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PubMedDate not supplied

Computed tomography of ilio-psoas muscle tumors.

A retrospective evaluation of computed tomographic scans done at our institution showing psoas muscle tumors is presented. The anatomy of the psoas muscle along with a discussion of normal psoas anatomy which can be mistaken for tumor are reviewed. Primary muscle tumors along with metastatic, neurogenic, and lymphomatous tumors affecting the psoas muscle is discussed. The use of computed tomography for assessment of extent, origin, and structure of psoas muscle tumors is demonstrated.

Adult↗
PubMed1997

Molecular aberrations of the G1-S checkpoint in myxoid and round cell liposarcoma.

Myxoid and round cell liposarcoma represents a morphological spectrum in which tumor progression from low-grade myxoid to high-grade round cell areas is frequently observed. A distinctive t(12;16)(q13;p11) reciprocal translocation rearranges the CHOP gene localized to 12q13 in most cases. Data concerning the occurrence of cell cycle aberrations in this subset of mesenchymal malignancies are very limited. Therefore, we analyzed a histologically homogeneous series of 21 cases of myxoid and round cell liposarcoma. The p53 pathway was studied by investigating the TP53 gene and protein, mdm2 protein, and p21Waf1 protein. The Rb-cyclin D pathway was analyzed by studying the pRb protein, the p16MTS1 gene, cyclin D1, cyclin D3, p27Kip1, cdk4, and cdk6 proteins. In contrast with the rare involvement of the TP53 gene in well differentiated liposarcoma, aberrations of the TP53 gene were observed in approximately 30% of cases of myxoid and round cell liposarcoma. Notably, mdm2 overexpression was seen in 56% of cases and correlated with histological grade, therefore indicating a possible role in tumor progression. Abnormalities involving the Rb-cyclin D pathway were observed in more than 90% of cases. pRb loss was present in one-third of cases and, at variance with that observed in other subsets of sarcoma, overexpression of cyclin Ds represented a rare event. Interestingly, upregulation of either cdk4 or cdk6 was demonstrated in 85% of cases.

Chromosome Aberrations↗
PubMed1997

Detection of local recurrence of soft-tissue sarcoma with positron emission tomography using [18F]fluorodeoxyglucose.

BACKGROUND: It is often difficult to detect a local recurrence of soft-tissue sarcomas due to disturbance of the normal anatomy by previous surgery and radiotherapy. The aim of this study was to assess the value of positron emission tomography (PET) with [18F]fluoro-2-deoxy-D-glucose (FDG) for detecting local recurrences. METHODS: In the period 1992-1995, 17 patients with proven or suspected local recurrence of soft-tissue sarcoma were examined using FDG-PET. Fifteen of these patients were ultimately proven to have a recurrence. RESULTS: Recurrence was visualized in 14 patients (93%). Small tumors (maximum diameter 0.5 cm) were as easily visible as large lesions (maximum diameter 20 cm). In one patient the PET scan was positive, but the recurrence could not be proven histologically. Recurrence was proven 1 year later. A recurrent low-grade liposarcoma was not visualized. The two patients with benign lesions had a negative PET scan. The mean glucose metabolic rate was calculated to be 13.2 mumol/100 g/min (range 1.9-28.4). A correlation was found between the histological malignancy grade and the metabolic rate (p < 0.05; Kruskal-Wallis). CONCLUSION: PET with FDG is a useful addition to the diagnostic armamentarium for detecting local recurrence of soft-tissue sarcomas and provides an indication of the malignancy grade of the recurrent lesion.

Adult↗
PubMed1986

[Bone and soft-tissue tumors].

There have been great advances in recent years in the treatment of malignant bone and soft-tissue tumors thanks to advanced diagnostic techniques, improved chemotherapy and improved surgery involving extensive resection of the area involved, with bone and joint replacement. Here, we present details of the pathological findings, treatment and prognosis of cases of malignant bone and soft-tissue tumors, such as osteosarcoma, bone MFH, soft-tissue MFH, rhabdomyosarcoma and synovial sarcoma.

