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Intra-Abdominal Desmoplastic Small Round Cell Tumor

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Authors
Park, Sung Hye; Park, Heum Rhye
Issue Date
1994-09
Publisher
Seoul National University College of Medicine
Citation
Seoul J Med 1994;35(3):217-224
Keywords
Small round cell tumordesmoplasticabdominalchildrenimmunohistochemical studyultrastructural study
Abstract
We report a case of malignant intraabdominal tumor in a 14 years old
Korean girl that presented with abdominal pain, distension and palpable mass. Emergency
laparotomy revealed bulky peritoneal masses with numerous satellite implanting nodules.
Ught microscopically the tumor was characterized by discrete islands of compact,
small epithelium-like cells encased in desmoplastic stroma. Immunohistochemically the
neoplastic cells expressed vimentin strongly and diffusely and also neuron specific enolase
strongly and focally. Ultrastructurally the neoplastic cells showed whorled intermediate
filaments, a fair number of mitochondria, dilated rough endoplasmic reticulum, a
few fat vacuoles and zonula-adherens type junctions. However there was no evidence of
specific differentiation. Histologic, immunohistochemical, and electronmicroscopic observations
indicated a primitive malignant neoplasm simultaneously expressing mesenchymal
and neural differentiation. Our case did not express any epithelial markers including
pancytokeratin, epithelial membrane antigen and CAM 5.2.
ISSN
0582-6802
Language
English
URI
http://hdl.handle.net/10371/7352
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 35 No.3 (1994)
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