7 June 2007 – Case of the Week #86

 

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This email is sponsored by Columbia University and New York - Presbyterian Hospital, who are sponsoring their 30th Annual Postgraduate Medicine Course, “Renal Biopsy in Medical Diseases of the Kidneys” on September 26-29, 2007.  The course will be held at The New York Academy of Medicine in New York City, New York. 

 

Please join them for an intensive 3 1/2 day course designed for pathologists interested in both a systematic review and an update on advances in diagnostic problems in medical renal diseases.

 

The faculty includes Drs. Charles Alpers (University of Washington), Robert Colvin (Harvard), William Couser (University of Washington), Ronald Falk (University of North Carolina), Agnes Fogo (Vanderbilt), Eli Friedman (Downstain Medical Center-Brooklyn), Gary Hill (Johns Hopkins), J Charles Jennette (University of North Carolina), Paul Klotman (Mount Sinai School of Medicine-New York), Marc Pohl (Cleveland Clinic), Helmut Rennke (Harvard), Pierre Ronco (Pierre and Marie Curie University-Paris) and Fred Silva (Medical College of Georgia).

 

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We thank Dr. Nazar Jawhar, University of Science & Technology Hospital, Sana’a, Yemen, for contributing this case.  We invite you to contribute a Case of the Week by emailing us with microscopic images (any size, we will shrink if necessary) in JPG or TIFF format, a clinical history, your diagnosis and any other images (gross, immunostains, etc.) that may be helpful or interesting.  We will write the discussion (unless you want to), list you as the contributor, and send you a check for $35 (US  dollars) for your time after we send out the case.  Please only send cases with a definitive diagnosis.

 

Case of the Week #86

 

Clinical History

 

A 39 year old woman presented with small intestinal obstruction, and a mass was discovered and excised.

 

Gross images:  image #1#2

Micro images:  image #3#4#5#6#7

 

What is your diagnosis?

 

 

 

 

 

 

 

 

 

 

 

 

 

  

 

 

 

 

 

 

 

 

 

Diagnosis:

 

Inflammatory fibroid polyp of the small intestine

 

Discussion

 

Inflammatory fibroid polyps are benign tumors that commonly appear in the distal stomach or terminal ileum as an obstructive mass or causing intussusception (Radiographics 1999;19:539).  They are polypoid submucosal masses that contain a mixture of spindle and inflammatory cells, particularly eosinophils, in a background of prominent capillaries and granulation tissue-like stroma (Mod Path 2003;16:366-free full text).  There is no atypia, and no/rare mitotic figures.  The spindle cells and blood vessels are immunoreactive for CD34, and negative for CD117, although mast cells are CD117+ (Mod Path 2000;13:1134-free full text).  Staining for smooth muscle actin is variable.  Excision is curative, as these tumors do not recur.

 

The differential diagnosis includes inflammatory myofibroblastic tumor, gastrointestinal stromal tumor and Crohn’s disease.  Inflammatory myofibroblastic tumor, also called inflammatory pseudotumor, often occurs in children.  It is also benign, but often recurs.  These tumors in general are larger than inflammatory fibroid polyps, and the overlying mucosa may be ulcerated.  They consist of spindle cells and an inflammatory infiltrate of plasma cells and lymphocytes, with few eosinophils.  Cellular areas may have mitotic activity.  The blood vessels are not as prominent as in inflammatory fibroid polyp.  Inflammatory myofibroblastic tumor is immunoreactive for smooth muscle actin and desmin, with variable staining for ALK, and negative staining for CD34 (Hum Path 2002;33:307).

 

Gastrointestinal stromal tumors of the small intestine are malignant in 30-50% of cases, and have a 5 year survival of only 50%.  The histologically benign cases may have plump spindle cells with eosinophilic cytoplasm, skenoid fibers (extracellular collagen globules), and muscle infiltration.  Most importantly, these tumors are immunoreactive for CD117, in contrast to inflammatory fibroid polyp.

 

Finally, inflammatory polyps of Crohn’s disease or ulcerative colitis have similar histology of inflamed mucosa in a granulation tissue stroma, with no/few mitotic figures.  Clinical information and histology of the remainder of the bowel are important distinguishing features.  Of interest, a case of inflammatory fibroid polyp in a Crohn’s patient has also been reported (Ann Ital Chir 2005;76:395)

 

 

 

Nat Pernick, M.D., President
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