In most cases, solid pseudopapillary tumours should be resected surgically, as there is a risk of malignancy cancer. Solid pseudopapillary tumors spt of the pancreas are rare usually benign pancreatic tumors. It affects mostly young females between the second and third decade of life. We sought to correlate the immunohistochemical staining of these tumours with that previously reported in the literature. Pathogenesis, histogenesis and differentiation of spt are still uncertain. We report the case of a 48yearold woman with a pseudopapillary tumour of the pancreas. Solid pseudopapillary tumor spt of the pancreas is a rare and probably misdiagnosed tumor with low malignant potential, accounting for 12% of all pancreatic tumors. The solid pseudopapillary neoplasm is a rare tumor of the pancreas. Mr imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. The tumor has been referred to with multiple different names, including. However, occurrences in children and elderly have also been reported5,6.
Diagnosis and treatment of solid pseudopapillary tumor of. Solid pseudopapillary tumor of the pancreas show diagnoses week 653. Jinping lai, md, phd solid pseudopapillary neoplasm of the pancreas is a rare entity with low malignant potential and excellent overall prognosis. At macroscopic examination, the tumor was located in the head of the pancreas, measuring. Solid pseudopapillary tumor of the pancreas radiographics. Solid pseudopapillary neoplasm spn of the pancreas is a rare tumor accounting for approximately 0. Due to rarity of the disease, most data on spt are. Solid pseudopapillary neoplasm of the pancreas jop. Histopathology donatella santini, francesca poli, stefania lega martinelli pathology unit, s.
The clear cell variant of solid pseudopapillary tumor of the pancreas. These features are correlated with a montage of contrast materialenhanced computed tomographic and magnetic resonance mr images, which demonstrate a welldefined, complex cystic mass arising in. Solid pseudopapillary neoplasm is a rare tumour of the exocrine pancreas. Solidpseudopapillary tumor of the pancreas spt has distinctive morphologic and biologic features but an unclear origin.
We report on our experience of the surgical management and outcomes of 11 patients with solid pseudopapillary tumour of the pancreas spt. Solid pseudopapillary tumor of the pancreas is a rare lowgrade malignant neoplasm. Solid pseudopapillary tumor was first described by dr. An algorithmic approach osamuyimen igbinosa saint peters university hospital.
Jahangir s, loya a, siddiqui mt, noreen n, yusuf ma. The disease is seen mostly in young female patients, and the detection rate of spts has increased recently 1. Pdf solidpseudopapillary neoplasm spn of the pancreas. Diagnosis and treatment of solid pseudopapillary tumor of the. We here report a 27yearold woman with solid pseudopapillary neoplasm, who presented with mild jaundice, mildly elevated liver function tests and right upper quadrant pain. The aim of this study was designed to evaluate the clinicopathologic feature, predictive factors of malignancy, and survival from experience of a single center. Stanford medicine school of medicine departments surgical pathology criteria solidpseudopapillary tumor of the pancreas. Solid pseudopapillary neoplasm spn of the pancreas is considered a lowmalignant neoplasm with a good prognosis.
Pathologic examination reveals that solid pseudopapillary tumor is usually a large, encapsulated mass composed of a mixture of cystic, solid, and hemorrhagic components. How is solidpseudopapillary neoplasm of pancreas treated. Extrapancreatic solid pseudopapillary neoplasm in the subhepatic. The major differential diagnosis includes pancreatic neuroendocrine tumor. Research article solidpseudopapillary tumor of the pancreas. Histologically the tumor was diagnosed as a solid and cystic pseudopapillary tumor of the pancreas. About 8384 cases of solid pseudopapillary neoplasms spn of pancreas have been published in english literature, from 1933 to 2018. Solid pseudopapillary tumor spt is a rare pathological entity of the pancreas accounting for 1. Surgical resection is known to offer excellent long term survival. Accuracy of diagnosis of solid pseudopapillary tumor of the. Solid pseudopapillary neoplasm of the pancreas spn is a very rare tumor with a low malignant potential. Solid pseudopapillary neoplasm spn is a rare pancreatic tumor that predominantly affects young females. Sptp occurs more frequently in the body and tail of. Spn accounts for less than 1% to 2% of exocrine pancreatic tumors.
