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Scientific letter
Anorectal melanoma: An atypical cause of rectorrhagia
Causa atípica de rectorragia: melanoma anorrectal
Cristina Saldaña Dueñasa,
Corresponding author
crisaldu@hotmail.com

Corresponding author.
, Silvia Goñi Esartea, Jose Francisco Juanmartiñena Fernándeza, Marta Montes Diazb, Rosa Iglesias Picazoa
a Digestive Department, Complejo Hospitalario de Navarra, Pamplona, Spain
b Department of Pathology, Complejo Hospitalario de Navarra, Pamplona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of an 81-year-old man with previous diagnose of hypercholesterolemia&#44; benign prostate hyperplasia and seborrheic keratosis&#46; He presented a two-months history of rectorrhagia episodes without any other symptoms&#46; In the laboratory tests there was no anemia and the tumor markers were negative&#46; A rectoscopy was performed identifying from the anus to 6<span class="elsevierStyleHsp" style=""></span>cm beyond the dental line a pseudo-polyp mameloned tumor with hyperpigmented base evolving a quarter of the whole circumference of the rectum &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>A&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">The biopsies showed extended small round cells proliferation with necrosis&#46; Neither melanic pigment nor mucosa was observed probably because superficial biopses where taken on the surface of the tumor&#46; The immunophenotype was negative to S-100 and positive to Melan-A and MITF &#40;microphthalmia-associated transcription factor&#41; so the diagnose of a rectal melanoma was performed &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>A and B&#41;&#46; The study was completed with colonoscopy &#40;three adenomatous polyps were removed&#41;&#44; pelvic magnetic resonance &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>B&#41; and computed tomography of the thorax and abdomen without mesorectal lymph nodes or distal metastasis&#46; The case was presented in the multidisciplinary comity and a surgical approach was chosen&#46; An endoanal resection was performed&#44; because of the absence of distal or local invasion and the patient age and comorbility&#44; without complications&#46; The surgical piece showed deep infiltration even throws muscular propia&#44; an ulcerated mucosa and the presence of melanophages&#46; The patient remains asymptomatic and with good quality of life&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Mucosal melanomas &#40;MM&#41; are rare &#40;0&#46;4&#8211;1&#46;6&#37; of total malignant melanomas&#41;&#46; Both cutaneous and mucosal melanomas contain melanocytes&#44; but have important differences such as the exposure of ultraviolet light &#40;clearly related with cutaneous melanoma&#41;&#44; localization&#44; appearance &#40;40&#37; in MM are amelanotic&#41; age of presentation and prognosis &#40;poorly in the case of MM&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1&#8211;5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The anus is the third most common location of melanomas after the skin and the eyes&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1&#44;3&#44;4</span></a> MM arise primarily in the head and neck &#40;55&#37;&#41;&#44; anorectal &#40;24&#37;&#41; and vulvovaginal &#40;18&#37;&#41; regions and are in 20&#37; of cases multifocal&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1&#44;3&#44;5</span></a> They are frequently found within 6<span class="elsevierStyleHsp" style=""></span>cm of the anal rim&#58; rectum &#40;42&#37;&#41;&#44; anal canal &#40;33&#37;&#41; or both&#46; Other infrequent sites include urinary tract&#44; gall bladder and small intestine&#46; The median age of presentation is 70 years &#40;except those located in oral cavity found in younger people&#41;&#46; There is a female preponderance of cases and HIV infection has been implicated as a risk factor for anal localization in young male patients&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1&#44;2&#44;6</span></a> The incidence of anorectal melanoma &#40;AM&#41; increases over time&#44; and it remains highly lethal&#44; with a 5-year survival rate of 6&#37; to 22&#37;&#46; The simplified staging system which was originally developed for melanomas of the head and neck in 1970 by Ballantyne is used for the primary site including three stages Stage I&#58; Localized disease&#59; Stage II&#58; Locoregional nodal involvement and Stage III&#58; distant metastatic involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">7</span></a> The most common presenting symptoms are nonspecific and include rectal bleeding followed by pain&#44; the sensation of a mass&#44; constipation&#44; diarrhea&#44; abdominal pain&#44; weight loss&#44; and tenesmus&#46; All of them present in such common conditions as are hemorrhoids&#44; rectal polyp or prolapse&#46; As the diagnose is usually late the tumors may attain a large size before being correctly diagnosed&#46; Metastases are present at the time of diagnosis in more than half of patients&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">2&#44;4&#44;5&#44;7-9</span></a> Although melanomas develop throughout the anus&#44; two thirds arise in the proximal portion of the pecten and transitional zone around the dentate line &#40;within 6<span class="elsevierStyleHsp" style=""></span>cm of the anal rim&#41;&#46; Twenty to fifty percent of tumors are pigmented but others remain amelanotic&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">1&#44;2</span></a> Histologically the cells are often large with an epithelioid appearance and finely distributed pigment giving the cytoplasm a dusty-tan appearance&#46; We can find large hyperchromatic nuclei with well-dispersed chromatin&#44; multinucleated and polymorphic giant cells&#46; Some melanomas &#40;small round cell melanomas&#41; contain atypical round cells resembling lymphocytes&#46; Intraepithelial extensions of malignant melanoma may be confused with Paget disease&#46; Immunohistochemistry studies can be useful&#44; as AM stains positive for anti S-100 protein&#44; HMB-45 &#40;as the S-100 is a monoclonal antibody&#44; HMB-45 that reacts against an antigen present in melanocytic tumors such as melanomas&#44; and stands for human melanoma black 45&#41; and Melan-A&#44; while are usually negative for AE1&#47;AE3 cytokeratines&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">4</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">A correct diagnose includes radiological exams including tomography scan &#40;for the evaluation of distal dissemination&#41;&#44; magnetic resonance &#40;for local evolving&#41; and even endoscopic procedures such as ecoendoscopy for a detailed examination of the area in order to a better surgical approach&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">8&#44;9</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Given the rarity of this neoplasia&#44; optimal treatment is still controversial and surgery seems to be the only curative treatment&#46; Surgical approaches include wide local excision &#40;WLE&#41; and abdominoperineal resection &#40;APR&#41;&#44; although no one has demonstrated survival advantage&#46; However with the wide and complete resection &#40;R0 oncological criteria&#41; the surveillance of patients for 5 years rounds the 19&#37; in contrast to the 6&#37; if the margins are affected&#46;<span class="elsevierStyleSup">7&#44;</span><a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">8&#44;10&#44;11</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">WLE is the procedure of choice when the complete excision of the tumor is possible&#44; as it is accompanied by a less morbidity rate and less functional limitations&#46; In contrast&#44; it presents higher rates of local recurrence&#46; If is not possible the complete excision with WLE&#44; in this situation&#44; APR and local radiotherapy may improve loco-regional control&#44; despite a lack of demonstrable impact upon overall survival&#46;<a class="elsevierStyleCrossRefs" href="#bib0115"><span class="elsevierStyleSup">8&#8211;11</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In conclusion&#44; primary anorectal melanoma is an extremely rare and aggressive malignancy with poor prognosis that it is often misdiagnosed&#46; High suspicious is mandatory to achieve early-diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">2&#44;8&#44;9</span></a></p></span>"
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es en pt

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