Case of Atypical Adenoid Hyperplasia of Lung with Polyostotic Fibrous Dysplasia and Diabetes Mellitus: A Random Coincidence or a Possible Unrecognized Syndromic Association?

Fibrous dysplasia (FD) is a benign intramedullary pathologic condition that is characterized by the replacement of bone with fibrous tissue. FD may be monostotic or polyostotic with the craniofacial bones, and the proximal femur is most commonly involved. A 39-year-old lady presented to our hospital, a tertiary care center with asymmetric swelling of her arms, for over 20 years. Radiographs revealed gross enlargement with marrow expansion of the right humerus, scapula with ground-glass matrix, along with a multiseptated cystic appearance. Positron emission tomography-computed tomography screening for the activity of these lesions incidentally demonstrated a few lung nodules, which on biopsy was found to be atypical adenomatous hyperplasia. The lady also had endocrine dysfunction in the form of diabetes mellitus, for which she was on treatment. In this article, we briefly review the available literature to decipher if any of the associated syndromes with polyostotic FD (being the dominant clinical feature) are known to have associations which explain the above findings. There is a need to recognize the underlying pattern so that appropriate genetic counseling, if any, can be provided to such patients.

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