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Presented by Dr. Andres Matoso and prepared by Dr. Monica Butcher
This case talks about an adult female with a bladder tumor
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Adult female with a bladder tumor
Diagnosis:
Correct
Correct: D
Histology: This tumor is stellate-shaped cells loosely arranged in a myxoid matrix with intermixed inflammatory cells, including eosinophils, neutrophils, lymphocytes and plasma cells.
Discussion: Also known as postoperative “spindle cell nodule,” inflammatory myofibroblastic tumors (IMTs) occur more commonly in the bladder but some also affect the prostate, or both. The mean age at presentation is 53 years with a wide range of 42 to 67 years, but can present at any age including children. Some patients have a history of instrumentation or prior transurethral resection but it can also present de novo. The most common clinical presentation is hematuria. There are many histologic patterns described and many patterns can be seen in the same tumor. A subset of IMTs are composed predominantly of stellate-shaped cells loosely arranged in a myxoid or hyalinized matrix with intermixed inflammatory cells, including eosinophils, neutrophils, lymphocytes and plasma cells. In other cases, the cells are more compact and arranged in fascicles or in a storiform architecture and with a more hyalinized stroma, resembling a scar. The spindle cells nuclei can be bland or display marked atypia with large and very prominent nucleoli.35
By immunohistochemistry, IMTs stain with myoid markers including actin, desmin, and caldesmon. Cytokeratin stains including AE1/3, CAM 5.2 or CK18 can also be frequently positive, which could lead to misinterpretation as sarcomatoid carcinoma. Occasionally, IMTs can be associated with sarcomatoid carcinoma but sarcomatoid carcinoma should display a more pronounced atypia and nuclear pleomorphism. P53 can also be positive in a large proportion of cases. Both, post-instrumentation and de novo cases can bear ALK -gene rearrangement in approximately two thirds of the cases, which can be detected either by FISH or by immunohistochemistry. Studies show a wide range of positivity for ALK by immunohistochemistry and there is evidence suggesting that different fusion partners result in different patterns of immunoreactivity, with cytoplasmic, nuclear or perinuclear staining.
IMTs are considered spindle cell neoplasms with borderline malignancy with a propensity to be locally infiltrative and recur after excision. A very small proportion (<2%) of extrapulmonary IMTs will give distant metastasis, and there is some evidence to suggest that those with an epithelioid morphology and with a specific ALK-RANBP2 translocation and the ALK-negative ones have such predisposition.References:
1. Liu C, Zhao X, Zhao Z, Lu P, Jin F, Li G. Malignant inflammatory myofibroblastic tumor of the prostate. J Clin Oncol. 2013;31(10):e144-147.
2. Zeng J, He RQ, Mo WG, et al. Inflammatory myofibroblastic tumor of the prostate after transurethral resection of the prostate with negative expression of anaplastic lymphoma kinase: a case report. Sao Paulo Med J. 2018;136(5):484-487.
3. Montgomery EA, Shuster DD, Burkart AL, et al. 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):1502-1512.
4. Hansel DE, Herawi M, Montgomery E, Epstein JI. Spindle cell lesions of the adult prostate. Mod Pathol. 2007;20(1):148-158.
5. Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol. 2007;31(4):509-520.
6. Chen ST, Lee JC. An inflammatory myofibroblastic tumor in liver with ALK and RANBP2 gene rearrangement: combination of distinct morphologic, immunohistochemical, and genetic features. Hum Pathol. 2008;39(12):1854-1858.
7. Patel AS, Murphy KM, Hawkins AL, et al. RANBP2 and CLTC are involved in ALK rearrangements in inflammatory myofibroblastic tumors. Cancer Genet Cytogenet. 2007;176(2):107-114.
8. Ma Z, Hill DA, Collins MH, et al. Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. Genes Chromosomes Cancer. 2003;37(1):98-105.
9. Bridge JA, Kanamori M, Ma Z, et al. Fusion of the ALK gene to the clathrin heavy chain gene, CLTC, in inflammatory myofibroblastic tumor. Am J Pathol. 2001;159(2):411-415.
10. Marino-Enriquez A, Wang WL, Roy A, et al. Epithelioid inflammatory myofibroblastic sarcoma: An aggressive intra-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol. 2011;35(1):135-144.
11. Debelenko LV, Arthur DC, Pack SD, Helman LJ, Schrump DS, Tsokos M. Identification of CARS-ALK fusion in primary and metastatic lesions of an inflammatory myofibroblastic tumor. Lab Invest. 2003;83(9):1255-1265.Incorrect
Correct: D
Histology: This tumor is stellate-shaped cells loosely arranged in a myxoid matrix with intermixed inflammatory cells, including eosinophils, neutrophils, lymphocytes and plasma cells.
