Sclerodermiform basal cell carcinoma

Synonym (s)

Keloid basal cell carcinoma, fibrosing basal cell carcinoma, morpheaant basal cell carcinoma, morpheiform basal cell carcinoma, sclerosing basal cell carcinoma.

Definition

Clinically "scarring" aspect, with a more pronounced therapeutic approach usually recommended.

Clinical features

Atrophic, slightly raised, scarred, partly keloid, rarely ulcerating, yellowish, waxy or ivory-coloured, indistinctly demarcated, dermal plaque with telangiectasia. One sees an increased tendency to infiltrate deep structures.

Diagnosis

Anamnesis
Clinic
Biopsy

Localization

Particularly the nose, forehead or cheeks.

Dermatopathology

A connective tissue, fibromatous reaction surrounds the tumour cell clusters. Unlike other forms of BCC, the typical palisading is mostly absent.

Prognosis

↑ Risk of recurrence

Therapy

Therapy of 1st choice according to Lebwohl	Evidence level
Curettage and electrodesiccation	B
Surgical excision	B
Moh's surgery (micrographically controlled)	B
Cryotherapy	B

Therapy of the 2nd choice after Lebwohl
Radiation therapy	B

Therapy of the 3rd choice according to Lebwohl
Interferon intralesional	B
Retinoids	D
Imiquimod	A
Photodynamic therapy	A
5-Fluorouracil	A
CO² laser	D
PEG-interleukin 2	D
NSAIDs	D
Ingenol mebutate	D
Vismodegib	A
Intralesional interleukin application	D
Systemic chemotherapy	D

Operative therapy

Excision

	BCC Ø < 2 cm		Infiltrative BCC
Safety distance	3 mm	4-5 mm	3 mm	5 mm	13-15 mm
Likelihood of tumour remnants remaining	15%	5%	34%	18%	5%

Source: Hauschild, A. (2016). Long version of the guideline "Basal cell carcinoma of the skin". Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/032-021.html

Curettage with/without electrodesiccation

Experimental therapies

Intralesional application of Dz13, a DNAzyme that targets JUN mRNA
Itraconazole has successfully disrupted the hedgehog signalling pathway in single cases.

Hedgehog inhibitor (vismodegib)
- In metastatic basal cell carcinoma, its use is indicated

Radiation therapy
- If surgical (re)excision is not possible
- Contraindicated: in Gorlin syndrome
- Radiation therapy alone: 84-96% remission
- Single doses between 2.0 and 3.0 Gy performed
- Total doses of 60 to 70 Gy
- Patients with basal cell carcinoma syndrome (Gorlin-Goltz) should not be treated with ionising radiation
- Cure rate: approx. 80-84% (for superficial basal cell carcinoma)
5-Fluorouracil (5% in cream), applied topically daily for four to six weeks
- Disadvantage: no control whether basal cell carcinoma has been completely removed
- Cure rate: approx. 81% (for superficial basaliomas)
Cure rates: 72-100%
- Imiquimod cream 5% daily 5x/week for a total of 6 weeks.

- Useful in superficial and in Gorlin syndrome.
Especially for superficial basal cell carcinoma.
Aesthetic results are worse.
Cure rates: 4-17%
- Laser therapy

- Photodynamic therapy
Recurrence rate: 3-19%
- Cryotherapy
The gold standard of basal cell carcinoma therapy
- Especially infiltrative growing and sclerodermiform basal cell carcinomas should be surgically excised.

- In the case of incomplete resection, re-excision should be performed except in well justified cases.

- Cure rate depends on tumour diameter, histological type and safety margin.

References

Clark CM, Furniss M, Mackay-Wiggan JM. Basal cell carcinoma: an evidence-based treatment update. Am J Clin Dermatol 2014;15:197-216.
Kwasniak LA, Garcia-Zuazaga J. Basal cell carcinoma: evidence-based medicine and review of treatment modalities. Int J Dermatol 2011;50:645-58.
Hauschild, A. (2016). Langfassung der Leitlinie "Basalzellkarzinom der Haut". Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/032-021.html