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Presented by Risa Mann, M.D. and prepared by Sharon Swierczynski, M.D., Ph.D.
Case 1: 75-year-old female with a ureter mass by CT scan
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1. Question
Week 154: Case 1
75-year-old female with a ureter mass by CT scanimages/092903case1fig1.jpg
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images/092903case1fig4.jpgCorrect
Answer: Amyloidosis
Histology: The specimen is a cross-section of a ureter which shows relatively normal-appearing urothelium with underlying scarring and chronic inflammation. The most striking abnormality is the deposition of amorphous pink material in the submucosa and wall of the ureter. This amorphous pink material is sometimes associated with foreign body giant cell reaction as well as ossification. When looking more carefully at the wall of the ureter, one can see that the amorphous pink material is not only deposited in the soft tissue surrounding the ureter, but is also strikingly present in many of the small vessels. There is no evidence of malignancy which was suspected clinically.
Discussion: Although this patient was clinically thought to have a tumor causing constriction of the ureter leading to hydronephrosis and pyelonephritis, in fact the thickened wall in this case was due to massive amyloid deposition involving the soft tissue as well as the walls of small vessels. When we studied this case, it was felt that the pink material most likely represented amyloid and the deposition of it in many small vessels is also typical of amyloid deposition. Foreign body giant cell reaction is also seen as a reaction to amyloid depositions. The Congo red stain confirmed this diagnosis and showed the typical green color with polarized illumination.
images/092903case1fig5.jpg
Amyloid deposition in the GU tract, particularly the bladder, can be part of a generalized process or can present as a localized nodular lesion. When the deposition forms a nodular mass, it is often clinically diagnosed as a tumor. The appearance of amyloid deposition in the bladder as well as the ureter is similar to that in other sites and can evoke a giant cell reaction, which is very prominent in this case. In most cases the amyloid protein deposition is of light chain derivation.
In those cases in which there has been adequate follow-up of amyloid deposition in the bladder, most of the patients have remained free of disease following local excision. Therefore, patients in which amyloid is identified should be evaluated for the possibility of a generalized plasma cell dyscrasia, but amyloid deposition such as this in the bladder and ureter should not be regarded as a definite manifestation of a myeloma.
Incorrect
Answer: Amyloidosis
Histology: The specimen is a cross-section of a ureter which shows relatively normal-appearing urothelium with underlying scarring and chronic inflammation. The most striking abnormality is the deposition of amorphous pink material in the submucosa and wall of the ureter. This amorphous pink material is sometimes associated with foreign body giant cell reaction as well as ossification. When looking more carefully at the wall of the ureter, one can see that the amorphous pink material is not only deposited in the soft tissue surrounding the ureter, but is also strikingly present in many of the small vessels. There is no evidence of malignancy which was suspected clinically.
Discussion: Although this patient was clinically thought to have a tumor causing constriction of the ureter leading to hydronephrosis and pyelonephritis, in fact the thickened wall in this case was due to massive amyloid deposition involving the soft tissue as well as the walls of small vessels. When we studied this case, it was felt that the pink material most likely represented amyloid and the deposition of it in many small vessels is also typical of amyloid deposition. Foreign body giant cell reaction is also seen as a reaction to amyloid depositions. The Congo red stain confirmed this diagnosis and showed the typical green color with polarized illumination.
images/092903case1fig5.jpg
Amyloid deposition in the GU tract, particularly the bladder, can be part of a generalized process or can present as a localized nodular lesion. When the deposition forms a nodular mass, it is often clinically diagnosed as a tumor. The appearance of amyloid deposition in the bladder as well as the ureter is similar to that in other sites and can evoke a giant cell reaction, which is very prominent in this case. In most cases the amyloid protein deposition is of light chain derivation.
In those cases in which there has been adequate follow-up of amyloid deposition in the bladder, most of the patients have remained free of disease following local excision. Therefore, patients in which amyloid is identified should be evaluated for the possibility of a generalized plasma cell dyscrasia, but amyloid deposition such as this in the bladder and ureter should not be regarded as a definite manifestation of a myeloma.
