An Unusual Case of Sturge-Weber Syndrome with Squamous Cell Carcinoma of Tongue
Sturge-Weber Syndrome (SWS) is a rare congenital neurocutaneous syndrome affecting skin and soft tissues in the distribution of cranial nerve V, the ipsilateral cerebral hemisphere, and eye, with an incidence of 1 in 20,000 to 50,000 births, with no overall predisposition to developing cancers. Ther...
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| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
JCDR Research and Publications Private Limited
2025-07-01
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| Series: | Journal of Clinical and Diagnostic Research |
| Subjects: | |
| Online Access: | https://jcdr.net/article_fulltext.asp?issn=0973-709x&year=2025&month=July&volume=19&issue=7&page=XD01-XD03&id=21225 |
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| Summary: | Sturge-Weber Syndrome (SWS) is a rare congenital neurocutaneous syndrome affecting skin and soft tissues in the distribution of cranial nerve V, the ipsilateral cerebral hemisphere, and eye, with an incidence of 1 in 20,000 to 50,000 births, with no overall predisposition to developing cancers. There is a dearth of literature for the use of microvascular free flaps in SWS, and none pertaining to its use in oncological surgery for these patients, to the best of our knowledge. Free flaps have been used to reconstruct port wine stains in such patients. Hereby, authors present a case of a 52-year-old male having SWS who developed a Squamous Cell Carcinoma (SCC) of the tongue and was managed surgically, with the reconstruction being done with a free Radial Artery Forearm Flap (RAFF). The patient complained of ulcer over tongue since three months. He was already diagnosed case of SWS. Patient’s extraoral examination also revealed large hyperpigmented pinkish-brown patch with multiple nodules over the left half of his forehead, extending from hairline till the upper eyelid. The histopathology report showed a moderately differentiated SCC tumour with a maximum dimension of 3.2 cm, for which patient also received adjuvant radiotherapy. This case report demonstrates the safe use of free flaps in patients with head and neck cancers who have a history of SWS. |
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| ISSN: | 2249-782X 0973-709X |