Introduction:
The rhabdoid phenotypic characteristic in digestive tumors is exceptional. In the
jejunum, the primitive undifferentiated adenocarcinoma rarely grows up to invade other
organs and appears as a cavitated mass.
Case report:
A 64 years-old patient with multifactorial anemia and transfusional requirements underwent
a normal gastroscopy and colonoscopy. This last procedure confirmed a 2 cm flat lesion
in the right colon and the histopathological analysis reported adenocarcinoma.
Furthermore, a CT confirmed a mass dependent of small bowel and a PET-CT reported
mesenteric implants with an extension on both adrenal glands and swollen lymph nodes.
The biopsy of a peripheral adenopathy was an undifferentiated adenocarcinoma.
Thus, an oral double-balloon enteroscopy was performed. The procedure showed a large
cavitated mass in jejunum consisting of a very irregular mucous membrane with proliferative,
necrotic areas and diffuse as well as a ongoing mild-bleeding. Biopsies were taken.
The enteroscope could assess the mass intracavitary, in retroversion, and the "outcoming"
inside of the small bowel was visualized.
Finally, an undifferentiated rhabdoid carcinoma (vimentin, CKae1-be3 +) was confirmed
by the histopathological analysis, excluding GIST or neural origin. The CT ruled out
pneumoperitoneum, showing peritoneal and pleural neoplastic progression. Subsequent
pathologic study of all samples of the various organs gave similar results. The patient
was treated by chemotherapy.
Conclusions:
Small bowel undifferentiated rhabdoid carcinoma is rare, with a great aggressiveness
and bad prognosis. At this location, there are only 11 cases described, almost the
half of them in the jejunum. This case has affected other organs (including colon)
with multiorgan metastases. Endoscopically, a big and cavitated jejunal is also a
rarity, only described in eccentrics GIST. There are no previous publications describind
this entity by flexible enteroscopy.
