Primary omental gastrointestinal stromal tumor (GIST).
BACKGROUND
We report herein a rare case of primary omental gastrointestinal stromal tumor (GIST).


METHODS
A 65 year-old man was referred to our hospital with a huge abdominal mass occupying the entire left upper abdomen as shown by sonography. On computed tomography (CT), this appeared as a heterogeneous low-density mass with faint enhancement. Abdominal angiography revealed that the right gastroepiploic artery supplied the tumor. With such an indication of gastric GIST, liposarcoma, leiomyosarcoma or mesothelioma laparotomy was performed and revealed that this large mass measured 20 x 17 x 6 cm, arising from the greater omentum. It was completely resected. Histopathologically, it was composed of proliferating spindle and epithelioid cells with an interlacing bundle pattern. Immunohistochemically, the tumor was positive for myeloid stem cell antigen (CD34), weakly positive for c-KIT (CD117) and slightly positive for neuron-specific enolase (NSE), but negative for cytokeratin (CK), alpha-smooth muscle actin (SMA) and S-100 protein. A mutation was identified in the platelet-derived growth factor alpha (PDGFRA) juxtamembrane domain (exon 12, codon561) and the tumor was diagnosed as an omental GIST. The postoperative course was uneventful. The patient is treated by Glevec(R) and is alive well with no sign of relapse.


CONCLUSIONS
Our case demonstrated a weak immunohistochemical expression of c-kit (CD117) and a point mutation in PDGFRA exon 12 resulting in an Asp for Val561 substitution. Imatinib therapy as an adjuvant to complete resection has been carried out safely. Because of the rarity of primary omental GISTs, it is inevitable to analyze accumulating data from case reports for a better and more detailed understanding of primary omental GISTs.
Todoroki
Takeshi
T
Department of Surgery, Motojima General Hospital, Ota, 373-0033 Japan. todoroki@ka2.so-net.ne.jp
Sano
Takaaki
T
Sakurai
Shinji
S
Segawa
Atsuki
A
Saitoh
Tamotsu
T
Fujikawa
Koichi
K
Yamada
Shuji
S
Hirahara
Nobutsune
N
Tsushima
Yoshito
Y
Motojima
Ryuji
R
Motojima
Teiji
T
D000368:Aged; D000792:Angiography; D001549:Benzamides; D001707:Biopsy, Needle; D017024:Chemotherapy, Adjuvant; D005500:Follow-Up Studies; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery*; D006801:Humans; D000068877:Imatinib Mesylate; D007150:Immunohistochemistry; D007813:Laparotomy / Q000379:methods; D008297:Male; D009852:Omentum / Q000473:pathology*; D010534:Peritoneal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery*; D010879:Piperazines / Q000008:administration & dosage; D011743:Pyrimidines / Q000008:administration & dosage; D018570:Risk Assessment; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler
D001549:Benzamides; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate