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A Revisit to Anorectal Malignant Melanoma (ARMM)

This article discusses the rare neoplasm of anorectal malignant melanoma, including its epidemiology, presentation, investigations, staging, prognosis, and treatment options such as surgery and adjuvant therapy.

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A Revisit to Anorectal Malignant Melanoma (ARMM)

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  1. A Revisit to AnorectalMalignant Melanoma (ARMM) Joint Hospital Surgical Grand Round 8 Nov 2014 Dr Jessie Chan Pamela Youde Nethersole Eastern Hospital

  2. Outline • Introduction • Epidemiology • Presentation • Investigations • Staging • Prognosis • Treatment • Surgery • Adjuvant therapy

  3. Introduction • Rare neoplasm • First reported by Moore in 1857 • Distinct biological and clinical entity from cutaneous melanoma with worse prognosis • No clear etiology elucidated • Arised from melanocytes distal to dentate line and extend proximally to rectum • Arised directly from melanocytes which present rarely in the mucosal epithelium of proximal anus or distal rectum

  4. ARMM • <1% of all malignant melanomas • <4% of anal canal malignancies • Primary • Secondary • Metastasis from cutaneous melanoma (2%)

  5. Epidemiology • Median age at presentation: 55 (range 29-92) • Reported incidence: 0.04 – 1.19% • No adequate population-based studies to draw definite conclusions for racial and gender difference

  6. Presentation • Non-specific symptoms • PR bleeding • Anal pain, anal mass • Tenesmus, pruritus • Change in bowel habit • Symptoms of metastatic disease • Most common sites of metastases: inguinal / mesenteric / hypogastric / para-aortic lymph nodes, liver, lung, skin, brain • Weight loss, groin mass, pelvic mass, bowel obstruction • Incidental finding

  7. Presentation • 80% lack obvious pigmentation • 20% histologically amelanotic

  8. Presentation • Up to 60% with locoregional lymphatic spread (mesorectal, pelvic side wall, inguinal lymph nodes) • Up to 40% with distant metastasis

  9. Investigations • Endoscopy and biopsy • Staging • Endoscopic ultrasound: role unclear • CT: regional lymphadenopathy, distant metastasis • PET scan: may be helpful

  10. Staging • Cutaneous melanoma: AJCC TNM system • ARMM • Clinical staging • Stage I: local disease • Stage II: local disease with regional lymph nodes • Stage III: distant metastatic disease

  11. Prognosis • Poor prognosis • Overall 5-year survival 3-22% • Survival <10 months with recurrent or metastatic disease

  12. Prognosis • Good prognostic factors • Tumour thickness <2mm • Poor prognostic factors • Tumour thickness >3mm • Tumour site above dentate line • Lymphovascular / perineural invasion • Necrosis Wanebo HJ, Woodruff JM, Farr GH, et al. Anorectal melanoma. Cancer. 1981 Apr 1; 47(7):1891-900. Brady MS, Kavolius JP, Quan SH. Anorectal melanoma. A 64-year experience at Memorial Sloan-Kettering Cancer Center.Dis Colon Rectum. 1995 Feb; 38(2):146-51. Perez DR, Trakarnsanga A, Shia J, et al. Locoregional lymphadenectomy in the surgical management of anorectal melanoma. Ann Surg Oncol. 2013;20:2339-2344.

  13. Treatment

  14. Treatment • Surgery • Wide local excision (WLE) • Abdominoperineal resection (APR) • Regional lymph node • Sentinel lymph node biopsy • Lymphadenectomy

  15. Treatment • Adjuvant therapy • Systemic • Chemotherapy • Biochemotherapy • Interferon • Vaccine / immunotherapy • Local • Radiation therapy • Electrochemotherapy

  16. Surgery • Mainstay of treatment • Controversies • APR vs local excision (LE) APR – en bloc excision with mesorectal lymph nodes ?oncological benefit • Inguinal lymphadenectomy

  17. Surgery • Meta-analysis by Akihisa Matsuda et al in Annals of Surgery 2014 • 31 studies • 1006 patients • APR vs LE • Overall survival • Relapse-free survival • Local recurrence rate Akihisa Matsuda, Masao Miyashita, Satoshi Matsumoto, et al. Abdominoperineal Resection Provides Better Local Control But Equivalent Overall Survival to Local Excision of Anorectal Malignant Melanoma – A Systemic Review. Ann Surg. 2014; 00: 1-8.

  18. APR vs LE • Overall survival – no difference (31 studies, N=1006)

  19. APR vs LE • Recurrence-free survival – no difference (14 studies, N=328)

  20. APR vs LE • Local recurrence rate – significantly lower in APR (13 studies)

  21. Surgery • No survival benefit from APR over LE • Higher local recurrence from LE which could be managed by salvage surgery • Less complications and morbidities with LE • Better body image • Better urinary and sexual function • Minimal impact on bowel function • Minimizing morbidities and maximizing quality of life merits consideration in such an aggressive disease

  22. Inguinal lymphadenectomy • Locoregional lymph node metastasis – no significant prognostic implication Perez DR, Trakarnsanga A, Shia J, et al. Locoregional lymphadenectomy in the surgical management of anorectal melanoma. Ann Surg Oncol. 2013;20:2339-2344.

  23. Inguinal lymphadenectomy • Systemic dissemination with micrometastasis in distant organs occurs early with unfavourable prognosis • Prophylactic – no improvement in survival, increased risk of complications • Therapeutic – seems not to contribute to improvement of survival Perez DR, Trakarnsanga A, Shia J, et al. Locoregional lymphadenectomy in the surgical management of anorectal melanoma. Ann Surg Oncol. 2013;20:2339-2344.

