old woman. Over a 3-year period, the patient underwent 11 biopsies or resections and 2 cytologic procedures. Multiple pathologists reviewed the histologic material including musculoskeletal pathologists but could not reach a definitive diagnosis. However, metastases eventually developed and were rapidly progressive and responsive to gemcitabine and docetaxel. Molecular characterization and ultrastructural analysis was consistent with smooth muscle origin, and amplification of unmutated chromosome 12p and 12q segments appears to be the major genomic driver of this tumor. Primary intraosseous STUMP is thought to be genetically related to leiomyosarcoma of bone, but likely representing an earlier stage of carcinogenesis. Wide excision and aggressive follow-up is warranted for this potentially life-threatening neoplasm.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Primary Intraosseous Smooth Muscle Tumor of Uncertain Malignant Potential: Original Report and Molecular Characterization

Kropp

Siegal

Frampton³

et al. 2016

Rare Tumors

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“…5 The differential diagnosis on histology includes fibrosarcoma, malignant fibrous histiocytoma, myofibrosarcoma, metastatic carcinoma and osteosarcoma. 15,20 The microscopy in the present case showed eosinophilic spindle cells with vesicular, ovoid to cigar-shaped nuclei, arranged in fascicles. The tumour cells showed high mitotic activity with necrotic areas.…”

Section: Case Reportmentioning

confidence: 92%

“…studies have described expression of smooth muscle actin (SMA), desmin, or vimentin in an objective identification of smooth muscle origin. 2,[4][5][6][7][8][9][10][11][12][13]15,18,19 Electron microscopy showed definite smooth muscle differentiation including cytoplasmic filaments with dense bodies, basement membranes and pinocytotic vesicles. 4,5,7,8,11,12,18 According to Young, immunohistochemistry allows the histological diagnosis to be made without the need to resort to ultrastructural studies.…”

Section: Case Reportmentioning

confidence: 99%

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Primary leiomyosarcoma of the first metatarsal bone: A case report

Singh¹,

Kumar²,

Kamau³

et al. 2017

SA orthop. j.

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A 60-year-old male presented with nine-month history of pain and progressively increasing swelling over the dorsomedial aspect of the right foot. It started as a small painful swelling over the dorsum of the foot. The patient developed a non-healing fungating ulcer at the biopsy site during the course of disease (Figures 1a and b). There was no history of trauma, fever or discharging sinus, and no history of any other swelling in the body. There was no history of radiation exposure. The patient was non-diabetic and did not present with any other systemic ailment. On examination, a diffuse, hard swelling about 10×15 cm was present over the dorsomedial aspect of the right foot. A fungating ulcer, about 4×4 cm in size, was present over the swelling. There was full range of motion of the ankle. Examination of the chest and abdomen was normal.Plain radiograph of the foot showed a poorly defined lytic lesion with complete destruction of the first metatarsal bone extending into the surrounding tissues, second metatarsal, medial and middle cuneiform (Figure 2). AbstractIntroduction: Primary leiomyosarcoma of bone is a rare entity which has never been reported in the foot. We report a case of primary leiomyosarcoma of metatarsal bone.

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Rare bone sarcomas: A retrospective analysis of 145 adult patients from the French Sarcoma Group

Boudou‐Rouquette

Martin

Kempf

et al. 2021

Intl Journal of Cancer

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The benefit of chemotherapy (CT) in rare bone sarcomas is not documented in prospective studies. Our retrospective study from the French sarcoma network for bone tumors ResOs was performed in adult patients (pts) from 1976 to 2014, with histologically verified diagnosis of leiomyosarcomas (LMS), undifferentiated pleomorphic sarcoma (UPS) or radiation-associated sarcomas of bone. The median follow-up was 4.7 years (95% CI: 3.7-6.5). Clinical features, treatment modalities and outcomes were recorded and analyzed from 145 pts (median age 53 years [range 20-87]). Site of disease was extremities (66%) or axial skeleton (34%), 111 (77%) presented with localized and potentially resectable disease. The most common histological subtypes were UPS (58%) and LMS (33%); 58% were high-grade tumors. Surgery was performed in 127 pts. In the 111 localized pts, 28 pts (25%) underwent upfront surgery or exclusive radiotherapy (RT; >50 Gy) without CT, whereas 83 pts (75%) received either neoadjuvant (n = 26) or adjuvant CT (n = 13) or both (n = 44). Neoadjuvant and adjuvant CT was mostly doxorubicin-based (95%/86%) and cisplatin-based (67%/63%). R0 resection was achieved in 59 pts, and a good histological response in 15 patients (25%). Adjuvant RT was performed in 24 (22%) pts. For the whole cohort (n = 145), the 5-year overall survival (OS) rate was 53% [42; 62]. In univariate analysis, age ≤ 60 was associated with a longer disease-free survival (DFS) (P = .0436). Neoadjuvant and adjuvant CT tended to be associated with better DFS (P = .056) with no significant impact on OS in this retrospective series.

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Primary leiomyosarcoma of bone—a clinicopathologic study of 8 uncommon cases with immunohistochemical analysis and clinical outcomes

Cited by 35 publications

References 31 publications

Primary Intraosseous Smooth Muscle Tumor of Uncertain Malignant Potential: Original Report and Molecular Characterization

Primary Intraosseous Smooth Muscle Tumor of Uncertain Malignant Potential: Original Report and Molecular Characterization

Primary leiomyosarcoma of the first metatarsal bone: A case report

Rare bone sarcomas: A retrospective analysis of 145 adult patients from the French Sarcoma Group

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