Leiomyosarcoma in bone: Primary or secondary? (original) (raw)
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Primary leiomyosarcoma of bone: a report on three patients
Clinical Imaging, 2008
Primary leiomyosarcoma of bone is a rare aggressive tumor that occurs mainly in older people. It resembles other malignancies clinically and radiologically, and differential diagnosis is based on histopathology, including immunohistochemistry. If leiomyosarcoma is found in bone, the possibility that it is a metastasis from a distant tumor should be investigated. The treatment is surgical excision with wide margins. Here we present three patients who had primary leiomyosarcoma of radius, ilium, and femur, respectively, and who were treated surgically.
Primary leiomyosarcoma of bone: A study of five cases
Cancer, 1980
Five cases of leiomyosarcoma of bone are described. The patients, 3 men and 2 women, ranged in age from 24 to 74 years. Four of the five tumors were located in the distal half of the femur. Radiographically, all tumors presented as purely osteolytic lesions. Angiography, performed in 3 cases, revealed hypervascularity and the presence of irregular, tortuous vessels at the site of the lesion in 1, hut not in 2 others. Histologically, the five tumors showed features characteristic of leiomyosarcomas. The most prominent features were blunt-ended, cigar-like, chromatin-rich nuclei in elongated acidophilic cells arranged in bundles which intersected each other at wide angles. The importance of trichrome stains, such as van Gieson stain, for the recognition of leiomyosarcomas is emphasized. Electron microscopic examination, performed in 3 cases, showed as the most prominent features spindle-shaped cells in parallel arrangement containing abundant myofilaments with elongated densities, pinocytotic vesicles, and basal lamina structures.
Leiomyosarcoma of Bone: A Case Report
Case Reports in Medicine, 2011
The aim of this paper is to present clinicopathologic features and immunohistochemical findings of a primary leiomyosarcoma of bone occurring in the proximal femur of a 46-year-old Caucasian male patient. Each case report on this exceedingly rare entity contributes to the notion of this disease.
Primary leiomyosarcoma of bone
Skeletal Radiology, 1987
Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light-and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years.
Annals of Diagnostic Pathology, 2011
Primary leiomyosarcoma of bone is a rare and a diagnostically challenging tumor entity. Over a 7year period, we identified 8 such cases that fulfilled the diagnostic criteria in 6 men and 2 women, with age ranging from 25 to 59 years (mean, 42.7 years). All cases were noted in the lower limbs, including femur and tibia as the commonly involved bones in 4 and 3 cases, respectively. On radiography, the most consistent feature was a solitary osteolytic lesion with cortical destruction, unassociated with matrix formation. On histopathology, all cases showed spindly sarcomatous cells, mostly arranged in fascicles and whorls. Of 8 cases, 6 (75%) were of high grade. Prominent vasculature was noted in 5 cases. Two cases displayed focal mineralization, including calcification and heterotropic woven bone formation in 1 case each, but lacked malignant osteoid or chondroid matrix. One case showed osteoclast-like giant cells. On immunohistochemistry, smooth muscle actin was diffusely positive in all cases (100%), desmin was positive in 6 (75%) of 8 cases, and h-caldesmon was positive in 5 (83.3%) of 6 cases. Five cases underwent surgery, including 3 amputations and 2 wide excisions. One case underwent chemotherapy. On follow-up, 5 cases developed metastasis, including 1 case with another, who died within 17 and 5 months. Leiomyosarcoma of bone is uncommon and diagnostically challenging. An index of suspicion is necessary for this diagnosis, especially in cases of lytic, destructive bone lesions, unassociated with matrix production, that show spindly sarcomatous cells on histopathology. Immunohistochemical analysis, including an optimum panel formed by smooth muscle actin (diffuse positivity), desmin, and h-caldesmon, is necessary for substantiating this diagnosis. Surgery forms the treatment mainstay. The prognosis appears to be dismal.
Primary Leiomyosarcoma of Bone: Analysis of Prognosis
Sarcoma, 2012
Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type of primary malignant bone tumour.Method. Retrospective review of data stored on a prospective database.Results. In a database of 3364 patients with primary malignant bone sarcomas, 31 patients were identified with a primary leiomyosarcoma of bone. There were 12 males and 19 females with a mean age of 46 and tumour size of 8 cm. The most common site was the distal femur followed by the proximal tibia. Treatment was with chemotherapy and surgical resection. Seven of the patients had metastases at diagnosis. Surgery was carried out in 28 patients, 8 having amputation and 20 limb salvage. Three patients developed local recurrence, but half developed metastases. All patient disease-specific s...
Primary leiomyosarcoma of bones – a rare entity in two different presentations
European Journal of Clinical and Experimental Medicine
Introduction. Leiomyosarcomas (LMS) originate from smooth muscle cells. They are very rare malignant neoplasms. Bony Leiomyosarcoma is a variant of spindle cell sarcoma, primarily affecting long bones, predominantly the distal femur and the proximal tibia followed by craniofacial skeleton. Aim. To describe clinical presentation and diagnostic approach of primary leiomyosarcoma of bones in two different patients. Description of the cases. Case 1. A 64-year-old male with a fracture of left distal femur after a fall was investigated and found to have a pathological fracture. An open biopsy of the fracture site confirms leiomyosarcoma. Case 2. A 58-year-old previously healthy female presented with a swelling on right side mandibular region. Orthopantomogram radiograph (OPG) of mandible and Cone beam CT (CBCT) mandible was taken initially and revealed a large area of bone destruction of the right side of the mandible associated with a soft tissue mass. Initial incisional biopsy made the ...
Primary Skeletal Leiomyosarcoma
Radiology Case Reports, 2012
A 28-year-old female presented with gradual onset of left-knee pain without a preceding traumatic event. Initially, her primary care physician diagnosed her with a meniscal tear based on physical exam. She was put into a brace, and the pain subsided after 2 weeks of conservative therapy. Two months later, the left-knee pain began increasing. She again saw her primary care doctor, who recommended an MRI, but because of insurance problems, she was unable to obtain one. A radiograph was not performed. She was given some narcotics, and again her pain improved. She subsequently moved across the nation, and the leftknee pain returned. A new physician examined her and diagnosed a "muscle strain," subsequently referring her to physical therapy. Her physical therapist recommended radiographs. The radiographs demonstrated a poorly defined lytic lesion arising from the distal left femur with a wide zone of transition, cortical destruction, and a small anterior soft-tissue component (Fig. 1). At the time of repeat examination after imaging, she denied any pain in her left knee except at night and with full extension. She also denied any fevers or chills, or any pain in her tibia or her hip. An MRI was obtained to further characterize the distal femoral lesion. This demonstrated a heterogeneously enhancing intramedullary mass within the distal metaphysis of the left femur (Figs. 2 and 3). There was cortical breakthrough anteriorly, with an enhancing soft-tissue component. 18fluorodeoxyglucose PET/CT and a Tc-99m MDP bone scan both showed marked radionuclide activity in the lesion (Figs. 4 and 5). Findings were most consistent with an osteosarcoma. At resection, the pathology demonstrated well-differentiated leiomyosarcoma that involved the medullary cavity, with focal extension through the cortex into the surrounding soft tissue (Fig. 6A). Histologically, the mass contained fascicles of highly mitotic spindle cells that
Primary bone leiomyosarcoma of distal femur: case report and literature review
Romanian Journal of Morphology and Embryology, 2022
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