Neurofibromatosis type 1, hyperparathyroidism, and osteosarcoma: interplay?
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.
Cinamon
Udi
U
Deaprtment of Otolaryngology, Head and Neck Surgery, The Edith Wolfson Medical Center, Holon, Israel. udicin@hotmail.co.il
Avinoach
Ilana
I
Harell
Moshe
M
D001859:Bone Neoplasms / Q000150:complications* / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D006961:Hyperparathyroidism / Q000150:complications*; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications*; D012516:Osteosarcoma / Q000150:complications* / Q000473:pathology / Q000628:therapy