Objectives: We report a case of primary melanoma of the small intestine. Primary intestinal melanoma (PIM) is an extremely rare neoplasm for which the cause is unknown.



 Materials and methods: A 67-year-old man was admitted to our department due to abdominal pain, constipation, a large, hard inguinal mass and severe anaemia. 



Results: After laboratory data, imaging techniques and histopathological examination, the diagnosis was confirmed. A surgical resection of the intestinal neoplasm, treatment with BRAF inhibitors and radiation therapy to the inguinal mass were performed.



Conclusion: PIM is rare and it is usually difficult to establish its exact origin

Gómez-­‐Moyano, Elisabeth

Herrero-­‐Lifona, Leticia

Pérez-­‐Belmonte, Luis M.

Pérez-­‐Díaz, José M.

Santín-­‐Piñero, Emilio

European Journal of Case Reports in Internal Medicine

Objectives: We report a case of primary melanoma of the small intestine. Primary intestinal melanoma (PIM) is an extremely rare neoplasm for which the cause is unknown.

 Materials and methods: A 67-year-old man was admitted to our department due to abdominal pain, constipation, a large, hard inguinal mass and severe anaemia. 

Results: After laboratory data, imaging techniques and histopathological examination, the diagnosis was confirmed. A surgical resection of the intestinal neoplasm, treatment with BRAF inhibitors and radiation therapy to the inguinal mass were performed.

Conclusion: PIM is rare and it is usually difficult to establish its exact origin

