Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases

Choi, Seung Hoon; Kim, Sang Woo; Oh, Jung Tak; Park, Jin Young; Seo, Jeong Meen; Lee, Suk Koo

doi:10.1016/j.jpedsurg.2006.08.024

Cited by 111 publications

(120 citation statements)

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“…The morphologic features suggesting a benign or malignant phenotype are inadequate to predict tumor biology accurately. [1][2][3][4][5][6][7][8] The application of simple DNA-based ancillary techniques has yielded mixed results as applied to high-and low-risk solid pseudopapillary neoplasms. Flow cytometry studies have demonstrated a trend toward DNA aneuploidy in malignant solid pseudopapillary neoplasms, but diploidy has been observed in both malignant and 'benign' solid pseudopapillary neoplasms.…”

Section: Discussionmentioning

confidence: 99%

“…However, aggressive malignant solid pseudopapillary neoplasms have been reported. [1][2][3][4][5][6] Attempts to separate aggressive from non-aggressive solid pseudopapillary neoplasms based on histologic criteria (such as vascular invasion, necrosis, increased mitotic rate, high nuclear grade) and size has yielded contradictory results with reports of histologically bland solid pseudopapillary neoplasms that have metastasized and histologically malignant solid pseudopapillary neoplasms with indolent outcomes. 4,7,8 Using morphologic features to predict the biological behavior of solid pseudopapillary neoplasms has limitations and is a major obstacle in stratifying patients into low-and highrisk groups as has been observed for other lesions, such as pheochromocytomas and neuroendocrine tumors.…”

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confidence: 99%

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Array comparative genomic hybridization analysis of solid pseudopapillary neoplasms of the pancreas

et al. 2008

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Solid pseudopapillary neoplasms of the pancreas are low-grade malignancies, but their biological behavior cannot be stratified solely on the basis of histopathologic criteria. Aside from mutations in b-catenin and lack of genetic changes common to pancreatic ductal adenocarcinomas, little is known about the chromosomal alterations in solid pseudopapillary neoplasms. We applied array comparative genomic hybridization to a series of 12 patients. The average age was 31 years (range 12-52 years) with 10 female and 2 male patients. The average tumor size was 7.3 cm (range 2-24 cm) with five lesions greater than 5 cm. All cases had 'bland' cytology without significant pleomorphism or high nuclear grade, but seven cases demonstrated at least one of these potentially aggressive histopathologic features: size 45 cm, tumor necrosis, lymphovascular invasion, perineural invasion and peripancreatic invasion. Clinically, one lesion demonstrated aggressive behavior. By array comparative genomic hybridization, chromosomal losses and/or gains were identified in eight cases; five cases had multiple (five or more) alterations. The most common alterations were gains at 13q, 17q, 1q and 8q. Six of the seven cases with at least one aggressive feature had genetic alterations, while only two cases without adverse features had genetic alterations (P ¼ 0.024). The single clinically aggressive tumor exhibited seven chromosomal gains and four aggressive histopathologic features. Our study demonstrates that genetic alterations detected by array comparative genomic hybridization are common in solid pseudopapillary neoplasms of the pancreas. Additional study and longer follow-up are needed to determine if these genetic abnormalities could help predict clinical behavior in these neoplasms.

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Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Array comparative genomic hybridization analysis of solid pseudopapillary neoplasms of the pancreas

et al. 2008

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“…En cuanto al tratamiento del TSP se ha descrito: pancreatectomía total, pancreatodoudenectomía, pancreatectomía distal, asociados o no, a esplenectomía o a disección sistemática de nódulos linfáticos 4,7,8,12,13 . La pancreaticoduodenectomía es comúnmente usada para resecar TSP localizado en la cabeza pancreática, mientras que la pancreatectomía distal es usada para los localizados en la cola.…”

Section: Discussionunclassified

“…La forma de presentación más frecuente es dolor abdominal y/o masa abdominal [1][2][3][4]7,8,[10][11][12] , se han descrito otros síntomas y signos los que incluyen dispepsia 6,7 , náuseas, vómitos 12 , amilasemia elevada 7 , ictericia 1,6,13 , o también, puede ser un hallazgo incidental 2,6 ; otra forma de presentación es como abdomen agudo por hemoperitoneo 2,4,10,13 . Se presenta característicamente en mujeres jóvenes entre la segunda y tercera década de la vida, siendo la relación hombre: mujer de 1:9 1,2,4,6,10,13 .…”

Section: Tumor Pancreáticounclassified

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Tumor sólido pseudopapilar de páncreas, reporte de un caso

et al. 2008

Rev. chil. pediatr.

