Purpose: Extracellular matrix metalloproteinase inducer (EMMPRIN) has been shown to stimulate fibroblasts to production
of matrix metalloproteinases (MMPs). MMPs comprise a family of proteolytic enzymes implicated in the degradation
of extracellular matrix which has been proposed to be one of the essential steps in tumor invasion and metastases. In the
present study we investigated the expression and location of mRNAs for EMMPRIN, matrix metalloproteinase-2 (MMP-2),
and membrane-type 1 matrix metalloproteinase (MT1-MMP) in mesenchymal tumors with different tendencies to recur or
metastasize.Subjects: Eight malignant fibrous histiocytomas (MFH), seven aggressive fibromatosis (AF), and six benign fibrous tumors
(BF).Method: The mRNA-expression of EMMPRIN, MMP-2 and MT1-MMP were studied using mRNA in situ hybridization
technique.Results: The mRNA-expression of EMMPRIN, MMP-2 and MT1-MMP respectively were found at varying frequency and
level in all tumor types. The mRNAs corresponding to EMMPRIN and MMP-2 were seen in neoplastic cells as well as in
endothelial cells both inside and outside the tumor pseudo-capsule, whereas MT1-MMP was seen only within the tumors.
The estimated mRNA levels of EMMPRIN and MMP-2 covariated significantly. Overall, the highest expression was found
in the MFH tumors and the lowest levels in the BF tumors.Discussion: These findings suggest that the MMP-inducer EMMPRIN and the extracellular matrix degrading system
involving the metalloproteinases MMP-2 and MT1-MMP is frequently activated in mesenchymal tumors. The covariation
between EMMPRIN and MMP-2 support previous findings that EMMPRIN may be an inducer of MMP-2. The high
levels of MMP-2 mRNA in MFH indicate a relationship between the proteolytic activity of MMP-2 and the tumor aggressiveness.

Jan Åhlén

Ulla Enberg

Catharina Larsson

Olle Larsson

Tony Frisk

Otte Brosjö

Anette von Rosen

Martin Bäckdahl

Malignant Fibrous Histiocytoma, Aggressive Fibromatosis and Benign Fibrous Tumors Express mRNA for the Metalloproteinase Inducer EMMPRIN and the Metalloproteinases MMP-2 and MT1-MMP

Purpose/Methods: The aP2 gene product (aP2 protein) is known to be expressed by preadipocytes and other immature fat
cells in vitro. A mouse monoclonal antibody raised against an 18 amino acid segment of the aP2 protein was found to react
with lipoblasts and fetal fat cells in paraffin sections of soft tissue tumours of adipose differentiation. In this immunohistochemical
study, we have further examined the diagnostic utility of aP2 expression in distinguishing tumours of adipose differentiation
from other benign and malignant soft tissue tumours.Result and discussion aP2 was strongly expressed by lipoblasts in lipoblastomas and all types of liposarcoma as well as brown
fat cells in hibernomas. Optimal conditions for immunohistochemical identification of lipoblasts in tumours of adipose differentiation
was noted when the antibody was diluted 1:30 to 1:50. Small lipoblast-like fat cells in pleomorphic lipoma and
spindle cell lipoma also showed variable staining for aP2 at this dilution of the antibody. Most benign and malignant soft
tissue tumours were distinguished by their absence of staining for aP2 protein, but some cases of myxoma, malignant fibrous
histiocytoma, synovial sarcoma and leiomyosarcoma contained tumour cells which reacted for aP2. aP2 protein expression
is likely to prove a useful means of distinguishing lipoblasts in liposarcoma but it should be used as part of a tumour panel
to exclude expression in other forms of mesenchymal tumour.

T. T. Yang

A. C. C. Reed

N. A. Athanasou

aP2 Protein Expression as a Diagnostic Marker in Soft Tissue Tumours

A report of alveolar soft part sarcoma metastatic to the small bowel is presented. Hematogenous metastases to the small
bowel from primary tumors outside the abdominal cavity are uncommon, and most remain asymptomatic and are not discovered
until autopsy. However, small bowel metastases can lead to intestinal obstruction, intussuseption or even perforation.
While metastases to the small bowel have been described for other tumor types, including melanoma and lung cancer, this
is extremely uncommon for sarcoma, especially alveolar soft part sarcoma. We describe a 42-year-old male with a long history
of alveolar soft part sarcoma, metastatic to the lung and brain, who developed an intussuseption from metastases to the small
bowel.

Michael S. Sabel

John F. Gibbs

Allan  Litwin

Brian  Mcgrath

William B. Kraybill

John J. Brooks

Alveolar Soft Part Sarcoma Metastatic to Small Bowel Mucosa Causing Polyposis and Intussuseption

Purpose: To report on an extremely rare tumour located in the cervical spine, its treatment and result.
Review of the literature.Patient: Case report of a 38-year-old woman with an intraosseous schwannoma of the cervical spine.Results: After local curettage no evidence for local recurrence at long-term follow-up.

H. W. Bart Schreuder

René P. H. Veth

Maciej Pruszczynski

J. Albert M. Lemmens

Erik W. van Laarhoven

Intraosseous Schwannoma (Neurilemmoma) of the Cervical Spine

Purpose. In the clinical work-up of a retroperitoneal mass, the diagnosis of soft tissue sarcoma is often not considered. Incidence
rates of various malignant and benign retroperitoneal tumours were studied to determine the incidence of soft tissue
sarcoma in comparison with other neoplasms in the retroperitoneal space.Method. Nation-wide data on retroperitoneal tumours, collected prospectively over a 5-year period (1 January 1989&#8211; 1 January
1994), were supplied by the Netherlands Cancer Registry and The Dutch Network and National Database for Pathology.Results. Seven hundred and six patients with a primary retroperitoneal neoplasm were identified; 566 patients had a malignant
tumour (80&#37;). A soft tissue sarcoma (STS) was the most frequently diagnosed malignant tumour (n &#61; 192), The agestandardised
incidence of retroperitoneal STS was 2.5 per million person-years. The male/female ratio for STS was 0.73. In
females, STS comprised 41&#37;of all malignant retroperitoneal tumours, carcinoma of unknown primary tumour site (CUP)
comprised 31&#37;, and malignant lymphomas (ML) comprised 22&#37;, whereas in males these values were 28&#37; (STS), 30&#37;
(CUP), and 32&#37; (ML), respectively.Discussion. Soft tissue sarcomas, albeit rare, are relatively common primary tumours in the retroperitoneum, especially in
women.

Thijs van Dalen

Jan-Willem W. Coebergh

Mariel K. Casparie

Charles H. F. Gimbr&#232;re

Harald J. Hoekstra

Bert N. Van Geel

Frits Van Coevorden

Adriaan  Hennipman

Soft Tissue Sarcoma: The Predominant Primary Malignancy in the Retroperitoneum

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Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.

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Sarcoma covers all aspects of connective tissue oncology research. It brings together work from scientists and clinicians carrying out a broad range of research in this field, including the basic sciences, molecular biology and pathology etc.

Sarcoma