ANTICANCER RESEARCH 30: 1793-1798 (2010)

Dermal Cylindroma of the Scalp (Turban Tumour) and Subjacent Calvarian Defects

REINHARD E. FRIEDRICH

Department of Oral and Maxillofacial Surgery, Eppendorf University Hospital, University of Hamburg, Germany

Abstract. This case report describes the diagnosis and Case Report therapy of a patient with a 40-year history of multiple potato-like tumours growing in the head and neck region. Patient. A seventy-five-year-old woman was referred to the The tumour proved to be a cylindroma, associated with Department of Oral and Maxillofacial Surgery from the calvarian defects. Further facial tumours were diagnosed as Department of Dermatology, Eppendorf University Hospital, trichoepitheliomas. This association of findings was for treatment of an extensive cylindroma of the entire scalp. pathognomonic for Brooke-Spiegler’s syndrome. Complete The patient first sought for medical advice in the University work-up of the resection specimen excluded any malignant Hospital 4 years previously but had refused any surgical transformation of the tumour. A long lasting history of treatment (Figure 1). On presentation, the patient was in cylindroma and evidence for bone destruction, not associated generally good condition. The primary reason the patient with a malignant transformation, is extremely rare in decided to seek surgical treatment was an ill-fit of her wig Brooke-Spiegler’s syndrome. (Figure 2). The patient’s medical history showed an early onset of the enlargement of the skin of her skull and the development Cylindroma is a slow growing, benign tumour of the skin (1), of potato-like tumours about 40 years earlier. At the same time, affecting preferentially the head and neck region, especially she noticed a growing baldness in the area of tumour growth the capillitium and forehead (2, 3). The term “turban tumour” and started wearing a wig. The patient could not provide is used to describe the topography of the tumour and its information about her family history because she and her sister pathognomonic size (4-10). A turban tumour can grow to an had been adopted as very small children and never knew their enormous size (covering the whole scalp) and cause severe parents. She had lost all contact with her sister several decades disfigurement. However, other regions of the body can also previously. Thus, the possibility of hereditary or sporadic be affected by cylindromas (11, 12), which may also occur tumour development could not be established. exclusively outside of the head and neck area (1, 13). To date, At the time of attendance, the tumour had grown circular there has been no report on bone destruction associated with around the capillitium, infiltrated both auricles, and further the occurrence of a turban tumour. Indeed, it is generally tumours of the head and neck area had grown on her chin and accepted that bone defects in a cylindroma are signs of neck. On her face, especially at the nasolabial folds, multiple malignant transformation (14, 15). This case report describes small tumours were seen resembling trichoepitheliomas. A the investigation and treatment of one patient with a total body examination revealed further trunk skin tumours. histologically proven cylindroma of the scalp and ears (16), who had developed multiple defects of the neurocranium Investigations. Computed tomography and magnetic subjacent to the tumour. Long-term follow-up did not show resonance imaging depicted a large extracranial tumour with any sign of malignant transformation, but only small diversely multiple septae, partially cystic or solid, with a maximum localised recurrences of the cylindroma in the head and neck. thickness of 5 cm (Figure 3). Defects of the cortical bone were identified bilaterally in the parietal region. These defects were associated with a honeycomb-like cystic arrosion of the adjacent calvaria (Figure 4). On the right side, the bone- Correspondence to: Professor Dr. R. E. Friedrich, Oral and destroying tumour was close to the dura (Figure 5). Maxillofacial Surgery, Eppendorf University Hospital, Martinist. 52, Angiography of the common and external carotid arteries D-20246 Hamburg, Germany. Tel.: +49 40428033259, Fax: +40 revealed numerous wavy-lined and elongated arteries inside 40428038120, e-mail: [email protected] the tumour (Figure 5). The occipital and the superficial Key Words: Dermal cylindroma, skull defects, Brooke-Spiegler temporal arteries were judged to be the nutrient vessels of the syndrome. tumour. The internal carotid artery did not supply the tumour.

