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Treatment planning and delivery in neutron radiotherapy of soft tissue sarcomas.

Well differentiated soft tissue sarcomas may benefit from fast neutron radiotherapy, in particular inoperable and recurrent tumors and tumors with residual disease after non-radical surgery. Treatment planning in a multidisciplinary pretherapeutic approach has to be based on tumor size and site and histopathology. Target volume definitions for potential microscopic spread and for the high risk region of local recurrence have to consider the preoperative tumor localization (imaging), the biological behavior and the extent of surgery. GI/II, T1-3 tumors after intralesional or marginal resection are indications for neutron therapy. Treatment planning and delivery has to take into account the narrow therapeutic range of fast neutrons and include individual immobilization devices, manufacturing of bolus and CT based computed dose calculations. Neutron radiotherapy techniques at the d, T generator are comparable to photon techniques except individual beam shaping, field size and portal verification. Total dose is 16 Gy neutrons in the high risk region and 12 to 13 Gy in the region of potential microscopic spread.

Combined Modality Therapy↗

Presence of steroid receptors in human soft tissue sarcomas of diverse histological origin.

The incidence of cytosol receptors for androgen, estrogen, glucocorticoid, and progesterone was determined in a series of histologically diverse soft tissue sarcomas. Receptor binding was characterized in tumor specimens from 29 adult patients (14 female, 15 male) obtained during surgery. Nineteen (66%) tumor cytosols bound at least one steroid. Seven (24%) had more than one receptor. Ten of 23 (43%) cytosols assayed for androgen binding were positive (Kd 0.97 +/- 0.23 X 10(-9) M; 60.6 +/- 25.9 fmol/mg protein), 7 of 29 (24%) for estrogen (Kd 1.00 +/- 0.19 X 10(-9) M; 33.2 +/- 10.9 fmol/mg protein), 9 of 26 (35%) bound glucocorticoid (Kd 4.22 +/- 1.77 X 10(-9) M; 231.0 +/- 155.0 fmol/mg protein), and 1 of 28 (4%) bound progesterone (Kd 0.41 X 10(-9) M; 18.7 fmol/mg protein). Density gradient analysis suggested that androgen and estrogen binding was located predominantly in the 6S and 7 to 8S regions, respectively, whereas receptor for glucocorticoid sedimented at 8S. Steroid binding was not related to patient age. Estrogen-positive (71%) and glucocorticoid-positive (78%) cytosols appeared predominantly in tumors from female patients. Receptor distribution also appeared to be correlated to tumor histogenetic origin.

Adult↗

Sarcomas of the female genital tract and pelvic soft tissues. A cluster of 14 cases in one year.

We report ten uterine and four extrauterine sarcomas encountered in a twelve month period (1989-90). The seven new cases of uterine sarcomas represent an estimated one third of all cases of malignancy of the corpus uteri, which were diagnosed at the gynaecology departments of St James's and Adelaide hospitals in one year. Overall, the prognosis was poor in the uterine sarcoma group-only one patient in clinically tumour free and five have succumbed to their disease. Delay in diagnosis especially in younger women with fibroid uterus contributed to this outcome.

Adenocarcinoma↗

[Renal sarcoma].

Among 322 cases of primary malignant renal tumours reviewed between 1969 and 1991, six were renal sarcoma (1.85%). They all presented earlier symptomatology which was not different from other renal tumours, and nephrectomy was performed in all cases, 5 of which died before 14 months. The histopathological diagnose showed two liposarcoma, two carcinosarcomas, all the others being fibrosarcomas.

Adult↗

Computed tomography of soft-tissue lesions of the extremities, pelvic and shoulder girdles: sonographic and pathological correlations.

Sixty-five patients with various soft-tissue tumours were examined by computed tomography (CT) and ultrasonography. Results were correlated with pathological findings. Although an accurate histological prediction could not be made in most cases, certain patterns appeared to be characteristic of specific types of tumours. A lipoma of uniformly low density may be differentiated from the heterogeneously denser liposarcoma. Multiple irregular linear densities with calcification are characteristic of haemangioma. An echo-free mass on the sonogram with soft-tissue density on CT suggests lymphoma or certain sarcomas. Small isodense subcutaneous lipomas or intramuscular lymphomatous masses may be clearly delineated on sonogram but not seen on CT. Our study indicates that the combined use of CT and ultrasound gives a very detailed basis for planning the surgical approach to the tumour.

