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Palmar Basal Cell Carcinoma in a Patient With Gorlin-Goltz Syndrome

Palmar Basal Cell Carcinoma in a Patient With Gorlin-Goltz Syndrome Report of a Case We describe herein a 67-year-old white woman affected with Gorlin-Goltz syndrome. In early adulthood, she developed various odontogenic cysts and multiple basal cell carcinomas (BCCs). Clinical examination revealed multiple small BCCs on the patient's trunk and numerous palmar pits. On her left palm, a waxy, flesh-colored, ulcerated nodule measuring 0.6 × 0.5 cm with telangiectases was observed (Figure 1). The patient reported that this lesion had slowly grown over years. Figure 1. View LargeDownload On our patient's left palm, we noted a waxy, flesh-colored, ulcerated nodule measuring 0.6 × 0.5 cm with telangiectases. Clinically, the differential diagnosis of BCC or eccrine poroma was considered. Under dermoscopy, we saw a whitish ulcerated lesion with prominent arborizing telangiectasis emerging from the periphery (Figure 2) and multiple pits with an irregularly shaped depressed aspect, geographic borders, and a linear pattern of arranged red globules following the furrows of the acral skin. Some of the pits were directly in contact with the tumor. Figure 2. View LargeDownload Under dermoscopy, a whitish ulcerated lesion was seen, with prominent arborizing telangiectasis emerging from the periphery. Since we could not distinguish any criteria for melanocytic lesion but the presence of ulceration and arborizing vessels, our diagnosis was nodular BCC. The lesion was removed and histopathologic analysis confirmed our suspicion of BCC. Comment In 1960, Gorlin and Goltz1 described the “nevoid basal cell carcinoma syndrome,” an infrequent autosomal dominant disorder predisposing to cancer and characterized by the presence of multiple BCCs, jaw cysts, and bifid ribs. This disorder is caused by germline mutations of a tumor suppressor gene (PTCH) on chromosome 9q22.3-q31.2 The main cutaneous manifestations of this syndrome are the presence of multiple BCCs and acral pits. Basal cell carcinoma lesions associated with Gorlin-Goltz syndrome differ from those in patients without the syndrome in their early onset and increased number, and they are less related to prolonged sun exposure. They also appear in unusual body sites, like the eyelid or the upper lip. The palm of the hand is the rarest site of the skin to be affected by BCC.3 The occurrence of BCC on acral sites has been related to xeroderma pigmentosum, trauma, intake of carcinogens such as arsenic, or Gorlin-Goltz syndrome.3 Howell and Freeman4 have suggested that palmar pits represent an initial form of BCC. In our case, the presence of the dermoscopic image of palmar pits in contiguity with the tumor may be suggestive of this origin. Since volar pits are more frequently found than volar BCC in Gorlin-Goltz syndrome, it might be that volar pits are predisposed to develop BCC instead of being initial forms of BCC. A coincidental co-occurrence of both lesions might also be considered. Histopathologically, palmar and plantar pits show small, well-defined areas of absence of dense keratin layer and irregularity of the epidermal rete ridges. They are often overlooked, but dermoscopy can be helpful in discovering them. Dermoscopy reveals small, flesh-colored, irregularly shaped depressed lesions with characteristic geographic borders and a typical pattern of linearly arranged red globules.5 To our knowledge, this is the first dermoscopic description of an acral BCC in contiguity with palmar pits. Our case highlights specific dermoscopic criteria for both acral BCC (ulceration and arborizing vessels) and palmar pits in the context of a patient affected with Gorlin-Goltz syndrome. Correspondence: Dr Puig, Center for Diagnosis of Pigmentary Lesions and Melanoma Unit, Department of Dermatology, Hospital Clinic, C/Villarroel 170, 08036 Barcelona, Spain (spuig@clinic.ub.es). Financial Disclosure: None reported. Back to top Article Information Funding/Support: This work was funded by grants 03/0019 and 06/0265 from the Fondo de Investigaciones Sanitarias, Madrid, Spain, grant RO-1 CA 83115 (fund 538226) from the National Cancer Institute, Bethesda, Md, and Project No. 018702, GenoMel, European Commission, Net of Excellence, Leeds, England (Drs Puig and Malvehy). References 1. Gorlin RJGoltz RW Multiple nevoid basal-cell epithelioma, jaw cysts and bifid ribs: a syndrome. N Engl J Med 1960;262908- 912PubMedGoogle ScholarCrossref 2. Gorlin RJEhrhart JC Naevoid basal cell carcinoma (Gorlin) syndrome. LeBoit PEBurg GWeedon DSarasin Aeds. WHO Classification of Tumours: Pathology and Genetics of Skin Tumours. Lyon, France IARC Press2006;285- 287Google Scholar 3. Salomão PKopke LFMachado-Pinto J Palmar basal cell carcinoma: Case report and literature review. Dermatol Surg 1999;25908- 910PubMedGoogle ScholarCrossref 4. Howell JBFreeman RG Structure and significance of the pits with their tumors in the nevoid basal cell carcinoma syndrome. J Am Acad Dermatol 1980;2224- 238PubMedGoogle ScholarCrossref 5. Kolm IPuig SIranzo PMalvehy J Dermoscopy in Gorlin-Goltz syndrome. Dermatol Surg 2006;32847- 851PubMedGoogle Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Palmar Basal Cell Carcinoma in a Patient With Gorlin-Goltz Syndrome

