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Abramson Cancer CenterNCI CANCERLIT® Search: Soft Tissue Sarcoma/Rhabdomyosarcoma - September 2002
National Cancer Institute®
Last Modified: September 1, 2002
- High-dose chemotherapy in adult soft tissue sarcoma.
- High-dose chemotherapy in soft tissue sarcoma in children.
- Soft-tissue sarcoma in childhood and adolescence.
- Systemic therapy for advanced soft-tissue sarcomas.
- Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysis.
- [Enhanced expression of matrix metalloproteinase-9 (MMP-9) under hypoxic and nutrient-deprived conditions]
- Primary intimal sarcoma of the aorta: role of transesophageal echocardiography.
- Differential Ki67 and bcl-2 immunoexpression in solid-glandular and spindle cell components of biphasic synovial sarcoma: a double
- Clinical features and outcome of initially unresected nonmetastatic pediatric nonrhabdomyosarcoma soft tissue sarcoma.
- Expression of multidrug resistance genes MVP, MDR1, and MRP1 determined sequentially before, during, and after hyperthermic isolated limb
- High-dose ifosfamide for soft tissue sarcomas: set the scene, or senescence?
- Phase I multicenter study of combined high-dose ifosfamide and doxorubicin in the treatment of advanced sarcomas. Swiss Group for
- Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical study with special emphasis on its distinction from ordinary
- Myofibrosarcoma of the bone: a clinicopathologic study.
- The regulatory region of Prague C v-Src inhibits the activity of the Schmidt-Ruppin A v-Src kinase domain.
- Color Doppler ultrasonography in the differentiation of uterine sarcomas from uterine leiomyomas.
- [Sarcomas]
- Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and peripheral nerve tumors.
- Alveolar soft part sarcoma. a report of 15 cases.
- ErbB-4 expression in limb soft-tissue sarcoma: correlation with the results of neoadjuvant chemotherapy.
- Detection of SYT-SSX fusion transcripts in paraffin-embedded tissues of synovial sarcoma by reverse transcription-polymerase chain reaction.
- Human herpesvirus 8 reactivation and human immunodeficiency virus type 1 gp120.
- The SYT-SSX1 fusion type of synovial sarcoma is associated with increased expression of cyclin A and D1. A link between t(X;18)(p11.2;
- CT differentiation of large exophytic renal angiomyolipomas and perirenal liposarcomas.
- Synovial sarcoma in pediatric patients.
- Low frequency of somatic mutations in the FH/multiple cutaneous leiomyomatosis gene in sporadic leiomyosarcomas and uterine leiomyomas.
- Contrast of response to dacarbazine, mitomycin, doxorubicin, and cisplatin (DMAP) plus GM-CSF between patients with advanced malignant
- Multidisciplinary management of soft-tissue sarcomas.
- GIST 1, chemotherapy 0, with a brand new hitter up next.
- Surgical management of primary breast sarcoma.
- Human herpesvirus type 8 seroprevalence among patients in immunosuppression state.
- Rhabdomyosarcoma of the head and neck in children.
- Interview with professor Bin Kroon.
- Malignant mixed mesodermal tumours: biology and clinical aspects.
- Preoperative chemoradiation treatment strategies for localized sarcoma.
- High bad and bcl-xL gene expression and combined bad, bcl-xL, bax and bcl-2 mRNA levels: molecular predictors for survival of stage 2 soft
- Mechanisms of actio of poly-plat, a novel platinum antineoplastic agent.
- Retrospective analysis of HHV-8 viremia and cellular viral load in HIV-seropositive patients receiving interleukin 2 in combination with
- High-resolution structure of the catalytic domain of avian sarcoma virus integrase.
- Phase I study of twelve-day prolonged infusion of high-dose ifosfamide and doxorubicin as first-line chemotherapy in adult patients with
- Novel mdm2 splice variants identified in pediatric rhabdomyosarcoma tumors and cell lines.
- No association between Langerhans cell histiocytosis and human herpes virus 8.
- Analysis of gene expression in Egr-1 transfected human fibrosarcoma cells.
- Radiographic response to neoadjuvant chemotherapy is a predictor of local control and survival in soft tissue sarcomas.
- Telomerase activity and human telomerase reverse transcriptase mRNA expression are correlated with clinical aggressiveness in soft tissue
- Inhibition of the transcription factor nuclear factor-kappa B by adenoviral-mediated expression of I kappa B alpha M results in tumor
- Mutation analysis of K-ras-2 in liver angiosarcoma and adjacent nonneoplastic liver tissue from patients occupationally exposed to vinyl
- Imatinib GIST keeps finding new indications: successful treatment of dermatofibrosarcoma protuberans by targeted inhibition of the
- MyoD1 and myogenin expression in human neoplasia: a review and update.
- Transcription mapping and expression patterns of genes in the major immediate-early region of Kaposi's sarcoma-associated herpesvirus.
- Leiomyosarcoma of the head and neck: a clinicopathological study.
- [Clinicopathological and immunohistochemical characteristics of stromal sarcoma of the duodenum]
- Prognostic significance of grading and staging systems using MIB-1 score in adult patients with soft tissue sarcoma of the extremities and trunk.
- Surgical management of soft tissue sarcomas of the hand and foot.
- CD44 stimulation by fragmented hyaluronic acid induces upregulation and tyrosine phosphorylation of c-Met receptor protein in human
- Analysis of the progression of fibroepithelial tumours of the breast by PCR-based clonality assay.
- Significance of loss of heterozygosity of the RB1 gene during tumour progression in well-differentiated liposarcomas.
- [CT manifestations of primary hepatic sarcoma]
- Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions.
- Dynamic magnetic resonance imaging in soft tissue tumors -- assessment of the diagnostic value of tumor enhancement rate indices.
- Technique and results of hyperthermic (41 degrees C) isolated lung perfusion with high-doses of cisplatin for the treatment of surgically
- Usefulness of Gd-DTPA contrast-enhanced dynamic MRI and serum determination of LDH and its isozymes in the differential diagnosis of
- Tissue and serum CA125 expression in endometrial cancer.
- Outcome of primary soft tissue sarcoma of the knee and elbow.
- CD117 in soft tissue sarcomas.
- KIT activation in soft tissue sarcomas.
- Endometrial stromal sarcomas with unusual histologic features: a report of 24 primary and metastatic tumors emphasizing fibroblastic and smooth
- Anti-p110 autoantibodies identify a subtype of "seronegative" myasthenia gravis with prominent oculobulbar involvement.
