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Cylindroma and Spiradenoma

  • Chapter
Cutaneous Adnexal Neoplasms

Abstract

Cylindroma and spiradenoma are two morphological variants of the same neoplasm.

Multiple cylindromas may appear on the scalp (turban tumor). They are inherited in an autosomal dominant fashion, often associated with multiple trichoepitheliomas, spiradenomas, and tumors of the parotid gland, association known as Brooke–Spiegler syndrome.

Several genetic studies have identified that the gene that encodes the hereditary transmission of the multiple familiar cylindromas is CYLD gene, located on the 16q12–q13 chromosome.

Histopathologically, cylindroma is composed of multiple epithelial aggregates of basaloid cells disposed like a jigsaw puzzle. Each epithelial aggregate is surrounded by a thick, eosinophilic homogeneous basement membrane, and round deposits of the same material are frequently observed within the aggregates. Cytologically, each epithelial aggregate consists of a peripheral layer of small basaloid cells and larger cells with a pale cytoplasm cells in the center. Spiradenoma consisting of a smaller number but larger in size of the same epithelial aggregates, peppered by lymphocytes, whereas basement membrane material is less abundant than in cylindroma.

Cylindroma and spiradenoma show the same immunohistochemical profile, with positivity of neoplastic cells for CEA, human milk globulin-1, alpha-smooth muscle actin, S-100 protein, and cytokeratins 8 and 18.

Cylindroma and spiradenoma are benign neoplasms. Malignant degeneration is rare, and it has been mostly described in cases of multiple lesions of the scalp.

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  1. Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain

    Luis Requena

  2. Department of Pathology, Wake Forest University School of Medicine, Winston Salem, North Carolina, USA

    Omar Sangüeza

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Requena, L., Sangüeza, O. (2017). Cylindroma and Spiradenoma. In: Cutaneous Adnexal Neoplasms. Springer, Cham. https://doi.org/10.1007/978-3-319-45704-8_14

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