AbstractThe management of patients with an incidentally detected pancreatic cyst is a significant clinical challenge. More than 80% are detected as incidental findings on CT scan or MRI. A case is presented of a 41-year-old patient with an asymptomatic pancreatic cyst which was detected during a check-up

Cano, Fernando

Chable-Montero, Fredy

Chan, Carlos

Felix-Saguchi, Carlos Isao

Gómez-Gutiérrez, Cristina

Téllez-Ávila, Félix Ignacio

Elsevier - Publisher Connector 

Endoscopia. 2016;28(2):67--70
www.elsevier.es/endoscopia
ENDOSCOPIA
CLINICAL CASE
Intraductal  papillary  mucinous  neoplasia  and
pancreatic cancer:  A  rare  but real  association
Cristina Gómez-Gutiérreza, Carlos Chanb, Fredy Chable-Monteroc,
Fernando Canoc, Carlos Isao Felix-Saguchid, Félix Ignacio Téllez-Ávilae,∗
a Gastroenterology  Department,  Medica  Sur  Clinic  and  Foundation,  Mexico  City,  Mexico
b Surgery  Department,  Instituto  Nacional  de  Ciencias  Médicas  y  Nutrición  Salvador  Zubirán,  Mexico  City,  Mexico
c Pathology  Department,  Medica  Sur  Clinic  and  Foundation,  Mexico  City,  Mexico
d Radiology  Department,  Medica  Sur  Clinic  and  Foundation,  Mexico  City,  Mexico
e Gastrointestinal  Endoscopy  Department,  Instituto  Nacional  de  Ciencias  Médicas  y  Nutrición  Salvador  Zubirán,  Mexico  City,
Mexico
Received 17  November  2015;  accepted  25  January  2016
Available  online  30  March  2016
KEYWORDS
Intraductal  papillary
mucinous  neoplasm;
Pancreas;
Diagnosis;
Tumors;
Computed
tomography
Abstract  The  management  of  patients  with  an  incidentally  detected  pancreatic  cyst  is  a  sig-
niﬁcant clinical  challenge.  More  than  80%  are  detected  as  incidental  ﬁndings  on  CT  scan  or  MRI.
A case  is  presented  of  a  41-year-old  patient  with  an  asymptomatic  pancreatic  cyst  which  was
detected during  a  check-up.
© 2016  Published  by  Masson  Doyma  Me´xico  S.A.  on  behalf  of  ASOCIACIO´N  MEXICANA  DE  ENDO-
SCOPIA GASTROINTESTINAL  A.C.  This  is  an  open  access  article  under  the  CC  BY-NC-ND  license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
PALABRAS  CLAVE
Neoplasia  papilar
mucinosa  intraductal;
Páncreas;
Diagnóstico;
Neoplasia  papilar  mucinosa  intraductal  y  desarrollo  de  cáncer  de  páncreas:  poco
frecuente  pero  real
Resumen  El  tratamiento  de  los  pacientes  con  un  quiste  pancreático  incidentalmente  detec-
tado es  un  reto  clínico  considerable.  Más  del  80%  son  detectados  como  hallazgos  incidentalesTumores; durante  la  realización  de  una  TAC  o  RM.  Presentamos  un  caso  de  un  paciente  de  41  an˜os  con  un
quiste pancreático  asintomático  detectado  durante  una  revisión.Tomografía
computarizada © 2016  Publicado  por  Masson  Doyma  Me´xico  S.A.  en  nombre  de  ASOCIACIO´N  MEXICANA  DE  ENDO-
SCOPIA GASTROINTESTINAL  A.C.  Este  es  un  art´ıculo  Open  Access  bajo  la  licencia  CC  BY-NC-ND
(http://creativecommons.org/licenses/by-nc-nd/4.0/).∗ Corresponding author at: Department of Endoscopy, Instituto Nacional
#15, Col. Sección XVI, Del. Tlalpan, Mexico CP 14000, Mexico. Tel.: +52 5
E-mail address: felixtelleza@gmail.com (F.I. Téllez-Ávila).
http://dx.doi.org/10.1016/j.endomx.2016.01.003
0188-9893/© 2016 Published by Masson Doyma Me´xico S.A. on behalf o
This is an open access article under the CC BY-NC-ND license (http://cre de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga
54870900; fax: +52 554870900.
f ASOCIACIO´N MEXICANA DE ENDOSCOPIA GASTROINTESTINAL A.C.
ativecommons.org/licenses/by-nc-nd/4.0/).
6 C.  Gómez-Gutiérrez  et  al.
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Figure  2  EUS-FNA  reported  a  cystic  tumor  in  the  pancreas
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creas  that  measured  20  ×  11  mm  (Fig.  1).  In January  2015,  an8  
ntroduction
ntraductal  papillary  mucinous  neoplasia  (IPMNs)  was  ﬁrst
escribed  in  1982.  Since  then,  its  classiﬁcation  and  man-
gement  have  evolved  dramatically.1 The  true  prevalence
f  IPMNs  is  unknown.  Most  patients  are  asymptomatic  at
