| Solid
pseudopapillary tumor of the pancreas. A report of 3 cases and a
review of the literature.
Chir Ital. 2006 Mar-Apr;58(2):235-45.
Solid
pseudopapillary tumours of the pancreas (SPTP) are a distinct clinico-pathological
entity that differs from the other cystic pancreatic neoplasms in the
young age of onset, the almost exclusive incidence in the female sex
and the low degree of malignancy. SPTP is a rare neoplasm that has
shown a progressive increase of incidence, passing from 0.17%-2.7% of
all exocrine tumours of the pancreas in the 1980's, to 6% in recent
reports in 2003. In addition, it accounts for about 5% of cystic
neoplasms of the pancreas. With the present paper, in the world
literature, updated to August 2005, 887 cases have been described in
248 articles. The histogenesis of these epithelial neoplasms remains
uncertain though it is likely that they originate from pluripotent
immature pancreatic cells. The tumour is generally of large size and
invariably presents a capsule. The diagnosis in most cases is based on
compressive symptoms, pain or finding of a palpable mass, while in
about 20% of the patients the finding is occasional during abdominal
imaging performed for other pathologies. CT and MR are not always
sufficient to differentiate with certainty between this type of tumour
and other cystic neoplasms of the pancreas such as pseudocysts,
parasitic cysts and congenital cysts. Cytological examination in most
cases permits the diagnosis of SPTP. The malignancy of these neoplasms
is attenuated and local with capsular invasion, lymp-node spread and,
only rarely, liver and peritoneal metastases. The surgical treatment
has to be radical since the malignancy can only be defined by
postoperative histological examination. The treatment consists of
three possible options: duodenocephalopancreatectomy, intermediate
pancreatectomy, and distal pancreatectomy with or without splenectomy.
Intraoperative histological examination is mandatory for the
diagnostic confirmation and for the evaluation of negativity of the
pancreatic resection margins. Survival after radical resection is
excellent. Moreover, in forma metastasizing to the liver an aggressive
attitude may be still curative and assure longer survival. The Authors
report their experience with three female patients with an average age
18 years (28,19 and 8 years) operated on between 1995 and 2000 for
SPTP. Two of the patients were asymptomatic and the finding of the
tumour was occasional. The third patient presented jaundice and
abdominal pain. The average diameter of the tumours was 6 cm (4, 7 and
7 cm). In all three cases tumour marker values (CEA, Ca19-9, alphaFP)
were normal. Only in one case was the preoperative diagnosis correct.
The surgical treatment depended on the location of the neoplasms: for
the two tumours in the head, in one case an enucleoresection was
performed in relation to an exophytic location, while, in the other, a
duodenocephalopancreatectomy was performed. In the somatopancreatic
tumour a distal splenopancreatectomy was performed. Only in one case
(the DCP) the capsule and the surrounding parenchyma were infiltreted
by neoplasm. In all cases there was immunohistochemical positivity for
alpha1-antitrypsin and for neuron-specific enolase. Neither mortality
nor operative morbidity were observed. Follow-up with CT found no
relapses in any of the three patients after 5, 7 and 10 years,
respectively, after the operation.
Solid
pseudopapillary tumor of the pancreas suggesting malignant potential.Pancreas.
2006 Apr;32(3):276-80.
OBJECTIVES: It
has been suggested that the histopathologic features associated with
malignant potential in solid pseudopapillary tumors (SPTs) of the
pancreas include cellular polymorphism, mitotic activity,
peripancreatic tissue invasion, neural invasion, lymphovascular
invasion, and lymph node metastasis. This study aimed to delineate the
features predicting SPTs with malignant potential, which can be easily
assessed in the clinical setting, and long-term results of patients
who underwent resection of SPT. METHODS: A retrospective review and
analysis were done of 33 patients who had SPTs that were surgically
treated. RESULTS: Eleven patients (33.3%) had SPTs suggesting
malignant potential among surgically treated patients with SPTs. The
local invasion to the peripancreatic tissue was the most frequent
malignant pathological feature (7/11 patients, 63.7%). Using
univariate analysis, tumor size greater than 5 cm was a significant
clinical factor to predict SPTs with malignant potential (P = 0.022).
The prognosis was excellent. No statistically significant survival
difference was noted between groups of malignant potential and benign
pathology (P = 0.1904). CONCLUSIONS: Malignant SPTs are low-grade
tumors with good prognosis. Adequate surgical intervention is
necessary. Especially, SPTs over 5 cm in diameter need to be treated
carefully because of the chance of malignant pathology. Careful
long-term follow-up is necessary in those patients in case of possible
tumor recurrences.
Four cases of solid
pseudopapillary tumors of pancreas: imaging findings and pathological
correlations.Eur
J Radiol. 2006 Apr;58(1):132-9.
