A patient with Loeys-Dietz syndrome treated with chemoradiotherapy for an oropharyngeal carcinoma.

We present the first published case of a patient with Loeys-Dietz syndrome (LDS) who was treated with radical chemoradiotherapy for an oropharyngeal carcinoma. In view of this newly recognised connective tissue disease, the uncertainty of severe toxicity from chemoradiotherapy to treat a potentially curative cancer posed a management challenge. The patient was treated with chemoradiotherapy and remains well with no evidence of recurrence at 3 years. Furthermore, we have observed minimal late effects secondary to chemoradiotherapy at 3 years following the completion of treatment suggesting that the underlying pathogenesis of LDS may provide an interesting human model to further elucidate the complex interactions of transforming growth factor ß1 (TGF-ß1) and tissue fibrosis secondary to chemoradiotherapy. A review of LDS as well as the association of TGF-ß1 expression and tissue fibrosis is presented.

Managing a locally advanced malignant thymoma complicated by nephrotic syndrome: a case report.

INTRODUCTION: The management of locally advanced inoperable malignant thymoma is difficult as there are no large randomized clinical trial data to guide treatment. However various case series have shown that malignant thymoma is often a chemosensitive disease. Cisplatin-based chemotherapy has been the gold-standard in the management of these patients. However when thymic cancers are complicated by paraneoplastic syndromes that damage kidney and neurological function, cisplatin use is often contraindicated. CASE PRESENTATION: We report a case of a 37 year old man with locally advanced malignant thymoma complicated by significant nephrotic syndrome and renal impairment. He responded to a novel combination of carboplatin, epirubicin and cyclophosphamide chemotherapy used as first line therapy. CONCLUSION: The treatment with chemotherapy of locally advanced malignant thymoma complicated by nephrotic syndrome and renal impairment is difficult due to the increase of toxicity. In this case, a novel chemotherapy combination with lesser toxicity was used successfully. In addition this chemotherapy combination did not impede the later use of conventional cisplatin-based chemotherapy. Therefore we suggest a course of carboplatin-based chemotherapy for locally advanced malignant thymoma in patients who are unsuitable for cisplatin.