National Cancer Institute®
Last Modified: November 21, 2001
1
UI - 21256548
AU - Cappello F; Barnes L
TI -
Synovial sarcoma and malignant mesothelioma of the pleura: review,
differential diagnosis and possible role of apoptosis.
SO - Pathology 2001 May;33(2):142-8
AD - Institute of Pathological Anatomy, University of Palermo, Italy.
francapp@hotmail.com
Synovial sarcoma of the pleural cavity is exceptionally rare and may be
confused, both clinically and histologically, with malignant
mesothelioma, with subsequent inappropriate therapy. To address this
dilemma, four biphasic synovial sarcomas (BSSs) and four biphasic
malignant mesotheliomas (BMMs) were studied with a panel of mucin and
immunohistochemical stains to determine if they would allow one to
distinguish between the two. The BMMs were all pleural-based. The BSSs
were extrapleural. The mucin and immunohistochemical stains were all
performed on formalin-fixed, paraffin-embedded tissue using standard
techniques, with appropriate positive and negative controls. Mucin
present in BSS is, in general, mucicarmine-positive and resistant to
both hyaluronidase and diastase. Of the immune markers evaluated, only
calretinin, Ber-Ep4 and bcl-2 were of limited discriminatory value.
Subsets of cytokeratins, CEA and CD 34 were not helpful. With the
exception of bcl-2, the apoptotic markers p53, bax and cpp32 (caspase)
also were not useful. However, when the apoptotic stains were viewed
collectively, variations in expression between the two tumours raised
the possibility that alterations in apoptotic activity might be
responsible for their pathogenesis and behavior. The diagnosis of BSS or
BMM of the pleural should be made only after total consideration of
clinical, radiological, histochemical and immunohistochemical findings.
Although mucin stains are useful in differential diagnosis, reliance
solely on immunohistochemical markers, with the possible exception of
calretinin, Ber-Ep4 and bcl-2, is not dependable. The role of apoptosis
in the pathogenesis of these tumours needs to be explored with a much
larger series.
2
UI - 21256550
AU - Tse GM; Law BK; Chan KF; Mas TK
TI -
Multinucleated stromal giant cells in mammary phyllodes tumours.
SO - Pathology 2001 May;33(2):153-6
AD - Department of Anatomical and Cellular Pathology, Prince of Wales
Hospital, Hong Kong SAR, China. garytse@cuhk.edu.hk
Mammary phyllodes tumour (PT) is an uncommon fibroepithelial neoplasm
with a prominent stromal component. We report five cases of PT (one
benign, three borderline, one malignant) with giant cells in the stroma.
All occurred in adults and ranged from 1.8 to 4.0 cm in size. The
overall cellularity, stromal cell pleomorphism and mitotic count was
higher for the malignant and borderline than the benign PT. The giant
cell number ranged from 18 to 35 cells per 10 high power fields, but
there was no relationship between this number and the grade of the PT.
Most giant cells were subepithelial, with multiple nuclei arranged in a
linear or irregular pattern, and moderate amount of cytoplasm. The
immunohistochemical profile of the giant cells was similar to the
stromal cells. In all cases, both giant cells and stromal cells
expressed vimentin strongly but not desmin; in two cases, both cell
populations expressed actin weakly. The respective percentage of giant
cells and stromal cells expressing MIB1 was also similar. This suggests
that these giant cells do not represent a different, more active stromal
population, despite the more bizarre appearance. In view of the small
number of cases, the significance of such giant cells on the prognosis
of PT remains uncertain.
3
UI - 21267675
AU - Calle Y; Palomares T; Castro B; del Olmo M; Alonso-Varona A
TI -
Removal of N-glycans from cell surface proteins induces apoptosis by
reducing intracellular glutathione levels in the rhabdomyosarcoma cell
line S4MH.
SO - Biol Cell 2000;92(8-9):639-46
AD - Department of Cell Biology and Morphological Sciences, School of
Medicine and Odontology, University of the Basque Country, Leioa,
Vizcaya, Spain.
Expression of determined Asn-bound glycans (N-glycans) in cell surface
glycoproteins regulates different processes in tumour cell biology.
Specific patterns of N-glycosylation are displayed by highly metastatic
cells and it has been shown that inhibition of N-glycan processing
restrains cell proliferation and induces cell death via apoptosis.
However, the mechanisms by which different N-glycosylation states may
regulate cell viability and growth are not understood. Since malignant
cells express high levels of intracellular glutathione (GSH) and a
reduction of intracellular GSH induces cell death via apoptosis, we
investigated whether GSH was involved in the induction of apoptosis by
removal of cell surface N-glycans. We found that removal of N-glycans
from cell surface proteins by treating the rhabdomyosarcoma cell line
S4MH with tunicamycin or N-glycosidase resulted in a reduction in
intracellular GSH content and cell death via apoptosis. Moreover, GSH
depletion caused by the specific inhibitor of GSH synthesis BSO induced
apoptosis in S4MH cells. This data indicates that adequate
N-glycosylation of cell surface glycoproteins is required for
maintenance of intracellular GSH levels that are necessary for cell
survival and proliferation.
4
UI - 21338546
AU - Park YK; Park HR; Chi SG; Ushigome S; Unni KK
TI -
Overexpression of p53 and absent genetic mutation in clear cell
chondrosarcoma.
SO - Int J Oncol 2001 Aug;19(2):353-7
AD - Department of Pathology, Kyung Hee University Hospital, #1 Hoeki-dong,
Dongdaemoon-ku, Seoul 130-702, Korea. ykpark@khmc.or.kr
Clear cell chondrosarcoma is one of the extremely rare chondrosarcomas.
