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Peripheral odontogenic myxoma in a 12-year-old girl: a rare entity.

ABSTRACT: Peripheral odontogenic myxoma is a rare odontogenic tumor representing an extra osseous counterpart of central odontogenic myxoma. It is commonly seen in gingiva between the 3rd and 4th decades of life and appears predominantly in females. Compared to central odontogenic myxoma, it is a less aggressive, slow-growing lesion with a low recurrence rate. However, close postoperative follow-up is required because of the unlimited growth potential of incompletely removed lesions. It shares many features with other soft tissue myxoid proliferations occurring in the oral cavity and hence needs to be differentiated from them. Very few cases of peripheral odontogenic myxomas have been reported and, to the best of our knowledge, no case has been reported in a pediatric patient. We present an unusual case of peripheral odontogenic myxoma occurring in a 12-year-old girl located in the anterior mandibular gingiva, with an emphasis on differential diagnosis.

SUBMITTER: Kanitkar S

PROVIDER: S-EPMC5529192 | biostudies-other | 2017 Jun

REPOSITORIES: biostudies-other

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Project description:BackgroundAbernethy malformation, also known as congenital extrahepatic portosystemic shunt, is an uncommon malformation resulting from aberrant development of the portal venous system. Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the CFTR gene. It mainly affects the exocrine glands of the respiratory, digestive and reproductive systems. It is considered extremely rare in the Asian population. We present a clinical case involving a pediatric patient of Asian descent who was diagnosed with Abernethy malformation and CF.Case summaryA 12-year-old girl presented with a medical history of recurring respiratory infections and hemoptysis, and chest computed tomography (CT) showed bronchiectasis. Whole exome sequencing was performed for the patient, yielding findings that revealed a compound heterozygous variant of the CFTR gene: c.233_c.234insT/p.Trp79fsTer3 (maternal origin); c.2909G>A/p.Gly970Asp (paternal origin). CF was diagnosed. The physician's attention was drawn to the presence of splenomegaly during disease progression. Abdominal enhanced CT revealed splenomegaly, compression of the left kidney, and multiple tortuous dilated vascular shadows were seen at the splenic hilum, which flowed back into the left renal vein and portal vein, suggesting Abernethy malformation type II. Intraoperatively, the abnormal blood flow was seen to merge into the inferior vena cava through the left renal vein without hepatic processing, and the pathology of liver biopsy showed hypoplastic, dilated or absent portal vein branches, both of which supported the diagnosis of Abernethy malformation type II. This represents the initial documented instance of Abernethy malformation accompanied by a CFTR gene mutation in the existing body of literature.ConclusionCoexisting Abernethy malformation and CF are rare. Detailed medical history information, abdominal enhanced CT, venography and genetic testing contribute to diagnosis as well as differential diagnosis.

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Peripheral odontogenic myxoma in a 12-year-old girl: a rare entity.

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