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

Inflammatory myofibroblastic tumor (IMT) of the urinary tract, also termed postoperative spindle cell nodule, inflammatory pseudotumor, and pseudosarcomatous fibromyxoid tumor, is rare and in the past was believed to reflect diverse entities. We reviewed a series of 46 IMTs arising in the ureter, bl...

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Published in:	The American journal of surgical pathology Vol. 30; no. 12; pp. 1502 - 1512
Main Authors:	MONTGOMERY, Elizabeth A, SHUSTER, Dawn D, BURKART, Ashlie L, ESTEBAN, Jose M, SGRIGNOLI, Anita, ELWOOD, Lori, VAUGHN, David J, GRIFFIN, Constance A, EPSTEIN, Jonathan I
Format:	Journal Article
Language:	English
Published:	Hagerstown, MD Lippincott Williams & Wilkins 01-12-2006
Subjects:	Adolescent Adult Aged Aged, 80 and over Biological and medical sciences Biomarkers, Tumor - metabolism Carcinoma, Transitional Cell - enzymology Carcinoma, Transitional Cell - genetics Carcinoma, Transitional Cell - pathology Child Child, Preschool Female Fibrosarcoma - enzymology Fibrosarcoma - genetics Fibrosarcoma - pathology Granuloma, Plasma Cell - enzymology Granuloma, Plasma Cell - genetics Granuloma, Plasma Cell - pathology Humans In Situ Hybridization, Fluorescence Inflammation - pathology Investigative techniques, diagnostic techniques (general aspects) Male Medical sciences Middle Aged Nephrology. Urinary tract diseases Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques Prostate - enzymology Prostate - pathology Protein-Tyrosine Kinases - genetics Protein-Tyrosine Kinases - metabolism Receptor Protein-Tyrosine Kinases Tumors of the urinary system Ureter - enzymology Ureter - pathology Urinary Bladder - enzymology Urinary Bladder - pathology Urinary tract. Prostate gland Urologic Diseases - enzymology Urologic Diseases - genetics Urologic Diseases - pathology Urothelium - enzymology Urothelium - pathology ALK Urinary tract tumor Urinary system disease Sarcoma Myofibroblast inflammatory myofibroblastic tumor Fibrosarcoma Inflammation Urinary tract disease Transitional cell carcinoma Malignant tumor Urinary tract High grade Case study Symptomatology Anatomic pathology Association Urinary system Urinary bladder Fluorescence in situ hybridization bladder pseudosarcoma FISH High malignancy
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Summary:	Inflammatory myofibroblastic tumor (IMT) of the urinary tract, also termed postoperative spindle cell nodule, inflammatory pseudotumor, and pseudosarcomatous fibromyxoid tumor, is rare and in the past was believed to reflect diverse entities. We reviewed a series of 46 IMTs arising in the ureter, bladder, and prostate, derived primarily from a large consultation practice. There were 30 male and 16 females aged 3 to 89 years (mean 53.6). Lesions were 1.2 to 12 cm (mean 4.2). There was a history of recent prior instrumentation in 8 cases. Morphology was similar to that previously described for IMT occurring in this region, with the exception of 1 case that focally appeared sarcomatous. Polypoid cystitis coexisted in 5 patients (11%). Mitoses were typically scant (0 to 20/10 hpf, mean 1). Necrosis was seen in 14 (30%) cases. Invasion of the muscularis propria was documented in 19 (41%). By immunohistochemistry (IHC), lesions at least focally expressed anaplastic lymphoma kinase (ALK) (20/35, 57%), AE1/3 (25/34, 73%), CAM5.2 (10/15, 67%), CK18 (6/6, 100%), actin (23/25, 92%), desmin (15/19, 79%), calponin (6/7, 86%), caldesmon (4/7, 57%, rare cells), p53 (10/13, 77%), and most lacked S100 (0/14), CD34 (0/13), CD117 (2/13, 15%), CD21 (0/5), and CD23 (0/3). ALK gene alterations were detected by fluorescence in situ hybridization (FISH) in 13/18 (72%) tested cases, including 2 with prior instrumentation; 13/18 (72%) showed agreement between FISH ALK results and ALK protein results by IHC. Most bladder IMTs were managed locally, but partial cystectomy was performed as the initial management in 7 cases and cystectomy in 1 (1 IMT was initially misinterpreted as carcinoma, 1 IMT was found incidentally as a separate lesion in a cystectomy specimen performed for urothelial carcinoma). Follow-up was available in 32 cases (range 3 to 120 mo; mean 33; median 24). There were 10 patients with recurrences (2 with 2 recurrences). Recurrences were unassociated with muscle invasion or with ALK alterations. In 2 cases, tumors of the urinary tract (TURs) showing IMT preceded (1 and 2 mo, respectively) TURs showing sarcomatoid carcinoma with high-grade invasive urothelial carcinoma accompanied with separate fragments of IMT. Even on re-review the IMT in these 2 cases were morphologically indistinguishable from other cases of IMT, with FISH demonstrating ALK alterations in the IMT areas in one of them. Both these patients died of their carcinomas. Lastly, there was 1 tumor with many morphological features of IMT and an ALK rearrangement, yet overtly sarcomatous. This case arose postirradiation for prostate cancer 4 years before the development of the lesion, with tumor recurrence at 4 months and death from intra-abdominal metastatic disease at 9 months. In summary, urinary tract IMTs are rare and share many features with counterparts in other sites, displaying similar morphology and immunogenotypic features whether de novo or postinstrumentation. Typical IMTs can be locally aggressive, sometimes requiring radical surgical resection, but none of our typical cases metastasized, although they can rarely arise contemporaneously with sarcomatoid urothelial carcinomas. For these reasons, close follow-up is warranted.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0147-5185 1532-0979
DOI:	10.1097/01.pas.0000213280.35413.1b