Fever and an Abdominal Mass .pdf



Nom original: Fever and an Abdominal Mass.pdfTitre: Case 24-2017Auteur: Shah Uzma, Goldstein Allan M., Gee Michael S., Deshpande Vikram

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The

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Case Records of the Massachusetts General Hospital
Founded by Richard C. Cabot
Eric S. Rosenberg, M.D., Editor
Virginia M. Pierce, M.D., David M. Dudzinski, M.D., Meridale V. Baggett, M.D.,
Dennis C. Sgroi, M.D., Jo‑Anne O. Shepard, M.D., Associate Editors
Allison R. Bond, M.D., Case Records Editorial Fellow
Emily K. McDonald, Sally H. Ebeling, Production Editors

Case 24-2017: An 8-Month-Old Girl
with Fever and an Abdominal Mass
Uzma Shah, M.D., Allan M. Goldstein, M.D., Michael S. Gee, M.D., Ph.D.,
and Vikram Deshpande, M.D.​​

Pr e sen tat ion of C a se
From the Departments of Pediatrics (U.S.),
Pediatric Surgery (A.M.G.), Radiology
(M.S.G.), and Pathology (V.D.), Massa‑
chusetts General Hospital, and the De‑
partments of Pediatrics (U.S.), Surgery
(A.M.G.), Radiology (M.S.G.), and Pa‑
thology (V.D.), Harvard Medical School
— both in Boston.
N Engl J Med 2017;377:574-82.
DOI: 10.1056/NEJMcpc1616393
Copyright © 2017 Massachusetts Medical Society.

574

Dr. Rajitha Venkatesh (Pediatrics): An 8-month-old girl was admitted to this hospital
because of fever and an abdominal mass.
The patient had been in her usual good health until approximately 6 days before
admission, when she passed a large stool surrounded by clotted blood. During the
next 4 days, she had intermittent fevers with associated irritability, as well as decreased oral intake without emesis. She continued to produce 6 to 8 wet diapers
per day and to pass soft, brown stools. During the day before admission, a mild
cough developed. The patient was evaluated in the office of her pediatrician, and
abdominal ultrasonography was scheduled for the next day. That evening, her
temperature rose to 40.3°C; her parents administered ibuprofen and took her to
the emergency department of another hospital.
On examination at the other hospital, the patient appeared to be in mild discomfort. The temperature was 39.4°C, the blood pressure 102/58 mm Hg, the pulse
170 beats per minute, the respiratory rate 32 breaths per minute, and the oxygen
saturation 99% while she was breathing ambient air. The weight was 9.53 kg (93rd
percentile). The fontanelle was open, soft, and flat. The abdomen was moderately
distended, with normal bowel sounds and no apparent tenderness on palpation;
the remainder of the examination was normal. Acetaminophen was administered,
and the temperature decreased to 36.1°C. Analysis of a urine specimen obtained by
means of catheterization revealed a pH of 5.0, a specific gravity of 1.005, a small
amount of blood, and a urobilinogen level of 0.2 mg per deciliter and was otherwise normal. A blood specimen could not be obtained.
Dr. Michael S. Gee: Abdominal radiography (Fig. 1A), performed with the patient
in an upright position, revealed the absence of bowel gas in the midabdomen and
peripheral displacement of multiple bowel loops, findings suggestive of an abdominal mass; there was a nonobstructive bowel-gas pattern and no free intraperitoneal air or abnormal calcifications. Abdominal ultrasonography (Fig. 1B)
revealed a large, thick-walled complex cystic mass in the midabdomen that had
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Case Records of the Massachuset ts Gener al Hospital