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Figure 1. Right lateral aspect of the patient on her first attendance at the age of 71 years. Figure 3. Coronal magnetic resonance imaging of the head. Large cystic areas are revealed inside the turban tumour. In the area of bone destruction the tumour cannot be differentiated clearly from the brain.

Figure 2. Right lateral aspect of the patient on at the age of 75 years. Note the enlargement of the tumour masses and the displacement of the right pinna due to the pressure of the tumour.

Therapy. The scalp was completely excised under general anaesthesia so that the tumour could be resected. The resection slices were 28 cm × 18 cm × 6 cm and 30 cm × 20 cm × 6 cm, respectively. The maximum diameter of single nodules was 8 cm. In this first operation, the bone was left intact in order to gain histological information of the tumour Figure 4. The computed tomography of the skull reveals a sharply biology prior to intracranial procedures. The defect was demarcated osseous defect of the right parieto-occipital region.

1794 Friedrich: Dermal Cylindroma of the Scalp

Figure 5. Angiographic radiogram depicting the feeding arteries of the tumour. The arteries originate from branches of the external carotid artery.

Figure 7. Biopsy of the resection specimen. The adenomateous tumour is segregated by thick septae, typical of a cylindroma. Hematoxylin-eosin, primary magnification ×120.

in maximum diameter. The dura was not invaded by the tumour and remained uninjured during the surgical manipulations. Multiple split-skin grafts were harvested to cover the calvarium completely. Healing of the wounds and recovery of the patient were both uneventful (Figure 6). The same differentiation of cylindroma was found in the bony Figure 6. Postoperative lateral aspect of the patient. Note the parieto- defects as in the large resection specimens (Figure 7). The occipital calvarian defect. periosteum was not infiltrated by the tumour. The tumours of the pinna caused mechanical impairment of hearing. After re-epithelialisation of the scalp, a third operation was temporarily covered. Histopathological work-up of the whole performed to reduce the auricular cylindromas, especially to specimen revealed a cylindroma in all sites and excluded contour the auditory meatus. Additionally, the cylindromas malignant transformation. A second operation was carried of the chin and the nasolabial tumours were excised, the out to resect the residual tumour and the subjacent bone latter proving to be trichoepitheliomas. Thirty-six months defects. Two craniotomies were performed to resect the after initial surgery, the patient was doing well with no signs residual tumour. Both the intracavitary tumour and adjacent of local recurrence and only some solitary tumours bone were excorporated. The largest defect was about 3 cm developing in her neck.

