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PubMed1998

Grading of subcutaneous soft tissue tumors by means of their relationship with the superficial fascia on MR imaging.

OBJECTIVE: To assess the diagnostic value of the relationship between subcutaneous tumors and the superficial fascia in the characterization of soft-tissue masses. DESIGN: MR studies of 64 soft tissue masses located in the subcutaneous space were reviewed. We established five grades of relationship between tumors and superficial fascia and analyzed the probability of the lesions in each group being malignant. Group 1 tumors did not contact the fascia; group 2 lesions contacted it slightly, with acute angles between the tumor and the fascia; group 3 lesions had wider contact with larger acute or right angles; group 4 tumors had even wider contact with obtuse angles with the fascia; and group 5 was composed of lesions that crossed the superficial fascia. RESULTS: The probability of a subcutaneous lesion that crosses the superficial fascia being malignant was 6.88 times greater than for lesions that did not cross the fascia. For lesions forming obtuse angles with the fascia the probability of malignancy was 6.3 times greater than that of tumors that did not present this sign. All histologically verified fascial infiltrations occurred in malignant lesions of groups 4 and 5. CONCLUSIONS: Malignant tumors of the subcutaneous compartment have a higher tendency to develop a close relationship with the fascia than benign lesions. Obtuse angles between superficial fascia and a subcutaneous mass or a lesion crossing the fascia strongly suggest malignancy.

Carcinoma, Squamous Cell↗
PubMed2003

Alpha-inhibin immunoreactivity in soft-tissue neoplasia.

Antibodies directed against alpha-inhibin have been previously reported as staining both sex cord-stromal neoplasms as well as adrenal cortical tumors. This relatively restricted immunoreactivity pattern is useful in the assessment of retroperitoneal masses, especially in a setting of limited tissue (e.g., needle biopsy). However, no study to date has evaluated alpha-inhibin immunoreactivity in soft-tissue neoplasms, which frequently enter the differential diagnosis of retroperitoneal masses. We investigate the incidence of alpha-inhibin staining in a variety of soft-tissue neoplasms by using formalin-fixed, paraffin-embedded tissue sections from 282 previously classified soft-tissue neoplasms with anti-alpha-inhibin (Serotec, 1:75). A modified avidin-biotin complex method was used after heat-induced epitope retrieval. Cytoplasmic granular staining was considered positive. Of the 282 tumors studied, a total of 8 (2.8%) demonstrated positive staining with anti-alpha-inhibin antibody. These included 4 of 25 liposarcomas (16%), 2 of 18 angiosarcomas (11%), 1 of 48 lipomas (2.1%), and 1 of 1 rhabdomyoma (100%). Negative staining was noted among hemangiomas (0/28), schwannomas (0/32), leiomyomas (0/16), fibrosarcomas (0/2), fibromas (0/11), dermatofibromas (0/9), neurofibromas (0/6), synovial sarcomas (0/15), rhabdomyosarcomas (0/10), Triton tumors (0/2), and malignant fibrous histiocytomas (0/59). We conclude that rare soft-tissue tumors, especially those exhibiting either lipomatous or vascular differentiation, demonstrate alpha-inhibin immunoreactivity. These findings re-emphasize the need for a well-construed antibody panel when immunohistochemical methods are employed in the evaluation of retroperitoneal neoplasms. However, the rarity of alpha-inhibin expression by soft-tissue neoplasms provides further support for its overall specificity as a marker of adrenal cortical differentiation in the biopsy evaluation of a retroperitoneal mass.

Humans↗
PubMed1983

Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors.

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.

Humans↗
PubMed2001

Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group.

