The alveolar subtype
has a gross appearance of more circumscribed edges and a firmer, more rounded consistency. Microscopically, these tend to contain rounded, small acidophilic cells septated by collagen tissue with a broken off appearance with occasional giant cell formation. They tend to have larger nuclei and heavier chromatin patterns than embryonal types. Immunohistochemical profiling Inhibitors,research,lifescience,medical is the same as embryonal but staining for anti-skeletal muscle antibody and S100 protein was also documented (42,43). A study of 24 RMS of different types, showed all cases staining positively for Ankyrin-Repeated Proteins “Arpp/Carp” (44). RMS is harder to diagnose in adults, with more advanced disease at presentation (34). This may be due to the fact that symptoms
(tenesmus, pain on defecation or Inhibitors,research,lifescience,medical fever along with mild leukocytosis in some cases) tend to present later, probably because of a larger space for the tumor to grow. There is also a higher risk of misdiagnosis with diseases of infectious or inflammatory origin, such as perianal abscesses (45-47). In addition, younger patients and especially infants tend to be examined more often by parents during diaper Inhibitors,research,lifescience,medical changes possibly explaining why RMS is picked up earlier (34). The mainstay of RMS treatment is resection to negative margins and a combination of chemotherapy and radiation therapy. Pediatric RMS guidelines state that microscopically negative margins should be obtained and ipsilateral lymph node basins (inguinal, selleck screening library retroperitoneal) should be excised in patients older than 10 years. Although unproven, these guidelines have been used for treating Inhibitors,research,lifescience,medical adults as well. The use of chemotherapy combining Vincristine, Actinomycin D, Cyclophosphamide and Adriamycin in conjunction with external beam Tipifarnib radiotherapy for patients with positive margins, and local node involvement has also been advocated (34). However, radiotherapy Inhibitors,research,lifescience,medical is often associated with dermopathy, anal stenosis and genitourinary complications (48). Because aggressive surgery often results in sacrificing
the function of the anorectum, Dacomitinib sporadic attempts have been made at neoadjuvant chemotherapy to reduce tumor size and improve resectability. Intraoperative radiation therapy also has been sporadically used. Twelve adult cases of anorectal RMS have been reported (45-47,49-56), ranging between the ages of 18 and 71. Seven patients had radical surgery (APR or anterior resection), three had local excision and two never underwent an operation (patient refusal and bony metastasis at presentation. Half the patients had adjuvant chemoradiation with a combination of Vincristine, Actinomycin-D, Adriamycin and/or ifosfamide. Neoadjuvant therapy was used in two patients. Ten of the twelve cases had follow up data reported. Among these, 3 patients recurred within 6 months of surgery.