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Case Reports
. 2007 Dec 29;1(1):4-9.
doi: 10.3315/jdcr.2007.1.1002.

Cylindroma transforming into basal cell carcinoma in a patient with Brooke-Spiegler syndrome

Justyna Sicinska  1 , Adriana Rakowska, Joanna Czuwara-Ladykowska, Andrzej Mroz, Marcin Lipinski, Anna Nasierowska-Guttmejer, Jolanta Sikorska, Katarzyna Sklinda, Monika Slowinska, Elzbieta Kowalska-Oledzka, Irena Walecka, Jerzy Walecki, Lidia Rudnicka
Affiliations
Case Reports

Cylindroma transforming into basal cell carcinoma in a patient with Brooke-Spiegler syndrome

Justyna Sicinska et al. J Dermatol Case Rep. .

Abstract

Background: Brooke-Spiegler syndrome is a rare condition with a predisposition to develop cutaneous adnexal neoplasms, especially cylindromas, trichoepitheliomas and spiradenomas. Malignant transformation of cylindromas is rare. In such cases usually cylindrocarcinomas develop within these lesions. We present an unusual case of basal cell carcinoma developing within a preexisting cylindroma.

Main observations: 58-year-old woman with a 30-year history of multiple dermal cylindromas extensively involving her scalp was referred for dermatological treatment. The patient reported that one of the long-lasting lesions, 5.5 cm in size, ulcerated within the foregoing few weeks. Histopathology confirmed cylindromas and basal cell carcinoma within the ulcerating tumor. Surgical excision of largest cylindroma tumors led to cosmetic and functional improvement. Magnetic resonance and computed tomography showed tumor infiltration into the skull lamina externa. Metastases were excluded by chest radiography and abdominal ultrasound examination.

Conclusion: Patients with Brooke-Spiegler syndrome should be followed-up for malignant transformation of skin tumors to prevent deep penetration and possible metastases.

Keywords: basal cell carcinoma; computed tomography; cylindroma; magnetic resonance imaging; syringoma; trichoepithelioma; turban tumor.

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Figures

Figure 1
Figure 1
Multiple cylindromas of the scalp forming a turban tumor.
Figure 2
Figure 2
Basal cell carcinoma within a cylindroma (A). Dermoscopy of ulcer's edge shows arborizing vessels, which are characteristic for both, basal cell carcinoma and cylindroma (B).
Figure 3
Figure 3
Cylindromas of the neck area (A). Dermoscopy shows prominent arborizing vessels on pink background and numerous whitish ovoid areas (B).
Figure 4
Figure 4
Basal cell carcinoma of the right cheek area and multiple trichoepitheliomas in the nasolabial area (A). Dermoscopic view of two trichoepitheliomas (B).
Figure 5
Figure 5
Multiple cylindromas on patient's back (A). Dermoscopy shows homogenous pink nodules with enlarged irregular vessels at the periphery. (B)
Figure 6
Figure 6
Cylindroma (H&E). The pattern of cylindroma consists of dermal nests, lobules or cords of basaloid tumor cells (A). Dilated thin-wall blood vessels corresponding to telangiectasia clinically are present between the tumor mass and epidermis. Lobules are surrounded by a dense, intensely eosinophilic hyaline membrane (B). Similar eosinophilic material is present inside the nests admixed with tumor cells. The pattern formed by these almost interlocking nests has been compared to a jigsaw puzzle. Within the nests are two cell types: palisaded small cells with oval dark nuclei situated peripherally and large cells with prominent vesicular nuclei located intralobularly (C).
Figure 7
Figure 7
BCC (H&E, 200x) Ulcerated tumor with the presence of basal cell carcinoma features underneath the ulceration with accompanying inflammatory cells.
Figure 8
Figure 8
Computer tomography scan of the scalp. Deformation of lamina externa of right frontal bone.
Figure 9
Figure 9
Magnetic resonance imaging of the scalp. Blurring of lamina externa of both parietal bones.
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References

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