- Interleukin (IL)-6, IL-8 and IL-10 in serum and CSF of Trypanosoma brucei gambiense sleeping sickness patients before and after treatment.
- Monophasic and biphasic synovial sarcomas abundantly express cancer/testis antigen NY-ESO-1 but not MAGE-A1 or CT7.
- Uterine sarcomas--the biggest challenge?
- Retrospective study of management of uterine sarcomas at Oxford 1990-1998: role of adjuvant treatment.
- Lack of ErbB-2 oncogene product overexpression in soft tissue sarcomas.
- Angiosarcoma treated with radiotherapy: impact of tumor type and size on outcome.
- Phase II trial of topotecan by continuous infusion in patients with advanced soft tissue sarcomas, a SWOG study. Southwest Oncology Group.
- Infrequent p53 gene mutations and lack of p53 protein expression in clear cell sarcoma of the kidney: immunohistochemical study and mutation
- Cancer-testis antigen expression in uterine malignancies with an emphasis on carcinosarcomas and papillary serous carcinomas.
- Epithelioid sarcoma: clinical, MR imaging and pathologic findings.
Table of Contents
1
UI - 11856592
AU - Reichardt P
TI -
High-dose chemotherapy in adult soft tissue sarcoma.
SO - Crit Rev Oncol Hematol 2002 Feb;41(2):157-67
AD - Medizinische Klinik m. S. Hamatologie, Onkologie und Tumorimmunologie,
Robert-Rossle-Klinik, Universitatsklinikum Charite der
Humboldt-Universitat zu Berlin, 13122, Berlin, Germany.
reichardt@rrk-berlin.de
Soft tissue sarcomas represent a rare and heterogeneous disease. Only
few drugs have been identified to be active, with doxorubicin,
epirubicin and ifosfamide being the only agents with response rates
above 20%. Combination chemotherapy results in higher response rates,
however, superiority against single agent chemotherapy in terms of
survival has not been established yet. Since a dose-response
relationship is suggested for the anthracyclines and especially
ifosfamide, high-dose or dose-intensive chemotherapy with bone marrow or
stem cell support has been evaluated by several investigators. The
studies are usually small, and included a very heterogeneous group of
patients. Randomized trials have not been done, so that definite
conclusions cannot be drawn to date. High-dose chemotherapy in soft
tissue sarcoma has to be considered highly investigational and should
not be performed outside clinical trials. Future studies should be
focused on the development of active regimens, resulting in complete
remission rates, that can be expected to translate into longer survival.
Finally, well designed and appropriately powered randomized trials,
using established prognostic and predictive factors, should be carried
out, preferably in younger patients and in the context of a potentially
curative multimodality approach.
2
UI - 11856594
AU - Atra A; Pinkerton R
TI -
High-dose chemotherapy in soft tissue sarcoma in children.
SO - Crit Rev Oncol Hematol 2002 Feb;41(2):191-6
AD - Department of Paediatric Oncology, The Royal Marsden Hospital NHS
Trust/Institute of Cancer Research, Downs Road, Sutton, SM2 5PT, Surrey,
UK.
Soft tissue sarcomas (STS) are highly malignant tumours that constitute
5-6% of all malignant childhood neoplasms. Of these, rhabdomyosarcoma
(RMS) is the most common in children, and has a characteristic two-peak
age incidence, 2-5 and 15-19 years. Most children with RMS are cured
with conventional chemotherapy and local therapy (surgery with or
without radiotherapy). Children with metastatic disease at presentation,
particularly those older than 10 years or with bone marrow or bone
involvement have a much poorer outcome. In this subgroup, high-dose
therapy with stem cell rescue has been studied over the last two
decades. Various single or multiagent chemotherapy regimens with or
without radiotherapy and autologous stem cell rescue have been used as
consolidation treatment with little success. Recent trials using
sequential high-dose chemotherapy in the early phase of treatment have
proved to be feasible, but the beneficial effect has to be confirmed.
The role of purging remains unclear. Collaboration between different
international groups is urgently required, in an attempt to improve the
poor outcome of children with high risk STS.
3
UI - 12044238
AU - Raney RB
TI -
Soft-tissue sarcoma in childhood and adolescence.
SO - Curr Oncol Rep 2002 Jul;4(4):291-8
AD - Division of Pediatrics, University of Texas, M.D. Anderson Cancer
Center, 1515 Holcombe Boulevard, Box 87, Houston 77030, USA.
Braney@mdanderson.org
This review summarizes and comments on the major articles that have been
published in English concerning pediatric soft-tissue sarcomas in the
past 2 years. Studies of rhabdomyosarcoma and undifferentiated sarcoma,
including late sequelae of treatment; nonrhabdomyosarcomatous
soft-tissue sarcoma; and the pathology of soft-tissue sarcomas are
included.
4
UI - 12044239
AU - Patel SR
TI -
Systemic therapy for advanced soft-tissue sarcomas.
SO - Curr Oncol Rep 2002 Jul;4(4):299-304
AD - Department of Sarcoma Medical Oncology, The University of Texas M.D.
Anderson Cancer Center, 1515 Holcombe Boulevard, Box 450, Houston 77030,
USA. spatel@mail.mdanderson.org
The field of oncology is experiencing a paradigm shift from
broad-spectrum cytotoxic therapies to more specific molecularly based
targeted therapies. The activity of imatinib mesylate in chronic
myelogenous leukemia and gastrointestinal stromal tumors (GIST) has
reinforced our faith in translational research and its potential impact
on the cure of cancer and improvement in quality of life for patients.
This breakthrough has been particularly exciting for the field of
sarcoma and for patients with advanced GIST, for whom no other effective
therapy was available. Unfortunately, as is becoming increasingly clear,
cancer is a very complex problem with multiple mechanisms and pathways
that function either independently or interdependently enabling cell
survival. We are therefore far away from having solved the problem.
Attempts at refining the currently available therapeutic armamentarium
to maximize the therapeutic index (dose intensification with growth
factor support) must continue in parallel with laboratory-based research
identifying critical targets to be inhibited or blocked. Identification
of new agents with some activity, such as gemcitabine and ecteinascidin
(ET-743), is also of paramount importance.
5
UI - 12075208
AU - Al-Khateeb T; Bataineh AB
TI -
Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a
retrospective analysis.
SO - Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002 May;93(5):580-5
AD - Jordan University of Science and Technology. Irbid, Jordan.