Adolescent↗
PubMedDate not supplied

Assessment of soft tissue lesions suspicious for liposarcoma by F18-deoxyglucose (FDG) positron emission tomography (PET).

BACKGROUND: F18-deoxyglucose (FDG) positron emission tomography (PET) is a promising imaging technique. The aim of this study was to investigate the use of FDG PET in patients with suspected liposarcomas (LS). PATIENTS AND METHODS: Forty-two masses were studied. The FDG uptake was estimated in tumor (T) and normal tissue (NT). The data were analyzed with respect to pathological findings. RESULTS: Pathology revealed 11 primary LS, 14 locally recurrent LS, 5 other sarcomas, 1 inflammation, 1 lymphoma and 10 benign lesions. FDG uptake (T-to-NT ratio) in 25 LS corresponded with the histological subtype. Pleomorphic, mixed and myxoid LS showed an increased T-to-NT ratio and were thus visualized. Four out of six well-differentiated LS presented a low FDG uptake. Like subtype, the tumor grade also corresponded to FDG uptake. The T-to-NT ratio of higher grade LS, contrary to low grade LS, was uniformly increased. Primary LS were distinguishable from benign tumors, while other sarcomas, inflammation and lymphoma were not. Recurrence was detected with a sensitivity of 86% and a specificity of 100%. False-negative diagnoses occurred only in well-differentiated recurrences. CONCLUSION: FDG uptake in LS correlates with the histological subtype and tumor grade. The diagnostic value of FDG PET in LS, therefore, is influenced by histomorphological parameters. Our data suggest that pleomorphic, mixed and higher-grade LS recurrences are preferentially amenable to FDG PET imaging.

Adolescent↗
PubMed1992

Soft tissue sarcomas in whites and blacks living in the United States of America.

Five hundred four hospitals volunteered reports on 2,355 patients in a long-term study and 645 institutes reported on 3,457 in a short-term study. Out of 5,623 cases of soft tissue sarcoma (STS) reported in white and blacks living in the United States of America, 574 cases (10.2%) were reported in blacks. No striking differences were found between blacks and whites concerning anatomic sites, histologic types, histologic grades, or clinical stages of STS. A higher percentage of patients with recurrences were reported in whites with liposarcoma (37.7% compared to 28.9% in blacks), and leiomyosarcoma (45% in whites compared to 39.1% in blacks). On the other hand recurrences were more frequent in fibrosarcoma and rhabdomyosarcoma in black patients. No significant differences in survival was found between white and black patients with STS.

Adult↗
PubMed2000

[99mTc-HMDP accumulation in soft tissue tumor].

Accumulation with bone scintigraphy using technetium-99m hydroxymethylene diphosphonate (99mTc-HMDP) in 68 cases with radiographically or pathologically verified soft tissue tumor was examined. Radiographical or histopathologic diagnoses of the 68 cases included; 14 lipomas, 11 liposarcomas, 11 neurinomas or neurofibromas, 6 malignant lymphomas, 5 malignant fibrous histiocytomas, 5 hemangioma, rhabdomyosarcomas, 2 Langerhans cell histiocytoses, 2 desmoid tumors and one each of neuroblastoma, hemangiopericytoma, angiomyxoma, plasmacytoma, liomyosarcoma, lymphangioma, fibrosarcoma, elastofibroma, synovial sarcoma, and ganglion. Thirty-seven (54%) showed positive accumulation and 31 were negative. One half of soft tissue tumors can be accumulated by 99mTc-HMDP.

Aged↗
PubMed1997

High dose tumor necrosis factor-alpha and melphalan administered via isolated limb perfusion for advanced limb soft tissue sarcoma results in a >90% response rate and limb preservation.