Luis M. Pérez-Belmonte

Emilio Santín-Piñero

Leticia Herrero-Lifona

Elisabeth Gómez-Moyano

José M. Pérez-Díaz

Directory of Open Access Journals

 	  Doi:	  10.12890/2014_000147	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   European	  Journal	  of	  Case	  Reports	  in	  Internal	  Medicine	   	  	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   ©	  EFIM	  2014	  Primary	  Melanoma	  of	  the	  Small	  Intestine	  with	  a	  Metastatic	  Lymph	  Mass	  in	  the	  Inguinal	  Canal:	  A	  Case	  Report	  	  Luis	  M.	  Pérez-­‐Belmontea,	  Emilio	  Santín-­‐Piñeroa,	  Leticia	  Herrero-­‐Lifonab,	  Elisabeth	  Gómez-­‐Moyanoc,	  José	  M.	  Pérez-­‐Díaza	  	  aDepartment	  of	  Internal	  Medicine,	  Axarquía	  County	  Hospital,	  Vélez-­‐Málaga,	  Spain	  bDepartment	  of	  Allergology,	  University	  Regional	  Hospital	  of	  Málaga,	  Málaga,	  Spain	  cDepartment	  of	  Dermatology,	  University	  Regional	  Hospital	  of	  Málaga,	  Málaga,	  Spain	  	  	  Abstract	  	  Objectives:	   We	   report	   a	   case	   of	   primary	   melanoma	   of	   the	   small	   intestine.	   Primary	   intestinal	   melanoma	   (PIM)	   is	   an	  extremely	  rare	  neoplasm	  for	  which	  the	  cause	  is	  unknown.	   	  	  Materials	   and	  methods:	   A	   67-­‐year-­‐old	  man	  was	   admitted	   to	   our	   department	   due	   to	   abdominal	   pain,	   constipation,	   a	  large,	  hard	  inguinal	  mass	  and	  severe	  anaemia.	   	   	  	  Results:	  After	   laboratory	  data,	   imaging	   techniques	  and	  histopathological	  examination,	   the	  diagnosis	  was	  confirmed.	  A	  surgical	  resection	  of	  the	  intestinal	  neoplasm,	  treatment	  with	  BRAF	  inhibitors	  and	  radiation	  therapy	  to	  the	  inguinal	  mass	  were	  performed.	   	  	  Conclusion:	  PIM	  is	  rare	  and	  it	  is	  usually	  difficult	  to	  establish	  its	  exact	  origin.	   	  	  Keywords:	  Abdominal	  pain,	  small	  intestine,	  melanoma	  	  	  	  	  Received:	  19/09/2014	  Accepted:	  27/09/2014	  Published:	  03/10/2014	  How	  to	  cite	  this	  article:	  Pérez-­‐Belmonte	  LM,	  Santín-­‐Piñero	  E,	  Herrero-­‐Lifona	  L,	  Gómez-­‐Moyano	  E,	  Pérez-­‐Díaz	  JM.	  Primary	  Melanoma	  of	  the	  Small	  Intestine	  with	  a	  Metastatic	  Lymph	  Mass	  in	  the	  Inguinal	  Canal:	  A	  Case	  Report,	  EJCRIM	  2014;1:doi:	  10.12890/2014_000147	  Conflicts	  of	  Interests:	  The	  authors	  declare	  that	  they	  have	  no	  conflicts	  of	  interest	  related	  to	  this	  research.	  	   	  Doi:	  10.12890/2014_000147	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   European	  Journal	  of	  Case	  Reports	  in	  Internal	  Medicine	   	  	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   ©	  EFIM	  2014	  Introduction	  Intestinal	  melanomas	  can	  be	  primary	  tumours	  or	  metastases	  of	  cutaneous,	  ocular	  or	  anal	  melanomas.	  Most	  melanomas	  in	   the	  small	   intestine	  are	  metastases	   from	  primary	  cutaneous	   lesions,	  but	  a	  melanoma	  can	  also	  develop	  as	  a	  primary	  mucosal	  tumour	  in	  the	  intestinal	  tract	  (less	  than	  2%)	  [1].	  Although	  it	  is	  important	  to	  distinguish	  primary	  from	  metastatic	  disease,	  establishing	  the	  correct	  origin	  of	  small	  bowel	  melanoma	  can	  be	  difficult	  or	  impossible.	  In	  this	  article,	  we	  report	  a	  patient	  presenting	  with	  primary	  melanoma	  of	  the	  small	  intestine	  [2].	  Case	  report	   	  A	  67-­‐year-­‐old	  German	  man	  was	  admitted	  to	  the	  Department	  of	  Internal	  Medicine	  due	  to	  3	  months	  of	  intermittent	  and	  diffuse	  abdominal	  pain,	  progressive	  constipation,	  vomiting,	  melaena	  and	  weight	   loss	   (over	  20	  kg).	  Also,	  he	   referred	  a	  large	  (9×6	  cm)	  and	  hard	  inguinal	  mass	  on	  the	  right	  side	  that	  had	  gradually	  grown	  in	  the	  previous	  6	  weeks	  (Fig.	  1).	   	  No	  other	  significant	  findings	  were	  noted	  at	  physical	  examination	  and	  his	  medical	  history	  was	  remarkable	  only	  for	  heavy	  smoking	  (60	  pack-­‐years)	  and	  alcohol	  abuse	  (120	  g/day).	   	  Laboratory	  data	  were	  within	  normal	  limits	  except	  for	  severe	  iron-­‐deficiency	  anaemia.	  