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Cylindroma of the scalp.

Mogaddam MR, Maleki N - J Res Med Sci (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Imam Khomeini Hospital, Ardabil University of Medical Sciences, Ardabil, Iran.

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The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern and separated by hyaline basement membrane material, which was suggestive of cylindroma [Figure 2]... They can be seen in conjunction with spiradenomas and trichoepitheliomas, and this condition is known as Brooke-Spiegler syndrome (BSS)... BSS is a rare autosomal dominant disorder caused by mutations of the cylindromatosis gene (CYLD) located at 16q12-q13... Histologically, the tumors are circumscribed, nonencapsulated dermal nodules composed of islands and cords of basaloid cells surrounded by a thick, hyalinized, periodic acid-Schiff (PAS)-positive basement membrane... Recently, it was suggested that targeting nuclear factor (NF)-κB in cylindroma therapy may be of therapeutic potential in patients with multiple tumors in the course of BSS... In one case of familial trichoepitheliomas, significant improvement was achieved with a combination of adalimumab and aspirin... This combination was chosen in order to block tumor necrosis factor alpha (TNFα)-induced activation of NF-κB at two different levels of the signaling pathway... Several therapeutic agents including sodium salicylate, cyclooxygenase (COX)2 inhibitors (i.e., celecoxib), corticoids and prostaglandins as well as phytomedical products inhibit NF-κB activity and thus, may be of benefit in BSS... MRM contributed in the conception of the work, conducting the study, and revising the draft... NM and NSA designed the study and were responsible for the overall study management... All authors approved the final version of the manuscript, and agreed with regard to all aspects of the work... There are no conflicts of interest.

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The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern
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Figure 2: The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern

Mentions: A 56-year-old woman presented with a long-standing history of slow-growing multiple scalp nodules. Her daughter and aunt also had a history of similar cutaneous lesions. Physical examination revealed multilobulated, large, epiphytic pink nodules, 2.5 cm by 6 cm in the greatest dimension, on the scalp [Figure 1]. The nodules were painless, solid, and movable from the underlying surface. Results of the routine laboratory examinations were normal. The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern and separated by hyaline basement membrane material, which was suggestive of cylindroma [Figure 2]. This patient underwent staged procedures, with debulking surgery followed by contouring with split-thickness skin graft. After 1 year, she was lost to follow-up.


Cylindroma of the scalp.

Mogaddam MR, Maleki N - J Res Med Sci (2015)

The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4696381&req=5

Figure 2: The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern
Mentions: A 56-year-old woman presented with a long-standing history of slow-growing multiple scalp nodules. Her daughter and aunt also had a history of similar cutaneous lesions. Physical examination revealed multilobulated, large, epiphytic pink nodules, 2.5 cm by 6 cm in the greatest dimension, on the scalp [Figure 1]. The nodules were painless, solid, and movable from the underlying surface. Results of the routine laboratory examinations were normal. The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern and separated by hyaline basement membrane material, which was suggestive of cylindroma [Figure 2]. This patient underwent staged procedures, with debulking surgery followed by contouring with split-thickness skin graft. After 1 year, she was lost to follow-up.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Imam Khomeini Hospital, Ardabil University of Medical Sciences, Ardabil, Iran.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

The histopathology of the scalp nodule revealed a well-defined dermal lesion composed of islands and cords of dark basaloid cells arranged in a jigsaw puzzle-like pattern and separated by hyaline basement membrane material, which was suggestive of cylindroma [Figure 2]... They can be seen in conjunction with spiradenomas and trichoepitheliomas, and this condition is known as Brooke-Spiegler syndrome (BSS)... BSS is a rare autosomal dominant disorder caused by mutations of the cylindromatosis gene (CYLD) located at 16q12-q13... Histologically, the tumors are circumscribed, nonencapsulated dermal nodules composed of islands and cords of basaloid cells surrounded by a thick, hyalinized, periodic acid-Schiff (PAS)-positive basement membrane... Recently, it was suggested that targeting nuclear factor (NF)-κB in cylindroma therapy may be of therapeutic potential in patients with multiple tumors in the course of BSS... In one case of familial trichoepitheliomas, significant improvement was achieved with a combination of adalimumab and aspirin... This combination was chosen in order to block tumor necrosis factor alpha (TNFα)-induced activation of NF-κB at two different levels of the signaling pathway... Several therapeutic agents including sodium salicylate, cyclooxygenase (COX)2 inhibitors (i.e., celecoxib), corticoids and prostaglandins as well as phytomedical products inhibit NF-κB activity and thus, may be of benefit in BSS... MRM contributed in the conception of the work, conducting the study, and revising the draft... NM and NSA designed the study and were responsible for the overall study management... All authors approved the final version of the manuscript, and agreed with regard to all aspects of the work... There are no conflicts of interest.

No MeSH data available.


Related in: MedlinePlus