BACKGROUND: Recombinant tumor necrosis factor-alpha (rTNF-alpha) is a highly potential antineoplastic agent. However, its systemic administration in humans has resulted in a life-threatening septic shock-like syndrome, and its use has been abandoned. The administration of high dose rTNF-alpha and melphalan via isolated limb perfusion (ILP) eliminated the systemic side effects and was shown to be very effective for metastatic melanoma confined to the limb. The purpose of the current study was to assess the role of rTNF-alpha and melphalan administered via ILP in patients with soft tissue sarcoma. Amputation is unavoidable in 10% of these patients despite aggressive conventional therapy. Limb preservation was the objective in this select group of candidates for amputation or mutilating surgery. METHODS: During a 36-month period, 35 patients with high grade soft tissue sarcoma underwent 41 ILPs with high dose rTNF-alpha (3-4 mg) and melphalan (1-1.5 mg/kg). There were 21 males and 14 females. The mean age was 48 years (range, 14-80 years). Histologic subtypes included malignant fibrous histiocytoma, synovial, liposarcoma, malignant schwannoma, desmoid, clear cell, epithelioid, rhabdomyosarcoma, leiomyosarcoma, and unclassifiable. Twenty-one patients presented with recurrent and 14 with very extensive primary tumors. The tumors were located in the upper extremity in 8 patients and in the lower extremity in 27 patients. Twenty-five patients were candidates for amputation and 10 for extensive mutilating surgery. ILP was performed via the corresponding vessels proximal to the tumor. Six patients with partial response (PR) insufficient to render them resectable underwent a second ILP. With the exception of 4 of 9 patients with multifocal lesions and 1 who refused surgery, resection of the residual tumor or tumor bed or limb was performed 6-8 weeks after ILP. RESULTS: Marked tumor softening occurred within 48 hours, and in tumors protruding through the skin hemorrhagic necrosis was evident within 24 hours. The overall response rate was 91%. Thirteen patients (37%) had a complete response and 19 (54%) had a PR; in 5 of these patients > 90% necrosis of the tumor occurred. In 3 patients (8.5%), only minimal regression was observed (stabilization of disease). The rate of limb sparing was 85% (29 of 34 patients). CONCLUSIONS: The combination of high dose rTNF-alpha and melphalan given via ILP appears to be effective in patients with advanced soft tissue sarcoma confined to the limb, achieving a high response rate and limb preservation.

Adolescent↗
PubMed1997

Cutaneous melanoma in patients with sarcoma.

BACKGROUND: The authors became interested in an association between cutaneous melanoma and sarcoma when they reviewed their experience with other malignancies occurring in patients with a diagnosis of sarcoma. METHODS: The authors identified 48 patients with both melanoma and bone or soft tissue sarcoma (STS) by a computer search of all sarcoma patients entered into their institution's cancer registry between 1943 and 1996 who had an additional diagnosis of melanoma. The medical records were reviewed and clinical and pathologic data collected. RESULTS: The median age at diagnosis was 46 years for patients with melanoma and 50 years for patients with sarcoma, which was consistent with population-based data. Among patients with STS (n = 41), malignant peripheral nerve sheath tumors (MPNT) were more common in patients with both diagnoses (5 of 41; 13%) when compared with all adults with STS admitted to the study center between 1982 to date (125 of 2901; 4%; P < 0.05). Liposarcoma occurred in only 1 patient with both melanoma and STS (1 of 41; 2%), despite the fact that it was the most common histologic diagnosis in all adults with STS (625 of 2901; 22%; P < 0.001). The anatomic site of STS was more commonly visceral (11 of 41; 27%) when compared with all adults with STS (424 of 2901; 15%; P < 0.05). A positive family history of cancer was noted in 50% of the patients, and 25% of patients had a third primary tumor. CONCLUSIONS: Although a distinct "melanoma/sarcoma" syndrome was not identified, MPNT as well as visceral sarcomas were more common than expected in this study. The authors also noted strong family histories of cancer as well as additional primary malignancies in patients with melanoma and sarcoma, suggesting a predisposition toward cancer.

Adolescent↗
PubMed1984

Perineal liposarcoma: diagnosis and management.