The pathogenesis and the molecular genetic events, which contribute to
the development of clear cell chondrosarcoma, are not well elucidated,
due in part to the lack of sufficient tumor tissue available. To
characterize the involvement of the p53 gene abnormality in this
disease, we analyzed expression and sequence alteration of p53 by
immunohistochemical analysis of the protein expression and quantitative
DNA/PCR and PCR-SSCP assays of the gene in 28 paraffin-embedded tissue
specimens. Immunohistochemical analysis demonstrated that 7 (25%) showed
patchy positive nuclear staining for p53 and 5 (18%) showed diffuse
positive nuclear staining patterns. Sixteen (57%) were negative for p53
immunostaining. Quantitative DNA/PCR analysis revealed that none of the
cases we studied showed significantly reduced levels of p53
amplification (<0.50), strongly suggesting an allelic deletion of the
p53 gene. In contrast, however, DNA/PCR-SSCP analysis failed to detect
any types of mutations resulting in amino acid substitution within exons
5-9 regions of the gene. Taken together, our data suggest that genetic
alteration of p53 is a relatively rare event in clear cell
chondrosarcomas but a substantial fraction of this type of tumors
carries abnormal overexpression of p53, which might result from an as
yet unidentified mechanism(s).
5
UI - 21372582
AU - Barile A; Caulo M; Zugaro L; Di Cesare E; Gallucci M; Masciocchi C
TI -
[Staging and re-staging of soft tissue sarcoma using MRI. Usefulness of
contrast media]
SO - Radiol Med (Torino) 2001 Jun;101(6):444-55
AD - Cattedra di Radiologia, Universita degli Studi, L'Aquila, Italy. antonio
barile@cc.univaq.it
PURPOSE: This study aims to evaluate the usefulness of paramagnetic
contrast medium (Gadolinium) in the staging and re-staging of soft
tissue tumors using Magnetic Resonance Imaging (MRI). MATERIAL AND
METHODS: Sixty patients affected by soft tissue sarcoma of different
histotype were retrospectively evaluated. Age ranged from 23 to 78
years. Data were obtained from the musculoskeletal tumor database of our
Department of Radiology and cases from the last 7 years (1993-1999)
entered this study. All the patients were submitted to ultrasound (US)
and to routine and enhanced MR examinations (SE T1-weighted and Fast-SE
T1 and T2-weighted before and after fat saturation pulse; GE T2-weighted
sequences). At least two different scan planes were obtained depending
on the major axis of the lesion. Once imaging information had been
obtained all the patients underwent surgery and histology. After
surgery, the mean follow-up period was 18 months. Paramagnetic contrast
medium (0,2mmol/Kg) was administered in all the MR follow-up
examinations. RESULTS: Lesions included adipose histology in 15 cases;
muscular histology in 12 cases; fibro-hystiocitic histology in 20 cases;
synovial histology in 8 cases; mixed histology in 5 cases. Excluding
lesions with mainly adipose content showing obvious high signal
intensity on T1-weighted sequences, the other lesions rarely presented
MR features specific to histological category also after contrast medium
administration. In all the cases MRI allowed an accurate definition of
the lesion boundaries to exclude or demonstrate neighbor region
invasion. In 11 cases, MRI was able to demonstrate the presence of a
recurrent tumor. In 2 out of 11 cases, recurrences were detected out
only after intravenous administration of Gadolinium, plain MRI having
failed to recognize them. DISCUSSION AND CONCLUSIONS: Despite the
absence of specific MRI signs of the different soft tissue tumor
histotypes also after Gadolinium administration, MRI remains the best
imaging technique to establish the exact morphology of the lesions and
to establish the invasion of the neighbor regions by the tumors. The
presence of regional spreading of the tumor has to be considered a sign
of malignancy. During the follow-up, the use of Gadolinium allows the MR
examinations to reach high sensitivity levels and makes it also possible
to recognize local recurrences of a very small size. Considering our
experience we recommend, whenever a follow-up of resected soft tissue
tumors is requested, to perform MRI examination before and after
intravenous Gadolinium administration.
6
UI - 21385121
AU - Koizumi K; Shimamoto Y; Azuma A; Wataya Y; Matsuda A; Sasaki T;
TI -
Fukushima M
Cloning and expression of uridine/cytidine kinase cDNA from human
fibrosarcoma cells.
SO - Int J Mol Med 2001 Sep;8(3):273-8
AD - The Second Cancer Laboratory, Taiho Pharmaceutical Co., Ltd., 1-27
Misugidai, Hanno-city, Saitama 357-8527, Japan.
Uridine/cytidine kinase which converts uridine and cytidine to their
corresponding monophosphates is a rate-limiting enzyme involved in the
salvage pathway of pyrimidine synthesis. We isolated cDNA encoding the
enzyme from human fibrosarcoma cells, then determined its nucleotide
sequence by the 5'-RACE method followed by confirmation employing the
human genome DNA library. The isolated uridine/cytidine kinase cDNA (UCK
cDNA) consisted of 786 nucleotides encoding 261 amino acids and was
found to have approximately 70% homology with mouse UCK cDNA. Northern
blot analysis of human leukemia RNAs with labeled UCK gene showed a
single band at 1.6 kb to be UCK mRNA, and southern blot analysis of the
UCK cDNA after digestion with BamHI, SacI and XbaI enzymes showed four
band signals, suggesting the UCK gene to have at least 4 exons. A
truncated form of UCK cDNA was expressed as the His-tag conjugated
protein in Escherichia coli. The expressed and purified protein
specifically converted uridine and cytidine to their corresponding
monophosphates and also phosphorylated antitumor nucleosides such as
5-fluorouridine, cyclopentenyl-cytosine and 3'-C-ethynylcytidine. The
present results suggest that our cloned human UCK cDNA encodes the
correct amino acid sequence for UCK protein, showing high intracellular
phosphorylation activity forward natural and synthetic pyrimidine
nucleosides.
