Solid and Papillary Epithelial Neoplasm of the Pancreas in Children

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.

Solid and Papillary Epithelial Neoplasm of the Pancreas in Children

Four children with solid and papillary epithelial neoplasm of the pancreas are reported. Three were girls. Mean age at operation was 12 years and 7 months (10-13 years). Clinical presentation included nausea, vomiting, and apalpable mass. One had hemoperitoneum due to tumor rupture. In two cases, tumors were in the body of the pancreas, and one the body and tail, and in one,the tail. Mean diameter of the tumors was 10.8 cm (8-15cm). Surgical procedures were distal pancreatectomy and splenectomy in 2 cases, distal pancreatectomy in one, and subtotal pancreatectomy and splenectomy in one. Mean follow-up period was 61 months (6-121 months). Three patients are still alive without any recurrence. However, in the one case of ruptured tumor, portal vein thrombosis and liver metastasis developed after subtotal pancreatectomy and splenectomy during the course of postoperative adjuvant chemotherapy.

Solid and Papillary Epithelial Neoplasm of the Pancreas in a Child: A case Report

Solid and papillary epithelial neoplasm (SPEN) of the pancreas is a rare tumor with low malignant potentiality that usually occurs in young females. Preoperative evaluation, especially radiologic tests, including ultrasonography and CT scan, is helpful in the diagnosis. These studies demonstrate a well-demarcated large mass with solid and cystic portions, frequently in the tail or body of the pancreas. Complete resection is usually curative, however local invasion and/or metastasis may occur. The authors report a case of a solid and papillary epithelial neoplasm of the pancreatic body in a 14-year old child at St. Benedict Hospital and review the literature.

A Clinical Review of Solid and Papillary Epithelial Neoplasm of the Pancreas / 한국간담췌외과학회지

BACKGROUND/AIMS: Solid and papillary epithelial neoplasm (SPEN) of the pancreas is a rare malignant tumor with a characteristic female predominance. This tumor has a low- grade malignant potential biologically, and shows favorable prognosis with surgical treatment clinically. METHODS: Nine patients with SPEN of the pancreas who were pathologically confirmed at the Dong-A University Medical Center were analyzed retrospectively. Clinical data, including clinical manifestation, diagnostic maneuvers, surgical treatment modality, and prognosis were evaluated. RESULTS: Six out of nine patients were females with a mean age of 28 (range, 15~52) years. Except in 2 cases, all patients presented with either a palpable abdominal mass (5 cases) or abdominal pain (2 cases). The tumors ranged in size from 3.8 to 18 (average size, 11.4) cm. Five were located in the body, 3 in the tail, and 1 in the head. SPEN was diagnosed preoperatively in 6 patients by abdominal ultrasonography and/or CT. All patients underwent surgical resection, which included subtotal pancreatectomies (4 cases), distal pancreatectomies (4 cases, 1 by laparoscopically) and local excision (1 case). All patiests are survived without evidence of recurrence with a mean follow up of 53.1 (range, 26~69) months. CONCLUSION: SPEN of the pancreas is low-malignant in character, and complete surgical excision is the treatment of choice which warrants a long-term survival.

A Case of Solid and Papillary Epithelial Neoplasm of Pancreas in a Young Adolescent / 대한소아소화기영양학회지

Solid and papillary epithelial neoplasm (SPEN) of the pancreas is an uncommon tumor and is found predominantly in young females. The most common clinical presentation is an abdominal mass. The tumor has a low grade malignant potential and complete removal is the treatment of choice. We report a case of SPEN in a 13-year-old girl who presented with abdominal pain and increasing size of an abdominal mass after abdominal trauma. CT and sonographic findings showed a well-demarcated mass in the pancreas tail with solid and cystic portion. She got a distal pancreatectomy and pathologic finding was SPEN of pancreas.

A Case of Solid and Papillary Epithelial Neoplasm of the Pancreas / 대한소아소화기영양학회지

Solid and papillary epithelial neoplasm of the pancreas is an uncommon low-grade malignant tumor found predominantly in young females. It is rare in childhood. The origin of the tumor is probably from a multipotential stem cell of the pancreas. Neoplasm usually behave like a very low grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. We report a case of solid and papillary epithelial neoplasm of the pancreas in a 14 year-old girl who presented with intermittent abdominal pain and abdominal mass. CT scan shows a large, about 7×6 cm sized, well-marginated hypodense round mass in the head of the pancreas with some ill defined enhancing solid internal portion. She had taken complete excision of the pancereatic mass and Roux-en-Y pancreaticojejunostomy and histologically comfirmed solid and papillary epithelial neoplasm of the pancreas. A brief review of literature was made.

