A 60 year old female presented with a mass on the right side of the face, since 20 years. The mass was painless and gradually increasing in size. There was no history of trauma prior to the appearance of lesions. There was no history of any discharge from the mass. On examination, there was a single soft sessile nodule of size 3 × 2 cm on the right cheek [Figure 1]. The nodule was mobile over the underlying bone. There was no evidence of discharge, ulceration or cervical lymphadenopathy. There were no other lesions on the scalp and other parts of the body.
Figure 1.
A solitary soft nodule
Punch biopsy and histopathological examination of the lesion was performed.
Microscopic examination revealed multiple basophilic tumour islands packed together in the dermis [Figure 2]. Higher magnification revealed masses of epithelial cells which are surrounded by the hyaline sheath. Island of cells showed dark nuclei at the periphery and pale nuclei at the centre [Figure 3].
Figure 2.
Basophilic tumour islands in the dermis
Figure 3.
Masses of epithelial cells showing dark nuclei at the periphery and pale nuclei at the centre with surrounding hyaline sheath
Question
What is the diagnosis?
Answer
Cylindroma.
Discussion
Cylindromas are rare, benign adnexal tumours that classically arise on the scalp, face and neck. They appear as smooth dome shaped nodules. Cylindromas can occur as multiple lesions or solitary lesions. Solitary cylindromas occur more commonly than multiple ones. Multiple cylindromas can occur as a part of a dominantly inherited condition, the Brooke-Spiegler syndrome and are associated with other adnexal tumours like trichoepithelioma and spiradenoma. Multiple tumours are more common on the scalp while solitary cylindroma is common on the face. Tumours on the scalp can be so large in number that they may cover the entire scalp like a turban, thus earning the name of turban tumour.[1] Cylindroma shows female preponderance and female to male ratios of 6:1 and 9:1 have been reported. Solitary cylindromas affect the face and scalp of older adults and usually are not inherited.
Histologically, a cylindroma shows multiple tumour islands of various size and shape lying closely to fit together, giving the appearance of a jigsaw puzzle. These tumour islands consist of two types of cells. Cells with dark nuclei are seen at the periphery while cells with lightly stained nuclei are seen in the centre. Often, thick hyaline eosinophilic, periodic acid-Schiff stain (PAS) positive membrane can be seen around the tumor islands. This hyaline membrane is diastase resistance and immunoreactive to collagen IV. Eosinophilic hyaline material can be seen in the form of globules within the tumor islands. A variable number of tubules can also be seen within the tumor islands. Cylindromas may show apocrine or eccrine differentiation, of which apocrine is commonly seen.[2] Cylindromas need to be surgically excised. Cylindromas rarely undergo malignant degeneration.[3,4]
Footnotes
Source of support: Nil
Conflict of Interest: Nil.
References
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