7
UI - 21398001
AU - Nishiguchi S; Shiomi S; Ofuji S; Ishizu H; Iwata Y; Sasaki N; Minamitani
TI -
S; Ochi H
Leiomyosarcoma of the liver detected by high F-18 fluorodeoxyglucose
positron emission tomographic uptake.
SO - Clin Nucl Med 2001 Sep;26(9):798-9
AD - Third Department of Internal Medicine, Osaka City University Medical
School, 1-4-3 Asahimachi, Abeno-ku, Osaka 545-8585, Japan.
8
UI - 21434723
AU - Wirbel RJ; Schulte M; Mutschler WE
TI -
Surgical treatment of pelvic sarcomas: oncologic and functional outcome.
SO - Clin Orthop 2001 Sep;(390):190-205
AD - Department of Trauma Surgery, University of Saarland, Homburg, Germany.
The experiences in treating 93 consecutive patients (56 males, 37
females; mean age, 38.5 years; range, 4-69 years), including 76 patients
with primary malignant bone tumors and 17 patients with soft tissue
sarcomas involving the innominate bone, are reported. Oncologic and
functional results were investigated in relation to the tumor stage, to
the achieved surgical margin, and to the surgical procedure
(hemipelvectomy, internal hemipelvectomy and endoprosthetic replacement,
and continuity resection). The mean followup was 48 months (range, 8-222
months). The 5-year survival was 86% in patients with low-grade
malignant bone tumors, 42% in patients with high-grade malignant bone
tumors, and 25% in patients with high-grade soft tissue sarcomas.
Survival was influenced by the grade of malignancy, the tumor stage, and
the achieved surgical margins. Forty-six patients who survived were
examined an average of 36 months after primary surgery. Excellent and
good functional results were seen in 82% of patients who underwent
continuity resection and in 55.5% of patients who underwent partial or
total internal hemipelvectomy. All patients who survived hemipelvectomy
had poor functional results. Surgical treatment of pelvic sarcomas is an
extensive procedure with a considerable incidence of complications. It
requires the knowledge of different techniques of resection and
reconstruction of bone, joints, soft tissue, and intrapelvic organs.
9
UI - 21436366
AU - Challine D; Roudot-Thoraval F; Sarah T; Laperche L; Boisson B;
TI -
Mauberquez S; Dubernet F; Rigot P; Lefrere F; Mercier B; Brossard Y;
Rouet F; Girot R; Loiseau P; Girard D; Claquin J; Loty B; Lerable J;
Mariotti M; Pawlotsky JM; Lefrere JJ
Seroprevalence of human herpes virus 8 antibody in populations at high
or low risk of transfusion, graft, or sexual transmission of viruses.
SO - Transfusion 2001 Sep;41(9):1120-5
AD - Bacteriology and Virology Laboratory, Henri Mondor Hospital, Paris XII
University, Creteil, France.
BACKGROUND: The routes of transmission of human herpes virus 8 (HHV-8)
remain unclear. In particular, HHV-8 transmission by blood components
and organ transplantation is still debated and raises public health
issues. The objective of this study was to determine the prevalence of
anti-HHV-8 in selected populations of persons or patients with or
without risk factors for the transmission of viral infections, in order
to determine the routes of HHV-8 transmission. STUDY DESIGN AND METHODS:
A total of 1431 persons or patients at low or high risk of sexually,
blood-, or graft-transmitted viral infections were tested by means of a
standardized immunofluorescence serologic assay detecting anti-HHV-8.
RESULTS: The persons or patients could be classified into three distinct
groups according to anti-HHV-8 prevalence: a low prevalence group (0.0%
to 5.0%), including healthy blood donors, healthy pregnant women,
multiply transfused patients with thalassemia major, and IV drug users;
an intermediate prevalence group (5.0% to 20.0%), including organ
donors, kidney transplant recipients, and multiply transfused patients
with sickle cell disease; a high prevalence group (>20.0%), including
HIV-negative persons at high risk of sexually-transmitted viral
infections, and HIV-infected homosexual men and heterosexuals.
CONCLUSION: The sexual route appears to be the main route of HHV-8
transmission; bloodborne transmission of HHV-8, if it exists, is rare.
In contrast, organ transplantation recipients might be exposed to HHV-8
transmission by the transplanted organ, which raises the issue of
systematic screening of organ donors.
10
UI - 21445657
AU - Meis-Kindblom JM; Sjogren H; Kindblom LG; Peydro-Mellquist A; Roijer E;
TI -
Aman P; Stenman G
Cytogenetic and molecular genetic analyses of liposarcoma and its soft
tissue simulators: recognition of new variants and differential
diagnosis.
SO - Virchows Arch 2001 Aug;439(2):141-51
AD - Department of Pathology and the Musculoskeletal Tumor Center, Goteborg
University, Sahlgrenska University Hospital, Sweden.
jeanne.meis-kindblom@llcr.med.gu.se
Liposarcoma is one of the most common sarcomas of adults. Its
differential diagnosis and accurate subclassification are often
problematic; the latter is also important with regard to appropriate
treatment and prognosis. We studied a series of 23 liposarcomas that had
unusual or previously undescribed features and 10 liposarcoma simulators
and correlated the morphologic, cytogenetic, and molecular genetic
findings. We found that use of cytogenetic-molecular genetic techniques
aids in the distinction between myxoid-round cell liposarcoma and their
simulators, chondroid lipoma, myxoid spindle cell-pleomorphic lipoma,
cellular intramuscular myxoma, and myxofibrosarcoma. Poorly
differentiated forms of round cell liposarcoma lacking morphologic
evidence of lipogenesis can also be diagnosed using these techniques;
however, the techniques do not aid in distinguishing low-grade myxoid
from high-grade round cell liposarcomas. This study also shows that
retroperitoneal liposarcomas with myxoid liposarcoma-like zones are part
of the morphologic spectrum of well-differentiated-dedifferentiated
liposarcoma rather than true myxoid liposarcomas. Perhaps most
importantly, our results provide the first molecular genetic evidence
that true mixed liposarcomas (mixed well-differentiated and myxoid
liposarcoma) do indeed exist. They also unequivocally demonstrate the
existence of small, round cell variants of pleomorphic liposarcoma that
closely simulate myxoid-round cell liposarcoma.
