An aggressive case of anorectal melanoma · 2015-03-31 · An aggressive case of anorectal melanoma...
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GE J Port Gastrenterol. 2014;21(4):169---171 www.elsevier.pt/ge IMAGES IN GASTROENTEROLOGY AND HEPATOLOGY An aggressive case of anorectal melanoma Um caso agressivo de melanoma anorretal Susana Marques a,b,∗ , Raquel Silva a,b , Miguel Bispo a,b , Francisco Silva a,b , Leopoldo Matos a,b a Gastroenterology Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal b Medicine II Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal Received 13 May 2013; accepted 10 June 2013 Available online 6 January 2014 Introduction All melanomas originate from the melanocyte. They may arise not only from the skin, but also from mucosal epithe- lium lining of the respiratory, alimentary and genitourinary tracts. Anorectal melanoma accounts for 1---3% of all anal tumors and 0.3% of all melanoma. The majority of cases arise from the mucocutaneous junction (dentate line). 1,2 It usually occurs in the 5th or 6th decades of life. The most common presenting complaints are bleeding, anal pain or mass, pruritus, tenesmus and change in bowel habits. Weigh loss, anemia and fatigue may indicate metastatic dis- ease. At presentation, 60% of the patients have lymph node involvement and 30% have distant metastases. This very rare tumor can be classified into three different stages of disease progression: localized disease (I), regional lymph node involvement (II) and distant metastases disease (III), which account for a median survival of 24, 17 and 8 months respectively. 3 Surgical resection is the gold standard treatment. Chemotherapy and radiation therapy alone have not been shown to be effective, but may provide some benefit when used as adjuvants. Despite treatment, the prognosis remains poor. 4 ∗ Corresponding author. E-mail address: [email protected] (S. Marques). Case report This case presents a 55-year-old female patient with a past medical history significant for hemorrhoids and prior depres- sion who was admitted to our medical facility for syncope. As associated symptoms, she reported intermittent rectal bleeding, usually after a bowel movement, and involuntary weigh loss over the past 6 months. Due to family history of colorectal cancer in her father at the age of 47, she had had a total colonoscopy the year before which confirmed just internal hemorrhoids. She also had had CEA and CA 19.9 measurements two months prior to admission and both markers were within normal range. On physical examination, conjunctiva pallor was noted and digital rectal exam revealed a 5 cm rectal mass palpated 3 cm from the anal verge. Admission laboratory studies were significant for iron-deficiency anemia. Chest X-ray showed multiple bilateral pulmonary cannon ball nodules suggestive of metastasis (Fig. 1). Flexible sigmoidoscopy was performed. A dark polypoid lesion measuring 5 cm in diameter was identified at the anorectal junction (Fig. 2). Histologically, tumor cells con- tained apparent brown pigment and were positive for S-100 protein and Melan-A immunohistochemical staining. These findings were consistent with the diagnosis of anorectal melanoma. Tumor staging included standard head, thoracic, abdom- inal and pelvic CT imaging. Multiple bilateral pulmonary 0872-8178/$ – see front matter © 2013 Sociedade Portuguesa de Gastrenterologia. Published by Elsevier España, S.L.U. All rights reserved. http://dx.doi.org/10.1016/j.jpg.2013.06.002
Transcript of An aggressive case of anorectal melanoma · 2015-03-31 · An aggressive case of anorectal melanoma...
GE J Port Gastrenterol. 2014;21(4):169---171
www.elsevier.pt/ge
IMAGES IN GASTROENTEROLOGY AND HEPATOLOGY
An aggressive case of anorectal melanoma
Um caso agressivo de melanoma anorretal
Susana Marquesa,b,∗, Raquel Silvaa,b, Miguel Bispoa,b, Francisco Silvaa,b,Leopoldo Matosa,b
a Gastroenterology Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugalb Medicine II Department, Centro Hospitalar de Lisboa Ocidental, Lisboa, Portugal
Received 13 May 2013; accepted 10 June 2013Available online 6 January 2014
Introduction
All melanomas originate from the melanocyte. They mayarise not only from the skin, but also from mucosal epithe-lium lining of the respiratory, alimentary and genitourinarytracts. Anorectal melanoma accounts for 1---3% of all analtumors and 0.3% of all melanoma. The majority of casesarise from the mucocutaneous junction (dentate line).1,2
It usually occurs in the 5th or 6th decades of life. Themost common presenting complaints are bleeding, anal painor mass, pruritus, tenesmus and change in bowel habits.Weigh loss, anemia and fatigue may indicate metastatic dis-ease. At presentation, 60% of the patients have lymph nodeinvolvement and 30% have distant metastases.
