Quiz-summary
0 of 1 questions completed
Questions:
- 1
Information
Presented by George Netto, M.D. and prepared by ChanJuan Shi, M.D., Ph.D.
Case 3: A 9 year boy presented with hematuria was found to have a 3.5 cm “polypoid/dome shaped” urinary bladder mass.
You have already completed the quiz before. Hence you can not start it again.
Quiz is loading...
You must sign in or sign up to start the quiz.
You have to finish following quiz, to start this quiz:
Results
Time has elapsed
Categories
- Not categorized 0%
- 1
- Answered
- Review
-
Question 1 of 1
1. Question
Week 367: Case 3
A 9 year boy presented with hematuria was found to have a 3.5 cm “polypoid/dome shaped” urinary bladder mass. Focal Desmin and cytokeratin staining was demonstrated.images/netto92908-3a.jpg
images/netto92908-3b.jpg
images/netto92908-3c.jpgCorrect
Answer: Inflammatory myofibroblastic tumor
Histology: Inflammatory myofibroblastic tumor (IMT) demonstrates a typical “fasciitis-like” morphologic features imparted by the proliferation of bland spindle cells containing somewhat monotonous nuclei set in a thinly vascular myxoid background with variable amount of associated Inflammatory cells.
Discussion: Also known as postoperative spindle cell nodules and pseudosarcomatous myofibroblastic proliferations, IMT lesions of the GU tract predominantly occur in the urinary bladder. Similar tumors are found in the prostate and prostatic urethra. A prior history of instrumentation (catheter or prior TURBT) is occasionally but not universally present.
The spindle cells react with cytokeratins (50-70%) and smooth muscle markers (including desmin) in approximately 80% of cases. Cytoplasmic expression of ALK has been shown in approximately 60% of GU tract IMTs. Corresponding genetic alterations involving the ALK gene have been demonstrated by FISH studies in over 70% of cases in a recent large series by Montgomery et al. The latter genetic alteration was also detected in 2 IMT lesions that appeared to be related to prior instrumentation. A recurrence rate of around 30% has been reported in IMT.
Alk postivity and a negative staining for myogenin was demononstrated in the current case.Given the fact that rare IMT may coexist with a non sampled sarcomatoid urothelial carcinoma, a recommendation for close follow up should accompany a transurethral biopsy diagnosis of IMT.
Reference(s):
– Montgomery EA, Shuster DD, Burkart AL, Esteban JM, Sgrignoli A, Elwood L, Vaughn DJ, Griffin CA, Epstein JI. Inflammatory myofibroblastic tumors of the urinary tract: a clinicopathologic study of 46 cases, including a malignant example inflammatory fibrosarcoma and a subset associated with high-grade urothelial carcinoma. Am J Surg Pathol. 2006;30(12):1502-12.
– Hirsch MS, Dal Cin P, Fletcher CD: ALK expression in pseudosarcomatous myofibroblastic proliferations of the genitourinary tract. Histopathology. 2006;48(5):569-78.Incorrect
Answer: Inflammatory myofibroblastic tumor
Histology: Inflammatory myofibroblastic tumor (IMT) demonstrates a typical “fasciitis-like” morphologic features imparted by the proliferation of bland spindle cells containing somewhat monotonous nuclei set in a thinly vascular myxoid background with variable amount of associated Inflammatory cells.
Discussion: Also known as postoperative spindle cell nodules and pseudosarcomatous myofibroblastic proliferations, IMT lesions of the GU tract predominantly occur in the urinary bladder. Similar tumors are found in the prostate and prostatic urethra. A prior history of instrumentation (catheter or prior TURBT) is occasionally but not universally present.
The spindle cells react with cytokeratins (50-70%) and smooth muscle markers (including desmin) in approximately 80% of cases. Cytoplasmic expression of ALK has been shown in approximately 60% of GU tract IMTs. Corresponding genetic alterations involving the ALK gene have been demonstrated by FISH studies in over 70% of cases in a recent large series by Montgomery et al. The latter genetic alteration was also detected in 2 IMT lesions that appeared to be related to prior instrumentation. A recurrence rate of around 30% has been reported in IMT.
Alk postivity and a negative staining for myogenin was demononstrated in the current case.Given the fact that rare IMT may coexist with a non sampled sarcomatoid urothelial carcinoma, a recommendation for close follow up should accompany a transurethral biopsy diagnosis of IMT.
Reference(s):
– Montgomery EA, Shuster DD, Burkart AL, Esteban JM, Sgrignoli A, Elwood L, Vaughn DJ, Griffin CA, Epstein JI. Inflammatory myofibroblastic tumors of the urinary tract: a clinicopathologic study of 46 cases, including a malignant example inflammatory fibrosarcoma and a subset associated with high-grade urothelial carcinoma. Am J Surg Pathol. 2006;30(12):1502-12.
– Hirsch MS, Dal Cin P, Fletcher CD: ALK expression in pseudosarcomatous myofibroblastic proliferations of the genitourinary tract. Histopathology. 2006;48(5):569-78.