11
UI - 21445658
AU - Nishio J; Iwasaki H; Ishiguro M; Ohjimi Y; Isayama T; Naito M; Kikuchi M
TI -
Identification of syt-ssx fusion transcripts in both epithelial and
spindle cell components of biphasic synovial sarcoma in small tissue
samples isolated by membrane-based laser microdissection.
SO - Virchows Arch 2001 Aug;439(2):152-7
AD - Department of Pathology, School of Medicine, Fukuoka University, Japan.
In order to confirm the presence of SYT-SSX fusion gene in epithelial
and spindle cell components of synovial sarcoma, we performed a nested
reverse transcriptase-polymerase chain reaction (RT-PCR) using microbeam
microdissection of membrane-mounted native tissue (MOMeNT) technique
applied on formalin-fixed, paraffin-embedded tumor specimens from two
biphasic synovial sarcomas and a control tissue of adamantinoma. Small
targeted portions of either an epithelial or spindle cell component of
the tumor tissue were microdissected together with the supporter
membrane, by using an ultraviolet (337-nm) pulsed laser microbeam
coupled into a robot-stage microscope with infinity optics. The SYT-SSX
fusion transcript was detected in epithelial and spindle cell components
of both biphasic synovial sarcomas, but not in the control tissue.
Southern blot analysis also confirmed that the detected messages were
derived from the SYT-SSX fusion gene. In conclusion, the microbeam
MOMeNT is a useful method for isolating selected small portions from
tissue sections. The SYT-SSX fusion gene is present in both cellular
components of biphasic synovial sarcoma and is involved in oncogenesis
of the synovial sarcoma rather than in morphologic epithelial
differentiation. Therefore, in spite of the variable proportions of each
component, our results confirm that the synovial sarcoma is of
monoclonal origin.
12
UI - 21439146
AU - Li WW; Takahashi N; Jhanwar S; Cordon-Cardo C; Elisseyeff Y; Jimeno J;
TI -
Faircloth G; Bertino JR
Sensitivity of soft tissue sarcoma cell lines to chemotherapeutic
agents: identification of ecteinascidin-743 as a potent cytotoxic agent.
SO - Clin Cancer Res 2001 Sep;7(9):2908-11
AD - Laboratories of Molecular Pharmacology, Memorial Sloan-Kettering Cancer
Center, New York, New York 10021, USA.
The cytotoxic effects of ecteinascidin-743(ET-743), a novel marine
natural product, were evaluated and compared with that of clinically
used anticancer agents methotrexate, doxorubicin, etoposide, and
paclitaxel in eight human soft tissue sarcoma (STS) cell lines. HT-1080,
a fibrosarcoma cell line, and HS-42, a malignant mesodermal cell line,
were the most sensitive of the cell lines to methotrexate, doxorubicin,
etoposide, and paclitaxel. Other cell lines (IC50s) varied considerably
and were more resistant to these agents. ET-743 was more potent than any
of these agents, with IC50s in the pM range in all of the cell lines.
Cytotoxicity of ET-743 was dose- and time-related (4-72 h exposure).
Cytotoxic concentrations of ET-743 produced a S/G2 block in all of the
cell lines tested. Three colon adenocarcinoma cell lines, HCT-8, HT-29,
and HCT-116, and one breast cancer cell line, MCF-7, were 1-2 logs less
sensitive to ET-743 than the STS cell lines. Cell lines were also
characterized as to expression of oncogenes and tumor suppressor genes
to attempt to correlate sensitivity of these cell lines to ET-743 and
other chemotherapeutic agents. All of the cell lines except M8805, a
malignant fibrous histiocytoma cell line, had mutations in p53 and/or
overexpressed the MDM2 protein. Only HS-18, a liposarcoma cell line,
lacked expression of the retinoblastoma protein. None of the cell lines
had detectable expression of P-glycoprotein as measured by
immunohistochemistry. ET-743 is an extremely potent cytotoxic agent
against human STS cell lines and is being evaluated as an antitumor
agent in this disease.
13
UI - 21441119
AU - Baillargeon J; Deng JH; Hettler E; Harrison C; Grady JJ; Korte LG;
TI -
Alexander J; Montalvo E; Jenson HB; Gao SJ
Seroprevalence of Kaposi's sarcoma-associated herpesvirus infection
among blood donors from Texas.
