Persistent Elevation of Serum

Department of Internal Medicine and Gastroenterology, Bentivoglio Hospital. Bologna, Italy
Context Serum CA 19-9 is a marker widely
used for digestive malignancies. However, in
various benign diseases its elevation can also
be observed; obstructive jaundice, liver
disease, acute and chronic pancreatitis,
diabetes mellitus, interstitial pulmonary
disease and collagen vascular disease are such
examples. We report a case of marked and
persistent increase of serum CA-19-9 in a
patient, currently in healthy conditions, who
was admitted to our hospital because of
severe diarrhoea.
Case report A 70-year-old woman was
referred to our unit in 2002 for acute watery
diarrhoea, lower left quadrant pain, vomit and
fever. In her past medical history there was an
episode of acute diarrhoea in 1960 and an
endoscopic resection of a villous adenoma
measuring 1.5 cm with low grade dysplasia in
1981. At admission, blood tests were normal
except for C-reactive protein (17.5 mg/dL),
haemoglobin (11.4 g/dL), albumin 2.2 g/dL,
and CA 19-9 (514 U/mL). Abdominal
ultrasonography, upper gastrointestinal series
and endoscopy were negative. Abdominal
CT-scan revealed normal liver and pancreas,
but thickened wall of the right colon and a
paracolic fluid collection. Colonoscopy
showed lesions that were characterized by
having a cobblestone surface, and exudative
yellow plaques from the transverse colon to
cecum
with hyperaemia
and focal
haemorrhage. Histology demonstrated the
presence of chronic, non specific,
inflammation of the lamina propria. Initial
treatment with mesalazine and antibiotics was
ineffective, while methylprednisolone, 60 mg
i.v. dramatically ameliorated the symptoms
within 2 weeks. The patient was discharged in
apparently healthy conditions. Serum CA 19-
9 was determined repeatedly: initially values
fluctuated from 620 U/mL to normal values
(in two sessions), but starting from 2003
levels were consistently elevated and a test
performed in march 2005 showed markedly
high values (1,331 U/mL). During follow up
she underwent abdominal ultrasonographies,
upper and lower GI endoscopies and CT scans
which were always normal. A positron
emission tomography performed in 2004 with
18F-FDG was also negative. At present, the
patient is in an apparently healthy condition,
her weight is stable, appetite is conserved and
bowel movements are regular. Current blood
tests are normal except for high triglyceride
(264 mg/dL) and cholesterol (225 mg/dL)
levels. Nitrites, leukocyte and bacteria are
present in urine. Serum CA 19-9 value is 528
U/mL, while all the other tumour markers are
normal.
MRCP
and
endoscopic
ultrasonography for the study of the pancreas
will be performed in the near future.
The Surgical Treatment of Neuroendocrine Pancreatoduodenal
Tumors in Multiple Endocrine Neoplasia Type 1
Zanini N, Casadei R, Tomassetti P, Calculli L, Pezzilli R, Migliori M, Campana D,
Pagogna S, Minni F
Dipartimento di Scienze Chirurgiche e Anestesiologiche, Chirurgia Generale ‘Minni’,
Policlinico S.Orsola-Malpighi. Bologna, Italy
Background Multiple endocrine neoplasia
type 1 (MEN 1) is an autosomal dominant
inherited syndrome, with significant
variability in its clinical expression. It is
classically characterized by tumors of the
parathyroid glands, pancreatic islets and

Page 59
JOP. J Pancreas (Online) 2005; 6(5 Suppl):477-535.
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JOP. Journal of the Pancreas – http://www.joplink.net – Vol. 6, No. 5 Supplement – September 2005. [ISSN 1590-8577]
535
anterior pituitary gland. About 35% to 75% of
individuals with MEN-1 developed
neuroendocrine neoplasms of the pancreas or
duodenum. They are often malignant tumors
and represent the most common MEN-1
related cause of death in MEN-1 kindreds.
Aim To evaluate our experience in the
surgical treatment of neuroendocrine
pancreaticoduodenal tumors in 12 cases of
MEN-1 observed from 1985 to 2005.
Methods From 1985 to 2005, 12 cases of
patients affected by MEN-1 syndrome, from 5
families, were observed in our Surgical
Department: 11 (91.7%) were male, 1 (8.3%)
female; in 12 out of 12 cases (100%)
pancreaticoduodenal involvement was
revealed at a mean age of 43 years (range 21-
76 years). Tumors were multiple in 9 (75%)
cases, single in 3 (25%) with a maximum size
of 10 cm and minimum of few mm. In 8
(66.7%) cases the tumors were localized in
the pancreatic parenchyma: 7 in body-tail, 1
in tail. In 4 (33.3%) cases pancreas and
duodenum were involved: 2 duodenum plus
pancreatic tail, 1 duodenum and pancreatic
head and, finally, 1 duodenum and the
pancreatic parenchyma diffusely. In 10
(83.3%) cases the histotype was single (6 non
functioning, 3 gastrinomas, 1 insulinoma), in
2 (16.7%) multiple (non functioning plus
insulinomas plus gastrinomas and
insulinomas plus gastrinomas). Nine (75%)
patients underwent surgical treatment with 10
pancreatic
resection:
8
subtotal
pancreatectomies, 1 total pancreatectomy and
1 enucleoresection of the tumors.
Results Patients not surgically treated (n=3):
1 lost to follow-up; 2 alive with stable disease
with medical therapy. Patients surgically
treated (n=9): total recurrence 5 (55.5%) of
which recurrence and no reoperation 4 cases,
recurrence and operation 1 case. Eight
patients are alive (median follow-up of 22
years and 6 months) of which 3 alive and
disease free and 5 alive with disease (2 with
pancreatic recurrence and hepatic metastases,
1 with duodenal gastrinomas and hepatic
metastases, 1 with duodenal gastrinomas, and
1 with gastric carcinoids). One patient dead
for the disease after 7 years and 4 months
from the first operation.
Conclusion Pancreaticoduodenal tumors in
patients affected by MEN-1 syndrome have to
be treated as soon as possible to prevent
hepatic metastases and the progression of the
disease. The extension of the pancreatic
resection have to be consider for the disease
diffusion and the high frequency of the
recurrences. We suggest large pancreatic
resection until total pancreatectomy.

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