Are You Confident of the Diagnosis?
Characteristic findings on physical examination
Spiradenoma presents as a skin-colored, blue, or pink nodule located most commonly on the upper trunk or extremities (Figure 1). The nodule is well demarcated and has a spongy or cystic feel. It is usually less than 1 cm in size; however, they can grow larger and may be painful or tender. The many pneumonics for painful skin tumors invariably include eccrine spiradenoma. Rarely, multiple spiradenomas may be present and may be arranged in a linear or zosteriform pattern.
Expected results of diagnostic studies
A biopsy will reveal a well-defined dermal or subcutaneous tumor composed of one or only a few round basophilic lobules (Figure 2). There is no epidermal connection, which helps to distinguish this tumor from a basal cell carcinoma. The cells are arranged in sheets and cords and there may be small duct-like structures. There are two main types of cells comprising the lobules. The cells lining the ducts and surrounding the abundant small blood vessels are basaloid cells with hyperchromatic nuclei. In between are larger polygonal cells with paler cytoplasm. On close examination, lymphocytes are found scattered throughout (Figure 3).
Under the microscope, cylindroma most closely resembles spiradenoma, but it is usually distinguished by the many small aggregates of cells arranged in a jigsaw puzzle pattern and surrounded by hyalin material. Spiradenomas with cylindromatous foci (spiradenocylindroma) have been described.
Malignant degeneration of a spiradenoma is a rare occurrence. Malignant eccrine spiradenoma is more likely to develop in a preexisting spiradenoma. Clinically, the patient may have noticed rapid enlargement, ulceration, pain, or a change in color. Histologically, the malignant spiradenoma may be differentiated from its benign counterpart by the presence of cytologic atypia, loss of a well circumscribed architecture, necrosis, infiltration of strands into the stroma, and numerous mitoses.
Multiple spiradenomas may be a sign of a familial spiradenoma syndrome or Brooke-Spiegler syndrome. To diagnose Brooke-Spiegler syndrome, in addition to spiradenomas, trichoepitheliomas and cylindromas must be present. The clinical differential diagnosis includes cylindroma, basal cell carcinoma, or a benign fibrous, vascular, or neural tumor. While spiradenoma is more likely to present with pain, a potential clue to the diagnosis, a deep biopsy (punch or excisional) is usually done to ascertain the correct diagnosis.
Who is at Risk for Developing this Disease?
Young to middle-aged adults of both sexes are most commonly affected. Those with Brooke-Spiegler syndrome or a familial eccrine spiradenoma syndrome are at risk for developing multiple spiradenomas.
What is the Cause of the Disease?
Despite the terminology, eccrine spiradenomas are thought to be of apocrine origin, but some may exhibit eccrine differentiation. Cylindromas and spiradenomas show overlapping features, including myoepithelial, ductal, apocrine, and eccrine features. It was speculated that they both may be derived from a pluripotent cell.
In another report, biopsies from a family with multiple hereditary trichoepitheliomas were studied. The biopsies showed trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma). The spectrum of lesions exhibited folliculosebaceous and apocrine differentiation. It was hypothesized that in this syndrome, tumors are derived from undifferentiated germinative cells of the folliculosebacous-apocrine unit. It also lended support to the theory that spiradenomas are apocrine in origin rather than eccrine.
Systemic Implications and Complications
There are no local complications for benign spiradenomas; however, the lesions can be cosmetically disfiguring. This is especially true for syndromes in which there are multiple spiradenomas present. Spiradenoma does not have systemic implications unless it turns malignant. If malignant, the patient is at risk for metastases. Metastases are most commonly found in the lymph nodes, lung, brain, and liver.
Treatment options are summarized in Table I.
|Medical Treatment||Surgical Treatment||Physical Modalities|
|None||Wide local excision||Carbon dioxide laser|
|Staged surgical excision|
|Mohs micrographic surgery|
|Bipolar scissor removal (in conjunction with laser therapy)|
Optimal Therapeutic Approach for this Disease
Wide local excision is the best option for both malignant and benign spiradenomas. Since the benign lesion may recur or have foci of malignant transformation, it is important that the spiradenoma is completely excised. For malignant spiradenoma, some authors have recommended 1-cm margins and excision depth down to the fascia. As with most surgical options, there is the risk of infection, bleeding, and scarring.
Excisional biopsy may be done diagnostically or therapeutically, but complete excision is recommended to avoid recurrence or chance of malignancy.
Staged surgical excision is useful for patients with multiple lesions where wide local excision of each spiradenoma is not feasible. This approach is also used in combination with laser therapy to debulk the tumor before laser treatment.
For spiradenomas in cosmetically concerning areas, Mohs micrographic surgery is recommended. Mohs surgery minimizes the scarring as compared to wide local excision, and this option also allows the surgeon to remove microscopic disease which may have been missed otherwise.
Carbon dioxide laser therapy has been used to vaporize multiple tumors located on the face. The laser was used in continuous wave mode, 10.0 W. The patient in the case report had an excellent cosmetic outcome and experienced relief from the pain associated with the lesions. There were no complications reported.
Bipolar scissors have been reported in debulking cylindromas, a similar lesion to eccrine spiradenoma, and prior to treatment with laser therapy. Two cases have been reported. The first patient had less severe cylindromas that were treated under local anesthesia with several passes of a continuous wave carbon dioxide laser at 10 to 15 W with a 1-cm focusing handpiece. The second patient had more extensive and severe cylindromas that were treated under general anesthesia by first debulking the tumors with bipolar scissors and then using the continuous wave carbon dioxide laser at 40 to 50 W. The second patient did have areas of alopecia and atrophy, but had no recurrence after 1 year.