SO - Ann Epidemiol 2001 Oct;11(7):512-8
AD - Department of Pediatrics, The University of Texas Health Science Center,
7703 Floyd Curl Drive, San Antonio, TX 78229-3900, USA.
PURPOSE: Kaposi's sarcoma-associated herpesvirus (KSHV), a
gammaherpesvirus recently discovered among AIDS patients with Kaposi's
sarcoma, is a potential candidate for screening in blood and plasma
donors. While a number of studies have assessed KSHV infection among
U.S. blood donors, larger-scale population-based studies would be
necessary to develop more refined estimates of the magnitude and
variation of KSHV infection across different geographic regions of the
U.S. blood supply. The goal of the present study, therefore, was to
determine the seroprevalence of KSHV infection and to assess demographic
correlates of KSHV infection among south Texas blood donors. METHODS:
KSHV infection was determined using specific serologic assays that
measure antibodies to KSHV latent and lytic antigens. RESULTS: The
overall seroprevalence of KSHV in Texas blood donors (15.0%) is
substantially higher than previously reported among blood donor and
general population samples in the United States. This high rate of KSHV
infection persisted across most of the sociodemographic subgroups under
study but was particularly elevated among participants with less than a
high school education. The infection rate also increased linearly with
age. CONCLUSIONS: The elevated infection rate reported in the present
study suggests that screening methods to detect KSHV infection in blood
donors should be considered. In view of the etiologic role of KSHV for
several malignancies, it would be important for future studies to
directly assess the risk of KSHV transmission via blood transfusion.
14
UI - 21455251
AU - Gaumann A; Tews DS; Mayer E; Dahm M; Petrow PK; Otto M; James C;
TI -
Kriegsmann J
Expression of apoptosis-related proteins, p53, and DNA fragmentation in
sarcomas of the pulmonary artery.
SO - Cancer 2001 Sep 1;92(5):1237-44
AD - Institute of Pathology, University of Mainz, Mainz, Germany.
a.gaumann@Kerckhoff.mpg.de
BACKGROUND: Apoptosis is a common feature in a variety of pathologic
conditions. Induction of apoptosis through apoptotic stimuli such as,
chemotherapy or radiation, presents new insights into tumor biology and
therapy. In particular, members of the Bcl-2 family as well as the Fas
system are known to be involved in the regulation of apoptosis in
different tumor entities. METHODS: In the current study, the expression
of the apoptosis-related molecules p53, Bax, Bcl-2, Fas (CD95),
Fas-Ligand and perforin was examined in 7 patients with a sarcoma of the
pulmonary artery. Furthermore, the TUNEL-method for the detection of
apoptotic cells was applied as well as sequencing of the p53 gene.
RESULTS: In the TUNEL assay, approximately 10% of the sarcoma cells
displayed DNA fragmentation. In addition, Bax was expressed in tumor
cells. Accumulation of p53 was evident in 4 of 7 patients (pAB 240
antibody), and 2 of them were positive for the pAB 1801 antibody. Only 1
case had a point mutation in Exon 5 of the p53 sequence. A few tumor
cells showed a double labeling of Bax and p53. Bcl-2 could be detected
only in tumor-associated lymphocytes. Finally, several lymphocytes could
be stained with perforin, but none of the specimens showed a reactivity
for Fas or Fas-Ligand. CONCLUSION: The expression of Bax indicated a
possible role of this molecule in programmed cell death in pulmonary
sarcomas. The limited coexpression of Bax and p53 suggested that
induction of Bax can occur independently of p53. The detection of
perforin in lymphocytes suggested a possible role for this molecule in
apoptosis of the sarcoma cells. In contrast, the Fas system did not seem
to play an essential role in sarcomas of the great vessels. Copyright
2001 American Cancer Society.
15
UI - 21455264
AU - Patel SR; Jenkins J; Papadopolous N; Burgess MA; Plager C; Gutterman J;
TI -
Benjamin RS
Pilot study of vitaxin--an angiogenesis inhibitor-in patients with
advanced leiomyosarcomas.
SO - Cancer 2001 Sep 1;92(5):1347-8
AD - Department of Sarcoma Medical Oncology, The University of Texas M. D.
Anderson Cancer Center, Houston, Texas 77030, USA. spatel@mdanderson.org
16
UI - 21284295
AU - Vanel D; De Paolis M; Monti C; Mercuri M; Picci P
TI -
Radiological features of 24 periosteal chondrosarcomas.
SO - Skeletal Radiol 2001 Apr;30(4):208-12
AD - Laboratorio di Ricerca Oncologica, Istituto Rizzoli, Bologna, Italy.
OBJECTIVE: To report the imaging findings of 24 periosteal
chondrosarcomas diagnosed, staged, treated and followed in a single
institution, to analyze and define their pattern, and discuss their
practical consequences. DESIGN AND PATIENTS: Plain films, 16 CT
examinations and four MRI examinations were reviewed, and compared with
the histological evaluation. RESULTS: There were 20 men and four women,
aged from 17 to 65 years. Twelve lesions involved the distal femoral
metaphyses (8 posteriorly), five the proximal humerus, two the proximal
metaphyses of the femur and two of the tibia, two the humeral shafts and
one the iliac wing. Size varied from 4 to 11 cm. The cortex was always
involved (thick, 15; thin, 13). Typical cartilaginous calcifications and
cartilaginous lobules were very frequent. Radial thick periosteal bone
formations (n = 6) indicated calcifications between the lobules of
cartilage. Medullary involvement was rare (n = 2). All patients are
alive and free of disease. CONCLUSIONS: Recognizing periosteal
chondrosarcoma is of paramount importance because the prognosis is
excellent after adequate local surgery alone. The patterns of other
surface tumors of bone are usually different.
17
UI - 21453547
AU - Loos BM; Wieneke JA; Thompson LD
TI -
Laryngeal angiosarcoma: a clinicopathologic study of five cases with a
review of the literature.
SO - Laryngoscope 2001 Jul;111(7):1197-202
AD - Department of Otolaryngology-Head and Neck Surgery, Georgetown
University Medical Center, Washington, DC, USA.
OBJECTIVE: Primary laryngeal angiosarcoma (LA) is rare without a
reported series evaluating these tumors. STUDY DESIGN/METHODS: Five
patients with LA were retrospectively retrieved from the
Otorhinolaryngic Registry of the Armed Forces Institute of Pathology.
