|Year : 2015 | Volume
| Issue : 2 | Page : 103-107
Solid pseudopapillary tumor of the pancreas
Edigara Govindaraj1, Nihal Ahemad2, Ravindranath M Meti2
1 Department of Oncosurgery, Vijayanagara Institute of Medical Sciences, Bellary, Karnataka, India
2 Department of Surgery, Vijayanagara Institute of Medical Sciences, Bellary, Karnataka, India
|Date of Web Publication||10-Apr-2015|
Room No. 5, 17th Block, VIMS Hostel, Cantonment, Bellary - 583 104, Karnataka
Source of Support: None, Conflict of Interest: None
The solid pseudopapillary tumor of the pancreas (SPPT) is an unusual low-grade malignant epithelial tumor affecting predominantly adolescent girls and young women, usually misdiagnosed as pancreatic pseudocyst. Although approximately 500 cases of SPTP have been described in the last 40 years, its pathogenesis remains uncertain. However, the clinical features of this neoplasm are very characteristic and SPPT must be suspected in any young woman with a cystic or partially cystic pancreatic mass. The most frequent symptom of SPPT is upper abdominal pain seen in nearly half the patients. The differential diagnosis of SPPT includes pseudocyst of pancreas, neuroendocrine tumor and ductal adenocarcinoma of pancreas. Diagnosis of SPPT is usually made only after operative biopsy. Surgical excision offers the best chance for cure and should always be attempted irrespective of the magnitude of resection involved. This case report highlights the importance of awareness of SPPT as a distinct class of pancreatic tumor in young females with excellent prognosis.
Keywords: Excellent prognosis, solid pseudopapillary tumor of pancreas, young females
|How to cite this article:|
Govindaraj E, Ahemad N, Meti RM. Solid pseudopapillary tumor of the pancreas. Int J Health Allied Sci 2015;4:103-7
| Introduction|| |
Solid pseudopapillary tumor (SPPT) of the pancreas is a rare entity accounting for 0.5-2.5% of primary pancreatic tumors with slow growth rate and excellent prognosis.  SPPT is usually seen in young females and is usually misdiagnosed as pancreatic pseudocyst. Although approximately 500 cases of SPPT have been described in the last 40 years, 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. Awareness of clinical, microscopic and macroscopic features along with sufficient sampling of tumor is necessary for correct diagnosis of SPPT. This case report highlights the importance of awareness of SPPT as a distinct class of pancreatic tumor in young females with excellent prognosis. Although the most common presentation is upper abdominal pain, this patient presented with an epigastric mass. Thus, this case is an uncommon presentation of a relative rare disease.
| Case report|| |
A 35-year-old female came to the outpatient Department of Vijayanagar Institute of Medical Sciences, Bellary, Karnataka with complaints of mass per abdomen of 3 months duration. Initially there was no pain but gradually it was associated with dull aching type of pain around the epigastric region, radiating to the back. No other specific complaints.
On examination, patient had no signs of pallor, icterus, cyanosis or generalized lymphadenopathy. Per abdomen revealed a mass of size roughly 10 cm × 12 cm in the epigastric and periumblical region. On palpation, mass was firm in consistency, freely mobile, nontender, nonpulsatile, not moving with respiration. No other mass was palpable. No other organomegaly. No free fluid in the abdomen.
Computed tomography (CT) scan revealed a well-defined, noncalcified, solid-cystic pancreatic mass lesion in the head of the pancreas about 14 cm × 20 cm size. Pancreatic duct and spleen was normal in size [Figure 1],[Figure 2] and [Figure 3].
Wide local excision of the tumor was done. Per-operative examination revealed a well-defined 14 cm × 20 cm solid-cystic mass in pancreatic head region. No evidence of peripancreatic lymphadenopathy was seen.
Cut section showed growth had granular appearance with embedded areas of hemorrhage and necrosis [Figure 4] and [Figure 5].
Partially encapsulated tumor arranged in sheets and trabeculae of small round cells (pseudopapillae) were seen. The cells had round to oval nuclei with finely dispersed chromatin, inconspicuous nuclei and eosinophilic cytoplasm. Occasionally cells showed nuclear grooving. Pseudorosetting along with presence of foam cells was also seen [Figure 6],[Figure 7] and [Figure 8].
With these histological features, final diagnosis of SPPT of pancreas was made.
The postoperative period was uneventful. The patient is on regular follow-up for a period of 1 year now and is completely asymptomatic.
| Discussion|| |
Cystic pancreatic neoplasms were first classified by Robson and Moynihan in 1903. In 1959, Frantz described a rare tumor known as papillary tumor of the pancreas, benign or malignant. This tumor was known by various names like Gruber-Frantz tumor, solid and cystic tumor, solid and papillary epithelial neoplasm, papillary cystic tumor and solid-cystic papillary epithelial neoplasm. In 1996, the World Health Organization renamed this tumor as SPPT for the international histological classification of tumors of the exocrine pancreas. 
