Right atrial lipoma in patient with Cowden syndrome

Fabrizio Ceresa

Right atrial lipoma in patient with Cowden syndrome

Fabrizio Ceresa

2010, Interactive CardioVascular and Thoracic Surgery

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Primary cardiac tumors are rarest form of cancer and the lipoma represent about 8% of these tumors. Cowden disease is a rare autosomal dominant disorder, associated to a germline mutation of the PTEN gene, characterized by multiple hamartomas and an increased risk of breast, thyroid and endometrial carcinomas. For the first time, we describe a right atrial lipoma in a patient affected by Cowden syndrome. The patient suffered of some episodes of atrial flutter. The echocardiogram showed a cardiac mass, suggestive of lipoma with cardiac magnetic resonance images. Right atrial mass was completely resected and the histological examination confirmed the diagnosis of lipoma. The patient was discharged from hospital without any complications.

ARTICLE IN PRESS doi:10.1510/icvts.2010.245001 Editorial www.icvts.org Case report - Cardiac general Fabrizio Ceresaa,*, Graziella Calarcob, Enrico Franzı̀c, Francesco Patanèa Historical Pages Brief Case Report Communication 䊚 2010 Published by European Association for Cardio-Thoracic Surgery Primary cardiac tumors are very rare and about 75% of them are benign w2x. These tumors are often asymptomatic until they grow enough to cause symptoms. Cardiac lipomas are 50 times less common than myxoma and they grow very slowly. They usually arise from the epicardial fat tissue, growing into the pericardial sac. The most frequent intracardiac location is the right atrium, where they can originate either from the atrial septum or the atrial roof. The symptoms are usually caused by the overgrowth and they are dyspnea, embolism, atrial and ventricular arrhythmias w3 x . Cowden syndrome is a rare autosomal dominant disorder, associated to a germline mutation of the PTEN gene, characterized by multiple hamartomas and an increased Nomenclature *Corresponding author. Tel.: q39 090 399 3309; fax: q39 090 399 3751. E-mail address: [email protected] (F. Ceresa). 3. Discussion Best Evidence A 62-year-old male with an history of hypertension, cerebral transient ischemic attack and affected by Cowden syndrome with gastrointestinal hamartomas, was referred to us for a cardiac mass into the right atrium. The presence of multiple gastrointestinal hamartomas is not needed for the diagnosis of Cowden syndrome, but it is one of the clinical manifestation of this disease. In our case, the diagnosis of Cowden disease was based on Consortium criteria and afterwards it was confirmed by the detection of the mutation in PTEN gene. After the recent onset of an episode of atrial flutter, the patient underwent a transthoracic echocardiogram that showed an atrial mass, arising from the atrial septum and taking up more than the half of the right atrium. The cardiac magnetic resonance images (MRIs) confirmed the relationship between the tumor and the atrial septum that was widely infiltrated. State-of-the-art 2. Case report Follow-up Primary cardiac tumors are very rare, ranging from 0.001 to 0.28% of all tumors, and the lipoma is responsible for about 8% of them w1x. Cowden disease, one of the several hamartoma syndromes, is characterized by hyperplastic lesions and hamartomas distributed on the whole body w2x. We describe the first case of right atrial lipoma as a clinical manifestation of Cowden syndrome. Moreover, the signal behavior of the mass, identical to fatty tissue, was suggestive of a cardiac lipoma, associated with a cystic component (Fig. 1a and b). A median sternotomy was performed. Cardiopulmonary bypass was initiated with distal ascending aorta cannulation and bicaval venous cannulation. The tumor appeared to arise from the atrial septum aneurysm, with a large base of plant. The mass and the part of atrial septum infiltrated were completely removed. We preferred to close the surgical defect with a direct running suture as the size of the septal aneurysm allowed for it. The histological examination confirmed the diagnosis of cardiac lipoma (Fig. 2a, b). The patients was discharged from hospital without any problems after one week. Negative 1. Introduction Proposal for Bail- Keywords: Cardiac lipoma; Cowden syndrome; Cardiac tumors ESCVS Primary cardiac tumors are rarest form of cancer and the lipoma represent about 8% of these tumors. Cowden disease is a rare autosomal dominant disorder, associated to a germline mutation of the PTEN gene, characterized by multiple hamartomas and an increased risk of breast, thyroid and endometrial carcinomas. For the first time, we describe a right atrial lipoma in a patient affected by Cowden syndrome. The patient suffered of some episodes of atrial flutter. The echocardiogram showed a cardiac mass, suggestive of lipoma with cardiac magnetic resonance images. Right atrial mass was completely resected and the histological examination confirmed the diagnosis of lipoma. The patient was discharged from hospital without any complications. 