Browsing by Author "Fantasia, John E"

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    Bilateral Central Giant Cell Granulomas of the Mandible in An Eight Year-Old Girl with Noonan Syndrome (Noonan-Like/Multiple Giant Cell Lesions Syndrome)
    (2005-03) Edwards, Paul C.; Fox, Joyce; Fantasia, John E; Goldberg, Jeff; Kelsch, Robert D
    A number of conditions can present with lesions that histologically are indistinguishable from the central giant cell granuloma (CGCG) of bone, including brown tumors of hyperparathyroidism, cherubism, and, less commonly, a number of inherited syndromes. We report a case of an eight-year girl who presented with bilateral CGCGs of the posterior mandible. Characteristic facial features, reported increased post-operative bleeding and history of pulmonary stenosis led us to suspect a diagnosis of Noonan syndrome. A medical geneticist confirmed this on further evaluation. This case report will discuss the salient features of this diagnosis.
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    Clinically aggressive central giant cell granulomas in two patients with neurofibromatosis 1
    (2006-12) Edwards, Paul C.; Fantasia, John E; Saini, Tamjit; Rosenberg, Tracey J; Sachs, Stephen A; Ruggiero, Salvatore
    Background Neurofibromatosis 1 (NF1) is an autosomal dominantly inherited disorder caused by a spectrum of mutations affecting the Nf1 gene. Affected patients develop benign and malignant tumors at an increased frequency. Clinical findings include multiple cutaneous café-au-lait pigmentations, neurofibromas, axillary freckling, optic gliomas, benign iris hamartomas (Lisch nodules), scoliosis, and poorly defined soft tissue lesions of the skeleton. Kerl first reported an association of NF1 with multiple central giant cell granulomas (CGCGs) of the jaws. There have since been 4 additional published cases of NF1 patients with CGCGs of the jaws. Clinical cases We report on 2 patients who presented with NF1 and aggressive CGCGs of the jaws. In both cases, the clinical course was characterized by numerous recurrences despite mechanical curettage and surgical resection. Conclusions We review proposed mechanisms to explain the apparent association between NF1 and an increased incidence of CGCGs of the jaws. While the presence of CGCGs of the jaws in patients with NF1 could represent either a coincidental association or a true genetic linkage, we propose that this phenomenon is most likely related to NF1-mediated osseous dysplasia. Compared to normal bone, the Nf1-haploinsufficient bone in a patient with NF1 may be less able to remodel in response to as of yet unidentified stimuli (e.g. excessive mechanical stress and/or vascular fragility), and consequently may be more susceptible to developing CGCG-like lesions. Alternatively, the CGCG in NF1 patients could represent a true neoplasm, resulting from additional, as of yet unidentified, genetic alterations to Nf1-haploinsufficient bone.
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    Foreign Body Reaction to Hyaluronic Acid (Restylane®): An Adverse Outcome of Lip Augmentation
    (2006-08) Edwards, Paul C.; Fantasia, John E; Iovino, Robert
    Non-animal source hyaluronic acid (Restylane®) is a relatively new redefining dermal filler that is being employed with increasing frequency in the fields of dermatology and cosmetic/facial plastic surgery. We report a case of a 74-year-old woman who presented with a firm submucosal nodule of the lower lip, which clinically was thought to represent a benign neoplasm. An excisional biopsy revealed the presence of multiple cyst-like vacuolated areas surrounded by granulomatous tissue composed predominantly of histiocytes and foamy macrophages, consistent with a foreign body reaction. Subsequent to the pathology findings, the patient acknowledged that she had received injections of Restylane® to the lips approximately 6 months before discovering the nodule. She had not mentioned this to her dentist or oral and maxillofacial surgeon to whom she had been referred because she believed that these two events were not related. Although hyaluronic acid-based dermal fillers reportedly have a low incidence of long term side effects, clinicians should be aware of the possible development of foreign body reactions to these injectable agents.
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    Salivary Heterotopia Of The Parathyroid Gland: A Report of Two Cases and Review of the Literature
    (2005-05) Edwards, Paul C.; Bhuiya, Taqfiq; Kahn, Leonard B; Fantasia, John E
    Two cases of periparathyroid salivary gland heterotopia are described. A review of the records of the Department of Pathology, Long Island Jewish Medical Center, over a 4-year period, identified 759 surgical specimens containing parathyroid gland tissue. Of these, 2 (0.26%) contained foci of ectopic salivary gland tissue. Both cases were associated with cyst formation. To date, 9 additional cases of heterotopic salivary gland tissue associated with the parathyroid gland have been described in the literature.