Dermatology Online Journal

Spiradenomas
Kathleen Tran MD MSc, Taylor DeFelice MD, Maria Robinson MD, Rishi Patel MD, Miguel Sanchez MD
Dermatology Online Journal 18 (12): 15

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

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Abstract

We report a 52-year-old man with a one-year history of multiple, firm, skin-colored nodules on the vertex of the scalp. Histopathologic examination was consistent with a spiradenoma, which is a rare, benign adnexal tumor of controversial histogenesis. Multiple spiradenomas may arise in association with Brooke-Spiegler syndrome, which is an autosomal dominant condition of multiple cyclindromas, trichoeptheliomas, and cyclindromas.

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History

Figure 1

A 52-year-old man presented to the Dermatology Clinic at Bellevue Hospital Center in September, 2011, with a one-year history of grouped, non-tender nodules on the vertex of his scalp. The nodules were slow-growing and occasionally were painful and pruritic. The patient had no family history of similar growths.

Physical examination

Six, firm, pink-to-skin-colored, smooth, exophytic papulonodules were present on the vertex of the scalp. The lesions were not tender and measured between five and ten millimeters in size.

Laboratory data

None.

Histopathology

Figure 2

Within the dermis, there are circumscribed aggregates of small epithelial cells, some of which have vesicular nuclei and are arranged around small lumina. The epithelial aggregates are partially rimmed by eosinophilic basement membrane material.

Discussion

Spiradenoma is a rare, benign, adnexal neoplasm, which typically presents as a slow-growing nodule on the head or trunk. It arises equally in men and women, most often in patients between 15 and 35 years of age, and has no racial predilection [1]. Although usually solitary, cases of multiple spiradenomas that form patterns described as linear, blashkoid, zosteriform, and nevoid have been reported [2, 3, 4]. The lesions may coalesce and form multinodular growths. Some giant vascular spiradenomas have grown to reach diameters of several centimeters [2, 3]. Rarely, the neoplasm arises in infancy or is congenital.

Multiple spiradenomas also have been described as a prominent feature of the autosomal-dominant Brooke-Spiegler syndrome, which is characterized by the development of multiple, adnexal tumors, predominantly spiradenomas, cylindromas, and trichoepthieliomas [4]. Most genetic mutations in Brooke-Spiegler syndrome have been mapped to the CYLD gene on chromosome 9 [5], but whether a specific genetic defect underlies isolated and sporadic spiradenomas has yet to be determined. Only about 50 cases of malignant degeneration into spiradenocarcinoma have been reported [6, 7].

On histopathologic examination, spiradenomas appear as multinodular, sharply- circumscribed, neoplasms of basaloid cells within the dermis and subcutis [11]. Within the nodules, there are aggregates of two cell types, which may form pseudoglandular rosettes. Small, basaloid cells with hyperchromatic nuclei tend to be located toward the periphery of the aggregates and larger cells with pale nuclei often are located closer to the center. There are duct-like structures and scattered droplets of eosinophilic, periodic acid-Schiff-positive hyaline material. Very large spiradenomas may develop cystic areas of degeneration or marked areas of vascular ectasia [6].

The pathogenesis of spiradenoma is largely unknown. Although long considered to be of eccrine gland differentiation, spiradenomas currently are considered to arise from cells of folliculosebaceous-apocrine lineage [4]. Not only can spiradenomas and cylindromas develop in the same patient, but lesions of cylindroma merging into a spiradenoma have been reported. Furthermore, both cylindromas and spiradenomas can arise within a nevus sebaceus [4]. One group of investigators found alterations in expression of beta-catenin, which is a protein previously linked to the pathogenesis of pilomatricoma, in a series of 12 spiradenomas using immunohistochemical stains [8].

The differential diagnosis of spiradenoma includes other adnexal tumors, such as cylindroma and poroma, as well as non-adnexal dermal tumors, such as leiomyoma and dermatofibroma. Diagnosis is best achieved by histopathologic examination. Treatment for painful spiradenomas consists of surgical excision. Recurrence is uncommon.

References

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2. Ekmekci TR, et al. Congenital blaschkoid eccrine spiradenoma on the face. Eur J Dermatol 2005; 15:73 [PubMed]

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8. Harada H, et al. The gene for multiple familial trichoepithelioma maps to chromosome 9p21. J Invest Dermatol 1996; 107:41 [PubMed]

9. Granter SR, et al. Malignant eccrine spiradenoma (spiradenocarcinoma): a clinicopathologic study of 12 cases. Am J Dermatopathol 2000; 22:97 [PubMed]

10. Kazakov DV, et al. Morphologic diversity of malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma based on the study of 24 cases, sporadic or occurring in the setting of Brooke-Spiegler syndrome. Am J Surg Pathol 2009 33:705 [PubMed]

11. Rapini, RP. Sweat gland neoplasms. In: Hodgson S, ed. Practical Dermatopathology: Philadelphia, PA: Elsevier Mosby, 2005:295

12. Im M, et al. Alteration of the beta-catenin pathway in spiradenoma. J Cutan Pathol 2011, 38: 657 [PubMed]

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