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“An Unusual Urological Tumour”: Above the Collar and below the Belt

“An Unusual Urological Tumour”: Above the Collar and below the Belt Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 480826, 3 pages doi:10.1155/2012/480826 Case Report “An Unusual Urological Tumour”: Above the Collar and below the Belt 1 1 1 2 Erik K. Mayer, Shabnam Undre, Daniel C. Cohen, Marjorie M. Walker, 1 1 Justin A. Vale, and Anup Patel Department of Urology, St. Mary’s Hospital, Imperial College Healthcare NHS Trust, Praed Street, London W2 1NY, UK Department of Histopathology, St. Mary’s Hospital, Imperial College Healthcare NHS Trust, Praed Street, London W2 1NY, UK Correspondence should be addressedtoErikK.Mayer, e.mayer@imperial.ac.uk andDanielC.Cohen, daniel.cohen@imperial.ac.uk Received 10 July 2012; Accepted 2 September 2012 Academic Editors: K. Aogi, L. Beex, G. Di Vagno, and P. F. Lenehan Copyright © 2012 Erik K. Mayer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Bladder lymphomas are rarely primary tumours and more commonly associated with systemic lymphoma, either as nonlocalised bladder lymphoma or as secondary bladder lymphoma. Primary bladder lymphomas (PBL) tend to be low-grade mucosa- associated lymphoid tissue (MALT) type, contrasting with diffuse large cell or follicular centre cell types more commonly seen in secondary bladder lymphoma. Bladder involvement by systemic lymphoma infers poor prognosis and patients often have no localising symptoms (typically a postmortem diagnosis). Other treatmentsare preferredoversurgery forall bladderlymphomas, except where diagnosis is uncertain or for relief of irritative bladder symptoms. We describe a unique case of systemic high-grade B-cell lymphoma with simultaneous cutaneous renal and bladder lesions at presentation. 1. Case Report more consistent with lymphoid than epithelial/transitional cell origin (Figure 2(a)). At cystoscopy, a 3 cm round A 70-year-old man presented to the dermatologists with a 2- submucosal lesion on the anterior aspect of the bladder year history of painless enlarging scalp nodules, which had wall was seen and resected. Histology confirmed high- rapidly worsened over the previous 6 months (Figure 1(a)). grade B-cell lymphoma in both bladder and kidney biopsies He had lost 7 kg in weight; there were no other systemic (Figure 2(b)). symptoms. He had neither clinical lymphadenopathy nor hepatosplenomegaly and his peripheral blood count, ESR, 2. Discussion protein electrophoresis, liver function tests, and LDH were normal. A skin biopsy from the scalp demonstrated high- To date there are few case reports of nonprimary bladder grade B-cell non-Hodgkin’s lymphoma. Staging CT showed lymphoma, reflecting its rarity as a presenting pathological multiple small cervical lymphadenopathy, lymphomatous entity. To our knowledge we describe the first reported case infiltration in both kidneys, and an area of mucosal thick- of disseminated lymphoma with simultaneous cutaneous, ening of the bladder, which was consistent with a primary renal, and bladder lesions demonstrable at presentation. bladder carcinoma. Staging was completed with an MRI of The incidence of non-Hodgkin’s lymphoma (NHL) is the head (Figure 1(b)) and bone marrow biopsy, which was increasing and maintains a geographical variability: in 2007 normal. there were 10,900 people diagnosed with NHL in the UK The patient described neither lower urinary tract symp- [1]. The incidence of NHL typically increases with age. Rates toms nor episodes of macroscopic haematuria and was a increase sharply in people over 50 and around two-thirds nonsmoker. A urine cytology specimen revealed atypical cells (68%) of all cases are diagnosed in people over 60 years. 2 Case Reports in Oncological Medicine (a) (b) Figure 1: (a) Scalp nodules as seen at presentation. (b) MRI demonstrating an extensive mixed signal, soft tissue mass, over the vertex of the skull. There is infiltration through the inner and outer tables of the skull vault and extension into the dural membranes. H and E CD20 (a) (b) Figure 2: (a) Urine cytology demonstrates a cluster of atypical lymphoid cells. (b) Biopsy of bladder tumour showing infiltration of the transitional epithelium and lamina propria by atypical B lymphocytes (CD20 positive on immunohistochemistry). The most recent classification system is that endorsed by of patients with bladder involvement experience no urinary the World Health Organisation (REAL