OBJECTIVE: Solid
pseudopapillary tumor of the pancreas (SPTP tumor) is a rare
pancreatic neoplasm with low malignant potential, which usually
affects female patients in the second or third decades of life. It is
a non-functional, slow-growing neoplasm that very often reaches
considerable size before the first symptoms appear. Symptomatology is
frequently related to tumor size. Surgical excision is usually
curative in most cases. Infrequently the tumor can appear in male
patients or in aged women, which can make the diagnosis more
difficult. Some patients develop liver metastases in the follow-up
that can be resected. Our purpose is to review the radiological and
pathological findings of SPTP with emphasis on these infrequent cases.
SUBJECTS AND METHODS: The medical records and radiological findings of
patients who underwent surgery for SPTP between 2000 and 2005 were
retrospectively reviewed. Study eligibility required that patients had
undergone surgical resection and that a SPTP had been pathologically
proved. RESULTS: Four cases of solid pseudopapillary tumor of the
pancreas were diagnosed and treated in our institution in the study
period. Two of the patients, developed on follow-up liver metastases,
and peritoneal, hepatic, and nodal metastases, respectively.
CONCLUSION: Solid pseudopapillary tumors are well-encapsulated
neoplasms that usually have a good prognosis after surgical excision.
A malignant behavior is uncommon and in this case lymph node
involvement, hepatic metastases and occasionally peritoneal invasion
may also occur. Resection of liver metastases can prolong the
long-term survival of the patients.
Solid-pseudopapillary
tumor of the pancreas: Clinical experience and literature review.
World J Gastroenterol 2005; 11(9): 1403-1409
http://www.wjgnet.com/1007-9327/11/1403.asp
Solid-pseudopapillary
tumours of the pancreas: 14 years experience.ANZ
J Surg. 2005 Aug;75(8):684-9.
BACKGROUND:
Solid-pseudopapillary tumour of the pancreas (SPTP) is a low-grade
malignancy lesion that is distinct from other pancreatic tumours.
Preoperative diagnosis is often inaccurate and treatment strategies
remain controversial. The present study reviews the clinical features,
diagnosis, treatment, and outcome of Asian patients with SPTP from a
single institution. METHODS: The medical records and images of
patients who underwent surgery for SPTP between June 1990 and December
2003 were retrospectively reviewed. Study eligibility required that
patients had undergone surgical resections and that the SPTP had been
pathologically demonstrated. RESULTS: Twenty-eight patients with SPTP
were identified. Eighteen patients (64.3%) reported the predominant
symptom of 'vague abdominal pain', five patients (17.8%) had an
apparent 'abdominal mass', and five patients (17.8%) without overt
symptoms had masses that were discovered incidentally during
screening. All patients underwent magnetic resonance (MR) imaging (n =
22) and/or computed tomography (n = 17), with a specificity of 90.9%
and 76.4%, respectively. All masses were well-circumscribed, except
for a tumour in one patient (3.6%), which adhered to the stomach wall
and metastasized to the liver. Two patients (7.1%) underwent
enucleation, while 25 patients (89.3%) received curative resection.
The patient with the liver metastases underwent distal pancreatectomy
and splenectomy with partial hepatectomy. The mean follow up was 66.9
months. No mortality occurred during follow up but the patient with
the liver metastasis had progressive deterioration. CONCLUSION:
Symptoms of SPTP are indistinct and preoperative diagnosis is often
inaccurate. Magnetic resonance imaging improves SPTP diagnosis. In
general, the prognosis of well-circumscribed SPTP is favourable after
curative resection.
Urgent Whipple
resection for solid pseudopapillary tumor of the pancreas. J
Hepatobiliary Pancreat Surg 2003; 10:386-9.
We report a
14-year-old boy who was urgently transferred to our institution after
a blunt abdominal trauma. CT scan and repeated ultrasound examinations
revealed a subhepatic mass, which slowly increased in size. Imaging
features were not specific enough to permit a precise diagnosis.
Because of the positive peritoneal signs and increased signs of
circulatory instability, the decision was made to perform an urgent
explorative laparotomy. The exploration revealed that the large
haematoma-like bleeding mass was a tumor arising from the head of the
pancreas, which ruptured on the surface. We performed a curative R0
Whipple resection. Histology of the resected specimen revealed a solid
pseudopapillary tumor of the pancreas, an extremely rare tumor
predominantly occurring in young and middle-aged women. It has a
favorable prognosis if resected radically. The boy is well 30 months
after the operation. To our knowledge, there are only two other
descriptions of pancreatic solid pseudopapillary tumor in boys treated
urgently by Whipple resection because of acute presentation after
blunt abdominal trauma.
Solid-pseudopapillary
tumor of the pancreas: a clinical study of five cases, and review of
the literature. Ann
Chir 2003; 128:543-8.
INTRODUCTION:
Solid pseudopapillary tumors of the pancreas are rare and their origin
is unknown. The aim of this work was to report five new cases.
