Autosomal dominant or
sporadic
STK11 gene (serine-threonine,
protein kinase) tumor suppressor gene
Presents in childhood
Clinical findings
Lentigines on mucosa,
perorificial skin, palms and digits
Gasterointestinal
hamartomatous polyps (More common in small intestine) may cause
bleeding, pain, intussuception, obstruction, adenocarcinoma
Ovarian cancer is the
most common malignancy
Breast cancer,
pancreatic cancer
Diagnosis:
monitor with
colonoscopy, polypectomy
Course:
normal lifespan
malignancy detected early
Benign
gastrointestinal polyps
Oral and labial
melanotic macules: can be associated with LEOPARD syndrome (Lentigines,
electrocardiographic conduction defects, ocular hypertelorism,
pulmonary stenosis, abnormal genetalia, retardation of growth,
and deafness
Laugier-Hunziker
syndrome: absence of intestinal polyps
Histology:
mild acanthosis and
hyperpigmentation of basal layer
Treatment:
cryosuergery (melanocytes
freeze at -4 to -7 degree centigrade, laser, tretinoin, chemical
pee)
Comment by; Dr.
Mehrdad Mehravaran, Szeged, Hungary: