Malignant Cylindroma of the Scalp Arising in a Setting of Multiple Cylindromatosis: A Case Report

Abstract

An unusual case of malignant cylindroma of the scalp arising in a 79-year-old white female with multiple cylindromatosis is presented. The tumor apparently arose from a cylindroma and had features of spiradenoma. Multiple cylindromatosis is an uncommon hereditary autosomal dominant disease, which is characterized by multiple skin adnexal tumors like cylindromas and trichoepitheliomas and occasional spiradenomas. Cylindroma is an uncommon benign tumor, which originates from skin appendages and is most commonly found on the scalp and face with a strong predilection for middle-aged and elderly females. Although cylindromas are usually benign neoplasms, carcinoma arising in such neoplasms is rare with only sporadic reports in literature. Her family history was negative for a similar disease. The patient's main concern was painful lesions over her right ear that interfered with wearing of her glasses. The clinical, histological immunohistochemical features, and treatment are presented along with a review of the literature.

Malignant cylindroma arising as a solitary neck lesion

“…A thirty-year-old male presented in 1981 with treatmentrefractory urticaria ( figure 1A), intermittent fever, muscle and bone pains, arthritis, anorexia, general malaise and persistently elevated inflammatory markers. The recent observation of an association between Waldenström's macroglobulinaemia and mutations in MyD88, a Toll-like Receptor signal transduction molecule with a role in the production of pro-IL-1␤, is intriguing and may have mechanistic implications for the disease [5]. An immunoglobulin M paraprotein was detected on electrophoresis (figure 1B), which increased over time, resulting in treatment for Waldenström's macroglobulinaemia.…”

“…Multiple cylindromas are often seen in a genetic condition known as Brooke-Spiegler syndrome (chromosome 9 defect) [1,2]. Gross features that may point to malignancy include rapid increase in size, ulceration and bleeding [5]. Cases involving the trunk and other locations have also been well documented [3].…”

“…She was treated with intravenous and oral ciprofloxacin hydrochloride, which improved the clinical symptoms over the next several weeks. Reactive arthritis also develops aseptic palmoplantar pustulosis together with arthritis [5]. Four months later, neck pain and lumbago relapsed.…”

Cylindroma: an update

“…2 The term arose for its cylindrical shape seen in transversal section. 4 Despite the identical nomenclature for those lesions, both the clinical behavior and prognosis can vary drastically. 4 Despite the identical nomenclature for those lesions, both the clinical behavior and prognosis can vary drastically.…”

Fine needle aspiration cytology of cylindroma of scalp: A case report

“…Most of the cases occur at head and neck region, specially over scalp in middle aged female patients. 3 Cylindromatosis or Brooke Spiegler syndrome (BSS) is a rare autosomal dominant inherited condition due to mutation of CYLD-1 gene. Multiple cylindromas are inherited as autosomal dominant pattern.…”