internal dependent debris and multiple mural
satellite cysts. The mass was located predominantly to the left of the midline, displaced the
left kidney laterally, and did not have internal
vascularity on Doppler imaging.
Dr. Venkatesh: The patient was transferred to
this hospital 4.5 hours after her arrival at the
other hospital.
The patient had been born after an uncomplicated gestation of 41 weeks’ duration. When she
was 6.25 months of age (7.5 weeks before this
evaluation), she was seen in the emergency department of the other hospital because she had
four episodes of vomiting over a 3-hour period;
she had associated grunting that was suggestive
of abdominal pain but had no constipation, fever,
or bleeding. On examination at that time, she was
afebrile and weighed 8.15 kg (84th percentile);
she had mild abdominal distention and voluntary guarding but had no palpable mass or organomegaly. Abdominal radiography revealed a nonspecific bowel-gas pattern. She was able to
receive several feedings without complication,
and she returned home.
The patient’s childhood immunizations were
current. She took no medications and had no
known allergies. She lived with her parents, a
sibling, and other relatives. Her maternal grandfather had had colon cancer, and a paternal aunt
had died at 20 years of age because of cancer
that had been initially manifested by an abdominal cyst; there was no family history of
inflammatory bowel disease.
On examination, the patient was fussy but
consolable. The temperature was 36.3°C, the blood
pressure 105/52 mm Hg, the pulse 122 beats per
minute, the respiratory rate 32 breaths per minute, and the oxygen saturation 98% while she was
breathing ambient air. The abdomen was soft
and distended. On palpation, there was a firm
fullness above the pubis but no discrete mass.
The remainder of the examination was unchanged. Blood levels of glucose, calcium, phosphorus, total protein, albumin, globulin, lactate
dehydrogenase, uric acid, amylase, alpha fetoprotein, and human chorionic gonadotropin
were normal, as were the results of coagulation
and liver-function tests; other test results are
shown in Table 1. A blood culture was obtained.
Dr. Gee: Computed tomography (CT) of the
abdomen (Fig. 2A and 2B), performed in accordance with a pediatric (low dose) protocol after
n engl j med 377;6

the administration of intravenous and oral contrast material, revealed a complex cystic mass
(measuring 14.5 cm in greatest dimension) in
the midabdomen. The mass filled the left side of
the abdomen and was separate from the bowel.
Several loculations of the cyst abutted the pancreatic body and tail. The mass did not contain
enteric contrast material, and the attenuation
values of the contents of the mass were consistent with fluid.
Ultrasound-guided percutaneous drainage of
A

B

*

Figure 1. Abdominal Imaging Studies Obtained
at the Other Hospital.
A radiograph (Panel A) shows the absence of bowel
gas in the midabdomen and displacement of adjacent
bowel loops. An ultrasound image (Panel B) shows a
complex cystic mass in the midabdomen, on the left
side, that has internal dependent debris (asterisk) and
a thick wall with additional loculations (arrows); the left
kidney is displaced laterally (arrowhead).

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Table 1. Laboratory Data.*
Reference Range,
Age-Adjusted†

On Admission,
This Hospital

Hematocrit (%)

33.0–39.0

29.1

Hemoglobin (g/dl)

10.5–13.5

9.2

6000–17,500

10,400

Neutrophils

17–49

38.4

Lymphocytes

67–77

49.4

Monocytes

4–11

11.5

Eosinophils

0–8

0.3

Variable

White-cell count (per

mm3)

Differential count (%)

Basophils

0–3

0.1

150,000–450,000

490,000

Sodium (mmol/liter)

135–145

135

Potassium (mmol/liter)

3.4–5.0

4.3

Chloride (mmol/liter)

98–106

100

Carbon dioxide (mmol/liter)

22–27

20

Urea nitrogen (mg/dl)

5–20

3

0.30–1.00

0.23

Magnesium (mg/dl)

1.7–2.4

2.6

Lipase (U/liter)

13–60

797

<8.0

134.4

Platelet count (per mm3)

Creatinine (mg/dl)

C-reactive protein (mg/liter)

* To convert the values for urea nitrogen to millimoles per liter, multiply by 0.357.
To convert the values for creatinine to micromoles per liter, multiply by 88.4.
To convert the values for magnesium to micromoles per liter, multiply by
0.4114.
† Reference values are affected by many variables, including the patient popu­
lation and the laboratory methods used. The ranges used at Massachusetts
General Hospital are age-adjusted for patients who are not pregnant and do
not have medical conditions that could affect the results. They may therefore
not be appropriate for all patients.

