A retrospective study of medical records of all patients treated from january 1997 until july 2015. It includes a summary of the current literature to provide a reference for the management of this rare clinical entity. Diagnosis and treatment of solid pseudopapillary tumor of. It typically affects young noncaucasian females in their second or third decade of life. Due to rarity of the disease, most data on spt are derived from case reports and small case series. It accounts for 2%3% of pancreatic tumors in adults but differs from other pancreatic neoplasms by a clear preponderance in females and a low. Solid pseudopapillary tumor spt of the pancreas is a rare exocrine pancreatic tumor, which represents only about 1% of all tumors of the pancreas. Surgical resection is generally curative and the prognosis is excellent. Asymptomatic versus symptomatic solid pseudopapillary. Solidpseudopapillary tumor of the pancreas is an enigmatic tumor, mostly presenting with benign course or, less frequently, with aggressive behaviour or relapse after resection. We present an unusual case of a 42yearold man who had a recurrence of his solid pseudopapillary tumor 4 years after undergoing a. In this report, a case of the rare solid pseudopapillary tumor of the pancreas is described.
The clear cell variant of solid pseudopapillary tumor of the pancreas. Tumor with prominent blood vessels and small cyst like spaces contrasts with normal pancreatic acini at right. Clinicopathological features of solid pseudopapillary tumor of the. Solid pseudopapillary tumor is a rare primary neoplasm of the pancreas that typically affects young women.
A solid pseudopapillary neoplasm spn of the pancreas is a rare pancreatic tumour. This neoplasm is also known by other names including solid and papillary epithelial neoplasm, solid and cystic tumor, papillarycystic neoplasm of the. The incidence of solid pseudopapillary neoplasm spn of the pancreas is rising. In this paper, therefore, we propose an algorithm for diagnosis and treatment of this rare type of tumor. Spn accounts for less than 1 % to 2 % of exocrine pancreatic tumors. Over time many terms have been used to describe the same tumor, such as frantzs tumor, solid and papillary tumor, papillary cystic tumor, solid cystic tumor, solid cystic and papillary epithelial neoplasm, benign or malignant papillary tumor of the pancreas, papillary epithelial neoplasm of pancreas in a child. 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 the pancreas 2. Pdf solidpseudopapillary tumor of the pancreas spt has distinctive morphologic and biologic features but an unclear origin. Spt of the pancreas is rare and was first described by franz in 1959. Its synonyms are solidcystic tumor, papillarycystic tumor, solid and. Discussion spts of the pancreas are rare 1%2% of exocrine pancreatic tumors at most institutions. Solidpseudopapillary neoplasm of the pancreas is a relatively uncommon tumor.
Overexpression of sox11 and tfe3 in solidpseudopapillary. Most pancreatic tumors are malignant and have a bad prognosis. Solid pseudopapillary tumor should be part of differential. All patients underwent preoperative abdominal ultrasound, computed assisted tomography, and tumor markers. Diagnosis and treatment of solid pseudopapillary tumor of the.
Solid pseudopapillary tumor of the pancreas springerlink. 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. It is most commonly seen in young female patients 2, 5, and only a few cases have been reported in male patients aged 60 years. Although the evidence for proper management is accumulating, we still lack diagnostic and therapeutic guidelines. Typical solid pseudopapillary tumor is characterized by a wellencapsulated mass with varying amounts of intratumoral hemorrhage. It is a relatively a benign tumor, with a favorable prognosis. Solidpseudopapillary neoplasm, pancreas clinics in oncology. Dec 25, 2018 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. The major differential diagnosis includes pancreatic neuroendocrine tumor pannet. Abdominal examination revealed a lump palpable in the right upper abdomen. Since 1970, several other terms have been used, including solid and cystic acinar cell tumor, papillary epithelial neoplasm, solid and papillary neoplasm, papillarycystic tumor, solid cystic tumor, and papillary tumor of the pancreas frantzs tumor. Virginia kneeland frantz in 1959 as a papillary cystic tumor of the pancreas in a 2 year old male patient. 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 is considered a lowgrade malignancy that is apparently curable with surgical complete excision in most instances.
They are generally large, encapsulated masses with mixture of solid, cystic, and hemorrhagic components. Solid pseudopapillary tumors spt are rare, slow growing tumors of the pancreas that typically affect young women. Clear cell variant of solid pseudopapillary neoplasm is also reported to be glycogen negative you are here. Notohara k, hamazaki s, tsukayama c, nakamoto s, kawabata k, mizobuchi k, sakamoto k, okada s, solid pseudopapillary tumor of the pancreas. Solid nests of poorly cohesive cells forming a cuff surrounding blood vessels, resulting in a pseudopapillary architecture. Objective to assess the specificity of increased 18f.