Other ablative therapies such as mechanical dermabrasion and radiotherapy have been mentioned in the literature, but there are no studies demonstrating results.
The patient should be advised that eccrine spiradenoma is usually a benign lesion that tends to remain stable over time. They should be aware that the presence of pain or tenderness is not necessarily a sign of malignant degeneration. A biopsy should be performed to confirm the diagnosis.
Spiradenomas, even when benign, should be excised in their entirety, since there is the possibility of malignant degeneration within the lesion. If the patient does not wish to have the spiradenoma completely excised, the clinical characteristics of a possible malignant change should be explained and the patient should be followed carefully. The risks and benefits of each procedure should be thoroughly explained, and Mohs surgery should be given as an option, especially for cosmetically important areas.
If the lesion is found to be a malignant spiradenoma, wide local excision or Mohs surgery should be recommended. The patient may need a referral to a surgeon and an oncologist. Screening for metastases, including a sentinel lymph node biopsy, may be indicated depending on the site, length of time present, and histologic features. Close clinical follow-up every 3 months the first year, every 6 months the second year, and annually thereafter has been recommended by some authors. They also state that an annual chest radiograph and liver function tests may be useful, since lung and liver are two of the top locations for metastases.
Unusual Clinical Scenarios to Consider in Patient Management
Familial eccrine spiradenomas are inherited in an autosomal dominant pattern. These patients exhibit multiple painful, growing nodules on their face, scalp, and upper trunk. A thorough family history may reveal several generations with similar findings. Genetic counseling is important if familial eccrine spiradenoma syndrome is suspected.
Spiradenomas have also been reported in association with the autosomal dominant Brooke-Spiegler syndrome. Other findings in this syndrome include multiple trichoepitheliomas and cylindromas. The genetic abnormality of this syndrome has been mapped to 16q12-13 where there is a loss of heterozygosity of the CYLD locus (tumor suppressor).
What is the Evidence?
Kersting, DW, Helwig, EB. “Eccrine spiradenoma”. AMA Arch Dermatol. vol. 73. 1956. pp. 199-227. (Spiradenomas are characterized and described both clinically and pathologically. The majority of the lesions are solitary, present with paroxysmal pain or pain out of proportion to trauma, and are located on upper trunk, face and upper extremities. They observed that if the lesion was not completely removed, it was likely to recur.)
Argenyi, ZB, Nguyen, AV, Balogh, K. “Malignant eccrine spiradenoma: a clinicopathologic study”. Am J Dermatopoathol. vol. 14. 1992. pp. 381-90. (Two cases of malignant eccrine spiradenoma are reported and discussed. They outline 22 cases of malignant eccrine spiradenoma with clinical features and outcomes. Conclusions made included discussion of the wide spectrum of histologic variants found in malignant eccrine spiradenoma.)
Meybehm, M, Fischer, HP. “Spiradenoma and dermal cylindroma: comparative immunohistochemical analysis and histogenetic considerations”. Am J Dermatopathol. vol. 19. 1997. pp. 154-61. (Both cylindromas and spiradenomas show myoepithelial, ductal, apocrine, and possibly eccrine features. They conclude that both tumors arise from pluripotent cells.)
Ter Poorten, MC, Barrett, K, Cook, J. “Familial eccrine spiradenoma: a case report and review of the literature”. Dermatol Surg.. vol. 29. 2003. pp. 411-4. (A case report of familial eccrine spiradenoma and review of this rare syndrome in the literature. Only two other cases had been reported prior. Familial eccrine spiradenoma is distinct from Brooke-Spiegler syndrome. Trichoepitheliomas and cylindromas have not been found in association with the familial eccrine spiradenoma syndrome, and therefore, it should be considered a separate entity.)
Hantash, BM, Chan, JL, Egbert, BM, Gladstone, HB. “De novo malignant eccrine spiradenoma: a case report and review of the literature”. Dermatol Surg. vol. 32. 2006. pp. 1189-98. (A case report of malignant eccrine spiradenoma arising on the forehead over a period of 1 week. This is unusual since most malignant eccrine spiradenomas arise within a preexisting benign spiradenoma. They recommend Mohs surgery for treatment, but the patient refused. Instead, the lesion was treated with wide excision with 1-cm margins.)
Martins, C, Bartolo, E. “Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser”. Dermatol Surg. vol. 26. 2000. pp. 877-80. (A report of two case of Brook-Spiegler syndrome, in which one case was treated with laser alone and one case was treated with bipolar scissor debulking and more aggressive laser therapy. They also report that in one of their cases the histology of one tumor showed features of both a cylindroma and an eccrine spiradenoma.)
Clarke, J, Ioffreda, M, Helm, KF. “Multiple familial trichoepitheliomas: A folliculosebaceous-apocrine genodermatosis”. Am J Dermatopathol. vol. 24. 2002. pp. 402-5. (Biopsies from a family with multiple hereditary trichoepitheliomas showed trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma), demonstrating a spectrum of lesions exhibiting folliculosebaceous and apocrine differentiation. It was hypothesized that the syndrome may represent tumors derived from undifferentiated germinative cells of the folliculosebacous-apocrine unit.)
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