Disclosures. Introduction. Reflections 3/8/2014. No relevant disclosures. Surgical Treatment of Other Anal Tumors

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1 Disclosures Surgical Treatment of Other Anal Tumors No relevant disclosures Mark Lane Welton, MD, MHCM Harry A Oberhelman Jr. Professor Chief, Colon & Rectal Surgery Stanford University School of Medicine Vice Chief of Staff, Stanford Hospital & Clinics Introduction Reflections Reflections Definitions Pleas Excuses Rare tumors More filler UCSF PGC April 21-23, 1994 Clostridium difficile: a common masquerader Panel of experts Larry Way wear the mantle of the expert 1

2 Reflections - Career trajectory When I was young other neoplasms of the pancreas Peak of my career controversial subjects Past my prime other anal tumors Landmarks Anal cancer? poorly defined Standard terminology not helpful Standard terminology why no help? Dentate line Squamous metaplasia Dentate line Anal verge Anal verge Junction with hair bearing Surgeons understand Anal Margin?? 2

3 Gynecologic Terminology New Terminology HPV 16 Anal Peri-anal HPV 16 Everyone understands Transformation zone Anal cancer/perianal cancer New Terminology Anal Vulvar HPV 16 Transformation zone Almost Everyone understands 3

4 Anal adenocarcinoma Extramucosal (perianal) adenocarcinoma Adenocarcinoma within anorectal fistulae, Adenocarcinoma of anal glands the prognosis of anal adenocarcinoma is poor, and there is little information on the optimal management. The relevant studies indicate that a combination of radical surgical resection and pre- or postoperative chemoradiotherapy offers the best chance of survival Anal adenocarcinoma 20 year experience 83-04: 34 pts (6 palliative) 28 total: 13 local v 15 radical excision median dis free:13 mos v 32 mos (p=0.055) 5 year survival 43% & 63% (p=0.3) multivariate analysis: radical surgery - overall (p=0.045) & disease free survival (p=0.004) Anwar S. Colorectal Dis 2013;15(2): Chang, GC Dis Colon Rectum 09 Aug UCSF Class of 2003 Basal cell carcinoma of the perianus Etiology unclear due to reportedly limited sun exposure (Rochester MN) Review of 20 year period (ended 12/96) 21 cases (15 male) with follow up for 19 7 with basal cells (multiple) at other sites 17 local excision, 1 Moh s, 1 cautery Recurrence - Zero Buschke-Lowenstein tumors Giant condyloma acuminatum Large with propensity to infiltrate Malignant transformation > 50% High recurrence rates with radical surgery Paterson CA, Dis Colon Rectum 99 Sep 4

5 Buschke-Lowenstein tumors Condylomata complicating existing abscess fistulous disease Local failure - transection of sinus/fistula Radical excision Paget s disease Dr. Finlayson other lesions Squamous cell papilloma Papillary hidradenoma Keratocanthoma Neuroendocrine tumors Malignant melanoma Mesenchymal & Neurogenic tumors Lymphomas Squamous cell papilloma Test for HPV If negative - burned out condylomas Welton ML 4 th ed WHO Class Tumors of Dig Syst 5

6 Papillary hidradenomas Arises in perianal apocrine glands Women >>> men 1 cm circumscribed nodule mistaken for hemorrhoid Local excision Anal melanoma 1-3% of anal tumors 5 th or 6 th decade Pain, bleeding or mass Incidental finding at hemorrhoidectomy Spread - regional nodes and liver Metastases are frequent at presentation Survival < 5 years Anal melanoma Radical surgery palliation only Medical Oncology trials ipilimumab human monoclonal antibody to a receptor on CTLA-4 allowing continued tumor killing activity Mesenchymal & Neurogenic tumors Hemangioma, Hemangiopericytoma, Leiomyoma, Malignant fibrous histiocytoma, Leiomyosarcoma, Rhabdomyoma in newborn, Rhabdomyosarcoma in adulthood, Fibrosarcoma, Neurilemmoma neurofibroma, Granular cell tumor, spindle cell lipoma, aggressive angiomyxoma 6

7 Anal Leiomyoma Exceedingly rare < 0.1% anorectal neoplasms Spindle cell tumor Arise from muscularis mucosa, muscularis propria or sphincters Removal with negative margins Extended follow up Canda AE Surgery 2010 Aggressive Angiomyxoma anal if we mean near the anus wide local excision if possible Soft without well defined fibrous capsule Finger-like projections High local recurrence rates 70% Hormonal therapies/oophorectomy Abu JI Int J Gynecol Cancer 2005 Lymphoma Rare in general population Men > women because of AIDS More common in AIDS patients Especially MSM B-cell type most common large cell immunoblastic or pleomorhpic Peralta E. Clin Colon Rectal Surg 2009 Lymphoma Presentation mass or ulceration; local adenopathy Age of presentation x 34 years AIDS related x age 65 non- AIDS related Non AIDS risk factors UC & other immune disorders Peralta E. Clin Colon Rectal Surg

8 Lymphoma Excisional biopsy enough tissue AIDS related - HAART, chemotherapy & GCSF Non-AIDS related - indolent mucosal-associated lymphatic associated tissue (MALT) NCCN guidelines Margins clear - Excise & observe Margins positive - Locoregional radiation Inflammatory cloacogenic polyp Arise in the anal transition zone Associated with mucosal prolapse Rounded irregular mass cm in size Hyperplastic rectal mucosa May be confused with rectal adenoma Excise Peralta E. Clin Colon Rectal Surg 2009 Welton ML 4 th ed WHO Class Tumors of Dig Syst Malacoplakia Greek for soft plaques Usually in immunocompromised patients Most common in GU tract ~70% 50 cases of cutaneous Most common perineal ~ 40% Perianal nodules Perianal granular cell tumor Letter & case report Uncommon benign lesions of soft tissue 1/3 in tongue; skin, sub Q of chest and UE GI tract uncommon & perianus rare (17 cases) Schwann cell derivation Local excision Mourra N Internat J of Colorectal Dis

9 Anal malignant fibrous histiocytoma Case report & review of literature 4 involving anorectum previously reported Abdominal perineal proctectomy Transanal excision plus radiation in this case because radical excision refused. Anal Leiomyosarcoma Fewer than 20 in world s literature Radical excision with clear margins APR Lesions < 2 cm may be considered for local excision if wide clear margins Kim BG World J Gastroenterol 2008 Thalheimer L Am Surgeon 2008 Anal neurofibroma Skin, subq, deep soft tissue and viscera Anal canal very rare with one other report One of two nerve sheath tumors (schwannoma) Slow growing benign Local excision Frick EJ Jr. Dis Colon Rectum 2000 Anal spindle cell lipoma Case report 3x3 cm mass in intersphincteric plane Spindle cells No comment c-kit Categorized as a GIST Radical excision after excisional biopsy Residual mural disease of 0.7 cm Tan GY Tech coloproctol

10 Summary of other anal tumors Rare tumors Often extrapolate from knowledge in other areas Many lumped in as anal are just near 10