䊚 2010 Published by European Association for Cardio-Thoracic Surgery. All rights reserved. Institutional Abstract out Procedure Report Article Results Topic Paper Downloaded from https://academic.oup.com/icvts/article/11/6/803/832166 by guest on 04 January 2023 Received 4 June 2010; received in revised form 19 August 2010; accepted 24 August 2010 Protocol a Division of Cardiac Surgery, ‘Ospedali Riuniti Papardo-Piemonte’ Hospital, Messina, Italy Division of Patologic Anatomy ‘Ospedali Riuniti Papardo-Piemonte’ Hospital, Messina, Italy c Division of Pediatric Cardiology ‘San Vincenzo Hospital’, Taormina, Italy b Work in Progress Report Right atrial lipoma in patient with Cowden syndrome New Ideas Interactive CardioVascular and Thoracic Surgery 11 (2010) 803–805 ARTICLE IN PRESS 804 F. Ceresa et al. / Interactive CardioVascular and Thoracic Surgery 11 (2010) 803–805 risk of breast, thyroid and endometrial carcinomas. The subcutaneous lipomas and cutaneous hemangiomas often appear w4–6x. We have searched in the literature for the cardiac involvement in Cowden disease without finding any citations. We want to underline certain features of the management of our case. First, the role of cardiac magnetic resonance imaging (MRI) to identify the nature of the cardiac tumor and its relationship with the near structures, allowing to plan the best surgical approach. Second, heart can be involved in Cowden syndrome, that caused the growth of cardiac tumor, such as lipomas, that often remain asymptomatic for long time. Therefore, echocardiography is an important tool to discover cardiac involvement of Cowden disease before growing enough to become symptomatic. We conclude that Cowden syndrome can cause right atrial lipoma and in this case the mainstay of the treatment Fig. 2. (a) Histological examination confirm the diagnosis of cardiac lipoma in Cowden disease. (b) High-power microscopic view. remains the complete surgical resection, even if these tumors can grow again. References w1x Joaquin MR, Arruda MV, Soares MJ. Right atrial lipoma resection and partial reconstruction using bovine pericardium. Rev Bras Circ Cardiovasc 2009;24:239–241. w2x Kraemer N, Balzer JC, Schoth F, Neizel M, Kuehl H, Günther RW, Krombach G. Atrial tumors in cardiac MRI. Rofo 2009;181:1038–1049. w3x Kitzing B. Cardiac lipoma in patient with a history of malignant tumors: a case report. Cases J 2008;1:41. w4x Gustafson F, Zbulk KM, Scacheri C, Enq C. Cowden syndrome. Semin Oncol 2007;34:428–434. w5x Umemura K, Takagi S, Ishigaki Y, Iwabuchi M, Kuroki S, Kinouchi Y, Shimosegawa T. Gastrointestinal polyposis with esophageal polyposis is useful for early diagnosis of Cowden’s disease. World J Gastrointest 2008;14:5755–5759. w6x Hanssen AM, Fryns JP. Cowden syndrome. J Med Genet 1995;32:117– 119. Downloaded from https://academic.oup.com/icvts/article/11/6/803/832166 by guest on 04 January 2023 Fig. 1. A cardiac magnetic resonance images (MRI) show a huge right atrial mass with a cystic components, arising from atrial septum (a and b). The signal behavior is suggestive for cardiac lipoma. ARTICLE IN PRESS F. Ceresa et al. / Interactive CardioVascular and Thoracic Surgery 11 (2010) 803–805 Institutional ESCVS Proposal for BailNegative Follow-up State-of-the-art out Procedure Report Article Results Topic Paper Downloaded from https://academic.oup.com/icvts/article/11/6/803/832166 by guest on 04 January 2023 Protocol w1x Ceresa F, Calarco G, Franzi E, Patane F. Right atrial lipoma in patient with Cowden’s syndrome. Interact CardioVasc Thorac Surg 2010;11:803– 805. w2x Vaughan CJ, Weremowicz S, Goldstein MM, Casey M, Hart M, Hahn RT, Devereux RB, Girardi L, Schoen FJ, Fletcher JA, Morton CC, Basson CT. A t(2;19) (p13;p13.2) in a giant invasive cardiac lipoma from a patient with multiple lipomatosis. Genes Chromosomes Cancer 2000;28:133– 137. Work in Progress Report References New Ideas Authors: Dimos Karangelis, Cardiovascular and Thoracic Surgery, Hospital of Larissa, Georgiou Rika 4, 41335 Larissa, Greece; Georgios Tagarakis, Athanasios Hevas, Nikolaos Tsilimingas doi:10.1510/icvts.2010.245001A After reading the interesting case report on right atrial lipoma in a patient with Cowden syndrome w1x we would like to make a few remarks. Although echocardiography is considered to be the most important diagnostic technique in such cases, magnetic resonance imaging can provide detailed characteristics and best visualization of the tumours. We are also aware that plain chest radiography findings are sometimes suggestive of primary cardiac tumours but not pathognomonic. Where there any suggestive findings of the tumour on a plain chest X-ray film, for example, a notable calcification or a widened mediastinum? We also assume that the authors performed a complete excision of the tumour reaching negative margins. In regard to the created defect, do they routinely use a direct running suture for the repair or do they consider a patch also? Finally, unlike cutaneous lipomas, cardiac lipomas present a rather confused genetic and molecular background. Vaughan et al. w2x reported an unusual translocation between chromosomes 2 and 19 in a patient with a large invasive cardiac lipoma. Besides the mutation in PTEN gene which was detected by the authors, were any cytogenetic studies indicative of the aforementioned perturbation? Editorial eComment: Benign primary cardiac tumours and Cowden’s syndrome 805 Best Evidence Nomenclature Historical Pages Brief Case Report Communication

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