Desmoid tumors: clinical features and outcome of an unpredictable and challenging manifestation of familial adenomatous polyposis

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art_CAMPOS_Desmoid_tumors_clinical_features_and_outcome_of_an_2015.PDF (750.79 KB)
Citações na Scopus
27
Tipo de produção
article
Data de publicação
2015
DOI
Scopus
Web of Science
PubMed
Título da Revista
ISSN da Revista
Título do Volume
Editora
SPRINGER
Autores
MARTINEZ, Carlos Augusto Real
Citação
FAMILIAL CANCER, v.14, n.2, p.211-219, 2015
Projetos de Pesquisa
Unidades Organizacionais
Fascículo
Resumo
Background/Aims Desmoid tumors (DTs) are rare, locally invasive neoplasms that may affect 10-25 % of familial adenomatous polyposis (FAP) patients. Our aim was to evaluate incidence and clinical presentation among our patients, the potential impact on FAP outcome and to discuss treatment. Materials and methods Charts from 133 FAP (1977-2013) were reviewed. Patients with DTs were separated to retrieve demographic, clinical and management data. Follow-up was focused on disease evolution causing complications or death. Results 19 (14.3 %) DTs were diagnosed, either after previous trauma (16) or during FAP surgery (3). This group comprised 8 men (42.1 %) and 11 women (57.9 %) with an average age of 32.9 years. Intervals from surgical trauma to DTs ranged from 7 to 60 months. ECMs were detected in 12 (63.1 %) patients. DTs were located in the abdominal wall (8), abdominal cavity (8), abdominal wall and cavity (2) and left arm (1). Five patients (26.3 %) referred family history of DTs. Patients presented severe complications such as small bowel obstruction (4) and hydronephrosis (2), being directly responsible for death in three patients. Conclusions (1) DTs developed in 14.3 % of FAP, mostly after surgical trauma; (2) 30 % caused severe morbidity; (3) identification of clinical risk factors may help surgeons to develop screening and therapeutic decisions.
Palavras-chave
Familial adenomatous polyposis, Desmoid tumors, Sulindac, Morbidity
URI
https://observatorio.fm.usp.br/handle/OPI/9780
Referências
  1. BERK T, 1992, CAN J SURG, V35, P393
  2. Bertario L, 2001, INT J CANCER, V95, P102, DOI 10.1002/1097-0215(20010320)95:2<102::AID-IJC1018>3.0.CO;2-8
  3. Bonvalot S, 2008, EJSO-EUR J SURG ONC, V34, P462, DOI 10.1016/j.ejso.2007.06.006
  4. Bulow S, 2003, GUT, V52, P742, DOI 10.1136/gut.52.5.742
  5. Calvert George T., 2012, Sarcoma, P726537, DOI 10.1155/2012/726537
  6. Campos FG, 2011, SURG LAPARO ENDO PER, V21, P327, DOI 10.1097/SLE.0b013e31822b0f65
  7. Campos FG, 2010, POLIPOSE ADENOMATOSA
  8. Campos FG, 2010, J GASTROINTEST SURG, V14, P1943, DOI 10.1007/s11605-010-1288-6
  9. CASPARI R, 1995, HUM MOL GENET, V4, P337, DOI 10.1093/hmg/4.3.337
  10. Church J, 2005, DIS COLON RECTUM, V48, P1528, DOI 10.1007/s10350-005-0018-8
  11. Church J, 2008, DIS COLON RECTUM, V51, P897, DOI 10.1007/s10350-008-9232-5
