CASE REPORT   

European Journal of Oral and Maxillofacial Surgery 2021 December;5(3):110-6

DOI: 10.23736/S2532-3466.20.00232-5

Copyright © 2020 EDIZIONI MINERVA MEDICA

lingua: Inglese

A rare presentation of myeloid sarcoma in coalition with Noonan Syndrome

Deepak PAI, Jency A. MATHEW ✉, Athiramol CHANDRAN, Divya AGRAWAL

Department of Oral and Maxillofacial Surgery, AJ Institute of Dental Sciences, Mangalore, India

The aim of this article is to provide knowledge regarding a rare case such as myeloid sarcoma, especially when in association with Noonan Syndrome and also provide guidance for the choice of investigation to come to a final diagnosis. A 45-year-old female patient presented with complaints of pain and swelling over the right side of the mandible which she had observed for 2 months. On examination the patient showed features of Noonan Syndrome with cardiac anomalies. Her radiographic evaluation revealed the presence of bilateral osteolytic lesions with respect to her mandible. The incisional biopsy suggested the presence of bilateral central giant cell granuloma for which she underwent segmental resection on one side and marginal mandibulectomy on the other. However, immunohistochemistry studies revealed the presence of myeloid sarcoma. Patients diagnosed with Noonan Syndrome, even though it is a rare entity, should be evaluated for systemic and local anomalies. In childhood, the cardiologic malformations should be the first priority followed by follow up for oncological evaluation. As with our current case of interest a detailed investigation including advanced radiographic and serological studies are advised to ascertain the nature of the pathology.

KEY WORDS: Noonan Syndrome; Myeloid sarcoma; Giant cell granuloma