Inverted Urothelial Papilloma of the Upper Urinary Tract: Description of Two Cases with Systematic Literature Review R

Santi et al. Diagnostic Pathology (2020) 15:40 https://doi.org/10.1186/s13000-020-00961-9 RESEARCH Open Access Inverted urothelial papilloma of the upper urinary tract: description of two cases with systematic literature review R. Santi1, I. C. Galli2, V. Canzonieri3,4, J. I. Lopez5 and G. Nesi2* Abstract Background: Inverted urothelial papilloma (IUP) of the upper urinary tract is an uncommon benign tumour that occasionally presents as a polypoid mass causing urinary obstruction. Histologically, IUP is characterised by a proliferating urothelium arranged in cords and trabeculae, in continuity with overlying intact epithelium, and extending into the lamina propria in a non-invasive, endophytic manner. Cytological atypia is minimal or absent. Top differential diagnoses include urothelial carcinoma with inverted growth pattern and florid ureteritis cystica. Although urothelial carcinomas of the upper urinary tract with prominent inverted growth pattern commonly harbour microsatellite instability, the role of the mutator phenotype pathway in IUP development is still unclear. The aim of this study was to describe two additional cases of IUP of the upper urinary tract, along with an extensive literature review. Case presentation: We observed two polypoid tumours originating in the renal pelvis and the distal ureter, respectively. Both patients, a 76-year-old woman and a 56-year-old man, underwent surgery because of the increased likelihood of malignancy. Histology was consistent with IUP and patients are alive and asymptomatic after long-term follow-up (6 years for the renal pelvis lesion and 5 years for the ureter lesion). The tumours retained the expression of the mismatch-repair protein MLH1, MSH2, and PMS2 whereas loss of MSH6 was found in both cases. Conclusions: When completely resected, IUP does not require rigorous surveillance protocols, such as those for urothelial carcinoma and exophytic urothelial papilloma. It is therefore important for the surgical pathologist to be aware of this rare entity in order to ensure correct patient management. Keywords: Inverted urothelial papilloma, Upper urinary tract, Molecular markers, Microsatellite instability Background It usually occurs at the bladder neck, trigone or prostatic Inverted urothelial papilloma (IUP) is a rare lesion, his- urethra, but is rare in the upper urinary tract. To the tologically similar to inverted papilloma of the nasal cav- best of our knowledge, 68 IUP cases of the renal pelvis ity and paranasal sinuses. First reported in 1927 by and ureter have been described in the English literature Paschkis as “polypoid adenoma of the bladder” [1], it (Tables 1 and 2)[3–52]. was later described in 1963 by Potts and Hirst as a dis- Histological diagnosis of IUP can be difficult and sev- tinct tumour entity of the urinary bladder [2]. IUP ac- eral pathological conditions may enter differential diag- counts for approximately 2% of all urothelial neoplasms. nosis, including other urothelial neoplasms with endophytic growth patterns (i.e. papillary urothelial neo- * Correspondence: [email protected] plasm of low malignant potential, low- and high-grade 2Pathology Section, Department of Health Sciences, University of Florence, Viale Pieraccini 6, 50139 Florence, Italy urothelial carcinoma), nested urothelial carcinoma, para- Full list of author information is available at the end of the article ganglioma, carcinoid tumour, florid von Brunn nest © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Santi et al. Diagnostic Pathology (2020) 15:40 Page 2 of 10 Table 1 IUP of the renal pelvis (RP) previously reported in the English Literature (NS = Not Stated; NA = Not Assessed) Reference Age Sex Presentation Site Gross/ Associated Urothelial Treatment Recurrence (Follow- Maximum Lesions Up) Diameter (cm) Matz et al. 68 M Haematuria, Left RP Nodule/1.5 None Nephroureterectomy None (2 ys) (1974) [3] flank pain Assor (1976) 79 M Haematuria, Right RP Sessile polyp/ None Partial resection NS [4] flank 1.5 discomfort Cameron 58 F NS RP (side NS) NS/3 None Nephroureterectomy Patient died of et al. (1976) carcinoma of the [5] endometrium four years later Di Cello et al. 53 M Asymptomatic Left uretero-pelvic Sessile polyp/ None Nephroureterectomy NS (1980) [6] junction 3 Theoret et al. 89 M Asymptomatic RP (side NS) NS None NA NA (1980) [7] (autopsy finding) Uyama et al. 73 M Haematuria Left RP NS/2.5 None Nephroureterectomy, None (5 ys) (1981) [8] radiation and chemotherapy Anderström 62 M Asymptomatic Left RP Nodule/3 