Pancreatic solid and cystic hamartoma in adults:
characterization of a new tumorous lesion.
Am J Surg
Pathol. 2005 Jun;29(6):797-800.
Nonneoplastic tumor-like lesions ("pseudotumors") of the pancreas
include cystic and noncystic varieties. We report on a solid and
cystic tumor-like lesion of the pancreas that occurred in 2 adult
patients. The lesions, located in the head and neck of the gland,
respectively, were well demarcated and composed of cystic ductal
structures embedded in focally inflamed stromal tissue. In addition,
one of the lesions showed irregularly arranged but
well-differentiated acini and small intralobular and interlobular
ducts embedded in hypocellular, fibrotic tissue. Discrete islets
were lacking, but immunohistochemical staining for chromogranin A
revealed individual scattered endocrine cells evenly distributed
between acinar and ductal cells. The surrounding pancreatic
parenchyma did not show significant chronic pancreatitis. After
tumor removal, the follow-up of the patients was uneventful. Because
of the irregular arrangement of otherwise mature tissue components
of the pancreas, the lesions were considered solid and
cystic hamartomas. Their pathogenesis is so
far unknown.
A case of solid
pancreatic hamartoma in a 58-year-old Japanese woman is presented.
She had no symptoms, and a pancreatic mass was incidentally found on
screening ultrasonography 4 months before admission. The patient was
not alcoholic and had no history of pancreatitis. Physical
examination and laboratory data were unremarkable. Preoperative
imaging demonstrated a nodule in the body of the pancreas, measuring
2.0 cm in maximum diameter, which showed marked delayed enhancement
during dynamic CT. The patient underwent a distal pancreatectomy
under the preoperative diagnosis of pancreatic endocrine tumor and
had an uneventful postoperative course. A well-demarcated solid
nodule, 1.9 cm in diameter, was evident in the body of the pancreas.
Microscopically, the lesion was composed of non-neoplastic,
disarranged acinar cells and ducts embedded in a sclerotic stroma
with elongated spindle cells, lacking discrete islets. The stromal
spindle cells were immunoreactive for CD34 and CD117. The
histological diagnosis was solid hamartoma of the pancreas. There
was no recurrence 5 months after surgery. Herein is reported a case
of solid hamartoma of the pancreas and review of the literature. A
search through the literature found only two cases of solid
hamartoma of the pancreas, among the 14 cases previously reported as
pancreatic hamartoma.
Paraduodenal pancreatitis: a clinico-pathologically
distinct entity unifying "cystic dystrophy of heterotopic pancreas",
"para-duodenal wall cyst", and "groove pancreatitis".Semin
Diagn Pathol. 2004 Nov;21(4):247-54.
A distinct
form of chronic pancreatitis occurring predominantly in and around
the duodenal wall (near the minor papilla) has been reported under
various names, including cystic dystrophy of heterotopic pancreas,
pancreatic hamartoma of duodenum, para-duodenal wall cyst,
myoadenomatosis, and groove pancreatitis. Our experience with these
lesions and the review of the literature show that these lesions
have the following common characteristics: (1) The duodenal wall
contains dilated ducts, some with inspissated secretions, and
pseudocystic changes as well as adjacent stromal reactions including
hypercellular granulation tissue, foreign-body type giant cell
reaction engulfing mucoprotein material, and myofibroblastic
proliferation. (2) Brunner's gland hyperplasia is typically present.
(3) Dense myoid stromal proliferation, with intervening rounded
lobules of pancreatic acinar tissue, creates a histologic picture
reminiscent of "myoadenomatosis," "pancreatic hamartoma," or even
leiomyoma in some cases. (4) Spillover of fibrosis into the adjacent
pancreas and soft tissue occurs, especially in the "groove" area
(between the pancreas, common bile duct and duodenum), including the
region around the common bile duct. (5) Clinically, these lesions
often mimic "pancreas cancer" or periampullary tumors, because of
marked scarring as well as the ill-defined borders of the process.
Patients with these findings are predominantly males, 40-50 years
old, with a history of alcohol abuse. That the process is often
centered in the region of minor papilla (and the adjacent pancreas)
suggests that an anatomic variation of the ductal system may render
this area particularly susceptible to the effects of alcoholic
injury, and the myo-adenomatoid and cystic changes on the duodenal
wall may in turn represent changes related to a localized recurrent
pancreatitis. In conclusion, these clinicopathologic findings
characterize a distinctive process that can be referred to as
paraduodenal pancreatitis.
Groove
pancreatitis and pancreatic heterotopia in the minor duodenal
papilla.Pancreas.
