Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature

Crucitti, Antonio; Grossi, Ugo; Giustacchini, Piero; Tomaiuolo, Pasquina M. C.; Bellantone, Rocco Domenico Alfonso

doi:10.1007/s13304-010-0006-y

Cited by 13 publications

(7 citation statements)

References 11 publications

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“…The female:male ratio is 10:114 with the incidence reported in females of 86% in adults and children 6. Historically, abdominal pain was the most common presentation of SPT 8.…”

Section: Discussionmentioning

confidence: 99%

“…Prior to this report, only 3 of 11 paediatric patients were successfully diagnosed preoperatively with SPT 42 44–52. Differential diagnoses include a non-functioning islet tumour, pancreatoblastoma, acinar cell cancer, mucinous cystic neoplasm, serous cystadenomas, lymphoma and pseudocyst 7 14 50 53 54. In this setting, an EUS-FNA was performed to rule out lymphoma—a Whipple is contraindicated for this diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Regardless, when evaluating all patients with SPT, surgical resection is the mainstay of therapy with overall 5-year survival greater than 95% (even in the presence of metastases) 14. Fried et al 66 described radiotherapy for unresectable SPT.…”

Section: Discussionmentioning

confidence: 99%

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Solid pseudopapillary tumour (Frantz’s tumour) of the pancreas in childhood

Escobar

Bond²,

Schopp³

2014

BMJ Case Reports

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An 11-year-old girl presented with acute pancreatitis and mass in the head of the pancreas. MRI revealed a heterogeneous right-upper quadrant retroperitoneal mass measuring 6.8×6.1×5.5 cm arising from the pancreatic head. Endoscopic ultrasound with fine-needle aspirate revealed a solid pseudopapillary tumour (SPT) of the pancreas. The patient underwent a pylorus-preserving Whipple procedure. Pathology confirmed SPT. First described by Frantz, SPT represents less than 3% of all exocrine tumours. It is especially rare in children and shows different clinical features compared with adults. In our patient, tumour cells were arranged at the periphery of fibrovascular cores, but they did not show definite gland formation, keratinisation or cytoplasmic pigment accumulation. A periodic acid-Schiff stain without diastase did not show appreciable glycogen within the tumour cells, classic for Frantz's tumour. The literature, diagnosis, management and pathogenesis on this rare entity in children are reviewed and discussed.

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“…The female:male ratio is 10:114 with the incidence reported in females of 86% in adults and children 6. Historically, abdominal pain was the most common presentation of SPT 8.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Solid pseudopapillary tumour (Frantz’s tumour) of the pancreas in childhood

Escobar

Bond²,

Schopp³

2014

BMJ Case Reports

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“…Während pädiatrische SPN häufig im Pankreaskopf lokalisiert sind (60-70 %), befindet sich bei Erwachsenen die Mehrzahl der Franz-Tumore im Pankreaskorpus oder -schwanz (80 %) [16]. Die überwiegende Mehrheit ( > 90 %) der Patienten ist weiblich und zwischen 18 und 35 Jahren alt, während männliche Patienten typischerweise 10 Jahre älter sind [1,6]. Aufgrund der langsam wachsenden abdominellen Tumormasse bleiben die Patienten häufig über lange Zeit asymptomatisch oder zeigen nur milde Symptome.…”

Section: Hintergrundunclassified

Solide pseudopapilläre Pankreasneoplasie bei Kindern und Jugendlichen

et al. 2022

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Zusammenfassung Hintergrund Pankreastumore im Kindes- und Jugendalter sind seltene Entitäten, die lange Zeit asymptomatisch verlaufen und häufig als Zufallsbefund bei bildgebenden Untersuchungen aus anderen Gründen oder durch abdominelle Schmerzen auffallen. Solide pseudopapilläre Pankreasneoplasien (SPN) sind seltene Vertreter dieser Gruppe. Die Therapie der SPN besteht in der primären R0 Resektion. Wir stellen vier Fälle von SPN vor und diskutieren das individualisierte diagnostische und therapeutische Vorgehen. Methode Retrospektive Fallstudie an Hand von vier Patientinnen mit SPNs, die in den Jahren 2015–2020 behandelt wurden. Ergebnisse Bei vier weiblichen Patienten (11–17 Jahre) wurde eine SPN diagnostiziert. Drei SPN waren im Pankreaskopf lokalisiert. Die histologische Sicherung erfolgte durch eine endosonographisch-, CT-gesteuerte oder offene Tumorbiopsie. Eine R0-Resektion wurde mittels pyloruserhaltender, partieller Duodenopankreatektomie nach Traverso-Longmire erzielt. In einem Fall war der Tumor im Pankreasschwanz lokalisiert. Es bestand zusätzlich eine Tumoradhärenz an den Milzgefäßen. Eine CT-gesteuerte Biopsie bestätigte den Verdacht auf eine SPN und es erfolgte eine Pankreasschwanzresektion mit Splenektomie. Im Follow-up (6–72 Monate, Median: 28,5 Monate) ergaben sich bei keinem Patienten Hinweise auf Tumorrezidive, Metastasen oder einen pankreopriven Diabetes. Schlussfolgerung Zur Therapieplanung ist eine präoperative histologische Diagnosesicherung von SPN notwendig. Aufgrund der individualisierten diagnostischen und therapeutischen Konzepte, ist eine Versorgung in Zentren mit spezialisierter Kinderchirurgie, Kinderonkologie, Kinder-Gastroenterologie, Kinderradiologie und Pathologie essentiell.

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“…SPT shows different clinical features in childhood. High survival rates can be achieved in most cases, warranting aggressive treatments even in metastatic disease 5 . A meticulous radiological survey prior to any surgical intervention may aid in the timely diagnosis of this condition.…”

Section: Introductionmentioning

confidence: 99%

Solid pseudopapillary tumour of the pancreas in a child: Case report

Mittal¹,

Nk²,

Mehta³

et al. 2013

OA Case Reports

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Introduction Solid pseudopapillary tumour of the pancreas, though rare, is a low-grade malignancy with indolent behaviour. It is encountered predominantly in young females, although it has been seen in males and children. Most patients present with vague nonspecific abdominal pain resulting in delayed diagnosis. Therefore diagnosis of this tumour may be an incidentalfindingduringdiagnosticimaging procedures or maybe assumed when a young women presents with an asymptomatic palpable mass. Traditionally the presence of a large bulky pancreatic tumour in a child should raise suspicion of the diagnosis of pancreotoblastoma. This study reports a case of a solid pseudopapillary tumour of the pancreas in a child. Case report We present a case of an eight-year-old child presenting with pain and a lump in the epigastric region. Computerised tomography was subsequently performed to localise the mass accurately. Computed tomography showed a minimally enhancing solid mass in the head and proximal body of the pancreas compressing the second and third part of the duodenum and measuring 7.2 × 6.9 × 6.4 cm. Conclusion This case report highlights the fact that with characteristic imaging features the diagnosis of solid pseudopapillary tumour of the pancreas should be considered irrespective of theageprofileofthepatient.

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Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature

Cited by 13 publications

References 11 publications

Solid pseudopapillary tumour (Frantz’s tumour) of the pancreas in childhood

Solid pseudopapillary tumour (Frantz’s tumour) of the pancreas in childhood

Solide pseudopapilläre Pankreasneoplasie bei Kindern und Jugendlichen

Solid pseudopapillary tumour of the pancreas in a child: Case report

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