292
development of unusual mass lesions. These are not uncommonly considered malignant until proven otherwise, and patients may undergo resection to define the diagnosis. This may be an inevitable course of action to avoid associated complications (e.g. bowel obstruction). However, in many cases, the disease may respond to immunosuppression, and there are reports of mass lesions regressing to the point that they are no longer discernible clinically or radiologically.2 Positive outcomes have also been reported following glucocorticoid treatment of IgG4-related sclerosing cholangitis.7 In conclusion, surgeons should be aware of the IgG4-RD, and gastrointestinal manifestations (e.g. sclerosing mesenteritis) in particular. Surgery will always maintain a role with regard to incipient complications, but clinicians should also be aware that the disease may respond to medical therapy and obviate the need for resection.
References 1. Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr. Opin. Rheumatol. 2012; 24: 60–9. 2. Minato H, Shimizu J, Arano Y et al. IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology. Pathol. Int. 2012; 62: 281–6. 3. Scudiere JR, Shi C, Hruban RH et al. Sclerosing mesenteritis involving the pancreas: a mimicker of pancreatic cancer. Am J Surg Pathol 2010; 34: 447–53.
Images for surgeons
4. Harrison JD, Rodriguez-Justo M. Commentary on IgG4-related sialadenitis: Mikulicz’s disease, Kuttner’s tumour, and eponymy. Histopathology 2011; 58: 1164–6. 5. Otsuki M, Chung JB, Okazaki K et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan–Korea Symposium on Autoimmune Pancreatitis. J. Gastroenterol. 2008; 43: 403–8. 6. Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J. Gastroenterol. 2007; 42 (Suppl. 18): 39–41. 7. Bjornsson E, Chari S, Silveira M et al. Primary sclerosing cholangitis associated with elevated immunoglobulin G4: clinical characteristics and response to therapy. Am. J. Ther. 2011; 18: 198–205.
Justin S. Gundara, MBBS (Hons) Julian C. Ip, MBBS Anthony R. Glover, FRACS Jaswinder S. Samra, DPhil, FRACS Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital, The University of Sydney, Sydney, NSW, Australia doi: 10.1111/ans.12435
Mullerianosis of the urinary bladder A 30-year-old woman presented with a 3-month history of increasing right iliac fossa pain. She has no active medical conditions, but is a current heavy cigarette and marijuana smoker. Her obstetrics history comprises of an ectopic pregnancy and normal vaginal delivery 10 and 6 years ago, respectively. She has normal menstrual cycles and has no history of endometriosis. Ultrasound imaging showed a large 39 × 37 × 32-mm lesion on the posterior surface of the bladder suspicious of a urothelial carcinoma. Further evaluation with a computed tomography urogram showed this lesion to be confined to the bladder wall (Fig. 1). She underwent cystoscopic evaluation where a polypoid submucosal lesion was seen in the right posterior bladder wall with intact epithelium. The lesion was resected to the level of the bladder wall and during the resection the appearance of glandular structures was evident. It was suspicious of intrusion by extravesical pathology. As a result, a gynaecologist was called into the theatre for further assessment. Hysteroscopy, endometrial curetting and cervical biopsies were taken. The hysteroscopy was unremarkable and the endometrial and cervical tissue were benign. Interestingly, the pathology of the resected bladder lesion returned as endosalpingiosis. The patient was reassured of the pathology and was planned for repeat cystoscopy in 3 months. However, 2 months later, she represented with severe right iliac fossa pain, haematuria and dysuria. Reimaging showed the recurrence of this mass, which was reconfirmed on cystoscopic evaluation. The patient underwent an open partial cystectomy where this large ulcerated mass was completely excised (Fig. 2). She was
Fig. 1. Coronal aspect on computed tomography urogram demonstrating the bladder lesion in the posterior/dome of the bladder (arrow).
reviewed 1 month after her operation and was asymptomatic. The histopathology of the lesion showed mullerianosis of the bladder comprising of endosalpingiosis and endometriosis components (Fig. 3). © 2013 Royal Australasian College of Surgeons
Images for surgeons
293
reported cases of malignant transformation of the endometriosis component into adenocarcinoma.6 Total resection via the transurethral route or open partial cystectomy is recommended. There have been cases where the use of gonadotropin-releasing hormone agonist being used; however, there also have been variable results. This is assumed that the nonendometrial elements in the lesion are not hormone sensitive and do not respond or regress.7 Mullerianosis of the urinary bladder is a rare condition, but it should be considered as a differential diagnosis of benign bladder lesions in premenopausal women. Surgical resection is the optimal treatment for appropriate disease and symptomatic control. Fig. 2. Ulcerated lesion in the posterior bladder wall at time of partial cystectomy.
