Der solid pseudopapilläre Pankreastumor (SPT) – eine seltene Raumforderung der Bauchspeicheldrüse

Zusammenfassung

Hintergrund: Der seltene, niedrig maligne SPT betrifft überwiegend junge Frauen. Das Langzeitüberleben nach Resektion liegt bei ca. 95 %. In Ausnahmefällen findet sich das solid pseudopapilläre Karzinom (SPC) mit eindeutigen Malignitätskriterien. Inoperable Lebermetastasen können mit RFA, TACE oder Chemotherapie behandelt werden. Methoden: Retrospektive Analyse des chirurgischen Vorgehens, der Immunhistochemie und des klinischen Verlaufs von fünf Patientinnen (1998 – 2007). Ergebnis: Das mittlere Alter betrug 16 Jahre (13 – 47). Die R0-Resektion wurde durch kephale Pankreoduodenektomie (n = 3), Pankreaslinksresektion (n = 1) und Enukleation (n = 1) erzielt. Es zeigte sich ein mittlerer Tumordurchmesser von 8 cm (6 – 15), eine Gefäßinvasion (3 / 5) und ein Lymphknotenbefall (1 / 5). Vier Patientinnen mit SPT sind nach 30 bis 101 Monaten rezidivfrei. Die Patientin mit SPC und Metastasierung lebt nach 98 Monaten unter Chemotherapie. Schlussfolgerung: Der SPT ist ein niedrig maligner Tumor. Eine radikale Resektion des SPT führt zu einem rezidivfreien Langzeitüberleben. Beim SPC mit inoperabler Metastasierung scheint die Chemotherapie das Überleben zu verlängern.

Abstract

Background: In general, the rare SPT is a tumour of low malignancy predominantly affecting young women. The outcome after radical resection is favourable. In exceptional cases the tumour presents as solid pseudopapillary carcinoma (SPC) with typical malignant features and even metastases. Unresectable liver metastases can be treated with RFA, TACE or chemotherapy. Methods: We retrospectively reviewed the surgical approach, immunohistochemistry and clinical outcome in five female patients (1998 – 2007). Results: The mean age was 16 years (range: 13 – 47 years). For radical tumour removal a pancreato – duodenectomy (n = 3), a distal pancreatectomy (n = 1) and an enucleation (n = 1) were performed. We encountered a mean tumour diameter of 8 cm (range: 6 – 15 cm), an angioinvasion (3 / 5) and a lymphatic infiltration (1 / 5). After 30 to 101 months follow-up four patients were free of recurrence. Chemotherapy has resulted in a survival of over 98 months in a case of SPC with liver metastases. Conclusion: SPT is a tumour of low malignancy. Radical resection is recommended for long-term recurrence-free survival. Chemotherapy may prolong survival in SPC with unresectable metastases.

Schlüsselwörter

solid pseudopapillärer Pankreastumor - solid pseudopapilläres Karzinom - Immunhistochemie - Lebermetastasen - Chemotherapie

Key words

solid pseudopapillary tumour - solid pseudopapillary carcinoma - immunohistochemistry - liver metastases - chemotherapy

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