A Case of a Solid and Papillary Epithelial Neoplasm of the Pancreas Associated with Malignant Change / 대한소화기내시경학회지

The solid and papillary epithelial neoplasm of the pancreas is a relatively uncommon disease. It accounts for approximately 1 to 2 percent of all exocrine pancreatic tumors. This benign or low grade malignant tumor is reported to occur predominantly in young women and rarely in men. Recurrence and development of metastasis after resection are found only in a small fraction of the general population. A case is herein reported involving a solid and papillary epithelial neoplasm of the pancreas which extensively spread to nearby organs, in a 34 year-old man. Chief complaints were black stool. Physical examination revealed tenderness on the left upper quadrant of the abdomen. Esophagogastroduodenoscopy revealed multiple cardiac variceal bleeding. Abdominal sonography and CAT scan findings showed a huge lobulated mass on the left upper quadrant area with an internal necrotic portion. Surgical findings showed determined a splenic vein tumor thromboembolus, portal vein involvement, distal stomach involvement, and multiple colonic invasion. Therefore, distal pancreatectomy, wedge resection of the stomach, splenetomy, segmental resection of the transverse colon, and excision of the mass were all performed. Pathologic examination revealed a solid and papillary epithelial neoplasm in the pancreatic tail with a marked dilated splenic vein filled with tumor thromboembolus. The patient has been under chemotherapy since then, and is being closely observed.

Surgical treatment of Solid and Papillary Epithelial Neoplasm of the Pancreas / 한국간담췌외과학회지

BACKGROUND: Solid and papillary epithelial neoplasm(SPEN) is a rare malignant tumor of the pancreas that typically occurs in young females and has an excellent prognosis. This tumor has a low malignant potential, and is highly curable with surgical treatment. METHODS AND MATERIALS: From January 1986 to July 1999, ten patients of solid and papillary epithelial neoplasm of the pancreas pathologically verified at Kyung Hee University Hospital were retrospectively analyzed. Pre-, intra-, and post-operative data were evaluated in all patients to determine optimal management with specific reference to surgical strategy. RESULTS: Nine patients out of ten were females, with a mean age of 21.8 years (range 12-38). Six patients presented with a palpable abdominal mass and four patients with abdominal pain. Remaining patients presented with fever, low back pain, and no specific symptom. Abdominal ultrasonography and/or CT scan showed an abdominal mass in all patients. The correct preoperative diagnosis of SPEN was made in six patients. Incorrect diagnosis included pseudocysts in two and cystadenoma in two. The tumor size ranged from 5 to 14.5 cm in diameter (average 8.85 cm). Four patients with tumor in the pancreatic head had a pylorus preserving pancreaticoduodenectomy (PPPD). Four patients with tumor in the pancreatc tail underwent distal pancreatectomy and splenectomy(without in one). Two patients were performed mass enucleation only. The histologic diagnosis was confirmed in all patients. There were no nuclear atypia and pleomorphism in microscopic findings. Postoperative complications were intraabdominal abscess in one, lymphatics leakages in one, and wound infection in one. All ten patients are alive without sign of recurrence with a mean follow-up of 5.36 years (range 0.1 to 13 years). CONCLUSIONS: SPEN is an indolent tumor of the pancreas with low malignant potential and excellent long-term prognosis and should be considered in the differential diagnosis of large pancreatic masses, especially in young females. Resection is the treatment of choice that is resectable.

Solid and Papillary Epithelial Neoplasms of the Pancreas / 한국간담췌외과학회지

BACKGROUND: Solid and papillary epithelial neoplasm(SPEN) of the pancreas is a rare tumor with low grade malignant potential and usually found in young female patients. The prognosis of this lesion is reported to be much more favorable than other pancreatic neoplasms. METHODS: We report four cases of SPENs of the pancreas treated at the department of surgery, Keimyung University Dongsan Medical Center. Three were women and one was a man and their mean age at the time of surgery was 19 years with the ranges between 11 and 29 years of age. Two patients were admitted with a palpable abdominal mass and another two patients with abdominal pain. Results of laboratory studies including serum amylases and tumor markers were all within normal limits. Diagnosis was made preoperatively in three patients by MRI and needle biopsies. Tumors were located in the head portion in three patients and in the body of the pancreas in one patient. RESULTS: All patients underwent complete resection which involved three enucleations and one pancreaticoduodenectomy. All patients are alive at the time of this report with mean follow up period of 69.5 month (the ranges between 8 and 105 months) without evidence of recurrence. CONCLUSION: In our experience, complete resection of this neoplasm is the treatment of choice and the prognosis is excellent.