Acknowledgement Dr Terry Brain for providing photos of histopathology slides.
Mullerianosis of the bladder is a rare condition where there have been fewer than 30 cases reported in the literature. We report the first documented case in Australia. Young and Clement first described this pathology in 1996 as a mixture of at least two of the three benign mullerian-derived glandular epithelial proliferation – endocervicosis, endosalpingiosis and endometriosis – which involves the lamina propria and muscularis propria of the bladder.1 It clinically manifests as lower abdominal and pelvic pain and also has urinary symptoms comprising of urgency, frequency, haematuria and dysuria.2 It is commonly seen in premenapausal women and in some cases the symptoms are cyclical to coincide with the menstrual cycle. There are two theories on its pathogenesis. The first is implantation of extrauterine mullerian tissue during pelvic surgery and the second theory is based on a metaplastic origin. The latter is presumed because of the presence of two or more mullerian tissue rather than one that could have been implanted during pelvic surgery. Also the location of the lesion is predominantly on the dome or posterior wall of the bladder, an area that corresponds to its peritoneal covering, which is receptive to female hormones.3 The differential diagnosis to mullerianosis includes cystitis cystica, cystitis glandularis and nephrogenic adenoma.4 The distinguishing feature of mullerianosis is that it involves the muscularis propria where as the other benign conditions are confined to the lamina propria. Its infiltrative picture can result it in being misdiagnosed as an adenocarcinoma. However, there is a lack of nuclear atypia, mitotic activity and stromal reaction.5 There have been
Fig. 3. (a) Section showing normal bladder serosa (black arrow) with endosalpingiosis deposit (white arrow) disrupting muscularis propia. No endometrial stroma between endosalpingiosis lining and normal muscularis propia of bladder wall. (b) Section showing normal muscularis propia of bladder with disruption by cyst-like lumen endometrium deposit. Presence of endometrial stroma (red arrow) and normal endometrial lining with tubule type epithelium and columnar cells.
© 2013 Royal Australasian College of Surgeons
References 1. Young RH, Clement PB. Mullerianosis of the urinary bladder. Mod. Pathol. 1996; 9: 731–7. 2. Guan H, Rosenthal DL, Erozan YS. Mullerianosis of the urinary bladder: report of a case with diagnosis suggested in urine cytology and review of literature. Diagn. Cytopathol. 2012; 40: 997–1001. 3. Donné C, Vidal M, Buttin X et al. Mullerianosis of the urinary bladder: clinical and immunohistochemical findings. Histopathology 1998; 33: 290–2. 4. Koren J, Mensikova J, Mukensnabl P, Zamecnik M. Mullerianosis of the urinary bladder: report of a case with suggested metaplastic origin. Virchows Arch. 2006; 449: 268–71. 5. Olivia Vella JE, Nair N, Ferryman SR, Athavale R, Latthe P, Hirschowitz L. Mullerianosis of the urinary bladder. Int. J. Surg. Pathol. 2011; 19: 548–51. 6. Garavan F, Grainger R, Jeffers M. Endometrioid carcinoma of the urinary bladder complicating vesical mullerianosis: a case report and review of the literature. Virchows Arch. 2004; 444: 587–9. 7. Margulis V, Lemack GE, Molberg K, Saboorian MH. Bladder ‘mullerianosis’ in a woman with lower urinary tract symptoms and hematuria. J. Urol. 2001; 165 (6 Pt 1): 1996–7.