the cystic mass was performed with the use of
an 8-French pigtail catheter (Fig. 2C). A 500-ml
sample of cloudy fluid was aspirated and sent for
microbiologic, hematologic, and chemical analysis. Then, iodinated contrast material was injected through the drainage catheter to identify sites
of communication with the cystic mass. On
fluoroscopic images obtained during the injection (Fig. 2D), a curvilinear branching structure
adjacent to the cyst cavity was filled with contrast material. A CT scan was obtained immediately after the injection (Fig. 2E and 2F) to determine the anatomical location of this structure.
During CT, only the necessary amount of radiation was used, in accordance with the Image
Gently principles outlined by the Alliance for

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Radiation Safety in Pediatric Imaging.1 Because
this was a follow-up examination targeted to a
specific anatomical region, a specific pediatric
(low dose) technique was used; the resultant effective dose was 1 mSv, which is equivalent to
less than 1 year of background exposure. The
findings on CT confirmed that the curvilinear
structure filled with contrast material was the
pancreatic duct (Fig. 2E). In addition, contrast
material was seen in the distal duodenum and
proximal jejunum (Fig. 2F); it had presumably
passed through the ampulla.
Dr. Venkatesh: Metronidazole, gentamicin, ampicillin, and fluids were administered intravenously,
and acetaminophen was administered as needed
for fevers. During the next 2 days, the patient
continued to have low-grade fevers.
Diagnostic procedures were performed.

Differ en t i a l Di agnosis
Dr. Uzma Shah: All the discussants are aware of
the diagnosis in this case. A cystic abdominal
mass originates from one of several possible
structures and may be identified incidentally.
Patients with a cystic abdominal mass may present with mass effect, obstruction, abdominal
pain, and infection (Table 2).2 This 8-month-old
girl presented with an abdominal mass and infection.
Findings on the initial imaging studies suggested that the mass was in close proximity to
the pancreas. The elevated lipase level indicated
a possible pancreatic origin. The differential diagnosis of pancreatic cysts is broad.3 Pancreatitis
may be associated with fluid collections around
the pancreas.4 Such fluid collections can be pancreatic pseudocysts, which can have intrapancreatic and extrapancreatic components. Pseudocysts are distinct from congenital cysts, since they
are fluid-filled spaces with a well-defined wall
that has no epithelial lining. Pseudocysts may
communicate with the pancreatic duct. They
may grow to be very large, and they develop at
least 4 weeks after an episode of pancreatitis. In
children, they tend to be associated with acute
pancreatitis and occur most frequently in the
context of traumatic pancreatitis.5 Although the
majority of pseudocysts resolve on their own,
pseudocysts that last longer than 3 months or
those that grow to be very large necessitate treat-

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Case Records of the Massachuset ts Gener al Hospital

A

B

D

C

F

E

Figure 2. Abdominal Imaging Studies Obtained at This Hospital.
CT images (Panels A and B), obtained after the administration of oral and intravenous contrast material, show a
multilobulated cystic mass, with several loculations abutting the pancreas (arrows). A fluoroscopic image (Panel C)
shows percutaneous drainage of the mass with a catheter. A fluoroscopic image obtained during the injection of
contrast material into the cyst cavity (Panel D) shows communication with an adjacent curvilinear structure (arrows).
CT images obtained after the injection (Panels E and F) confirm that the structure is the pancreatic duct (Panel E,
arrow) and ampulla (Panel E, arrowhead) and show contrast material in the proximal jejunum (Panel F, arrows),
findings consistent with pancreatic communication.

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Table 2. Cystic Abdominal Masses.
Hepatobiliary
Congenital cyst
Choledochal cyst
Hamartoma
Caroli’s disease
Hydrops of the gallbladder
Cystic neoplasm
Abscess
Splenic
Congenital cyst
Abscess
Vascular malformation
Renal
Hydronephrosis
Cystic nephroma
Multicystic dysplastic kidney
Wilms’ tumor
Rhabdoid tumor
Mesentery and bowel
Mesenteric cyst
Duplication cyst
Lymphatic malformation
Pancreatic
Inflammatory condition (e.g., pseudocyst or walled-off
necrosis)
Cystic neoplasm
Nonneoplastic condition (e.g., congenital cyst, retention
cyst, mucinous neoplasm, or lymphoepithelial cyst)
Congenital condition (e.g., enterogenous duplication
cyst or pancreatic hamartoma)