A Case of Solid and Papillary Epithelial Neoplasm in Head of Pancreas in a 11-year-old Female / 대한소아소화기영양학회지

Solid and Papillary epithelial neoplasm of pancreas is a rare and low grade malignant tumor. It develops in 2nd or 3rd decades of young female and located in tail of pancreas predominantly. Prognosis is good despite its various histologic features, which suggest a malignant appearance. We report one case of solid and papillary epithelial neoplasm in head of pancreas in a 11-year-old girl who had been suffered from vomiting and right upper abdominal pain for 3 weeks.

Solid and Papillary Epithelial Neoplasm of the Pancreas

BACKGROUND: Solid and papillary neoplasms of the pancreas are very rare tumors that occur predominantly in young women. Most of them are diagnosed because of their large sizes and because they are present with an asymptomatic abdominal mass. Most reports indicate that these large palpable, abdominal masses occur in females in their second and third decades of life. Only a few cases of solid and papillary epithelial neoplasms of the pancreas have been reported. METHOD: Here in, eight cases were reviewed which were treated at the Department of Surgery, Catholic University Medical College affiliated hospital from 1988 to 1996. RESULT: The diagnosis is often implied by radiologic examination with ultrasonography, UGI, ERCP, and computed tomography. Most cases showed well-encapsulated, round, or lobulated masses consisting of both cystic and solid areas. The hallmark histologic pattern of this tumor is a solid and papillary epithelial pattern in a pancreatic neoplasm. There is no specific marker for this neoplasm which could elucidate the obscure histogenetic origin and the phenotypic differentiation. Concluion: Therefore, surgical excision is the primary form of treatment and has favorable results.

A Case of a Solid and Papillary Epithelial Neoplasm of the Pancreas with Local Invasion into the Duodenum / 대한소화기내시경학회지

A solid and papillary epithelial neoplasm of the pancreas is a very rare and low-grade type of malignancy, although an increasing number of cases have been reported in recent years. Patients with a solid and papillary epithelial neoplasm of the pancreas have a good prognosis and may be cured if the disease is diagnosed before metastasis and diffuse local invasion. A 23-year-old female who had a 4-year history of recurrent abdominal pain, was admitted due to lower abdominal pain. Incidentally a calcified, 6 4 cm sized ovoid mass was found in the right upper quadrant, from a simple abdominal X-ray. An ERCP, abdominal sonography, and CT were performed, as well as a pylorus preserving pancreatoduodenectomy. Gross pathologic examination revealed a well encapsulated mass with cystic degeneration and hemorrhagic necrosis. Microscopically, the tumor had papillary fronds with a fibrovascular connective tissue core. There was focal infiltration of tumor cells into the duodenal wall and heterotropic pancreatic tissue in the submucosa. Immunohistochemistry revealed positivity for neuron-specific enolase. The patient maintained a healthy status for one year since the operation.

Solid and papillary epithelial neoplasm of the pancreas in an adult male: A case report and review of the literature / 한국간담췌외과학회지

Solid and papillary epithelial neoplasm of the pancreas(SPENP) is an uncommon low grade malignant tumor histologically distinct from the usual ductal adenocarcinoma and amenable to cure by surgical excision. The main features of SPENP are a peculiar morphology, favorable prognosis, and prediction for women 10 and 40 years of age. In men, the occurrence of SPENP seems to be exceedingly rare. The tumor is usually large at the time of presentation, and surgical excision is the treatment of choice. Gross pathologic examination revealed apparent encapsulation, cystic degeneration, and hemorrhagic necrosis. Microscopically, the tumor was characterized by distinctive solid and papillary patterns. This benign or low-grade malignant epithelial tumor is composed of monomorphous cells variably expressing epithelial, mesenchymal, and endocrine markers. The tumor is known to have good prognosis; although local invasion and infiltration of the capsule may occur. Despite growing recognition of this tumor, its histogenesis remains a matter of controversy i.e. pancreatic ductal cell origin, acinar cell origin, pluripotential cell origin. A 44-year-old male presented with an one-year history of postprandial abdominal pain. Following abdominal ultrasonography, computed axial tomography and angiography, a SPENP suspected. The patient underwent exploratory laparotomy. The tumor was located in the head of pancreas. And thus the patient underwent a pylorus-preserving pancreaticoduodenectomy. His postoperative course was uneventful, and he remains well.