RESULTS: Three men and 2 women, aged 29 to 71 years, presented with
hoarseness (n = 4) and hemoptysis (n = 1). Two patients had previous
neck radiation. The tumors involved the supraglottis (n = 4) with a mean
size of 3.1 cm. Histologically, all tumors had anastomosing vascular
channels lined by remarkably atypical endothelial cells protruding into
the lumen, frequent atypical mitotic figures, and hemorrhage. All cases
tested (n = 4) demonstrated immunoreactivity with antibodies to Factor
VIII-RA and CD34. All patients had surgery followed by postoperative
radiation (n = 3 patients). Three patients died with disease (mean, 17
mo), whereas one patient is alive with no evidence of disease at 18
years. CONCLUSIONS: LA is a rare tumor, frequently associated with
previous radiation, usually involving the supraglottis with
characteristic histomorphologic and immunophenotypic features. LA has a
poor prognosis, making appropriate separation from other conditions
important.
18
UI - 21390914
AU - Zhang Q; Yang Z; Bu G
TI -
[Surgery of advanced clival tumors]
SO - Zhonghua Er Bi Yan Hou Ke Za Zhi 1998 Feb;33(1):21-3
AD - Third Teaching Hospital, Bethune University of Medical Sciences,
Changchun 130031.
OBJECTIVE: To investigate the effects of surgical procedures on tumor 27
patients with advanced clival tumors during the last 9 years. METHODS:
Four different kinds of surgical approaches were selected and used
according to preoperative clinical and radiographic findings, and the
complications and the long-term survival rates were evaluated after the
surgical procedures. RESULTS: All of the 8 patients with benign clival
tumors (6 cases with chordomas and 2 with meningiomas) had over 2-8
years survival and no severe complications after the surgical procedures
and adjuvant radiotherapy. Fourteen of the 19 patients with malignant
tumors had over 2-6 years survival (a survival rate of 73.7%) after the
surgical procedures, the adjuvant chemotherapy and radiotherapy.
CONCLUSION: Surgery of the clivus appears to be worthwhile procedure for
improving outcome of the patients with advanced clival tumors.
19
UI - 21418712
AU - Cunha A; Costa SC; Lima CS; Ortega M; Costa FF
TI -
Low incidence of human herpesvirus 8 in bone marrow samples from
Brazilian patients with multiple myeloma.
SO - Acta Haematol 2001;105(4):247-8
AD - Department of Internal Medicine and Department of Clinical Pathology,
University of Campinas (UNICAMP), CEP Campinas, SP, 13083-970, Brazil.
20
UI - 21423990
AU - Katz RA; DiCandeloro P; Kukolj G; Skalka AM
TI -
Role of DNA end distortion in catalysis by avian sarcoma virus
integrase.
SO - J Biol Chem 2001 Sep 7;276(36):34213-20
AD - Fox Chase Cancer Center, Institute for Cancer Research, Philadelphia,
Pennsylvania 19111, USA. R_Katz@fccc.edu
Retroviral integrase (IN) recognizes linear viral DNA ends and
introduces nicks adjacent to a highly conserved CA dinucleotide usually
located two base pairs from the 3'-ends of viral DNA (the "processing"
reaction). In a second step, the same IN active site catalyzes the
insertion of these ends into host DNA (the "joining" reaction). Both DNA
sequence and DNA structure contribute to specific recognition of viral
DNA ends by IN. Here we used potassium permanganate modification to show
that the avian sarcoma virus IN catalytic domain is able to distort
viral DNA ends in vitro. This distortion activity is consistent with
both unpairing and unstacking of the three terminal base pairs,
including the processing site adjacent to the conserved CA. Furthermore,
the introduction of mismatch mutations that destabilize the viral DNA
ends were found to stimulate the IN processing reaction as well as
IN-mediated distortion. End-distortion activity was also observed with
mutant or heterologous DNA substrates. However, further analyses showed
that using Mn(2+) as a cofactor, processing site specificity of these
substrates was also maintained. Our results support a model whereby
unpairing and unstacking of the terminal base pairs is a required step
in the processing reaction. Furthermore, these results are consistent
with our previous observations indicating that unpairing of target DNA
promotes the joining reaction.
21
UI - 21424528
AU - Groves AK; Cotter MA; Subramanian C; Robertson ES
TI -
The latency-associated nuclear antigen encoded by Kaposi's
sarcoma-associated herpesvirus activates two major essential
Epstein-Barr virus latent promoters.
SO - J Virol 2001 Oct;75(19):9446-57
AD - Medical Scientist Training Program, Cell and Molecular Biology Graduate
Program, Department of Microbiology and Immunology, University of
Michigan Medical Center, Ann Arbor, Michigan 48109-0934, USA.
The latency-associated nuclear antigen (LANA) encoded by the Kaposi's
sarcoma-associated herpesvirus (KSHV) is expressed in the majority of
KSHV-infected cells and in cells coinfected with Epstein-Barr virus
(EBV). In coinfected body cavity-based lymphomas (BCBLs), EBV latent
membrane protein 1 (LMP1), which is essential for B-lymphocyte
transformation, is expressed. EBNA2 upregulates the expression of LMP1
and other cellular genes through specific interactions with cellular
transcription factors tethering EBNA2 to its responsive promoters. In
coinfected BCBL cells, EBNA2 is not detected but LANA, which is
constitutively expressed, contains motifs suggestive of potential
transcriptional activity. Additionally, recent studies have shown that
LANA is capable of activating cellular promoters. Therefore, we
investigated whether LANA can affect transcription from two major EBV
latent promoters. In this study, we demonstrated that LANA can
efficiently transactivate both the LMP1 and C promoters in the human
B-cell line BJAB as well as in the human embryonic kidney 293 cell line.
