Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome: A case report

Fidelis O. Ojevwe, Cindy D. Ojevwe, James P. Zacny, Arkadiusz Z. Dudek, Amy Lin, Patrick Kohlitz

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background: Nevoid basal cell carcinoma syndrome (NBCCS), which is also known by other names, including Gorlin-Goltz syndrome and multiple basal-cell carcinoma (BCC) syndrome, is a rare multi-systemic disease inherited in a dominant autosomal manner with complete penetrance and variable expressivity. The main clinical manifestations include multiple BCCs, odontogenic keratocysts of the jaw, hyperkeratosis of the palms and soles, skeletal abnormalities, intracranial calcifications and facial deformities. Patients and Methods: A 31-year-old male diagnosed with Gorlin-Goltz syndrome with multiple unresectable facial BCCs was treated with the Hedgehog inhibitor vismodegib. Results: After one month of therapy on vismodegib, there were significant reductions in the size of multiple BCCs on the patient's face. The patient remains on this therapy. Conclusion: Hedgehog pathway inhibition is an effective strategy to treat unresectable BCCs from Gorlin-Goltz syndrome. Although vismodegib shows some promising clinical results in the early phase of its use, there are concerns of possible resistance developing within months. Duration of therapy, role of maintenance treatment and drug modification to reduce resistance need to be explored in future case studies.

Original languageEnglish (US)
Pages (from-to)1777-1781
Number of pages5
JournalAnticancer Research
Volume35
Issue number3
StatePublished - Mar 1 2015

Fingerprint

HhAntag691
Basal Cell Nevus Syndrome
Odontogenic Cysts
Penetrance
Therapeutics
Jaw
Names
Multiple basal cell carcinoma
Pharmaceutical Preparations

All Science Journal Classification (ASJC) codes

  • Oncology
  • Cancer Research

Cite this

Ojevwe, F. O., Ojevwe, C. D., Zacny, J. P., Dudek, A. Z., Lin, A., & Kohlitz, P. (2015). Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome: A case report. Anticancer Research, 35(3), 1777-1781.
Ojevwe, Fidelis O. ; Ojevwe, Cindy D. ; Zacny, James P. ; Dudek, Arkadiusz Z. ; Lin, Amy ; Kohlitz, Patrick. / Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome : A case report. In: Anticancer Research. 2015 ; Vol. 35, No. 3. pp. 1777-1781.
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abstract = "Background: Nevoid basal cell carcinoma syndrome (NBCCS), which is also known by other names, including Gorlin-Goltz syndrome and multiple basal-cell carcinoma (BCC) syndrome, is a rare multi-systemic disease inherited in a dominant autosomal manner with complete penetrance and variable expressivity. The main clinical manifestations include multiple BCCs, odontogenic keratocysts of the jaw, hyperkeratosis of the palms and soles, skeletal abnormalities, intracranial calcifications and facial deformities. Patients and Methods: A 31-year-old male diagnosed with Gorlin-Goltz syndrome with multiple unresectable facial BCCs was treated with the Hedgehog inhibitor vismodegib. Results: After one month of therapy on vismodegib, there were significant reductions in the size of multiple BCCs on the patient's face. The patient remains on this therapy. Conclusion: Hedgehog pathway inhibition is an effective strategy to treat unresectable BCCs from Gorlin-Goltz syndrome. Although vismodegib shows some promising clinical results in the early phase of its use, there are concerns of possible resistance developing within months. Duration of therapy, role of maintenance treatment and drug modification to reduce resistance need to be explored in future case studies.",
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Ojevwe, FO, Ojevwe, CD, Zacny, JP, Dudek, AZ, Lin, A & Kohlitz, P 2015, 'Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome: A case report', Anticancer Research, vol. 35, no. 3, pp. 1777-1781.

Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome : A case report. / Ojevwe, Fidelis O.; Ojevwe, Cindy D.; Zacny, James P.; Dudek, Arkadiusz Z.; Lin, Amy; Kohlitz, Patrick.

In: Anticancer Research, Vol. 35, No. 3, 01.03.2015, p. 1777-1781.

Research output: Contribution to journalArticle

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AU - Kohlitz, Patrick

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N2 - Background: Nevoid basal cell carcinoma syndrome (NBCCS), which is also known by other names, including Gorlin-Goltz syndrome and multiple basal-cell carcinoma (BCC) syndrome, is a rare multi-systemic disease inherited in a dominant autosomal manner with complete penetrance and variable expressivity. The main clinical manifestations include multiple BCCs, odontogenic keratocysts of the jaw, hyperkeratosis of the palms and soles, skeletal abnormalities, intracranial calcifications and facial deformities. Patients and Methods: A 31-year-old male diagnosed with Gorlin-Goltz syndrome with multiple unresectable facial BCCs was treated with the Hedgehog inhibitor vismodegib. Results: After one month of therapy on vismodegib, there were significant reductions in the size of multiple BCCs on the patient's face. The patient remains on this therapy. Conclusion: Hedgehog pathway inhibition is an effective strategy to treat unresectable BCCs from Gorlin-Goltz syndrome. Although vismodegib shows some promising clinical results in the early phase of its use, there are concerns of possible resistance developing within months. Duration of therapy, role of maintenance treatment and drug modification to reduce resistance need to be explored in future case studies.

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Ojevwe FO, Ojevwe CD, Zacny JP, Dudek AZ, Lin A, Kohlitz P. Treatment of multiple unresectable basal cell carcinomas from gorlin-goltz syndrome: A case report. Anticancer Research. 2015 Mar 1;35(3):1777-1781.