Small bowel (small intestine)
Benign tumors / tumor-like conditions
Lipomatosis of the ileocecal valve

Author: Kieran Doyle
Senior Author: Jian-Hua Qiao, M.D.
Editorial Board Member: Raul S. Gonzalez, M.D.
Editor-in-Chief: Debra Zynger, M.D.
Kieran Doyle
Jian-Hua Qiao, M.D.

Topic Completed: 5 June 2019

Revised: 27 June 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Lipomatosis of the ileocecal valve


Kieran Doyle
Jian-Hua Qiao, M.D.
Page views in 2019 to date: 634
Cite this page: Doyle K, Qiao J. Lipomatosis of the ileocecal valve. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/smallbowellipomatosisileocecalvalve.html. Accessed August 26th, 2019.
Definition / general
Essential features
  • Diffuse infiltration of the fatty tissue mainly in the submucosal layer is characteristic
  • Usually asymptomatic and found incidentally
  • Lack of encapsulation differentiates it from lipoma
  • Cause of the fat deposition is not known
Terminology
  • Lipomatosis of the ileocecal valve
  • Lipohyperplasia
ICD coding
  • ICD-10: E88.2 - lipomatosis, not elsewhere classified
Epidemiology
  • M > F
  • Uncommon under age 40
  • More common with obesity
Sites
  • Ileocecal valve and ileum
  • Also can occur in cecum and ascending colon
Pathophysiology
  • Benign mature adipocyte proliferation within the submucosal layer of the ileocecal valve
Etiology
  • Unknown
Clinical features
  • Commonly asymptomatic
  • May present with
    • Right lower quadrant pain
    • Constipation
    • Diarrhea
    • Obstruction
    • Bleeding
    • Hemorrhage
    • Intussusception
  • Can mimic appendicitis
  • Risk factors: female, obese, age > 40
Diagnosis
  • Incidental diagnosis of right colon resection, which usually includes terminal ileum and ileocecal valve
Radiology description
Radiology images

Images hosted on other servers:

Hypoechoic on ultrasound

CT with submucosal fat

Multidetector CT

Case reports
Treatment
  • Usually asymptomatic
  • Surgical resection if causing obstruction, significant symptoms or concern for malignancy
Clinical images

Images hosted on other servers:

Smooth ileocecal lesion

Gross description
  • Prominent and thickened ileocecal valve, usually an incidental finding during gross exam of right colectomy specimen
Gross images

Contributed by Jian-Hua Qiao, M.D.

Thickened ileocecal valve

Donut shaped ileocecal valve

Microscopic (histologic) description
  • Ileocecal valve mucosa with extensive submucosal proliferation of lipomatous mature adipocytes
  • Fibrous septa and vascular congestion are usually present
  • No surrounding fibrous capsule
Microscopic (histologic) images

Contributed by Jian-Hua Qiao, M.D.

Unencapsulated submucosal adipose

Villi resembling terminal ileum mucosa

Unencapsulated submucosal adipose

Well defined crypts resembling cecal mucosa

Immunohistochemistry
  • Not indicated
Sample pathology report
  • Right hemicolectomy
    • Adenocarcinoma of ascending colon (see synoptic report)
    • Lipomatosis of ileocecal valve (incidental finding)
    • 20 mesenteric lymph nodes negative for metastatic tumor (0/20)
Differential diagnosis
  • Lipoma
    • True lipoma of ileocecal valve is rare
    • Has demarcating capsule around the fatty tissue and is confined to only one of the ileocecal lips
  • Crohn's disease
    • Crohn's ileocolitis can be associated with lipomatosis of the ileocaecal valve
    • Can cause difficulty in diagnostic imaging studies
    • Gross and microscopic examination will show characteristic fissuring, skipping ulceration and transmural lymphoid aggregates
  • Non-Hodgkin lymphoma (NHL)
    • Can involve ileocecal valve and forming obstructive mass lesion or circumferential infiltration
    • Microscopic examination will show malignant lymphoma cells with specific IHC staining patterns
  • Adenomatous polyp
    • Can involve ileocecal valve
    • Grossly, it is usually focal polypoid lesion rather than diffuse thickening
    • Microscopic exam will show adenomatous tubules
  • Adenocarcinoma of the ileocecal valve
    • Usually an irregular mass lesion with ulceration and obstruction
    • Microscopic exam will show malignant tumor glands
Board review question #1
A middle aged man underwent a right hemicoloectomy for cecal adenocarcinoma. Grossly, the ileocecal valve was prominent and diffusely thickened but no tumor was noted. What is your diagnosis?



  1. Crohn's disease
  2. Lipoma of the ileocecal valve
  3. Lipomatosis of the ileocecal valve
  4. Non-Hodgkin lymphoma
  5. Normal ileocecal valve
Board review answer #1
C. Lipomatosis of the ileocecal valve. Submucosal nonencapsulated adipose tissue proliferation is diagnostic for lipomatosis of the ileocecal valve.

Comment here

Reference: Small bowel (small intestine) - Lipomatosis of the ileocecal valve
Board review question #2
A 45 year old woman presents with sudden right lower quadrant abdominal pain. She has been experiencing on and off abdominal pain for the past 4 weeks. CT abdomen reveals small bowel obstruction near the ileocecal valve. She undergoes exploratory laparotomy and is later diagnosed with lipomatosis of the ileocecal valve by pathological examination. Which of the following is diagnostic for lipomatosis of the ileocecal valve?

  1. Adipocyte proliferation in the muscularis propria not contained within a capsule
  2. Adipocyte proliferation in the submucosa contained within a capsule
  3. Adipocyte proliferation in the submucosa not contained within a capsule
  4. Diffuse gland proliferation
  5. Inflammatory cells predominately lymphocytes
Board review answer #2
C. Adipocyte proliferation in the submucosa not contained within a capsule is diagnostic for lipomatosis of the ileocecal valve.

Comment here

Reference: Small bowel (small intestine) - Lipomatosis of the ileocecal valve
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