Syn: Solid and cystic tumour of the pancreas ; Papillary-cystic tumour; Solid and papillary epithelial neoplasm.

Histopathology Images of Solid Pseudopapillary Tumour

Solid-pseudopapillary tumour was first described by Frantz

in 1959.

It is a rare tumour which has a low-grade malignant potential.

Clinical presentation:

This uncommon tumour occurs almost exclusively in

adolescent girls and young women with a median

age of 26 years.

Rare cases have been reported in men.

It appears to be more aggressive in older males.

Patients usually present with an enlarging and painful

abdominal mass, but asymptomatic cases are

found incidentally at laparotomy or by imaging

technique.

The diagnosis depends on histologic confirmation, but

its appearance on imaging is fairly characteristic,

being a large well-encapsulated mass with calcification

and areas of hemorrhagic degeneration.
 

Gross features: Grossly, the tumour may occur anywhere

in the pancreas as a round, well - circumscribed lesion,

measuring 2-17 cm in diameter (average 8 cm).

Cut surface shows a solid mass with cystic zones.

Areas of necrosis and hemorrhage are common.

Microscopic features: Image Link1 ; Image Link2 ; Image Link3

The histological appearance is very distinctive and is

considered diagnostic.

1. The solid portion is composed uniform, small and round cells

arranged in sheets, cords and trabeculae with a rich vascular

network.

The cytoplasm is eosinophilic or vacuolated, often containing

PAS-positive droplets.

Nuclei are round or ovoid, having finely dispersed

chromatin and inconspicuous nucleoli.

Mitoses are few.

Many of the cells, farthest from the vessels undergo

degeneration and death causing remaining cells around

the vessels to form pseudorosette mimicking

endocrine tumour.

2. The cystic zones result from more degenerative changes

with prominent necrosis and hemorrhage.

Cholesterol granulomas may be present.

3. Malignant forms show vascular and nerve sheath invasion.

Tumours show positive immunostaining for alpha-1-antitrypsin,

alpha-antichymotrypsin, neuron-specific enolase,

and vimentin.

Positive immunostaining for progesterone nuclear receptor

antibody has been reported.

One recent case shows a distinctive, unbalanced translocation

between chromosomes 13 and 17.

Majority of the tumours have a borderline malignant potential,

but most patients are symptom-free many years

after surgery.

Frankly malignant tumors are characterized by vascular

invasion and/or metastases to lymph nodes or liver.

Surgical resection has generally been curative, but close

follow up is advisable, particularly when the histologic

characteristics suggest a more aggressive tumour.

Differential diagnosis:  Any solid or cystic pancreatic disease

entities,  such as - mucinous cystic tumor, microcystic

adenoma, islet cell tumor, cystadenocarcinoma,

acinar cell carcinoma, inflammatory pseudocyst, mucus

 secreting tumor, pancreatoblastoma, and a vascular

tumor-like hemangioma.

The first four are usually seen in older patients and have no

particular gender preponderance .

Pancreatoblastoma is usually found in younger individuals

of either sex.

Radiologically, a linear sunburst pattern of calcification

is the usual finding in microcystic adenoma.

A hypervascular pattern on angiography is suggestive of

islet cell tumour rather than solid-pseudopapillary tumour.

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.

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BACKGROUND: Solid-pseudopapillary tumors (SPTs) of the pancreas have been reported as rare lesions with "low malignant potential" occurring mainly in young women. This study was designed to define the clinicopathological characteristics and the effect of surgical intervention. METHODS: A retrospective review from January 1985 to July 2000 was performed. Clinicopathological, operative, and survival data were obtained. The Kaplan-Meier method and chi2 analysis were performed. All cases were re-reviewed by a senior pathologist. RESULTS: During this time, 24 patients were diagnosed as having SPTs (0.9%). Twenty females and four males were identified, with a median age of 39 years (range, 12-79). The median size of the lesions was 8.0 cm (range, 1-20). Two patients' tumors were found to be unresectable at initial presentation because of vascular invasion; both patients have remained alive with disease, one for 13 years and the other 1 year. At a median follow-up of 8 years, one recurrence occurred in 17 patients who underwent complete resection. Microscopic margin positive (P = .26), invasion of surrounding structures (P = .51), and size >5 cm (P = .20) were not significant predictors of survival. Four patients presented with synchronous liver metastasis and underwent resection of the primary tumor and the liver metastasis, with one patient dying of progression of metastatic disease at 8 months, another alive with recurrence in the liver at 6 years, and the last two alive without evidence of disease at 1 month and 11 years. CONCLUSIONS: SPT occurs predominantly in women (82%), although it can occur in men; all age groups are affected. Complete resection is associated with long-term survival even in the presence of metastatic disease.

