CCS is an inherited disorder that commonly involves the development of multiple but on occasion a single or few tumors that closely resemble, and may be confused with, the sporadic SA and SAC tumors described here. CCS spiradenoma and CCS spiradenocarcinoma tumors must be distinguished form the sporadic SA and SAC tumors reviewed here in order to afford genetic counseling to individuals with CCS as well as to the close family members of these individuals.Currently, SA and SAC are usually treated by complete excision making sure that no tumor cells are left behind at the surgical site. This is particularly important in SAC where the incomplete surgical removal of all tumor cells may result in recurrence of the tumor at the surgical site and/or its metastasis to the local lymph nodes draining the surgical site and/or to distant tissues. In addition to surgical resection, some cases of SAC tumors have been treated with adjuvant therapy that includes radiotherapy and/or chemotherapy. It is not clear that these adjuvant treatments improve patient prognoses. Further studies are needed to determine the best treatment(s) for localize SA, localized SAC, and, in particular, metastatic SAC tumors. Presentation
Spiradenoma tumors occur in individuals of various ages but tend to develop in middle-aged and elder adults (e.g. in a study of 27 patients, the median age at diagnosis was 62 years). However, cases have been reported in children as young as 2 years. These tumors usually present as a solitary, sometimes painful, slowly growing, 1–2 cm (i.e.
In a review of 136 reported cases treated for SA or SAC: 1) 4.4% and 14.7% of individuals with SA and SAC tumors, respectively, had died (time between treatment and death was not reported); 2) patients who presented with metastasis to distant tissues had a median survival time of 16 months (11 of these patients were treated with adjuvant therapy in addition to surgery [adjuvant treatments included 5 patients treated with radiotherapy, 3 treated with chemotherapy, and 2 treated with both radiotherapy and chemotherapy]); and 3) Surgery with adjuvant therapy yielded a median survival time of 20 months (this survival time did not significantly differ from that found in individuals treated with surgery alone). See also
Malignant acrospiroma
List of cutaneous conditions
Dermal cylindroma
Trichoepithelioma
CYLD cutaneous syndrome
List of cutaneous neoplasms associated with systemic syndromes

References


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