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Discussion The pinna can be severely affected by cylindroma, causing further disfigurement and hearing impairment. A patient with a turban-like tumour was first reported by Surgical resection of the tumour masses with Ancell (17). The clinicopathological features of cylindroma reconfiguration of the auricle and outer meatus has were described in detail by Spiegler (3) and Brooke (5). The prompted acceptable results (7, 12). cylindroma of the skin arises from eccrine or apocrine glands To the Author’s best knowledge, there has been no prior (18). The tumour forms thick layers of collagen-rich report of a cylindroma of the head and neck region (turban extracellular matrix (11, 19, 20). tumour) where evidence was presented for multiple lesions The medical history of patients with cylindroma usually of the calvarium and definitive exclusion of malignant covers years or even decades of their life. Women are transformation proved during a follow-up period of 36 affected predominantly, with the male-to-female ratio months. ranging from 1:3 to 1:9 (3, 9). A cylindroma may occur in either solitary or multiple lesion form. The solitary type is Acknowledgements usually of sporadic origin, whereas the multiple-lesion type is often a predominantly inherited disease (12). These The expert histological diagnosis of the resection specimen provided tumours can vary in size from papules to large dome-shaped by Professor Dr. mult. K. Donath, Institute of Pathology, Eppendorf nodules. The capillitium is the most frequently affected site University Hospital, Hamburg, Germany, is kindly appreciated. in the head and neck area (7-9, 17). Other anatomical regions of the face and neck are occasionally covered by References cylindromas (16) and these tumours can also be found in the skin of the trunk, arms and legs (4, 21). The diameter of 1 Rubin MG and Mitchell AJ: Generalized cutaneous cylindromatosis. Cutis 33: 568-569, 1984. these tumours rarely exceeds 4-6 cm (22). 2 De Backer A, Vanden Houte K, Duinslaeger L and Wylock P: Brooke-Fordyce trichoepitheliomas are frequently found Familial dermal eccrine cylindroma (turban tumor) - a report of in patients with dermal cylindromas (5). The term “Brooke- two cases with emphasis on the surgical approach. Eur J Plast Spiegler” syndrome reflects this close association of Surg 9: 113-116, 1986. cylindroma and trichoepithelioma as pathognomonic features 3 Spiegler E: Ueber Endotheliome der Haut. Arch Dermatol Syph of this syndrome. Further studies revealed the evidence of a (Berlin) 50: 163-176, 1899. single genetic entity for these phenotypes (23), which was 4 Batstone JHF: Turban tumour. Br J Surg 54: 567-569, 1967 5 Brooke HG: Epithelioma adenoides cysticum. Br J Dermatol 4: recently linked to having a molecular basis (24, 25). In the 269-287, 1892. current case, there was no information regarding family 6 Blandy LJ, Gammie WFP and Stovin PGI: Turban tumours in history (24); however, there was evidence of brother and sister. Br J Surg 49: 136-146, 1961. trichoepitheliomas of the nasolabial fold (12). Therefore, the 7 Fabian RL and Shugar MA: Florid dermal cylindroma (turban reported case fulfilled the main diagnostic criteria of a tumor). Head Neck Surg 4: 165-169, 1981. Brooke-Spiegler-syndrome. 8 Given K, Pickrelli K and Smith D: Dermal cylindroma (turban The macroscopic differential diagnosis of a cylindroma tumor). Plast Reconstr Surg 59: 582-587, 1977. 9 Harper PS: Turban tumors (Cylindromatosis). In: Bergsma D includes a variety of skin tumours, such as trichoepithelioma, (ed.). The Third Conference on the Clinical Delineation of Birth tricholemmoma, basal cell carcinoma, angiolymphoid Defects, part XII: Skin, Hair and Nails. Baltimore, Williams and hyperplasia (26) or neurofibromatosis (1, 27). The tentative Wilkins, pp. 338-341, 1971. diagnosis of this benign tumour, based on physical 10 Alawi MH, Hobby JAE and Lesna M: Familial dermal examination, may not be hampered in cases with an cylindroma with involvement of the parotid gland. Br J Plast excessive growth resembling a turban. However, malignant Surg 35: 167-170, 1982. transformation of a cylindroma has to be considered (28). 11 van Balkom IDC and Hennekam RCM: Dermal eccrine cylindromatosis. J Med Genet 31: 321-324, 1994. Thus, thorough investigation of patients with turban tumour 12 Burrows NR, Russell Jones R and Smith NP: The is imperative. The penetration of bone by local extension in clinicopathological features of familial cylindromas and a case of cylindroma contains a reference to a malignant trichoepitheliomas (Brooke-Spiegler syndrome): a report of two transformation (14). families. Clin Exp Dermatol 17: 332-336, 1992. The extension of the tumour is best depicted with 13 Grimmer H: Erkrankungen des äußeren weiblichen Genitale. magnetic resonance or computed tomography. Angiography Adenextumoren. Zylindrom (Spiegler´scher Tumor). Z Haut may disclose hypertrophied branches of the external carotid Geschl Krankh 46: 123-130, 1971 14 Lyon JB and Rouillard LM: Malignant degeneration of turban artery supplying the turban tumour (7). tumor of the scalp. Trans St Johns Hosp Dermatol Soc 46: 74- Treatment consists of surgical excision (7, 29-33). In 77, 1961. extensive lesions, resurfacing with split skin-grafts is the 15 Luger A: Das Zylindrom der Haut und seine maligne method of choice for covering the defects (4, 7, 29-31). Degeneration. Arch Dermatol Syph (Berl) 188: 155-180, 1949.