he  time  of  diagnosis,  and  the  cysts  are  usually  discovered
s  an  incidental  ﬁnding  on  imaging.2 The  use  of  computed
omography  (CT)  and  magnetic  resonance  imaging  (MRI)
ay  partially  explain  the  recent  rise  in  prevalence  of  these
esions.3 The  detection  rate  of  pancreatic  cysts  by  conven-
ional  MRI  has  been  estimated  to  be  as  high  as  13.5%  in
symptomatic  populations.4 IPMNs  represent  about  20--50%
f  all  pancreatic  cystic  neoplasms  and  38%  of  resected  pan-
reatic  cysts.  The  average  age  of  diagnosis  peaks  at  60--70
ears  old,  with  a  slightly  higher  prevalence  in  men  and  with
ecurrent  acute  pancreatitis  without  recognized  biliary  or
lcoholic  etiology  as  the  most  frequent  expression.5 There
re  three  varieties,  the  main  duct  tumor  (MDT),  the  branch
uct  tumor  (BDT),  and  mixed,  having  different  incidences  of
alignancy  and  prognoses.6 The  MDT  has  a  frequency  malig-
ancy  of  62%  (36--100%)  and  BDT  has  a  2--3%  of  malignancy
nnual  risk.7 At  the  time  of  diagnostics  of  IPMNs,  approxi-
ately  10--20%  of  cases  harbor  invasive  carcinoma,  10--20%
n  situ  carcinoma,  and  the  remaining  60--80%  intraductal  or
ntracystic  adenoma  with  low  degree  dysplasia.  There  are
ome  risk  factors  for  malignancy  in  IPMNs  such  as  size  ≥3  cm,
 dilated  main  pancreatic  duct,  or  the  presence  of  an  asso-
iated  solid  component,  and  if  there  present  more  than  2
isk  factors,  should  be  performed  de  EUS--FNA  that  has  a
ensitivity  of  approximately  60%  and  a  speciﬁcity  of  90%  for
he  risk  of  malignancy  in  IPMNs.8 The  molecular  analyses  of
he  cyst  ﬂuid  for  diagnosis  are  still  evolving  and  there  is  not
arker  that  distinguishes  benign  from  malignant.7 Histolog-
cally,  the  gastric  type  is  typically  low  grade,  with  normally
mall  percentage  developing  into  carcinoma,  although  if  a
arcinoma  does  develop  in  these  patients,  it  is  usually  of  the
ubular  type  and  behaves  a  conventional  pancreatic  duc-
al  adenocarcinoma.  In  the  published  series,  resectability
n  patients  with  IPMNs  was  90--100%,  while  mortality  and
igure  1  CT  scan  with  hypodense  cystic  lesion  located  in
he head  and  uncinate  process  of  the  pancreas  that  measures
0 ×  11  mm.
e
(
w
F
i
i
aead with  a  diameter  of  3.4  ×  3.2  cm,  without  vascular  involve-
ent, and  with  no  communication  with  the  pancreatic  duct
bserved.
orbidity  in  patients  with  cancer  related  to  IPMN  were
imilar  to  those  related  to  pancreatic  surgery  for  ductal
denocarcinoma.  Five-year  survival  is  100%  for  adenomas,
0--90%  for  in  situ  carcinomas,  and  50--70%  for  invasive
arcinomas.8--10 Therefore,  an  integral  approach  is  impor-
ant  to  make  a correct  diagnosis  and  initiate  the  proper
anagement  as  soon  as  possible.
ase presentation
n  November  2014,  a 41-year-old  female  with  an  asymp-
omatic  history  was  admitted  for  general  check-up.  She
ad  relevant  health  record,  and  on  physical  examination
he  only  thing  that  stood  out  was  a  BMI  of  27  kg/m2.  Dur-
ng  studies,  non-contrast  CT  detected  a  hypodense  cystic
esion  located  in  the  head  and  uncinate  process  of  the  pan-ndoscopic  ultrasonography-guided  ﬁne-needle  aspiration
EUS-FNA)  was  made,  with  a  heterogeneous  cystic  tumor,
ith  solid  part,  located  in  the  head  of  the  pancreas  without
igure  3  The  histopathological  ﬁndings  were  compatible  with
nﬁltrative  ductal  adenocarcinoma  of  intestinal  type  originating
n intraductal  papillary  mucinous  neoplasia  with  dysplasia  high
nd low  degree.
IPMN  and  pancreatic  cancer  69
d
T
c
DFigure  4  Surgical  specimen  with  no  involvement  of  main  pan-
creatic  duct.
vascular  involvement,  no  communication  with  the  pancre-
atic  duct  and  main  pancreatic  duct  in  a  maximum  diameter
of  3  mm  in  the  head.  The  lesion  was  suggestive  of  IPMNs  of