Discussion: Also known as postoperative “spindle cell nodule,” inflammatory myofibroblastic tumors (IMTs) occur more commonly in the bladder but some also affect the prostate, or both. The mean age at presentation is 53 years with a wide range of 42 to 67 years, but can present at any age including children. Some patients have a history of instrumentation or prior transurethral resection but it can also present de novo. The most common clinical presentation is hematuria. There are many histologic patterns described and many patterns can be seen in the same tumor. A subset of IMTs are composed predominantly of stellate-shaped cells loosely arranged in a myxoid or hyalinized matrix with intermixed inflammatory cells, including eosinophils, neutrophils, lymphocytes and plasma cells. In other cases, the cells are more compact and arranged in fascicles or in a storiform architecture and with a more hyalinized stroma, resembling a scar. The spindle cells nuclei can be bland or display marked atypia with large and very prominent nucleoli.35
By immunohistochemistry, IMTs stain with myoid markers including actin, desmin, and caldesmon. Cytokeratin stains including AE1/3, CAM 5.2 or CK18 can also be frequently positive, which could lead to misinterpretation as sarcomatoid carcinoma. Occasionally, IMTs can be associated with sarcomatoid carcinoma but sarcomatoid carcinoma should display a more pronounced atypia and nuclear pleomorphism. P53 can also be positive in a large proportion of cases. Both, post-instrumentation and de novo cases can bear ALK -gene rearrangement in approximately two thirds of the cases, which can be detected either by FISH or by immunohistochemistry. Studies show a wide range of positivity for ALK by immunohistochemistry and there is evidence suggesting that different fusion partners result in different patterns of immunoreactivity, with cytoplasmic, nuclear or perinuclear staining.
IMTs are considered spindle cell neoplasms with borderline malignancy with a propensity to be locally infiltrative and recur after excision. A very small proportion (<2%) of extrapulmonary IMTs will give distant metastasis, and there is some evidence to suggest that those with an epithelioid morphology and with a specific ALK-RANBP2 translocation and the ALK-negative ones have such predisposition.References:
1. Liu C, Zhao X, Zhao Z, Lu P, Jin F, Li G. Malignant inflammatory myofibroblastic tumor of the prostate. J Clin Oncol. 2013;31(10):e144-147.
2. Zeng J, He RQ, Mo WG, et al. Inflammatory myofibroblastic tumor of the prostate after transurethral resection of the prostate with negative expression of anaplastic lymphoma kinase: a case report. Sao Paulo Med J. 2018;136(5):484-487.
3. Montgomery EA, Shuster DD, Burkart AL, et al. 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):1502-1512.
4. Hansel DE, Herawi M, Montgomery E, Epstein JI. Spindle cell lesions of the adult prostate. Mod Pathol. 2007;20(1):148-158.
5. Coffin CM, Hornick JL, Fletcher CD. Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases. Am J Surg Pathol. 2007;31(4):509-520.
6. Chen ST, Lee JC. An inflammatory myofibroblastic tumor in liver with ALK and RANBP2 gene rearrangement: combination of distinct morphologic, immunohistochemical, and genetic features. Hum Pathol. 2008;39(12):1854-1858.
7. Patel AS, Murphy KM, Hawkins AL, et al. RANBP2 and CLTC are involved in ALK rearrangements in inflammatory myofibroblastic tumors. Cancer Genet Cytogenet. 2007;176(2):107-114.
8. Ma Z, Hill DA, Collins MH, et al. Fusion of ALK to the Ran-binding protein 2 (RANBP2) gene in inflammatory myofibroblastic tumor. Genes Chromosomes Cancer. 2003;37(1):98-105.
9. Bridge JA, Kanamori M, Ma Z, et al. Fusion of the ALK gene to the clathrin heavy chain gene, CLTC, in inflammatory myofibroblastic tumor. Am J Pathol. 2001;159(2):411-415.
10. Marino-Enriquez A, Wang WL, Roy A, et al. Epithelioid inflammatory myofibroblastic sarcoma: An aggressive intra-abdominal variant of inflammatory myofibroblastic tumor with nuclear membrane or perinuclear ALK. Am J Surg Pathol. 2011;35(1):135-144.
11. Debelenko LV, Arthur DC, Pack SD, Helman LJ, Schrump DS, Tsokos M. Identification of CARS-ALK fusion in primary and metastatic lesions of an inflammatory myofibroblastic tumor. Lab Invest. 2003;83(9):1255-1265.