Moreover, we demonstrated that specific domains of LANA containing the
putative leucine zipper and the glutamic acid-rich region are highly
effective in upregulating these viral promoters, while the
amino-terminal region (435 amino acids) exhibited little or no
transactivation activity in our assays. We also specifically tested
truncations of the LMP1 promoter element and showed that the -204 to +40
region had increased levels of activation compared with a larger region,
-512 to +40, which contains two recombination signal-binding protein J
kappa binding sites. The smaller, -204 to +40 promoter region contains
specific binding sites for the Ets family transcription factor PU.1,
transcription activating factor/cyclic AMP response element, and Sp1,
all of which are known to function as activators of transcription. Our
data therefore suggest a potential role for LANA in regulation of the
major EBV latent promoters in KSHV- and EBV-coinfected cells.
Furthermore, LANA may be able to activate transcription of viral and
cellular promoters in the absence of EBNA2, potentially through
association with transcription factors bound to their cognate sequences
within the -204 to +40 region. This regulation of viral gene expression
is critical for persistence of these DNA tumor viruses and most likely
involved in mediating the oncogenic process in these coinfected cells.
22
UI - 21424534
AU - Hwang S; Gwack Y; Byun H; Lim C; Choe J
TI -
The Kaposi's sarcoma-associated herpesvirus K8 protein interacts with
CREB-binding protein (CBP) and represses CBP-mediated transcription.
SO - J Virol 2001 Oct;75(19):9509-16
AD - Department of Biological Sciences, Korea Advanced Institute of Science
and Technology, Daejeon 305-701, Korea.
Kaposi's sarcoma-associated herpesvirus (KSHV) open reading frame K8
encodes a basic region-leucine zipper protein of 237 amino acids that
homodimerizes with its bZIP domain. KSHV K8 shows significant homology
to the Epstein-Barr virus (EBV) immediate-early protein Zta, a key
regulator in the reactivation and replication of EBV. In this study, we
report that K8, like its homolog EBV Zta, interacts with cellular
CREB-binding protein (CBP) in vivo and in vitro. This interaction
requires the C/H3 domain of CBP and the basic region of K8. K8 represses
CBP-mediated transcription by competing with limited amounts of cellular
CBP, exemplified by the reduced expression from the AP-1 and human
immunodeficiency virus long terminal repeat promoters.
23
UI - 21424854
AU - Graham D
TI -
Management of soft tissue sarcoma.
SO - Nurs Clin North Am 2001 Sep;36(3):553-65, xi
AD - Advanced Practice, Education and Research, Memorial Sloan-Kettering
Cancer Center, New York, New York 10021, USA. grahamd@mskcc.org
Sarcomas are rare and unusual neoplasms that can be found in any tissue
and can have life-threatening outcomes. Caring for these patients can be
a great challenge and requires a multidisciplinary team.
24
UI - 21427147
AU - Agus V; Tamborini E; Mezzelani A; Pierotti MA; Pilotti S
TI -
Re: A novel fusion gene, SYT-SSX4, in synovial sarcoma.
SO - J Natl Cancer Inst 2001 Sep 5;93(17):1347-9
25
UI - 21433392
AU - Zietz C; Rumpler U; Sturzl M; Lohrs U
TI -
Inverse relation of Fas-ligand and tumor-infiltrating lymphocytes in
angiosarcoma: indications of apoptotic tumor counterattack.
SO - Am J Pathol 2001 Sep;159(3):963-70
AD - Institute of Pathology, Ludwig Maximilians University, Munich, Germany.
christian.zietz@lrz.uni-muenchen.de
Fas and Fas-L regulate immune responses through the induction of cell
death. Fas-L is commonly expressed in activated immune cells and in the
endothelium. In the latter it contributes to the inhibition of
transvascular cell migration by the induction of apoptosis in
Fas-bearing lymphocytes. Here we investigated whether the Fas/Fas-L
system may regulate lymphocyte invasion into angiosarcomas. Fas and
Fas-L expression was quantitatively determined in different grade
angiosarcomas (n = 40) and related to the number of extravasated
tumor-infiltrating lymphocytes (TILs). Fas expression was detected in <
50% of the cases. In positive tumors both the number of Fas-positive
cells and the staining intensity were highly variable and did not
correlate with the number of TILs, the mean time of survival, and the
histopathological tumor grade. By contrast, Fas-L expression was
detected in >70% of the cases and the relative numbers of Fas-L-positive
cells correlated inversely with the numbers of CD3- and CD8-positive
TILs (P < or = 0.004). The survival times of patients with high
Fas-L-expressing angiosarcomas were significantly reduced as compared to
patients with low Fas-L-expressing tumors. Our results show that
angiosarcomas with low Fas-L expression are characterized by numerous
TILs, whereas sarcomas with high Fas-L expression show significantly
reduced numbers of TILs. These results suggest that the Fas/Fas-L system
may repress TIL invasion into angiosarcoma and by this may contribute to
the evasion of the anti-tumor immune surveillance of angiosarcoma in the
course of an apoptotic tumor counterattack mechanism.
26
UI - 21443303
AU - Worley BS; van den Broeke LT; Goletz TJ; Pendleton CD; Daschbach EM;
TI -
Thomas EK; Marincola FM; Helman LJ; Berzofsky JA
Antigenicity of fusion proteins from sarcoma-associated chromosomal
translocations.
SO - Cancer Res 2001 Sep 15;61(18):6868-75
AD - Metabolism Branch, National Cancer Institute, Bethesda, Maryland
20892-1578, USA.