Solid pseudopapillary tumor of the pancreas springerlink. The diagnosis of solid pseudopapillary tumour of the pancreas is usually confirmed with endoscopic ultrasound eus guided fine needle aspiration fna of the lesion. Mr imaging features of solid pseudopapillary tumor of the. Solid pseudopapillary neoplasm spn of the pancreas. Pancreatic pseudopapillary tumor in a male child abdulwahed n meshikhes1, ramez atassi2 1department of surgery, dammam central hospital and 2department of pathology, regional laboratory and central blood bank, directorate of health. Solid pseudopapillary neoplasm of the pancreas is a lowgrade malignant tumor generally associated with a good prognosis. These tumors have low malignant potential and a strong predilection for young women, with less than 10% of cases reported in men 1. Solid pseudopapillary tumor of the pancreas radiology key. Feb 28, 2014 solid pseudopapillary tumor is a rare primary neoplasm of the pancreas that typically affects young women. Different methods of resection of solid pseudopapillary. Although most tumors show benign behavior, malignant degeneration may. Differentiation of solidpseudopapillary tumors of the. Acinar cell carcinoma neuroendocrine solid and papillary epithelial neoplasm solid cystic pseudopapillary tumor of pancreas solid pseudopapillary neoplasm solid pseudopapillary tumor of the pancreas week 105.
Solid pseudopapillary tumor in a pancreatic rest of the. Solid pseudopapillary tumor of the pancreas gross appearance in situ. Solid pseudopapillary tumors of the pancreas, known as frantz tumors, are rare pancreatic tumors that occur predominantly in women, with very few cases. Discussion spts of the pancreas are rare 1%2% of exocrine pancreatic tumors at most institutions. It consists of about % of all the pancreatic neoplasm, and mostly occurred in young women 2, 3. Solidpseudopapillary tumor of the pancreas frantz tumor. Although rare, this type of tumor is increasingly seen in clinical practice because of widespread availability of imaging modalities and better awareness of the disease.
Solid pseudopapillary tumor of the pancreas sptp is a rare type of pancreatic tumor. Most pancreatic tumors are malignant and have a bad prognosis. Solid pseudopapillary tumour of the pancreas is a rare neoplasm which, for the most part, affects young women and has a relatively favourable prognosis with low malignant potential. Surgical resection is generally curative and the prognosis is excellent. It is a relatively a benign tumor, with a favorable prognosis. Although most tumors show benign behavior, malignant degeneration may occur. Solid nests of poorly cohesive cells forming a cuff surrounding blood vessels, resulting in a pseudopapillary architecture stroma usually shows various degrees of hyalinization or evidence of degeneration such as hemorrhage, foamy macrophages, calcification and cholesterol clefts.
Solid and papillary tumors of the pancreas spts are unusual benign or lowgrade malignant epithelial tumors occurring predominantly in women under the age of 25 years 91% 15. It generally occurs in young women and the prognosis is usually good after complete. Solid pseudopapillary neoplasm spn is a rare but distinctive pancreatic neoplasm that typically affects young women. Also known as solid pseudopapillary tumor, papillary epithelial neoplasm, papillary cystic neoplasm, solid and papillary neoplasm, low grade papillary neoplasm and hamoudi or frantz tumor epidemiology. Drawing of a solid pseudopapillary tumor of the pancreas shows the thick fibrous capsule of the mass surrounding a hemorrhagic and necrotic center. Solid pseudopapillary neoplasm of the pancreas with liver. How is solid pseudopapillary neoplasm of pancreas treated. We conducted a pubmed search and found only 4 cases of sptp arising in ectopic pancreas in the worlds literature. Solid pseudopapillary neoplasm of the pancreas in a young.
However, 5% to 15% of patients with spns develop metastatic disease, most commonly in the liver. In 1996, the world health organization renamed this tumor solid pseudopapillary tumor for the international histologic classification of tumor of the exocrine pancreas. This is a lowgrade tumor that usually occurs in children but is rare in adults and, in exceptional cases, can show extrapancreatic localization. Solid pseudopapillary neoplasm of the pancreas with high.
Risk factors of the recurrences of pancreatic solid. Solid pseudopapillary neoplasm spn of the pancreas is a rare tumor of low malignant potential. The finding of solid pseudopapillary tumor in a pancreatic rest is extremely rare. Solid pseudopapillary neoplasm of the pancreas sciencedirect. Solid pseudopapillary tumor is a very rare neoplasm of the pancreas, accounting for only 12% of all exocrine pancreatic tumors. Until it was defined by the world health organization who in 1996 as solid pseudopapillary tumor of the pancreas, this tumor was described by using various names including solid cystic tumor. A retrospective study of medical records of all patients treated from january 1997 until july 2015. Solid pseudopapillary tumor of the pancreas, otherwise known as solid and cystic tumor or frantz tumor, is an unusual form of pancreatic carcinoma, with unknown etiopathogenesis, that. Solid pseudopapillary tumours are typically round, welldemarcated, measuring 217 cm in diameter average 8 cm, with solid and cystic areas with hemorrhage on cut sections. Oct 31, 2017 solid pseudopapillary tumor of the pancreas is an uncommon pancreatic tumor and represents 12% of exocrine pancreatic tumors1. The clinical data and pathological findings from 11 chil dren with sptp were.