  24. Adjuvant Therapy

  25. Chemotherapy • Role remains unclear • ?Adjuvant • ?Palliative • Dacarbazine, cisplatin, vinblastine, vincristine, nimustine, bacillus Calmette-Guérin, levamisole, temozolomide • Single or combination – poor results

  26. Interferon • Interferon alpha • Combination of direct activities and indirect immune-mediated effects • Parenteral route / intratumoural injection • No standard regimen established • Insufficient data

  27. Biochemotherapy • Biologic agent (interferon, interleukin) + traditional cytotoxic chemotherapeutic agent • Longer disease-free and median survival in metastatic ARMM when compared with chemotherapy alone Kim K B, Sanguino A M, Hodges C, et al. Biochemotherapy in patients with metastatic anorectal mucosal melanoma. Cancer. 2004;100:1478–1483. Atkins MB, Lotze MT, Dutcher JP, et al. High-dose recombinant interleukin 2 therapy for patients with metastatic melanoma: analysis of 270 patients treated between 1985 and 1993. J Clin Oncol. 1999 Jul; 17(7):2105-16.

  28. Biochemotherapy • Role • Best available adjuvant therapy after surgery • Considered in advanced or metastatic ARMM

  29. Vaccine / Immunotherapy • Immunize against melanoma cell antigen • BRAF and cKIT mutations • Insufficient data • Area of significant research effort and may play an important part of the non-operative treatment of melanoma in the future

  30. Radiation Therapy • Locoregional disease control • Primary lesion, inguinal and pericolic lymph nodes • Utility unclear, evidence conflicting

  31. Radiation Therapy • Ballo et al • Local excision + post-op RT • Local control rate comparable with APR • 5-year local control rate: 74% • No improvement in survival • Moozar et al • Pre-op RT little effect on tumour burden • Post-op RT did not change local recurrence • No survival benefit Ballo MT, Gershenwald JE, Zagars GK, et al. Sphincter-sparing local excision and adjuvant radiation for anal-rectal melanoma.J Clin Oncol. 2002 Dec 1; 20(23):4555-8. Moozar KL, Wong CS, Couture J. Anorectal malignant melanoma: treatment with surgery or radiation therapy, or both.Can J Surg. 2003 Oct; 46(5):345-9.

  32. Electrochemotherapy • Injection of chemotherapy directly into the lesions, followed by application of electric pulses using a needle electrode • Electrical stimulation to the tissues creating a transient permeabilization of the plasma membrane • Allows direct access of the chemotherapeutic agents into the cytosol of tumor cells • Known to provide effective local control for cutaneous melanoma

  33. Electrochemotherapy • Convert APR to sphincter-preserving local excision • Overall success rate unclear Snoj M, Rudolf Z, Cemazar M,et al. Successful sphincter-saving treatment of anorectal malignant melanoma with electrochemotherapy, local excision and adjuvant brachytherapy.Anticancer Drugs. 2005 Mar; 16(3):345-8.

  34. Summary • ARMM is a rare and highly malignant disease • Survival predicted by status of regional and distant metastasis but not method of surgery for local control • Minimizing surgical morbidities and maximizing quality of life should be the major consideration in formulating the treatment plan • Role of all adjuvant therapies is still unclear

  35. References • P Carcoforo, M.T Raiji, G.M Palini, et al. Primary Anorectal Melanoma: An Update.J Cancer. 2012; 3:449-453. doi:10.7150/jca.5187. • Marc Singer and Matthew G. Mutch.Anal Melanoma.Clin Colon Rectal Surg. May 2006; 19(2): 78–87. • Akihisa Matsuda, Masao Miyashita, Satoshi Matsumoto, et al. Abdominoperineal Resection Provides Better Local Control But Equivalent Overall Survival to Local Excision of Anorectal Malignant Melanoma – A Systemic Review. Ann Surg. 2014; 00: 1-8. • Perez DR, Trakarnsanga A, Shia J, et al. Locoregional lymphadenectomy in the surgical management of anorectal melanoma. Ann Surg Oncol. 2013;20:2339-2344. • Wanebo HJ, Woodruff JM, Farr GH, et al. Anorectal melanoma. Cancer. 1981 Apr 1; 47(7):1891-900. • Brady MS, Kavolius JP, Quan SH. Anorectal melanoma. A 64-year experience at Memorial Sloan-Kettering Cancer Center.Dis Colon Rectum. 1995 Feb; 38(2):146-51.

  36. References • Kim K B, Sanguino A M, Hodges C, et al. Biochemotherapy in patients with metastatic anorectal mucosal melanoma. Cancer. 2004;100:1478–1483. • Ulmer A, Metzger S, Fierlbeck G. Successful palliation of stenosing anorectal melanoma by intratumoral injections with natural interferon-β.Melanoma Res. 2002;12:395–398. • Atkins MB, Lotze MT, Dutcher JP, et al. High-dose recombinant interleukin 2 therapy for patients with metastatic melanoma: analysis of 270 patients treated between 1985 and 1993.J Clin Oncol. 1999 Jul; 17(7):2105-16. • Snoj M, Rudolf Z, Cemazar M,et al. Successful sphincter-saving treatment of anorectal malignant melanoma with electrochemotherapy, local excision and adjuvant brachytherapy.Anticancer Drugs. 2005 Mar; 16(3):345-8. • Ballo MT, Gershenwald JE, Zagars GK, et al. Sphincter-sparing local excision and adjuvant radiation for anal-rectal melanoma.J Clin Oncol. 2002 Dec 1; 20(23):4555-8. • Moozar KL, Wong CS, Couture J. Anorectal malignant melanoma: treatment with surgery or radiation therapy, or both.Can J Surg. 2003 Oct; 46(5):345-9.

  37. Thank You

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