1/5. Mucinous adenocarcinoma in chronic anorectal fistula.adenocarcinoma in association with chronic anal fistula is a rare disease which gives rise to difficult problems of diagnosis and treatment. A case of mucinous adenocarcinoma arising on a long standing fistula in ano is described. A patient with a long history of mucinous discharge, pain and perianal induration underwent a biopsy of the external opening of the fistula that showed mucinous infiltrating adenocarcinoma. After a colonoscopy and a preoperative abdominal CT scan, she underwent a successful abdominoperineal resection with adjuvant chemoradiation therapy. diagnosis of this condition is often difficult; deep and multiple biopsies of the fistulous tracks or perianal mass are necessary to establish the diagnosis. An accurate staging of the neoplasm, using endorectal ultrasound, NMR or CT scans is needed to plan the appropriate treatment. Recent studies have shown that locally advanced anal adenocarcinomas could benefit from pre or postoperative chemoradiation therapy. However, an accurate and complete removal of the tumor, which usually entails abdominoperineal resection, is often necessary to achieve radicality. Despite new therapy protocols, the prognosis of mucinous adenocarcinoma is still poor, mostly due to its advanced nature at the time of diagnosis. This reinforces the importance of biopsy of all perianal abscesses and fistulas for early detection and treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/5. Giant condyloma acuminatum of the anorectum: trends in epidemiology and management: report of a case and review of the literature.PURPOSE: Giant condyloma acuminatum (Buschke-Loewenstein tumor) of the anorectum is a rare disease with a potentially fatal course. Controversy exists as to the epidemiology, pathologic nature, and management of the tumor. methods: We present a 42-year-old male with a 12-cm x 10-cm exophytic mass of the anal verge. Treatment included wide local excision and partial closure with rotation flaps. pathology revealed a giant condyloma acuminatum with foci of well-differentiated squamous-cell carcinoma. We identified 51 reported cases of giant condyloma acuminatum in the English literature, and to our knowledge this is the largest review to date. RESULTS: Giant condyloma acuminatum presents with a 2.7:1 male-to-female ratio. For patients younger than 50 years of age, this ratio is increased to 3.5:1. The mean age at presentation is 43.9 years, 42.9 in males and 46.6 in females (P = 0.44). There seems to be a recent trend toward a younger presentation. The most common presenting symptoms are perianal mass (47 percent), pain (32 percent), abscess or fistula (32 percent), and bleeding (18 percent). Giant condyloma acuminatum has been linked to human papilloma virus and has distinct histologic features. Foci of invasive carcinoma are noted in 50 percent of the reports, "carcinoma in situ" in 8 percent, and no invasion in 42 percent. Historically, treatment strategies have included topical chemotherapy, wide local excision, abdominopelvic resection, and the frequent addition of adjuvant and neoadjuvant systemic chemotherapy and radiation therapy. recurrence is common. CONCLUSION: There seems to be a trend toward younger age at presentation and male predominance of giant condyloma acuminatum of the anorectum. Foci of invasive cancer within giant condyloma specimens are of uncertain significance and do not seem to correlate with recurrence or prognosis. Local invasion and local recurrence are the major source of morbidity in this disease. Complete excision is the preferred initial therapy when feasible. Wide local excision, fecal diversion, or abdominoperineal resection have been used. Chemotherapy with 5-fluorouracil and focused radiation therapy may be used in certain cases of recurrence or extensive pelvic disease, with unpredictable response. Controlled, prospective, multi-institutional studies are necessary to further define the nature and treatment of this rare disease.- - - - - - - - - - ranking = 2keywords = rare disease (Clic here for more details about this article) |
3/5. Perianal melanoma disguised as hemorrhoids: case report and discussion.Perianal melanoma is a very rare disease that carries a grave prognosis. The difficulty in making the diagnosis and the rarity of the disease results in failure of recognition until the disease is widespread. Ultimately, the burden is on the physician to uncover this ominous cancer. Not doing so is one of the most significant correlates to poor outcome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/5. Anorectal malignant melanoma: report of two cases from Buddhachinnaraj Hospital.Anorectal malignant melanoma is a rare disease and has a uniformly poor prognosis. The following are two reported cases from Buddhachinaraj Hospital. A 55-year-old and a 65-year-old female patients presented with rectal bleeding. Large anorectal masses with regional lymph node involvement were detected initially. They exhibited different histological features which were atypical round cell resembling lymphocytes or were small cell appearance and spindle cell appearance similar to sarcoma. The diagnosis was confirmed by expression of S100 protein and HMB45. Abdominoperineal resection (APR) was the treatment of choice in both patients. The former case died in the fourth month after diagnosis because of distance metastasis and congestive heart failure. The latter case is receiving postoperative adjuvant therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/5. Long-term survival after locally aggressive anorectal melanoma.Anorectal melanoma is a rare disease and, unlike cutaneous melanoma, there are few guidelines regarding optimal management. It has a reputation for having a poor prognosis, which has been attributed to a delay in diagnosis and to a lack of effective systemic therapy. It has also been suggested that the biology of this tumor may differ from that of cutaneous melanoma. An interesting case of anorectal melanoma is presented which highlights the unique considerations and challenges encountered by medical oncologists and surgeons who treat this disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |