Cinamon Udi, Avinoach Ilana, Harell Moshe
Deaprtment of Otolaryngology, Head and Neck Surgery, The Edith Wolfson Medical Center, Holon, Israel.
Eur Arch Otorhinolaryngol. 2002 Nov;259(10):540-2. doi: 10.1007/s00405-002-0497-3. Epub 2002 Jul 9.
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.
1型神经纤维瘤病（NF 1）是一种易发生良性和恶性肿瘤的综合征。在恶性肿瘤中，骨肉瘤罕见但已有报道。有一些关于1型神经纤维瘤病患者合并甲状旁腺腺瘤和甲状旁腺功能亢进的报告。此外，有研究表明甲状旁腺激素在体外对骨肉瘤的调节中起作用。我们报告了一名50岁患有1型神经纤维瘤病的女性，有3年未经治疗的甲状旁腺功能亢进和甲状旁腺腺瘤病史。该患者发生了下颌骨肉瘤。据我们所知，这是首例报道发生在下颌骨的病例。1型神经纤维瘤病患者不寻常的肿瘤部位、与甲状旁腺功能亢进的关联以及骨肉瘤的快速生长都很引人关注。