Solid pseudopapillary neoplasm spn is a rare tumor with malignant potential which is generally located in the tail of pancreas. Solidpseudopapillary tumor of the pancreas radiographics. What are the lab findings in pancreatic solid pseudopapillary neoplasm cyst fluid. It classically presents as a large tumor with cystic and solid components. It primarily affects women 87%90% of cases, with average age of 20 to 30 years, rendering, in our case, eusfnb with immunohistochemistry mandatory for this improbable, according to the epidemiological data, diagnosis. Solid pseudopapillary tumor of the pancreas is a rare pancreatic tumor that predominantly occurs in young noncaucasian women. Solid pseudopapillary tumor of the pancreas frantzs tumor. Terminology the tumor has been referred to with multiple different names, including. Solid pseudopapillary neoplasia spn of the pancreas is an extremely rare epithelial tumor of low malignant potential. Different names for this tumor were reported until it was defined by the world health organization who in 1996 as a solid pseudopapillary tumor of. Most patients present with nonspecific symptoms until the tumor becomes.
Also unclear are its neuroendocrine differentiation, its capability to express alpha1antitrypsin aat and, in view of the tumor s. Solid pseudopapillary pancreatic tumor tumor of frantz. Solidpseudopapillary tumor of the pancreas johns hopkins. Solid pseudopapillary tumor of the pancreas request pdf. Bologna, italy summary a olidspseudopapillary tumor is an uncommon and enigmatic pancreatic neoplasm, and the term encompasses the two. A neoplasm with distinct and highly characteristic cytological features. Solid pseudopapillary tumor spt of the pancreas is a rare exocrine pancreatic tumor, which represents only about 1% of all tumors of the pancreas. The purpose of this pictorial essay is to illustrate the various appearances of solid pseudopapillary tumor of the pancreas. Enucleation of pancreatic solid pseudopapillary neoplasm.
Solid pseudopapillary tumor of the pancreas pankreas. We recommend image characterization with contrastenhanced helical ct scan. Solid pseudopapillary tumor of the pancreas radiology case. This study was conducted to describe the mr imaging features of solid pseudopapillary tumor of the pancreas. Pdf solid pseudopapillary tumor of the pancreas frantzs. Solid pseudopapillary tumor of the pancreas in children. Spts are rare tumors which occupy % of all pancreatic tumors and 1015 % of cystic tumors of the pancreas.
It was first described by frantz in 1959 and also known as frantzs tumor. Conclusion the unclear preoperative diagnoses, together with incidence of potential malignancy as well as good outcome with resection, suggest that all suspected cystic tumors of the pancreas should. Solid pseudopapillary neoplasms show peculiar morphologic features, but sometimes the differential diagnosis with other pancreatic neoplasms ie, pancreatic neuroendocrine tumors can be a challenging task, especially in. Intraoperative photographs of two patients show a a large, ovoid, encapsulated mass with smooth surface in the head of the pancreas and b a yellowish slightly lobulated mass in the body and tail of the pancreas arrows. Although previously suggested, more recent publications have addressed the lack of race predominance. Terminology the tumor has been referred to with multiple. It constitutes approximately 1% of pancreatic neoplasms and 3% of cystic lesions of the pancreas.
Solid pseudopapillary tumor of the pancreas in a child. Solid pseudopapillary neoplasm of the pancreas spn is a rare pancreatic disorder that first described by frantz in 1959. Solid pseudopapillary tumor spt of the pancreas is a rare enigmatic tumor of low malignant potential that most frequently occurs in young noncaucasian women. Solid pseudopapillary tumor represents 1% to 2% of pancreatic malignant neoplasm. Solidpseudopapillary tumor of pancreas is a very rare neoplasm of low malignant potential and unknown origin. The treatment methods solidpseudopapillary neoplasm of pancreas are determined by several factors, such as the size and location of tumor, how advanced the condition is, the overall health of the affected individual, as well as hisher personal preferences. Solid pseudopapillary neoplasm of the pancreas references 1. Solid pseudopapillary neoplasm spn of the pancreas, a rare type of tumour accounting for 0. Spns are usually indolent but they do have malignant potential. Solid pseudopapillary tumors spt are rare, slow growing tumors of the pancreas that typically affect young women. The solid pseudopapillary neoplasm spn of the pancreas was first described by frantz in.
Notohara k, hamazaki s, tsukayama c, nakamoto s, kawabata k, mizobuchi k, sakamoto k, okada s, solid pseudopapillary tumor of the pancreas. Dec 05, 2008 solid pseudopapillary tumor of the pancreas is a rare pancreatic tumor that predominantly occurs in young noncaucasian women. Clear cell variant of solid pseudopapillary neoplasm is also reported to be glycogen negative you are here. Clinical update on the management of pseudopapillary tumor. Spns are fundamentally solid tumours, although with increasing size, they become solid.
