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NCI CANCERLIT® Search: Soft Tissue Sarcoma/Rhabdomyosarcoma - September 2001
Last Modified: November 1, 2001

Table of Contents

CancerMail from the National Cancer Institute

1
UI - 21237090
AU - Hagiwara A; Inoue Y; Nakayama T; Yamato K; Nemoto Y; Shakudo M; Daikokuya H; Nakayama K; Yamada R
TI - The "botryoid sign": a characteristic feature of rhabdomyosarcomas in the head and neck.
SO - Neuroradiology 2001 Apr;43(4):331-5
AD - Department of Radiology, Kobe City General Hospital, 4-6 Minatojimanakamachi, Chuo-ku, Kobe 650-0046, Japan. akirakun@z2.zzz.or.jp
We investigated nine patients with rhabdomyosarcoma in the head and neck (6-53 years of age), using CT and MRI. The tumours originated in the paranasal sinuses (3), cheek (2), soft palate (1), orbit (1), sternocostoclavicular muscle (1) and parapharyngeal space (1). The histological subtype was embryonal in five, alveolar in three and pleomorphic in one case. The tumours enhanced markedly and heterogeneous on CT and MRI. The masses were isointense or gave slightly higher signal than surrounding muscles on T1- and heterogeneously high signal on T2-weighted images. In four tumours, multiple ring enhancement resembling bunches of grapes. This appears to be characteristic of rhabdomyosarcoma and probably reflects a component of botryoid-type rhabdomyosarcoma in which mucoid-rich stroma is covered with a thin layer of tumour cells. We have named this imaging feature the "botryoid sign".

2
UI - 21261126
AU - Zocchetti C
TI - [Liver angiosarcoma in humans: epidemiologic considerations]
SO - Med Lav 2001 Jan-Feb;92(1):39-53
AD - Regione Lombardia, Osservatorio Epidemiologico e Flussi Informativi, Via Pola, 9-20124 Milano. Carlo_Zocchetti@regione.lombardia.it
A review of the available literature on angiosarcoma of the liver in an epidemiologic perspective shows that this cancer is rare (0.5-2.5 cases every 10,000,000 persons). It escapes current mortality statistics; it presents peculiar diagnostic difficulties even when histologic material is scrutinized by panels of pathologists; it occurs in both gender at every age (also in children); estimated latency is long (10-40 years, in occupational cases) or very long (60 years and above, in non-occupational cases). Thorotrast, arsenic, and vinyl chloride monomer are frequently listed as risk factors but, along with other substances (steroids, hemochromatosis, diethylstilbestrol, phenelzine, urethane, cyclophosphamide, oral contraceptives) they contribute to explaining the occurrence of no more than 20% of the published cases. Focussing on subjects exposed to vinyl chloride monomer, the published cases were all males aged 35-75 years, with an average latency of 22 years (range 10-40), in jobs with a potential for a high, or very high exposure to VCM (mainly as autoclave cleaner) started before 1970, and limited to a small number of factories. No evidence of a relationship between environmental (non occupational) exposure to VCM and angiosarcoma of the liver emerges from the available literature. We conclude that in more than 25 years of research, apart from the case of vinyl chloride, the etiology of this type of cancer has remained unchanged and largely unknown.

3
UI - 21292755
AU - Gravis G; Mousseau M; Douillard JY; Dorval T; Fabbro M; Escudier B; Mignot L; Viens P; Groupe Francais d'Immunotherapie
TI - Can interleukin-2 reverse anthracyclin chemoresistance in metastatic soft tissue sarcoma patients. Results of a prospective phase II clinical trial.
SO - Eur Cytokine Netw 2001 Apr-Jun;12(2):239-43
AD - Oncology Unit, Institut Paoli-Calmettes, 232, boulevard Sainte-Marguerite, 13273 Marseille Cedex 09, France.
Anthracyclin-based chemotherapy is the most efficient chemotherapy for advanced or metastatic soft tissue sarcoma (STS). Development of anthracyclin chemoresistance has been widely documented. In a previous clinical trial, we evaluated a possible reversal of anthracyclin chemoresistance after exposure to subcutaneous IL-2. The current phase II clinical study entered 17 proven metastatic STS patients, refractory to anthracyclin chemotherapy, who received IL-2, and subsequent anthracyclin-based chemotherapy. Subcutaneous IL-2 was administered at 18 million Units/day, 5 days a week for two consecutive weeks. Treatment was administered safely at the full dose for 16 out of 17 patients, and toxicity was mild. One patient had treatment stopped because of rapidly progressive disease. As soon as patients met biological and clinical criteria, chemotherapy was administered. The median delay was 12 days (2-23) from the end of IL-2 administration. Only 13 patients received anthracyclin chemotherapy after IL-2. The other 4 patients did not receive chemotherapy for progressive disease. One partial response was observed out of 13 evaluable patients (7.7% overall response, 95% confidence interval: 0.2 to 36). The overall response rate was 5.9% (95% CI: 0.15 to 29), so the study was stopped due to lack of efficacy. In previous and current studies, a few patients have developed restored anthracyclin chemosensitivity following exposure to IL-2. No conclusive evidence of IL-2 chemoresistance reversal was obtained from this study. Further investigations need to be performed with perhaps a larger group of more carefully selected patients using a different schedule and sequence of combined cytokines and chemotherapy.