Solidpseudopapillary tumor of the pancreas radiographics. In most cases, solid pseudopapillary tumours should be resected surgically, as there is a risk of malignancy cancer. Solidpseudopapillary neoplasms spns are rare, accounting for 1% to 2% of all exocrine pancreatic tumors. Solid pseudopapillary tumors spt of the pancreas are rare usually benign pancreatic tumors. Nov 20, 2015 solid pseudopapillary tumor of the pancreas is extremely rare in children. Solid pseudopapillary tumor of the pancreas sptp is a rare type of pancreatic tumor. Organization renamed this tumor solid pseudopapillary tumor of the exocrine pancreas. Solid pseudopapillary tumor of the pancreas is a rare nonfunctioning tumor. Although most commonly presenting in females in the second to fourth decade of life, it. Some patients, however, may present with an abdominal mass or have discomfort. It is extremely rare and has low malignant potential 1,2.
This study was conducted to describe the mr imaging features of solid pseudopapillary tumor of the pancreas. 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. Solid pseudopapillary tumor of the pancreas in children. Tumor cells usually have a moderate amount of eosinophilic. Typical solid pseudopapillary tumor is characterized by a wellencapsulated mass with varying amounts of.
Solid pseudopapillary neoplasm of the pancreas is a rare entity with low malignant potential and excellent overall prognosis. Solid pseudopapillary neoplasm of the pancreas jop. Recurrent solid pseudopapillary neoplasm of pancreas. They were first described by frantz in 1959 as a papillary tumor of the pancreas, benign or malignant. Approximately 500 cases of spt have been described in the last 40 years 2,4,7. Solid pseudopapillary tumor of the pancreas spt, frantzs tumor is very rarely diagnosed in children. Jan 07, 2009 solid pseudopapillary neoplasm of the pancreas is a relatively uncommon tumor. Solid pseudopapillary tumour of the pancreas is a rare neoplasm 1%. Solid pseudopapillary neoplasm of the pancreas sciencedirect. Diagnosis and surgical treatment of solid pseudopapillary neoplasm. The aim of this study is to report our experience with spn of the pancreas. Solid pseudopapillary tumor of the pancreas is a pancreas tumor which is occasionally seen in pediatric patients. This tumour primarily affects young women and is usually treated with surgical resection with a relatively favourable prognosis.
Solid pseudopapillary tumor of the pancreas request pdf. It classically presents as a large tumor with cystic and solid components. A, a solidcystic tumor arrows is shown in the head of the pancreas on gray scale sonography. The diagnosis of solid pseudopapillary tumour of the pancreas is usually confirmed with endoscopic ultrasound eus guided fine needle aspiration fna of the lesion. Solid pseudopapillary neoplasm of the pancreas spn is a very rare tumor with a low malignant potential. The lesion appears round with a welldefined margin and mixed cysticsolid. Solid pseudopapillary neoplasm spn of the pancreas is a rare tumor which predominantly occurs in young female. Solid pseudopapillary tumor of the pancreas radiology case.
It has received different denominations, including frantz tumor, cystic solid tumor, papillary cystic. These tumors predominantly affect young female patients and usually have a favorable prognosis 1, 2. Solid pseudopapillary neoplasm spn of the pancreas is a rare tumor of low malignant potential. Solid pseudopapillary tumour of pancreas bmj case reports. It was also known as franzs tumor or hamoudi tumor until the world health organization who labeled it as a solid pseudopapillary tumor in 1996. Stroma usually shows various degrees of hyalinization or evidence of degeneration such as hemorrhage, foamy macrophages, calcification and cholesterol clefts. The most frequent presenting symptom is abdominal pain. We present a case of solid pseudopapillary pancreatic tumor that represented a challenge to the radiologists. 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. Although solid pseudopapillary neoplasms spns are considered tumors of low malignant potential, pa. Histologic features of this neoplasm are usually specific. Laparoscopic surgery for solid pseudopapillary tumor of. Management edit in most cases, solid pseudopapillary tumours should be resected surgically, as there is a risk of malignancy cancer.
The entity was first described in 1959 by pathologist virginia kneeland frantz and in 1996 reclassified by the world health organization who. Solidpseudopapillary neoplasms spns of the pancreas are rare tumors that predominantly occur in young women. In contrast to other pancreatic tumors, the semimalignant solid pseudopapillary tumor has a favorable prognosis. The purpose of this pictorial essay is to illustrate the various appearances of solid pseudopapillary tumor of the pancreas. Solid pseudopapillary tumor of the pancreas spt is a rare tumor of low malignant potential, mostly described in young women. Solid pseudopapillary neoplasms of the pancreas are uncommon and usually occur in young women. Surgical resection is the treatment of choice and its prognosis is excellent.
