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Adrenal masses in patients with familial adenomatous polyposis

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Diseases of the Colon & Rectum

Abstract

PURPOSE: The aims of this study were 1) to report the characteristics and the clinical outcome of familial adenomatous polyposis (FAP) patients with adrenal masses in the FAP registry at the Cleveland Clinic Foundation and 2) to estimate the prevalence of adrenal masses detected by computed tomography in FAP patients compared with that expected in a normal population. METHODS: A retrospective review was undertaken of the FAP registry database at our institution. Only 738 patients treated at the Cleveland Clinic Foundation were included in the study. A metaanalysis was conducted to determine the relative risk of adrenal incidentaloma in this series of FAP patients and in a general population as reported in the four largest pertinent studies published in the past 15 years. RESULTS: Fifteen patients (11 females) were identified. Two patients had symptoms related to cortisol hypersecretion (arterial hypertension) and underwent surgery. The final pathology was adrenocortical carcinoma and bilateral nodular hyperplasia. Adrenal masses were found incidentally (incidentalomas) in 13 patients: 12 were detected by computed tomography and one during laparotomy for total abdominal colectomy. Only one patient underwent left adrenalectomy for a 5-cm mass. Pathologic report revealed adrenocortical adenoma. Among the 738 patients considered in this study, only 162 underwent abdominal computed tomographic scan, mainly for assessing intra-abdominal desmoid. The prevalence of incidentaloma in our series compared with that reported in the literature is significantly different (7.4 vs.0.6–3.4 percent;P <0.001 (chi-squared test)). DISCUSSION: Although the presence of other extracolonic manifestations represents a selection bias for computed tomographic study in our series, the incidence of incidentalomas in FAP patients seems to be higher than in a general population. However, incidental detection of an adrenal mass in FAP patients has probably a limited clinical relevance, and the management should be the same as that for the normal population.

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References

  1. Bussey HJ. Familial polyposis coli. Family studies, histopathology, differential diagnosis, and results of treatment. Baltimore: Johns Hopkins University Press, 1975:1–75.

    Google Scholar 

  2. Nelson RL, Orsay CP, Pearl RK, Abcarian H. The protean manifestations of familial polyposis coli. Dis Colon Rectum 1988;31:699–703.

    PubMed  Google Scholar 

  3. Gardner EJ. A genetic and clinical study of intestinal polyposis, a predisposing factor for carcinoma of the colon and rectum. Am J Hum Genet 1951;3:167–73.

    PubMed  Google Scholar 

  4. Bulow S. Familial adenomatous polyposis. Ann Med 1989;21:299–307.

    PubMed  Google Scholar 

  5. Jagelman DG. Extracolonic manifestations of familial polyposis coli. Cancer Genet Cytogenet 1987;27:319–25.

    PubMed  Google Scholar 

  6. Kinzler KW, Nilbert MC, Su LK,et al. Identification of FAP locus genes from chromosome 5q21. Science 1991;253:661–5.

    PubMed  Google Scholar 

  7. Nishisho I, Nakamura Y, Miyoshi Y,et al. Mutations of chromosome 5q21 genes in FAP and colorectal cancer patients. Science 1991;253:665–9.

    PubMed  Google Scholar 

  8. Davies DR, Armstrong JG, Thakker N,et al. Severe Gardner syndrome in families with mutations restricted to a specific region of the APC gene. Am J Hum Genet 1995;57:1151–8.

    PubMed  Google Scholar 

  9. Olschwang S, Tiret A, Laurent-Puig P, Muleris M, Parc R, Thomas G. Restriction of ocular fundus lesions to a specific subgroup of APC mutations in adenomatous polyposis coli patients. Cell 1993;75:959–68.

    PubMed  Google Scholar 

  10. Devic A, Bussy MM. Un cas de polypose adenomateuse: generalisee a tout l'intestin. Arch Mal Appar Dig Mal Nutr 1912;6:278–89.

    Google Scholar 

  11. MacDonald JM, Davis WC, Crago HR, Berk AD. Gardner's syndrome and periampullary malignancy. Am J Surg 1967;113:425–30.

    PubMed  Google Scholar 

  12. Coli RD, Moore JP, La Marche PH, DeLuca FG, Thayer WR Jr. Gardner's syndrome; a revisit to a previously described family. Am J Dig Dis 1970;15:551–68.