4
UI - 21378866
AU - Johnson EP; Bushman FD
TI - Paired DNA three-way junctions as scaffolds for assembling integrase complexes.
SO - Virology 2001 Aug 1;286(2):304-16
AD - Infectious Disease Laboratory, The Salk Institute, La Jolla, California 92037, USA.
Early steps of retroviral replication involve reverse transcription of the viral RNA genome and integration of the resulting cDNA copy into a chromosome of the host cell. The initial DNA breaking and joining steps of integration are carried out by the virus-encoded integrase enzyme. Integrases bind specifically to the ends of the unintegrated viral cDNA but nonspecifically to target DNA. Conventional assays in vitro reveal primarily the nonspecific DNA binding mode, complicating studies of integrase--DNA complexes. Here, we report an investigation of unconventional DNA structures useful for positioning integrase at predetermined sites. We find that paired DNA three-way junctions can be used to mimic branched DNAs normally formed as reaction intermediates. The three-way junctions differ from authentic intermediates in the connectivity of the DNAs, which, in contrast to the authentic intermediate, allow formation of stable DNA structures under physiological conditions. Assays in vitro showed that integrase can direct hydrolysis at sequences resembling the viral cDNA ends within the three-way junction, but not on junctions with mutant sequences. Changing the spacing between the paired three-way junctions disrupted the cleavage pattern, emphasizing the importance of the correct DNA scaffold. DNase I footprinting studies revealed protection of specific bases at the terminus of the LTR in the three-way junction complex, but not on control linear DNA, specifying the locations of tight interactions between integrase and DNA. Paired DNA three-way junctions are attractive reagents for structural studies of integrase-DNA complexes. Copyright 2001 Academic Press.

5
UI - 21388259
AU - Grunbaum U; Meye A; Bache M; Bartel F; Wurl P; Schmidt H; Dunst J; Taubert H
TI - Transfection with mdm2-antisense or wtp53 results in radiosensitization and an increased apoptosis of a soft tissue sarcoma cell line.
SO - Anticancer Res 2001 May-Jun;21(3B):2065-71
AD - Institute of Pathology, Martin Luther University Halle-Wittenberg, Halle/Saale, Germany.
Soft tissue sarcomas (STS) are mostly resistant after radiation treatment and are characterized by a rather low rate of apoptosis. The aim of this study was to test, in the p53 mutant STS cell line US8-93, the effect of a combined treatment with DNA transfection--either with mdm2 antisense oligodesoxynucleotides (mdm2-AS) or with a wild-type p53-plasmid (wtp53)--and the effects of irradiation on radiosensitivity. Mdm2-sense oligodesoxynucleotides (mdm2-SE) and a GFP-plasmid (GFP) were applied as controls. In order to evaluate the treatment radiation sensitization (clonogenic survival), apoptotic cell death and P53/MDM2-protein expression were determined. A moderately increased radiation sensitization was observed when comparing clonogenic survival after 2 Gy irradiation between cells transfected either with the control mdm2-SE (48%) or with mdm-2 AS (30%). At the same irradiation dose, clonogenic survival of wtp53-plasmid transfected cells (32%) was about 2-fold less than in the cells transfected with the control GFP-plasmid (61%). This enhancement factor of radiation sensitization was increased by about 3-fold at 4 Gy irradiation. Furthermore, an increase in apoptotic cells was already detectable by up to 7.7% (mdm2-AS) in comparison to 3.1% (mdm2-SE control) 72 hours after transfection. In parallel, the percentage of apoptotic cells could be further elevated after subsequent irradiation with 12 Gy by up to 15% (mdm2-AS) compared to 5.7% (mdm2-SE control). A striking result was obtained with the combined treatment of a wtp53 and 12 Gy irradiation which produced in 25% and 38.9% of apoptotic cells 48 hours and 72 hours after transfection, respectively. We can therefore conclude that the sensitivity of radiation therapy is enhanced by DNA transfection with wtp53 or mdm-2 AS ODNs for the correction of the p53-mdm2 balance in STS in vitro.

6
UI - 21396385
AU - Hill KA; Gonzalez-Crussi F; Chou PM
TI - Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: a histological and immunohistochemical comparison.
SO - Mod Pathol 2001 Aug;14(8):784-90
AD - Department of Surgical Pathology, Children's Memorial Hospital, Northwestern University, 2300 Children's Plaza, Chicago, IL 60614, USA.
Calcifying fibrous pseudotumor (CFP), a recently described lesion, is characterized by a predominantly lymphoplasmacytic infiltrate with abundant hyalinized collagen and psammomatous or dystrophic calcifications. The cause and pathogenesis are unclear, but it has been postulated that CFP may represent a sclerosing end stage of inflammatory myofibroblastic tumor (IMT). We compared the histological and immunohistochemical profiles of seven cases diagnosed as CFP and seven as IMT. Histologically, the CFP demonstrated varying degrees of calcifications in addition to fibroblastic proliferation admixed with inflammatory cells composed of lymphocytes, eosinophils, and mast cells. The IMTs rarely contain calcifications and had a myofibroblastic proliferation varying from hyalinized acellular collagen to florid fibroblastic proliferations simulating sarcoma. The inflammatory component was composed primarily of plasma cells and lymphocytes, sometimes arranged as lymphoid aggregates with germinal centers. All CFP cases were diffusely positive for factor XIIIa and negative for smooth muscle actin, muscle-specific actin, and CD34. All IMTs demonstrated diffuse positivity for actin, variable positivity for CD34, and focal positivity for Factor XIIIa. This study demonstrates certain distinct histologic, immunohistochemical, and electron microscopic features between IMTs and CFPs.

7
UI - 21406759
AU - Pollock J
TI - Radiation-induced secondary malignancy of the esophagus.
SO - Ann Thorac Surg 2001 Aug;72(2):669

