To describe the case of successful radiotherapeutic treatment of a woman suffering from Brooke-Spiegler syndrome who had multiple disfiguring cylindromas on the entire scalp and further tumors on the trunk.
After decades of treatment with conventional therapies including surgery and topically applied salicylic acid, the 73-year-old woman agreed to undergo radiotherapeutic treatment. She received 60 Gy to the scalp and 36 Gy to painful nodules in the lumbar spine region.
Over a follow-up period of 14 and 11 years, respectively, the scalp nodules almost completely regressed, while the lumbar nodules became painless and considerably smaller. Apart from alopecia, no late adverse effects of treatment remain.
This case should remind us of the potential role that radiotherapy could play in treating Brooke-Spiegler syndrome. The required dose for treatment of such extensive disease is still a matter of debate due to the scarcity of radiotherapeutic experience. This case demonstrates that for scalp tumors, 30 × 2 Gy can result in long-term tumor control, while other dose prescriptions may be adequate for tumors in other locations.

Cylindromas are rare tumors of adnexal structures in adults. They are predominantly found on the scalp and face. More impressive than incidentally diagnosed solitary cylindromas are multiple tumors in patients with familial Brooke-Spiegler syndrome. If many large cylindromas appear on the head, they are termed turban tumor. Sudden growth or ulceration should raise suspicion for malignant transformation.

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Dermal cylindroma is a benign neoplasm of the eccrine sweat glands, which presents in the head and neck area in majority of cases. In its most florid presentation, the entire scalp can be involved, leading to the descriptive label turban tumor. They most commonly occur on the head and neck as solitary or multiple tumors. Solitary cylindromas occur sporadically and typically are not inherited. Multiple tumors are observed in an autosomal dominantly inherited manner. When nodules enlarge and coalesce on the scalp, they form the distinctive turban tumor feature. We present a case of multiple form of dermal cylindroma, occupying the anterior scalp in a 38-year-old male patient.

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