title: Hyperphosphatemic familial tumoral calcinosis: odontostomatologic management and pathological features.
abstract: BACKGROUND
  Hyperphosphatemic familial tumoral calcinosis (HFTC) is to a rare autosomal recessive disorder characterized by cutaneous and sub-cutaneous calcified masses, usually adjacent to large joints. The aim of the current study was to report on the clinico-pathological features of a patient with HFCT, with emphasis on alterations in the jawbones and teeth and the subsequent therapeutic interventions.
  
  
  METHODS
  A 13-year-old male patient with HFTC diagnosis came to our attention for dental anomalies and maxillary and mandibular hypoplasia. OPT highlighted multiple impacted teeth, short and bulbous teeth, and pulp chamber and canal obliterations. Lateral cephalometric radiograms pointed out retrusion of both jaws, skeletal class II malocclusion, and deep-bite. He underwent orthopedic, orthodontic, conservative, and surgical treatments, allowing the correction of maxillo-facial and dental abnormalities and dysmorphisms without adverse effects. The surgical samples were sent for conventional and confocal laser scanning microscope (CLSM) histopathological examination, which highlighted several metaplastic micro- and macro-calcifications in the soft tissues, and typical islands of homogenous, non-tubular, dentino-osteoid calcified structures in dentinal tissues.
  
  
  CONCLUSIONS
  The management of maxillo-facial abnormalities in patients affected by HFTC is very difficult and, requires a combined therapeutic approach. To date, very few indications have been published in the literature.
authors.affiliation: Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology, Aldo Moro University, Barii, Italy.
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Favia
authors.affiliation: Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology, Aldo Moro University, Barii, Italy.
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Lacaita
authors.affiliation: Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology, Aldo Moro University, Barii, Italy.
authors.forename: Luisa
authors.initials: L
authors.lastname: Limongelli
authors.affiliation: Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology, Aldo Moro University, Barii, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Tempesta
authors.affiliation: Department of Biomedical Science and Human Oncology, Complex Operating Unit of Pediatrics, Aldo Moro University, Barii, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Laforgia
authors.affiliation: Department of Interdisciplinary Medicine, Complex Operating Unit of Odontostomatology, Aldo Moro University, Barii, Italy.
authors.forename: Angela Pia
authors.initials: AP
authors.lastname: Cazzolla
authors.affiliation: Department of Emergency and Organ Transplantation, Pathological Anatomy, Aldo Moro University, Barii, Italy.
authors.forename: Eugenio
authors.initials: E
authors.lastname: Maiorano
mesh_terms: D000293:Adolescent; D002114:Calcinosis / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006801:Humans; D006958:Hyperostosis, Cortical, Congenital / Q000150:complications* / Q000473:pathology / Q000601:surgery; D054559:Hyperphosphatemia / Q000150:complications* / Q000473:pathology / Q000601:surgery; D007571:Jaw Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D008297:Male; D019647:Oral Surgical Procedures; D009971:Orthodontics, Corrective; D014076:Tooth Diseases / Q000209:etiology / Q000473:pathology / Q000601:surgery*