classification) and symptoms, urinary urgency, frequency, and haematuria have includes subtypes such as diffuse large B-cell, follicular, all been described [3]. It has been reported that a high and small lymphocytic. Determinations of specific subtypes proportion (47%) of patients in this group experience of disease along with patient’s prognostic characteristics ureteric obstruction [3]. gleaned from the International Prognostic Index are crucial Nonprimary bladder lymphomas tend to be a dif- to treatment plans and subsequent management. ferent histological type than those found in the PBL Lymphomas of the bladder are rare and account for 0.2% group and are commonly of the diffuse large cell or of all bladder neoplasms [2]. There is a female predominance follicular centre cell types [3]. In the nonprimary group, and average age of presentation of 58 years. The most the bladder involvement by systemic lymphoma infers a common type is extranodal marginal zone lymphoma of poorer prognosis than that seen in PBL as it implies mucosal-associated lymphoid tissue (MALT) first described multisystem disease. However, there is a clear prognostic in 1990 [3], while Hodgkin’s type is even rarer. Lymphomas distinction between the secondary bladder lymphoma and are classed into the primary group if they present primarily non-localised lymphoma groups with median survival of with bladder symptoms and there is no evidence of systemic 0.6 years and 9 years, respectively [3]. Part of this might lymphoma at presentation. be explained by some patients being classified in the non- More commonly, although relatively few case studies localised group when a dominant bladder mass was only exist, lymphoma of the bladder is seen in association associated with local extension to adjacent organs or to with systemic lymphoma; this can either be as bladder regional lymph nodes [3]. The authors argued that although involvement with active systemic lymphoma (nonlocalised some might classify this category of patient into a “PBL” bladder lymphoma), as reported in this case study, or as group, their tumour histology resembled more closely that lymphoma recurrence in the bladder after a period of of the general population and the patient and disease remission from previous lymphoma (secondary lymphoma) characteristics were distinct from those seen in the PBL [3]. It is rare for disseminated lymphoma to present group [3]. with symptoms of bladder involvement and more typically Non-Hodgkin’s lymphoma involving the bladder can remains a postmortem diagnosis [2]. Although a percentage be successfully treated using chemotherapy, radiotherapy, Case Reports in Oncological Medicine 3 immunotherapy, surgery, and combinations of these modal- ities [4]. The treatment of choice is determined by the histology and extent of disease. The systemic nature of non- primary bladder lymphoma warrants a systemic approach to treatment. Lymphomas with aggressive phenotypes such as diffuse large B-cell are best managed with chemotherapy. The cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (CHOP/R) regime is most commonly used [4]. Adjuvant radiotherapy is used for involved fields in localised disease. Follicular lymphoma treatments range from a watch-and-wait policy to chemoimmunotherapy or radioimmunotherapy. Immunotherapy generally forms a standard component of management of advanced disease [4]. The indolent nature of the MALT lymphomas means that local radiotherapy can be used with curative intent, such as in PBL. Surgical resection has a limited role in primary bladder lymphoma, beyond obtaining biopsy specimens, and rarely adds anything to management or prognosis. There are reports, however, of patients having undergone cystectomy due to unclear diagnosis, or to relieve irritative symptoms [5]. There is a report of a low-grade MALT-type PBL being managed with transurethral resection and adjuvant intravesical mitoxantrone [6]. In this case, the patient’s disease constituted stage IV B. He underwent eight cycles of CHOP/R chemotherapy followed by radical radiotherapy to scalp and brain. Follow- up MRI showed dramatic improvement in the scalp and dural mass with minimal residual dural enhancement. A repeat CT showed complete remission with no residual disease in either kidneys or bladder. The patient remained in complete remission for 14 months but then developed a gradual deterioration in his balance and thereafter a marked peripheral neuropathy. Repeat imaging confirmed recurrent widespread