Basal cell carcinoma resection in an Ecuadorian patient with Gorlin-Goltz syndrome
Andrea Villarreal-Juris1; Diana Galarza-Estrella2; Bairon Sabando-Farías1; Gabriela Castillo-Andrade1; Jimmy Casares-Tamayo3
1Department of General Surgery, Luis Vernaza Hospital, Guayaquil-Ecuador
2Pontifical Catholic University of Ecuador, Quito-Ecuador
3Department of General Surgery, Metropolitan Hospital, Quito-Ecuador
*Andrea Villarreal-Juris, Department of General Surgery, Luis Vernaza Hospital, Guayaquil-Ecuador
Figure 1: Facial skin lesions and previous left maxillary keratocyst
Physical examination revealed multiple surgical scars on the left upper eyelid with tumoral activity on the eyelid margin, as well as on the outer third of the ipsilateral lower eyelid and on the right side.
Figure 2: Surgical scars with tumoral activity in the left and right eyelids.
In October, a wide resection of the left upper and lower eyelid was planned, plus reconstruction and transoperative study, which are performed without complications.
Figure 3 : Surgical planning
Figure 4 : Tumor resection involving the upper and lower eyelids with safety margins and surgical piece
Figure 5 : Confection of the left fronto-parietal flap
Figure 6: Immediate reconstruction results
Subsequently, the patient attended scheduled control, reporting the presence of a left superciliary nodular lesion. Physical examination revealed multiple lesions located in the left superciliary region, left helix, concha, and antihelix, in the inner corner of the left lower eyelid, in the left parieto-temporal and occipito-temporal regions, and other small bilateral genian lesions. There were no alterations in the flap. Left external campimetry was limited. It was decided to perform a facial bone and skull simple and contrasted tomography (CT) and laboratory tests.
Then, resection of the previously mentioned lesions and resection of the lower eyelid with transoperative study, shield-type incision and external canthoplasty of the lower left eyelid was planned. A plastic surgeon was included in the surgical team.
Figure 7: Shield-type incision
Altogether, 9 skin lesions located in the left superciliary, left frontal, interparietooccipital, posterior occipital, auricular, and left retroauricular regions were resected, which were positive for basal cell carcinoma.
In the subsequent control, the patient's campimetry showed improvement.
Figure 8 : Final result
- Holíková Z, Massi D, Lotti T, Hercogová J. Insight into the pathogenesis of sporadic basal cell carcinoma. Int J Dermatol. 2004 Dec;43(12):865–9.
- Thomas N, Vinod Vs, George A, Varghese A. Gorlin–Goltz syndrome: An often missed diagnosis. Ann Maxillofac Surg. 2016;6(1):120.
- Bartoš V, Kullová M, Adamicová K, Paucinová I. Gorlin-goltz syndrome. Klin Onkol. 2019;32(2):124–8.
- Hahn H, Wicking C, Zaphiropoulos PG, Gailani MR, Shanley S, Chidambaram A, et al. Mutations of the human homolog of drosophila patched in the nevoid basal cell carcinoma syndrome. Cell. 1996 Jun 14;85(6):841–51.
- Yamamoto K, Yoshihashi H, Furuya N, Adachi M, Ito S, Tanaka Y, et al. Further delineation of 9q22 deletion syndrome associated with basal cell nevus (Gorlin) syndrome: Report of two cases and review of the literature. Congenit Anom (Kyoto). 2009 Mar;49(1):8–14.
- Kumar NN, Padmashree S, Jyotsna T, Shastry S. Gorlin-Goltz syndrome: A rare case report. Contemp Clin Dent. 2018 Jul 1;9(3):478–83.
- Binkley GW, Johnson HH. Epithelioma adenoides cysticum: Basal cell nevi, agenesis of the corpus callosum and dental cysts: A clinical and autopsy study. A M A Arch Dermatology Syphilol. 1951;63(1):73–84.
- Gorlin RJ, Goltz RW. Multiple Nevoid Basal-Cell Epithelioma, Jaw Cysts and Bifid Rib. N Engl J Med. 1960 May 5;262(18):908–12.
- Rayner CRW, Towers JF, Wilson JSP. What is Gorlin’s syndrome? The diagnosis and management of the basal cell naevus syndrome, based on a study of thirtyseven patients. Br J Plast Surg. 1977 Jan 1;30(1):62–7.
- Kimonis VE, Singh KE, Zhong R, Pastakia B, Digiovanna JJ, Bale SJ. Clinical and radiological features in young individuals with nevoid basal cell carcinoma syndrome. Genet Med. 2013 Jan;15(1):79–83.
- Bresler SC, Padwa BL, Granter SR. Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome). Head Neck Pathol. 2016 Jun 1;10(2):119–24.
- Evans DGR, Farndon PA, Burnell LD, Rao Gattamaneni H, Birch JM. The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer. 1991;64(4):959–61.
- Coffin CM. Case 1 congenital cardiac fibroma associated with gorlin syndrome. Fetal Pediatr Pathol. 1992;12(2):255–62.
- Moramarco A, Himmelblau E, Miraglia E, Mallone F, Roberti V, Franzone F, et al. Ocular manifestations in Gorlin-Goltz syndrome. Orphanet J Rare Dis. 2019 Sep 18;14(1).
- Jensen MF, Roser SM. Gorlin’s syndrome with ameloblastoma. Oral Surgery, Oral Med Oral Pathol. 1978;45(2):325–6.
- Schultz SM, Twickler DM, Wheeler DE, Hogan TD. Ameloblastoma associated with basal cell nevus (Gorlin) syndrome: CT findings. J Comput Assist Tomogr.
- Gupta SR, Jaetli V, Mohanty S, Sharma R, Gupta A. Nevoid basal cell carcinoma syndrome in Indian patients: A clinical and radiological study of 6 cases and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;113(1):99– 110.
- MacDonald-Jankowski DS. Keratocystic odontogenic tumour: Systematic review. Vol. 40, Dentomaxillofacial Radiology. Dentomaxillofac Radiol; 2011. p. 1–23.
- Baliga SD, Rao SS. Nevoid-basal cell carcinoma syndrome: a case report and overview on diagnosis and management. J Maxillofac Oral Surg. 2010 Mar 1;9(1):82–6.
- Hasan A, Akintola D. An Update of Gorlin-Goltz Syndrome. Prim Dent J. 2018 Sep 1;7(3):38–41.
- Kleinerman RA. Radiation-sensitive genetically susceptible pediatric subpopulations. In: Pediatric Radiology. Pediatr Radiol; 2009.
- Ramesh M, Krishnan R, Chalakkal P, Paul G. Goltz-Gorlin Syndrome: Case report and literature review. J Oral Maxillofac Pathol. 2015 May 1;19(2):267.
- Palacios-Álvarez I, González-Sarmiento R, Fernández-López E. Gorlin Syndrome. Vol. 109, Actas Dermo-Sifiliograficas. Elsevier Doyma; 2018. p. 207– 17.
- Longo B, Nicolotti M, Ferri G, Belli E, Santanelli F. Sagittal split osteotomy of the fibula for modeling the new mandibular angle. J Craniofac Surg. 2013 Jan;24(1):71–4.
- Buonaccorsi S, Terenzi V, Pellacchia V, Indrizzi E, Fini G. Reconstruction of an acquired subtotal ear defect with autogenous septal cartilage graft. Plast Reconstr Surg. 2007 May;119(6):1960–1.