Abdominal	  ultrasound,	  barium	  examination	  and	  computerized	  tomography	  revealed	  an	   infiltrative	  mass	   that	   involved	  segments	   of	   ileal	   loop	  with	   aneurismal	   dilatation	   and	   a	   large	   inguinal	   lymph	  mass	  without	   other	   pathological	   lymph	  nodes	  (Fig.	  2).	   	  A	   whole	   positron	   emission	   scan	   confirmed	   these	   findings	   (Fig.	   3)	   and	   together	   with	   total	   body	   computerized	  tomography	  and	  urological,	  ocular	  and	  anal	  examinations	  ruled	  out	  malignancies	  in	  other	  locations.	   	   	   	  	  	  	  	  	  	  	  Figure	  1:	  Inguinal	  mass	   Figure	  2:	  Computer	  tomography	  image	  Figure	  3:	  Positron	  emission	  scan	  image	   	  Doi:	  10.12890/2014_000147	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   European	  Journal	  of	  Case	  Reports	  in	  Internal	  Medicine	   	  	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   ©	  EFIM	  2014	  After	   several	   blood	   transfusions	   and	   intensive	   intravenous	   iron	   therapy,	   a	   surgical	   resection	   of	   the	   ileal	   mass	   was	  performed.	  During	  laparotomy,	  an	  8-­‐cm	  mass	  was	  identified,	  involving	  segments	  of	  the	  ileum.	  There	  was	  no	  evidence	  of	  other	  abdominal	  lesions.	   	  The	  pathological	  examination	  of	  the	  resected	  specimen	  confirmed	  the	  diagnosis	  of	  melanoma	  of	  the	  small	  intestine.	  It	  showed	  a	  precursor	  lesion	  in	  the	  intestinal	  mucosa.	  The	  BRAF	  Val600	  mutation	  was	  positive.	  Based	  on	  these	   findings	  and	  the	  patient’s	  clinical	  presentation,	  he	  was	  given	  a	  diagnosis	  of	  primary	  melanoma	  of	   the	  small	  intestine	  with	  a	  metastatic	  lymph	  mass	  in	  the	  inguinal	  canal.	  The	   postoperative	   course	   was	   uneventful	   and	   the	   clinical	   evolution	   was	   initially	   favourable,	   without	   requiring	  more	  blood	   transfusions	   or	   iron	   supplements.	   The	   patient	   underwent	   a	   combination	   treatment	   with	   BRAF	   inhibitors	   and	  radiation	   therapy	   to	   the	   inguinal	  mass,	   improving	   his	   physical	   status	   and	   reducing	   the	  mass.	   Four	  months	   later,	   his	  physical	  condition	  gradually	  worsened	  and	  the	  inguinal	  mass	  grew,	  with	  several	  inguinal	  lymph	  nodes	  appearing	  on	  the	  right	  side.	  At	  the	  present	  time,	  the	  patient	  is	  undergoing	  palliative	  care.	   	   	   	   	   	  	  Discussion	  The	  vast	  majority	  of	  cases	  of	  intestinal	  melanomas	  are	  metastatic	  lesions,	  originating	  from	  a	  primary	  cutaneous	  lesion	  or,	  less	  frequently,	  an	  ocular,	  urological	  or	  rectal	  lesion.	  Melanoma	  can	  also	  develop	  as	  a	  primary	  mucosal	  neoplasm	  in	  the	  gastrointestinal	  tract,	  although	  it	  is	  extremely	  rare	  [1].	  Establishing	  the	  exact	  origin	  (primary	  or	  secondary)	  of	  small	  bowel	  melanoma,	  especially	  metastatic	   lesions	  from	  an	  unknown	  or	  a	  regressed	  primary	  cutaneous	  melanoma	  can	  be	  difficult	   or	   impossible	   [3].	   In	   this	   case,	   on	   further	   extensive	   dermatological,	   ophthalmological,	   urological	   and	   rectal	  examination,	  we	  did	  not	  have	  evidence	  of	  another	  primary	  lesion,	  giving	  the	  diagnosis	  of	  primary	  melanoma	  of	  the	  small	  intestine	  with	   a	  metastatic	   lymph	  mass	   in	   the	   inguinal	   canal.	   The	   histopathological	   examination	   also	   supported	   this	  diagnosis.	  It	  usually	  shows	  a	  precursor	  lesion	  or	  melanosis	  in	  the	  intestinal	  mucosa,	  although	  histopathological	  features	  should	  not	  be	  considered	  alone	  [2,	  4].	   	  Men	  are	  more	  prone	  to	  primary	  intestinal	  melanoma	  (PIM)	  than	  women,	  and	  the	  ileum	  is	  the	  most	  commonly	  affected	  area	   [1,	   5].	   In	   addition,	   it	   tends	   to	   be	   more	   aggressive	   and	   is	   associated	   with	   a	   worse	   prognosis	   than	   cutaneous	  melanoma.	  