Adolescent↗

The gray scale sonographic appearance of intra-abdominal mesenchymal sarcomas.

Twenty-eight patients with intra-abdominal mesenchymal sarcomas were examined with gray scale ultrasonography. Masses were present in the mesentery, abdominal hollow viscera, liver, and retroperitoneal areas. Four different ultrasonic patterns were observed in the extra hepatic abdominal masses: (a) hyperechoic masses intermixed with anechoic zones; (b) hyperechoic masses with central fluid-filled zones; (c) homogeneous hyperechoic masses; and (d) homogeneous hypoechoic masses. Three of 6 patients with hepatic masses were totally anechoic alongside other more typical metastatic patterns. The presence of a large mass with sonographic signs of necrosis should strongly suggest the possibility of sarcoma. The differential diagnosis is discussed.

Abdominal Neoplasms↗

Bone tumors of mixed origin: osteo-liposarcoma and osteo-rhabdomyosarcoma.

1. A complete perusal of the literature revealed twenty cases of primary liposarcoma of bone acceptable as such to the authors. These were tabulated as to location and age. 2. Eight cases of osteo-liposarcoma, primary in bone, were encountered in the literature and an additional case was reported by the authors. 3. The authors described for the first time in the literature a new primary tumor of bone of mixed origin: osteo-rhabdomyosarcoma. After careful perusal of the literature they added three additional cases: two cases, previously reported as primary rhabdomyosarcoma of bone, which on careful evaluation of the radiographs in said publications and the paucity of microphotographs they considered to be osteo-rhabdomyosarcomas, and the other case, previously reported as malignant mesenchymoma of the sternum following radiotherapy for breast cancer. 4. The authors prefer to classify these tumors (osteo-liposarcoma and osteo-rhabdomyosarcoma) as "Tumors of Mixed Origin" and not as "Malignant Mesenchymomas". 5. A complete review of the literature revealed 219 reported "dedifferentiated" chondrosarcomas, or chondrosarcomas "with additional mesenchymal component", among which only nine (9) contained a bona fide rhabdomyosarcomatous component. The rest exhibited other mesenchymal tumors as osteogenic sarcoma, fibrosarcoma, malignant fibrous histiocytoma, angiosarcoma, and undifferentiated sarcoma. The authors recommend to continue classifying these tumors as chondrosarcomas with additional mesenchymal component or even as "dedifferentiated" chondrosarcomas but not as malignant mesenchymomas.

Adolescent↗

Grading in soft tissue tumors: principles and problems.

Histologic grading has been considered the most important prognostic factor for soft tissue sarcomas. Several grading systems have been proposed based on the assessment of morphologic features in heterogeneous groups of sarcomas. Currently, the French Federation of Cancer Centers (FNCLCC) and the National Cancer Institute (NCI) grading systems are the most commonly used. These systems are based on a few morphologic predictors of biologic behavior, which is justifiable because of the rarity of soft tissue sarcomas. Nonetheless, over- or underestimation of prognosis may occur because of an uneven representation of specific sarcomas with rather distinct biologic behaviors among studies of grading systems. In addition, lack of standardization of morphologic criteria and frequent omission of the influence of clinical factors on the final survival analyses preclude universal acceptance of a particular grading system. New advances in diagnostic imaging, quantitative morphometric technologies, cytogenetics, and molecular genetics, allied with alternative analytic data systems, may provide better validation, reproducibility, and prognostic capabilities for current and future grading systems. This article summarizes and critically analyzes the various important grading systems that have thus far been proposed and suggests alternatives for the elaboration of more reproducible systems with higher predictive capabilities.

Adolescent↗

Cytogenetics and experimental models of sarcomas.

Bone and soft tissue sarcomas are diagnostically challenging. Recognition of specific cytogenetic abnormalities in these neoplasms has significantly reduced some of the associated difficulties and has provided valuable information on histopathogenesis. Commonly, translocations involving an exchange of chromosomal material and creation of novel chimeric genes are detected. These fusion genes frequently function as aberrant transcription factors that contribute to sarcomagenesis. New studies indicate that less commonly occurring variant fusion genes are also present in some tumors, eg, Ewing's sarcoma and alveolar rhabdomyosarcoma. The clinical consequences, if any, of these variant hybrids are not yet known. Reverse transcriptase polymerase chain reaction and are useful approaches in detecting these transcripts. In addition to translocations, supernumerary ring chromosomes are often encountered in sarcomas, particularly those of intermediate or borderline malignancy. Traditional fluorescence in situ hybridization, and recently, comparative genomic hybridization have uncovered the chromosomal composition of these rings as well as some associated gene amplifications in well-differentiated liposarcoma and dermatofibrosarcoma protuberans.