8
UI - 21379843
AU - Tamborini E; Papini D; Mezzelani A; Riva C; Azzarelli A; Sozzi G; Pierotti MA; Pilotti S
TI - c-KIT and c-KIT ligand (SCF) in synovial sarcoma (SS): an mRNA expression analysis in 23 cases.
SO - Br J Cancer 2001 Aug 3;85(3):405-11
AD - Department of Pathology, Istituto Nazionale per lo Studio e la Cura dei Tumori, Milano, 20133, Italy.
In a previous immunophenotypic molecular-based analysis it was shown that bcl2 over-expression characterizes the SS gene profile in addition to the non-random translocations. Here we show that the over-expression of an additional potentially antiapoptotic gene, the c-KIT gene, is associated with this tumour. Interestingly, whereas bcl2 over-expression appears to be restricted to the spindle cell tumoral component, c-kit mainly involves the epithelial component of biphasic SS. Twenty-three primary and metastatic samples from 21 patients were analysed by immunophenotyping (23/23), immunoprecipitations and Western blotting (3/23), and RT-PCR (23/23). Ten cases were biphasic and 13 monophasic in sub-type. Twelve, 10 and 1 case carried the SYT-SSX1, SYT-SSX2 and SYT-SSX4 fusion transcript, respectively. Co-presence of both c-Kit and SCF mRNA was observed in almost all cases (20/23), suggesting the occurrence of an autocrine loop. Immunophenotyping, confirmed by biochemical analyses, showed a modulation of c-Kit expression which was faint in the spindle and strong in the epithelial component, respectively. The study was complemented by c-Met/HGF receptor/ligand expression and c-Met protein analysis with results superimposable to those already reported. Since in each tumour, epithelial and spindle cell components harbour the same type of translocation t(X;18) the present findings suggest a shifting of the anti-apoptotic role from BCL2 to c-KIT gene during the transition from the uncommitted spindle to the differentiated epithelial cells. Copyright 2001 Cancer Research Campaign.

9
UI - 21175026
AU - el-Zeftawy H; Heiba SI; Jana S; Rosen G; Salem S; Santiago JF; Abdel-Dayem HM
TI - Role of repeated F-18 fluorodeoxyglucose imaging in management of patients with bone and soft tissue sarcoma.
SO - Cancer Biother Radiopharm 2001 Feb;16(1):37-46
AD - Saint Vincent's Catholic Medical Centers of New York, Section of Nuclear Medicine, Department of Radiology, New York Medical College, Valhalla, NY, USA.
AIM OF THE STUDY: To assess the impact of repeated F-18 FDG studies on the management of patients with bone and soft tissue (B&S) sarcomas. MATERIAL AND METHODS: Twenty patients with B&S tissue tumors (11 M and 9 F age 17-72 years) had 52 F-18 FDG Dual Head Coincidence Imaging (DHCI) studies. 7 patients were followed for 6 months to 2 years clinically after removal of the primary tumor. Thirteen patients were evaluated for suspected recurrences. Patient's preparation, F-18 FDG injection and imaging procedure were done according to department protocol. Attenuation corrected images were interpreted visually by 3 trained physicians. Tumor to background ratios were calculated for all lesions. RESULTS: In 13 patients having both studies, baseline FDG and CT/MRI were concordant in 8 patients, FDG detected more lesions in 3 patients but it did not detect 4 metastatic pulmonary nodules in 2 patients. Follow up studies showed stable disease in 10 patients while 6 patients who showed worsening disease needed to change their chemotherapy. Surgery was avoided in 2 patients and 2 patients showed improved response. CONCLUSION: Repeated F-18 FDG DHCI examinations proved to have an impact on the clinical management of patients with malignant bone and soft tissue sarcoma. It helps to differentiate postoperative changes from local recurrence.

10
UI - 21244949
AU - Craciunescu OI; Das SK; McCauley RL; MacFall JR; Samulski TV
TI - 3D numerical reconstruction of the hyperthermia induced temperature distribution in human sarcomas using DE-MRI measured tissue perfusion: validation against non-invasive MR temperature measurements.
SO - Int J Hyperthermia 2001 May-Jun;17(3):221-39
AD - Department of Radiation Oncology, Duke University Medical Center, Durham, NC 27710, USA. oana@radonc.duke.edu
Essential to the success of optimized thermal treatment during hyperthermia is accurate modelling. Advection of energy due to blood perfusion significantly affects the temperature. Without accurate estimates of the magnitude of the local tissue blood perfusion, accurate estimates of the temperature distribution can not be made. It is shown here that the blood mass flow rate per unit volume of tissue in the Pennes' bio-heat equation can be modelled using a relative perfusion index (RPI) determined with dynamic-enhanced magnetic resonance imaging (DE-MRI). Temperature distributions in two patients treated with hyperthermia at Duke University Medical Center for high-grade leg tissue sarcomas are modelled, and the resultant temperatures are compared to measured temperatures using a non-invasive MR thermometry technique. Significant correlations are found between the DE-MRI perfusion images, the MR temperature images, and the numerical simulation of the temperature field. The correlation between DE-MRI measured values and advective heat loss in tissue is used to scale the perfusion distribution, thereby allowing the continuum model to account for the local thermal impact of vasculature in the tumour. Large vessels in tumour and neighbouring healthy tissue need to be taken into account in order to accurately describe the complete temperature distribution.

11
UI - 21367088
AU - Sosa C; Benetucci J; Hanna C; Sieczkowski L; Deluchi G; Canizal AM; Mantina H; Klaskala W; Baum M; Wood C
TI - Human herpesvirus 8 can be transmitted through blood in drug addicts.
SO - Medicina (B Aires) 2001;61(3):291-4
AD - University of Nebraska School of Biological Sciences, Lincoln, NE, USA.
Human Herpes virus type-8 (HHV-8) seroprevalence was studied in a population of HIV positive intravenous drug users (IVDUs) from Argentina. Analysis of this population also indirectly made it possible to study HHV-8 blood transmission, because these individuals frequently engage in needle sharing behavior and are capable of acquiring a broad array of blood borne pathogens, including Hepatitis B/C virus. The seroprevalence of HHV-8 in IVDUs was compared to a group of non-IVDUs and HIV negative individuals. Of the 223 individuals tested, 13.45% were HHV-8 positive, 16.99% in the IVDUs group, and 5.71% in the non-IVDUs. Among HIV positive IVDUs, 25/144 (17.36%) were also HHV-8 seropositive. The seropositivity rate of HHV-8 in HIV negative IVDUs was 11.1%. In contrast, HHV-8 seroprevalence in HIV negative heterosexual individuals without drug usage behavior was even lower (5.71%). The rate of HHV-8 infection in HIV positive IVDUs was three times as high compared to the non IVDU HIV negative individuals, suggesting that IVDU is a risk for HHV-8 infection. Furthermore, it was found that IVDUs showed a very high rate of Hepatitis B/C (52.77%), which also correlate with HHV-8 infection in this population (23.68%). All Hepatitis B/C positive individuals were also HIV positive. Our data confirm other studies showing that individuals who share needles are at risk for acquiring Hepatitis B/C and HIV infections. In addition, our results suggest that they are also at risk to acquiring HHV-8 infection by the same route.

