Cutaneous cylindroma: it's all about MYB

Abstract

Cutaneous cylindroma is a rare benign tumour that occasionally turns into malignant cylindrocarcinoma. The cancer can be sporadic or emerge in the context of Brooke-Spiegler syndrome (BSS), an inheritable condition characterized by mutation of the gene CYLD, encoding a tumour suppressor protein that controls the activity of the transcription factor NF-kB. Sporadic cylindromas present histological features shared with adenoid cystic carcinoma (ACC), a head and neck cancer originating from salivary or other exocrine glands. Like ACCs, sporadic cylindromas express, although at lower frequency, the aberrant fusion transcript MYB-NFIB. In a paper recently published in the Journal of Pathology, the research teams led by Neil Rajan and Goran Stenman demonstrate that CYLD-defective cyclindromas in BSS patients are negative for the MYB-NFIB fusion. Only the wild-type MYB oncoprotein is activated in the majority of these tumours. RNA interference studies in cells derived from BSS patients indicate that ablating MYB expression results in a striking reduction of cylindroma cell proliferation, suggesting that MYB plays a pivotal role in the biology of this cancer. The take-home message of the study is that activation of MYB, in its wild-type form or fusion derivatives, is a common feature of spontaneous and hereditary cylindromas, constituting a potentially actionable therapeutic target.

30 Years of NF-κB: A Blossoming of Relevance to Human Pathobiology

“…For example, CYLD deficiency may cause overexpression of the c-myb oncogene as a feature of cylindromas (Corda and Sala, 2016). However, in addition to NEMO, activated CYLD can remove the lysine 63-linked polyubiquitin chains of BCL-3 (an atypical IκB), thus preventing BCL-3 translocation into the nucleus together with p52 and p50, which may also promote cell proliferation.…”

Polymorphous sweat gland carcinoma found to have MYB rearrangement

“…In ACC, increased MYB expression is coordinated with TP63 expression in myoepithelial cells and Notch expression in luminal epithelial cells to orchestrate the characteristic cribriform histological pattern seen in ACC . MYB – NFIB fusions have been found in ACC and, to a lesser degree, in cylindroma, which shares histological features with ACC …”

Periodic acid Schiff's: A definitive stain in histopathological diagnosis of Cylindroma

“…1 As the author has rightly pointed out that survey done by NPCB 2 has taken the cutoff of vision <3/60 for screening purpose for criteria of blindness. Some more studies such as conducted by Murthy et al 3 and Kanagarajan et al 4 have taken the visual acuity <6/60 as screening criteria to see the prevalence of cataract. To see the actual prevalence of different eye diseases in these islands, a large well design population survey is required.…”

“…The tumor occurs sporadically or as part of the Brooke-Spiegler syndrome. 3 In both cases, there is activation of MYB in its wild type or as part of fusion derivatives. 3 Treatment involves wide local excision or use of electrocautery/CO 2 laser for smaller lesions.…”

“…3 In both cases, there is activation of MYB in its wild type or as part of fusion derivatives. 3 Treatment involves wide local excision or use of electrocautery/CO 2 laser for smaller lesions. 4 The histomorphology basically constitutes basaloid cells arranged in tubules and nests with hyaline membrane.…”