  12. Church JM, 1995, SEMINARS COLON RECTA, V6, P29
  13. Clark SK, 1998, BRIT J SURG, V85, P970
  14. Clark SK, 1999, BRIT J SURG, V86, P1185, DOI 10.1046/j.1365-2168.1999.01222.x
  15. Dobbie Z, 1996, J MED GENET, V33, P274, DOI 10.1136/jmg.33.4.274
  16. Elayi E, 2009, DIS COLON RECTUM, V52, P1762, DOI 10.1007/DCR.0b013e3181b5518a
  17. Fallen T, 2006, FAM CANCER, V5, P191, DOI 10.1007/s10689-005-5959-5
  18. Fiore M, 2009, ANN SURG ONCOL, V16, P2587, DOI 10.1245/s10434-009-0586-2
  19. Galle TS, 1999, SCAND J GASTROENTERO, V34, P808
  20. Groen EJ, 2008, ANN SURG ONCOL, V15, P2439, DOI 10.1245/s10434-008-9981-3
  21. GURBUZ AK, 1994, GUT, V35, P377, DOI 10.1136/gut.35.3.377
  22. Hartley JE, 2004, DIS COLON RECTUM, V47, P334, DOI 10.1007/s10350-003-0063-0
  23. Heiskanen I, 1996, INT J COLORECTAL DIS, V11, P157, DOI 10.1007/s003840050034
  24. JONES IT, 1986, ANN SURG, V204, P94, DOI 10.1097/00000658-198607000-00014
  25. Latchford AR, 2006, BRIT J SURG, V93, P1258, DOI 10.1002/bjs.5425
  26. Lefevre JH, 2008, BRIT J SURG, V95, P1136, DOI 10.1002/bjs.6241
  27. Lynch HT, 2001, DIGEST DIS SCI, V46, P2325, DOI 10.1023/A:1012330626600
  28. Lynch HT, 1996, AM J GASTROENTEROL, V91, P2598
  29. Middleton SB, 2003, DIS COLON RECTUM, V46, P481, DOI 10.1097/01.DCR.0000054676.08829.DC
  30. Mitchell G, 1998, Sarcoma, V2, P149, DOI 10.1080/13577149877902
  31. Moeslein G, 2006, FAM CANCER, V5, P287, DOI 10.1007/s10689-005-5677-z
  32. Nieuwenhuis MH, 2008, CLIN GASTROENTEROL H, V6, P215, DOI 10.1016/j.cgh.2007.11.011
  33. Nieuwenhuis MH, 2011, BRIT J CANCER, V104, P37, DOI 10.1038/sj.bjc.6605997
  34. Nieuwenhuis MH, 2011, INT J CANCER, V129, P256, DOI 10.1002/ijc.25664
  35. Nieuwenhuis Marry H, 2010, Ned Tijdschr Geneeskd, V154, pA2235
  36. PENNA C, 1993, SURG GYNECOL OBSTET, V177, P263
  37. RODRIGUEZBIGAS MA, 1994, CANCER, V74, P1270, DOI 10.1002/1097-0142(19940815)74:4<1270::AID-CNCR2820740415>3.0.CO;2-7
  38. Anaya Daniel A, 2008, Clin Colon Rectal Surg, V21, P263, DOI 10.1055/s-0028-1089941
  39. Sinha A, 2012, BRIT J RADIOL, V85, P254
  40. Sinha A, 2011, COLORECTAL DIS, V13, P1222, DOI 10.1111/j.1463-1318.2010.02345.x
  41. Soravia C, 2000, DIS COLON RECTUM, V43, P363, DOI 10.1007/BF02258303
  42. Speake D, 2007, BRIT J SURG, V94, P1009, DOI 10.1002/bjs.5633
  43. Sturt NJH, 2006, FAM CANCER, V5, P275, DOI 10.1007/s10689-005-5675-1
  44. Tulchinsky H, 2005, ARCH SURG-CHICAGO, V140, P159, DOI 10.1001/archsurg.140.2.159
  45. Vasen HFA, 2008, GUT, V57, DOI 10.1136/gut.2007.136127
  46. Vogel JD, 2005, DIS COLON RECTUM, V48, P662
  47. Wallis YL, 1999, J MED GENET, V36, P14
  48. Xhaja X, 2013, COLORECTAL DIS, V15, P1489, DOI 10.1111/codi.12416

Coleções
Artigos e Materiais de Revistas Científicas - FM/MGT
Artigos e Materiais de Revistas Científicas - HC/ICHC
Artigos e Materiais de Revistas Científicas - LIM/35
Artigos e Materiais de Revistas Científicas - ODS/03