It	  is	  a	  result	  of	  aggressive	  tumour	  behaviour	  with	  fast	  growth	  in	  the	  rich	  vascular	  and	  lymphatic	  supply	  of	  the	  intestinal	  mucosa	  and	  late	  diagnosis	  [2,	  3].	  The	  clinical	  picture	  of	  small	  bowel	  melanoma	  is	  similar	  to	  the	  clinical	  presentation	  of	  other	  tumours	  involving	  the	  small	  intestine.	  The	  most	  common	  symptoms	  are	  abdominal	  pain,	  intestinal	  obstruction,	  constipation,	  haematosis,	  melaena,	  anaemia,	  fatigue,	  weight	  loss	  and	  the	  presence	  of	  a	  palpable	  abdominal	  mass.	  Substantial	  lymphadenopathies	  can	  also	  be	  found.	   	  Diagnosis	   should	   be	  made	   by	   a	   combination	   of	   imaging	   techniques	   to	   improve	   the	   sensitivity	   and	   specificity	   of	   the	  radiological	   imaging.	   Transabdominal	   ultrasonography,	   conventional	   barium	   contrast	   studies,	   endoscopy,	   computer	  tomography	  and	  positron	  emission	  scan	  may	  be	  essential	  for	  diagnosis	  of	  small	  bowel	  melanoma.	  However,	  the	  rate	  of	  clinical	  detection	  is	  low	  (only	  10%–20%)	  [2,	  3].	  No	   standard	   therapy	   exists	   for	   the	   treatment	   of	   intestinal	  melanoma,	   although	   surgical	   removal	   is	   the	   treatment	   of	  choice	  in	  all	  patients	  with	  resectable	  melanoma	  [1,	  3].	  Wide	  surgical	  resection	  of	  the	  tumour	  with	  sufficient	  free	  margins	   	  Doi:	  10.12890/2014_000147	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   European	  Journal	  of	  Case	  Reports	  in	  Internal	  Medicine	   	  	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   	   ©	  EFIM	  2014	  proximally	  and	  distally	  from	  the	  lesion,	  together	  with	  a	  subtended	  wedge	  of	  mesentery	  to	  remove	  regional	  lymph	  nodes,	  is	  recommended.	  Treatment	  of	  PIM	  with	  chemotherapy	  has	  very	  low	  response	  rates	  [1,	  2].	  In	  conclusion,	  melanoma	  typically	  develops	  where	  melanocytes	  are	  found	  (skin,	  eyes,	  meninges	  and	  anal	  region)	  but	  can	  also	  develop	  as	  a	  primary	  tumour	  in	  the	  small	   intestine,	  although	  this	   is	  very	  rare.	  However,	  the	  majority	  of	  cases	  are	  metastases	   from	   cutaneous	   melanoma.	   This	   rare	   case	   report	   is	   interesting	   because	   we	   present	   a	   PIM	   with	   a	   large	  metastatic	   lymph	  mass	   in	  the	  inguinal	  canal,	  presenting	  a	  diagnostic	  dilemma	  for	  us.	  PIM	  should	  be	  considered	  in	  the	  differential	   diagnosis	   of	   small	   bowel	   tumours,	   which	   usually	   remain	   a	   challenge	   for	   radiologists,	   pathologists	   and	  clinicians.	   	  	  Learning	  Points	  	  • PIM	   is	  an	  extremely	  rare	  neoplasm	  which	  should	  be	  considered	   in	   the	  differential	  diagnosis	  of	  small	   intestine	  tumours.	  • A	  clear	  distinction	  between	  PIM	  and	  metastatic	  intestinal	  melanoma	  can	  be	  difficult.	   	  • A	  combination	  of	  imaging	  techniques	  is	  crucial	  for	  its	  early	  diagnosis.	   	  	  References	  	   	  1. Patel	  K,	  Ward	  ST,	  Packer	  T,	  Brown	  S,	  Marsden	  J,	  Thomson	  M	  et	  al.	  Malignant	  melanoma	  of	  the	  gastro-­‐intestinal	  tract:	  a	  case	  series,	  Int	  J	  Surg	  2014;12:523–527.	   	  2. Lens	  M,	  Bataille	  V,	  Krivokapic	  Z.	  Melanoma	  of	  the	  small	  intestine,	  Lancet	  Oncol	  2009;10:516–521.	   	  3. Schuchter	  LM,	  Green	  R,	  Fraker	  D.	  Primary	  and	  metastatic	  diseases	  in	  malignant	  melanoma	  of	  the	  gastrointestinal	  tract,	  Curr	  Opin	  Oncol	  2000;12:181–185.	   	  4. Blecker	  D,	  Abraham	  S,	  Furth	  EE,	  Kochman	  ML.	  Melanoma	  in	  the	  gastrointestinal	  tract,	  Am	  J	  Gastroenterol	  1999;94:3427–3433.	   	  5. Resta	  G,	  Anania	  G,	  Messina	  F.	  Jejuno-­‐jejunal	  invagination	  due	  to	  intestinal	  melanoma,	  World	  J	  Gastroenterol	  2007;13:310–312.	   	  	  

Primary Melanoma of the Small Intestine with a Metastatic Lymph Mass in the Inguinal Canal: A Case Report

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Primary Melanoma of the Small Intestine with a Metastatic Lymph Mass in the Inguinal Canal: A Case Report

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