Perineal liposarcoma is excessively rare. Its clinical and pathological behavior can only be predicted by comparison with the behavior of liposarcoma in other parts of the body. We describe the management of a man who presented with symptoms related to bladder outlet obstruction and was found to have a prostatic mass on digital examination of the rectum. A needle biopsy was performed and the tissue was histologically consistent with sarcoma of the prostate. Computerized tomography suggested that the mass extended through the urogenital diaphragm and was contiguous with the corpus spongiosum and corpora cavernosa of the penis. Complete surgical extirpation was achieved through a perineal approach, although local extension of the growth beneath the internal anal sphincter and the urogenital diaphragm necessitated reconstruction of these structures. The surgical margins were free of tumor. We advocate local surgical extirpation rather than pelvic exenteration in these cases.

Adult↗
PubMed2005

Significance of HDMX-S (or MDM4) mRNA splice variant overexpression and HDMX gene amplification on primary soft tissue sarcoma prognosis.

The product of the HDMX (or MDM4) gene is structurally related to the MDM2 oncoprotein and is also capable of interacting with the tumor suppressor protein p53. The aim of our study was to determine the amplification status of the HDMX gene and the expression of the HDMX mRNA (particularly that of the HDMX-S splice variant) in soft-tissue sarcomas (STS). Patients with STS were evaluated for the status of HDMX gene amplification (n = 66) and HDMX-S mRNA expression (n = 57) within their tumors. DNA, total RNA and protein were isolated from frozen tumor tissue. We determined that the HDMX-S splice variant transcript was predominant in a subset (14%) of tumor samples and that its expression was correlated with decreased patient survival (15 vs. 53 months, p < 0.0001, log-rank test) and with a 17-fold increased risk of a tumor-related death (p < 0.0001, multivariate Cox's regression model). The tumors from these patients also expressed elevated levels of HDMX-S protein. The HDMX gene was amplified in 17% of STSs, and the gene amplification was associated with poor prognosis (RR = 6.5, p < 0.0001). There was no correlation between the HDMX gene amplification and overexpression of the HDMX-S splice variant. In summary, our data indicate that both the overexpression of the HDMX-S transcript as well as HDMX gene amplification are important prognostic markers for STS.

Adult↗
PubMedDate not supplied

Management of liposarcoma occurring in pregnant women.

BACKGROUND: The coexistence of pregnancy and liposarcoma is rare. Only 12 cases of pregnancy-associated liposarcoma have previously been reported in the English-language literature. MATERIALS AND METHODS: We present two cases of liposarcoma occurring in pregnant patients: one myxoid liposarcoma in a 29-year-old woman at 29 weeks' gestation; another well-differentiated liposarcoma in a 44-year-old woman at 22 weeks' gestation. RESULTS: The first patient's infant was delivered by cesarean section after the confirmation of pulmonary maturity of the fetus. The patient was subsequently treated with chemotherapy and radiotherapy. The second patient awaited delivery until 32 weeks' gestation with no treatment of the tumor because she had a low-grade sarcoma. After cesarean section, surgical treatment followed. CONCLUSION: Treatment of sarcomas occurring during pregnancy is difficult. Both mother and fetus should be appropriately managed, depending on the trimester at the time of diagnosis and the histological type and grade of the tumor.

Adult↗
PubMed1994

MDM2 gene amplification correlates with ring chromosome in soft tissue tumors.

