Hamartoma of the abdominal cavity and retroperitoneum
– a review and a case report

Czech version

Authors: A. Paclík; P. Dytrych; D. Hoskovec; R. Jakša; Z. Krška
Authors‘ workplace: I. chirurgická klinika 1. LF Univerzity Karlovy a VFN v Praze přednosta: prof. MUDr. Z. Krška, DrSc.
Published in: Rozhl. Chir., 2017, roč. 96, č. 9, s. 375-382.
Category: Review

Overview

Methods:
We have reviewed recent as well as older literature with the intention of compiling a summary report on hamartomas of the abdominal cavity and retroperitoneum.

Introduction:
Hamartoma of the abdominal cavity and retroperitoneum is a rare condition which has received relatively little attention.

Results:
The most commonly affected organs are the liver, spleen, digestive tract, pancreas and kidneys. By its nature, hamartoma is a benign lesion with a good prognosis. However, neoplastic transformation may occur and hamartomas need to be regarded as potentially malignant.

Patients are very often asymptomatic. Signs and symptoms depend on the organ involved and are caused, to different degrees, by obstruction, surrounding tissue compression, rupture or tumor bleeding. In imaging diagnostics, it is usually not possible to safely distinguish between a hamartoma and a malignant lesion. Therefore, the patient is preferably indicated for surgery (or endoscopic removal) for the purpose of histological verification and definitive treatment.

Conclusion:
Hamartomas of the abdominal cavity and retroperitoneum are relatively rare, benign and often asymptomatic diseases which, however, may manifest as compression, obstruction, perforation, bleeding or even malignant transformation.

The recent case report of splenic hamartoma presented at the end of this review proves that surgical management is often the only rational option to obtain definitive confirmation of the diagnosis.

Key words:
splenic hamartoma − hamartoma of the liver − hamartoma of the pancreas − hamartomatous polyps of the colon − multiple hamartoma syndrome

Sources

1. Manxhuka-Kerliu S, Sahatciu-Meka V, Kerliu I, et al. Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature. J Med Case Rep 2014;8:321.

2. Jelsig AM, Qvist N, Brusgaard K, et al. Hamartomatous polyposis syndromes: a review. Orphanet J Rare Dis 214;9:101.

3. Tanaka N, Seya T, Onda M, et al. Myoepithelial hamartoma of the small bowel: report of a case. Surg Today 1996;26:1010−13.

4. Theodosiou E, Voulalas G, Salveridis N, et al. Neuromesenchymal hamartoma of small bowel-an extremely rare entity: a case report. World J Surg Oncol 2009;7:92.

5. Brosens LA, Langeveld D, van Hattem WA, et al. Juvenile polyposis syndrome. World J Gastroenterol 2011;17:4839–44.

6. Uppal S, Mistry D, Coatesworth AP. Cowden disease: a review. Int J Clin Pract 2007;61:645–52.

7. Lloyd KM, Dennis M. Cowden disease. A possible new symptom complex with multiple system involvement. Ann Intern Med 1963;58:136.

8. Kato M, Mizuki A, Hayashi T, et al. Cowden’s disease diagnosed through mucocutaneous lesions and gastrointestinal polyposis with recurrent hematochezia, unrevealed by initial diagnosis. Intern Med 2000;39:559.

9. Curatolo P, Bombardieri R, Jozwiak S. Tuberous sclerosis. Lancet 2008;372:657.

10. Van Lier MG, Wagner A, Mathus-Vliegen EM, et al. High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. Am J Gastroenterol 2010;105:1258.

11. Veale AM, McColl I, Busey HJ. Juvenile polyposis coli. J Med Genet 1966;3:5–16.

12. Grotsky HW, Rickert RR, Smith WD, et al. Familial juvenile polyposis coli. A clinical and pathologic study of a large kindred. Gastroenterology 1982;82:494.

13. Soto-Medina CA, Mier-Escurra EA, Treviño-Garza F, et al. Splenic hamartoma. Case report. Cir Cir 2014;82:328−31.

14. Lee H, Maeda K. Hamartoma of the spleen. Arch Pathol Lab Med. 2009;133:147–51.

15. Sankar S, Thanka J, Jagdishchandrabose S, et al. Splenic hamartoma: A rare vascular space occupying lesion of the Spleen. Indian J Pathol Microbiol 2011;54:223−5.