cerebral metastasis. He died following a cardiac arrest before palliative radiotherapy could be given. References [1] Cancer Research UK, http://info.cancerresearchuk.org/cancer- stats/types/nhl/?script=true. [2] A. W. Bates, A. J. Norton, and S. I. Baithun, “Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases,” Journal of Clinical Pathology, vol. 53, no. 6, pp. 458– 461, 2000. [3] C.L.Kempton,P.J.Kurtin, D. J. Inwards, P. Wollan,and D. G. Bostwick, “Malignant lymphoma of the bladder: evidence from 36 cases that low-grade lymphoma of the malt-type is the most common primary bladder lymphoma,” American Journal of Surgical Pathology, vol. 21, no. 11, pp. 1324–1333, 1997. [4] S. M. Ansell and J. Armitage, “Non-Hodgkin lymphoma: diagnosis and treatment,” Mayo Clinic Proceedings, vol. 80, no. 8, pp. 1087–1097, 2005. [5] K. Horasanli, M. Kadihasanoglu, O. T. Aksakal, A. Ozagari, and C. Miroglu, “A case of primary lymphoma of the bladder managed with multimodal therapy,” Nature Clinical Practice Urology, vol. 5, no. 3, pp. 167–170, 2008. [6] P. Porter, A. J. Cornaby, M. Al-Hilali, and A. M. Chakrabarti, “Primary lymphoma of the bladder treated successfully with mitozantrone gel,” Postgraduate Medical Journal, vol. 75, no. 888, pp. 609–610, 1999. MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 International Journal of Journal of Immunology Research Endocrinology Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Submit your manuscripts at http://www.hindawi.com BioMed PPAR Research Research International Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Research and Treatment Cellular Longevity Neurology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Case Reports in Oncological Medicine Hindawi Publishing Corporation

“An Unusual Urological Tumour”: Above the Collar and below the Belt

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Publisher
Hindawi Publishing Corporation
Copyright
Copyright © 2012 Erik K. Mayer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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2090-6706
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2090-6714
DOI
10.1155/2012/480826
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Abstract

Hindawi Publishing Corporation Case Reports in Oncological Medicine Volume 2012, Article ID 480826, 3 pages doi:10.1155/2012/480826 Case Report “An Unusual Urological Tumour”: Above the Collar and below the Belt 1 1 1 2 Erik K. Mayer, Shabnam Undre, Daniel C. Cohen, Marjorie M. Walker, 1 1 Justin A. Vale, and Anup Patel Department of Urology, St. Mary’s Hospital, Imperial College Healthcare NHS Trust, Praed Street, London W2 1NY, UK Department of Histopathology, St. Mary’s Hospital, Imperial College Healthcare NHS Trust, Praed Street, London W2 1NY, UK Correspondence should be addressedtoErikK.Mayer, e.mayer@imperial.ac.uk andDanielC.Cohen, daniel.cohen@imperial.ac.uk Received 10 July 2012; Accepted 2 September 2012 Academic Editors: K. Aogi, L. Beex, G. Di Vagno, and P. F. Lenehan Copyright © 2012 Erik K. Mayer et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Bladder lymphomas are rarely primary tumours and more commonly associated with systemic lymphoma, either as nonlocalised bladder lymphoma or as secondary bladder lymphoma. Primary bladder lymphomas (PBL) tend to be low-grade mucosa- associated lymphoid tissue (MALT) type, contrasting with diffuse large cell or follicular centre cell types more commonly seen in secondary bladder lymphoma. Bladder involvement by systemic lymphoma infers poor prognosis and patients often have no localising symptoms (typically a postmortem diagnosis). Other treatmentsare preferredoversurgery forall bladderlymphomas, except where diagnosis is uncertain or for relief of irritative bladder symptoms. We describe a unique case of systemic high-grade B-cell lymphoma with simultaneous cutaneous renal and bladder lesions at presentation. 