Congenital gastrointestinal pacemaker cell tumor

The interstitial cells of Cajal complex within the gut wall function as a p acemaker to direct peristalsis, Their neoplastic counterpart is the gastroi ntestinal pacemaker cell tumor, a spindle and/or epithelioid cell mesenchym al tumor previously known as gastrointestinal stromal tumor or incorrectly called leiomyosarcoma in some cases of older reports, Although numerous cas es of gastrointestinal leiomyosarcomas have been documented in the English- language literature, no pediatric case of gastrointestinal stromal tumor or gastrointestinal pacemaker cell tumor has, to our knowledge, been recorded . Herein, we report a case of congenital gastrointestinal pacemaker cell tu mor confirmed by immunohistochemistry and electron microscopy in a full-ter m male newborn.