Animals↗

What to do with deep lipomatous tumors.

Although most deep soft-tissue sarcomas are fairly easily recognized as heterogenous masses that are bright on T2-weighted MRI and dark on T1-weighted MRI, liposarcomas are often difficult to distinguish from lipomas and other benign fatty tumors. Because clinical features overlap considerably between lipomas and liposarcomas, the radiographic features are of utmost importance in differentiating these entities. Key MRI features, such as homogeneity of fatty signal, absence of gadolinium enhancement, and low signal on short tau inversion recovery images, strongly support the diagnosis of benign lipoma, but the absence of these findings is not specific for liposarcoma. Unfortunately, biopsy frequently results in false negative findings; therefore, early referral to an orthopaedic oncologist is indicated when any radiographic features suggest that the mass could represent a liposarcoma.

Biopsy↗

Management of retroperitoneal sarcomas.

The records of 116 adult patients who underwent operative treatment for retroperitoneal sarcomas at the Mayo Clinic during the years 1963 to 1982 were reviewed. Clinical, pathologic, and treatment variables were analyzed for their influence on recurrence and death from disease. Leiomyosarcomas, liposarcomas, and malignant fibrous histiocytomas represented 93% of the tumors. The primary tumor was completely excised in 54% of patients. Recurrent tumor developed in 68% of patients (median time to recurrence, 1.3 years). Tumor fixation to adjacent structures (T3 tumor) or a high-grade tumor (G2-4) identified patients at increased risk for recurrent disease. Five-year and 10-year survival rates were 40% and 22%, respectively. Survival was significantly better for patients who had (1) complete surgical excision of their tumors, (2) low-grade tumors (G1), (3) tumors not fixed to adjacent retroperitoneal structures (T1 and T2 sarcomas), and (4) tumors without metastases when initially seen. Complete surgical excision offers patients with retroperitoneal sarcomas the best chance for long-term survival, but recurrent disease remains a vexing problem. The therapeutic challenges in the treatment of retroperitoneal sarcomas continue to be the development of therapy that will increase the rate of complete resection, decrease the rate of local recurrence, and enhance patient survival.

Adult↗

Retroperitoneal soft tissue sarcomas: prognosis and treatment of primary and recurrent disease in 117 patients.

BACKGROUND: The objective of this study was to define prognostic factors for patients with primary soft tissue sarcomas (STS) arising from the retroperitoneum. PATIENTS AND METHODS: One hundred and seventeen consecutive patients, resected in our institutions between July 1972 and November 2002, were reviewed. RESULTS: The prognostic factors predicting survival were incomplete resection, a tumor of high grade (G3), metastases to lymph nodes and distant metastasis. Patients with a malignant fibrous histiocytoma (MFH) or a malignant peripheral nerve sheath tumor (MPNST) had a worse prognosis than those patients with other tumors. The prognostic factors predicting local recurrence were incomplete resection and high grade (G3). The prognostic factors predicting metastasis were incomplete resection, lymph node metastasis at the time of the resection of the primary tumor and tumor histology. CONCLUSION: Since only complete tumor resection offers a chance for cure, it is mandatory, and local control remains the most significant challenge in the management of retroperitoneal sarcomas. Other therapies can support surgical treatment, depending on the tumor localization and histological entity. The management of patients with a STS should be provided by a specialized team of surgeons, oncologists and radiotherapists, and patients should be enrolled in a treatment study whenever possible.

Chemotherapy, Adjuvant↗

Metastatic patterns in soft-tissue sarcomas.

In 242 patients with recurrent soft-tissue sarcomas, the most common sites of initial recurrence were the primary site in 47.5% of patients and the lungs in 38% of patients. Further recurrences in the course of the disease concerned the lungs, bones, liver, and brain. Total survival and survival after recurrence were influenced by the histologic type, which also affected the site of recurrence. In the management of local recurrence, a five-year disease-free survival rate of 38% was achieved with surgical treatment, while radiation or chemotherapy alone was ineffective. Local recurrences resulted in significantly higher survival rates than those involving other organs. The disease-free interval was a significant prognostic indicator of subsequent survival in the whole group of patients and among those with local recurrence.

Adolescent↗