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Gorlin's syndrome presenting with myolipoma of tongue base

  • L Barker (a1), S Lo (a1) and R Sudderick (a1)

We report the first case of tongue base myolipoma associated with Gorlin's syndrome.


Case report and review of world literature.


A 39-year-old man with known Gorlin's syndrome presented with progressive dysphagia. Subsequent magnetic resonance imaging scan and biopsy confirmed the rare diagnosis of myolipoma arising from the tongue base. In view of the benign nature of this mass, it was debulked rather than completely excised in order to preserve swallowing function.


To our knowledge, this is the first report of the coexistence of two rare conditions in an atypical fashion. This case appears to represent a new variant in the broad spectrum of features of Gorlin's syndrome.

Corresponding author
Address for correspondence: Dr Stephen Lo, Department of Otolaryngology, Royal Surrey County Hospital, Guildford GU2 7XX, UK. Fax: 01483 464108 E-mail:
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1PJW Stoelinga , JH Peter , WJB Van de Staak , MM Cohen Jr. Some new findings in the basal cell naevus syndrome. Oral Surg 1973;36:686–92

2RJ Gorlin , RW Goltz . Multiple nevoid basal cell epithelioma, jaw cyst and bifid rib: a syndrome. New Engl J Med 1960;262:908–12

3AE Bale . The nevoid basal cell carcinoma syndrome: genetics and mechanism of carcinogenesis. Cancer Invest 1997;15:180–6

4E Shafei-Benaissa , JR Savage , P Babin , M Larregue , D Papworth , J Tanzer The naevoid basal-cell carcinoma syndrome (Gorlin syndrome) is a chromosomal instability syndrome. Mutat Res 1998;397:287–92

5PA Farndon , RG Del Mastro , DG Evans , MW Kilpatrick . Location of gene for Gorlin syndrome. Lancet 1992;339:581–2

6RL Johnson , AL Rothman , J Xie , LV Goodrich , JW Bare , JM Bonifas Human homolog of patched, a candidate gene for the basal cell naevus syndrome. Science 1996;272:1668–71

7VE Kimonis , SG Mehta , JJ Digiovanna , SJ Bale , B Pastakia . Radiological features in 82 patients with nevoid basal cell carcinoma (NBCC or Gorlin) syndrome. Genet Med 2004;6:495502

8MR Gailani . Development defects in Gorlin syndrome related to a putative tumour suppressor gene on chromosome 9. Cell 1992; 69:111–17

9H Hahn , C Wicking , PG Zaphiropoulous , MR Gailani , S Shanley , A Chidambaram Mutations of the human homolog of Drosphilia patched in the nevoid basal cell carcinoma syndrome. Cell 1996;85:841–51

11K Tabuchi , H Okubo , K Takahashi , I Uemaetomari , H Shimoyamada , Y Nakayama Nevoid basal cell carcinoma syndrome: a report of an aggressive case with ethmoid sinus invasion. Auris Nasus Larynx 2003;30:421–3

12E Hermes , T Liekenbrocker , M Korner , A Kapp , B Wedi . Hexadactyly of hand and feet in a patient with basal cell nevus syndrome [in German]. Hautarzt 2002;53:672–6

15R Cowan , P Hoban , A Kelsey , JM Birch , R Gattamaneni , DG Evans . The gene for the naevoid basal cell carcinoma syndrome acts as a tumour suppressor gene in medulloblastoma. Br J Cancer 1997;76:141–5

18B Yilmaz , LH Goldberg , NR Schechter , BL Kemp , H Ruiz . Basal cell nevus syndrome concurrent with adenoid carcinoma of salivary gland. J Am Acad Dermatol 2003;48(suppl 5):S64–6

19PA Winkler , B Guyuron . Multiple metastases from basal cell naevus syndrome. Br J Plast Surg 1987;40:528–31

22SG Honavar , JA Shields , CL Shields , RC Eagle Jr, H Demirci , EZ Mahmood . Basal cell carcinoma of the eyelid associated with Gorlin-Goltz syndrome. Ophthalmology 2001;108:1115–23

23KA Behranwala , K Chettiar , M El-Bahrawy , G Stamp , AK Kakker . Retroperitoneal myolipoma. World J Surg Oncol 2005;3:72

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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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