Eccrine porocarcinoma is a rare malignant sweat gland tumor arising from the intra dermal part of the gland and accounts for only 0.005% of all epithelial cutaneous tumors. Commonly involved site includes extremities and face. Scalp is a rare site for porocarcinoma with less than 20 reported cases so far. Wide local excision with clear margins remains the treatment of choice. Review of literature revealed a local recurrence rate of 37.5% and a nodal involvement risk of 20%. Porocarcinoma of the scalp is peculiar in that the primary tumor may be large at presentation, making surgery with adequate margins difficult. Adjuvant radiotherapy must be considered in a case to case basis due to the high local recurrence rates compared to other sites of porocarcinoma and should be given to all patients with close margins and extra capsular extension.
AB has two distinct grades with higher-grade tumors having significantly poor survival. Maximal safe surgery followed by adjuvant radiation and temozolomide should be advocated for these tumors.
Objectives Xanthoastrocytoma (XA) is a low-grade glial tumor seen in young adults and there is lack of robust data on treatment of this rare tumor. In this systematic review and individual patient's data analysis, we aimed to look into the demography, pattern of care, survival outcomes, and prognostic factors in patients with both Grade II and III XA.
Methods A comprehensive search was conducted with the Medical Subject Heading terms: “Xanthoastrocytoma; Pleomorphic Xanthoastrocytoma; Anaplastic Xanthoastrocytoma; Xanthoastrocytoma AND treatment; and Anaplastic Xanthoastrocytoma AND survival” to find all possible publications.
Results A total of 325 individual patients from a total of 138 publications pertaining to XA were retrieved. Median age of the entire cohort was 19 years. About 56.1% of the patients underwent a gross total resection (GTR) and 31.4% underwent a subtotal resection. Nearly, 76.6% of the patients had a Grade II tumor and adjuvant radiation was delivered in 27.4% of the patients. Estimated 2- and 5-year progression-free survival (PFS) were 68.5 and 51.2%, respectively. Age, grade, and extent of surgery were significant factors affecting PFS. Estimated 2- and 5-year overall survival (OS) was 88.8 and 78%, respectively. The median OS for Grade II and Grade III tumors were 209 and 49 months, respectively. Age and extent of surgery were significant factors affecting OS.
Conclusion XA is a disease of young adults with favorable prognosis. Younger patients (<20 years), patients who undergo a GTR, and patients with a lower grade tumor have a better treatment outcome.
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