Download Nevoid basal cell carcinoma syndromeJC

Case Study
Jordan Crawford
Date: 11/9/10
Name of Syndrome:
Patient:
Age:
18
Gender:
Male
Height:
6’2’’
Weight:
185
Vital Signs:
HR:66
Respiratory rate: 18
Blood Pressure:120/82
Temperature:89.2
Chief
complaint
Medical
Alert
The appearance of skin cancer
Nevoid basal cell
carcinoma syndrome
Cause of Syndrome - Medical History (if
applicable)
Basal cell nevus syndrome is a rare
genetic condition. The gene linked to
the syndrome is passed down through
families as an autosomal dominant trait.
That means you will get the syndrome if
either parent passes the gene down to
you.
Age/race/sex predilections and
Systemic Clinical Features:
Sex
Men and women are affected equally. The
male-to-female ratio is estimated to be 1:1.3.
Age
The disease is present (inherited) at birth and
most commonly manifests itself with either
BCCs (usually multiple) occurring at a young
age (third decade or earlier) or odontogenic
keratocysts presenting in the second or third
decade.
Race
The syndrome is found in all races.
However, a definite but smaller percentage of
African Americans present with skin cancer
and have fewer skin cancers than whites who
are affected.
Orofacial Clinical Features
• Broad nose
• Heavy, protruding brow
• Jaw that sticks out (in some cases)
• Wide-set eyes
• Cleft lip or palate occurs in 3-5% of patients.
Radiographic Features of this Syndrome
• Cysts in the jaw, which can lead to abnormal
tooth development or jaw fractures.
Special considerations in Treatment of this patient? How is it Diagnosed?
How common is it? Is it a horse or zebra?
•Treatment of patients with nevoid basal cell carcinoma syndrome (NBCCS)
involves surveillance for and treatment of the associated findings. Because most of
the findings involve tumors (benign and malignant), treatment is often surgical.
•Oral surgery involving cyst enucleation followed by mechanical curettage or use of
peripheral ostectomy may be required for odontogenic keratocysts. The incidence of
recurrence following treatment is high.
•No routine laboratory studies are helpful for nevoid basal cell carcinoma syndrome
(NBCCS).
•Molecular genetic testing: Although the diagnosis is usually made in adults or older
children based on clinical findings, molecular testing is available to confirm the
diagnosis in patients with atypical findings or possibly for prenatal diagnosis.
•Biopsy of skin lesions
•Biopsy of jaw cysts
•X-rays
It is estimated that about one in 40,000
people have NBCCS.
Pictures of Disease or Syndrome
Intraoral Pictures
List Sources and References (This should be more than your text book!)
"Nevoid Basal Cell Carcinoma Syndrome | Cancer.Net." Doctor-approved Cancer Information from
ASCO | Cancer.Net. Web. 08 Nov. 2010. <http://www.cancer.net/patient/Cancer Types/Nevoid
Basal Cell Carcinoma Syndrome>.
"Nevoid Basal Cell Carcinoma Syndrome: Differential Diagnoses & Workup - EMedicine
Dermatology." EMedicine - Medical Reference. Web. 08 Nov. 2010.
<http://emedicine.medscape.com/article/1101146-diagnosis>.
"Nevoid Basal Cell Carcinoma Syndrome." WebMD - Better Information. Better Health. Web. 08
Nov. 2010. <http://www.webmd.com/cancer/nevoid-basal-cell-carcinoma-syndrome>.