Spiradenoma Clinical Presentation

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 2, 2011
 

History

Spiradenoma lesions tend to arise on the head; the neck; the trunk; and, less commonly, the arms followed by the legs. Lesions tend to be about 1 cm in diameter, and they remain stable in size.

The lesions are mostly solitary, but they can be multiple, giant, linear, blaschkoid, or grouped.

Mambo[9] performed a clinical and pathologic study of 49 spiradenomas occurring in 46 patients. While textbooks say spiradenomas are painful, this was not reported in most patients. Pain, increased sensation, and/or tenderness were noted in only 23% of the 35 patients who had well-documented clinical histories. In 2 patients, the spiradenoma underwent malignant transformation but did not recur in the 35 patients who underwent surgical removal.

Malignant spiradenomas can occur in long-standing lesions.[10] In malignant spiradenomas of the breast, a long-standing cutaneous nodule begins to enlarge rapidly. The growth is often associated with an ulceration and a change in color.

Granter et al[11] reported clinicopathologic features of 12 cases, as follows:

  • In one study of approximately 10 patients, the male-to-female ratio of patients with spiradenomas was 1:1; most patients were in their 60s.
  • Spiradenomas occurred most commonly on the trunk. The next most common regions were the extremities and the head and neck. The average size of the spiradenoma was 7.5 cm in diameter. According to the history, the spiradenoma had been present 7 months to 30 years before surgical removal. Only 1 patient had metastatic spread of the spiradenoma. In this patient, an apparent remission occurred with dissection of the lymph nodes.
  • When biopsy specimens were examined, areas of benign spiradenomas and malignant spiradenomas coexisted, with more malignant spiradenoma tissue usually present. The cancerous nature of malignant spiradenomas was revealed by an increased mitotic rate, necrosis, nuclear atypia, pleomorphism, hyperchromasia, loss of nested and trabecular growth patterns, and an absence of a dual cell population.
  • Malignant spiradenomas with pulmonary metastasis has been reported.[12]
  • Granter et al[11] found 2 distinctive histologic patterns in malignant spiradenomas (both with focal squamous differentiation). The first was an abrupt transition between a benign spiradenoma and a high-grade carcinoma component. The second was a contiguity between benign spiradenomas and malignant spiradenomas. In the later case, the diagnosis was defined by an increased nuclear-to-cytoplasmic ratio, hyperchromasia, and marked mitotic activity.

Kao et al[13] noted that spiradenomas rarely (< 1%) occur in infancy. spiradenomas in infants differ from the conventional spiradenomas by the presence of superficial dermal nodules. The nodules display a less distinct 2-cell pattern of immature adnexal epithelial cells. Ductule formation is rare. In infants and young adults, these tumors may be mistaken for mesenchymal neoplasms involving the skin and the subcutis.

Spiradenomas can mimic other painful tumors, in particular angiolipomas, because of their similar color.

Brooke-Spiegler syndrome with combined lesions containing cylindromatous, spiradenomatous, trichoblastomatous, and sebaceous differentiation have been reported.[14]

Spiradenomas have been noted to occur in the ears in several case reports,[15] including that by Nadig et al[16] from 2004. External auditory canal eccrine spiradenocarcinoma has also been noted.[17] Additionally, papular lesions of the proximal nail fold have been noted to be spiradenomas.[18]

Vulvar carcinosarcoma arising from a eccrine spiradenoma has been reported.[19]

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Physical

Spiradenomas are usually gray, pink, purple, red, or blue nodules about 1 cm in diameter. The lesions tend to occur on the scalp, the neck, and the upper part of the torso. About 5 cases have been reported on the eyelid.[20, 21] Spiradenomas tend to be soft and are sometimes painful to palpation.

Malignant spiradenomas tend to be larger than benign spiradenomas. Malignant spiradenomas tend to ulcerate. Malignant spiradenomas tend to preferentially involve the trunk and the extremities (92% of reported cases). The average size of malignant spiradenomas at presentation is 3.9 cm (range, 0.5-15 cm) in diameter. Many malignant spiradenoma lesions occur on the scalp.[22, 23] Malignant spiradenomas have been reported to occur on a traumatized area.[24] Malignant spiradenoma can result in lymph node, diffuse pulmonary or brain, and liver metastases (in order of occurrence). Chase et al[25] noted a malignant spiradenoma of the vulva, underlying that malignant spiradenomas can occur in almost any location. Malignant spiradenomas have been reported to occur on the toes.[26]

Giant eccrine spiradenoma of the hand was reported by Siegel et al.[27] Turhan-Haktanir et al in 2008 noted an eccrine spiradenoma arising in nevus sebaceous in an adolescent girl.[28] Congenital linear eccrine spiradenoma was noted as a presentation of spiradenoma by Rodríguez-Martín et al in 2009.[29]

Multiple eccrine spiradenomas in a segmental distribution have been reported.[30]

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Causes

Spiradenomas seem to be caused by a defective tumor suppressor gene. In Brooke-Spiegler syndrome, a defect exists in the CYLD gene located on chromosome 9. The actual cause of a solitary spiradenoma has yet to be defined.

The cause of malignant spiradenomas is unclear. The expression of TP53 seems to be increased in malignant spiradenomas, but the significance of this observation is unclear.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Specialty Editor Board

Evan R Farmer, MD  Clinical Professor of Pathology and Dermatology, Department of Pathology, Virginia Commonwealth University School of Medicine

Evan R Farmer, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, and International Society of Dermatology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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