12
UI - 21374481
AU - Wiener ES; Anderson JR; Ojimba JI; Lobe TE; Paidas C; Andrassy RJ; Raney RB; Qualman SJ; Donaldson SS; Maurer HM; Link MP; Crist WM; Grier HE
TI - Controversies in the management of paratesticular rhabdomyosarcoma: is staging retroperitoneal lymph node dissection necessary for adolescents with resected paratesticular rhabdomyosarcoma?
SO - Semin Pediatr Surg 2001 Aug;10(3):146-52
AD - Soft Tissue Sarcoma Committee of the Children's Oncology Group, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
PURPOSE: Use of retroperitoneal lymph node dissection (RPLND) in paratesticular rhabdomyosarcoma (PTRMS) is controversial and has changed over the past 2 decades. The Intergroup Rhabdomyosarcoma Study Group (IRSG) required ipsilateral RPLND (IRPLND) for all patients with PTRMS treated on IRS-III (1984-91), but changed to clinical evaluation of RPLNs using computerized tomography (CT) in IRS-IV (1991 through 1997). In IRS-IV, only those patients with identified lymph node involvement on CT required surgical evaluation of the RPLNs. Nodal radiation therapy was administered only to patients with RPLNs recognized as positive; such patients received more intensive chemotherapy as well. Thus, they compared the incidence of recognized RPLN involvement using these 2 different approaches. They then analyzed patient outcome to determine whether this change in management affected outcome. METHODS: Eligible patients with group I or II PTRMS who were treated on IRS III (n = 100) or IRS IV (n = 134) were analyzed. Failure-free survival (FFS) and survival (S) rates were estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: There was a significant change in the distribution of patients with group I versus II tumors from IRS-III to IRS-IV (group I, 68% in IRS-III versus 82% in IRS-IV). This was the result of decreased node recognition when CT was used to stage RPLNs in IRS-IV and was most notable for adolescents (>10 years of age). Overall, 3-year FFS was 92% for patients treated on IRS-III and 86% for those treated on IRS-IV (P =.10), whereas survival estimates were 96% and 92%, respectively (P =.30). Adolescents were at higher risk of RPLN relapse than were children (<10 years of age) and their FFS and survival were worse, regardless of IRS protocol. Furthermore, adolescents with recognized group II tumors experienced better 3-year FFS than those with group I tumors on IRS-IV (100% versus 68%, P =.06), most likely as a result of receiving radiotherapy and intensified chemotherapy. CONCLUSIONS: Use of only CT scan evaluation of RPLN in IRS-IV led to a decrease in identification of RPLN involvement in boys who present with localized PTRMS, and a higher rate of regional relapse as compared with IRS-III. Adolescents had much higher likelihood of RPLN disease, and they fared significantly worse than did younger children on both studies. Furthermore, adolescent boys with group I tumors experienced worse FFS than those with Group II tumors on IRS-IV, probably because some patients with group II tumors were not identified by CT imaging and thus received less effective therapy. These data suggest that adolescents should have ipsilateral RPLN dissection as part of their routine staging, and those with positive lymph nodes require intensified chemotherapy as well as nodal irradiation. Copyright 2001 by W.B. Saunders Company

13
UI - 21242248
AU - Benda JA
TI - Pathology of smooth muscle tumors of the uterine corpus.
SO - Clin Obstet Gynecol 2001 Jun;44(2):350-63
AD - Department of Pathology, University of Iowa College of Medicine, 200 Hawkins Drive, 5244A RCP, Iowa City, IA 52242-1009, USA.

14
UI - 21249641
AU - Pollock R
TI - Soft tissue sarcoma: setting the stage.
SO - Ann Surg Oncol 2001 May;8(4):273-4

15
UI - 21249643
AU - Ramanathan RC; A'Hern R; Fisher C; Thomas JM
TI - Prognostic index for extremity soft tissue sarcomas with isolated local recurrence.
SO - Ann Surg Oncol 2001 May;8(4):278-89
AD - Royal Marsden Hospital, London, United Kingdom. ramanathanrc@msx.upmc.edu
BACKGROUND: Local recurrence occurs in 10% to 20% of patients with extremity soft tissue sarcomas despite optimal treatment. The association of local recurrence with subsequent survival is controversial and conflicting. There is a need for a staging system to predict outcome in this subset of patients and also to plan optimal treatment, including adjuvant systemic therapy. METHODS: Data collected from 110 patients with locally recurrent extremity soft tissue sarcomas were studied. The influence of clinical and pathologic factors on local recurrence, distant metastasis, and disease-specific survival were analyzed by univariate and multivariate techniques. RESULTS: Of the 110 patients who presented with local recurrence, 92 had an isolated local recurrence and 18 had prior or concomitant distant metastases. The 5-year disease-specific survival for all patients was 63% and for those with isolated local recurrence, it was 69%. Histologic grade, malignant fibrous histiocytoma histology, pathologic margins, previous local recurrence, and prior radiotherapy were independent prognostic factors for subsequent local recurrence. Tumor size, histologic grade, and time to local recurrence were independent prognostic factors for distant metastasis and disease-specific survival. A prognostic index was calculated by assigning a score of 1 to 3 for each of the three independent prognostic factors for survival and added to give the prognostic index for each patient. As the prognostic index increased from 3 to 9, there was a progressive increase in the hazard ratios and a corresponding deterioration in survival. The patients were then categorized into three prognostic groups based on the hazard ratios for disease specific survival. The differences in the survival curves were highly statistically significant (P < .0001). CONCLUSIONS: Tumor size, histologic grade, and time to local recurrence are the primary determinants of distant metastases and survival in locally recurrent extremity soft tissue sarcomas. The impact of local recurrence on survival varies considerably. The nature of the local recurrence, rather than its presence per se, is a more useful guide to prognosis. We propose a simple staging system based on size, grade, and time to recurrence that correlates extremely well with prognosis and may serve as a guide to make therapeutic decisions in patients with locally recurrent extremity soft tissue sarcomas.

