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| Gastrointestinal pacemaker cell tumor (GIPACT) - Gastrointestinal stromal tumors show phenotypic characteristics of the interstitial cells of Cajal |
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| Author(s): Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM |
| Source: AMERICAN JOURNAL OF PATHOLOGY Volume: 152 Issue: 5 Pages: 1259-1269 Published: MAY 1998 |
| Times Cited: 633 References: 50 |
| Abstract: The interstitial cells of Cajal (ICC) form a complex cell network within the gastrointestinal tract wall where the function as a pacemaker system. Expression of the kit proto-oncogene is essential for the development of this system. The aim of our study was to examine the hypothesis that gastrointestinal stromal tumors differentiate toward cells with an ICC phenotype. Ultrastructurally, 58 stromal tumors were characterized and found to share many features with ICC. Seventy-eight stromal tumors were immunophenotyped, particularly with regard to the kit receptor. AU 78 tumors revealed strong, homogeneous immunoreactivity for the kit receptor as did ICC of adjacent and control gastrointestinal walls. Focal hyperplasia and hypertrophy of kit receptor positive cells were also observed in the gastrointestinal wall adjacent to the tumors. CD34 immunoreactivity observed in interstitial cells surrounding Auerbach's ganglia suggests that a subpopulation of ICC is CD34 positive and may explain why 56 of 78 stromal tumors were CD34 positive. Thirty control tumors, including gastrointestinal leiomyomas and leiomyosarcomas, were all negative for the kit receptor, We conclude that gastrointestinal stromal tumors show striking morphological and immunophenotypic similarities with ICC and that they may originate from stem cells that differentiate toward a pacemaker cell phenotype. We propose that the noncommittal name "gastrointestinal stromal tumor" be replaced by gastrointestinal pacemaker cell tumor. |
| Document Type: Article |
| Language: English |
| Reprint Address: Kindblom, LG (reprint author), Univ Gothenburg, Dept Pathol, Sahlgrens Univ Hosp, Gothenburg Musculoskeletal Tumor Ctr, S-41345 Gothenburg, Sweden |
Addresses:
1. Univ Gothenburg, Dept Pathol, Sahlgrens Univ Hosp, Gothenburg Musculoskeletal Tumor Ctr, S-41345 Gothenburg, Sweden |
| Publisher: AMER SOC INVESTIGATIVE PATHOLOGY, INC, 428 EAST PRESTON ST, BALTIMORE, MD 21202-3993 USA |
| Subject Category: Pathology |
| IDS Number: ZL742 |
| ISSN: 0002-9440 |
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