BACKGROUND: Surgery is the main prognostic factor in retroperitoneal sarcoma. However, despite progress, surgery alone is rarely curative, and analysis of the causes of failures and of other prognostic factors are warranted to ascertain treatment orientations. METHODS: Data of patients treated from 1.80 to 12.94 for primary retroperitoneal sarcoma were extracted from the French Federation of Cancer Centers Sarcoma Group registry. Univariate and multivariate analysis were performed for initial local control and for local and general outcome. One hundred sixty-five patients (median age, 54 years; range, 16--82 years) were identified. Median tumor size was 15 cm (range, 2--70 cm); 31% of tumors presented with neurovascular or bone involvement. Liposarcoma, leiomyosarcoma, and malignant fibrous histiocytoma represented 66% of the tumors. Eighty-four percent of the tumors were of high or intermediate grade. Twenty patients had initial metastases. Multimodality treatment included surgery (150 patients), radiotherapy (92 patients), and chemotherapy (77 patients). Complete excision was achieved in 94 of 145 nonmetastatic patients. Median follow-up was 47 months (range, 3--160 months). RESULTS: Actuarial overall 5-year survival rate (median) was 46% (51 months). The main prognostic factors for survival were initial metastases and surgery, which represented the major treatment-linked factor. High-grade of tumors affected local recurrence, metastatic recurrence, and survival. Adjuvant radiotherapy was significantly associated with reduced local recurrence. Various evolutive patterns were observed with histologic subtypes. CONCLUSIONS: Aggressive surgery remains mandatory in retroperitoneal sarcoma, but a randomized trial is needed to evaluate the place of radiotherapy for local control.

Adolescent↗
PubMed1986

Primary peritoneal, omental, and mesenteric tumors in childhood.

Of 21 primary peritoneal, omental, and mesenteric tumors, 14 were cysts and seven were solid. Most of the cysts were omental-mesenteric lymphangiomas; the sole exception was a cystic mesothelioma in a newborn. The solid tumors included three multicentric hamartomas, three sarcomas of undetermined histogenesis, and one immature teratoma. Since cystic lymphangiomas carry a good prognosis following surgical excision, they must be distinguished from cysts of mesothelial origin. Solid hamartomas may simulate malignant tumors because of multicentricity and immature tissue composition. Although peritoneal sarcomas of undetermined histogenesis are apt to be large, composed of primitive cells, and disposed to the development of implants, current treatment modalities may control the disease. One of three treated patients survived for 8 years. A child with a solid immature teratoma without a malignant germ-cell component remains free of disease 5 years after diagnosis. Our review, which represents the combined experience of two children's hospitals over several decades, did not include tumors such as malignant mesothelioma, lipoma, liposarcoma, leiomyoma, mesenchymoma, fibromatosis, and other neoplasms, which may rarely arise in the peritoneum, omentum, and mesentery of children.

Age Factors↗
PubMed1994

Outcome and prognosis in retroperitoneal soft tissue sarcoma.

PURPOSE: To retrospectively evaluate the outcome of treatment and identify factors prognostic for survival and locoregional and distant disease control for patients with retroperitoneal soft tissue sarcoma. METHODS AND MATERIALS: The records of 104 patients with retroperitoneal soft tissue sarcoma (RSTS) managed with surgery and irradiation at Princess Margaret Hospital between 1975 and 1988 were retrospectively reviewed. Univariate log-rank analysis was used to evaluate potential prognostic factors. RESULTS: Presentation was new primary disease, 74; primary recurrence, 20; metastases, 10. Pathology was liposarcoma for 42, leiomyosarcoma for 22, malignant fibrous histiocytoma for 19, and 21 with other histologies. Grade was low for 36, high for 35, and 33 were not graded. Median tumor size was 17 cm. Grossly complete surgical excision was achieved for 45 (43%), of whom 6 (6%) also had clear surgical margins. Adjuvant postoperative irradiation was administered to 36 patients to a median dose of 40 Gy/20 fractions/4 weeks and 16 received adjuvant chemotherapy. Nine patients received no adjuvant postoperative radiotherapy. Gross residual tumor was present postoperatively in 57 patients. The overall 5- and 10-year survival rates were 36% and 14%, respectively. The locoregional relapse free rate (RFR) was 28% at 5 years and 9% at 10 years, and the distant RFR was 76% at 5 years and 60% at 10 years. For the 45 patients treated with complete excision, survival was 55% and 22% at 5 and 10 years, and locoregional RFR was 50% and 18% at 5 and 10 years. Univariate analysis demonstrated that complete surgical removal was the only factor significant for improved survival, locoregional RFR, and distant RFR. Liposarcoma histology predicted for improved survival (p = 0.02), and leiomyosarcoma histology for a lower distant RFR, compared to other histologies (p = 0.003). Patients under 62 years had an improved survival (p = 0.002) and local RFR (p = 0.02), and patients presenting with recurrent disease had improved survival (p = 0.03). Sex, tumor size, or grade, or the use of adjuvant chemotherapy were not predictive for any of the endpoints tested. Those who received adjuvant irradiation following gross surgical clearance experienced a prolonged median locoregional RFR over those who did not, and this approached statistical significance for those receiving radiation doses > 35 Gy. (103 months vs. 30 months, p = 0.06). Statistical significance was reached (p = 0.02) if only the infield RFR was considered. CONCLUSIONS: This study demonstrates that failure to achieve local control is the primary cause of treatment failure for patients with RSTS, and that postoperative irradiation in doses > 35 Gy after complete surgery delayed, but did not prevent local recurrence. Improvements in outcome for patients with RSTS will require alternate treatment strategies, and preoperative irradiation with an aggressive surgical attempt at complete excision is currently under investigation.