1. Case Report more consistent with lymphoid than epithelial/transitional cell origin (Figure 2(a)). At cystoscopy, a 3 cm round A 70-year-old man presented to the dermatologists with a 2- submucosal lesion on the anterior aspect of the bladder year history of painless enlarging scalp nodules, which had wall was seen and resected. Histology confirmed high- rapidly worsened over the previous 6 months (Figure 1(a)). grade B-cell lymphoma in both bladder and kidney biopsies He had lost 7 kg in weight; there were no other systemic (Figure 2(b)). symptoms. He had neither clinical lymphadenopathy nor hepatosplenomegaly and his peripheral blood count, ESR, 2. Discussion protein electrophoresis, liver function tests, and LDH were normal. A skin biopsy from the scalp demonstrated high- To date there are few case reports of nonprimary bladder grade B-cell non-Hodgkin’s lymphoma. Staging CT showed lymphoma, reflecting its rarity as a presenting pathological multiple small cervical lymphadenopathy, lymphomatous entity. To our knowledge we describe the first reported case infiltration in both kidneys, and an area of mucosal thick- of disseminated lymphoma with simultaneous cutaneous, ening of the bladder, which was consistent with a primary renal, and bladder lesions demonstrable at presentation. bladder carcinoma. Staging was completed with an MRI of The incidence of non-Hodgkin’s lymphoma (NHL) is the head (Figure 1(b)) and bone marrow biopsy, which was increasing and maintains a geographical variability: in 2007 normal. there were 10,900 people diagnosed with NHL in the UK The patient described neither lower urinary tract symp- [1]. The incidence of NHL typically increases with age. Rates toms nor episodes of macroscopic haematuria and was a increase sharply in people over 50 and around two-thirds nonsmoker. A urine cytology specimen revealed atypical cells (68%) of all cases are diagnosed in people over 60 years. 2 Case Reports in Oncological Medicine (a) (b) Figure 1: (a) Scalp nodules as seen at presentation. (b) MRI demonstrating an extensive mixed signal, soft tissue mass, over the vertex of the skull. There is infiltration through the inner and outer tables of the skull vault and extension into the dural membranes. H and E CD20 (a) (b) Figure 2: (a) Urine cytology demonstrates a cluster of atypical lymphoid cells. (b) Biopsy of bladder tumour showing infiltration of the transitional epithelium and lamina propria by atypical B lymphocytes (CD20 positive on immunohistochemistry). The most recent classification system is that endorsed by of patients with bladder involvement experience no urinary the World Health Organisation (REAL classification) and symptoms, urinary urgency, frequency, and haematuria have includes subtypes such as diffuse large B-cell, follicular, all been described [3]. It has been reported that a high and small lymphocytic. Determinations of specific subtypes proportion (47%) of patients in this group experience of disease along with patient’s prognostic characteristics ureteric obstruction [3]. gleaned from the International Prognostic Index are crucial Nonprimary bladder lymphomas tend to be a dif- to treatment plans and subsequent management. ferent histological type than those found in the PBL Lymphomas of the bladder are rare and account for 0.2% group and are commonly of the diffuse large cell or of all bladder neoplasms [2]. There is a female predominance follicular centre cell types [3]. In the nonprimary group, and average age of presentation of 58 years. The most the bladder involvement by systemic lymphoma infers a common type is extranodal marginal zone lymphoma of poorer prognosis than that seen in PBL as it implies mucosal-associated lymphoid tissue (MALT) first described multisystem disease. However, there is a clear prognostic in 1990 [3], while Hodgkin’s type is even rarer. Lymphomas distinction between the secondary bladder lymphoma and are classed into the primary group if they present primarily non-localised lymphoma groups with median survival of with bladder symptoms and there is no evidence of systemic 0.6 years and 9 years, respectively [3]. Part of this might lymphoma at presentation. be explained by some patients being classified in the non- More commonly, although relatively few case studies localised group when a dominant bladder mass was only exist, lymphoma of the bladder is seen in association associated with local extension to adjacent organs or to with systemic lymphoma; this can either be as bladder regional lymph nodes [3]. The authors argued that although involvement with active systemic lymphoma (nonlocalised some might classify this category of patient into a “PBL” bladder lymphoma), as reported in this case study, or as group, their tumour histology resembled more closely that lymphoma recurrence in the bladder after a period of of the general population and the patient and disease remission from previous lymphoma (secondary lymphoma) characteristics were distinct from those seen in the PBL [3]. It is rare for disseminated lymphoma to present group [3]. with symptoms