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Congenital pancreatic cysts are very rare. They
may be enteric duplication cysts or pancreatic
hamartomas that arise as a developmental aberration. To date, 23 pancreatic hamartomas have
been identified in the literature, and thus, data
and details on the cause of these cysts, their
progression, and the disease course are limited.
The youngest patient in whom a pancreatic hamartoma has been identified was 27 weeks of
age.7-12 Pancreatic hamartomas may grow to be
very large (up to 10 cm in diameter); on histologic examination, they are composed of cysts
that are lined by cuboidal epithelium and may
contain a variety of pancreatic cell types. In some
cases, they have been reported to occur with
asphyxiating thoracic dystrophy and hemihypertrophy. However, a genetic basis has not been
defined. The stromal cells have been reported to
express CD34 and CD117.13
Our initial impression was that this patient
had a large, multilobulated cystic mass that
seemed to be pancreatic in origin. Although
there was an increase in the blood lipase level,
there was no medical history suggestive of acute
pancreatitis or abdominal trauma. In this patient’s age group, abdominal trauma would be a
more likely cause of pancreatitis with a pseudocyst.6 Ultimately, we considered the possibility
that the patient had a congenital cyst rather than
a pseudocyst due to pancreatic inflammation or
injury.

Dr . Uzm a Sh a h’s Di agnosis
Congenital pancreatic cyst.

Dermoid or epidermoid cyst
Ovarian

Discussion of M a nagemen t

Follicular cyst

Dr. Allan M. Goldstein: This otherwise healthy
8-month-old girl presented with fever and poor
Miscellaneous
appetite that had lasted for several days. Her
Urachal cyst
examination was notable for abdominal distenCystic teratoma
tion without tenderness. Abdominal ultrasound
images and subsequently obtained axial CT imAdrenal hemorrhage
ages showed a large, multilobulated cystic mass.
Omental cyst
The presence of an elevated lipase level led to the
Cystic neuroblastoma
presumptive diagnosis of pancreatitis. The absence of hyperamylasemia did not argue against
ment with either endoscopic drainage by means this diagnosis, given the very low level of panof cystogastrostomy or surgical obliteration. creatic amylase production in infants.14 The inAnalysis of the pancreatic fluid tends to reveal a fant was admitted to the hospital, and treatment
very high lipase level.6
with intravenous fluids and broad-spectrum antiCorpus luteum cyst

578

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Case Records of the Massachuset ts Gener al Hospital

biotic agents was begun because of the fever and
elevated C-reactive protein level.
The fevers persisted. On hospital day 4, ultrasound-guided percutaneous drainage of the cystic mass was performed, and a 500-ml sample of
cloudy fluid was obtained. An 8-French pigtail
catheter was left in the largest cystic space. Contrast material was injected into the catheter and
filled a branching structure that resembled the
pancreatic duct. An axial CT image that was
obtained after the injection confirmed that contrast material had filled the pancreatic duct and
showed contrast material in the duodenum.
Analysis of the fluid sample revealed an amylase level of 5000 U per liter and a lipase level
higher than 1 million U per liter, with abundant
neutrophils but no organisms. Cultures of the
fluid sample were negative. After the drainage,
the patient became ill, with tachycardia, fever,
emesis, abdominal tenderness, and leukocytosis.
She was treated in the pediatric intensive care
unit with fluids, antibiotics, bowel rest, and nasogastric decompression, and over the next 3 days,
her condition improved. The catheter drained
200 to 300 ml of clear fluid daily, and the infant
was discharged from the hospital on day 13 with
the catheter still in place. At home, she remained
well; she was afebrile, active, and feeding well,
and the catheter drained approximately 300 ml
daily.
After extensive discussion with the family, the
decision was made to proceed with laparotomy.
The patient had a preoperative diagnosis of either a congenital pancreatic cyst or a pancreatic
pseudocyst. For a congenital cyst, we would perform a complete resection. For a pancreatic pseudocyst, we would perform internal drainage by
means of cystojejunostomy because of the size
and location of the mass.15
Given the possibility that this patient had a
pseudocyst, we performed the operation 5 weeks
after the initial presentation to allow the cyst
wall time to mature and develop the capacity to
hold sutures. The abdomen was entered through
a transverse upper abdominal incision, and a
grapefruit-sized, multilobulated cystic structure
was identified (Fig. 3A). The cysts had thin walls
and were filled with fluid. The mass was clearly
a congenital cyst, rather than a pseudocyst. Therefore, we proceeded with resection of the lesion.
Extensive inflammatory adhesions were bound
to the omentum, colon, and duodenum, and we

