Pendular stromal tumour of the stomach with dominant PDGFRA immunoexpression: Case report and short literature review

Abstract

Introduction. Gastrointestinal stromal tumours are most frequent mesenchimal tumours of the gastrointestinal tract that originate from Cajal?s interstitial cells that are most frequently CD-117 positive. Stromal tumours of the stomach are the most frequent mesenchimal tumours of the gastrointestinal tract. Such tumours are usually sessile, but rarely pendular when they can be easily removed with a limited local excision of the stomach wall around the pedicle. Major stomach resections are rarely necessary. Case Outline. In a 54-year-old woman with abdominal pain and fever of unknown aetiology, a large spherical mobile and almost painless mass was found within the upper right abdomen. US and CT showed a mainly cystic, partly solid tumour, of 15.5?12.5 cm in diameters. Laboratory data including tumour markers were within normal limits. At operation a mobile and free tumour of the stomach attached to the anterior wall with a 2.5 cm pedicle was found and easily excised. Abdominal mucosa was normal. There was no liver metastasis or peritoneal dissemination. Hystology and imunohistochemistry showed a rare sclerosing sincitial subtype of stromal tumour with imunophenotype heterogenicity with a dominant PDGFRA and rare CD-117 immunoexpression. The postoperative recovery was uneventful. The patient was symptom-free with no sign of recurrence after a year and a half. Conclusion. A rare subtype of histological highly malignant stromal tumour of the stomach, macroscopically of pendular type, that was easily excised, was presented which so far showed a favourable evolution with no signs of recurrence.Uvod. Gastrointestinalni stromalni tumori su najcesci mezenhimni tumori gastrointestinalnog trakta, a poticu od Kahalovih (Cajal) intersticijalnih celija, koje su najcesce CD117 pozitivne. Stromalni tumori zeluca su najcesci mezenhimni tumori gastrointestinalnog trakta. Cesce su sesilni, a redje pendularni, kada se ogranicenom lokalnom ekscizijom zida zeluca oko peteljke mogu lako odstraniti. Vece resekcije zeluca po pravilu nisu potrebne. Prikaz bolesnika. Kod 54-godisnje zene kod koje su se javili bol u trbuhu i neobjasnjiva febrilnost napipan je loptast, mobilan, blago bolan, prominirajuci tumefakt u desnom gornjem kvadrantu abdomena. Ultrazvukom i kompjuterizovanom tomografijom ustanovljen je pretezno cisticni, a manjim delom solidni tumor velicine 15,5?12,5 cm. Laboratorijski nalazi, ukljucujuci i tumorske markere, bili su u referentnim granicama. Tokom operacije uocen je slobodan tumor koji je polazio od prednjeg zida zeluca, s kojim je bio vezan peteljkom precnika oko 2,5 cm. Tumor je odstranjen lokalnom ekscizijom peteljke. Na mukozi zeluca nije bilo promena. Nije bilo ni metastaza na jetri, kao ni peritonealne diseminacije. Histoloski i imunohistohemijski dokazan je redak sklerozirajuci sincicijalni podtip stromalnog tumora zeluca s imunofenotipskom heterogenoscu s dominantnom imunoekspresijom gena PDGFRA i veoma retkom imunoekspresijom CD117. Postoperacioni tok je protekao bez komplikacija. Godinu i po dana posle operacije bolesnica je bez tegoba i znakova recidiva oboljenja. Zakljucak. Prikazan je redak tip histoloski visokorizicnog stromalnog tumora zeluca, makroskopski pendularnog tipa, koji je odstranjen bez teskoca i gde klinicki nije bilo znakova recidiva oboljenja godinu i po dana od operacije.