Khateeb@just.edu.jo
OBJECTIVE: The objective was to study the clinicopathologic features of
rhabdomyosarcoma (RMS) of the oral and maxillofacial region in
Jordanians. STUDY DESIGN: Data were collected from records of patients
treated between 1989 and 2000 at the Maxillofacial Unit of Jordan
University of Science and Technology. The main outcome measures were
age, gender, location, stage of disease, histopathologic type, treatment
received, follow-up period, and eventual outcome. RESULTS: Nine patients
with RMS aged 4 to 17 years were found, with a male to female ratio of
2:1. Six (67%) bony sites and 7 (78%) soft tissue sites were involved.
The extent of disease was locoregional in 8 (89%) cases, nodal in 3
(33%) cases, and distant metastatic in 2 (22%) cases. The Intergroup
Rhabdomyosarcoma Study classification of clinical groups was as follows:
2 (22%) cases in stage II, 5 (56%) cases in stage III, and 2 (22%) cases
in stage IV. The histopathologic types found were 6 (67%) embryonal, 2
(22%) alveolar, and 1 (11%) undifferentiated. Triple agent chemotherapy
was used in the treatment of 8 cases, singly or in combination with
surgery or radiotherapy. Two patients are still alive, and 5 patients
have died of disease. CONCLUSIONS: The clinicopathologic features of
maxillofacial RMS in this group of Jordanians are different from those
of people from other countries. Further studies are needed to have a
better understanding of the behavior of RMS in the oral and
maxillofacial area.
6
UI - 12187831
AU - Suzuki A
TI -
[Enhanced expression of matrix metalloproteinase-9 (MMP-9) under hypoxic
and nutrient-deprived conditions]
SO - Hokkaido Igaku Zasshi 2002 Jul;77(4):351-7
AD - Hokkaido University Graduate School of Medicine, Sapporo 060-8638,
Japan.
7
UI - 11999659
AU - Rhee MY; Myong NH; Park YB
TI -
Primary intimal sarcoma of the aorta: role of transesophageal
echocardiography.
SO - Circ J 2002 Jan;66(1):111-3
AD - Department of Internal Medicine, College of Medicine, Dankook
University, Cheonan, Choongnam, Korea. rheemy@anseo.dankook.ac.kr
Diagnosis of primary tumors of the aorta is difficult. A patient who had
a primary intimal sarcoma of the aorta with metastasis presented with
obstructive symptoms and computed tomography showed a thrombus-like mass
in the aorta. However, transesophageal echocardiography revealed an
inhomogeneous and echo-dense mass with an outer membrane, unlike a
thrombus, and suggestive of a primary aortic tumor. Pathologic
examination of specimens from exploration and autopsy revealed a primary
intimal sarcoma.
8
UI - 12010367
AU - Lopes JM; Nesland JM; Reis-Filho JS; Holm R
TI -
Differential Ki67 and bcl-2 immunoexpression in solid-glandular and
spindle cell components of biphasic synovial sarcoma: a double
immunostaining assessment with cytokeratin and vimentin.
SO - Histopathology 2002 May;40(5):464-71
AD - Institute of Molecular Pathology and Immunology (IPATIMUP) and Porto
Medical School, University of Porto, Porto, Portugal.
AIMS: Synovial sarcoma is a malignant soft tissue of uncertain
histogenesis that may show a biphasic (spindle and solid/glandular
components) or a monophasic histological appearance. In previous
studies, we demonstrated that the solid/glandular component possesses
higher proliferation rates than the spindle cell component of biphasic
synovial sarcomas and that the spindle cell component may exhibit a
progressive transition from or to the solid-glandular component in
biphasic synovial sarcoma. To evaluate this hypothesis further, we
designed a novel approach to correlate immunoexpression of Ki67, bcl-2
and bax in the spindle cell and in the solid-glandular component of
biphasic synovial sarcomas. We also performed a
double-immunohistochemical assessment of the Ki67 proliferative indices
and the immunoexpression of anti-apoptotic protein bcl-2 in neoplastic
cells expressing either vimentin or cytokeratin. METHODS AND RESULTS:
Immunohistochemistry for vimentin (10 cases), bcl-2 (10 cases), Ki67(10
cases), cytokeratin (10 cases), and bax (eight cases), and
double-immunostaining for vimentin/Ki67 (10 cases), vimentin/bcl-2 (nine
cases), cytokeratin/Ki67 (10 cases), and cytokeratin/bcl-2 (10 cases)
assays were performed in 10 cases of primary biphasic synovial sarcoma.
Semiquantitative assessment was adopted for each case in both
components. Statistical analysis was performed using Fisher's exact test
or chi2 test. On conventional immunohistochemistry, the solid/glandular
component revealed more expression of Ki67, bax and cytokeratin than the
spindle cell component (P=0.0004, P=0.082, and P < 0.0001,
respectively); on the other hand, the latter showed higher expression of
bcl-2 and vimentin than the former (P=0.0281 and P=0.059, respectively).
Double immunohistochemistry analysis revealed higher co-expression
levels of cytokeratin/Ki67 and cytokeratin/bcl-2 than the spindle cell
component (P=0.015 and P < 0.0001, respectively); conversely, the latter
presented higher co-expression of vimentin/bcl-2 than the former
(P=0.0007). All cases showed no more than 10% of cells coexpressing
cytokeratin/bcl-2, cytokeratin/Ki67, and no case revealed cells
coexpressing vimentin/Ki67. CONCLUSION: Our findings indicate that in
biphasic synovial sarcoma the acquisition of epithelial phenotype
(solid/glandular component) is associated with a high expression of
pro-apoptotic proteins and a high proliferative differentiation status,
and conversely, mesenchymal phenotype (spindle cell component) is
associated with a high expression of apoptosis-inhibitor bcl-2 and a low
proliferative terminal-type differentiation status.
9
UI - 12149295
AU - Spunt SL; Hill DA; Motosue AM; Billups CA; Cain AM; Rao BN; Pratt CB;
TI -
Merchant TE; Pappo AS
Clinical features and outcome of initially unresected nonmetastatic
pediatric nonrhabdomyosarcoma soft tissue sarcoma.