Synchronous grade 2 Extracorporeal Patient died of et al. (1982) transitional cell resection of ureter metastatic poorly [9] carcinoma of and RP and differentiated contralateral RP and autotransplant of squamous cell non-invasive grade 2 kidney to bladder carcinoma of the transitional cell carcin- bladder three years oma of the bladder; later; no recurrence history of recurrent in the kidney where grade 2 transitional IUP was diagnosed cell carcinoma of the bladder Anderström 49 M Ureteral colic Right RP Nodule/NS None NS NS et al. (1982) [9] Watters et al. 65 M Haematuria Left RP Pedunculated None Nephroureterectomy NS (1983) [10] polyp/1 Lausten et al. 63 M NS Right RP Pedunculated Grade 3 invasive Nephrectomy None (8.5 ys) (1984) [11] polyp/1 polypoid transitional cell carcinoma in the contralateral RP after 8 years Taylor et al. 65 M Haematuria Right RP Sessile polyp/ None Nephroureterectomy None (2 ys) (1986) [12] NS Schulze et al. 53 M Haematuria Right RP Sessile polyp/ None Nephroureterectomy NS (1986) [13] 2.5 Schulze et al. 55 M Haematuria Left RP and ureter Not apparent None Nephrectomy NS (1986) [13] at gross examination Romanelli 52 M Haematuria, Right RP Sessile polyp/ None Nephroureterectomy NS (1986) [14] renal colic, 2.1 Yamaguchi 73 M Haematuria Left RP Pedunculated Synchronous low Nephrectomy None (1 y) et al. (1988) polyp/0.6 grade transitional cell [15] carcinoma of the bladder (ureteral orifice) Schultz et al. 58 M Haematuria Left RP NS Synchronous Pyelotomy and IUP of the bladder 1 (1988) [16] superficial grade 2 endoscopic resection y later transitional cell carcinoma of the contralateral ureter (nephroureterectomy Santi et al. Diagnostic Pathology (2020) 15:40 Page 3 of 10 Table 1 IUP of the renal pelvis (RP) previously reported in the English Literature (NS = Not Stated; NA = Not Assessed) (Continued) Reference Age Sex Presentation Site Gross/ Associated Urothelial Treatment Recurrence (Follow- Maximum Lesions Up) Diameter (cm) with excision of the bladder cuff) Aubert et al. 34 M Haematuria Left RP NS None Nephroureterectomy None (18 months) (1988) [17] Kyriakos et al. 73 F Asymptomatic Multiple lesions: Polyp/2.6 (I); None Nephroureterectomy None (11 months) (1989) [18] Junction between slightly a upper pole elevated major calyx and nodule/1 (II); right RP (I); right polyp/0.5 (III); calix (II); distal polyp/1.2 (IV) right ureter (III and IV) Bagley et al. 64 M Haematuria Right RP Nodule/1 Recurrent transitional Ureteropyeloscopy None (6 months) (1990) [19] cell carcinoma of the with endoscopic bladder resection Bassi et al. 51 M Haematuria, Left RP Sessile polyp/ None Partial resection NS (1991) [20] flank pain 0.5 Vlassopopulos 59 M Haematuria, Left RP Sessile polyp/ None Nephroureterectomy None (12 months) et al. (1992) flank pain 2 [21] Ueda T et al. 71 M Asymptomatic Right RP Nodule/4 None Nephrectomy Synchronous clear (1992) [22] cell carcinoma of the homolateral kidney, treated with surgery and anticancer drugs. No recurrence from IUP (21 months) Spevack et al. 64 M Haematuria Right RP Pedunculated None Partial resection None (42 months) (1995) [23] polyp/2.5 Chiura et al. 63 M Haematuria Right RP NS/3 Transitional cell Nephroureterectomy None (1 y after (1998) [24] carcinoma of the left surgery for distal ureter three carcinoma) years later, treated with surgery, radiation therapy and chemotherapy Chiura et al. 53 M Haematuria RP NS Pyelitis cystica Nephroureterectomy NS (1998) [24] Chiura et al. 64 M Asymptomatic Right RP NS Recurrent transitional Ureteroscopy and Transitional cell (1998) [24] cell carcinoma of the biopsy carcinoma in the bladder (previous and homolateral kidney subsequent to IUP and ureter 9 ys later diagnosis) Darras et al. 52 M Haematuria, Left RP Polyp/NS Synchronous IUP of Partial resection None (NS) (2005) [25] occasional the bladder discomfort in the lower abdomen Luo et al. 62 M Asymptomatic Right RP Pedunculated None Nephroureterectomy None (NS) (2012) [26] polyp Luo et al. 66 M Haematuria Left RP Pedunculated None Nephroureterectomy None (NS) (2012) [26] polyp Luo et al. 64 M Haematuria Left RP Pedunculated None Nephroureterectomy None (NS) (2012) [26] polyp Luo et al. 73 F Flank pain Right RP Pedunculated None Nephroureterectomy None (NS) (2012) [26] polyp Santi et al. Diagnostic Pathology (2020) 15:40 Page 4 of 10 Table 2 IUP of the ureter (U) previously reported in the English Literature (NS = Not Stated; NA = Not Assessed) Reference Age Sex Presentation Site Gross/Maximum Associated Urothelial Lesions Treatment Rrecurrence Diameter (cm) (Follow-Up) Geisler et al.

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