The cell of origin of SPPT is unknown. Some authors have reported SPPT as neoplasm of uncommitted cells with most cells similar to intercalated duct cells or centroacinar cells. 
The most frequent symptom of SPPT is upper abdominal pain seen in nearly half the patients. SPPT may occur anywhere in the pancreas, but is most frequently found in the head or tail. The classic CT features of SPPT are a large well-encapsulated mass with varying solid and cystic components caused by hemorrhagic degeneration. Calcifications and enhancing solid areas may be present at periphery of mass.  Atypical appearances include: Metastasis to liver, ductal obstruction, extracapsular invasion, dense calcification and male patients.  Nishihara et al. reported venous invasion, diffuse growth pattern, extensive tumor necrosis, significant nuclear atypia and high mitotic rate as indicators of malignant potential of SPPT. 
Magnetic resonance imaging typically demonstrates a well-defined lesion with heterogeneous signal intensity on T1- and T2-weighted images, which reflects the complex nature of the mass. Areas of high signal intensity on T1-weighted images and low or inhomogeneous signal intensity on T2-weighted images can help identify blood products and may also help differentiate SPPTs from islet cell tumors, whose cystic components have moderately increased signal intensity on T1-weighted images and increased signal intensity on T2-weighted images. Moreover, the peripheral portions of SPPTs do not demonstrate the hypervascularity typically seen in islet cell tumors. 
Ultrasonography guided fine-needle aspiration cytology (FNAC) remains an useful adjunct to the diagnosis of SPPT. The highly cellular smears show numerous papillary tissue fragments with slender branching fibrovascular stalks that are characteristic of this tumor. The tumor cells form two or several layers on the fibrovascular core. Pseudorosette formations are also described. Apart from these papillary fragments, there may be many discrete tumor cells. The individual tumor cells have been described as being monomorphous having round to oval eccentric nuclei, bland nuclear chromatin pattern with small nucleoli and exhibiting longitudinal grooves and convolutions; the cytoplasm being eosinophilic. 
In addition to perivascular and papillary fragmentation Jayaram et al. considered the presence of intracytoplasmic inclusions as a distinctive feature of this neoplasm.  Cappellari et al. considered the nuclear folds or grooves to be a characteristic of this tumor.  Binucleation and multinucleation of tumor cells are also described. Mucinous change in the stalks of papillae, foamy cells and multinucleated giant cells are also mentioned earlier in reports.
The differential diagnosis of SPPT includes pseudocyst of the pancreas, neuroendocrine tumor and ductal adenocarcinoma of pancreas. Ductal adenocarcinoma is most commonly seen in elderly male and grows in a infiltrative manner. Neuroendocrine tumors present with a solid or microacinar pattern and nuclei are small, round, smoothly contoured with fine chromatin. Moreover, peripheral portions of neuroendocrine tumors show hypervascularity on arterial phase CT images. Absence of history of pancreatitis in presence of solid and cystic pancreatic mass in young age group patient must raise suspicion for SPPT. ,
Final diagnosis of SPPT is usually made only after operative biopsy. SPPT has very good prognosis unlike ductal adenocarcinoma of the pancreas. Even benign SPPPT tumors have malignant potential and correct treatment by total surgical excision is curative.  Our case report thus highlights the importance of awareness of SPPT as a distinct class of pancreatic tumor in young females with excellent prognosis.
A single institution study of 14 cases conducted by Tata memorial hospital Mumbai (one of the largest study for SPPT in India) also concluded that surgical excision offers the best chance for cure and should always be attempted irrespective of the magnitude of resection involved. 
Dr. Sharma and Hiralal from Department of Radiodiagnosis, Sanjay Gandhi PGIMS, Lucknow reported a case series of three cases in their institution. All were well encapsulated tumors involving body of the pancreas. Distal pancreatectomy with spleen preserving surgery was performed. 
In another case series done in Dayananda Medical College and Hospital, Ludhiana they reviewed retrospectively five such cases of SPPT over a period of 6 years. In their series, all the patients who underwent resection were disease free on a follow-up of 1 year. 
Solid pseudopapillary tumors show 15% metastatic rate involving liver and peritoneum. Even in the presence of disseminated disease, the clinical course is usually protracted and overall 5 years survival rate is 97%. 
A recent series from Memorial Sloan-Kettering Cancer Center, New York, USA, recommends complete surgical excision with even metastatectomy if required. These authors conclude that long-term survival improves with complete surgical resection of primary with metastatectomy for synchronous or metachronous lesions. 
| Conclusion|| |
A very high degree of clinical suspicion is necessary to diagnose SPPT of the pancreas. This diagnosis should always be borne in mind when any young female presents with a pancreatic mass. CT scan and if possible endoscopic ultrasound are essential to the preoperative diagnosis. FNAC appears to aid in the specific diagnosis of SPPT. Surgical excision offers the best chance for cure and should always be attempted irrespective of the magnitude of resection involved and other adverse factors. Patients with SPPT have an excellent prognosis after surgical excision.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]