JOURNAL of
ONCOLOGICAL
SCIENCES

CASE REPORT

Coexistence of Atrial Myxoma and Gastrointestinal Stromal Tumor
Received Date : 16 Oct 2019
Accepted Date : 19 Apr 2020
Available Online : 24 Jun 2020
Doi: 10.37047/jos.2019-71885 - Article's Language: EN
J Oncol Sci.2020;6(2):119-22
This is an open access article under the CC BY-NC-ND license
ABSTRACT
Gastrointestinal stromal tumors (GISTs) are primarily mesenchymal tumors with specific histological features found in the gastrointestinal tract and the abdomen. They are identified and diagnosed by the expression of the protooncogene protein called CD117 detected using immunohistochemistry. Atrial myxoma is the most common benign primary tumor of the heart. Carney triad is considered to be a specific type of multiple endocrine neoplasia. Three classically related tumors are the GIST, pulmonary chondroma, and extra-adrenal paraganglioma. Carney syndrome is an autosomal dominant syndrome with multiple components, characterized by myxomatous neoplasms (cardiac, endocrine, cutaneous, and neural), pigmented lesions of the skin and mucosae, and multiple endocrine tumors.The patient in this study was a 75-year-old man with no known illness. He had a tumor in the stomach and was operated. The pathology was reported as low-grade GIST; therefore, adjuvant imatinib treatment was not recommended. Further, owing to the shortness of breath in the third month of follow-up, echocardiography was requested. Left atrial mass was detected and operated. His pathology was compatible with atrial myxoma. After both operations, the patient is still being followed up in remission in our clinic.Only one case has been reported worldwide documenting the coexistence of atrial myxoma and GIST, and none has been seen in Turkey. Thus, to the best of our knowledge, this is the second case in the literature and first report from Turkey.
REFERENCES
  1. Kindblom LG, Remotti HE, Aldenborg F, Meis-Kindblom JM. Gastrointestinal pacemaker cell tumour (GIPACT): gastrointestinal stromal tumours show phenotypic characteristics of the interstitial cells of Cajal. Am J Pathol. 1998;152(5):1259-1269. [PubMed] 
  2. de Silva CM, Reid R. Gastrointestinal stromal tumors (GIST): C-kit mutations, CD117 expression, differential diagnosis and targeted cancer therapy with Imatinib. Pathol Oncol Res. 2003;9(1):13-9. [Crossref]  [PubMed] 
  3. Burke A, Virmani R. Classification and Incidence of Cardiac Tumors. Tumors of the heart and great vessels. 1st ed. Atlas of tumor pathology. Washington DC: Armed Forces Institute of Pathology; 1996:1-11.
  4. Engin G, Asoglu O, Kapran Y, Mert G. A gastrointestinal stromal tumor with mesenteric and retroperitoneal invasion. World J Surg Oncol. October 2007;5:121. [Crossref]  [PubMed]  [PMC] 
  5. Gold JS, Dematteo RP. Combined surgical and molecular therapy: the gastrointestinal stromal tumor model. Ann Surg. 2006;244(2):176-184. [Crossref]  [PubMed]  [PMC] 
  6. Bölükbaşı H, Nazlı O, Tansug T, et al. Gastrointestinal stromal tumors (GISTs): analysis of 20 cases. Hepatogastroenterology. 2006;53(69):385-388. [PubMed] 
  7. Gonzalez-Juanatey C, Regueiro-Abel M, Lopez-Agreda H, Pe-a-Martínez F, Gonzalez-Gay MA. Giant left atrial myxoma mimicking severe mitral valve stenosis Int J Cardiol. 2008;127(3):e110-112. [Crossref]  [PubMed] 
  8. Reynen K. Cardiac myxomas. N Engl J Med. 1995;333(24):1610-1617. [Crossref]  [PubMed] 
  9. Panos A, Kalangos A, Sztajzel J. Left atrial myxoma presenting with myocardial infarction. Case report and review of the literature. Int J Cardiol. 1997;62(1):73-75. [Crossref]  [PubMed] 
  10. Vogt PR, Jenni R, Turina MI. Infected left atrial myxoma with concomitant mitral valve endocarditis. Eur J Cardiothorac Surg. 1996;10(1):71-73. [Crossref]  [PubMed] 
  11. Carney JA. The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review. Medicine (Baltimore). 1983;62(3):159-169. [Crossref]  [PubMed] 
  12. Carney JA, Gordon H, Carpenter PC, Shenoy BV, Go VL. The complex of myxomas, spotty pigmentation, and endocrine overactivity. Medicine (Baltimore). 1985;64(4):270-283. [Crossref]  [PubMed] 
  13. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab. 2001;86(9):4041-4046. [Crossref]  [PubMed] 
  14. Almeida MQ, Stratakis CA. Carney complex and other conditions associated with micronodular adrenal hyperplasias. Best Pract Res Clin Endocrinol Metab. 2010;24(6):907-914. [Crossref]  [PubMed]  [PMC] 
  15. Joensuu H, Eriksson M, Hall KS, et al. One vs three years of adjuvant imatinib for operable gastrointestinal stromal tumor: a randomized trial. JAMA. 2012;307(12):1265-1272. [Crossref]  [PubMed] 
  16. Kuon E, Kreplin M, Weiss W, Dahm JB. The challenge presented by right atrial myxoma. Herz. 2004;29(7):702-709. [Crossref]  [PubMed] 
  17. Pinede L, Duhaut P, Loire R. Clinical presentation of left atrial cardiac myxoma. A series of 112 consecutive cases. Medicine (Baltimore). 2001;80(3):159-172. [Crossref]  [PubMed] 
  18. İpek G, Erentug V, Bozbuga N, et al: Surgical management of cardiac myxoma. J Card Surg. 2005;20(3):300-304. [Crossref]  [PubMed] 
  19. Aspres N, Bleasel NR, Stapleton KM. Genetic testing of the family with a Carney-complex member leads to successful early removal of an asymptomatic atrial myxoma in the mother of the patient. Australas J Dermatol. 2003;44(2):121-122. [Crossref]  [PubMed] 
  20. Q Shafiq, I Mohamed. Concomitant occurrence of the left atrial myxoma and gastrointestinal stromal tumor (GIST): a case report. The Internet Journal of Cardiology. 2008;7(2):1-4. [Crossref]