Trilateral retinoblastoma : Is the location of the intracranial tumor important?

✍ Scribed by Annette C. Moll; Saskia M. Imhof; Antoinette Y. N. Schouten-van Meeteren; Maarten Boers

• Publisher: John Wiley and Sons
• Year: 2000
• Tongue: English
• Weight: 48 KB
• Volume: 88
• Category: Article
• ISSN: 0008-543X
• DOI: 10.1002/(sici)1097-0142(20000215)88:4<965::aid-cncr30>3.0.co;2-n

✦ Synopsis

and Review of the Literature with Special Reference to Carney Syndrome W e read the article of Kiryu et al. with great interest. 1 They re- ported a case of multiple chondromatous hamartoma of the lung and reviewed the literature. However, the case that we published in 1997 was not included among the 15 cases they cited, although the article is in fact a review of the medical literature. 2 Our case had multiple and bilateral lesions that mimicked metastatic carcinoma radiologically. The diagnosis was established with open biopsy and mediastinoscopy. Our research for any accompanying disorder was negative. Two distinct types of hamartoma have been defined, namely, the chondromatous and leiomyomatous types. 3 Multiple hamartomas are usually of the leiomyomatous type. Pulmonary hamartomas may be a rare manifestation of a genetic syndrome, such as Cowden syndrome or Carney triad. 4,5 Cowden disease is multiple hamartoma syndrome characterized by mucocutaneous lesions; multiple benign tumors of internal organs; and increased risk for breast, thyroid, urogenital and digestive tract cancers. 4 The association of gastric leimyoblastoma, pulmonary hamartoma, and extra-adrenal paraganglioma is known as Carney triad. 5 Genetic studies have suggested a tumorigenic relation between hamartomas of the lung and benign tumors of the other organs. 6 No such association was present in our patient, who is now well without any disease progression or presentation of any signs of an accompanying disorder 4 years later.