Solide pseudopapilläre Pankreasneoplasie bei Kindern und Jugendlichen

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.

Abstract

Background Pancreatic tumors in children and adolescents are rare entities that can stay asymptomatic for long periods. They often become apparent as incidental findings or due to clinical symptoms, like abdominal pain. Solid pseudopapillary neoplasms of the pancreas (SPN) are rare representatives of this group, being low-grade malignant processes and requiring radical surgical treatment. We present four cases of SPN with different diagnostic and therapeutic approaches.

Methods A retrospective analysis of four cases of SPN treated between 2015 and 2020 was performed.

Results Four female patients (11–17 years) were found to have SPN during diagnostic procedures. Three of them were located in the pancreatic head. Histological confirmation was obtained with endosonographic-, CT-guided and open biopsy, respectively. R0 resection was achieved by a pylorus preserving, partial duodenopancreatectomy according to Traverso-Longmire. In one patient the tumor was located in the pancreatic tail with tumor adherence to the splenic vessels. A CT guided biopsy confirmed an SPN. A distal pancreatectomy and splenectomy was performed. Follow-up (6 months – 6 years) revealed no evidence of tumor recurrence, metastasis, or pancreoprive diabetes.

Conclusion For the treatment plan preoperative histological confirmation of SPN is necessary. Based on the tailored diagnostic and operative concepts, treatment at a center with a specialized pediatric surgery, pediatric oncology, pediatric gastroenterology, pediatric radiology and pathology is mandatory.

Schlüsselwörter

Pankreastumor - Solide pseudopapilläre Neoplasien - Pankreasresektion - Pankreasinsuffizienz

Key words

pancreatic tumors - solid pseudopapillary neoplasia - pancreatic surgery - pancreatic insufficiency

Publication History

Received: 30 June 2021

Accepted: 23 December 2021

Article published online:
21 February 2022

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