It is proposed that the blockade of NF-κBs actions prolongs cell survival, stimulates high rates of cell growth, and perhaps has other effects which contribute to the formation and/or progression of SA and SAC tumors. Diagnosis
SA and SAC tumors have been confused with leiomyoma, angiolipoma, granular cell tumor, basal cell carcinoma, squamous cell carcinoma, metastatic adenocarcinoma, and adenoid cystic carcinoma tumors; the giant vascular SA form of these tumors has been mistaken for a hemangioma, angiolipoma, calcifying epithelioma, neuroma, malignant melanoma, angiosarcoma, vascular malformation, and venous thrombosis. CT scans, FDG-positron emission tomography, and magnetic resonance imaging to detect 1) the shape and local invasiveness of these tumors and 2) the presence of metastases and 2) the clinical presentation of individuals of these tumors often suggest the correct diagnosis. However, definitive separation of SA, SAC, and giant vascular SA from the just cited lesions requires histopathological examinations. The diagnosis of SA versus SAC tumors is often suggested by their clinical presentations, e.g. rapid enlargement or onset of pain in a long-standing SA tumor strongly suggests that it has become a SAC. Findings that a known SA or undiagnosed tumor has areas of SA histology transitioning to SAC histology (see above Pathology section) is virtually diagnostic for SAC.The sporadic SA and SAC tumors reviewed here must also be distinguished from the very similar appearing SA-like and SAC-like tumors in individuals afflicted with the CYLD cutaneous syndrome. CCS is a familial disease uniformly associated with the inheritance of inactivating mutations in the CYLD gene.
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).