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16 Wolf BA, Gluckman JL and Wirman JA: Benign dermal 26 Vázquez Botet M and Sánchez JL: Angiolymphoid hyperplasia cylindroma of the external auditory canal: a clinicopathological with eosinophilia: report of a case and review of the literature. report. Am J Otolaryngol 6: 35-38, 1985. J Dermatol Surg Oncol 4: 931-933, 1978. 17 Ancell H: History of a remarkable case of tumours, developed 27 Baden HP: Cylindromas simulating neurofibromatosis. N Engl on the head and face, accompanied with a similar disease in the J Med 267: 296-297, 1962. abdomen. Med Chir Trans R Med Chir Soc Lond 25: 227-247, 28 Lin PY, Fatteh SM and Lloyd KM: Malignant transformation in 1842. a solitary dermal cylindroma. Arch Pathol Lab Med 111: 765- 18 Al-Qattan MM: Turban tumour: eccrine or apocrine in origin 767, 1987. (letter). Br J Plast Surg 44: 318, 1991. 29 Freedman AM and Woods JE: Total scalp excision and auricular 19 Bruckner-Tuderman L, Pfaltz M and Schnyder UW: Cylindroma resurfacing for dermal cylindroma (turban tumor). Ann Plast overexpresses collagen VII, the major anchoring fibril protein. Surg 22: 50-57, 1989. J Invest Dermatol 96: 729-734, 1989. 30 Goldman B: Total excision of the scalp and portions of the face; 20 Pfaltz M, Bruckner-Tuderman L and Schnyder UW: Type VII restoration by skin grafting: the surgical management of massive collagen is a component of cylindroma basement membrane cylindroma of the scalp and face. Ann Surg 133: 555-560, 1951. zone. J Cutan Pathol 16: 388-395, 1989. 31 Irwin LR, Bainbridge LC, Reid CA, Piggot TA and Brown HG: 21 Guzzo C and Johnson B: Unusual abdominal location of a Dermal eccrine cylindroma (turban tumor). Br J Plast Surg 43: dermal cylindroma. Cutis 56: 239-240, 1995. 702-705, 1990. 22 Korting GW and Denk R (Hrsg.): Dermatologische 32 Sherman JE, Hoffman S and Goulian D: Dermal cylindroma: Differentialdiagnose. Schattauer, Stuttgart, pp. 667-668, 1974. surgical approach. Plast Reconstr Surg 68: 596-602, 1981. 23 Welch JP, Wells RS and Kerr CB: Ancell-Spiegler cylindromas 33 Sharma HS, Meorkamal MZ, Zainol DCP and Dharap MS: (turban tumors) and Brooke-Fordyce trichoepitheliomas: Eccrine cylindroma of the ear canal - report of a case. J Laryngol evidence for a single gene entity. J Med Genet 5: 29-35, 1968. Otol 108: 706-709, 1994. 24 Biggs PJ, Wooster R, Ford D, Chapman P, Mangion J, Quirk Y, Easton DF, Burn J and Stratton MR: Familial cylindromatosis (turban tumour syndrome) gene localised to chromosome 16q12- q13: evidence for its role as a tumour suppressor gene. Nat Genet 11: 441-443, 1995. 25 Biggs PJ, Chapman P, Lakhani SR, Burn J and Stratton MR: The cylindromatosis gene (CYLD1) on chromosome 16q may be the Received August 19, 2009 only tumour suppressor gene involved in the development of Revised April 19, 2010 cylindromas. Oncogene 12: 1375-1377, 1996. Accepted April 21, 2010

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