Solid pseudopapillary tumors of the pancreas. Pathology report of 13 cases. Gastroenterol Clin Biol 1999; 23:207-14.

OBJECTIVES: Solid pseudopapillary tumors of the pancreas are exceptional. The aims of our study were to reevaluate the mode of presentation of these tumors and to analyze the role of pathological examination in diagnostic assessment and prognostic evaluation. PATIENTS: We report the clinical, radiological and pathological findings in a retrospective series of 13 patients in whom a diagnosis of solid pseudopapillary tumor of the pancreas was made between 1983 and 1997. There were 12 females (median age: 22.5 years) and one male, aged 73. RESULTS: The tumor was discovered incidentally (3 cases) or because of nonspecific digestive symptoms (10 cases). Biological data were uninformative. The tumor was pancreatic in 12 cases and duodenal in 1. In all cases, imaging techniques showed an heterogeneous lesion with no or poor vascularization. A cystic component was identified in 4 cases. Surgical resection was performed in all cases. Pathological examination showed an encapsulated tumor in 8 cases, a non-encapsulated but well-limited lesion in 3 cases and an infiltrative tumor in 2 cases. At the time of diagnosis, multiple liver metastases were present in 1 case. Mean duration of follow-up was 24 months (range: 3-168). At last follow-up, all patients, including the patient with synchronous metastatic disease, were alive, without local recurrence. CONCLUSION: Our study confirms that most cases of solid pseudopapillary tumors of the pancreas present with a suggestive clinical picture, including their occurrence in young women and their good prognosis after surgical resection. However, our results also underline the occurrence of cases presenting with unusual features, including old age, male sex, extra-pancreatic localization and malignant evolution. Histopathological examination is essential for the establishment of the diagnosis but morphological data are of little prognostic value.

Pancreatic solid-cystic-papillary tumor: clinicopathologic features in eight patients from Hong Kong and review of the literature. World J Surg 1999; 23:1045-50.

Solid-cystic-papillary tumors (SCPTs) of the pancreas are rare. The clinicopathologic features and pathogenesis of these tumors have attracted a number of investigations, but the results remain unclear. We investigated the clinicopathologic data, immunohistochemical expression of the pan-endocrine markers, hormones, steroid receptors, and p53 overexpression in pancreatic SCPTs from eight Chinese patients (seven women, one man) collected over a 24-year period. They accounted for 2.5% of the primary pancreatic tumors. The tumors were seen in young women (mean age 27 years). They were large (mean size of resected tumor was 8.4 cm), benign, had solid and cystic areas, and were evenly distributed in the pancreas. The main differential diagnosis was pancreatic endocrine tumor. The tumors were negative for pan-endocrine markers, hormones, estrogen receptor, progesterone receptor, and p53. To date, 452 pancreatic SCPTs have been documented in the English literature. They occurred in patients of different ethnic groups, particularly in non-Caucasians. The tumors were frequently noted in young females. Uncommon cases of malignant pancreatic SCPTs were often found in older men and had indolent behavior. It was concluded that pancreatic SCPTs have distinct clinicopathologic characteristics. The present observations, together with a review of the literature suggests that overexpression of p53 or estrogen receptor is not important in the pathogenesis of pancreatic SCPTs.

Normal Islets of Langerhans

The Apud Concept

An approach to reporting of pancreatic specimen

Reporting of pancreatic biopsies for the diagnosis of neoplastic lesions

Reporting of ampullary and periampullary biopsies for the diagnosis of neoplastic lesions

Reporting of Pancreatico duodenectomy (Whipple's operation) specimen

Reporting of Distal Pancreatectomy Specimen

Developmental Defects of Pancreas

Nesidioblastosis

Pancreas Divisum

Aberrant(Ectopic)Pancreas

Annular Pancreas

Pancreatic Agenesis

Non-Neoplastic Pancreatic Cysts 

Pancreatitis

Acute Pancreatitis

Chronic Pancreatitis

Autoimmune Pancreatitis

Herpes Simplex Pancreatitis

Diabetes Mellitus

Neoplasms of the Endocrine Tumours

Islet Cell Tumours

Glucagonomas

Insulinomas

Somatostatinoma

VIPomas

Pancreatic Polypeptide-Secreting Tumours

Enterochromaffin Cell (Carcinoid) Tumours

Pancreatic Gastrinoma

Corticotropinoma

Parathyrinoma

Multiple Endocrine Neoplasia (MEN) Syndrome

Carcinoma of the Pancreas