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Erschienen in: International Journal of Colorectal Disease 12/2016

28.09.2016 | Original Article

Prophylactic total gastrectomy in the management of hereditary tumor syndromes

verfasst von: Dimitrios Pantelis, Robert Hüneburg, Ronja Adam, Stefanie Holzapfel, Heidrun Gevensleben, Jacob Nattermann, Christian P. Strassburg, Stefan Aretz, Jörg C. Kalff

Erschienen in: International Journal of Colorectal Disease | Ausgabe 12/2016

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Abstract

Purpose

Germline mutations in several genes confer a relevant lifetime risk of gastric cancer. In this context, an increasing involvement of a surgeon can be seen, mainly with the question of performing a prophylactic operation.

Methods

Patients with hereditary tumor syndromes predisposing for gastric cancer who received care leading to prophylactic total gastrectomy in our Center for Hereditary Tumor Syndromes were analyzed. For each patient, the multidisciplinary decision-making process, the perioperative course, and the histopathologic findings were assessed. Short-term morbidity was evaluated based on the medical reports.

Results

The analysis includes nine patients (six female, three male) with a median age of 41.6 (range 23–60) years. Indication for prophylactic total gastrectomy was based on family history and genetic analysis (eight patients with a germline mutation of the CDH1 gene and one patient with a SMAD4 mutation). Removal of the entire gastric mucosa was documented intraoperatively by fresh frozen section examination. Extended (DII) lymphadenectomy was performed in four patients. Histopathologic examination of gastrectomy specimens revealed six patients (6/9, 67 %) with multifocal signet ring cell carcinomas. In our series, prophylactic total gastrectomy was a safe procedure without mortality and low morbidity.

Conclusions

Patients with hereditary syndromes predisposing for gastric cancer should be evaluated for this curative procedure in a specialized center. Further research is necessary, and the implementation of nationwide registers including patients with prophylactic gastrointestinal operations due to hereditary tumor syndrome is advisable.
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Metadaten
Titel
Prophylactic total gastrectomy in the management of hereditary tumor syndromes
verfasst von
Dimitrios Pantelis
Robert Hüneburg
Ronja Adam
Stefanie Holzapfel
Heidrun Gevensleben
Jacob Nattermann
Christian P. Strassburg
Stefan Aretz
Jörg C. Kalff
Publikationsdatum
28.09.2016
Verlag
Springer Berlin Heidelberg
Erschienen in
International Journal of Colorectal Disease / Ausgabe 12/2016
Print ISSN: 0179-1958
Elektronische ISSN: 1432-1262
DOI
https://doi.org/10.1007/s00384-016-2656-9

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Inhalte des Webinars zur S1-Leitlinie „Empfehlungen zur Therapie der akuten Appendizitis bei Erwachsenen“ sind die Darstellung des Projektes und des Erstellungswegs zur S1-Leitlinie, die Erläuterung der klinischen Relevanz der Klassifikation EAES 2015, die wissenschaftliche Begründung der wichtigsten Empfehlungen und die Darstellung stadiengerechter Therapieoptionen.

Dr. med. Mihailo Andric
Berufsverband der Deutschen Chirurgie e.V.