16. Zissin R, Lishner M, Rathaus V. Case report: Unusual presentation of splenic hamartoma; computed tomography and ultrasonic findings. Clin Radiol 1992;45:410−1.

17. Wang JH, Ma XL, Ren FY, et al. Multi-modality imaging findings of splenic hamartoma: a report of nine cases and review of the literature. Abdom Imaging 2013;38:154–62.

18. Namikawa T, Kitagawa H, Iwabu J, et al. Laparoscopic splenectomy for splenic hamartoma: case management and clinical consequences. World J Gastrointest Surg 2010;2:147–52.

19. Tan CL, Tan SH, So JB, et al. Muco-submucosal elongated polyps of the gastrointestinal tract: a case series and a review of the literature. World J Gastroenterol 2013;19:1845−9.

20. Manxhuka-Kerliu S, Sahatciu-Meka V, Kerliu I, et al. Small intestinal gastrointestinal stromal tumor in a young adult woman: a case report and review of the literature. J Med Case Rep 2014;8:321.

21. Brosens LA, Langeveld D, van Hattem WA, et al. Juvenile polyposis syndrome. World J Gastroenterol 2011;17:4839–44.

22. Jiangang Sun, Yongshun Gao, Bo Yang, et al. Intestinal obstruction caused by giant ileal hamartoma: a case report. Ann Transl Med 2016;4:138.

23. Sun JG, Qi J, Yang B, et al. The clinical characteristics and treatment of intestinal hamartomas. Lasers Med Sci 2016;31:1761.

24. Marques M, Antunes J, Coelho R, et al. Single-balloon enteroscopy efficacy and degree of concordance with noninvasive evaluation of small bowel. Endosc Int Open. 2017;5: E96–E102.

25. Akahoshi K, Akahane H, Motomura Y, et al. A new approach: endoscopic submucosal dissection using the clutch cutter (R) for early stage digestive tract tumors. Digestion 2012;85:80–4.

26. Brookes MJ, Manjunatha S, Allen CA, et al. Malignant potential in a Brunner’s gland hamartoma. Postgrad Med J 2003;79:416–7.

27. Botsford TW, Crowe P, Croker DW. Tumors of the small intestine. A review of experience with 115 cases including a report of a rare case of malignant hemangio-endothelioma. Am J Surg 1962;103:358–65.

28. Levine JA, Burgart LJ, Batts KP, et al. Brunner’s gland hamartomas: clinical presentation and pathological features of 27 cases. Am J Gastroenterol 1995;90:290–4.

29. Merine D, Jones B, Ghahremani GG, et al. Hyperplasia of Brunner glands: the spectrum of its radiographic manifestations. Gastrointest Radiol 1991;16:104–8.

30. Perez A, Saltzman JR, Carr-Locke DL, et al. Benign nonampullary duodenal neoplasms. J Gastrointest Surg 2003;7:536–41.

31. Debraj Sen, Y.S. Gulati, Anusree Majumder. Hepatic cystic mesenchymal hamartoma. Med J Armed Forces India 2015; 71(Suppl 2): S574–7.

32. Chung EM, Cube R, Lewis RB, et al. Pediatric liver masses: radiologic-pathologic correlation part 1. Benign tumors. Radiographics 2010;30:801–26.

33. Ros P, Goodman Z, Ishak KG, et al. Mesenchymal hamartoma of the liver: radiologic-pathologic correlation. Radiology 1986;158:619–24.

34. Chung EM, Lattin GE, Jr, Cube R, et al. From the archives of the AFIP: Pediatric liver masses: radiologic-pathologic correlation. Part 2. Malignant tumors. Radiographics 2011;31:483–507.

35. Ramanujam TM, Ramesh JC, Goh DW, et al. Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature. J Pediatr Surg 1999;34:1684–6.

36. Barnhart DC, Hirschl RB, Garver KA, et al. Conservative management of mesenchymal hamartoma of the liver. J Pediatr Surg 1997;32:1495–8.