16
UI - 21249644
AU - Clary BM; DeMatteo RP; Lewis JJ; Leung D; Brennan MF
TI - Gastrointestinal stromal tumors and leiomyosarcoma of the abdomen and retroperitoneum: a clinical comparison.
SO - Ann Surg Oncol 2001 May;8(4):290-9
AD - Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
INTRODUCTION: The present study was undertaken to define the clinical differences between leiomyosarcomas (LMS) occurring within the abdomen and retroperitoneum and gastrointestinal stromal tumors (GIST). METHODS: It was a retrospective, single-institution review of patients treated for intra-abdominal and retroperitoneal GIST and LMS from July 1, 1982 through August 1, 1999. RESULTS: A total of 561 patients, 239 with GIST and 322 with LMS, were identified. Patients with GIST were older, with a median age of 58 years versus 54 years in the LMS group (P < .01). The majority of patients with GIST were male (58%), whereas 68% of LMS patients (excluding gender-specific sites) were female (P < .01). The 5-year disease-specific survival for GIST and LMS were 28% and 29%, respectively. The presentation status and ability to achieve a complete surgical resection were the main independent predictors of outcome for both GIST and LMS. Local and distant recurrence was common in both. The pattern of distant recurrence differed: 50% of all first-site GIST recurrences involved the liver, whereas 30% of all LMS first-site recurrences involved the lungs. CONCLUSIONS: Although the two patient populations appear to be distinct, their clinical courses are similar. The pattern of distant spread follows the known patterns of hematogenous dissemination. Complete surgical resection is the cornerstone of treatment for primary GIST and LMS and in selected patients with local and distant recurrence.

17
UI - 21372512
AU - Bauer M; Meyer M; Sautter J; Gasser T; Ueffing M; Widmer HR
TI - Liposome-mediated gene transfer to fetal human ventral mesencephalic explant cultures.
SO - Neurosci Lett 2001 Aug 10;308(3):169-72
AD - Department of Neurology, Klinikum Grobetahadern, University of Munich, Marchioninistrabetae 15, 81366, Munich, Germany.
The feasibility of non-viral gene transfer using liposomes is described for human fetal nigral tissue. Ventral mesencephalic explants from 6 to 12 week old fetuses were grown as free-floating roller tube cultures. For the transfection, a vector coding for beta-galactosidase driven by the Rous Sarcoma Virus promoter was used. The developmental stage of the human tissue, time in vitro and the amount of vector DNA used significantly influenced the transfection efficiency. Optimal transfection results were obtained with tissue from a 10 week old fetus, cultured for 4 days and transfected with mixtures containing 4 microg vector DNA. Histological analysis suggested that a specific population of ventral mesencephalic precursor cells were the target for the gene transfer. This finding might have implications for gene delivery and cell replacement strategies in Parkinson's disease.

18
UI - 21371953
AU - Guillen DR; Cockerell CJ
TI - Cutaneous and subcutaneous sarcomas.
SO - Clin Dermatol 2001 May-Jun;19(3):262-8
AD - Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas 75235, USA.

19
UI - 21390891
AU - Cai X; Wang T; Shi L
TI - [Nonepithelial malignant neoplasms of the larynx and hypopharynx]
SO - Zhonghua Er Bi Yan Hou Ke Za Zhi 1998 Apr;33(2):113-6
AD - First Affiliated Hospital of Shandong Medical University, Jinan 250012.
OBJECTIVES: Nonepithelial Malignant Neoplasms of the larynx and hypopharynx are rare. Their clinical features, diagnosis and treatment were discussed. METHODS: From 1985 to 1996, 23 cases of nonepithelial malignant neoplasms of the larynx and 3 cases of hypopharynx were diagnosed. There were 21 males and 5 females. Age of the patients ranged from 16 to 65 years. Laryngeal tumours included 11 cases of sarcomas, 1 extramedullary plasmocytoma, 9 lymphomas and 2 malignant melanomas. Hypopharyngeal tumours included 1 leiomyosarcoma and 2 malignant melanomas. Extensive resection was the first treatment for 21 patients. Then, according to their histopathologic features, irradiation or/and chemotherapy were chosen. RESULTS: Follow-up period was from 6 months to 10 years. There were 12 patients survived for 4 to 10 years, including 6 cases of laryngeal sarcomas, 2 supraglottic lymphomas, 2 malignant melanomas of the larynx, 1 malignant melanoma and 1 leiomyosarcoma of the hypopharynx. CONCLUSIONS: Characteristics for these tumours were: 1. usually covered by intact mucosa; 2. laryngeal tumours rarely metastasize to the cervical lymph nodes; 3. difficulties in histopathological diagnosis, and immunohistochemistry is helpful to differentiation and determination.

20
UI - 21397697
AU - Afolabi OC; Odukoya O; Arole G; Banjo AF
TI - Nucleolar organizer regions in jaw tumours of cartilaginous origin.
SO - West Afr J Med 2001 Jan-Mar;20(1):17-21
AD - Nigerian Institute of Medical Research, Yaba, Nigeria.
Nucleolar organizer regions [NORs] are loops of DNA that transcribe to ribosomal RNA. They can be visualized as intranuclear black dots by histochemical staining with a colloid silver solution. Silver-stained nucleolar proteins [AgNORs] were counted in cases comprising of primary chondrosarcomas of three histologic grades, in chondromyxoid fibroma and in controls comprising of normal bone and cartilage tissues of the jaw bones. The AgNOR counts increased step-wisely from normal bone tissue [1.11 0.4], chondromyxoid fibroma [2.66 0.78], grade I chondrosarcoma [3.94 0.34], grade II chondrosarcoma [4.32 0.52], and grade III chondrosarcoma [5.54 0.44]. There was a statistically significant difference in the mean AgNOR counts between grade 1 and grade III chondrosarcoma [p < 0.05]. The mean AgNOR counts for benign cartilaginous [chondromyxoid fibroma] tumour was significantly lower than the mean, AgNOR count for malignant cartilaginous tumours [chondrosarcomas] [p < 0.05]. The results in the present study indicate that silver colloid staining is a useful technique for evaluating the proliferative activity of chondrosarcoma and benign cartilaginous tumour such as chondromyxoid fibroma.