surgically divided these. Cephalad retraction of
the stomach revealed that the mass arose from
the inferior aspect of the neck of the pancreas.
This attachment was divided from the pancreas
with the use of a soft-tissue stapler. Care was
taken not only to avoid leaving behind any part
of the cyst wall but also to avoid injuring the
pancreatic duct (Fig. 3B). A catheter was left at
the pancreatic resection margin. The patient was
discharged home on postoperative day 5 with
the catheter in place, since it was still draining
60 ml of lipase-rich fluid daily.

Pathol o gic a l Discussion
Dr. Vikram Deshpande: The resected mass was 14.5 cm
by 11.0 cm by 5.5 cm. Sectioning of the mass
revealed multiple trabeculated, smooth-lined cysts
that contained reddish-brown to cloudy grayishyellow fluid. On microscopic examination, the
lesion was unencapsulated but well circumscribed
and was composed of multiple thin-walled cysts,
some of which were filled with bloody serous
fluid. No solid component was identified. On
histologic examination, the cysts were lined by a
single layer of low cuboidal epithelium (Fig. 3C
and 3D). The cells did not have intracellular mucin or cytologic atypia. Some of the larger cysts
were denuded of epithelium and instead lined by
inflammatory granulation tissue accompanied
by sheets of neutrophils. Lobular aggregates of
smaller cysts surrounded by a dense collar of
cellular mesenchymal stroma were also identified (Fig. 3E). The mesenchymal cells did not
have cellular atypia and were strongly positive
for desmin (Fig. 3F); this immunophenotype is
consistent with a myofibroblastic lineage. The
cellular stroma had a superficial resemblance to
ovarian stroma; however, on immunohistochemical staining, these cells were negative for estrogen receptor. In addition to the presence of
mature-appearing pancreatic tissue at the periphery of the lesion, intratumoral immature-appearing acini without accompanying pancreatic islets
were identified. Immunohistochemical staining
for chromogranin, insulin, and glucagon was performed to identify intralesional islets. Although
entrapped pancreatic islets were identified at the
periphery of the lesion, intratumoral islets were
absent. The regional lymph nodes showed reactive changes.
Pancreatoblastoma is the most common ma-

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A

B

C

D

E

F

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Figure 3. Resection Specimens of the Pancreatic Mass.
Panel A shows a multilobulated cystic mass arising from the pancreas. The stomach is being held toward the pa‑
tient’s head (to the left), and the multilobulated cystic mass is seen arising from the lesser sac, draped over by the
omentum. The catheter is being held (upper right). Panel B shows the pancreatic resection margin after the removal
of the cystic mass. The stomach is retracted cephalad (to the left), and the transverse colon is shown (right). The
pancreatic resection margin, where the cystic mass was removed, is being held by forceps. Hematoxylin and eosin
staining of resection specimens was performed. Panel C shows lobular aggregates of microcysts surrounded by a
cellular mesenchymal stroma. Panel D shows that the cysts are lined by a single layer of low cuboidal epithelium
without cellular atypia. Panel E shows cystic structures surrounded by cellular mesenchymal stroma. An immuno‑
histochemical stain for desmin was performed; Panel F shows that the mesenchymal cells are positive for desmin
and shows the lobular arrangement of the cysts.

