Prophylactic total gastrectomy in the management of hereditary tumor syndromes.
OBJECTIVE
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.
Department of General, Visceral, Thoracic and Vascular Surgery, University Hospital of Bonn, Bonn, Germany. dimitrios.pantelis@ukb.uni-bonn.de.|Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany. dimitrios.pantelis@ukb.uni-bonn.de.
Dimitrios
D
Pantelis
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany.
Robert
R
Hüneburg
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Institute of Human Genetics, University Hospital Bonn, Bonn, Germany.
Ronja
R
Adam
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Institute of Human Genetics, University Hospital Bonn, Bonn, Germany.
Stefanie
S
Holzapfel
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Institute of Pathology, University Hospital Bonn, Bonn, Germany.
Heidrun
H
Gevensleben
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany.
Jacob
J
Nattermann
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Department of Internal Medicine I, University Hospital Bonn, Bonn, Germany.
Christian P
CP
Strassburg
Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.|Institute of Human Genetics, University Hospital Bonn, Bonn, Germany.
Stefan
S
Aretz
Department of General, Visceral, Thoracic and Vascular Surgery, University Hospital of Bonn, Bonn, Germany.|Center for Hereditary Tumor Syndromes, University Hospital Bonn, Bonn, Germany.
Jörg C
JC
Kalff
D000328:Adult; D005260:Female; D005743:Gastrectomy; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D012307:Risk Factors; D013274:Stomach Neoplasms / Q000235:genetics / Q000473:pathology / Q000517:prevention & control* / Q000601:surgery*; D055815:Young Adult
CDH1; Hereditary diffuse gastric cancer; Juvenile polyposis syndrome; Prophylactic total gastrectomy; SMAD4
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