2005 May;30(4):e92-5.
Groove
pancreatitis is a rare form of segmental chronic pancreatitis that
involves the anatomic space between the head of the pancreas, the
duodenum, and the common bile duct. We report 2 cases of groove
pancreatitis with pancreatic heterotopia in the minor papilla.
Patients were a 44-year-old woman and a 47-year-old man. Both had a
past history of alcohol consumption and presented with abdominal
pain, vomiting, and weight loss caused by duodenal stenosis.
Abdominal computed tomography revealed thickening of the duodenal
wall and enlargement of the pancreatic head in both patients. In 1
patient, ultrasound endoscopy showed a dilated duct in the head of
the pancreas. Pancreaticoduodenectomy was performed to rule out
pancreatic adenocarcinoma and because of the severity of the
symptoms. In both cases, gross and microscopic examinations showed
fibrous scar of the groove area. The Santorini duct was dilated and
contained protein plugs in both patients, with abscesses in 1 of
them. In both cases, there were microscopic foci of heterotopic
pancreas with mild fibrosis in the wall of the minor papilla. Groove
pancreatitis is often diagnosed in middle-aged alcoholic men
presenting with clinical symptoms caused by duodenal stenosis. The
pathogenesis of this rare entity could be because of disturbance of
the pancreatic secretion through the minor papilla. Pancreatitis in
heterotopic pancreas located in the minor papilla and chronic
consumption of alcohol seem to be important pathogenic factors.
- Accessory spleen.
Accessory spleen presenting as a mass in the tail of
the pancreas.
Ann Diagn Pathol. 2007 Aug;11(4):277-81.
In this case
report, we describe an accessory spleen that presented as a mass in
the tail of the pancreas and mimicked a neoplasm. Intrapancreatic
accessory spleen have a relatively high prevalence and can be
mistaken for tumors. We present a case of intrapancreatic accessory
spleen in a 40-year-old man, which was discovered incidentally
during a workup for an aortic dissection. Computerized axial
tomography and magnetic resonance imaging scans demonstrated a
hypervascular mass in the tail of the pancreas. The clinical and
radiological differential diagnosis included pancreatic mucinous
cystic neoplasm, pancreatic endocrine neoplasm, solid
pseudopapillary tumor, ductal adenocarcinoma, and metastasis. After
a distal pancreatectomy was completed, microscopic examination
revealed heterotopic splenic tissue.
Intrapancreatic accessory spleen: a differential diagnosis of
pancreatic tumour. Zentralbl Chir. 2007
Feb;132(1):73-6.
BACKGROUND:
According to autoptic studies, accessory spleens may be found in
10-15% of the population and most of them are usually located at or
near the splenic hilum. Only in 1-2% they are located in the
pancreatic tail. We report a rare case of intrapancreatic accessory
spleen which radiologically mimicked a tumor in the tail of the
pancreas. PATIENT: A 54-year-old man was diagnosed with a tumor at
the pancreatic tail. In the preoperative computed tomography (CT),
there was a lesion (2.6 cm in diameter) in the pancreatic tail and
two locoregional lesions (1 and 1.5 cm in diameter), which had
intensive contrast enhancement. The diagnosis of a nonfunctioning
endocrine pancreatic tail carcinoma with lymph node metastasis was
made. RESULTS: Intraoperative examination showed two accessory
spleens nearby the pancreatic tail. As pancreatic cancer could not
be excluded because of the local findings, an oncological left
pancreatectomy was performed. Histological examination excluded
cancer and revealed an intrapancreatic accessory spleen and two
accessory spleens nearby the pancreatic tail. CONCLUSION:
Intrapancreatic accessory spleen should be included in the
differential diagnosis of pancreatic neoplasm.
Intrapancreatic accessory spleen. A rare cause of a pancreatic mass.
Int J Pancreatol. 1999 Feb;25(1):65-8.
CONCLUSION:
The clinical significance of intrapancreatic accessory spleens
resides in the mimicry of pancreatic cancer. Radionuclide tests (Octreotide
scan and Tc99m sulfur colloid scan) should be undertaken to
distinguish these lesions from neuroendocrine tumors, hypervascular
metastases and pancreatic carcinoma. If the tests are equivocal,
diagnostic laparotomy or laparoscopy is recommended. BACKGROUND:
Despite its relatively common occurrence, intrapancreatic ectopic
splenic tissue is rarely detected owing to its asymptomatic nature.
METHODS: We report a case of a clinically asymptomatic patient in
which abdominal computed tomography (CT) scans revealed a mass of
1.5 cm in diameter in the distal pancreas. The tumor markers CA 19-9
and carcinoembryonic antigen (CEA) were slightly elevated, and
pancreatic neoplasm was suspected. RESULTS: Left pancreatic
resection and splenectomy were performed. The removed specimen
disclosed the presence of an accessory spleen within the pancreatic
tail.
