[Telangiectatic osteosarcoma secondary to a liposclerosing myxofibrous tumor: a case report]. / Ostéosarcome de type télangiectasique développé sur une tumeur fibromyxoïde liposclérosante : à propos d'un cas.

Malignant transformation of a fibrous dysplasia into an osteosarcoma is very rare. We report the case of an 84-year-old man with telangiectatic osteosarcoma of the upper femur arising in a previous fibrous dysplasia also known as liposclerosing myxofibrous tumor. The tumor was expressing the epithelial membrane antigen. This is the first described case of a malignant transformation into an osteosarcoma arising in a liposclerosing myxofibrous tumor. We discuss the main differential diagnosis with a review.

A p53 gene mutation in malignant fibrous histiocytoma associated with bone infarction.

Transformed sarcomas rarely arise from bone infarct lesions, although the majority of bone sarcomas are primary in origin. However, the pathogenesis of the condition is unknown. In this report, we describe a malignant fibrous histiocytoma with a p53 gene mutation. A 59-year-old woman complained of having pain in her left knee for three months. Plain radiographs of the distal metaphysis of her left femur revealed an ill-defined lytic lesion, which was consistent with a malignant tumor in the infarct lesion. An open biopsy specimen did not show any evidence of malignancy. Immunohistochemical examination of the biopsy specimen failed to show p53 protein-positive cells. However, a mutation in the p53 gene was detected when polymerase chain reaction/single-strand conformation polymorphism (PCR-SSCP) analysis was performed. A functionally relevant p53 missense mutation in codon 273 of exon 8 [CGT (Arg) -> CAT (His)] was confirmed by direct sequencing. We concluded that this lesion was a malignant bone tumor arising from the bone infarct lesion, and we thus performed a wide resection. The histopathological diagnosis of the resected specimen was that it was a malignant fibrous histiocytoma associated with bone infarction. Immunohistochemistry revealed that the tumor cells were positive for the p53 protein. To our knowledge, our patient is the first patient having a bone infarct-associated sarcoma with a p53 gene mutation. Identification of the p53 mutation helps in diagnosing the malignant transformation of the bone infarct lesion. One pathogenesis of this condition may be a mutation in the p53 gene.

VEGF expression in osteosarcoma correlates with vascular permeability by dynamic MRI.

Dynamic enhanced magnetic resonance imaging has been used to assess tumor angiogenesis in osteosarcoma. Vascular endothelial growth factor has been shown to correlate with pulmonary metastasis and a poor prognosis in osteosarcoma. The purpose of this investigation was to determine whether vascular endothelial growth factor expression in osteosarcoma correlates with vascular permeability detected by dynamic enhanced magnetic resonance imaging and to explore the role of dynamic enhanced magnetic resonance imaging as a noninvasive means of assessing tumor angiogenic activity. Fifty-five osteosarcoma patients with osteosarcoma enrolled in a treatment protocol that included dynamic enhanced magnetic resonance imaging. In 15 patients, tumor tissues were available for vascular endothelial growth factor immunohistochemical studies. A two-compartment model used the exchange rate constants (kep) between the plasma and tumor compartments to quantify vascular permeability during dynamic magnetic resonance imaging studies. Immunohistochemical staining for vascular endothelial growth factor was graded according to the intensity and number of positively stained cells. Vascular endothelial growth factor-positive tumors showed higher mean vascular permeability when compared with vascular endothelial growth factor-negative tumors. Vascular permeability also correlated with increasing vascular endothelial growth factor expression. The preliminary results in this study show an association between vascular endothelial growth factor and dynamic MR signal enhancement in osteosarcoma. Dynamic enhanced magnetic resonance imaging should be investigated as a means to prognosticate osteosarcoma patients with osteosarcoma according to their tumor angiogenic activity.

The role of microvascular surgery in limb-sparing procedures for malignant tumors of the knee.

Limb-sparing surgery has proven to be a feasible alternative to amputation for treatment of malignant tumors about the knee. Microvascular surgery and the possibility of providing healthy, stable soft-tissue coverage to the affected limb have expanded the possible role of limb-sparing techniques. Microvascular free flaps were utilized in 26 patients undergoing resection for large malignant tumors of the knee or adjacent to the knee. Patients were then followed for a minimum of 3 years to a maximum of 7 years and evaluated for tumor recurrence and limb function. Survival and disease-free interval were 68 and 77 percent, respectively, in stage II patients and only 50 and 0 percent, respectively, in stage III patients. Function, as determined by the MSTS functional evaluation system, was found to be fair to good in all patients. Microvascular free-tissue transfer has proven to be a valuable adjunct in limb-sparing surgery.

[Ultrasound monitoring of bone lesions in the treatment of Ewing's sarcoma]. / Monitorowanie zmian kostnych przy pomocy ultrasonografii w trackcie leczenia miesaka Ewinga.