MATERIAL AND METHODS: Retrospective study of data from patients
operated on from 1983 to 2002 in a university hospital specialized in
pancreatic surgery. Patients were identified in a prospectively
constituted database of pathologic examinations. RESULTS: Five
patients (three men and two women, aged from 15 to 69 years) underwent
pancreatectomy for a solid pseudopapillary tumor, which was discovered
fortuitously by imaging in three cases. Tumor diameter ranged from 4
to 15 cm. Diagnosis was made preoperatively in only one patient. There
were three pancreaticoduodenectomies and two left pancreatectomies,
with extension to the transverse colon due to vascular reasons in two
cases. Only one significant complication occurred (one colonic
fistula). With a follow-up ranging from 6 months to 6 years, all
patients are alive without recurrence. CONCLUSIONS: Solid
pseudopapillary tumors are not exceptional in men. Complete resection
can need extension to neighboring organs but allows good long-term
survival.
Solid-pseudopapillary
tumor of the pancreas: a neoplasm with distinct and highly
characteristic cytological features. Diagn Cytopathol 2002; 27:325-34.
The solid-pseudopapillary
tumor of the pancreas (SPTP) is an unusual low-grade malignant
epithelial tumor affecting predominantly adolescent girls and young
women. Although approximately 500 cases of SPTP have been described in
the last 40 yr, its pathogenesis remains uncertain. However, the
clinical features of this neoplasm are very characteristic and SPTP
must be suspected in any young woman with a cystic or partially cystic
pancreatic mass. In this report, we describe the cytologic features of
seven cases of SPTP investigated by preoperative fine-needle
aspirates. The analysis of the cytologic features in these cases and
in 43 cases collected from the literature reveals that they are highly
characteristic and quite distinct from those of other cystic or solid
tumors of the pancreas. On this basis, a cytologic diagnosis of SPTP
may be rendered with great confidence, not only in clinically typical
examples, but also in unusual presentations, such as in older
patients, in males, in ectopic locations, and in metastatic sites.
A case of solid
pseudopapillary neoplasm of the pancreas and tumor doubling time.
Pancreatology 2002; 2:495-8.
BACKGROUND:
Solid pseudopapillary neoplasm of the pancreas is a rare tumor with
unique clinicopathological features. Although it is considered to grow
slowly, little is known about its doubling time. METHOD: We
encountered a young Japanese woman with a solid pseudopapillary
neoplasm of the pancreas that had been detected at another hospital 5
years previously but was resected at our hospital. The tumor showed
only a solid component without any cysts. Computed tomographic scans
taken 5 years apart were compared to determine the tumor doubling
time. RESULT: The tumor doubling time according to the formula of
Schwartz and co-workers was determined to be 765 days. CONCLUSION:
These findings confirm that this tumor grows quite slowly and also
support long disease-free periods even in patients who have recurrence
or metastases.
Solid-cystic
pseudopapillary tumor of the pancreas: acute post-traumatic
presentation. Case report and review of the literature. Rev
Gastroenterol Mex 2002; 67:93-6.
OBJECTIVE: To
report an infrequent case of pancreatic pseudopapillary solid-cyst
tumor, known as Frantz tumor, discovered after blunt abdominal trauma,
and to carry out a review of the literature. BACKGROUND: Frantz's
tumor is a very uncommon low-grade malignant papillary-cystic neoplasm
of the pancreas that is relatively frequent in young black women and
was first described by Frantz in 1959. CASE REPORT: We present the
case of a 15-years-old female patient who, 24 h after blunt abdominal
trauma, came to the emergency room with intense abdominal and atypical
pain and a palpable mass in the left upper quadrant. It was initially
diagnosed as a post-traumatic pancreatic pseudocyst. The patient
underwent distal pancreatectomy with spleen preservation; the
histopathologic report was a pseudopapillary solid and cystic tumor of
the pancreas without malignant cells. Clinical evolution has been
satisfactory without recurrence of the tumor at 14 months of
follow-up. CONCLUSIONS: Frantz's tumor has benign biologic behavior
and treatment of choice consists of surgical resection. Outcome after
surgical resection is excellent with 90% survival in the long term.
Tumoral recurrence has been described in approximately 10% of
patients.
Solid and papillary epithelial neoplasms of the pancreas. Surgery
1990; 108: 457-480.
Seven patients
with solid and papillary epithelial neoplasms of the pancreas are
reported. All were young women with a mean age of 22 years (range, 16
to 33 years old). Each patient had a large asymptomatic abdominal
mass. The tumors ranged in size from 7 to 20 cm (average size, 13 cm)
and were evenly distributed throughout the head, body, and tail of the
pancreas. One patient had a metastatic tumor to her liver, which was
unresectable. All other patients underwent resection, which included
two distal pancreatectomies, two total pancreatectomies, one
pancreaticoduodenectomy, and one local excision. Four of the seven
patients had evidence of local invasions alone, and one had a liver
metastasis and local invasion. All patients had the characteristic
histologic pattern of a solid and papillary epithelial pancreatic
neoplasm. All patients are alive with a mean follow-up of 10 years
(range, 4 to 20 years). This is an unusual malignant neoplasm of the
pancreas occurring predominantly in young women. Even though they are
locally invasive, long-term survival is the rule. Surgical therapy
should be aggressive, since liver metastasis may occur.
|
