Histopathology of sarcomas.
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A group of 21 adults patients with soft tissues sarcomas of the extremities were studied by CT. CT was a diagnostic procedure in only one case. However it has always permitted a precise local work-up particularly in demonstrating the vasculo-nervous connections of the lesions. The place of CT with regard to the other radiological procedures is discussed. by its contribution to the locoregional and general pretherapeutic work-up and to a better determination of radiotherapeutical dosimetry, CT appears to have an up standing place in the staging and the follow-up of soft tissues sarcomas.
The growth rate of pulmonary metastases was analyzed in eleven patients with soft tissue sarcoma. In cases where more than two examinations were available the growth rate seemed to be exponential. In all but one case microscopic pulmonary spread was calculated to be present when the primary tumor was diagnosed. Tumor doubling time varied between 8 and 198 days in different patients. The variation in growth rate between various nodules in the same patient was much less pronounced but nevertheless considerable, which might, at least partly, be explained by tumor cell polyclonality. Computed tomography of the chest may detect pulmonary metastases earlier than conventional radiography and is therefore recommended in the preoperative work-up in soft tissue sarcoma.
Pleomorphic liposarcoma (PLS) is defined as a high-grade pleomorphic sarcoma containing multivacuolated lipoblasts. PLS arises most frequently in the extremities of older individuals and often metastasizes to the lungs. The prognosis of PLS parallels that of many other high-grade sarcomas. In a small analysis combining clinicopathologic data available from 16 previously reported and 8 additional cases of PLS arising in the extremities, the 5-year overall survival rate for the 24 patients was 40% (median survival, 48 months). By univariate analysis, patients with tumors located in the upper extremities (P = .021) and greater than 10 cm (P = .047) had decreased overall survival. Older age, female sex, epithelioid phenotype, and radiotherapy or chemotherapy administration were not significantly associated with an adverse outcome.
Soft tissue sarcomas (STS) are a heterogeneous group of uncommon tumors that show a broad range of differentiation that may reflect etiologic distinction. Routine tabulations of STS are not morphology-specific. Further, the lack of inclusion of sarcomas arising in all organs in most standard evaluations underestimates the true rates. We analyzed the 1978-2001 Surveillance, Epidemiology and End Results program incidence rates of STS regardless of primary site, except bones and joints, using the 2002 criteria of the WHO classification. There were 26,758 cases available for analysis. Leiomyosarcomas accounted for 23.9%, malignant fibrous histiocytomas 17.1%, liposarcomas 11.5%, dermatofibrosarcomas 10.5%, rhabdomyosarcomas 4.6% and angiosarcomas 4.1%. Almost half (47.9%) of the sarcomas arose in the soft tissues, 14.0% in the skin and 7.0% in the uterus. Overall, incidence rates were highest among black women (6.26/100,000 woman-years) and the lowest among white women (4.60/100,000). Age-adjusted rates increased at 1.2% and 0.8% per year among white males and females, respectively, both trends statistically significant, while rates among blacks declined slightly. About 40% of leiomyosarcomas among women were uterine in origin, with a black/white rate ratio of 1.7. This rate ratio increased to 2.0 when we accounted for the lower prevalence of intact uteri among black compared to white women. Total STS rates rose exponentially with age. Rates for both uterine leiomyosarcoma and dermatofibrosarcoma increased rapidly during the childbearing years, peaking at about age 40 and 50, respectively. Incidence patterns of STS varied markedly by histologic type, supporting the notion that these tumors may be etiologically distinct.
Brachytherapy delivered within the early post-operative period has been associated with delayed healing and wound breakdown. The objective of this study was to determine whether reconstruction with a microvascular free flap reduced the incidence of wound breakdown after early post-operative brachytherapy and wide excision of soft tissue sarcomas. Four patients with sarcomas underwent excision and free flap reconstruction. Brachytherapy was administered in the early post-operative period using Iridium-192 wires via tubes inserted intraoperatively. In three of the four patients the wounds healed uneventfully, demonstrating that brachytherapy can be delivered in the early post-operative period following free flap reconstruction without an increase in the frequency of wound breakdown. There has been no local recurrence to date in this group of patients.
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Cancer-testis or germ cell antigens (GCAs) are a category of tumor antigens expressed by male germ cells and by cancers of diverse histological origin, but not usually by normal adult somatic tissue. These antigens include products encoded by the MAGE, BAGE, GAGE, SSX, and LAGE/NY-ESO-1 families that encode antigenic peptides recognized by T lymphocytes. In this study, we exploit oligonucleotide technology to identify genes in melanoma and soft tissue sarcoma (STS) that display a cancer-testis/GCA expression profile. We identified 59 such genes, including GCAs we knew to be recognized by T lymphocytes. Among our findings are the expression of PRAME in monophasic synovial sarcoma, PRAME and NY-ESO-1 in myxoid/round cell liposarcoma, and SSX2 and members of the GAGE family in malignant fibrous histiocytoma. Furthermore, the proto-oncogene DBL/MCF2 was identified as encoding a novel candidate GCA expressed by clear cell sarcoma/melanoma of soft parts (MSP). DBL/MCF2 peptides that are bound to HLA-A*0201 were identified and recognized by T lymphocytes. These results show the utility of high-throughput expression analysis in the efficient screening and identification of GCA candidates in cancer, and its application to the discovery of candidate targets for T cell immunity against GCAs expressed by cancer.
