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Related Experiment Videos

Familial gigantiform cementoma.

S J Finical1, W J Kane, R P Clay

  • 1Division of Plastic Surgery, Mayo Clinic and Mayo Foundation, Rochester, Minn 55901, USA.

Plastic and Reconstructive Surgery
|March 17, 1999
PubMed
Summary

Familial gigantiform cementoma, a rare benign tumor, can cause facial disfigurement. Early surgical intervention and reconstruction yield good functional and aesthetic outcomes with no recurrence observed.

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Area of Science:

  • Oral and Maxillofacial Surgery
  • Genetics
  • Oncology

Background:

  • Familial gigantiform cementoma (FGC) is a rare autosomal dominant condition.
  • Tumors can lead to significant facial disfigurement and skeletal abnormalities.
  • Delayed treatment can result in massive tumor growth requiring complex surgical intervention.

Purpose of the Study:

  • To report on the treatment and outcomes of five patients from two families with FGC.
  • To highlight the importance of early diagnosis and intervention for FGC.
  • To describe the surgical management and reconstructive techniques used for extensive FGC.

Main Methods:

  • Case series involving five patients with FGC from two families.
  • Surgical resection of massive cementoma tumors.

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  • Multi-stage reconstruction of facial soft tissues and skeleton.
  • Pre- and post-operative assessment of relevant clinical and biochemical markers.

    • Main Results:

    • Three adult patients presented with massive tumors requiring extensive, multi-stage resection and reconstruction.
    • Two female patients required hysterectomy due to anemia from uterine adenomas prior to FGC treatment.
    • Elevated alkaline phosphatase levels in three patients normalized post-surgery.
    • No tumor recurrence was observed during a 3-year follow-up period.

    Conclusions:

    • Extensive resection and complex reconstruction can achieve favorable functional and aesthetic results in FGC patients.
    • Early treatment is crucial to prevent severe disfigurement and complications associated with FGC.
    • Multidisciplinary management is essential for addressing systemic complications and achieving optimal outcomes.