37. Desmet VJ. Pathogenesis of ductal plate malformation. J Gastroenterol Hepatol 2004;19(Suppl 7):S356–60.

38. Kin HK, Jin SY. Cholangiocarcinoma arising in von Meyenburg complexes. Korean J Hepatol. 2011;17:161–4.

39. Zheng RQ, Zhang B, Kudo M, et al. Imaging findings of biliary hamartomas. Wolrd J Gastroenterol 2005;11:6354–9.

40. Semelka RC, Hussain SM, Marcos HB, et al. Biliary hamartomas: solitary and multiple lesions shown on current MR techniques including gadolinium enhancement. J Magn Reson Imaging. 1999;10:196–201.

41. Sinakos E, Papalavrentios L, Chourmouzi D et al. The clinical presentation of Von Meyenburg complexes. Hippokratia 2011;15:170–3.

42. Zen Y, Terahata S, Miyayama S, et al. Multicystic biliary hamartoma: a hitherto undescribed lesion. Hum Pathol 2006;37:339–44.

43. Beard R, Yee E, Mortele K, et al. Multicystic biliary hamartoma: A report of a rare entity and a review of the literature. Int J Surg Case Rep. 2014;5:919–23.

44. Craig J, Peters R, Edmundson H. Tumors of the liver and intrahepatic bile ducts, Fasc 26, 2nd ed, DC Armed Forces Institute of Pathology, Washington DC 1989.

45. Wanless IR, Mawdsley C, Adams R. On the pathogenesis of focal nodular hyperplasia of the liver. Hepatology 1985;5:1194.

46. Haber M, Reuben A, Burrell M, et al. Multiple focal nodular hyperplasia of the liver associated with hemihypertrophy and vascular malformations. Gastroenterology 1995;108:1256.

47. Wanless IR, Gryfe A. Nodular transformation of the liver in hereditary hemorrhagic telangiectasia. Arch Pathol Lab Med 1986;110:331.

48. Matsushita D, Kurahara H, Mataki Y, et al. Pancreatic hamartoma: a case report and literature review. BMC Gastroenterol 2016;16:3.

49. Nagata S, Yamaguchi K, Inoue T, et al. Solid pancreatic hamartoma. Pathol Int 2007;57:276–80.

50. Sueyoshi R, Okazaki T, Lane GJ, et al. Multicystic adenomatoid pancreatic hamartoma in a child: Case report and literature review. Int J Surg Case Rep 2013;4:98–100.

51. Wu SS, Vargas HI, French SW. Pancreatic hamartoma with Langerhans cell histiocytosis in a draining lymph node. Histopathology 1998;33:485–7.

52. Pauser U, Kosmahl M, Kruslin B, et al. Pancreatic solid and cystic hamartoma in adults: characterization of a new tumorous lesion. Am J Surg Pathol 2005;29:797–800.

53. Beger HG, Siech M, Poch B, et al. Limited surgery for benign tumours of the pancreas: A systematic review. World J Surg 2015;39:1557–66.

54. Jinzaki M, Silverman SG, Akita H. Renal angiomyolipoma: a radiological classification and update on recent developments in diagnosis and management. Abdom Imaging 2014;39:588–604.

55. Fittschen A, Wendlik I, Oeztuerk S, et al. Prevalence of sporadic renal angiomyolipoma: a retrospective analysis of 61,389 in- and out-patients. 1. Abdom Imaging 2014;39:1009–13.

56. Becker JA, Kinkhabwala M, Pollack H, et al. Angiomyolipoma (Hamartoma) - angiographic review. Acta Radiol 1973;14:561−8.

57. Allen TD, Risk W. Renal angiomyolipoma. J Urol 1965;94:203−7.

58. Clark ER, Palubinskas AJ. The angiographic spectrum of renal hamartoma. Am J Roentgenol Radium The Nucl Med 1972;114:715−21.

59. Khrlnam M, Wolf BS. Hamartoma of the kidney, clinical and roentgenographic manifestation. Am Roentgenol Radium Ther Nucl Med 1961;86:830−41.

60. Aydin H, Magi-Galluzzi C, Lane BR. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. Am J Surg Pathol 2009;33:289-97.

61. Vít V, Pacík D, Čermák A, et al. Je CT vyšetření dostatečně spolehlivé při hodnocení neoplasmatických procesů ledvin? Čes urol 2004;3:17–20.

62. Sooriakumaran P, Gibbs P, Coughlin G, et al. Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated. BJU Int 2010;105:101.