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Case Report
Cylindroma with malignant transformation in a patient with Brooke-Spiegler syndrome
1 Resident, McMaster University Department of Pathology & Molecular Medicine, Hamilton, ON, Canada
2 Pathologist, St. Joseph’s Healthcare Hamilton, Hamilton, ON, Canada
Address correspondence to:
Man Hymn Edwin Ho
1280 Main Street West, Hamilton, ON,
Canada
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Article ID: 100048Z11MH2021
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Ho MHE, Alowami S, Gohla G, Salama S. Cylindroma with malignant transformation in a patient with Brooke-Spiegler syndrome. J Case Rep Images Pathol 2021;7:100048Z11MH2021.ABSTRACT
Introduction: Classic cylindromas are benign skin adnexal tumors of sweat duct origin that can be sporadic or arise multiply with other tumors in association with genetic syndromes such as Brooke-Spiegler syndrome. Malignant transformation is seldom seen, with less than 50 cases in literature. We report an extremely rare case of carcinoma ex-cylindroma in a genetic syndromic context.
Case Report: A woman with a history of Brooke-Spiegler syndrome presented with hemorrhagic and ulcerative change in a longstanding scalp lesion. After elective excision, gross description revealed a multinodular mass with a well-circumscribed smooth tan-pink component and a hemorrhagic, ulcerative component with yellow areas. Microscopy revealed both a residual benign cylindroma component with irregular solid nests of basaloid cells in a “jigsaw puzzle” architecture with surrounding hyaline sheath, and a malignant ulcerated component with loss of organization, cellular atypia, brisk mitoses, and necrosis. A diagnosis of malignant cylindroma, completely excised, was made. The patient will be seen in clinical follow-up.
Conclusion: This case represents a rare example of malignant transformation in a pre-existing cylindroma in a patient with Brooke-Spiegler syndrome. The scenario exemplifies the risk of genetic syndromes and role of follow-up of these so-called benign cylindromas, as malignant transformation needs to be identified for early management. Malignant cylindromas have potential for both locally aggressive recurrence and metastasis. Awareness and correct pathologic diagnosis is necessary preceding further future studies on natural history, prognosis, and treatment in this rare entity.
Keywords: Brooke-Spiegler syndrome, Malignant cylindroma
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Author Contributions
Man Hymn Edwin Ho - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Salem Alowami - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Gabriela Gohla - Conception of the work, Design of the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Samih Salama - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementPatient information has been de-identified for the purposes of confidentiality.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Man Hymn Edwin Ho et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
