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2004, Number 1

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Cir Gen 2004; 26 (1)

Glucagonoma: Report of one patient

Hermosillo SJM, Marino D, Ramírez CV, Zermeño RJJ, Carranza RJM, Aguilera SJ

Language: Spanish
References: 38
Page: 49-54
PDF size: 593.95 Kb.


ABSTRACT

Objective: To present the case of a patient with glucagonoma.
Design: Report of a case.
Setting: Third level health care hospital.
Case description: Man of 49 years of age, comes to the hospital due to dermal lesions of 18 months evolution in the face and gluteus that extend to the feet, legs, and thighs, constituted by vesicles and ampullae. Lesions expanded, curing centrally after forming a central crust, while the borders remained well defined and rounded. Besides, he refers weight loss and glossitis. The biopsy reported: necrolytic migratory erythema. The clinical and histopathological findings suggest glucagonoma syndrome. Axial computed tomography reveals a mass in the body and tail of the pancreas, without metastasis. Preoperative management included parenteral nutrition and octreotide, remitting the dermal lesions in more than 80%. He is subjected to surgery, finding a neoplasm in the pancreas of approximately 7 x 5 x 3 cm, involving the body and tail, of rubber-like consistency and nodular aspect surface, without glandular affection or distant metastases, with thrombosis of the splenic vein and splenomegaly. Distal pancreatectomy and splenectomy are performed. The anatomopathological study of the pancreas reveals macroscopically a congestive surface with fatty tissue adherences and the section reveals a yellowish pancreatic parenchyma with characteristic trabecular surface, occupying only 20% of it, since the remainder 80% is occupied by the tumor, of brownish-grayish color. The histopathological and immunohistochemical studies reported: glucagonoma. During the postoperative period, the patient presents deep venous thrombosis, which is medically treated yielding a good response. The patient is discharged being in good condition and without dermal lesions.
Conclusion: During a following period of two years the patient it’s asymptomatic.


REFERENCES

  1. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine (Baltimore) 1996; 75: 53-63.

  2. Boden G. Glucagonomas and insulinomas. Gastroenterol Clin North Am 1989; 18: 831-45.

  3. Becker SW, Kahn D, Rothman S. Cutaneous manifestations of internal malignant tumors. Arch Dermatol Syphilol 1942; 45: 1069-80.

  4. McGavran MH, Unger RH, Recant L, Polk HC, Kilo C, Levin ME. A glucagons-secreting alpha-cell carcinoma of the pancreas. N Engl J Med 1966; 2741: 408-13.

  5. Wilkinson DS. Necrolytic migratory erythema with carcinoma of the pancreas. Trans St Johns Hosp Dermatol Soc 1973; 59: 244-50.

  6. Zinner MJ. Endocrine tumors of pancreas. In: Beauchamp RD, Thompson JC. Maingot’s Abdominal Operations. 10th Edition. Appleton and Lange. A Simon and Schuster Company, Stanford, Connecticut. 1997, 1849-50.

  7. Roggli VL, Judge DM, McGavran MH. Duodenal glucagonoma: a case report. Hum Pathol 1979; 10: 350-3.

  8. Edney JA, Hofmann S, Thompson JS, Kessinger A. Glucagonoma syndrome is an underdiagnosed clinical entity. Am J Surg 1990; 160: 625-8; discussion 628-9.

  9. Gleeson MH, Bloom SR, Polak JM, Henry K, Dowling RH. Endocrine tumour in kidney affecting small bowel structure, motility, and absorptive function. Gut 1971; 12: 773-82.

  10. Staccpoole PW. The glucagonoma syndrome. Clinical features, diagnosis and treatment. Endocrinol Rev 1981; 2: 347-61.

  11. Haga Y, Yanagi H, Urata J, Inada M, Shimada S, Nitahata N, et al. Early detection of pancreatic glucagonoma. Am J Gastroenterol 1995; 90: 2216-23

  12. Jaffe BM. Surgery for gut hormone-producing tumors. Am J Med 1987; 82(5B): 68-76.

  13. Leichter SB. Clinical and metabolic aspects of glucagonoma. Medicine (Baltimore) 1980; 59: 100-13.

  14. Higgins GA, Recant L, Fischman AB. The glucagonoma syndrome: surgically curable diabetes. Am J Surg 1979; 137: 142-8.

  15. Lokich J, Anderson N, Rossini A, Hadley W, Federman M, Legg M. Pancreatic alpha cell tumors: case report and review of the literature. Cancer 1980; 45: 2675-83.