Nieroshan Rajarubendra, MBBS Yit Leang, MBBS Michael Monsour, MBBS, FRACS Urology Department, Launceston General Hospital, Launceston, Tasmania, Australia doi: 10.1111/ans.12430
Copyright of ANZ Journal of Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
development of unusual mass lesions. These are not uncommonly considered malignant until proven otherwise, and patients may undergo resection to define the diagnosis. This may be an inevitable course of action to avoid associated complications (e.g. bowel obstruction). However, in many cases, the disease may respond to immunosuppression, and there are reports of mass lesions regressing to the point that they are no longer discernible clinically or radiologically.2 Positive outcomes have also been reported following glucocorticoid treatment of IgG4-related sclerosing cholangitis.7 In conclusion, surgeons should be aware of the IgG4-RD, and gastrointestinal manifestations (e.g. sclerosing mesenteritis) in particular. Surgery will always maintain a role with regard to incipient complications, but clinicians should also be aware that the disease may respond to medical therapy and obviate the need for resection.
References 1. Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr. Opin. Rheumatol. 2012; 24: 60–9. 2. Minato H, Shimizu J, Arano Y et al. IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology. Pathol. Int. 2012; 62: 281–6. 3. Scudiere JR, Shi C, Hruban RH et al. Sclerosing mesenteritis involving the pancreas: a mimicker of pancreatic cancer. Am J Surg Pathol 2010; 34: 447–53.
Images for surgeons
4. Harrison JD, Rodriguez-Justo M. Commentary on IgG4-related sialadenitis: Mikulicz’s disease, Kuttner’s tumour, and eponymy. Histopathology 2011; 58: 1164–6. 5. Otsuki M, Chung JB, Okazaki K et al. Asian diagnostic criteria for autoimmune pancreatitis: consensus of the Japan–Korea Symposium on Autoimmune Pancreatitis. J. Gastroenterol. 2008; 43: 403–8. 6. Chari ST. Diagnosis of autoimmune pancreatitis using its five cardinal features: introducing the Mayo Clinic’s HISORt criteria. J. Gastroenterol. 2007; 42 (Suppl. 18): 39–41. 7. Bjornsson E, Chari S, Silveira M et al. Primary sclerosing cholangitis associated with elevated immunoglobulin G4: clinical characteristics and response to therapy. Am. J. Ther. 2011; 18: 198–205.
Justin S. Gundara, MBBS (Hons) Julian C. Ip, MBBS Anthony R. Glover, FRACS Jaswinder S. Samra, DPhil, FRACS Upper Gastrointestinal Surgical Unit, Royal North Shore Hospital, The University of Sydney, Sydney, NSW, Australia doi: 10.1111/ans.12435
Mullerianosis of the urinary bladder A 30-year-old woman presented with a 3-month history of increasing right iliac fossa pain. She has no active medical conditions, but is a current heavy cigarette and marijuana smoker. Her obstetrics history comprises of an ectopic pregnancy and normal vaginal delivery 10 and 6 years ago, respectively. She has normal menstrual cycles and has no history of endometriosis. Ultrasound imaging showed a large 39 × 37 × 32-mm lesion on the posterior surface of the bladder suspicious of a urothelial carcinoma. Further evaluation with a computed tomography urogram showed this lesion to be confined to the bladder wall (Fig. 1). She underwent cystoscopic evaluation where a polypoid submucosal lesion was seen in the right posterior bladder wall with intact epithelium. The lesion was resected to the level of the bladder wall and during the resection the appearance of glandular structures was evident. It was suspicious of intrusion by extravesical pathology. As a result, a gynaecologist was called into the theatre for further assessment. Hysteroscopy, endometrial curetting and cervical biopsies were taken. The hysteroscopy was unremarkable and the endometrial and cervical tissue were benign. Interestingly, the pathology of the resected bladder lesion returned as endosalpingiosis. The patient was reassured of the pathology and was planned for repeat cystoscopy in 3 months. However, 2 months later, she represented with severe right iliac fossa pain, haematuria and dysuria. Reimaging showed the recurrence of this mass, which was reconfirmed on cystoscopic evaluation. The patient underwent an open partial cystectomy where this large ulcerated mass was completely excised (Fig. 2). She was
Fig. 1. Coronal aspect on computed tomography urogram demonstrating the bladder lesion in the posterior/dome of the bladder (arrow).
reviewed 1 month after her operation and was asymptomatic. The histopathology of the lesion showed mullerianosis of the bladder comprising of endosalpingiosis and endometriosis components (Fig. 3). © 2013 Royal Australasian College of Surgeons
Images for surgeons
293
reported cases of malignant transformation of the endometriosis component into adenocarcinoma.6 Total resection via the transurethral route or open partial cystectomy is recommended. There have been cases where the use of gonadotropin-releasing hormone agonist being used; however, there also have been variable results. This is assumed that the nonendometrial elements in the lesion are not hormone sensitive and do not respond or regress.7 Mullerianosis of the urinary bladder is a rare condition, but it should be considered as a differential diagnosis of benign bladder lesions in premenopausal women. Surgical resection is the optimal treatment for appropriate disease and symptomatic control. Fig. 2. Ulcerated lesion in the posterior bladder wall at time of partial cystectomy.
