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Evaluating Causes of Death in Familial Adenomatous Polyposis

  • Original Article
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Journal of Gastrointestinal Surgery Aims and scope

Abstract

Background

Familial adenomatous polyposis is a genetic syndrome associated with an increased risk of colorectal cancer (CRC) and different extracolonic manifestations.

Goals

The goal of this study is to evaluate the frequency of death causes.

Material and Methods

Charts from 97 patients treated from 1977 to 2008 were reviewed. Retrieved data and family information allowed us to classify causes of death in those related to CCR to other malignancies or other causes.

Results

There were analyzed data from 46 men (47.4%) and 51 women (52.6%) with an average age of 35.1 years (14 to 82). At diagnosis, 57 patients (58.7%) already had CRC-associated polyposis. There were performed 93 colectomies, one internal diversion, and one partial resection. Two patients were not operated on. Results from 19 deceased patients (19.5%) were analyzed. CRC, other tumors (desmoid tumors, lymphoma, and gastric cancer), and other causes (complication of duodenal cancer surgery, complication after ileorectal anastomosis (IRA), and coronary disease) were responsible for 12 (63.1%), four (21.1%), and three (15.8%) of all deaths, respectively. Death from CRC occurred in the context of either systemic, rectal, or pouch recurrence. Desmoid disease was the second cause of death (10.5% of all causes), leading to a fatal outcome 22% of all patients who developed DT during the study period. Upper digestive carcinomas were responsible for other two death cases.

Conclusions

(1) CRC is still the most prevalent cause of death; (2) even after curative resections, CRC can cause death through rectal or pouch malignization; (3) long-term survival was also strongly related to the development of extracolonic neoplasia, especially desmoid tumors and gastroduodenal carcinoma; (4) our results raise the need for local improvement in familiar screening and help us to define follow-up strategies and patient-information standards.

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Authors and Affiliations

  1. Colorectal Surgery Unit, Gastroenterology Department, Hospital das Clínicas, University of São Paulo Medical School, São Paulo, Brazil

    Fábio Guilherme C. M. de Campos, Rodrigo Oliva Perez, Antônio Rocco Imperiale, Víctor Edmond Seid, Sérgio Carlos Nahas & Ivan Cecconello

  2. Rua Padre João Manoel, 222 Cj 120, São Paulo, 01411-001, Brazil

    Fábio Guilherme C. M. de Campos

Authors
  1. Fábio Guilherme C. M. de Campos
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  2. Rodrigo Oliva Perez
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  3. Antônio Rocco Imperiale
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  4. Víctor Edmond Seid
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  5. Sérgio Carlos Nahas
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  6. Ivan Cecconello
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Corresponding author

Correspondence to Fábio Guilherme C. M. de Campos.

Additional information

Campos FG, Perez RO, Imperiale AR, Seid VE, Nahas SC, and Cecconello I. Evaluating causes of death in Familial Adenomatous Polyposis.

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de Campos, F.G.C.M., Perez, R.O., Imperiale, A.R. et al. Evaluating Causes of Death in Familial Adenomatous Polyposis. J Gastrointest Surg 14, 1943–1949 (2010). https://doi.org/10.1007/s11605-010-1288-6

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  • DOI: https://doi.org/10.1007/s11605-010-1288-6

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