No abstract available.
Carney Complex* ; Humans ; Neurilemmoma*

No abstract available.
Carney Complex* ; Hyperplasia* ; Myxoma* ; Nevus, Blue* ; Testis*

No abstract available.
Asian Continental Ancestry Group ; Carney Complex* ; Female ; Humans ; Nevus, Blue*

Although cardiac myxoma is the most commonly encountered benign cardiac tumor in cardiac surgery practice, recurrent cardiac myxoma is very rare, is most commonly related to the Carney complex, and usually requires multiple cardiac operations with specific requirements in terms of perioperative management. In this report, we describe a patient who experienced the fourth recurrence of cardiac myxoma and review the diagnostic criteria of the Carney complex. This is the first report of such a case in Korea.
Carney Complex* ; Heart Neoplasms ; Humans ; Korea ; Myxoma* ; Recurrence* ; Thoracic Surgery

Carney Complex ; Humans ; Jugular Foramina ; Myxoma ; Neoplasm Recurrence, Local

Carney complex is an autosomal dominant disorder that occurs due to a mutation in PRKAR1A, which encodes protein kinase A. The clinical features are multiple endocrine gland neoplasms, skin tumors, pigmented skin lesions, myxomas, and schwannomas. In Carney complex, the cardiac myxoma is a common co-morbidity. It occurs in multiples, during young age, regardless of gender and cardiac chamber and is known to recur frequently. Therefore there are high risks of adhesion and massive bleeding due to repeated surgeries. Such surgical risks account for over 50% of disease-specific mortality of Carney complex patients. Here, we present anesthetic experiences of myxoma removal surgery in two patients with Carney complex.
Anesthesia ; Carney Complex ; Cyclic AMP-Dependent Protein Kinases ; Endocrine Gland Neoplasms ; Hemorrhage ; Humans ; Myxoma ; Neurilemmoma ; Skin

Ten percent of all myxomas are the familial form. Familial myxomas appear to have autosomal dominant transmission. We experienced two siblings with familial myxomas. A left atrial myxoma was surgically removed in a 21-year-old woman. Six years later, other myxomas were found in the right atrium and the left atrium and these were also surgically removed. Right ventricular and right atrial myxomas were surgically excised in her brother. The two siblings were found to have frame-shift mutations in the PRKAR1A gene (c.537delA; p.Gly180GlufsX26), which is the causative gene for Carney complex. Obtaining the genetic diagnosis makes it possible to prepare more effective therapeutic strategies for these patients and the gene carriers. Complete excision, ruling out multicentricity and proper postoperative follow up are all necessary to avoid recurrence of myxoma.
Carney Complex ; Female ; Follow-Up Studies ; Heart Atria ; Humans ; Myxoma ; Recurrence ; Siblings ; Young Adult

Carney complex (CNC) is a rare disease characterized by myxoma, spotty skin pigmentation, and multiple neuroendo crine tumors. Here, we present a case of CNC, diagnosed 11 years after resection of recurrent cardiac myxomas. We had previously reported a 37-year-old woman in 1984 as biatrial myxomas and in 1994 as recurrent left atrial and ventricular myxomas, and at those times, she did not have any other myxomas and skin lesions. On her present admission, she had skin pigmentation and myxomatosis of breast, nostril and forearm. Upon the examination of her relatives, no characteristics of CNC were found on them. She was diagnosed as a sporadic form of CNC. If cardiac myxomas tend to be multiple and recurrent, we should consider the possibility of CNC, even other characteristics of CNC except cardiac myxomas are not present at diagnosis.
Adult ; Breast ; Carney Complex* ; Diagnosis ; Female ; Forearm ; Humans ; Myxoma* ; Rare Diseases ; Skin ; Skin Pigmentation

Epithelioid blue nevus is a rare variant of blue nevus that was first described in patients with Carney complex, a familial lentiginosis and multiorgan neoplasm (spotty cutaneous pigmentation, cutaneous/cardiac/mammary myxomas, endocrine overactivity, and psammomatous melanotic schwannomas). Epithelioid blue nevus is an intradermal melanocytic nevus composed of heavily pigmented globular and polygonal cells and lightly pigmented spindle and dendritic cells. Recently, cases of epithelioid blue nevi have been reported in patients without Carney complex. Herein, we describe a child who developed epithelioid blue nevus on her right foot without Carney complex symptoms and review the relevant literature.
Carney Complex* ; Child ; Dendritic Cells ; Foot ; Humans ; Lentigo ; Myxoma ; Nevus, Blue* ; Nevus, Pigmented ; Pigmentation

A solitary cutaneous myxoma, referred to as superficial angiomyxomas, is relatively uncommon benign myxoid soft tissue tumors. The lesion usually present on the head, neck, and trunk of adults as a slowly growing asymptomatic nodule. Herein, we describe a case of a pedunculated solitary cutaneous myxoma arising on the vertex of a 48-year-old man, but with no evidence of Carney syndrome. Histologically, the tumor was composed of vascular, mucinous matrix with a network of fine collagen and reticulin fibers, containing stellate and spindle-shaped fibroblasts. There was no evidence of malignancy
Adult ; Carney Complex ; Collagen ; Fibroblasts ; Head ; Humans ; Middle Aged ; Mucins ; Myxoma* ; Neck ; Reticulin