Solid pseudopapillary tumor of the pancreas spt is an extremely rare pancreatic tumor, which has a low malignant potential and occurs mainly in young women. It includes a summary of the current literature to provide a reference for the management of this rare clinical entity. It is of low malignant potential and occurs most frequently in young females. In the second patient the tumor was located in the body of her pancreas, with portal vein occlusion and welldeveloped collateral circulation. Solidpseudopapillary tumor of pancreas request pdf. Solid pseudopapillary tumor of the pancreas sptp is a rare neoplasm of low malignant potential with uncertain behavior, diagnosed mainly in young women. Epigenetics of solid pseudopapillary neoplasm of the pancreas. Three of these cases were reports of solid and papillary epithelial tumors arising in ectopic pancreatic tissue in the mesocolon. We report a case of spt arising from the head of the pancreas in a yearold girl who presented with pain in the epigastrium and was treated successfully.
Solid pseudopapillary neoplasms spns are the most common pediatric pancreatic tumor. Such tumours histologically comprise poorly cohesive epithelial cells. However, solid pseudopapillary tumor of the pancreas spt is an unusual lowgrade malignancy that rarely metastasizes. Solid pseudopapillary tumor spt of the pancreas is a rare clinical entity, with a reported incidence of 2%3% of all pancreatic tumors 16.
Solid pseudopapillary tumor of the pancreas radiology reference. Accurate diagnosis is essential in the management of spn. We report our experience with the diagnostic and therapeutic management of these tumors over 10 years. Although most commonly presenting in females in the second to fourth decade of life, it has been reported in the pediatric population with an incidence of 816. Printable solid pseudopapillary neoplasm of the pancreas. Solid pseudopapillary tumor spt of the pancreas, other wise known as solid and cystic tumor or frantz tumor, is a rare but characteristic neoplasm, with unknown etiopatho genesis, accounting for 0. Solid pseudopapillary tumor of the pancreas in children koreamed. Solid pseudopapillary neoplasm of the pancreas is a rare tumor characterized by its occurrence in young women and its limited malignant potential. Solid pseudopapillary tumor of the pancreas sptp is a rare neoplasm of uncertain origin, often indolent biologic behavior, and distinctive pathologic features. Tumor with prominent blood vessels and small cyst like spaces contrasts with normal pancreatic acini at right. Solid pseudopapillary tumor of the pancreas spt is a rare pancreatic tumor with an unclear pathogenesis and good prognosis after resection. Solidpseudopapillary tumor spt of pancreas has been described by many other synonyms, such as solidcystic tumor, papillarycystic tumor, solid and papillary epithelial neoplasm, lowgrade papillary tumor, and frantzs tumor because of the first report by the frantz in 1959. Full text solid pseudopapillary tumor of the pancreas. The aim of this study is to report our experience with spn of the pancreas.
A solid pseudopapillary tumour of the pancreas springerlink. New brunswick, nj, usa abstract context solidcystic pseudopapillary tumor of the pancreas is a rare tumor, but has favorable prognosis even in the presence of distant metastases. Solid pseudopapillary tumor is a rare primary neoplasm of the pancreas that typically affects young women. Pancreatic solid pseudopapillary neoplasm, originally identified by frantz in 1959, was defined as solid pseudopapillary tumor spt by the world health organization who in 1996. Eastern province, dammam, saudi arabia abstract context solid pseudopapillary tumors are exceedingly rare in males. Case report solid pseudopapillary tumor of the pancreas. It is classified among the pancreatic epithelial tumors, though many are reported to be negative for cytokeratin. Report of five cases cameron d adkisson, adam s harris, mellena d bridges, justin h nguyen, horacio j asbun, john a stauffer abstract introduction. It has received different denominations, including frantz tumor, cystic solid tumor, papillary cystic tumor, papillary epithelial neoplasia, among others.
The treatment methods solid pseudopapillary neoplasm of pancreas are determined by several factors, such as the size and location of tumor, how advanced the condition is, the overall health of the affected individual, as well as hisher personal preferences. Solid pseudopapillary tumor of the pancreas radiology. Solid pseudopapillary tumor of the pancreas, a tumor typically seen in young women, is a large, welldefined, encapsulated lesion with heterogeneous high or low signal intensity on t1weighted, heterogeneous high signal intensity on t2weighted, and. Pdf solidpseudopapillary tumor of the pancreas johann. Clinical update on the management of pseudopapillary tumor of. The patient was submitted to whipples surgery and the surgical specimen was referred to the pathology anatomy service of the hospital. Solid and cystic pseudopapillary tumor of the pancreas.
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