SO - J Clin Oncol 2002 Aug 1;20(15):3225-35
AD - Department of Hematology-Oncology, St Jude Children's Research Hospital,
University of Tennessee College of Medicine, 332 N. Lauderdale Street,
Memphis, TN 38105-2794, USA. sheri.spunt@stjude.org
PURPOSE: To describe the clinical features, response to therapy, and
outcome of pediatric patients with initially unresected nonmetastatic
nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). PATIENTS AND METHODS:
We retrospectively reviewed the presenting clinical features and tumor
characteristics of all 40 pediatric patients with initially unresected
information was available was analyzed to determine whether response to
therapy was associated with local disease control and event-free and
overall survival. RESULTS: More than 70% of the 40 patients had tumors
with high-risk features (tumor size > 5 cm, high grade, invasiveness).
For the 27 patients included in the outcome analysis, 5-year event-free
survival and survival estimates were 33% +/- 9% and 56% +/- 10%,
respectively. Ten (37%) of these patients had a complete or partial
response to neoadjuvant chemotherapy and/or radiotherapy, and only two
of the 10 had residual tumor after surgery. Combined chemotherapy and
radiotherapy seemed more effective than either modality alone in
inducing a response, but the response to neoadjuvant therapy did not
predict outcome. Most treatment failures were local, and postrelapse
survival was poor (19% +/- 10%). CONCLUSION: Initially unresected NRSTS
constitutes a unique subgroup of pediatric sarcomas that commonly
present with high-risk features and respond poorly to neoadjuvant
therapy. Only about one third of patients treated with multimodal
therapy remain disease-free, and local control is the major limiting
factor in achieving cure. More effective risk-directed treatments are
needed for this unique subgroup of patients.
10
UI - 12149303
AU - Stein U; Jurchott K; Schlafke M; Hohenberger P
TI -
Expression of multidrug resistance genes MVP, MDR1, and MRP1 determined
sequentially before, during, and after hyperthermic isolated limb
perfusion of soft tissue sarcoma and melanoma patients.
SO - J Clin Oncol 2002 Aug 1;20(15):3282-92
AD - Division of Surgery and Surgical Oncology, Charite, Humboldt University,
Campus Berlin-Buch, Robert Rossle Hospital and Tumor Institute,
Robert-Rossle-Strasse 10, 13092 Berlin, Germany. ustein@mdc-berlin.de
PURPOSE: Isolated, hyperthermic limb perfusion (ILP) with recombinant
human tumor necrosis factor alpha and melphalan is a highly effective
treatment for advanced soft tissue sarcoma (STS) and locoregional
metastatic malignant melanoma. Multidrug resistance (MDR)-associated
genes are known to be inducible by heat and drugs; expression levels of
the major vault protein (MVP), MDR1, and MDR-associated protein 1 (MRP1)
were determined sequentially before, during, and after ILP of patients.
PATIENTS AND METHODS: Twenty-one STS or malignant melanoma patients were
treated by ILP. Tumor tissue temperatures were recorded continuously and
ranged from 33.4 degrees C initially to peak values of 40.4 degrees C
during ILP. Serial true-cut biopsy specimens from tumor tissues were
routinely microdissected. Expression analyses for MDR genes were
performed by real-time reverse transcriptase polymerase chain reaction
and immunohistochemistry. RESULTS: In 83% of the patients, MVP
expression was induced during hyperthermic ILP. MVP-mRNA inductions
often paralleled the increase in temperature during ILP. Increased MVP
protein expressions either were observed simultaneously with the
MVP-mRNA induction or were delayed until after the induction at the
transcriptional level. Inductions of MDR1 and MRP1 were observed in only
13% and 27% of the specimens analyzed. Temperatures and drugs applied
preferentially led to an induction of MVP and were not sufficient to
induce MDR1 and MRP1 in the majority of tumors. CONCLUSION: This study
is the first to analyze the expression of MDR-associated genes
sequentially during ILP of patients and demonstrates that treatment
might lead to increased levels of MVP, whereas enhanced levels of MDR1
and MRP1 remain rare events.
11
UI - 9789601
AU - Verweij J
TI -
High-dose ifosfamide for soft tissue sarcomas: set the scene, or
senescence?
SO - Ann Oncol 1998 Aug;9(8):807-9
12
UI - 9789611
AU - Leyvraz S; Bacchi M; Cerny T; Lissoni A; Sessa C; Bressoud A; Hermann R
TI -
Phase I multicenter study of combined high-dose ifosfamide and
doxorubicin in the treatment of advanced sarcomas. Swiss Group for
Clinical Research (SAKK).
SO - Ann Oncol 1998 Aug;9(8):877-84
AD - Serge.Leyvraz@chuv.hospvd.ch
Ifosfamide and doxorubicin are the most active agents in the treatment
of sarcomas and are characterized by a marked dose-response
relationship. The objective of this study was to determine the maximum
tolerated dose (MTD) of both agents in combination under
granulocyte-macrophage colony-stimulating factor (GM-CSF) cover.
PATIENTS AND METHODS: Thirty-three patients with untreated sarcomas
(soft tissue: n = 20; gynecological: n = 11; bone: n = 2) were treated
with ifosfamide 12 g/m2 by continuous i.v. infusion over five days and
doxorubicin with dose escalation from 50 mg/m2 i.v. bolus divided on two
days, then to 60 mg/m2 bolus divided on three days. Ifosfamide was
reduced to 10 g/m2 and doxorubicin was further escalated up to 90 mg/m2.
GM-CSF (5 micrograms/kg/day subcutaneously) was started 24 hours after
chemotherapy and continued for 10 days. RESULTS: The MTD was reached
with the combination of ifosfamide at 12 g/m2 and doxorubicin at 60
mg/m2. But with ifosfamide 10 g/m2 and doxorubicin 90 mg/m2 the MTD was
not obtained. While severe leukopenia and granulopenia were observed at
all-dose levels, severe anemia was more frequently related to the
highest dose of ifosfamide. Severe thrombopenia and mucositis were more
commonly observed at the highest dose of doxorubicin. Ifosfamide 10 g/m2
and doxorubicin 90 mg/m2 induced WHO grade 4 leukopenia in 58%, grade
3-4 thrombopenia in 42%, and anemia in 31% of cycles. Mucositis was
minor in 50% of cycles. The overall response rate among 31 evaluable
patients was 55% (95 confidence interval (CI): 36%-73%), with four (13%)
complete responders and 13 (42%) partial responders. Response rates
based on soft-tissue sarcomas or gynecological sarcomas alone were
similar. Ten patients could be treated by elective surgery and/or
radiotherapy. The total group of patients reached a median survival of
two years, with 25% (SE 8%) survivors after three years. CONCLUSIONS:
The dose level of ifosfamide 10 g/m2 and doxorubicin 90 mg/m2 with
supportive GM-CSF is manageable in a multicenter setting and should be
further tested in regular phase II trials, including patients with
gynecological and soft-tissue sarcomas. Transient toxicity with
myelosuppression should be accepted in order to obtain a high antitumor
activity of this regimen and a potential improvement in survival.