In the paper, a patient suffering from Ewing's sarcoma is presented. She was treated with chemo- and radiotherapy. As one of the methods of monitoring of the course of treatment and follow-up after finishing it has been ultrasound examination. In the ultrasound examination I have observed gradual diminishing of the tumor, up to it's complete disappearance. In the bone there have remained only two small hyper-echogenic focuses not changing their shape or size. At the beginning in Doppler examination a large arterial flow was found in the tumor, having low vessel resistance. In the course of treatment systolic velocity decreased and vessel resistance increased up to complete disappearance of the flow in the visible small hyper-echogenic focuses in the bone.

Combined MRI and MRA for limb salvage planning.

Magnetic resonance angiography (MRA) was performed in two patients with primary bone neoplasms being staged for differing types of limb-salvage procedures. A gated two-dimensional (2D) phase contrast MRA sequence that is capable of variable velocity encoding throughout the cardiac cycle was used to acquire the MRA images. The resulting cardiac phase images were added with a matched filter algorithm to create a single high signal-to-noise ratio image. An SE MR image in the same plane and field of view was then added to the MRA image. This displayed the relationship of vascular and stationary tissues within a composite picture: a "2D combined angiographic-stationary tissue" image. The anatomy represented gave information equivalent to conventional angiography. While this technique is not an ideal 3D rendering, it is easier to use. Along with other SE sequences acquired as needed for staging, it provides a complete preoperative evaluation of a tumor bed or donor site for a vascularized graft harvest.

Microvascular anastomosis of a rectus abdominis free flap into a prosthetic vascular bypass graft.

Distal bypass grafts are frequently employed to revascularize lower extremities and to replace major peripheral vessels following excision of large soft-tissue or bone tumors. Autogenous vein grafts are generally used for such bypasses, when available. Large wounds associated with peripheral vascular disease or excision of massive tumors frequently require concomitant free-tissue transfers. Anastomosis of a free-tissue transfer pedicle to a vein bypass graft has been reported. The first successful case of an anastomosis of a flap pedicle to a Gore-tex bypass graft is presented.

Three-phase skeletal scintigraphy for suspected bone tumors.

The advantage of using three-phase skeletal scintigraphy rather than merely conventional delayed imaging in the investigation of suspected bone tumors is illustrated by two cases in which this procedure provided greater information on the site and extent of the lesion than other single modalities.

Hibernoma: distinctive light and electron microscopic features and relationship to brown adipose tissue.

Four hibernomas and samples of developing human brown and white adipose tissue were observed. Distinctive features of hibernomas were 1) lobules of closely apposed large polygonal cells and capillaries; 2) three principal cell types (granular eosinophilic, multivacuolated, and univacuolated) varying in prominence from case to case; 3) investment of each tumor cell by basal lamina; 4) an inverse relationship between lipid droplet size and the number of mitochondria per unit of cytoplasm; 5) pleomorphic mitochondria with dense matrixes or large, round mitochondria with transverse lamellar cristae; 6) undulating plasmalemmal invaginations; 7) micropinocytotic vesicles; 8) periodic short plasmalemmal densities; and 9) a conspicuous lack of cytoplasmic membrane systems. The frequent association of micropinocytotic vesicles and undulating plasmalemmal invaginations in proximity to capillaries strongly suggests that the invaginations represent a localized cell membrane adaptation for efficient endocytosis. In human fetal brown adipose tissue, which is first recognizable in fetuses of 21 weeks' gestational age, the most characteristic cell was the polygonal multivacuolated cell. Univacuolated cells were present in brown adipose tissue of older fetuses, and in infants and adults entire lobules containing univacuolated cells coexisted with lobules of multivacuolated cells and granular eosinophilic cells. The ultrastructure of human brown adipose tissue resembled that of hibernomas and was similar to previously described features of this tissue in animals. Developing white adipose tissue differed from brown adipose tissue by its loose plexiform arrangement of capillaries and spindle-shaped cells in less circumscribed lobules and by the absence of polygonal multivacuolated cells. The authors did not identify centripetal lobular maturation in white adipose tissue, but peripheral growth "caps" were a common finding in maturing brown adipose tissue. They consider brown adipose tissue to be a special form of adipose tissue, the variable cytologic composition of which is reflected in the histologic spectrum of hibernomas.

Angiography in giant-cell bone tumors.

Angiography was performed on 48 patients with giant-cell bone tumors. Sixty-four per cent were hypervascular; 25% were hypo- or moderately vascular; and 10% were avascular. Findings were similar in the 45 benign and 3 malignant tumors. There was no correlation between the findings and degree of histological aggressiveness. There was a high (60%) incidence of aneurysmal bone cyst component in the hypovascular group. Hyperemic synovium, occasionally simulating tumor, was seen in 80% of surgically proved lesions. Angiography was useful in assessing tumor extent, particularly when an extraosseous component was present.