Basal cell carcinoma of the external auditory canal and Gorlin-Goltz syndrome: a case report

Lobo, C. J.; Timms, M. S.; Puranik, V. C.

doi:10.1017/s0022215100138782

Cited by 6 publications

(3 citation statements)

References 5 publications

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“…In the case by Lobo et al the patient presented with a BCC in the right external auditory canal, treated by a nearly circumferential excision up to the tympanic membrane. 7 Another patient with a BCC of the ear was discussed by Lasso et al The tumor originated from the temporal region and involved the middle ear, the intrapetrous part of the facial nerve and the dura mater. Resection required extensive use of local and free flaps.…”

Section: Discussionmentioning

confidence: 99%

Tonsillar Hypertrophy in Goltz-Gorlin Syndrome: Case Report and Literature Review

Lavrysen¹,

Loeys²,

Vanderveken³

et al. 2024

B-ENT

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Goltz-Gorlin syndrome, also known as focal dermal hypoplasia or nevoid basal cell carcinoma syndrome, is a rare multisystemic disease caused by autosomal dominant mutations in the PORCN gene. The characterizing features are keratocystic odontogenic tumors in the jaw, multiple basal cell carcinomas, calcification of the falx cerebri, palmar or plantar pits, and skeletal abnormalities. This paper reports the case of a 3-yearold girl with Goltz-Gorlin syndrome, showing progressively increasing tonsillar hypertrophy with obstructive effect on the oropharynx. The lymphatic tissue was remarkably papillomatous in aspect. Pathologic examination after tonsillectomy showed hyperplastic, morphologically normal tonsillar tissue, without arguments for papilloma, active inflammation, or malignancy. This paper highlights the rare manifestations of ENT pathologies in GGS which demand attention early on and long-term follow-up.

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Section: Discussionmentioning

confidence: 99%

Tonsillar Hypertrophy in Goltz-Gorlin Syndrome: Case Report and Literature Review

Lavrysen¹,

Loeys²,

Vanderveken³

et al. 2024

B-ENT

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“…Khalifa et al reported a case of endometrial adenocarcinoma associated with ovarian fibroma and Gorlin's syndrome. The external auditory meati may also be involved, 15,21 and orbital or tarsal BCCs may also extend into and/or obliterate the orbit and ethmoidal sinuses. 17 Yilmaz et al reported the concurrence of basal cell naevus syndrome and adenoid cystic carcinoma in a patient who also had distant metastases in the lungs, 18 a feature seen by other authors in this and other sites.…”

Section: Introductionmentioning

confidence: 99%

“…Computed tomography scans revealed a large, cystic mass in the left maxillary sinus, protruding into the nasal cavity. The external auditory meati may also be involved, 15 , 21 and orbital or tarsal BCCs may also extend into and/or obliterate the orbit and ethmoidal sinuses 8 , 22 . To date, no cases with involvement of the upper aerodigestive tract have been documented.…”

Section: Introductionmentioning

confidence: 99%