Pancreatic pseudotumors: non-neoplastic solid lesions
of the pancreas that clinically mimic pancreas cancer.Semin
Diagn Pathol. 2004 Nov;21(4):260-7.
In the
pancreas, a variety of non-neoplastic conditions may form solid
masses that may mimic cancer. Up to 5% of pancreatectomies performed
with the preoperative clinical diagnosis of carcinoma will prove to
be non-neoplastic by pathologic examination, although this figure is
decreasing with improved diagnostic modalities. Chronic inflammatory
lesions are the leading cause of this phenomenon ("pseudotumoral
pancreatitis"), and among these, autoimmune and paraduodenal
pancreatitides (discussed separately in this issue) are most
important. In this article, we will focus on the noninflammatory
lesions that may form tumor-like lesions of the pancreas.
Adenomyomatous hyperplasia of ampulla of Vater is a subtle lesion
that is difficult to define; larger examples (>5 mm) have been found
to be the cause of obstructive jaundice. Accessory (heterotopic)
spleen may form a well-defined nodule within the tail of the
pancreas and is typically mistaken for endocrine neoplasm.
Lipomatous hypertrophy is the replacement of pancreatic tissue with
mature adipose tissue that occasionally leads to moderate to marked
enlargement of the pancreas. Hamartomas are very rare if the entity
is defined strictly. They are characterized by irregularly arranged
mature pancreatic elements admixed with stromal tissue. A cellular,
spindle-cell variant with c-kit (CD117) expression is recognized.
Pseudolymphoma forms well-defined nodules composed of hyperplastic
lymphoid tissue. Rarely, foreign-body deposits, granulomatous
inflammations (such as sarcoidosis or tuberculosis), and congenital
lesions may form tumoral lesions. In conclusion, it is important to
recognize the types of conditions that form pseudotumors in the
pancreas so that they can be distinguished from ductal
adenocarcinomas, especially clinically, but also pathologically.
Nonspecific terms such as "inflammatory pseudotumor" ought to be
avoided, and every attempt should be made to classify a "pseudotumor"
into a more specific diagnostic category discussed above.
Non-neoplastic
cystic and cystic-like lesions of the pancreas: may mimic pancreatic
cystic neoplasms.
ANZ J Surg. 2006
May;76(5):325-31.
BACKGROUND:
Cystic lesions of the pancreas consist of a broad range of
pathological entities. With the exception of the pancreatic
pseudocyst, these are usually caused by pancreatic cystic neoplasms.
Non-neoplastic pancreatic cystic and cystic-like lesions are
extremely rare. In the present article, the surgical experience with
these unusual entities over a 14-year period is reported. METHODS:
Between 1991 and 2004, all patients who underwent surgical
exploration for a cystic lesion of the pancreas were retrospectively
reviewed. Patients with a pancreatic pseudocyst were excluded. There
were 106 patients of whom 8 (7.5%) had a final pathological
diagnosis consistent with a non-neoplastic pancreatic cystic or
cystic-like lesion, including 3 patients with a benign epithelial
cyst, 2 with a pancreatic abscess (one tuberculous and one foreign
body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic
cyst. These eight patients are the focus of this study. RESULTS:
There were six female and two male patients with a median age of
61.5 years (range, 41-71 years). All the patients were of Asian
origin including seven Chinese and one Indian. Four of the patients
were asymptomatic and their pancreatic cysts were discovered
incidentally on radiological imaging for other indications. All the
patients underwent preoperative radiological investigations,
including ultrasonography, computed tomography or magnetic resonance
imaging, which showed a cystic lesion of the pancreas. Three
patients, all of whom were symptomatic, were diagnosed
preoperatively with a malignant cystic neoplasm on the basis of
radiological imaging. Two patients were eventually found to have a
pancreatic abscess, one tuberculous and the other, secondary to
foreign body perforation. The third patient was found on final
histology to have chronic pancreatitis with retention cysts. The
remaining five patients had a preoperative diagnosis of an
indeterminate cyst; on pathological examination, they were found to
have a benign epithelial (congenital) cyst (n = 3), retention cyst
(n = 1) and mucinous non-neoplastic cyst (n = 1). At a median follow
up of 20 months (range, 3-34 months), none of the patients had any
evidence of recurrent disease. CONCLUSION: Non-neoplastic cystic and
cystic-like lesions of the pancreas are rare causes of pancreatic
cystic lesions that are generally benign and do not require surgery
when asymptomatic. However, despite advances in diagnostic
investigations such as endoscopic ultrasound with fluid aspirate and
magnetic resonance imaging, the preoperative diagnosis remains
unreliable. Hence, the challenge for all clinicians is to recognize
these lesions preoperatively and to avoid 'unnecessary' surgery.