This very rare tumor can be classified into three differentstages of disease progression: localized disease (I), regionallymph node involvement (II) and distant metastases disease(III), which account for a median survival of 24, 17 and8 months respectively.3
Surgical resection is the gold standard treatment.Chemotherapy and radiation therapy alone have not beenshown to be effective, but may provide some benefit whenused as adjuvants. Despite treatment, the prognosis remainspoor.4
∗ Corresponding author.E-mail address: [email protected] (S. Marques).
Case report
This case presents a 55-year-old female patient with a pastmedical history significant for hemorrhoids and prior depres-sion who was admitted to our medical facility for syncope.As associated symptoms, she reported intermittent rectalbleeding, usually after a bowel movement, and involuntaryweigh loss over the past 6 months.
Due to family history of colorectal cancer in her fatherat the age of 47, she had had a total colonoscopy theyear before which confirmed just internal hemorrhoids. Shealso had had CEA and CA 19.9 measurements two monthsprior to admission and both markers were within normalrange.
On physical examination, conjunctiva pallor was notedand digital rectal exam revealed a 5 cm rectal mass palpated3 cm from the anal verge. Admission laboratory studies weresignificant for iron-deficiency anemia. Chest X-ray showedmultiple bilateral pulmonary cannon ball nodules suggestiveof metastasis (Fig. 1).
Flexible sigmoidoscopy was performed. A dark polypoidlesion measuring 5 cm in diameter was identified at theanorectal junction (Fig. 2). Histologically, tumor cells con-tained apparent brown pigment and were positive for S-100protein and Melan-A immunohistochemical staining. Thesefindings were consistent with the diagnosis of anorectalmelanoma.
Tumor staging included standard head, thoracic, abdom-inal and pelvic CT imaging. Multiple bilateral pulmonary
0872-8178/$ – see front matter © 2013 Sociedade Portuguesa de Gastrenterologia. Published by Elsevier España, S.L.U. All rights reserved.http://dx.doi.org/10.1016/j.jpg.2013.06.002
170 S. Marques et al.
Figure 1 Chest X-ray showing multiple canon ball nodules.
metastases of varying sizes were observed on thoracic CT(Fig. 3). In addition, head CT revealed six lesions involv-ing the frontal and temporal lobes bilaterally, some showingnecrotic degeneration. The greatest was 3 cm in diameterand presented extensive surrounding edema, causing lateralventricular displacement (Fig. 4).
She was immediately placed on high dose corticosteroidtherapy and prophylactic antiepileptic drugs. On day 5, shedeveloped rapid focal progressive neurological deteriorationwith confusion, aphasia, right hemiparesis and sphinctercontrol loss. Due to the advanced tumor stage, she was dis-charged and referred to a palliative care center just oneweek after admission. She passed away 8 weeks after theinitial diagnosis.
Ethical responsibilities
Protection of human and animal subjects. The authorsdeclare that no experiments were performed on humans oranimals for this investigation.
Figure 2 Polypoid lesion identified with flexible sigmoi-doscopy.
Figure 3 Thoracic CT confirming bilateral pulmonar metasta-sis.
Figure 4 Head CT showing a right frontal and temporal lobelesion.
Confidentiality of Data. The authors declare that theyhave followed the protocols of their work center on thepublication of patient data and that all the patients includedin the study have received sufficient information and havegiven their informed consent in writing to participate in thatstudy.
Right to privacy and informed consent. The authorsdeclare that no patient data appear in this article.
Conflicts of interest
The authors have no conflicts of interest to declare.
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References
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2. Cagir B, Whiteford MH, Topham A, Rakinic J, Fry RD. Chang-ing epidemiology of anorectal melanoma. Dis Colon Rectum.1999;42:1203---8.
3. Iddings DM, Fleisig AJ, Chen SL, Faries MB, Morton DL. Practicepatterns and outcomes for anorectal melanoma in the USA,reviewing three decades of treatment: is more extensive sur-gical resection beneficial in all patients? Ann Surg Oncol.2010;17:40---4.
4. Singer M, Mutch MG. Anal melanoma. Clin Colon Rectal Surg.2006;19:78---87.
