Primary leiomyosarcoma of bone

Abstract

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.

Primary Leiomyosarcoma of Bone: Analysis of Prognosis

“…Generally, it is felt that there is an equal distribution between the sexes for primary leiomyosarcomas [2, 7, 8]; however, our series reflects a slight male predominance, as do several other smaller studies [9, 11, 12]. In terms of tumour locality, our cohort agreed with the consensus that long bones are primarily affected, predominantly the distal femur and proximal tibia with 45% and 26% of patients, respectively [2, 5, 13, 14]. Interestingly, other studies have found the craniofacial skeleton to be the second most common area affected, while none of our patients had skull tumours, this most likely reflecting our local referral practice [15–19].…”

Leiomyosarcoma of sacrum: imaging and histopathologic findings

“…Most recently, Jeanrot et al described 5 cases of PLB [5]. There are few radiologic reports of this entity in the literature; a clear gender predilection has not been established [3,6,7]. In our case, gynecologic study and pelvic sonography ruled out a primary lesion of the uterus.…”

“…Sclerosis at the infero-medial margin of the tumor (arrows) is observed Fig. Berlin et al [3] performed angiography in six patients with PLB. The tumor extends to the sacroiliac joint, presacral fat (solid arrow) and S1 nerve root (arrows).…”

Computed tomography appearance of bone metastases of leiomyosarcoma

“…Leiomyosarcoma rarely causes osseous lesions, either as a primary or metastatic disease [1,2,10,12]. Radiologically, primary leiomyosarcoma presents most often as a purely osteolytic lesion with highly malignant characteristics [1,2,7].…”