13
UI - 11474287
AU - Oda Y; Miyajima K; Kawaguchi K; Tamiya S; Oshiro Y; Hachitanda Y; Oya
TI -
M; Iwamoto Y; Tsuneyoshi M
Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical
study with special emphasis on its distinction from ordinary
leiomyosarcoma and malignant fibrous histiocytoma.
SO - Am J Surg Pathol 2001 Aug;25(8):1030-8
AD - Department of Anatomic Pathology, Graduate School of Medical Sciences,
Kyushu University, Fukuoka, Japan.
Pleomorphic leiomyosarcoma (PLMS) was recently described as a
morphologic variant of leiomyosarcoma; however, its diagnostic criteria,
as shown by morphologic features and biologic behavior, remain
controversial. We describe 28 cases of pleomorphic sarcoma with
pleomorphic areas in more than two thirds of the tumor and an ordinary
leiomyosarcomatous fascicular area covering less than one third as PLMS.
PLMS comprised 8.6% of all the leiomyosarcomas (322 cases) registered in
our institute. Patients ranged in age from 31 to 89 years (average, 57.9
years). Seventeen patients (60.7%) were male and 11 were female. Tumor
location was as follows: the extremities in 17 cases, the
retroperitoneum or abdominal cavity in 7 cases, the chest/abdominal wall
in 3 cases, and the scalp in 1 case. Histologically, all cases showed at
least small foci of fascicles consisting of smooth muscle tumor cells,
in addition to pleomorphic areas mimicking storiform-pleomorphic
malignant fibrous histiocytoma. The border between pleomorphic and
leiomyosarcomatous fascicular areas was sharp in 3 cases, gradual in 2
cases, and blending in 23 cases. Sixteen cases (57.1%) showed a typical
storiform pattern, 6 cases revealed extensive stromal hyalinization, 6
cases showed a chronic inflammatory infiltrate, 2 cases had the foci of
foamy xanthomatous cells, and 7 cases contained myxoid malignant fibrous
histiocytoma-like areas covering less than 50% of the tumor. The tumors
had a tendency to be of a morphologically higher grade (10 tumors were
French Federation of Cancer Centers grade 2, 18 were grade 3). Five of
28 cases (18%) showed rhabdoid features. Immunohistochemically, all of
the 28 tumors examined showed a positive reactivity for at least one
smooth muscle marker (desmin, muscle-specific actin, and alpha-smooth
muscle actin) in the leiomyosarcomatous fascicular areas. In the
pleomorphic areas the expression of smooth muscle markers (desmin 10 of
28, muscle-specific actin 13 of 28, and alpha-smooth muscle actin 14 of
28) was significantly reduced, compared with that in leiomyosarcomatous
fascicular area (desmin 18 of 28, muscle-specific actin 26 of 28, and
alpha-smooth muscle actin 24 of 28). No significant difference was
observed between the MIB-1 labeling index in the leiomyosarcomatous
fascicular areas (26.10 on average) and that in the pleomorphic areas
(26.17 on average). However, the MIB-1 labeling index in PLMS was
significantly higher than that in ordinary leiomyosarcoma (n = 20, 12.86
on average) or storiform-pleomorphic malignant fibrous histiocytoma (n =
16, 16.63 on average). In 23 patients follow-up data were available with
a duration of 1-239 months. Eleven patients developed metastases, and
lung accounted for the most common site of metastasis (9 cases). Fifteen
of 23 patients (65.2%) died of disease. Our results indicate that PLMS
should be differentiated from ordinary leiomyosarcoma because of its
high proliferative activities and rather aggressive biologic behavior.
14
UI - 11717539
AU - Watanabe K; Ogura G; Tajino T; Hoshi N; Suzuki T
TI -
Myofibrosarcoma of the bone: a clinicopathologic study.
SO - Am J Surg Pathol 2001 Dec;25(12):1501-7
AD - Pathology Division, Fukushima Medical University School of Medicine
Hospital, Fukishima City, Japan. w-kazuo@fmu.ac.jp
Myofibroblastic tumors are fairly recently established soft tissue
neoplasms. Although most of them appear to be benign, myofibrosarcoma of
the soft tissue, seemingly their malignant counterpart, have been
reported. We describe the clinicopathologic and radiologic features of
four cases of myofibrosarcoma arising from the bone. All but one of the
patients were women ranging in age from 60 to 71 years. Two tumors
occurred in the metaphyses of distal femurs and the others arose in the
iliac bones. On radiologic examination all tumors exhibited
well-demarcated lytic destructive lesions without periosteal reaction.
Two tumors were localized in the bone, whereas the other two extended
into surrounding soft tissues. Histologically, all tumors were composed
principally of a mixture of a cell-rich fascicular area and a
hypocellular fibrous area. In the former area tumor cells had rather
eosinophilic spindle-shaped wavy cytoplasm and were arranged in
interlacing fascicles and small storiform patterns with variable numbers
of inflammatory cells. Tumors occasionally showed prominent
pleomorphism, and large cells with hyperchromatic nuclei were seen. In
contrast, hypocellular areas had various features, including
collagenous, hyalinous scar-like and rarely keloid-like areas. Focal
coagulation necroses were present in all but one tumor.
Immunohistochemically, the tumors were positive for vimentin, muscle
actin (HHF35), alpha-smooth muscle actin, calponin, and desmin, whereas
all of them were negative for high molecular weight caldesmon. On
follow-up there was one fatal case with distant metastases, whereas the
clinical courses of other cases after wide resection were excellent.
Myofibrosarcoma of the bone has distinctive histopathologic features,
which should be distinguished from those of other bone tumors with myoid
differentiation.
15
UI - 11871857
AU - Brabek J; Mojzita D; Hamplova L; Folk P
TI -
The regulatory region of Prague C v-Src inhibits the activity of the
Schmidt-Ruppin A v-Src kinase domain.
SO - Folia Biol (Praha) 2002;48(1):28-33
AD - Department of Physiology, Charles University, Prague, Czech Republic.