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Case Records of the Massachuset ts Gener al Hospital

lignant pancreatic neoplasm to occur in children.
Other pancreatic neoplasms that are reported in
children include solid pseudopapillary neoplasms
and pancreatic endocrine neoplasms. Pseudocysts
are the most common intrapancreatic cystic lesion to occur in children.5 In this case, the lesion
had no histologic resemblance to a neoplasm,
and the presence of epithelial lining ruled out a
pseudocyst. The possibility of a mucinous cystic
neoplasm was also considered; however, the absence of mucin, as well as the absence of estrogen receptor–positive ovarian stroma (a defining
feature of this cystic neoplasm), ruled out this
possibility. The intraductal papillary mucinous
neoplasm is the most common cystic pancreatic
neoplasm to occur in adults; it is distinctly uncommon for this neoplasm to occur in the first
decade of life, but it may arise in children with
a germline STK11 mutation.16 In this case, the
presence of ductal epithelium, immature mesenchymal tissue, and pancreatic acinar tissue is
suggestive of a pancreatic hamartoma.
Pancreatic hamartomas are uncommon in
adults and even less common in children.17,18 The
histologic features of pancreatic hamartomas vary
considerably, but all pancreatic hamartomas contain an abnormal mixture of pancreatic elements,
including ductal, mesenchymal, acinar, and endocrine tissue. In retrospect, it is possible that
some cases of groove pancreatitis, which is
manifested by an inflammatory lesion involving
the head of the pancreas and duodenal wall, may
have been misclassified as hamartomas.19,20
Among the four cystic hamartomatous pancreReferences
1. Goske MJ, Applegate KE, Bulas D, et al.
Image Gently: progress and challenges in
CT education and advocacy. Pediatr Radiol
2011;​41:​Suppl 2:​461-6.
2. Ranganath SH, Lee EY, Eisenberg RL.
Focal cystic abdominal masses in pediatric patients. AJR Am J Roentgenol 2012;​
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3. Basturk O, Coban I, Adsay NV. Pancreatic cysts: pathologic classification, differential diagnosis, and clinical implications.
Arch Pathol Lab Med 2009;​133:​423-38.
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5. Benifla M, Weizman Z. Acute pancre-

atic lesions reported in children,8,9,11,12 a multicystic adenomatoid hamartoma of the pancreas
most closely resembles the lesion seen in this
patient.
Dr. Goldstein: The patient returned to the outpatient clinic 7 days after discharge with fever,
irritability, abdominal pain, and draining of
cloudy fluid. She was admitted to the hospital,
and abdominal CT revealed a fluid collection
(measuring 2 cm by 3 cm) near the pancreatic
body. The fluid was cultured and grew methicillinsusceptible Staphylococcus aureus. The patient was
treated with intravenous antibiotics and was discharged 6 days later with a prescription for oral
antibiotics. The catheter that had been placed at
the time of surgery was removed on the day of
discharge. Follow-up ultrasonography that was
performed 2 weeks later revealed persistence of
the fluid collection near the pancreas, but the
patient was doing well, with no fever and a normal abdominal examination. The antibiotics were
therefore discontinued. Follow-up ultrasonography that was performed 3 months after the operation revealed no residual fluid collection, and
the patient continued to do well, with no fever
or abdominal pain.

A nat omic a l Di agnosis
Congenital pancreatic hamartoma (multicystic
adenomatoid hamartoma).
This case was presented at Pediatric Grand Rounds.
Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.

atitis in childhood: analysis of literature
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Multicystic adenomatoid hamartoma of the
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Pediatr Dev Pathol 2008;​11:​314-20.

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Wang C. Pancreatic hamartoma, a rare
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lesion with immunohistochemical and
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pancreatic hamartoma. Pediatr Radiol
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resembling gastrointestinal stromal tumor:
a solid tumor of the pancreas expressing c-kit (CD117). Mod Pathol 2005;​18:​
1211-6.

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14. Lebenthal E, Lee PC. Development of

functional responses in human exocrine
pancreas. Pediatrics 1980;​66:​556-60.
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DW, Ho KY. Systematic review comparing
endoscopic, percutaneous and surgical
pancreatic pseudocyst drainage. World J
Gastrointest Endosc 2016;​8:​310-8.
16. Jiao Y, Lumpkins K, Terhune J, et al.
Intraductal papillary mucinous neoplasm
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sulinism and a de novo germline SKIL
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Klimstra DS, Klöppel G. Pancreatic solid
and cystic hamartoma in adults: characterization of a new tumorous lesion. Am J
Surg Pathol 2005;​29:​797-800.
18. Yamaguchi H, Aishima S, Oda Y, et al.
Distinctive histopathologic findings of pancreatic hamartomas suggesting their “ham-

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