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Abstracts of the 22
nd
National Congress of Digestive Diseases / Digestive and Liver Disease 48S2 (2016) e67–e231
e151
Background and aim:
Neuroendocrine neoplasms (NENs) are
a heterogeneous group of neoplasms, which differ in biologic
behavior, histologic features and response to treatment.
The behavior and therapeutic options of NENs depend on the
location of the primary tumors.
NENs with unknown primary site (NENs-UP) account for 10 to 13
percent of all NENs.
Aim:
To describe clinical and histological findings and the diagnostic
work-up of patients with histologically proven metastatic NENs of
unknown primary.
Material and methods:
Study period: Between January 2005 to
January 2015, 93 patients with metastatic gastrointestinal NENs
were referred to our Institution.
Patients:
Among 93 patients, 17 (18%) presented with immuno
histochemically proven neuroendocrine metastases, without
evidence of primary site.
Of these, 11 were male and six female. The median age at the
diagnosis was 64 years (range 33-74). In all the cases, an exhaustive
work-up based on computed tomography (CT), magnetic resonance
(MRI) and somatostatin receptor scintigraphy (SRS) )/ Gallium-68
PET was performed.
Results:
14 of the 17 patients with NENs-UP (82%), showed
hepatic metastases, whereas in three patients an abdominal nodal
involvement was detected.
In 14 of the 17 cases (82%), liver or nodal metastases were firstly
diagnosed by abdominal ultrasound, performed during surveillance
of chronic diseases in six patients and for gastrointestinal symptoms
in eight.
16 of the 17 tumors (88%) were well differentiated (G1 in five cases
and G2 in 11), with a poorly differentiated carcinoma in one patient.
Hormonal syndrome was diagnosed in 11 cases (65%). In particular
carcinoid syndrome was present in 8 patients, Zollinger-Ellison
syndrome in two and Verner-Morrison syndrome in one.
In the course of a strict work-up, the primary tumor was diagnosed
in 12 cases (71%), after a median of 8.5 months (range 3-120), of
these eight (67%) had a functioning form.
The primary site was identified as pancreas by a repeated abdominal
CT (#3); terminal ileum (#2) and colon (#1) by colonoscopy;
central ileum by double balloon enteroscopy (#1) and pancreas by
endoscopic ultrasound (EUS) (#1).
Again, laparoscopy identified a jejunal, ileal, Meckel’s diverticulum
and pancreatic primary tumor in further four patients.
In our series, five cases are still classified as NENs-UP.
Conclusions:
Despite the continuous advances in diagnostic
techniques, including radiologic, endoscopic and immunohisto
chemical methods, metastatic NENs still represent a clinical
challenge, involving multidisciplinary expertise.
In the present series, NENs remain of unknown primary origin in
a relevant proportion of cases (5/93, 5.3%), even after an in-depth
work-up. Data from our series suggest that NENs-UP may derive
more frequently from the gastrointestinal tract. The presence of
hormone-related symptoms may help to better localize the primary
site.
P.05.10
DUODENAL NEUROENDOCRINE TUMORS – DATA FROM A SINGLE
CENTRE
Rossi R.E.*, Cavalcoli F., Conte D., Massironi S.
Department of Gastroenterology and Endoscopy, Fondazione IRCCS
Ca’ Granda, Ospedale Maggiore Policlinico, and Department of
Pathophysiology and Organ Transplantation, Università degli Studi di
Milano, milano, Italy
Background and aim:
Duodenal neuroendocrine neoplasms
(D-NENs) are heterogeneous tumors, previously classified as foregut
NET, whose prognosis can be good as for gastric neuroendocrine
tumors or bad when facing with more aggressive forms. Their
optimal management remains to be clarified, even if endoscopic
resection is increasingly performed instead of surgery. Present
series was aimed at reporting a single-centre experience.
Material and methods:
Retrospective analysis of patients with
histologically confirmed diagnosis of D-NENs managed at our
Institution.
Results:
From 2004 to 2014, 12 patients (9 M and 3 F, median
age of 67 years, range 26-79) were diagnosed and treated. The
D-NEN was single in all but one patient who was diagnosed with
MEN1 syndrome and had both a D-NEN and multiple pancreatic
NENs. Two patients had a peri-ampullary D-NEN. The median
diameter was 20 mm (range 5-37). A non-functioning D-NEN was
incidentally diagnosed in seven cases, a gastrinoma in three and
a somatostatinoma in two cases, respectively. According to 2010
WHO classification, D-NEN was G2 in three cases and G1 in nine.
At enrollement, four patients (33%) had metastases, to lymph nodes
(#2), liver (#1) and both (#1). D-NEN was removed in 10 cases
(83%), endoscopically (#3) or surgically (#7). An elder patient, with
8 mm non metastatic gastrinoma unsuitable for surgery, was only
followed-up. One patient was lost at follow-up. Over a median
follow-up of 33 months (range 2-133), two patients died of the
disease (metastatic somatostatinoma and gastrinoma, repectively,
both progressive after surgery and not responsive to somatostatin
analog, chemotherapy and peptide receptor radionuclide therapy
treatments).
Conclusions:
D-NENs may be metastatic at the diagnosis in up to 33%
of the cases, thus nuclear imaging should be performed to exclude
distant metastases. Endoscopy and surgery play a primary role in
the management of the disease. Prognosis may be highly variable.
Further studies are needed to better define standardised dedicated
guidelines for D-NENs, including the optimal management and the
perfect follow-up intervals.
P.05.11
UNUSUAL ONSET OF COLONIC SARCOIDOSIS: A CASE REPORT
Vigliardi G.*, Pastena F., Erra P.
U.O.C. Chirurgia Generale Ospedale “F.Veneziale”, Isernia, Italy
Background and aim:
Sarcoidosis is a multisystem chronic
inflammatory condition of unknown etiology that has the potential
to involve every tissue in the body. Sarcoidosis in the gastrointestinal
system, and particularly the colon, is very rare.
Material and methods:
We report a case of a 57-year-old man in
apparent good health who presented with newly onset abdominal
pain and symptoms related to colonic obstruction. The patient
was healthy until three to four weeks prior to presentation, when
new-onset constipation developed and he began passing pencil-
like stools. A physical examination of the patient revealed mild
abdominal distention and tenderness of the right lower abdominal
quadrant. Routine hematology and biochemistry analyses were
normal; Carcinoembryonic antigen levels were slightly elevated (3.6
ng/mL). A colonoscopy revealed a stenotic obstructive lesion at the
cecum-ascending colon transition and we were unable to advance
the colonoscope beyond this area. Biopsy specimens obtained from
this site showed no evidence of a neoplastic lesion, but rather an
acute inflammatory infiltration of the submucosa. An abdominal CT
revealedmarked symmetric concentricwall thickening in the cecum-
ascending colon, which notably reduced the enteral lumen and was
associated with a heterogeneous hyperdensity of perivisceral fat
tissue and multiple satellite lymphadenopathies along the ileocolic
vessels. A malignant colonic lesion was suspected, despite the
unclear histology results. The patient underwent an exploratory
laparotomy.