21
UI - 21414561
AU - Kok KY; Telesinghe PU; Yapp SK
TI - Treatment and outcome of cystosarcoma phyllodes in Brunei: a 13-year experience.
SO - J R Coll Surg Edinb 2001 Aug;46(4):198-201
AD - Department of Surgery, Ripas Hospital, Bandar Seri Begawan, Brunei. koky@brunet.bn
The purpose of this article is to discuss the benefits of and to illustrate a framework for appraisal. The place of career advice in this process is mentioned, as is a brief discussion on Assessment. From the point of view of the individual doctor, information to help him/her choose an appropriate career path should be readily available. It is more likely that a doctor will perform well throughout their career, if in a career or occupation that suits them.

22
UI - 21433647
AU - Fleming JB; Cantor SB; Varma DG; Holst D; Feig BW; Hunt KK; Patel SR; Benjamin RS; Pollock RE; Pisters PW
TI - Utility of chest computed tomography for staging in patients with T1 extremity soft tissue sarcomas.
SO - Cancer 2001 Aug 15;92(4):863-8
AD - Sarcoma Center, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030-4009, USA.
BACKGROUND: National Cancer Center Network (NCCN) and Society of Surgical Oncology (SSO) practice guidelines recommend chest computed tomography (CT) as part of the staging evaluation of patients with extremity soft tissue sarcoma (STS). In the current study, the authors evaluated the use and yield of chest roentgenography (CXR) and selective chest CT to screen for pulmonary metastases in patients with T1 STS. METHODS: The utility of these staging studies was evaluated retrospectively in a cohort of 125 consecutive patients who presented to a tertiary care cancer center with T1 primary (nonrecurrent) extremity STS. Two diagnostic strategies (CXR alone vs. CXR plus chest CT) were evaluated using an incremental cost-effectiveness ratio. RESULTS: The majority of tumors (70%) were high grade. The median sarcoma size was 3.0 cm; 64 of the tumors (51%) were located deep to the investing fascia of the extremity. All patients underwent staging CXR; 1 CXR (< 1%) was suspicious for metastatic disease. Fifty-one patients (41%) also underwent chest CT; 1 chest CT, performed in the patient with a suspicious CXR, revealed metastatic disease. With a median follow-up of 76 months, 19 patients (15%) developed metachronous pulmonary metastases. The relatively low yield resulted in an incremental cost-effectiveness ratio of $59,772 per case of synchronous pulmonary metastasis detected by CXR plus chest CT. CONCLUSIONS: Less than 1% of patients with T1 primary extremity STS were found to have pulmonary metastases that were detectable using a staging algorithm that employs routine CXR with the selective use of chest CT. The findings of the current study do not support current NCCN or SSO practice guidelines for patients with high-grade T1 STS. Copyright 2001 American Cancer Society.

23
UI - 21433648
AU - Hoos A; Stojadinovic A; Mastorides S; Urist MJ; Polsky D; Di Como CJ; Brennan MF; Cordon-Cardo C
TI - High Ki-67 proliferative index predicts disease specific survival in patients with high-risk soft tissue sarcomas.
SO - Cancer 2001 Aug 15;92(4):869-74
AD - Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
BACKGROUND: Soft tissue sarcomas (STSs) are heterogeneous neoplasms that have variable clinical outcome. Several clinical parameters and few molecular markers, including Ki-67 proliferative index, have been shown to correlate with patient prognosis. To the authors' knowledge, no definitive report exists to identify one molecular marker that can be analyzed easily in a clinical setting and that predicts survival in a cohort of patients with high-risk STS of identical clinical characteristics but variable outcome. METHODS: The influence of clinical prognostic factors was eliminated by selecting two patient groups with identical high-risk characteristics: large (> 10 cm), high-grade, deep, completely resected primary extremity STS (n = 47). Patients in the first group remained disease free (no evidence of disease [NED]) after primary tumor treatment (n = 19), whereas patients in the second group subsequently died of disease (DOD; n = 28). Triplicate 0.6-mm core biopsies from defined morphologic areas of paraffin embedded primary tumors were assembled on a tissue microarray and analyzed by immunohistochemistry with the MIB-1 antibody, and Ki-67 proliferative indices were correlated with patient outcome. RESULTS: High Ki-67 proliferative index, defined as greater than 30% tumor cells showing nuclear immunoreactivity, was significantly more frequent in the DOD group than in the NED group and was associated with tumor-related mortality (P = 0.02). This marker identifies an especially aggressive malignant phenotype within a cohort of high-risk tumors that is based on well established clinical and pathologic parameters alone and is easy to use in a clinical setting. CONCLUSIONS: On the basis of these data and previous reports, high Ki-67 proliferative index is suggested as a significant factor for predicting the prognosis of patients with high-risk STS and should be evaluated prospectively based on clinical trials. Copyright 2001 American Cancer Society.

