Lecture notes for medical students

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5. This will be an ACTIVE LEARNING SESSION x
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7. See notes for bibliography.

2. Learning Objectives
At the end of this session the learner will be able to
describe-
• Aetiology
• Clinical Features
• Management
Of
Benign Tumors of Small Intestine

3. Learning Objectives
1. Introduction & History
2. Relevant Anatomy, Physiology
3. Aetiology
4. Pathophysiology
5. Pathology
6. Classification
7. Clinical Features
8. Investigations
9. Management
10. Controversies
11. Prevention
12. Guidelines
13. Take home messages

4. Introduction & History.
•

5. Introduction & History.
• Small bowel tumours are rare and in total
account for less than 10% of gastrointestinal
neoplasia.
• Benign tumors of the small bowel often
remain asymptomatic throughout life.
• The majority of small bowel neoplasms are
benign and often remain asymptomatic
throughout life.

6. Relevant Anatomy
•

7. Relevant Anatomy
• Despite comprising 75% of the length and
90% of the surface area of the
gastrointestinal (GI) tract, the small bowel
harbors relatively few primary neoplasms
and fewer than 2% of Gimalignancies.

8. Relevant Physiology
•

9. Aetiology
• Idiopathic
• Traumatic
• Infections /Infestation
• Neoplastic (Benign/Malignant)
• Congenital/ Genetic
• Nutritional Deficiency/excess
• Autoimmune
• Degenerative / lifestyle
• Iatrogenic
• Psychosomatic
• Poisoning/ Toxins/ Drug induced

10. Etiology of Etiology
•

11. Aetiology of Aetiology
• Idiopathic
• Traumatic
• Infections /Infestation
• Neoplastic (Benign/Malignant)
• Congenital/ Genetic
• Nutritional Deficiency/excess
• Autoimmune
• Degenerative / lifestyle
• Iatrogenic
• Psychosomatic
• Poisoning/ Toxins/ Drug induced

12. Pathophysiology

13. Pathophysiology
• Factors suggested to explain low occurrence
of neoplasms in small bowel-
– Rapid intestinal transit through the small bowel limits
contact time to the small-bowel mucosa
– Greater fluidity of small-bowel chyme may dilute
luminal irritants
– Alkaline pH may play a role
– Low bacterial colony counts of the small bowel
– Higher levels of benzyl peroxidase (thought to detoxify
potential carcinogens) have been detected in the small
bowel
– High levels of immunoglobulin A and widespread gut
lymphoid tissue.

14. Pathology
•

15. Pathology
• Benign small-bowel tumors may be found
throughout the duodenum, jejunum, and ileum (in
order of increasing frequency).
• Tumors may be single, multiple, or widespread
(ie, as part of a polyposis syndrome).
•

16. Pathology
• The following three growth patterns have
been identified:
– Intraluminal
– Infiltrative
– Serosal
• Intraluminal lesions are most often associated with
the development of secondary bowel obstruction
and intussusception, whereas serosal lesions are
linked to small-bowel volvulus.

17. Classification

18. Classification
• Benign-
– Hyperplastic polyps
– Hamartomas
– Adenomas
– Gut stromal tumors
– Lipomas
– Hemangiomas
– Those associated with Peutz-Jeghers syndrome
• Malignant-
– Adenocarcinoma,
– Neuroendocrine tumours (nets),
– Lymphomas
– Gastrointestinal stromal tumours (GISTs).

19. Clinical Features
•

20. Clinical Features
• Demography
• Symptoms
• Signs
• Prognosis
• Complications

21. Demography

22. Demography
• Incidence & Prevalence
• Geographical distribution.
• Race
• Age
• Sex
• Socioeconomic status
• Temporal behaviour

23. Demography
• Incidence & Prevalence-

24. Demography
Incidence & Prevalence-
• Less than 10% of gastrointestinal neoplasia.

25. Demography
• Geographical distribution.

26. Demography
Geographical distribution-
• Nil

27. Demography
• Race.

28. Demography
Race-
• Nil.

29. Demography
• Age

30. Demography
Age-
• Occurs in persons of all age groups, though
the mean age of presentation is between the
fifth and sixth decades of life.

31. Demography
• Sex

32. Demography
Sex-
• Slight male predominance.

33. Symptoms

34. Symptoms and Signs
• Asymptomatic –
Detected incidently.
• Abdominal pain
• Constipation
• Melena
• Perforation
• Nausea
• Diarrhea
• Gastrointestinal (GI)
haemorrhage.
• Volvulus
• Vomiting
• Palpable mass
• Obstruction
• Anorexia
• Early satiety
• Anemia
• Intussusception

35. Complications

36. Complications
• Intususeption.
• Bowel obstruction
• Volvulus
• GI bleeding
• Perforation

37. Hamartoma
• Hamartoma is very rare benign condition
associated with
– An abnormal location
– And abnormal arrangement
of tissues normally found in small intestine.

38. Hamartoma
• Diagnosis is usually made by histological
examination.
• The most common presenting symptoms are
of intestinal obstruction due to either
intussusception or stricture.
• GI bleeding.

39. Hamartoma
• Types of hamartomas -
– Neuromuscular and vascular hamartoma
(NMVH),
– Neuromesenchymal hamartoma (NMH),
– Myoepithelial hamartoma (MEH)
– Cowden hamartomatous syndrome.

40. Cowden Hamartomatous
Syndrome.
• Cowden disease, also termed Cowden
syndrome and multiple hamartoma
syndrome, is an autosomal dominant
condition with variable expression that can
be associated with a mutation in
the PTEN gene on arm 10q.