The human homolog of the murine double minute type 2 gene (MDM2) has been cloned and mapped to 12q13-14. The gene presumably functions as a cellular regulator and mediator of TP53 function. Amplification of the MDM2 gene has recently been observed in soft tissue sarcoma and in osteosarcoma. We studied MDM2 amplification in a series of 94 mesenchymal tumors and found 3-20-fold amplification in 20 tumors: in 10 of 49 malignant fibrous histiocytomas (MFH), in 1 of 2 pleomorphic liposarcomas, in 6 of 7 atypical lipomas, and in 3 of 12 typical lipomas. Normal hybridization patterns were detected in all 16 myxoid liposarcomas, in all 3 leiomyosarcomas, and in all 5 leiomyomas studied. The MDM2 amplification correlated with the presence of marker ring chromosomes; of the 10 MFH with MDM2 amplification, 5 had ring chromosomes, compared to 4 of 39 without MDM2 amplification, and all 9 liposomas with MDM2 amplification had ring chromosomes, in 5 of the tumors as the sole karyotypic anomaly. The correlation between ring chromosomes and MDM2 gene amplification indicates that the marker rings of MFH and of atypical lipoma often harbor genetic material derived from chromosome 12.

Adult↗
PubMed1996

A rare case of burn scar malignancy.

Malignant changes in burn scars have long been reported in the literature. Most cases are squamous cell carcinomas and some other epithelial cell carcinomas. Soft tissue tumours are considered rare. A rare case of sarcoma arising in a scar on the sole of the foot in a 64-year-old male is reported. He had suffered fire burns 25 years previously. Histological findings indicated that the tumour was a pleomorphic liposarcoma. Amputation was performed, but he died 3 months after the operation. To our knowledge, this is only the second case of liposarcoma developing in a burn scar.

Burns↗
PubMed1981

Primary retroperitoneal sarcomas: the role of surgical treatment.

Attempting to understand the role surgical excision plays in the therapeutic efforts to provide cure or palliation in patients with retroperitoneal sarcomas, the literature on the subject was reviewed and 13 such cases were analyzed. A palpable abdominal mass was recorded in 10 patients. Intravenous pyelography suggested the diagnosis in nine patients. The diagnosis was difficult to establish before celiotomy, which was performed in 12 patients. In six the tumor was unresectable; it was partially resected in five and completely removed in one. This study suggests that the primary mode of spread of these tumors is direct invasion. The only objective benefit of operation was to provide a tissue-proven diagnosis. Radiation therapy, after the diagnosis was established, appeared to be the most effective therapeutic measure for retroperitoneal sarcomas. The value of additional chemotherapy could not be established.

Adult↗
PubMed1993

Edatrexate in patients with soft tissue sarcoma. Activity in malignant fibrous histiocytoma.

BACKGROUND: Chemotherapy has had little impact on the natural history of soft tissue sarcoma, and often is associated with serious toxicity. Edatrexate, an investigational antifolate, is active in patients with lung cancer, and has cytotoxic activity in human sarcoma cell lines. METHODS: Edatrexate was administered to 36 patients with measurable, advanced soft tissue sarcoma who had not previously received chemotherapy. The drug was given weekly for 5 weeks, then every other week. The initial dose, 80 mg/m2, was escalated by 10 mg/m2 every 2 weeks in the absence of toxicity. Eleven patients had leiomyosarcoma, 7 had malignant fibrous histiocytoma (MFH), and 5 had liposarcoma; the remainder of cell types included hemangiopericytoma (4), angiosarcoma (3), synovial (2), spindle cell (2), extraosseous chondrosarcoma (1), and fibrosarcoma (1). RESULTS: Thirty-five patients are evaluable. Partial response (PR) was seen in five of the seven patients with MFH; no other major responses occurred. Overall, the response frequency was 14% (two-sided 95% confidence interval, 3% to 26%). Median duration of PR was 6 months (range, 4-18 months). One patient had a minor tumor regression, and six had stable disease. Myelosuppression was generally mild; only three patients had grade 3 hematologic toxicity. Modification of dose or schedule was required in 50% of patients for mucositis. Fatigue was a common toxicity, seen in 66% of patients, but was tolerable in the majority. A rash was seen in 46% of patients; one patient had hepatic toxicity. CONCLUSIONS: Overall, the activity of edatrexate in this study, dominated by patients with either visceral or vascular sarcoma, was poor. However, the responses observed in patients with metastatic MFH suggests that further evaluation of edatrexate in patients with soft tissue sarcoma is warranted.

Adult↗