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Crystal-deficient alveolar soft-part sarcoma with cutaneous involvement: a case report.
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- Additional Information
- Source:
Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 7911005 Publication Model: Print Cited Medium: Internet ISSN: 1533-0311 (Electronic) Linking ISSN: 01931091 NLM ISO Abbreviation: Am J Dermatopathol Subsets: MEDLINE
- Publication Information:
Publication: Hagerstown, MD : Lippincott Williams & Wilkins
Original Publication: New York, Masson Publishing USA.
- Subject Terms:
- Abstract:
Alveolar soft-part sarcoma (ASPS) is a rare distinctive sarcoma, in most cases involving deep soft tissues of the extremities. It is associated with a specific unbalanced translocation, der(17)t(X;17)(p11;q25) that results in the formation of an ASPL-TFE3 fusion gene. Microscopically, it is typified by an alveolar growth of large cells containing typical periodic acid-Schiff-positive rod-shaped crystals, often serving as a diagnostic clue. Other distinctive features include nuclear immunoreactivity for transcription factor 3 (TFE3) protein and a typical ultrastructural finding of large crystals with a rectangular or rhomboid shape. The authors present an unusual case of ASPS with cutaneous involvement, which did not exhibit typical large crystals; there were striking round granules. Molecular genetic study revealed fusion transcript ASPL-TFE3, type 2. To the best of our knowledge, cutaneous involvement of a crystal-deficient ASPS has not been reported.
- Accession Number:
0 (ASPSCR1 protein, human)
0 (Basic Helix-Loop-Helix Leucine Zipper Transcription Factors)
0 (Intracellular Signaling Peptides and Proteins)
0 (Oncogene Proteins, Fusion)
0 (TFE3 protein, human)
- Publication Date:
Date Created: 20090423 Date Completed: 20090601 Latest Revision: 20191210
- Publication Date:
20240829
- Accession Number:
10.1097/DAD.0b013e31819d097a
- Accession Number:
19384069
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