    PubMed  Google Scholar 

  13. Schneider WR, Cubilla AL, Chaganti RS. Association of endocrine neoplasia with familial multiple colon polyps. Am J Hum Genet 1980;32:128A.

    Google Scholar 

  14. Puig R, Herrera JM, Cassinelli JF. Gardner's syndrome. Prensa Med Argent 1969;56:831–6.

    PubMed  Google Scholar 

  15. Gosserez M, Tréheux A, Hoefell JC, Stricker M, Fays J, Valentin F. Une etiologie rare de tumeurs des maxillaries: le syndrome de Gardner. J Radiol Electrol Med Nucl 1970;51:503–6.

    PubMed  Google Scholar 

  16. Marshall WH, Martin FI, MacKay IR. Gardner's syndrome with adrenal carcinoma. Aust Ann Med 1967;16:242–4.

    PubMed  Google Scholar 

  17. Naylor EW, Gardner EJ. Adrenal adenomas in a patient with Gardner's syndrome. Clin Genet 1981;20:67–73.

    PubMed  Google Scholar 

  18. Painter TA, Jagelman DG. Adrenal adenomas and adrenal carcinomas in association with hereditary adenomatosis of the colon and rectum. Cancer 1985;55:2001–4.

    PubMed  Google Scholar 

  19. Gross MD, Shapiro B. Clinical review 50: clinically silent adrenal masses [review]. J Clin Endocrinol Metab 1993;77:885–8.

    PubMed  Google Scholar 

  20. Gajraj H, Young AE. Adrenal incidentaloma [review]. Br J Surg 1993;80:422–6.

    PubMed  Google Scholar 

  21. Osella G, Terzolo M, Borretta G,et al. Endocrine evaluation of incidentally discovered adrenal masses (incidentalomas). J Clin Endocrinol Metab 1994;79:1532–9.

    PubMed  Google Scholar 

  22. Siren JE, Haapiainen RK, Huikuri KT, Sivula AH. Incidentalomas of the adrenal gland: 36 operated patients and review of literature. World J Surg 1993;17:634–9.

    PubMed  Google Scholar 

  23. Glazer HS, Weyman PJ, Sagel SS, Levitt RG, McClennan BL. Nonfunctioning adrenal masses: incidental discovery on computed tomography. AJR Am J Roentgenol 1982;139:81–5.

    PubMed  Google Scholar 

  24. Abecassis M, McLoughlin MJ, Langer B, Kudlow JE. Serendipitous adrenal masses: prevalence, significance, and management. Am J Surg 1985;149:783–8.

    PubMed  Google Scholar 

  25. Belldegrun A, Hussain S, Seletzer SE, Loughlin KR, Gitten RF, Richie JP. Incidentally discovered mass os the adrenal gland. Surg Gynecol Obstet 1986;163:203–8.

    PubMed  Google Scholar 

  26. Herrera MF, Grant CS, van Heerden JA, Sheedy PF II, Ilstrup DM. Incidentally discovered adrenal tumors: an institutional perspective. Surgery 1991;110:1014–21.

    PubMed  Google Scholar 

  27. Staren ED, Prinz RA. Selection of patients with adrenal incidentalomas for operation [review]. Surg Clin North Am 1995;75:499–509.

    PubMed  Google Scholar 

  28. Ross MS, Aron DC. Hormonal evaluation of the patient with an incidentally discovered adrenal mass. N Engl J Med 1990;323:1402–5.

    Google Scholar 

  29. Bernardino ME, Walther MM, Phillips VM,et al. CT-guided adrenal biopsy: accuracy, safety, and indications. AJR Am J Roentgenol 1985;144:67–9.

    PubMed  Google Scholar 

  30. Yankaskas BC, Staab EV, Craven MB, Blatt PM, Sokhandan M, Carney CN. Delayed complications from fine-needle biopsies of solid masses if the abdomen. Invest Radiol 1986;21:325–8.

    PubMed  Google Scholar 

  31. Katz RL, Shirkoda A. Diagnostic approach to incidental adrenal nodules in the cancer patient: results of a clinical, radiologic, and fine-needle aspiration study. Cancer 1985;55:1995–2000.

    PubMed  Google Scholar 

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Marchesa, P., Fazio, V.W., Church, J.M. et al. Adrenal masses in patients with familial adenomatous polyposis. Dis Colon Rectum 40, 1023–1028 (1997). https://doi.org/10.1007/BF02050923

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