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PubMed2002

Radiation-associated synovial sarcoma: clinicopathologic and molecular analysis of two cases.

Development of a soft-tissue sarcoma is an infrequent but well-known long-term complication of radiotherapy. Malignant fibrous histiocytomas, extraskeletal osteosarcomas, fibrosarcomas, malignant peripheral nerve sheath tumors, and angiosarcomas are most frequently encountered. Radiation-associated synovial sarcomas are exceptional. We report the clinicopathologic, immunohistochemical, and molecular features of two radiation-associated synovial sarcomas. One tumor developed in a 42-year-old female 17 years after external irradiation was given for breast carcinoma; the other occurred in a 34-year-old female who was irradiated at the age of 7 years for a nonneoplastic condition of the left hand. Both lesions showed morphologic features of monophasic spindle cell synovial sarcoma, were immunoreactive for cytokeratins, epithelial membrane antigen, CD99, CD117 (c-kit), and bcl-2 and bore the t(X;18) (SYT-SSX1) translocation. We conclude that synovial sarcoma has to be added to the list of radiation-associated soft-tissue sarcomas.

Adult↗
PubMed1994

Sarcoma of the breast: implications for extent of therapy. The M. D. Anderson experience.

BACKGROUND: Sarcoma of the breast is a rare clinical entity for which there are no prospective data about extent of surgery indicated or role of multimodality therapy. The purpose of this study was to examine one of the largest single institutional experiences to shed light on these clinical issues. METHODS: This study retrospectively reviewed 60 cases of sarcoma of the breast (cystosarcoma phyllodes excluded). RESULTS: For the entire series there was a median overall survival time (OS) of 67 months and a disease-free survival period (DFS) of 18 months. Tumors smaller than 5 cm were associated with a better DFS (p < 0.04) and OS (p < 0.009). Patients with tumors less than 5 cm in diameter did equally well whether treated by wide local excision or mastectomy. Angiosarcoma histologic characteristics were associated with longer OS than stromal sarcoma (p = 0.017), malignant fibrous histiocytoma (p = 0.075), or fibrosarcoma (p = 0.08). Axillary dissections did not recover any nodal metastasis; moreover, treatment in 75% of the patients with negative nodes subsequently failed. Regional lymph node metastases were always and only in the context of disseminated disease. Adjuvant chemotherapy and/or radiotherapy was associated with prolonged DFS (p = 0.015). There was a trend toward improved local control with adjuvant radiotherapy (p = 0.14). CONCLUSIONS: Lesions less than 5 cm should be treated by breast-preserving wide local excision, and adjuvant radiotherapy should be considered for selected subgroups. For tumors 5 cm or larger, a more aggressive approach seems appropriate; consideration should be given to neoadjuvant chemoradiation followed by margin-negative surgery (if possible). There is no demonstrable staging or therapeutic role for routine axillary dissection.

Adolescent↗
PubMed1990

[A well differentiated fibrosarcoma arising in left neck region].

The frequency of a fibrosarcoma, based on current criteria, is less than 10% of soft tissue sarcomas. It is most common in the region of extremities and the trunk and relatively rare in the head and neck region. Therefore, of interest is a case of a well differentiated fibrosarcoma arising in left neck region of a 69-year-old woman that is reported. The excised tumor consisted of a firm, partially lobulated mass with a fibrous capsule that measured 8 cm at its greatest diameter. Histologically, the tumor tissue was rather uniform and often had a fasciculated growth pattern consisting of spindle shaped cells and interwoven collagenous fiber in a parallel fashion. To achieve a diagnosis, it was necessary to differentiate this type of fibrosarcoma from aggressive fibromatosis and myogenic tumor (leiomyoma or leiomyosarcoma).

Aged↗
PubMed1990

Tyrosine protein kinase activity of the HZ4-feline sarcoma virus P80gag-kit-transforming protein.

The Hardy-Zuckerman 4 feline sarcoma virus (HZ4-FeSV), isolated from a feline fibrosarcoma, is a replication defective acute transforming feline retrovirus that originated by transduction of feline c-kit sequences with feline leukemia virus (FeLV). The v-kit sequences of the HZ4-FeSV, a segment of 1106 nucleotides, correspond to sequences of the cytoplasmic domain of the c-kit receptor kinase. The HZ4-FeSV is known to encode an 80-kilodalton protein with FeLV gag and kit determinants. The P80gag-kit protein and its associated activities from HZ4-FeSV-transformed mink cells were characterized. The P80gag-kit protein was found to be myristoylated, suggesting a membrane association for this protein. In agreement with the predicted relationship with tyrosine kinases, by using the in vitro immune complex-kinase procedure, the P80gag-kit protein was shown to display a tyrosine-specific autophosphorylation activity. In vivo, the P80 protein was found to be phosphorylated on serine and threonine and to a lesser degree on tyrosine. In addition, potential in vivo protein substrates for tyrosine-specific phosphorylation mediated by the P80gag-kit kinase were identified in HZ4-FeSV-transformed cells.