Acknowledgement Dr Terry Brain for providing photos of histopathology slides.
Mullerianosis of the bladder is a rare condition where there have been fewer than 30 cases reported in the literature. We report the first documented case in Australia. Young and Clement first described this pathology in 1996 as a mixture of at least two of the three benign mullerian-derived glandular epithelial proliferation – endocervicosis, endosalpingiosis and endometriosis – which involves the lamina propria and muscularis propria of the bladder.1 It clinically manifests as lower abdominal and pelvic pain and also has urinary symptoms comprising of urgency, frequency, haematuria and dysuria.2 It is commonly seen in premenapausal women and in some cases the symptoms are cyclical to coincide with the menstrual cycle. There are two theories on its pathogenesis. The first is implantation of extrauterine mullerian tissue during pelvic surgery and the second theory is based on a metaplastic origin. The latter is presumed because of the presence of two or more mullerian tissue rather than one that could have been implanted during pelvic surgery. Also the location of the lesion is predominantly on the dome or posterior wall of the bladder, an area that corresponds to its peritoneal covering, which is receptive to female hormones.3 The differential diagnosis to mullerianosis includes cystitis cystica, cystitis glandularis and nephrogenic adenoma.4 The distinguishing feature of mullerianosis is that it involves the muscularis propria where as the other benign conditions are confined to the lamina propria. Its infiltrative picture can result it in being misdiagnosed as an adenocarcinoma. However, there is a lack of nuclear atypia, mitotic activity and stromal reaction.5 There have been
Fig. 3. (a) Section showing normal bladder serosa (black arrow) with endosalpingiosis deposit (white arrow) disrupting muscularis propia. No endometrial stroma between endosalpingiosis lining and normal muscularis propia of bladder wall. (b) Section showing normal muscularis propia of bladder with disruption by cyst-like lumen endometrium deposit. Presence of endometrial stroma (red arrow) and normal endometrial lining with tubule type epithelium and columnar cells.
© 2013 Royal Australasian College of Surgeons
References 1. Young RH, Clement PB. Mullerianosis of the urinary bladder. Mod. Pathol. 1996; 9: 731–7. 2. Guan H, Rosenthal DL, Erozan YS. Mullerianosis of the urinary bladder: report of a case with diagnosis suggested in urine cytology and review of literature. Diagn. Cytopathol. 2012; 40: 997–1001. 3. Donné C, Vidal M, Buttin X et al. Mullerianosis of the urinary bladder: clinical and immunohistochemical findings. Histopathology 1998; 33: 290–2. 4. Koren J, Mensikova J, Mukensnabl P, Zamecnik M. Mullerianosis of the urinary bladder: report of a case with suggested metaplastic origin. Virchows Arch. 2006; 449: 268–71. 5. Olivia Vella JE, Nair N, Ferryman SR, Athavale R, Latthe P, Hirschowitz L. Mullerianosis of the urinary bladder. Int. J. Surg. Pathol. 2011; 19: 548–51. 6. Garavan F, Grainger R, Jeffers M. Endometrioid carcinoma of the urinary bladder complicating vesical mullerianosis: a case report and review of the literature. Virchows Arch. 2004; 444: 587–9. 7. Margulis V, Lemack GE, Molberg K, Saboorian MH. Bladder ‘mullerianosis’ in a woman with lower urinary tract symptoms and hematuria. J. Urol. 2001; 165 (6 Pt 1): 1996–7.
Nieroshan Rajarubendra, MBBS Yit Leang, MBBS Michael Monsour, MBBS, FRACS Urology Department, Launceston General Hospital, Launceston, Tasmania, Australia doi: 10.1111/ans.12430
Copyright of ANZ Journal of Surgery is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
![[Melanosis of the urinary bladder].](/assets/img/hold.png)