Existing variants of the oncogene v-src differ in their transforming
potential as well as in the range of their hosts. We compared the
protein kinase activities of two Prague C v-Src variants (PRC and H19),
reported to be of low oncogenic potential (Plachy et al., 1995), with
the highly oncogenic Schmidt-Ruppin A v-Src (SRA). We employed in vitro
kinase assays of affinity-purified proteins expressed in rabbit
reticulocyte lysate and in S. cerevisiae. In both systems used, the
specific kinase activity of the Prague C v-Src kinases amounted to only
ca 20% of the activity of SRA. This positions the PRC Src close to
activated c-Src, despite the lack of the regulatory C-terminal tail in
PRC. We constructed chimeras between PRC and SRA v-Src and tested them
for specific kinase activity in S. cerevisiae. Remarkably, the
regulatory N-terminal part of PRC, when fused to the SRA-derived kinase
domain, lowered the chimeras' PK activity to ca 20%, suggesting that it
is the regulatory part of PRC that is responsible for its low
phosphotransferase activity.
16
UI - 11876388
AU - Szabo I; Szantho A; Csabay L; Csapo Z; Szirmai K; Papp Z
TI -
Color Doppler ultrasonography in the differentiation of uterine sarcomas
from uterine leiomyomas.
SO - Eur J Gynaecol Oncol 2002;23(1):29-34
AD - Department of Obstetrics and Gynaecology, Semmelweis University Medical
School, Budapest, Hungary.
OBJECTIVE: The aim of this study was to investigate uterine vascularity
in cases of uterine leiomyomas and uterine sarcomas, as well as to
determine the efficiency of uterine blood flow analysis in
differentiating between them. MATERIALS AND METHODS: Transvaginal color
and pulsed Doppler findings obtained from 117 patients with
histologically proven uterine leiomyoma and 12 with uterine sarcoma were
retrospectively assessed. RESULTS: The mean intratumoral resistance
index (RI) and pulsatility index (PI) were significantly lower and the
intratumoral peak systolic velocity (PSV) was significantly higher in
patients with sarcomas than in patients with uterine leiomyomas. Marked
reduction of RI and PI and increased PSV could be found in 14 of the
leiomyoma cases which showed large size and/or necrotic, degenerative
and inflammatory changes. When a cut-off value of 0.5 for the RI was
considered, the detection rate for uterine sarcoma was 67% and the
false-positive rate was 11.8%. CONCLUSION: These results suggest that
the intratumoral RI detected by color and pulsed Doppler ultrasonography
in themselves could be poor for the preoperative differential diagnosis
of uterine sarcoma.
17
UI - 12082855
AU - Nielsen OS; Keller JO; Dombernowsky P
TI -
[Sarcomas]
SO - Ugeskr Laeger 2002 Jun 3;164(23):3036-9
AD - Arhus Universitetshospital, Arhus Kommunehospital, onkologisk afdeling,
ortopaedkirurgisk afdeling.
18
UI - 12088451
AU - Boyle JL; Haupt HM; Stern JB; Multhaupt HA
TI -
Tyrosinase expression in malignant melanoma, desmoplastic melanoma, and
peripheral nerve tumors.
SO - Arch Pathol Lab Med 2002 Jul;126(7):816-22
AD - Department of Pathology, Pennsylvania Hospital, Philadelphia, PA 19107,
USA.
CONTEXT: Pathologists may encounter problems in the differential
diagnosis of malignant melanoma, spindle and epithelioid neoplasms of
peripheral nerves, and fibrohistiocytic tumors. Tyrosinase has been
demonstrated to be a sensitive marker for melanoma. OBJECTIVE: To
determine the specificity of tyrosinase expression in the differential
diagnosis of melanoma, desmoplastic melanoma, and peripheral nerve
sheath tumors. DESIGN: Immunoreactivity for tyrosinase, HMB-45
(anti-gp100 protein), S100 protein, CD34, and vimentin was studied in 70
tumors, including 15 melanomas (5 desmoplastic, 4 amelanotic, 6
melanotic), 13 malignant peripheral nerve sheath tumors; 10 schwannomas
(1 pigmented), 12 neurofibromas (4 pigmented), and 20 fibrohistiocytic
tumors (10 dermatofibrosarcoma protuberans and 10 dermatofibromas).
Microwave-based antigen retrieval was performed in 10mM citrate buffer,
pH 6.0, for 20 minutes at 121 degrees C. RESULTS: All melanomas
demonstrated positive immunostaining for tyrosinase, HMB-45, and S100
protein. Immunoreactivity for HMB-45 was generally stronger than that
for tyrosinase in amelanotic lesions and significantly stronger in 1 of
the desmoplastic lesions. The 4 pigmented neurofibromas were focally
positive for tyrosinase, but did not stain for HMB-45. The pigmented
schwannoma was focally positive for both tyrosinase and HMB-45. The
malignant peripheral nerve sheath tumors, dermatofibrosarcoma
protuberans, and dermatofibromas were nonreactive for tyrosinase and
HMB-45. CONCLUSIONS: Our results support the sensitivity of tyrosinase
expression and demonstrate the relative specificity of tyrosinase as a
marker for melanocytic lesions, including desmoplastic melanoma,
although pigmented peripheral nerve tumors may demonstrate focal
positive staining. Immunoreactivity for tyrosinase and HMB-45 may have
been enhanced by the microwave-based antigen-retrieval technique used in
this study.
19
UI - 12091061
AU - van Ruth S; van Coevorden F; Peterse JL; Kroon BB
TI -
Alveolar soft part sarcoma. a report of 15 cases.
SO - Eur J Cancer 2002 Jul;38(10):1324-8
AD - Department of Surgery, The Netherlands Cancer Institute/Antoni van
Leeuwenhoek Hospital, Plesmanlaan 120, 1066 CX Amsterdam, The
Netherlands.
The aim of this study was to evaluate the presentation, course and
treatment outcome of 15 patients with this rare type of sarcoma. The
files of the patients were retrospectively analysed. Overall survival
was calculated according to the Kaplan-Meier method. There were 15
patients, 8 male and 7 female. The mean age at diagnosis was 29 years
for men and 24 years for women. The median survival was 48 months with
an overall 5-year survival of 38%. 5 patients had haematogenic
metastases at the time of diagnosis. For the remaining 10 patients with
localised disease, the median survival was 48 months and the 5-year
survival 48%. The median disease-free survival for these patients was 12
months with a 5-year disease free survival of 40%. After the occurrence
of haematogenic metastases, patients survived a median period of 8
months (range 0-45 months). 5 patients are still free of disease after a
median period of 234 months (12-295 months). Alveolar soft part sarcoma
is found especially in young adults. When diagnosed, it is often
metastasised with a poor prognosis. However, when radically resected,
long-term survival is possible.
