no evidence of metastases) SAC tumors and SAC tumors that have metastasized to local lymph nodes are commonly treated by surgical removal (with ≥1 cm margins) of the tumor plus surgical resection (i.e. removal) of the local lymph nodes. Individuals presenting with lymph node metastasis have also been treated with one or both of theses regimens plus, where possible and indicated, adjuvant radiation therapy to the areas of the removed tumors and lymph nodes. Chemotherapeutic agents such as methotrexate, cisplatin, etoposide, and 5-fluorouracil have been used to treat SAC with distant tissue metastases but the success of these drug treatments has been limited. Prognoses
In the only meta-analysis study reported on the treatment of SA and SAC tumors to date, 35 individuals with SA tumors and no evidence of local or distant metastasis who were treated with local resection (3 of these individuals underwent additional lymph node resections after removal of their primary tumor) all 35 were disease-free with a mean follow-up period of 33 months. Among individuals with SAC tumors that had metastasized to lymph nodes but not distant tissues, 3 individuals who had surgical excisions of their tumors but not their involved lymph nodes died of metastatic disease (range 39–49 months, mean survival range 45 months); one individual was alive with disease 24 months after lymph node dissection; and 8 individuals (6 of whom had lymph node dissection) lacked evidence of recurrent disease (observation times 2 to 97 months, mean observation time 47 months).
Individuals with CCS commonly develop an increasingly large number of skin tumors, including SA and SAC, over time. Usually, these individuals are readily distinguished from sporadic SA and SAC by their family history of the disease and the presence of large numbers of tumors. The rare cases of CCS that present with one or a few tumors are distinguished form CCS by testing for the CYLD gene: all individuals with CCS carry a mutated CYLD gene in their tumor as well as other tissues such as blood leucocytes while individuals with sporadic SA or SAC carry the mutated CYLD gene in their tumor but not other tissues (see Genetics of CCS). It is important to afford individuals with CCS and the close family members of these individuals access to in depth genetic counselling. Treatments
There is no consensus on the best treatment(s) for SA and SAC tumors. Further research, including meta-analysis comparisons of different treatment regimens, is needed to define the best treatment(s) for these tumors. In all cases, however, regular follow-ups of individuals with SA and SAC are strongly recommended in order to detect as early as possible: the formation of new tumors; changes in old tumors that suggest their progression to more aggressive or malignant formss; local recurrences of surgically removed tumors; and the development of local or distant metastases. One recommended follow-up strategy prescribes patients to have check-ups ever three months in the first year after treatment, every six months in the second year after treatment, and annually thereafter.