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References (6)

Publisher
American Medical Association
Copyright
Copyright © 2007 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.143.6.813
pmid
17576964
Publisher site
See Article on Publisher Site

Abstract

Report of a Case We describe herein a 67-year-old white woman affected with Gorlin-Goltz syndrome. In early adulthood, she developed various odontogenic cysts and multiple basal cell carcinomas (BCCs). Clinical examination revealed multiple small BCCs on the patient's trunk and numerous palmar pits. On her left palm, a waxy, flesh-colored, ulcerated nodule measuring 0.6 × 0.5 cm with telangiectases was observed (Figure 1). The patient reported that this lesion had slowly grown over years. Figure 1. View LargeDownload On our patient's left palm, we noted a waxy, flesh-colored, ulcerated nodule measuring 0.6 × 0.5 cm with telangiectases. Clinically, the differential diagnosis of BCC or eccrine poroma was considered. Under dermoscopy, we saw a whitish ulcerated lesion with prominent arborizing telangiectasis emerging from the periphery (Figure 2) and multiple pits with an irregularly shaped depressed aspect, geographic borders, and a linear pattern of arranged red globules following the furrows of the acral skin. Some of the pits were directly in contact with the tumor. Figure 2. View LargeDownload Under dermoscopy, a whitish ulcerated lesion was seen, with prominent arborizing telangiectasis emerging from the periphery. Since we could not distinguish any criteria for melanocytic lesion but the presence of ulceration and arborizing vessels, our diagnosis was nodular BCC. The lesion was removed and histopathologic analysis confirmed our suspicion of BCC. Comment In 1960, Gorlin and Goltz1 described the “nevoid basal cell carcinoma syndrome,” an infrequent autosomal dominant disorder predisposing to cancer and characterized by the presence of multiple BCCs, jaw cysts, and bifid ribs. This disorder is caused by germline mutations of a tumor suppressor gene (PTCH) on chromosome 9q22.3-q31.2 The main cutaneous manifestations of this syndrome are the presence of multiple BCCs and acral pits. Basal cell carcinoma lesions associated with Gorlin-Goltz syndrome differ from those in patients without the syndrome in their early onset and increased number, and they are less related to prolonged sun exposure. They also appear in unusual body sites, like the eyelid or the upper lip. The palm of the hand is the rarest site of the skin to be affected by BCC.3 The occurrence of BCC on acral sites has been related to xeroderma pigmentosum, trauma, intake of carcinogens such as arsenic, or Gorlin-Goltz syndrome.3 Howell and Freeman4 have suggested that palmar pits represent an initial form of BCC. In our case, the presence of the dermoscopic image of palmar pits in contiguity with the tumor may be suggestive of this origin. Since volar pits are more frequently found than volar BCC in Gorlin-Goltz syndrome, it might be that volar pits are predisposed to develop BCC instead of being initial forms of BCC. A coincidental co-occurrence of both lesions might also be considered. Histopathologically, palmar and plantar pits show small, well-defined areas of absence of dense keratin layer and irregularity of the epidermal rete ridges. They are often overlooked, but dermoscopy can be helpful in discovering them. Dermoscopy reveals small, flesh-colored, irregularly shaped depressed lesions with characteristic geographic borders and a typical pattern of linearly arranged red globules.5 To our knowledge, this is the first dermoscopic description of an acral BCC in contiguity with palmar pits. Our case highlights specific dermoscopic criteria for both acral BCC (ulceration and arborizing vessels) and palmar pits in the context of a patient affected with Gorlin-Goltz syndrome. Correspondence: Dr Puig, Center for Diagnosis of Pigmentary Lesions and Melanoma Unit, Department of Dermatology, Hospital Clinic, C/Villarroel 170, 08036 Barcelona, Spain (spuig@clinic.ub.es). Financial Disclosure: None reported. Back to top Article Information Funding/Support: This work was funded by grants 03/0019 and 06/0265 from the Fondo de Investigaciones Sanitarias, Madrid, Spain, grant RO-1 CA 83115 (fund 538226) from the National Cancer Institute, Bethesda, Md, and Project No. 018702, GenoMel, European Commission, Net of Excellence, Leeds, England (Drs Puig and Malvehy). References 1. Gorlin RJGoltz RW Multiple nevoid basal-cell epithelioma, jaw cysts and bifid ribs: a syndrome. N Engl J Med 1960;262908- 912PubMedGoogle ScholarCrossref 2. Gorlin RJEhrhart JC Naevoid basal cell carcinoma (Gorlin) syndrome. LeBoit PEBurg GWeedon DSarasin Aeds. WHO Classification of Tumours: Pathology and Genetics of Skin Tumours. Lyon, France IARC Press2006;285- 287Google Scholar 3. Salomão PKopke LFMachado-Pinto J Palmar basal cell carcinoma: Case report and literature review. Dermatol Surg 1999;25908- 910PubMedGoogle ScholarCrossref 4. Howell JBFreeman RG Structure and significance of the pits with their tumors in the nevoid basal cell carcinoma syndrome. J Am Acad Dermatol 1980;2224- 238PubMedGoogle ScholarCrossref 5. Kolm IPuig SIranzo PMalvehy J Dermoscopy in Gorlin-Goltz syndrome. Dermatol Surg 2006;32847- 851PubMedGoogle Scholar

Journal

Archives of DermatologyAmerican Medical Association

Published: Jun 1, 2007

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