20
UI - 12091063
AU - Merimsky O; Issakov J; Bickels J; Kollender Y; Flusser G; Soyfer V;
TI -
Schwartz I; Inbar M; Meller I
ErbB-4 expression in limb soft-tissue sarcoma: correlation with the
results of neoadjuvant chemotherapy.
SO - Eur J Cancer 2002 Jul;38(10):1335-42
AD - Department of Oncology, The Tel-Aviv Sourasky Medical Center, 6 Weizman
Street, Israel. merimsky@sahav.net.il
ErbB-4 is a recently described growth factor receptor. Relatively little
is known about its expression in human tumours. In this study, we
assessed the possible role of erbB-4 as a tissue marker for soft-tissue
sarcomas (STS) and its correlation with the response to chemotherapy.
The histological specimen of 29 patients with STS of a limb who had
received preoperative doxorubicin (ADR)-based chemotherapy were studied
for the degree of necrosis and the expression of erbB-4 (by an
avidin-biotin-peroxidase technique). ErbB-4 expression in the
preoperative tissue samples was compared with the expression in the
postchemotherapy resected tumour. The true objective response rate to
preoperative chemotherapy was 34%. Wide resection of the tumour was done
in 12 patients, marginal in 14, amputation in 2 and no surgery in 1. The
tumour necrosis was above 90% in 9 patients, 60-90% in 12, and less than
60% in 7 patients. An increase in erbB-4 expression was more common in
cases with no response to chemotherapy, while no change or a decrease in
erbB-4 was more common in responsive tumours (P=0.004). No correlation
could be found between the degree of necrosis or the chemotherapeutic
regimen and the change in expression of erbB-4. The median disease-free
survival (DFS) was longer for patients with a decrease or no change in
expression of erbB-4 than for patients with increased expression. It is
believed that postchemotherapy new expression or no downregulation of
the erbB-4 molecule represents tumour aggressiveness and increased
capability of growth and spread.
21
UI - 12150740
AU - Wei Y; Wang J; Zhu X; Shi D; Hisaoka M; Hashimoto H
TI -
Detection of SYT-SSX fusion transcripts in paraffin-embedded tissues of
synovial sarcoma by reverse transcription-polymerase chain reaction.
SO - Chin Med J (Engl) 2002 Jul;115(7):1043-7
AD - Department of Pathology, Cancer Hospital, Fudan University, Shanghai
200032, China.
OBJECTIVE: To assess the feasibility of detecting SYT-SSX fusion
transcripts in paraffin-embedded tissues of synovial sarcoma by reverse
transcription-polymerase chain reaction (RT-PCR). METHODS: RT-PCR was
used to amplify the SYT-SSX fusion transcripts using archival
formalin-fixed paraffin-embedded tumor specimens from a series of 37
synovial sarcoma cases. To investigate the specificity of the SYT-SSX
fusion transcripts, a variety of non-synovial sarcoma tumors were
included in the study as negative controls. The detected messages
derived from fusion genes were confirmed by subsequent sequence
analysis. RESULTS: SYT-SSX fusion transcripts were detected in 33 of 37
(89.2%) synovial sarcomas. None of the 34 cases of non-synovial sarcoma
tumors showed amplified products of SYT-SSX fusion transcripts, although
PBGD mRNA was detected in all specimens. Among 33 SYT-SSX-positive
synovial sarcomas, 22 tumors had an SYT-SSX 1 fusion transcript, whereas
6 tumors had an SYT-SSX2 fusion transcript. Fusion types can not be
distinguished in the remaining 5 cases. There was a significant
relationship between SYT-SSX fusion type and histologic subtype. All 10
biphasic synovial sarcomas had the SYT-SSX1 fusion, whereas all tumors
with SYT-SSX2 were of monophasic morphology (P < 0.05). CONCLUSIONS:
RT-PCR can be applied to archival formalin-fixed paraffin-embedded tumor
tissues as a sensitive and reliable technique for the diagnosis and
differential diagnosis of synovial sarcoma. There is an association
between SYT-SSX fusion type and histological subtype. SYT-SSX2 fusion
transcripts can only be found in monophasic synovial sarcoma.
22
UI - 12171492
AU - Lu C; Gordon GM; Chandran B; Nickoloff BJ; Foreman KE
TI -
Human herpesvirus 8 reactivation and human immunodeficiency virus type 1
gp120.
SO - Arch Pathol Lab Med 2002 Aug;126(8):941-6
AD - Department of Pathology and Skin Cancer Research Laboratories, Cardinal
Bernardin Cancer Center, Loyola University Medical Center, Maywood, Ill
606153, USA.
CONTEXT: Human herpesvirus 8 (HHV-8) is the presumed etiologic agent of
Kaposi sarcoma (KS), the most common neoplasm in patients with acquired
immunodeficiency syndrome. Current evidence indicates HHV-8 is
necessary, but not sufficient, for KS development without the
involvement of other cofactors. One potentially important cofactor is
human immunodeficiency virus type 1 (HIV-1). Although HIV-1 is not
essential for development of KS, studies have shown factors released
from HIV-1-infected cells, including HIV-1 proteins and cytokines,
promote the growth of KS cells in vitro. Recently, studies have shown
that coculture of HIV-1-infected T cells with HHV-8-infected primary
effusion lymphoma cell lines results in HHV-8 reactivation. This
response was due, in part, to cytokines. However, only a portion of
induced HHV-8 replication could be accounted for by cytokine
stimulation, indicating that other factors, including HIV-1-associated
proteins, may also be involved. OBJECTIVE: To investigate a possible
role for HIV-1 gp120 in HHV-8 reactivation. DESIGN: Using an in vitro
model system, we examined the effect of recombinant HIV-1 gp120 protein
on HHV-8 replication in latently infected primary effusion lymphoma cell
lines. MAIN OUTCOME MEASURES: Reactivation of HHV-8 was analyzed using
Northern blot analysis and quantitative polymerase chain reaction for
ORF26 messenger RNA expression, a gene encoding for the HHV-8 minor
capsid protein produced only during reactivation. The results were
extended and confirmed using a luciferase reporter construct driven by
the HHV-8 ORF50 promoter, the first promoter activated during HHV-8
replication. RESULTS: No evidence of enhanced HHV-8 replication was
found following treatment with HIV-1 gp120. In addition, HIV-1 gp120 was
unable to act synergistically with interferon-gamma or hepatocyte growth
factor/scatter factor to enhance reactivation of the virus in infected
primary effusion lymphoma cell lines. CONCLUSIONS: HIV-1 gp120 does not
appear to be responsible for the reactivation of HHV-8 demonstrated in
our previous studies. Further studies are necessary to determine if
other HIV-associated proteins, particularly Tat, gp160, and/or gp41,
which are also released from infected cells, may be important in
inducing HHV-8 reactivation.