of bladder involvement and more typically Non-Hodgkin’s lymphoma involving the bladder can remains a postmortem diagnosis [2]. Although a percentage be successfully treated using chemotherapy, radiotherapy, Case Reports in Oncological Medicine 3 immunotherapy, surgery, and combinations of these modal- ities [4]. The treatment of choice is determined by the histology and extent of disease. The systemic nature of non- primary bladder lymphoma warrants a systemic approach to treatment. Lymphomas with aggressive phenotypes such as diffuse large B-cell are best managed with chemotherapy. The cyclophosphamide, doxorubicin, vincristine, prednisone, and rituximab (CHOP/R) regime is most commonly used [4]. Adjuvant radiotherapy is used for involved fields in localised disease. Follicular lymphoma treatments range from a watch-and-wait policy to chemoimmunotherapy or radioimmunotherapy. Immunotherapy generally forms a standard component of management of advanced disease [4]. The indolent nature of the MALT lymphomas means that local radiotherapy can be used with curative intent, such as in PBL. Surgical resection has a limited role in primary bladder lymphoma, beyond obtaining biopsy specimens, and rarely adds anything to management or prognosis. There are reports, however, of patients having undergone cystectomy due to unclear diagnosis, or to relieve irritative symptoms [5]. There is a report of a low-grade MALT-type PBL being managed with transurethral resection and adjuvant intravesical mitoxantrone [6]. In this case, the patient’s disease constituted stage IV B. He underwent eight cycles of CHOP/R chemotherapy followed by radical radiotherapy to scalp and brain. Follow- up MRI showed dramatic improvement in the scalp and dural mass with minimal residual dural enhancement. A repeat CT showed complete remission with no residual disease in either kidneys or bladder. The patient remained in complete remission for 14 months but then developed a gradual deterioration in his balance and thereafter a marked peripheral neuropathy. Repeat imaging confirmed recurrent widespread cerebral metastasis. He died following a cardiac arrest before palliative radiotherapy could be given. References [1] Cancer Research UK, http://info.cancerresearchuk.org/cancer- stats/types/nhl/?script=true. [2] A. W. Bates, A. J. Norton, and S. I. Baithun, “Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases,” Journal of Clinical Pathology, vol. 53, no. 6, pp. 458– 461, 2000. [3] C.L.Kempton,P.J.Kurtin, D. J. Inwards, P. Wollan,and D. G. Bostwick, “Malignant lymphoma of the bladder: evidence from 36 cases that low-grade lymphoma of the malt-type is the most common primary bladder lymphoma,” American Journal of Surgical Pathology, vol. 21, no. 11, pp. 1324–1333, 1997. [4] S. M. Ansell and J. Armitage, “Non-Hodgkin lymphoma: diagnosis and treatment,” Mayo Clinic Proceedings, vol. 80, no. 8, pp. 1087–1097, 2005. [5] K. Horasanli, M. Kadihasanoglu, O. T. Aksakal, A. Ozagari, and C. Miroglu, “A case of primary lymphoma of the bladder managed with multimodal therapy,” Nature Clinical Practice Urology, vol. 5, no. 3, pp. 167–170, 2008. [6] P. Porter, A. J. Cornaby, M. Al-Hilali, and A. M. Chakrabarti, “Primary lymphoma of the bladder treated successfully with mitozantrone gel,” Postgraduate Medical Journal, vol. 75, no. 888, pp. 609–610, 1999. MEDIATORS of INFLAMMATION The Scientific Gastroenterology Journal of World Journal Research and Practice Diabetes Research Disease Markers Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 International Journal of Journal of Immunology Research Endocrinology Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Submit your manuscripts at http://www.hindawi.com BioMed PPAR Research Research International Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Journal of Obesity Evidence-Based Journal of Journal of Stem Cells Complementary and Ophthalmology International Alternative Medicine Oncology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 Parkinson’s Disease Computational and Behavioural Mathematical Methods AIDS Oxidative Medicine and in Medicine Research and Treatment Cellular Longevity Neurology Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation Hindawi Publishing Corporation http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014 http://www.hindawi.com Volume 2014

Journal

Case Reports in Oncological MedicineHindawi Publishing Corporation

Published: Sep 29, 2012

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