Histopathological studies made a definitive diagnosis of solidpseudopapillary tumor. Patients diagnosed with spn between 1998 and 2012 were identified from the national cancer data base ncdb n389. Solid pseudopapillary tumor of the pancreas spt, also known as frantzgruber tumor, was originally established as a separate entity in 1959 1 and represents 1% to 3% of all pancreatic tumors. Jinping lai, md, phd solid pseudopapillary neoplasm of the pancreas is a rare entity with low malignant potential and excellent overall prognosis. Solid pseudopapillary tumor of the pancreas is a rare neoplasm usually found in young women. Solid pseudopapillary neoplasia spn of the pancreas is an extremely rare epithelial tumor of low malignant potential. It has nonspecific clinical presentations such as abdominal pain and nausea, with vague radiologic features. Solid pseudopapillary tumor of the pancreas frantzs tumor. 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. The tumor has been referred to with multiple different names, including. It typically affects young women, has nonspecific clinical and radiologic manifestations, and can be readily diagnosed by ultrasoundguided fineneedle aspiration and histopathologic evaluation.
Characteristics and treatment outcomes were compared between genders. The patient was submitted to whipples surgery and the surgical specimen was referred to the pathology anatomy service of the hospital. Solid pseudopapillary neoplasm of the pancreas archives of. Solid pseudopapillary tumor of the pancreas is typically large at presentation 10cm on average, has a proponderance for the tail of pancreas and contains both solid and cystic components. They are rare and thought to account for 12% of exocrine pancreatic tumors. Spn accounts for less than 1% to 2% of exocrine pancreatic tumors. In this retrospective study from january 2000 december 2010, there. Solidpseudopapillary neoplasm spn of the pancreas radiologypathology correlation thomas loehfelm, md, phd aarti sekhar, md department of radiology and imaging sciences. Solid pseudopapillary neoplasms spns are the most common pediatric pancreatic tumor. Asymptomatic versus symptomatic solid pseudopapillary tumors. 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 t1. From 1997 to 20, spt was diagnosed in 18 patients among 451 pancreatic cystic neoplasms 3.
Printable solid pseudopapillary neoplasm of the pancreas. It has a enhancing capsule, may contain hemorrhagic components and in less than 10% contains calcification. Solid pseudopapillary neoplasm spn is a type of pancreatic neoplasm. Sox11 is widely expressed during embryogenesis, including the development of fetal pancreas, but absent in the majority of adult tissues. Pdf solidpseudopapillary tumor of the pancreas johann. Solid pseudopapillary tumor of the pancreas frantzs. We report an 18yearold female patient presenting with moderate grade abdominal pain for 5 weeks. Treatment involves complete excision of the tumor which. Solid pseudopapillary neoplasm of the pancreas is one of the rarest forms of pancreatic neoplasm. This is a lowgrade tumor that usually occurs in children but is rare in adults and, in exceptional cases, can show extrapancreatic localization. At macroscopic examination, the tumor was located in the head of the pancreas, measuring. They tend to present in young noncaucasian females around the 2 nd and 3 rd decades of life. However, solidpseudopapillary tumor of the pancreas spt is an unusual lowgrade malignancy that rarely metastasizes.
This research analyzes characteristics and outcomes of this disease by gender. Incidental lesions, solid pseudopapillary tumor, pancreas, computed tomography background solid pseudopapillary tumors spts of the pancreas used to be called frantz tumors, solid cystic tumors, or solid pseudopapillary neoplasms. Surgical resection is known to offer excellent long term survival. Pdf diagnosis and treatment of solid pseudopapillary tumor of the. Solid pseudopapillary tumor of the pancreas in a 45yearold man. The solid pseudopapillary neoplasm spn of the pancreas was first described by frantz in. Solid pseudopapillary neoplasm of the pancreasproposed. Clinical update on the management of pseudopapillary tumor of. Pdf solid pseudopapillary neoplasia spn of the pancreas is an extremely rare. Longterm outcomes after surgical resection are excellent and recurrences after an r0 resection are extremely rare. Epigenetics of solid pseudopapillary neoplasm of the pancreas. How is solidpseudopapillary neoplasm of pancreas treated. This tumor was first described by frantz in 1959 as a papillary tumor of the pancreas. Solid pseudopapillary neoplasm of the pancreas in a young.
Mr imaging features of solid pseudopapillary tumor of the. Very graphic content related to the type of surgery, organs. Jahangir s, loya a, siddiqui mt, noreen n, yusuf ma. About 8384 cases of solid pseudopapillary neoplasms spn of pancreas have been published in english literature, from 1933 to 2018. Preoperative diagnosis is substantially made by ct or mri in most cases. Approximately 90% of spns occur in young women, with. 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. Fdopa uptake in patients who underwent petct for suspicion of isolated pancreatic neuroendocrine tumor pnet. Different methods of resection of solid pseudopapillary. Solid pseudopapillary neoplasm of pancreas is a rare tumor with a low potential for metastasis and recurrence. Solid pseudopapillary neoplasm of the pancreas is a rare tumor, with low potential of malignancy, of uncertain lineage, and favorable prognosis in most cases it has received different denominations, including frantz tumor, cystic solid tumor, papillary cystic tumor, papillary epithelial neoplasia, among others. The solid pseudopapillary neoplasm is a rare tumor of the pancreas. Solid pseudopapillary neoplasm spn is a rare tumor with malignant potential which is generally located in the tail of pancreas. Solid pseudopapillary tumor of the pancreas radiology.