Combined Modality Therapy↗
PubMed1998

Metastizing low-grade clear cell leiomyosarcoma of the uterus.

An unusual case of uterine clear cell tumor is reported in a 56-year-old woman. The neoplasm was identified as a peculiar low-grade leiomyosarcoma, composed mainly of watery, clear large cells with round, fairly regular nuclei. No appreciable pleomorphism or high mitotic activity was noted. There was no necrosis. The presence of occasional areas of transition between these cells and typical spindle leiomyosarcoma cells, together with the immunohistochemical results, allowed the recognition of a smooth muscle origin of this clear cell tumor. The patient was treated by hysterectomy. Six years later she developed an inguinal metastasis resembling an hibernoma, and then later developed a large retroperitoneal metastasis, which was composed entirely of clear cells and was distinguished from a liposarcoma by immunohistochemistry.

Actins↗
PubMed1991

The role of multimodality therapy in soft-tissue sarcoma.

Soft-tissue sarcomas are uncommon malignancies. The development during a period of 8 years, in one institution, of a prospective data base incorporating more than 1600 patients with these tumors is described. The most common sites for occurrence are the extremities, but they can occur in any of the soft tissues of the body. Liposarcoma and leiomyosarcoma are the most common histopathologic conditions identified. Prognostic factors for both recurrence and survival include site, histopathology, size, grade, and adequacy of resection. A prospective randomized trial of the use of adjuvant radiation by the brachytherapy technique in extremity lesions has shown a decrease in local recurrence, but no impact on survival. Eligible patients not randomized to the trial show no difference in local recurrence or survival, regardless of whether they received adjuvant radiation.

Adolescent↗
PubMed2004

Operative management of primary retroperitoneal sarcomas: a reappraisal of an institutional experience.

OBJECTIVE: To review our recent experience with primary retroperitoneal sarcomas, determine prognostic factors for disease recurrence and patient survival, and compare them to our previous results. BACKGROUND: Medical therapies have shown little efficacy in the management of retroperitoneal sarcomas, making total surgical extirpation the best chance for patient cure. METHODS: The case histories of all patients operated upon for retroperitoneal sarcomas between January 1983 and December 1995 were retrospectively reviewed. RESULTS: Ninety-seven patients underwent attempted surgical resection of a primary retroperitoneal sarcoma. There were 54 (56%) men and 43 (44%) women, with a mean age of 59 years. Seventy-six (78%) patients underwent gross total resection, 13 (14%) had residual disease, and 8 (8%) underwent biopsy only with an actuarial 1-year survival of 88%, 51%, and 47%, respectively (P = 0.001). The actuarial 5- and 10-year survivals for patients who underwent gross total resection were 51% and 36%, respectively. Thirty-three patients (43%) developed locoregional recurrence, and 20 patients (26%) developed distant metastases at a median time of 12 months. The cumulative probability at 5 years was 44% for locoregional recurrence and 29% for distant metastases. On univariate analysis, factors associated with improved survival were complete resection of the tumor (P = 0.001), nonmetastatic disease at presentation (P = 0.01), low-grade tumors (P = 0.02), liposarcomas (P = 0.003), and no disease recurrence (P = 0.0001). Contrary to previous reports, the histologic subtype (P = 0.04) was the only significant factor predicting survival on multivariate analysis. CONCLUSIONS: Compared with our earlier experience, the rates of complete resection and overall survival have improved. Local control continues to be a significant problem in the management of retroperitoneal sarcomas. Because new surgical options for this problem are limited, further outcome improvement requires novel adjuvant therapies.

