Introduction

Cylindromas (also referred to as turban tumours in Brooke-Spiegler syndrome) are exceptionally rare, benign tumours of the skin. Only isolated cases have been described in the medical literature to date [1]. Histologically, they are classified as neoplasms arising from sweat glands, most frequently located in the head and neck region [1]. The condition is more commonly observed in women [2]. Solitary cylindromas occur sporadically and are generally not inherited [3]. These lesions typically affect middle-aged and elderly individuals [1]. The occurrence of multiple cylindromas is often associated with Brooke-Spiegler syndrome, an autosomal dominant inherited condition [4]. Malignant transformation into adenoid cystic carcinoma has been rarely reported [1]. The mainstay of treatment remains complete surgical excision of the lesion [1]. This article presents the case of a patient with a tumour demonstrating morphological features of both cylindroma and/or spiradenoma (cylindrospiradenoma).

Case report

We present the case of a 69-year-old Caucasian woman with a tumour located in the left preauricular region, who was referred to the Department of Otolaryngology. Physical examination revealed a lesion in the left preauricular area measuring 5.6 × 3.8 × 2.5 cm. The tumour had a pink-red coloration with numerous dilated blood vessels visible on its surface. The lesion was painless and exhibited slow growth; however, due to its size and location, it significantly interfered with the patient’s daily functioning.

The tumour was surgically excised via an incision made in the tragal part of the left auricle. During dissection, multiple vascular malformations were encountered, necessitating haemostasis of profuse bleeding.

Histopathological examination revealed a skin fragment containing a well-circumscribed subepidermal tumour composed of numerous nests of two distinct cell populations – smaller basaloid cells and larger, paler cells. No salivary gland tissue was identified in the specimen, supporting the cutaneous origin of the lesion.

The histomorphological features were consistent with a benign adnexal neoplasm exhibiting characteristics of a cylindroma or a tumour with mixed features of cylindroma and eccrine spiradenoma (cylindrospiradenoma).

Following surgical excision and histopathological confirmation, the patient was scheduled for regular otolaryngological follow-up every 6 months, including ultrasound monitoring (Figures 1, 2).

Discussion

Cylindromas can present in three distinct forms: as cutaneous lesions (as in the case presented), as tumours arising in the salivary glands – which may exhibit malignant behaviour – and as multiple lesions associated with genetic syndromes such as Brooke-Spiegler syndrome [5].

A review of the literature confirms that only a limited number of cylindroma cases have been documented to date [1], particularly those involving lesions of considerable size observed in this report. Malignant transformation into cylindrocarcinoma is extremely rare and has been infrequently described in the literature [6].

There are also reports of cylindromas occurring in atypical and rare anatomical locations such as the breast [7] or the external auditory canal [8].

Cylindromas are typically asymptomatic, although pain or paraesthesia may occur in certain cases due to nerve compression [1]. As described in the literature, solitary lesions are generally well-demarcated, firm, rubbery nodules that range in colour from red to pink or bluish, with diameters varying from a few millimetres to several centimetres. In the multiple form, numerous nodules of similar appearance are present [1]. The most frequent site for solitary cylindromas is the head and neck region, as observed in the case described. In multiple forms, the lesions also predominantly affect the head and neck, but may extend to involve the trunk, extremities, and genital region [9]. Clinically, they may mimic malignant neoplasms [10].

Cutaneous cylindromas are associated with a relatively high recurrence rate [1]. Therefore, complete surgical excision and consistent long-term follow-up are essential.

The case presented herein is of particular interest due to the rarity of the tumour type and the substantial/large size of the lesion. Early referral for surgical treatment is critical, particularly when the tumour is located in anatomically complex areas or has reached considerable/large size, as incomplete resection is associated with a higher risk of recurrence. In such cases, close and regular follow-up is imperative.

Funding

No external funding.

Ethical approval

Not applicable.

Conflict of interest

The authors declare no conflict of interest.

References