24
UI - 20345110
AU - Coscoy L; Ganem D
TI - Kaposi's sarcoma-associated herpesvirus encodes two proteins that block cell surface display of MHC class I chains by enhancing their endocytosis.
SO - Proc Natl Acad Sci U S A 2000 Jul 5;97(14):8051-6
AD - Howard Hughes Medical Institute and Departments of Microbiology and Medicine, University of California, San Francisco, CA 94143, USA.
Down-regulation of the cell surface display of class I MHC proteins is an important mechanism of immune evasion by human and animal viruses. Herpesviruses in particular encode a variety of proteins that function to lower MHC I display by several mechanisms. These include binding and retention of MHC I chains in the endoplasmic reticulum, dislocation of class I chains from the ER, inhibition of the peptide transporter (TAP) involved in antigen presentation, and shunting of newly assembled chains to lysosomes. Kaposi's sarcoma (KS)-associated herpesvirus (KSHV) is a human herpesvirus strongly linked to the development of KS and to certain AIDS-associated lymphoproliferative disorders. Here we show that KSHV encodes two distinctive gene products that function to dramatically reduce cell surface MHC I expression. These viral proteins are localized predominantly to the ER. However, unlike previously described MHC I inhibitors, they do not interfere with the synthesis, translocation, or assembly of class I chains, nor do they retain them in the ER. Rather, they act to enhance endocytosis of MHC I from the cell surface; internalized class I chains are delivered to endolysosomal vesicles, where they undergo degradation. These KSHV proteins define a mechanism of class I down-regulation distinct from the mechanisms of other herpesviruses and are likely to contribute importantly to immune evasion during viral infection.

25
UI - 20493630
AU - Fogel BL; McNally MT
TI - A cellular protein, hnRNP H, binds to the negative regulator of splicing element from Rous sarcoma virus.
SO - J Biol Chem 2000 Oct 13;275(41):32371-8
AD - Department of Microbiology and Molecular Genetics, Medical College of Wisconsin, Milwaukee, Wisconsin 53226, USA.
Incomplete RNA splicing is a key feature of the retroviral life cycle. This is in contrast to the processing of most cellular pre-mRNAs, which are usually spliced to completion. In Rous sarcoma virus, splicing control is achieved in part through a cis-acting RNA element termed the negative regulator of splicing (NRS). The NRS is functionally divided into two parts termed NRS5' and NRS3', which bind a number of splicing factors. The U1 and U11 small nuclear ribonucleoproteins interact with sequences in NRS3', whereas NRS5' binds several proteins including members of the SR [corrected] family of proteins. Among the proteins that specifically bind NRS5' is a previously unidentified 55-kDa protein (p55). In this report we describe the isolation and identification of p55. The p55 binding site was localized by UV cross-linking to a 31-nucleotide segment, and a protein that binds specifically to it was isolated by RNA affinity selection and identified by mass spectrometry as hnRNP H. Antibodies against hnRNP H immunoprecipitated cross-linked p55 and induced a supershift of a p55-containing complex formed in HeLa nuclear extract. Furthermore, UV cross-linking and electrophoretic mobility shift assays indicated that recombinant hnRNP H specifically interacts with the p55 binding site, confirming that hnRNP H is p55. The possible roles of hnRNP H in NRS function are discussed.

26
UI - 21238961
AU - Creager AJ; Cohen JA; Geradts J
TI - Aberrant expression of cell-cycle regulatory proteins in human mesenchymal neoplasia.
SO - Cancer Detect Prev 2001;25(2):123-31
AD - Department of Pathology, University of North Carolina School of Medicine, Chapel Hill, USA.
We previously demonstrated that approximately one-half of soft-tissue sarcomas were devoid of either pRB, the product of the retinoblastoma gene, or 16, the product of the MTS1/CDKN2 gene, while a smaller subset of aggressive mesenchymal tumors without metastatic potential did not express RB by immunohistochemistry. We now studied the expression of two additional important cell-cycle regulators, namely cyclin D1 and p53, in the same cohort of high- and low-grade lesions. In the aggregate, our data provide a comprehensive overview of the importance of cell-cycle deregulation in mesenchymal neoplasia. Paraffin sections of 58 sarcomas and 23 soft-tissue tumors of low malignant potential (STT-LMP) were reacted with monoclonal antibodies against cyclin D1 and p53, using optimized immunohistochemical staining protocols. The staining data were correlated with expression of pRB and p15 and with a variety of pathologic parameters. A total of 33 of 58 sarcomas (57%) and 9 of 23 STT-LMP (39%) overexpressed p53. Fourteen sarcomas (24%) and 4 STT-LMP (17%) overexpressed cyclin D1. There was no correlation between expression of these two genes and histologic tumor type or grade. Loss of RB and loss of p16 or overexpression of cyclin D1 were mutually exclusive events. Considering all four cell-cycle regulators, sarcomas had a significantly higher abnormality rate than did STT-LMP (P < .005). Only 10% of the sarcomas but 39% of STT-LMP showed normal expression of all four gene products. Based on our findings, overexpression of cyclin D1 and (presumably mutant) p53 appear to be among the most common molecular alterations in human mesenchymal neoplasia, and abrogation of cell-cycle control is observed in the great majority of sarcomas; it is present significantly less frequently in low-grade lesions.

27
UI - 21331832
AU - Jang KA; Ahn SJ; Choi JH; Sung KJ; Moon KC; Koh JK; Shim YH
TI - Polymerase chain reaction (PCR) for human herpesvirus 8 and heteroduplex PCR for clonality assessment in angiolymphoid hyperplasia with eosinophilia and Kimura's disease.
SO - J Cutan Pathol 2001 Aug;28(7):363-7
AD - Department of Dermatology, Seoul Paik Hospital, Inje-University, Seoul, Korea. jang722@netsgo.com
BACKGROUND: Recently, human herpesvirus 8 (HHV-8) has been isolated from almost all cases of Kaposi's sarcoma. It has not been found in most cutaneous hemangioproliferative disorders other than Kaposi's sarcoma. Benign vascular lesions including Kimura's disease were not found to contain the HHV-8 DNA sequence. However, there has been contradictory data concerning the presence of HHV-8 in angiolymphoid hyperplasia with eosinophilia (ALHE). Clonality studies in ALHE and Kimura's disease were rare. METHODS: We performed polymerase chain reaction (PCR)-based analysis to determine whether HHV-8 is present and heteroduplex analysis of rearranged T-cell receptor (TCR) gene for clonality assessment in paraffin-embedded skin biopsy samples of 7 ALHE and 2 Kimura's disease, taken from immunocompetent patients. RESULTS: HHV-8 could not be identified in all the cases of ALHE and Kimura's disease. Although 2 cases (2/7) of ALHE and 2 cases (2/2) of Kimura's disease showed positive result for PCR analysis of TCR, all the cases were negative for heteroduplex-PCR. CONCLUSIONS: We suggest that HHV-8 may not involve in a pathogenetic role in ALHE and Kimura's disease and the failure to demonstrate clonality may be consistent with the reactive nature of these diseases and lack of malignant transformation. In addition, heteroduplex-PCR can be applied to confirm doubtful cases of lymphoma in that heteroduplex-PCR is more specific than PCR as seen in our study.