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Pancreas pseudo-papillary tumor, a case-report Pancreatic tumors are extremely rare, specially in children, where the pancreas pseudo-papillary tumor corresponds to 1% of primary pancreatic tumors, characterized by its low malignant potential. We report the case of a solid pancreas pseudo-papillary tumor in a 5 year-old female patient, presenting with prolonged abdominal pain. An abdominal ultrasonography and computed tomography were performed, showing 2 tumors at the pancreas head and tail, with normal tumor markers (alpha-fetoprotein, carcinoembryonic antigen, β-HCG). The surgical treatment consisted on distal pancreatectomy with tumor enucleation at the pancreas head and tail, afterwards presenting acute pancreatitis that was medically treated. The biopsy confirmed pancreas solid pseudo-papillary tumor. In the 22 months follow-up period, the patient has remained disease-free. RESUMENObjetivo: Las neoplasias de páncreas son muy infrecuentes, más aún en la edad pediátrica, y el tumor sólido pseudopapilar de páncreas corresponde alrededor del 1% de los tumores primarios de páncreas, caracterizándose por su bajo potencial maligno. Reportamos el caso clínico de un tumor sólido pseudopapilar de páncreas en paciente pediátrico. La paciente es una niña de cinco años, la cual consultó por dolor abdominal prolongado, dentro de su estudio se realizó ecografía y escáner abdominal demostrando dos masas en cabeza y cola de páncreas, con marcadores tumorales (alfafetoproteína, antígeno carcinoembrionario, β-HCG) normales. Se realizó pancreatectomía distal y enucleación del tumor de cabeza de CASO CLÍNICO CASE REPORT

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Clinical Features and Outcome of Solid Pseudopapillary Neoplasm

Lee¹

2008

Arch Surg

140

101

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To delineate the clinical and pathological characteristics of solid pseudopapillary neoplasm (SPN), compare them between adults and children, and determine the predictive features suggesting malignant potential. Design: Retrospective analysis of patients who underwent surgery for a pathologically confirmed SPN. Setting: Tertiary care referral center. Patients: Sixty-two consecutive patients who underwent surgery for a pathologically confirmed SPN between 1985 and 2006. Main Outcome Measures: Demographic information and clinical presentation, radiological details, surgical data, pathological characteristics, postoperative course, and long-term survival. Results: Among 62 patients, 47 patients were adults (mean age, 36 years; range, 18-63 years) and 15 patients were children (mean age, 12 years; range, 8-13 years). A palpable mass was the most common presenting symptom in children (9 of 15; 60%) and an incidentally detected pancreatic mass, in adults (18 of 47; 38.3%) (P =.001). The mean tumor size in children was significantly larger than in adults (8.0 vs 6.0 cm; PϽ.03). In children, the tumor was located in the head of the pancreas (10 of 15; 66.7%) and in adults, in the body or tail (38 of 47; 80.9%) (P =.001). Nine patients (14.5%) had malignant SPN. There was no significant clinical factor suggesting malignant potential. Two patients had a tumor recurrence. They were still alive after debulking surgery. There were no tumor-related deaths. Conclusion: Solid pseudopapillary neoplasm had different clinical features in adults and children. Because long-term survival can be achieved, even with the synchronous or metachronous metastatic lesions, SPN should be treated aggressively, with complete resection, even if this requires metastatectomy.

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Solid pseudopapillary tumor of the pancreas: a multicenter study of 23 pediatric cases

Cited by 111 publications

References 28 publications

Array comparative genomic hybridization analysis of solid pseudopapillary neoplasms of the pancreas

Array comparative genomic hybridization analysis of solid pseudopapillary neoplasms of the pancreas

Tumor sólido pseudopapilar de páncreas, reporte de un caso

Clinical Features and Outcome of Solid Pseudopapillary Neoplasm

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