Limits...
Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome.

Booms P, Harth M, Sader R, Ghanaati S - Ann Maxillofac Surg (2015 Jan-Jun)

Bottom Line: Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors.Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib.The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation.

View Article: PubMed Central - PubMed

Affiliation: Frankfurt Orofacial Regenerative Medicine, University Hospital Frankfurt, Frankfurt am Main, Germany.

ABSTRACT
Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors. They are major criteria for the diagnosis of Gorlin syndrome, also called nevoid basal cell carcinoma syndrome. Clinical features of Gorlin syndrome are reported, and the relevance of hedgehog signaling pathway inhibition by oral vismodegib for maxillofacial surgeons is highlighted. In summary, progressed basal cell carcinoma lesions are virtually inoperable. Keratocystic odontogenic tumors have an aggressive behavior including rapid growth and extension into adjacent tissues. Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib. Due to radio-hypersensitivity in Gorlin syndrome, avoidance of treatment by radiotherapy is strongly recommended for all affected individuals. Vismodegib can help in those instances where radiation is contra-indicated, or the lesions are inoperable. The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation. Vismodegib, a new and approved drug for the treatment of advanced basal cell carcinoma, is a specific oncogene inhibitor. It also seems to be effective for treatment of keratocystic odontogenic tumors and basal cell carcinomas in Gorlin syndrome, rendering the surgical resections less challenging.

No MeSH data available.


Related in: MedlinePlus

Images of a patient with Gorlin-Goltz syndrome: (a) Calcification of the falx cerebri (). (b) Lesions () of the mandible. (c) Mandibular cystic lucencies (). (d) Considerable loss of bone () in the mandible. (e and f) Mandibular odontogenic keratocysts ()
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4555941&req=5

Figure 1: Images of a patient with Gorlin-Goltz syndrome: (a) Calcification of the falx cerebri (). (b) Lesions () of the mandible. (c) Mandibular cystic lucencies (). (d) Considerable loss of bone () in the mandible. (e and f) Mandibular odontogenic keratocysts ()

Mentions: KOTs mostly occur in the jaws [Figure 1], with potential for substantial (facial) disfigurement and speech impediment. The current treatment option is complete surgical resection, however, the procedures are often technically challenging, because of the location of the cyst to inaccessible sites – due to complex anatomical structures of the head and neck region. Therefore, complete resection is not always achievable, and subsequently, there are high recurrence rates. More than 65% of patients with GS have KOTs. The KOTs that are related to GS grow to sizes larger than any other.[6] These KOTs have an aggressive behavior including rapid growth and extension into adjacent tissues.[7] Obviously, this is of importance for the maxillofacial surgeons.


Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome.

Booms P, Harth M, Sader R, Ghanaati S - Ann Maxillofac Surg (2015 Jan-Jun)

Images of a patient with Gorlin-Goltz syndrome: (a) Calcification of the falx cerebri (). (b) Lesions () of the mandible. (c) Mandibular cystic lucencies (). (d) Considerable loss of bone () in the mandible. (e and f) Mandibular odontogenic keratocysts ()
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4555941&req=5

Figure 1: Images of a patient with Gorlin-Goltz syndrome: (a) Calcification of the falx cerebri (). (b) Lesions () of the mandible. (c) Mandibular cystic lucencies (). (d) Considerable loss of bone () in the mandible. (e and f) Mandibular odontogenic keratocysts ()
Mentions: KOTs mostly occur in the jaws [Figure 1], with potential for substantial (facial) disfigurement and speech impediment. The current treatment option is complete surgical resection, however, the procedures are often technically challenging, because of the location of the cyst to inaccessible sites – due to complex anatomical structures of the head and neck region. Therefore, complete resection is not always achievable, and subsequently, there are high recurrence rates. More than 65% of patients with GS have KOTs. The KOTs that are related to GS grow to sizes larger than any other.[6] These KOTs have an aggressive behavior including rapid growth and extension into adjacent tissues.[7] Obviously, this is of importance for the maxillofacial surgeons.

Bottom Line: Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors.Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib.The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation.

View Article: PubMed Central - PubMed

Affiliation: Frankfurt Orofacial Regenerative Medicine, University Hospital Frankfurt, Frankfurt am Main, Germany.

ABSTRACT
Vismodegib hedgehog signaling inhibition treatment has potential for reducing the burden of multiple skin basal cell carcinomas and jaw keratocystic odontogenic tumors. They are major criteria for the diagnosis of Gorlin syndrome, also called nevoid basal cell carcinoma syndrome. Clinical features of Gorlin syndrome are reported, and the relevance of hedgehog signaling pathway inhibition by oral vismodegib for maxillofacial surgeons is highlighted. In summary, progressed basal cell carcinoma lesions are virtually inoperable. Keratocystic odontogenic tumors have an aggressive behavior including rapid growth and extension into adjacent tissues. Interestingly, nearly complete regression of multiple Gorlin syndrome-associated keratocystic odontogenic tumors following treatment with vismodegib. Due to radio-hypersensitivity in Gorlin syndrome, avoidance of treatment by radiotherapy is strongly recommended for all affected individuals. Vismodegib can help in those instances where radiation is contra-indicated, or the lesions are inoperable. The effect of vismodegib on basal cell carcinomas was associated with a significant decrease in hedgehog-signaling and tumor proliferation. Vismodegib, a new and approved drug for the treatment of advanced basal cell carcinoma, is a specific oncogene inhibitor. It also seems to be effective for treatment of keratocystic odontogenic tumors and basal cell carcinomas in Gorlin syndrome, rendering the surgical resections less challenging.

No MeSH data available.


Related in: MedlinePlus