41. Cowden Hamartomatous
Syndrome.
• Causes hamartomatous neoplasms of the
skin and mucosa, GI tract, bones, CNS,
eyes, and genitourinary tract.
• Cowden disease is associated with the
development of several types of
malignancy-
• Breast,
• Thyroid,
• Colon,
• Renal Cell Carcinoma

42. Peutz-Jeghers syndrome
• Peutz-Jeghers syndrome is an autosomal
dominant disorder featuring
– Mucocutaneous pigmentation (eg, on the face,
lips, and buccal mucosa)
– And benign GI hamartomas.

43. Peutz-Jeghers syndrome
• The hereditary defect is associated with
mutation on the LKB1 gene (19p2,3).

44. Peutz-Jeghers syndrome
• These polyps may be found in the small
bowel in up to 90% of affected individuals.
• The stomach and the colon are frequently
involved, and tumors outside the GI tract
are also described.
• Histologically- frondlike appearance with a
stromal/smooth muscle core covered by
acinar glands and normal mucosa. Nuclear
atypia is absent.

45. Peutz-Jeghers syndrome
• Complications are common-
– Colicky abdominal pain
– GI bleeding
– Obstruction
– Intussusception
– Malignant transformation
– Non-GI cancers may be found concomitantly.

46. Peutz-Jeghers syndrome
• Current surveillance recommendations for
the small-bowel lesions include biannual
barium upper GI series and flexible
endoscopy beginning at age 10 years.

47. Hyperplastic polyps
• Hyperplastic polyps are benign mucosal
growths frequently observed in the
duodenum and proximal ileum.
• Frequently discovered upon routine upper
endoscopy,
• The polyps may be single or multiple.
• They are generally asymptomatic with no
malignant potential
• May be removed endoscopically with
biopsy forceps or an Endosnare.

48. Adenomas
• Three types of small-bowel adenomas have
been described, as follows:
– Adenomatous polyps
– Brunner gland adenomas
– Villous adenomas
• In general, they may develop as single or
multiple lesions, both sessile and
pedunculated.
• Histology-intraluminal extensions of the mucous
membrane and submucosal architecture with multiple acini
supported on a central fibrovascular core. Varying degrees
of differentiation within tumors.

49. Adenomas
• Complications –
– Obstruction
– Bleeding
– Intussusception
– malignant degeneration.

50. Brunner gland Adenomas
• Brunner gland adenomas develop most
often along the posterior wall of the
duodenum at the junction of the first and
second portions.
• Focal, multifocal, or diffuse, they exhibit
benign proliferation of the Brunner glands
with scattered ductal and stromal elements.

51. Villous Adenoma
• Villous adenoma very rare
• Most frequently found in the duodenum.
• Bleeding and obstruction are their most
common complications, though, like their
counterparts in the colon and stomach, they
may be associated with malignant
degeneration.
• Villous adenomas larger than 4 cm are at
particular risk for malignant elements.

52. Gut Stromal Tumors
• Aka. Leiomyomas and leiomyosarcomasare
the most common symptomatic small-bowel
lesions.
• Found in all areas of the small bowel,
including within the meckel diverticulum.
• Focal lesions and anular lesions.
• Intramural lesions may form intraluminal
masses, extraluminal masses, or transmural
(dumbbell-shaped) lesions.

53. Gut Stromal Tumors: Histology
• Nests of spindle-shaped cells located
between the muscularis propria and the
muscularis mucosa.
• Histologic features of smooth muscle may
or may not be seen with light microscopy.
•

54. Gut Stromal Tumors: Histology
• Differentiating between benign gut stromal
tumors and malignant gut stromal tumors is
difficult.
• Diagnostic criteria for malignancy -
– Tumor cell size
– Degree of cellular differentiation
– More than two mitotic figures per 10 hpf

55. Gut Stromal Tumors
• Complications-
– Bleeding
– Bowel obstruction
– Intussusception
– Tumor perforation
– Malignant degeneration.

56. Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histology
– Germ line Testing and Molecular Analysis
• Diagnostic Laparotomy.

57. Investigations
• Laboratory Studies
– Routine
– Special
• Imaging Studies
• Tissue diagnosis
– Cytology
• FNAC
– Histology
– Germ line Testing and Molecular Analysis
• Diagnostic Laparotomy.

58. Investigations
• Laboratory Studies
–

59. Investigations
• Laboratory Studies
– Anemia.

60. Diagnostic Studies
Imaging Studies
• X-Ray
• USG
• CT
• Angiography
• MRI
• Endoscopy
• Nuclear scan

61. Diagnostic Studies
Imaging Studies
• X-Ray- Barium
• USG
• CT
• Angiography
• MRI
• Endoscopy- enteroscopy, intraoperative
enteroscopy,Capsule endoscopy.
• Nuclear scan

62. Differential Diagnosis

63. Differential Diagnosis
• Colon Cancer
• Colonic Polyps
• Intestinal Carcinoid Tumor
• Intestinal Perforation
• Intestinal Polypoid Adenomas
•

64. Management

65. Operative Therapy

66. Operative Therapy
• Exploratory laparotomy with excision of the
lesion provides the safest and most direct
method for lesion identification and
treatment.
• Tumors discovered incidentally at
laparotomy should be removed to prevent
future symptom development and secondary
complications.

67. Minimally invasive Therapy

68. Minimally invasive Therapy
• Laparoscopic, endoscopic, and robotic-
assisted approaches have been described.

69. Futuristic

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