Synovial sarcoma (SS), clear cell sarcoma (CCS), and desmoplastic small
round cell tumor (DSRCT) are soft-tissue malignancies occurring
primarily in adolescents and young adults. These tumors contain specific
chromosomal translocations that fuse the 5' region of one gene with the
3' region of another, resulting in the formation of characteristic
fusion proteins. These translocations are unique to tumor cells and may
be required for persistence, thereby serving as targets for
immunotherapy. It was hypothesized that the fusion breakpoint sequences
associated with SS, CCS, and DSRCT can serve as tumor-specific
neoantigens. To test this, peptides corresponding to the fusion
breakpoints were designed and assessed for ability to bind to various
class I HLA molecules. Two peptides derived from the SS breakpoint
specifically bind the HLA-B7 antigen, and a 10-amino acid minimal
epitope was identified for this interaction. Specific binding of a SS
peptide and a CCS peptide to HLA-B27 molecule was also observed.
Finally, a peptide designed from the DSRCT breakpoint specifically binds
the HLA-A3 molecule, and a 9-amino acid optimal epitope was identified
for this interaction. The physiological/immunological relevance of these
peptide/MHC interactions was demonstrated by the induction of
SS-specific CTLs from normal donor lymphocytes using in vitro
stimulation with autologous, peptide-pulsed dendritic cells and by the
ability of these CTLs to lyse human SS tumor cells endogenously
expressing the full-length fusion protein. These results suggest that
sequences in the fusion region of sarcoma-associated chimeras can bind
class I HLA molecules and serve as neoantigens. These may be useful for
the development of novel immunotherapies for sarcoma patients with
appropriate HLA molecules and tumors bearing these translocations.
27
UI - 21453163
AU - Callister MD; Ballo MT; Pisters PW; Patel SR; Feig BW; Pollock RE;
TI -
Benjamin RS; Zagars GK
Epithelioid sarcoma: results of conservative surgery and radiotherapy.
SO - Int J Radiat Oncol Biol Phys 2001 Oct 1;51(2):384-91
AD - Department of Radiation Oncology, University of Texas M. D. Anderson
Cancer Center, Houston, Texas 77030, USA.
PURPOSE: To determine the outcome and prognostic factors for patients
with localized epithelioid sarcoma treated with conservative surgery and
radiotherapy (RT). METHODS AND MATERIALS: The medical records of 24
patients with nonmetastatic epithelioid sarcoma treated with
conservative surgery and RT were reviewed. Preoperative RT was given to
3 patients (median 46.4 Gy) and postoperative RT to 21 patients (median
64.5 Gy). A local (limb-sparing) surgical procedure was performed in all
patients. RESULTS: At a median follow-up of 131 months, 14 patients had
relapsed and 13 patients had died. The actuarial overall and
disease-free survival rate at 10 years was 50% and 37%, respectively.
Local, nodal, and metastatic failure occurred in 7, 4, and 10 patients,
respectively, yielding a 10-year actuarial local, nodal, and metastatic
control rate of 63%, 81%, and 56%, respectively. Univariate analysis
revealed that size < or =5 cm and extremity location were favorable
prognostic factors for overall, disease-free, and metastasis-free
survival. The actuarial 5-year overall, disease-free, and
metastasis-free survival rate was 79% vs. 25% (p = 0.002), 51% vs. 13%
(p = 0.03), and 79% vs. 13% (p <0.001), respectively, for lesion size <
or =5 vs. > 5 cm. The actuarial 5-year overall, disease-free, and
metastasis-free survival rate was 77% vs. 39% (p = 0.002), 56% vs. 0% (p
= 0.01), and 78% vs. 17% (p = 0.01), respectively, for extremity vs.
nonextremity location. Multivariate analysis of the factors correlating
with the overall, disease-free, and metastasis-free survival confirmed
the favorable prognostic significance of small lesion size. The
prognostic significance of extremity location on univariate analysis was
explained by an imbalance in the mean tumor sizes. CONCLUSIONS:
Epithelioid sarcoma is an aggressive soft-tissue sarcoma, with high
rates of local and distant relapse. Local control with conservative
surgery and RT compares favorably to published surgical series. The poor
outcome for tumors > or =5 cm in size emphasizes the need for effective
systemic therapy.
28
UI - 21453164
AU - Noel G; Habrand JL; Mammar H; Pontvert D; Haie-Meder C; Hasboun D;
TI -
Moisson P; Ferrand R; Beaudre A; Boisserie G; Gaboriaud G; Mazal A;
Kerody K; Schlienger M; Mazeron JJ
Combination of photon and proton radiation therapy for chordomas and
chondrosarcomas of the skull base: the Centre de Protontherapie D'Orsay
experience.
SO - Int J Radiat Oncol Biol Phys 2001 Oct 1;51(2):392-8
AD - Centre de Protontherapie d'Orsay, Orsay, France. noel@ipno.in2p3.fr
PURPOSE: Prospective analysis of local tumor control, survival, and
treatment complications in 44 consecutive patients treated with
fractionated photon and proton radiation for a chordoma or
chondrosarcoma of the skull base. METHODS AND MATERIALS : Between
years (14-85) were treated using a 201-MeV proton beam at the Centre de
Protontherapie d'Orsay, 34 for a chordoma and 11 for a chondrosarcoma.
Irradiation combined high-energy photons and protons. Photons
represented two-thirds of the total dose and protons one-third. The
median total dose delivered within the gross tumor volume was 67 cobalt
Gray equivalent (CGE) (range: 60-70). RESULTS: With a mean follow-up of
30.5 months (range: 2-56), the 3-year local control rates for chordomas
and chondrosarcomas were 83.1% and 90%, respectively, and 3-year overall
survival rates were 91% and 90%, respectively. Eight patients (18%)
failed locally (7 within the clinical tumor volume and 1 unknown). Four
patients died of tumor and 2 others of intercurrent disease. In
univariate ana