28
UI - 21392729
AU - Malo M; Davis AM; Wunder J; Masri BA; Bell RS; Isler MH; Turcotte RE
TI - Functional evaluation in distal femoral endoprosthetic replacement for bone sarcoma.
SO - Clin Orthop 2001 Aug;(389):173-80
AD - University of Montreal, Quebec, Canada.
A multicenter study of successfully treated patients (mean age, 36.7 years) with a minimum 1-year followup (average, 35.4 months) after distal femoral endoprosthetic replacement for bone sarcoma was done using the 1987 and 1993 versions of the Musculoskeletal Tumor Society, the Short Form-36, and the Toronto Extremity Salvage Score functional evaluation criteria. Fifty-six patients (28 women and 28 men) fulfilled the criteria. Thirty-one Kotz prostheses (fixed hinge, uncemented) and 25 Modular Replacement System Prostheses (rotating hinge, cemented) were used. Thirty-five patients walked without aids, 19 used a cane, and two used crutches or a walker. The Musculoskeletal Tumor Society 1987 mean score was 28.1. The Musculoskeletal Tumor Society 1993 mean score was 80.4. The Toronto Extremity Salvage Score mean was 81.6. The Short Form-36 Physical Component Score had a mean of 43.2 and Mental Component Score mean of 54.2. The two groups of implants were comparable, except for the length of bone resection. Multivariate regression analysis revealed that patient age, existence of a pathologic fracture, and type of prosthesis all significantly accounted for differences in functional outcome as measured by the Musculoskeletal Tumor Society 1993, the Toronto Extremity Salvage Score, and the Short Form-36 Physical Component Score scales. Although both implants provided satisfactory function, the Musculoskeletal Tumor Society 1993 and the Toronto Extremity Salvage Score results were significantly better with the Modular Replacement System prosthesis. The effect of possible differences among surgeons or institutions was not addressed.

29
UI - 21387135
AU - Guilhot F
TI - [Anti-tyrosine kinase: the beginning of molecular therapies of cancer and initial results]
SO - Bull Cancer 2001 Jul;88(7):659-60
AD - Service d'oncologie hematologique et de therapie cellulaire, CHU La Miletrie, 86021 Poitiers. f.guilhot@chu-poitiers.fr

30
UI - 21387136
AU - Ray-Coquard I; Cesne AL; Blay JY
TI - [STI571 and gastro intestinal stromal tumors]
SO - Bull Cancer 2001 Jul;88(7):661-2
AD - Centre Leon-Berard. 28, rue Laennec, 69008 Lyon, France. ray@lyon.fnclcc.fr

31
UI - 21398564
AU - Hyun TS; Subramanian C; Cotter MA 2nd; Thomas RA; Robertson ES
TI - Latency-associated nuclear antigen encoded by Kaposi's sarcoma-associated herpesvirus interacts with Tat and activates the long terminal repeat of human immunodeficiency virus type 1 in human cells.
SO - J Virol 2001 Sep;75(18):8761-71
AD - Cellular and Molecular Biology Graduate Program, University of Michigan Medical School, Ann Arbor, Michigan 48109-0934, USA.
The latency-associated nuclear antigen (LANA) is constitutively expressed in cells infected with the Kaposi's sarcoma (KS) herpesvirus (KSHV), also referred to as human herpesvirus 8. KSHV is tightly associated with body cavity-based lymphomas (BCBLs) in immunocompromised patients infected with human immunodeficiency virus (HIV). LANA, encoded by open reading frame 73 of KSHV, is one of a small subset of proteins expressed during latent infection and was shown to be important in tethering the viral episome to host chromosomes. Additionally, it has been shown that LANA can function as a regulator of transcription. However, its role in the progression of disease is still being elucidated. Since KS is one of the most common AIDS-associated cancers in the United States and BCBLs appear predominantly in AIDS patients, we examined whether LANA is able to regulate the HIV type 1 (HIV-1) long terminal repeat (LTR). Using luciferase-based transient transfection assays, we found that LANA was able to transactivate the HIV-1 LTR in the human B-cell line BJAB, human monocytic cell line U937, and the human embryonic kidney fibroblast cell line 293T. Moreover, we observed that the virus-encoded HIV transactivator protein Tat cooperated with LANA in activation of the LTR in a dose-response fashion with increasing amounts of LANA. Surprisingly, LANA alone was sufficient to transactivate the HIV-1 LTR in BJAB cells. In similar assays using a HIV-1 LTR construct with the core enhancer elements deleted; the activity of LANA was diminished but not abolished, indicating a mechanism which involves the cooperation of the core enhancer elements and downstream elements which include Tat. Furthermore, transient transfection of an infectious clone of HIV with LANA demonstrated effects similar to those seen in the reporter assays based on Western blot analysis of HIV Gag polypeptide p24. Interestingly, we also demonstrated that the carboxy terminus of LANA associates with Tat in cells and in vitro. These experiments suggest a role for LANA in activating the HIV-1 LTR through association with cellular molecules targeting the core enhancer elements and Tat and may have important consequences in increasing the levels of HIV in infected individuals and, hence, the disease state.

32
UI - 21407954
AU - van Kampen M; Eble MJ; Lehnert T; Bernd L; Jensen K; Hensley F; Krempien R; Wannenmacher M
TI - Correlation of intraoperatively irradiated volume and fibrosis in patients with soft-tissue sarcoma of the extre

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