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BACKGROUND: Vulvar liposarcoma is rare. Three cases have been reported since 1966. Primary sarcomas of the vulva constitute 1-2% of all vulvar malignancies. Because of their location and appearance, they may be easily misdiagnosed as a more benign process. CASE: A 65-year-old, black woman had a vulvar lesion that appeared clinically to be a Bartholin's gland abscess. The lesion was a soft tissue mass, was excised in toto and was sent for pathologic study with a presumed diagnosis of lipoma. The pathology report indicated pleomorphic liposarcoma. CONCLUSION: Only three prior cases of this disease have been reported. The clinician should heighten his or her awareness in a patient with a vulvar mass. Early diagnosis is imperative for a favorable outcome.
OBJECTIVE: The authors sought to identify prognostic factors in the management of extremity soft tissue sarcoma. SUMMARY BACKGROUND DATA: The surgical management of soft tissue sarcoma has evolved because of advances in therapy, resulting in increased limb preservation and quality of life. However, identifying a subset of patients most likely to benefit from adjuvant chemotherapy has been difficult to achieve. METHODS: A retrospective analysis of a prospective data base of 182 patients with extremity sarcomas from 1970 to 1992 was performed. RESULTS: A histologic diagnosis of Ewing's sarcoma, synovial sarcoma, and angiosarcoma was associated with a 13-fold increased risk of death compared with liposarcoma, fibrosarcoma, and malignant peripheral nerve sheath histologic types after having adjusted for the other prognostic factors (p < 0.001). In addition to histologic type, high-grade sarcomas (p = 0.018), sarcomas greater than 10 cm in size (p = 0.006), and age at diagnosis (p = 0.016) were found to be important prognostic factors for survival but not for local recurrence. For the first time to their knowledge, the authors showed that mean mitotic activity has prognostic value after having adjusted for other prognostic factors, such as grade (p = 0.005). The only prognostic factors predictive for local recurrence were whether the patient presented with locally recurrent disease (p = 0.0001) or had microscopically positive margins (p = 0.052). CONCLUSIONS: The use of mitotic activity along with grade, size, histologic type, and age at diagnosis is prognostic for survival in extremity soft tissue sarcoma. The use of an objective pathologic feature, such as mean mitotic activity, is also useful in selecting patients for future systemic neoadjuvant or adjuvant trials and primary therapy.
The data presented indicate that the combination of function-preserving surgery and radiation therapy is of value in the treatment of soft tissue sarcomas of the extremity. Local control is obtained in approximately 85% of patients and with survival results comparable to those obtained in patients treated with radical surgery. The one randomized series of patients treated with conservative resection and radiation compared to amputation has shown no difference in overall survival. These local control results have been obtained while maintaining good functional results. Combined local resection and radiation is an appropriate treatment option in a large proportion of patients with soft tissue sarcomas.
Liposarcoma is known to be the most common soft-tissue sarcoma, and the treatment of retroperitoneal disease is surgical excision. We present a case of complete surgical removal with concomitant nephrectomy using a hand-assisted laparoscopic approach and provide a review of the published studies.
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BACKGROUND: Liposarcoma of the head and neck region represents approximately 1% of head and neck sarcomas. Therefore, there are few data on the natural history, presentation, treatment, and prognosis of this neoplasm. METHODS: This study is a report of data from 76 patients with head and neck liposarcoma of whom 4 were treated at The Royal Marsden Hospital during the past 50 years. RESULTS: The median age of patient presentation was the seventh decade (range, 6 months-86 years), and 65% of the patients were male. The commonest site of presentation was the neck (28%), followed by the larynx (20%) and pharynx (18%). Sixty-two percent of tumors were low grade (well differentiated and myxoid), and 38% were high grade (pleomorphic and round cell). The principal determinant of outcome was histologic grade. Five-year survival by life-table analysis was 67% overall and varied with tumor type as follows: well differentiated 100%, myxoid 73%, pleomorphic 42%, and round cell 0%. Site appears to have had some influence on prognosis. Oral liposarcoma had a poor prognosis with a 5-year survival of 50%, despite the low grade of all tumors; however, the 5-year survival for laryngeal (89%) and head (83%) liposarcoma was considerably better. Tumor size did not affect prognosis. The mainstay of treatment was surgical excision, used alone in 70% of the cases. Radiotherapy was used with other treatments in 25% of the cases. Prognosis was best for patients treated with surgery only (5-year survival, 83%), compared with those receiving surgery plus radiotherapy (5-year survival, 63%), chemotherapy (5-year survival, 33%), and radiotherapy alone (5-year survival, 0%). CONCLUSIONS: Liposarcoma rarely involves the head and neck region. The prognosis for patients with this disease appears to be better than for those with liposarcoma arising elsewhere, particularly in the retroperitoneum. Prognosis is principally dependent on histologic grade. Complete surgical excision provides the most effective treatment.
There are no specific MRI features which are diagnostic in bone and soft tissue sarcomas, but a combination of certain findings allows an accurate diagnosis in some cases including giant cell tumour, chondrosarcoma, liposarcoma, neurofibrosarcoma, aggressive fibromatosis and pigmented villonodular synovitis. MRI is the method of choice for staging bone and soft tissue sarcomas. It is of particular use for identifying satellite nodules and skip lesions within the same bone or anatomical compartment. CT scan is important for screening for pulmonary metastases, and bone scan remains useful for screening for distant skeletal disease. The biopsy should be planned and performed after any MRI examination. MRI is the most sensitive post-therapy evaluation for local recurrence of bone and soft tissue sarcoma.
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