Pancreatic
malignant fibrous histiocytoma, inflammatory myofibroblastic tumor,
and inflammatory pseudotumor related to autoimmune pancreatitis:
characterization and differential diagnosis.Virchows
Arch. 2006 May;448(5):552-60.
Malignant
fibrous histiocytoma (MFH) and inflammatory myofibroblastic tumor (IMT)
are uncommon primary non-epithelial cell tumors of the pancreas. In
addition, there are inflammatory pseudotumors (IPT) that may arise
in the course of autoimmune pancreatitis (AIP). In the English
language literature, only 24 cases of IMT and nine cases of MFH in
the pancreas have been reported to date. We investigated three
individual spindle cell tumors of the pancreas that were identified
as MFH, IMT, and IPT, respectively, using immunohistochemical and
molecular analysis. Both the MFH and the IMT, but not the IPT,
showed nuclear p53 expression and mutations of the p53 gene. The MFH
and the IMT also had higher mitotic and Ki-67 (MIB-1) indexes than
the IPT. The IPT was found to be a tumor-like case of AIP. Many
IgG4-positive plasma cells, which are considered to be a feature of
AIP, were found in all three tumors. It is concluded that in this
series of spindle cell tumors of the pancreas, apart from
immunohistochemical features, the demonstration of p53 mutations may
be helpful in distinguishing true neoplastic tumors from
pseudotumors such as IPTs arising in the context of AIP.
Peripancreatic tuberculous lymphadenitis mimicking carcinoma: report
of a case.Acta
Chir Belg. 2004 Jun;104(3):338-40.
Peripancreatic
tuberculous lymphadenitis is a rare clinical entity and it usually
raises serious diagnostic problems. We report a case of a solitary
abdominal tuberculoma. A 45-year old woman was admitted to hospital
with obstructive jaundice. An exploratory laparotomy was performed.
A conglomerated mass, penetrating into the pancreas was found. Since
exact diagnosis could not be obtained by peroperative frozen
sections, standard Whipple procedure, segmental portal vein
resection and reconstruction with autogenous saphenous vein were
performed. Histopathological examination of the resected specimen
revealed tuberculous lymphadenitis. The patient was given an anti-tuberculous
treatment and a good response was noted. Abdominal tuberculoma is
often mistaken for a malignant neoplasm and a high grade of
suspicion is neccessary in order to make the exact diagnosis and
optimal medical treatment of this entity.
Pancreatic pseudotumor due to peripancreatic
tuberculous lymphadenitis.Pancreatology.
2002;2(6):561-4.
Peripancreatic
tuberculous lymphadenitis is a very rare and difficult diagnosis. We
report herein a patient with a clinically solitary abdominal
tuberculoma. A 68-year-old woman was admitted to our hospital with
moderate-level obstructive jaundice due to a mass located between
pylorus and head of the pancreas. There were no clinical signs or
symptoms of tuberculosis in lungs or abdomen. After the diagnosis of
a neoplasm of the pancreas was made, exploratory laparotomy was
performed which revealed a conglomerated mass penetrating into the
pancreas. Since an exact diagnosis could not be reached on the basis
of frozen sections prepared during the operation, a standard Whipple
procedure was performed. After the histopathological examination of
the resected specimen revealed tuberculous lympadenitis, the patient
was given antituberculous medication. The patient recovered well. An
abdominal tuberculoma is often mistaken for a malignant neoplasm,
and nonsurgical diagnosis and treatment of this entity continues to
be a challenge.
Foreign body granuloma of the head of the pancreas
caused by a fish bone--a rare differential diagnosis in head of the
pancreas tumor.Chirurg.
1999 Dec;70(12):1489-91.
Despite highly
developed scanning methods there is no absolute certainty of
delineating malignant pancreatic tumor from chronic pancreatitis.
Pancreatitis caused by foreign bodies has occasionally been
mentioned in literature. Our report is on the first case of a
foreign body granuloma of the pancreatic head caused by a fish-bone
transduodenally perforating the pancreas. On preoperative CT and
ultrasound as well as by intraoperative inspection and palpation the
lesion appeared malignant, so we saw the indication for Whipple's
operation. Although the histological examination showed a benign
state, taking into account the generally bad prognosis, in case of
suspected malignant pancreatic tumor we plead for resection as the
only possible form of curative therapy.