A Clinicopathological Study of Solid and Papillary Neoplasm of Pancreas / 영남의대학술지

Solid and papillary epithelial neoplasm of pancreas is a rare tumor, usually affecting young women, and its histogenesis is still controversial. This study was performed to define the clinicopathologic features and cellular origin of this tumor. Eight female cases of solid and papillary epithelial neoplasm of pancreas were studied by analyzing the clinicopathologic findings and immunohistochemical and electron-microscopic findings. The age of eight cases ranged from 21 to 54 years (mean, 34 years). The tumors developed in the tail (4 cases), body-tail (2 cases), body (1 case) and head (1 case). The mean diameter of tumors was 9.3 cm (range, 5.5 to 13 cm). Tumors showed solid, cystic and hemorrhagic areas. Histologically, the tumor cells were uniformly round or polygonal in shape, and formed solid sheets and papillary pattern. On the immunohistochemical stain, 8 cases (100%) were immunoreactive for alpha1-antitrypsin, 7 cases (87.5%) for cytokeratin, 7 cases (87.5%) for progesterone receptor, 6 cases (75%) for vimentin, and 1 case (12.5%) for synaptophysin, respectively. None of them were immunoreactive for estrogen receptor. Electron microscopic examination showed many mitochondria, annulate lamellae and canaliculi-like gap. These findings suggest that solid and papillary epithelial tumor of pancreas possibly originates from totipotential stem cells.

Solid and Papillary Epithelial Neoplasm of the Pancreas in Children

Six children with solid and papillary epithelial neoplasm of the pancreas were studied retrospective manner. There were 2 boys and 4 girls in this series. The mean age at operation was 11 years (range, 8 to 13 years). There were three incidental abdominal masses, two nontender abdominal masses and one tender abdominal mass. The size ranged from 6.5x6.0cm to 10.5x8.0cm. Five tumors were located in the head of pancreas, whereas one tumor was in the tail of pancreas. On exploration, all patients had no local invasion or metastasis. All patients underwent complete resection, which included three pylorus-preserving pancreaticoduodenectomy, two Whipple's operation and one distal pancreatectomy. All patients had the characteristic histologic pattern of a solid and papillary epithelial neoplasm of the pancreas. All patients are alive with a mean follow up of 5.0 years (range, 0.5 to 12.0 years) without recurrence. Compare to the adult, solid and papillary epithelial neoplasm of the pancreas in children had a slight higher incidence in male. We speculate that this tumor have the characteristic of low-grade malignancy. So complete resection is the treatment of choice for the neoplasm arising anywhere in the pancreas.

Papillary and Solid Epithelial Neoplasm of the Pancreas with Multiple Metastases

Papillary and solid epithelial neoplasm is a rare pancreatic tumor of low-grade malignancy. We report a case of a 23 year old female having solid and papillary neoplasm of the pancreatic tail with mutiple omental and peritoneal metastases. Microscopically, the main tumor showed typical histologic findings including solid and papillary areas with cystic change. But the metastasizing nodules were largely solid and the tumor cells demonstrated increased nuclear pleomorphism, hyperchromasia and an increased mitotic rate. The tumor cells contained considerable amount of intracellular and extracellular eosinophilic inclusions which were ultrastructually zymogen-like granules. These inclusions were more frequently found in the metastatic nodules. By flow cytometric study, the tumor was hyperdiploid. The DNA index was not significant.

Solid and Papillary Epithelial Neoplasm of the Pancreas Diagnosed by Percutaneous Fine Needle Aspiration Biopsy

Solid and papillary epithelial neoplasm of the pancreas is a rare, low grade malignant tumor of adolescence. The tumor we described was diagnosed by following preoperative percutaneous fine needle aspiration biopsy and confirmed by tissue examination. Characteristic cytologic findings were monotonous cells arranged in papillary fronds, acinus, and cords in necrotic and hemorrhagic backgrounds. Occasional foamy histiocytes and hyaline globules were present. The cytoplasm of the tumor cells were moderate in amount, pale eosinophilic or vacuolated with distinct borders. The nuclei were centrally located and round. There were one or two small nucleoli and finely granular chromatin throughout the nucleus.