Adolescent↗
PubMed1997

Loss of heterozygosity on chromosome 9q21 (p16 gene) uncommon in soft-tissue sarcomas.

Chromosome region 9p21 contains a tumor suppressor locus (p16) that may be involved in the genesis of several kinds of malignant tumors. To characterize the role of this gene in the development of soft-tissue tumors (STTs), we investigated the frequency of loss of heterozygosity (LOH) at this locus. DNA was obtained from 77 tumors and the peripheral blood of 23 of the patients with the tumors. Using one microsatellite marker distal to p16(D9S171) and one intragenic sequence-tagged site (STS) marker (c5.1), we observed LOH in only one liposarcoma and one malignant schwannoma (2.6%). Homozygous deletions of the p16 markers were not found. The osteosarcoma cell line MG-63 was used as a control for loss of the p16 gene. Because of the low LOH frequency, we hypothesize that the p16 gene is not essential for STT oncogenesis.

Chromosomes, Human, Pair 9↗
PubMedDate not supplied

[Treatment of soft tissue sarcomas of the extremities and the trunk by conservative surgery and postoperative irradiation. Apropos of a series of 96 patients].

AIM OF STUDY: Retrospective study of a series of 96 patients presenting with soft tissues sarcoma. Homogeneous treatment between 1980-1992 with conservative surgery and post operative irradiation. PATIENTS AND METHODS: Median age of the 96 patients was 58 years. Tumor site was: upper limb 20, lower limb 46, trunk 30. In 35 cases largest diameter of the tumor was 5 cm or less (T1). All patients were M0. The most frequent pathological sub type was: malignant histiocytofibroma 28, liposarcoma 28. A gross complete surgery was performed in 89 cases. Radiotherapy was performed with cobalt or x 18 MV photons. The dose delivered was 50 Gy with a boost of 10 Gy. No adjuvant chemotherapy was given. RESULTS: Mean follow up was 68 months. Local relapse was seen in 19 patients, six were salvaged by surgery, a limb amputation rates were necessary in 4 cases. The 5 and 10 year-overall survival was 70% and 64%. There was no severe radiation toxicity requiring surgery. A good function of the limb was preserved in all cases. CONCLUSION: These results are in agreement with those of the literature and justify a conservative approach for these soft tissues sarcomas.

Adolescent↗
PubMed2002

Dedifferentiated chordoma--a case report.

A case of a recurrent sacro-coccygeal chordoma is reported which histologically showed an admixture of a conventional chordoma and a pleomorphic sarcoma. These tumours are called dedifferentiated chordomas and carry an ominous prognosis.

Bone Neoplasms↗
PubMed2004

[The analysis of different treatment methods for 184 cases of soft tissue sarcoma].

OBJECTIVE: To study the proper way of using combined postoperative chemo-radiotherapy and prognostic factors of soft tissue sarcoma. METHODS: The clinical data of 184 patients were retrospectively reviewed. These patients were devided into surgery group (S, 94 patients), surgery plus postoperative radiotherapy group (S + R, 62 patients) and surgery plus chemotherapy group (S + C, 28 patients). RESULTS: The 5-year survival rates of S, S + R and S + C groups were 39.4%, 48.4% and 28.6%, respectively. Combined multitherapy was the key to improve survival rate and life quality. Clinical stage, pathological type and therapeutic method were also important prognostic factors for the long term survival. CONCLUSION: Surgery plus postoperative radiotherapy can improve the 5-year survival rate of soft tissue sarcoma.

Adolescent↗