Animals↗
PubMed1986

Reexamination of histological findings of ileal sarcomas induced in rats given diet containing bracken fern.

The ileal sarcomas induced in rats given diet containing bracken fern were examined microscopically and electron microscopically. These ileal sarcomas, which were induced at high incidence (80%-44%), have previously been identified as spindle cell fibrosarcomas. However, studies showed that they should be diagnosed as malignant fibrous histiocytomas (MFH) being classified into the following four histological types; fibrous, pleomorphic, giant cell and myxoid type. No types of sarcomas other than MFH were found in the ileum.

Animals↗
PubMedDate not supplied

[Surgical treatment of irradiation-induced sarcoma after treatment for breast carcinoma, personal experience: 4 case reports] ].

In the Department of Plastic Surgery of "Dubrava" University Hospital from 1993 to 1999 four patients were treated for radiation induced sarcoma. All of the patients were formerly operated for breast cancer and irradiated postoperatively. The mean time span between radiotherapy and development of sarcoma was 4.75 years. Four patients were treated with wide excision and immediate reconstruction with local flaps. One of the patients had fibrosarcoma, two patients had lymphangiosarcoma, and one patient had osteosarcoma. Two patients died within two years. Radiation induced sarcomas are therapy resistant, and the review of literature did not show large controlled investigations which would offer the most optimal treatment. Most frequently a rapid progression of the disease is seen.

Aged↗
PubMed1983

The Hardy-Zuckerman 2-FeSV, a new feline retrovirus with oncogene homology to Abelson-MuLV.

There is substantial evidence that oncogenes (v-onc) of acute transforming retroviruses have been acquired by transduction of cellular genes (c-onc) with retroviruses. Feline leukaemia virus (FeLV)-associated feline fibrosarcomas have proven to be extremely useful for the isolation of acute transforming retroviruses of a mammalian species. Three different v-onc genes have been identified in five acute transforming feline retroviruses. The Susan McDonough feline sarcoma virus (SMFeSV) contains the oncogene fms (ref. 4). The Snyder-Theilen (ST) and Gardner-Arnstein (GA) FeSVs contain the oncogene fes (ref. 4), which is homologous to the oncogene fps of the avian sarcoma viruses FSV, RRCII, PRCIV and 16L (refs 7, 8). The v-onc sequences of the Parodi-Irgens (PI) FeSV have recently been found to be homologous with the v-sis sequences of the simian sarcoma virus. We report here the isolation of another acute transforming feline retrovirus from a naturally occurring feline fibrosarcoma, designated the Hardy-Zuckerman 2 feline sarcoma virus (HZ2-FeSV) and demonstrate that the HZ2-FeSV and Abelson murine leukaemia virus (A-MuLV) have homologous oncogenes.

Abelson murine leukemia virus↗
PubMed1999

The impact of margin of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue sarcoma.

BACKGROUND/PURPOSE: Because the management of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) is determined by extrapolation from adult studies, the effect of margin of tumor resection and postoperative radiation therapy (RT) on local tumor recurrence in children has not been assessed. METHODS: Records of NRSTS patients from a single institution were reviewed with regard to demographic data, TNM staging, grade, histological type and site of primary tumor, RT, and local tumor recurrence. The margin of resection was determined by pathological review and did not necessarily reflect operative margins. RESULTS: Eighty-eight clinical group I patients were treated over a 30-year period. The most common histological tumor subtypes were synovial cell sarcoma (n = 26), malignant fibrous histiocytoma (n = 17), and fibrosarcoma (n = 7). The mean age was 9.4 years (range, 0 to 29 years). Thirty-four patients had high-grade tumors. Two of ten patients with low-grade tumors and margins less than 1 cm, including one of five who had received RT, had a local recurrence. Patients with low-grade tumors and margins greater than 1 cm (n = 44) had a lower recurrence rate (2 of 44, 4.5%). None of these patients had received RT. Fourteen patients with high-grade tumors had margins less than 1 cm. Seven of these had RT and had no recurrence. Three of the seven patients who received no RT had a recurrence (42.9%). None of the 20 patients with high-grade tumors and margins greater than 1 cm received RT; four of these patients had recurrences (20%). Seven of the 12 irradiated patients (58.3%) had serious radiation-associated complications (wound dehiscence, fracture, growth retardation, and joint dysfunction). CONCLUSIONS: Grade alone does not determine the rate of local recurrence. In both low- and high-grade tumors, a pathological margin of resection greater than 1 cm reduced local recurrence. Radiotherapy provided no advantage in low grade tumors but did decrease local recurrence rates in high-grade tumors with less than 1 cm pathological margins.

Adolescent↗