Carney Complex with Biatrial Cardiac Myxoma

Abstract

Cardiac myxomas make up approximately 50% of all benign cardiac tumors and represented 86% of all surgically treated cardiac tumors. Most of them originated from the left atrium, in some cases from both of atria. We report a case of male patient with biatrial myxomas and other extra-cardiac involvement: hypophyseal adenoma, enlargement of thyroid gland, tubular adenoma polyp of colon and bilateral large cell calcifying Sertoli cell tumor (LCCSCT) of testis. These findings led to the diagnosis of Carney's complex, which is a syndrome with multiple neoplasias, cardiac myxomas, lentigines, and endocrine abnormalities. A genetic test confirm this diagnosis. was revealed and resected the left atrial myxoma. This tumor was of identic consistence, 6 × 5 × 3 cm, attached to the free wall of the atrium by a small stalk 3 × 1 cm. The diagnosis of bilateral atrial myxomas was confirmed by histologic studies (Fig. 2). We assumed association between the endocrine abnormalities-hypophyseal adenoma, struma nodosa and benign tumors (bilateral LCCSCT of testis and biatrial tumor), and therefore we signed this patient with Carney complex. Carney complex is an autosomal dominant condition most commonly caused by mutations in the PRKAR1A gene on chromosome 17q23-q24. In our patient mutation c.491-492delTG/p.V164Dfs*5 in exon 4 of PRKAR1A gene was identified and the diagnosis of Carney complex was confirmed (Fig. 3). The patient was discharged in good condition. Keywords DiscussionAlthough primary tumors of the heart are rare, the myxoma is the most frequent benign primary heart tumor as it accounts for 0.3% of open-heart surgery. 4) Cardiac myxomas usually appear as a sporadic isolated condition in the left atrium of middle-aged women with no other coincidental pathology. A small percentage of primary cardiac tumors have familial penetrance. Recurrence is more frequent in patients with the family history of myxoma, and familial myxomas frequently appear et early ages, with atypical and multicentric location. 5) In 1985, Carney and others described in young people a special complex group of cardiac myxomas associated to a distinctive complex pathology, giving identity to the There is a marked familial trend (52%), a high incidence of recurrence (20%). 6) The most frequent extra-cardiac involvements are pigmented skin lesions, cutaneous myxomas, adrenal cortical disease, myxoid mammary fibroadenoma, pituitary adenoma, melanotic schwannomas, thyroid disease and male patients with testes tumors. The diagnosis is made when two or more of these criteria are present. The myxom in CNC is usually in left atrium, biatrial myxomas are rare. The four chambers of the heart should be examined at surgery for typical myxoma locations, right atriotomy and combined superior-transseptal approach improve exposure of the cavities. In our patient, the tumor size and attachment were identified by echocardiogram, and a safe and efficient transseptal incision was achieved. Myxoma is known to recur in 30%-70% of patients with familial myxomas. Close follo...

Cardiac myxoma misdiagnosed as infective endocarditis: a case of Carney complex

“…While vegetation in IE is known to cause systemic embolism, cardiac myxoma can also be an important cause of stroke. CM is responsible for 0.5% of all strokes and 10–30% of patients with CM were reported to develop cerebral embolic infarction [ 2 ]. Although infected CM is a rare condition, it should also be considered in the differential diagnosis of fever and embolism [ 3 ].…”

Dueling Myxomas: A Rare Finding

“…Primary cardiac tumors are rare, with the most common being myxomas, accounting for almost half 3, 4, 5, 8, 9. Myxomas are typically pedunculated, gelatinous tumors of stromal cells that originate from multipotent mesenchyme capable of both neural and endothelial differentiation 4, 6.…”

“…Carney complex, or Carney syndrome, is a rare autosomal-dominant endocrine disorder characterized by multiple benign tumors, such as myxomas, as well as changes in skin pigmentation (lentigines and blue nevi) 5, 8. Patients with Carney complex are usually young men and have at least two criteria to make the diagnosis, including multicentric tumors and spotty skin pigmentation Our patient did not have any evidence of pigmented skin lesions, nor did he have evidence of any other endocrine tumors to suggest this diagnosis.…”

“…Primary cardiac tumors are rare and although most often benign are associated with high embolic potential 3, 4. Myxomas are the most common primary tumors of the heart, most commonly located in the left atrium 3, 4, 5, 6, 7, 8. The discovery of multiple myxomas is a rare finding.…”

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“…The most common symptom in our review was dyspnea or exertional dyspnea, [ 6 – 10 ] followed by syncope [ 3 , 11 ] and palpitation. [ 12 , 13 ] Two patients complained of cough [ 10 , 14 ] and 1 complained of chest pain. [ 15 ]…”