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Case Reports
. 2018 May 3:10:2036361318772938.
doi: 10.1177/2036361318772938. eCollection 2018.

Giant morphea-form basal cell carcinoma of the umbilicus: Successful debulking with vismodegib

Mariana Orduz Robledo  1 , Eve Lebas  1 , Marie-Annick Reginster  2 , Mahmoud Baghaie  3 , Sabine Groves  4 , Arjen F Nikkels  1   5   6
Affiliations
Case Reports

Giant morphea-form basal cell carcinoma of the umbilicus: Successful debulking with vismodegib

Mariana Orduz Robledo et al. Rare Tumors. .

Abstract

Basal cell carcinoma of the umbilicus is very rare. The nodular subtype is the main representative. Giant basal cell carcinomas represent around 1% of all basal cell carcinomas. The hedgehog pathway inhibitor vismodegib is indicated for advanced basal cell carcinoma and CD56-negative immunostaining seems indicative for successful treatment. A 54-year-old man presented a 10 cm × 14 cm large and 4.5 cm deep morphea-form basal cell carcinoma with faint immunohistochemical CD56 expression arising from the umbilicus. A sequential treatment was initiated with debulking using vismodegib 150 mg per day for 4 months, followed by reconstructive surgery. To the best of our knowledge, this is the first report of a giant basal cell carcinoma of the morphea-form type of the umbilicus. The sequential treatment plan reduces the duration of vismodegib inherent adverse effects and significantly reduces the tumor mass prior to surgery. Besides increasing adherence to vismodegib treatment, this approach facilitates the surgical technique and improves cosmetic outcome.

Keywords: Morphea-form basal cell carcinoma; neuroendocrine differentiation; umbilicus; vismodegib.

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Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Clinical evolution of the giant morphea-form BCC after: (a) 1 day, (b) 30 days, (c) 60 days and (d) 120 days.
Figure 2.
Figure 2.
(a) Histopathologic suspicion of squamous cell carcinoma on the initial 4-mm punch biopsy, (b) Deep infiltrating morphea-form BCC on the excisionnal biopsy.
Figure 3.
Figure 3.
(a) H/E staining illustrating the sclerodermiform BCC, (b) faint CD56 immunostaining, and (c) strong immunohistochemical BerEp4 expression.
Figure 4.
Figure 4.
MRI illustrating the tumoral invasion until the abdominal fascia, (a) transversal view, (b) sagittal view.
Figure 5.
Figure 5.
Histologic aspect of the skin after 4 months of vismodegib therapy, showing cicatricial tissue without residual tumor tissue.
See this image and copyright information in PMC

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