Neurofibromatosis type 1, hyperparathyroidism, and osteosarcoma: interplay? |
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Authors: | Udi Cinamon Ilana Avinoach Moshe Harell |
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Institution: | Deaprtment of Otolaryngology, Head and Neck Surgery, The Edith Wolfson Medical Center, Holon, Israel. udicin@hotmail.co.il |
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Abstract: | Neurofibromatosis type 1 (NF 1) is a syndrome with a predisposition for benign and malignant tumor development. Of the malignant neoplasms, osteogenic sarcomas are rare but have been described. There are some reports of patients with neurofibromatosis type 1 with a parathyroid adenoma and hyperparathyroidism. Also, there are studies that imply that the parathyroid hormone plays a role in the regulation and modulation of oseogenic sarcomas in vitro. We report about a 50-year-old female suffering from neurofibromatosis type 1, with a 3-year documented history of untreated hyperparathyroidism and a parathyroid adenoma. The patient developed a mandibular osteogenic sarcoma. To our knowledge, this is the first reported case occurring in the mandible. The unusual tumor site for a patient with neurofibromatosis type 1, the conjugation with hyperparathyroidism and the rapid growth of an osteogenic sarcoma are intriguing. |
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