We thank Dr. Yalçin and Dr. Kars for their comments on our recently published article and for their additional data.1, 2 The interval between the onset of one component of Carney syndrome and another is very long in some cases. Carney et al. reported that the mean interval between detection of the first and second tumors was 8.4 years (median, 6 years); the 5 longest intervals were 21, 21, 24, 24, and 26 years in a follow-up study of 79 patients with Carney syndrome.3 Moreover, we also showed that the duration between the onset of multiple chondromatous hamartomas of the lung and time of recurrence is very long; the average was 12.7 years, and the shortest and longest were 5 and 30 years, respectively.1 There is the possibility that the triad might consist of familial and inherited disorders. Carney et al. reported that a sibling of each of two patients with gastric sarcoma and paraganglioma had bilateral carotid body paragangliomas.3 Furthermore, it is noteworthy that gene abnormalities have been reported in some cases of pulmonary hamartoma. Johansson et al. documented that cytogenetic analysis of short term cultures from seven pulmonary hamartomas revealed an abnormal karyotype in six of them, especially recombinations of chromosomal bands 6p21 and 14q24.5 The risk of lung carcinoma has been estimated to be 6.3 times higher in patients with pulmonary hamartoma than in the general population by Van Den Bosch et al. They showed that 11 of 154 patients (7%) also had bronchial carcinoma, which was synchronous in 6 patients and metachronous in 5 patients.6 Again, we must emphasize the necessity of careful and elaborate follow-up for any patients with one or more components of Carney syndrome, keeping in mind that neoplastic lesions may appear in any anatomic site except in the stomach, lung, extraparaganglion, adrenal gland, and esophagus after a remarkably long period. Takuji Kiryu M.D.*, Shimpei Kawaguchi M.D.*, Eisuke Matsui M.D.*, Hiroaki Hoshi M.D.*, Mitsuharu Kokubo M.D. , Kuniyasu Shimokawa M.D. , * Department of Radiology, Gifu University School of Medicine, Gifu, Japan, First Department of Surgery, Gifu University School of Medicine, Gifu, Japan, Department of Laboratory Medicine, Gifu University School of Medicine, Gifu, Japan
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