  16. Kaplan EL, Michelassi F. Endocrine tumors of the pancreas and their clinical syndromes. Surg Annu 1986; 18: 181-223.

  17. Holmes A, Kilpatrick C, Proietto J, Green MD. Reversal of a neurologic paraneoplastic syndrome with octreotide (Sandostatin) in a patient with glucagonoma. Am J Med 1991; 91: 434-6.

  18. Klein S, Jahoor F, Baba H, Townsend CM Jr, Shepherd M, Wolfe RR. In vivo assessment of the metabolic alterations in glucagonoma syndrome. Metabolism 1992; 41: 1171-5.

  19. Wynick D, Hammond PJ, Bloom SR. The glucagonoma syndrome. Clin Dermatol 1993; 11: 93-7.

  20. Lax E, Leibovici V, Fields SI, Gordon RL. Neglected radiologic signs of the glucagonoma syndrome. Diagn Imaging Clin Med 1986; 55: 321-6.

  21. Montenegro-Rodas F, Samaan NA. Glucagonoma tumors and syndrome. Curr Probl Cancer 1981; 6: 1-54.

  22. van Hecke E, Geerts ML, Kint A, Debersaques J, Naeyaert JM. Glucagonoma syndrome. Curr Probl Dermatol 1991; 20: 24-33.

  23. Stark DD, Moss AA, Goldberg HI, Deveney CW. CT of pancreatic islet cell tumors. Radiology 1984; 150: 491-4.

  24. Vandersteen PR, Scheithauer BW. Glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultra-structural study. J Am Acad Dermatol 1985; 12: 1032-9.

  25. Leibovici V, Granot E, Eid A, Goldin E, Okon E. The glucagonoma syndrome-report of a case without overt diabetes. J Dermatol 1987; 14: 491-6

  26. van der Loos TJ, Lambrecht ER, Lambers JC. Successful treatment of glucagonoma-related necrolytic migratory erythema with decarbazine. J Am Acad Dermatol 1987; 16(2 Pt2): 468-72.

  27. Moertel CG, Lefkopoulo M, Lipsitz S, Hahn RG, Klaassen D. Streptozocin-doxorubicin, streptozocin-fluorouracil, or chlorozotocin in the treatment of advanced islet-cell carcinoma. N Engl J Med 1992; 326: 519-23.

  28. Modlin IM, Lewis JJ, Ahlman H, Bilchik JA, Kumar RR. Management of unresectable malignant endocrine tumors of the pancreas. Surg Gynecol Obstet 1993; 176: 507-18.

  29. Shepherd ME, Raimer SS, Tyring SK, Smith EB. Treatment of necrolytic migratory erythema in glucagonoma syndrome. J Am Acad Dermatol 1991; 25(5 Pt2): 925-8.

  30. Sohier J, Jeanmougin M, Lombrail P, Passa P. Rapid improvement of skin lesions in glucagonomas with intravenous somatostatin infusion. Lancet 1980; 1: 40.

  31. Schmid R, Allescher HD, Schepp W, Holscher A, Siewert R, Sehusdziarra V, et al. Effect of somatostatin on skin lesions and concentrations of plasma amino acids in a patient with gl=Z

  32. Siller GM, Strutton GM, Moore GA, Kanowski DM, Nedwich JA. Glucagonoma syndrome with increased lactate dehydrogenase isoenzymes: octreotide treatment. Australas J Dermatol 1994; 35: 11-4.

  33. Boden G, Ryan IG, Eisenschmid BL, Shelmett JJ, Owen OE. Treatment of inoperable glucagonoma with the long-acting somatostatin analogue SMS 201-995. N Engl J Med 1986; 314: 1686-9.

  34. Kvols LK, Buck M, Moertel CG, Schutt AJ, Rubin J, O’Connell MJ, et al. Treatment of metastatic islet cell carcinoma with a somatostatin analogue (SMS 201-995). Ann Intern Med 1987; 107: 162-8.

  35. Arnold R, Frank M, Kajdan U. Management of gastroenteropancreatic endocrine tumors: the place of somatostatin analogues. Digestion 1994; 55 Suppl 3: 107-13

  36. Wynick D, Anderson JV, Williams SJ, Bloom SR. Resistance of metastatic pancreatic endocrine tumours after long-term treatment with the somatostatin analogue octreotide (SMS 2001-995). Clin Endocrinol (Oxf) 1989; 30: 385-8.

  37. Burton JL. Zinc and essential fatty acid therapy for necrolytic migratory erythema. Arch Dermatol 1993; 129: 246.

  38. Blackford S, Wright S, Roberts DL. Necrolytic migratory erythema without glucagonoma: the role of dietary essential fatty acids. Br J Dermatol 1991; 125: 460-2.




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