23
UI - 12173050
AU - Xie Y; Skytting B; Nilsson G; Grimer RJ; Mangham CD; Fisher C; Shipley
TI -
J; Bjerkehagen B; Myklebost O; Larsson O
The SYT-SSX1 fusion type of synovial sarcoma is associated with
increased expression of cyclin A and D1. A link between t(X;18)(p11.2;
q11.2) and the cell cycle machinery.
SO - Oncogene 2002 Aug 22;21(37):5791-6
AD - Department of Oncology and Pathology, CCK R8: 04, Karolinska Hospital,
Stockholm, Sweden.
A recent large multi-centre study convincingly confirmed previous
observations that the SYT-SSX1 fusion type, compared to SYT-SSX2, of
synovial sarcoma is associated with a worse clinical outcome. Apart from
the clinical impact, this fact also suggests (1) that the SYT-SSX fusion
gene may influence molecular mechanisms involved in tumour growth and
progression; and (2) that the SYT-SSX1 fusion type has a stronger
influence on these mechanisms than SYT-SSX2. The nature of the
underlying mechanisms is, however, still unknown. In this study we made
use of the SYT-SSX1 vs SYT-SSX2 concept to investigate whether major,
tumour relevant, and growth regulatory proteins (e.g. cyclins and
cyclin-dependent kinases) may be involved. Using Western blotting
analysis on 74 fresh, fusion variant-typed, tumour samples from
localized synovial sarcoma, we found a significant correlation between
SYT-SSX1 and high expression of cyclin A (P=0.003) and D1 (P=0.025). Our
data suggest that SYT-SSX may influence the cell cycle machinery, and
that the more aggressive phenotype of the SYT-SSX1 variant is due to an
accelerated tumour cell proliferation.
24
UI - 12185060
AU - Israel GM; Bosniak MA; Slywotzky CM; Rosen RJ
TI -
CT differentiation of large exophytic renal angiomyolipomas and
perirenal liposarcomas.
SO - AJR Am J Roentgenol 2002 Sep;179(3):769-73
AD - Department of Radiology, Division of Abdominal Imaging, HW 202, New York
University Medical Center, 560 First Ave., New York, NY 10016, USA.
OBJECTIVE: The purpose of our study was to describe the imaging findings
and CT characteristics that lead to accurate distinction of large
exophytic renal angiomyolipomas from retroperitoneal perirenal
liposarcomas, which at times can be confused on imaging studies and even
at pathologic examination. MATERIALS AND METHODS: We retrospectively
analyzed CT images of 15 large exophytic renal angiomyolipomas and 12
well-differentiated perirenal liposarcomas. Pathologic correlation was
available for six of 15 angiomyolipomas and all of the liposarcomas. All
examinations were evaluated for lesion size, renal parenchymal defect,
enlarged vessels, kidney displacement, lesion encapsulation or
margination, associated hemorrhage, and additional angiomyolipomas. The
records of patients with tuberous sclerosis or the forme fruste of that
condition were excluded from the study. RESULTS: The average size of the
angiomyolipomas was 14 x 10 cm. They showed a renal parenchymal defect
(n = 15), enlarged vessels (n = 12), renal displacement (n = 14), good
margination without a distinct capsule (n = 14), hemorrhage (n = 1), and
additional (one or two) angiomyolipomas (n = 4). The average size of the
liposarcomas was 18 x 11.6 cm. They showed enlarged vessels (n = 3),
renal displacement (n = 11), and encapsulation (n = 4); none showed a
renal parenchymal defect, hemorrhage, or associated angiomyolipomas.
CONCLUSION: Although large exophytic angiomyolipomas and
well-differentiated retroperitoneal liposarcomas may have similar
appearances on imaging, careful evaluation for a defect in the renal
parenchyma combined with the presence of enlarged vessels in
angiomyolipomas should enable accurate differentiation in almost all
cases. Achieving an accurate diagnosis can have a significant impact on
patient treatment.
25
UI - 12185067
AU - McCarville MB; Spunt SL; Skapek SX; Pappo AS
TI -
Synovial sarcoma in pediatric patients.
SO - AJR Am J Roentgenol 2002 Sep;179(3):797-801
AD - Department of Diagnostic Imaging, Mail Stop 210, St. Jude Children's
Research Hospital, 332 N. Lauderdale St., Memphis, TN 38105-2794, USA.
26
UI - 12177782
AU - Barker KT; Bevan S; Wang R; Lu YJ; Flanagan AM; Bridge JA; Fisher C;
TI -
Finlayson CJ; Shipley J; Houlston RS
Low frequency of somatic mutations in the FH/multiple cutaneous
leiomyomatosis gene in sporadic leiomyosarcomas and uterine leiomyomas.
SO - Br J Cancer 2002 Aug 12;87(4):446-8
AD - Section of Cancer Genetics, Haddow Laboratories, Institute of Cancer
Research, 15 Cotswold Road, Sutton, Surrey SM2 5NG, UK.
Germline mutations in the fumarate hydratase gene at 1q43 predispose to
dominantly inherited skin and uterine leiomyomata and leiomyosarcomas.
The enzyme, which is a component of the tricarboxylic acid cycle, acts
as a tumour suppressor. To evaluate fumarate hydratase in respective
sporadic tumours, we analysed a series of 26 leiomyosarcomas and 129
uterine leiomyomas (from 21 patients) for somatic mutations in fumarate
hydratase and allelic imbalance around 1q43. None of the 26
leiomyosarcomas harboured somatic mutations in fumarate hydratase. Fifty
per cent of leiomysarcomas tested showed evidence of allelic imbalance
at 1q, but this was not con
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