BDT  (Fig.  2).  The  cytological  abnormalities  were  compatible
with  BD-IPMN  with  moderate  dysplasia,  and  no  adenocarci-
noma  cytological  data  were  identiﬁed.  In  August  2015,  the
patient  underwent  Whipple  surgery  with  preserving  pylorus.
The  histopathological  ﬁndings  were  compatible  with  inﬁltra-
tive  ductal  adenocarcinoma  of  intestinal  type  originating  in
BD-IPMN  with  dysplasia  high  and  low  degree  with  surgical
margins  tumor-free  and  no  affection  of  main  pancreatic
T
(
a
s
Any of the following high-risk stigmata for malignancy:
1. Obstructive jaundice with head injury pancreas
2. Solid component reinforces intralesional
3. Main pancreatic duct>10mm
Yes
Yes Any of the following characteristics:
1. Mural nodules
2. Damage to the pancreatic duct
3. Suspected / positive cytology
Present any of the following characteristics:Consider
surgery
No
1. Pancreatitis
2. Above 3 cm
3. 5-9 mm main pancreatic duct
4. Mural nodule that does not enhance
5. Abrupt change in caliber of the pancreatic d
Yes, perform EUS
<1cm
CT/MRI in 2-3 years
1-2 cm
MRI/CT annually for
two years, then
extended
Figure  6  Algorithm  for  diagnosisFigure  5  MUC2  and  CK19  positive.
ucto  (Figs.  3  and  4),  MUC2  and  CK19  positive  (Fig.  5).
he  post-surgical  evolution  was  favorable  with  hospital  dis-
harge.
iscussionhis  year,  the  American  Gastroenterological  Association
AGA)  presented  its  guidelines  for  the  management  of
symptomatic  neoplastic  pancreatic  cysts  and  a decision
upport  tool  for  clinicians.  Most  of  the  guidelines  are
uct with distal atrophy
No
Lesion size
Undeterminated
No
2-3 cm
EUS 3-6 months, then
extend the range
>3cm
Closely monitored with
MRI and EUS every 3-6
months
 of  cystic  pancreatic  lesions.
7c
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R
10. Baiocchi GL, Molﬁno S, Frittoli B. Increased risk of sec-0  
onditional  recommendations  based  on  very  low  quality  evi-
ence.  Based  on  retrospective  cohorts,  the  prevalent  risk
f  malignancy  in  cysts  >3  cm  compared  with  those  <3  cm
n  size  mildly  increased,  that  increased  the  risk  of  malig-
ancy  approximately  3  times,  as  well  as  the  risk  of  cancer
n  cysts  associated  with  a  dilated  main  pancreatic  duct  and
olid  component,  the  latter  increased  the  risk  of  malignancy
pproximately  8  times.8 These  recommendations  are  con-
istent  with  the  Sendai  guidelines  (Fig.  6).  In  the  case  of
ur  patient,  the  cyst  was  detected  during  a  CT  scan  for  a
heck-up.  The  hypodense  pancreatic  cystic  lesion  measured
0  ×  11  mm  in  CT,  without  any  of  the  components  of  malig-
ancy  risk.  The  AGA  recommends  doing  an  MRI  every  two
ears  after  a  one-year  follow-up  for  cysts  without  >2  high-
isk  features,  then  stopping  surveillance  entirely  for  cysts
hat  have  no  concerning  features  and  seem  stable  by  MRI
maging  for  >ﬁve  years,  regardless  of  size.  We  have  to  recon-
ider  to  base  our  decisions  on  a  particular  patient  guided
nly  by  the  size  of  the  lesion.  Our  patient  is  an  example  of
 natural  history  of  IPMN  and  risk  of  malignancy.
thical responsibilities
rotecting  people  and  animals.  The  authors  declare  that
his  research  has  not  been  conducted  experiments  on
umans  or  animals.
ata  privacy.  The  authors  declare  that  this  article  does  not
ppear  patient  data.
ight  to  privacy  and  informed  consent.  The  authors
eclare  that  no  patient  data  appears  in  this  article.unding
he  authors  did  not  receive  sponsorship  to  undertake  this
rticle.C.  Gómez-Gutiérrez  et  al.
onﬂict of interests
he  authors  declare  no  conﬂict  of  interest.
eferences
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Intraductal papillary mucinous neoplasia and pancreatic cancer: A rare but real association 

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Intraductal papillary mucinous neoplasia and pancreatic cancer: A rare but real association

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