CASE REPORT – OPEN ACCESS International Journal of Surgery Case Reports 39 (2017) 271–272
Contents lists available at ScienceDirect
International Journal of Surgery Case Reports journal homepage: www.casereports.com
Perianal pseudolymphoma: A case report and review of the literature Tahleesa Cuda a,b,∗ , Pieter Prinsloo b a b
Royal Brisbane and Women’s Hospital, Brisbane, Australia Department of Surgery, Cairns Private Hospital, Cairns, Queensland, Australia
a r t i c l e
i n f o
Article history: Received 7 May 2017 Received in revised form 11 August 2017 Accepted 11 August 2017 Available online 17 August 2017 Keywords: Perianal pseudolymphoma Benign intralymphatic lymphoma Case report
a b s t r a c t INTRODUCTION: Perianal pseudolymphoma is a rare lymphoproliferative disorder that can mimic cutaneous lymphoma. The condition can present acutely with concerns of perianal sepsis. The purpose of this case review is to review the current literature on perianal pseudolymphoma and emphasise to the surgical community its presentation, suggested aetiology and management. CASE PRESENTATION: In this case report, we detail the case of a 28-year-old female who presented with perianal pseudolymphoma. Malignant lymphoproliferative diseases required exclusion. Conservative management of this rare condition was successful. DISCUSSION: We highlight the differential diagnoses, investigations and treatment options for a rare condition. A review of the literature on the limited published data on perianal pseudolymphoma is briefly discussed. CONCLUSION: The aim of this case report is to highlight the existence of such a rare condition that can imitate an acute surgical presentation and its successful conservative management. © 2017 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Perianal pseudolymphoma is a rare, polyclonal lymphoproliferative disorder of B- or T-cell origin. It is a benign condition, which clinically and histologically mimics cutaneous lymphoma. It is difficult to discern one condition from the other, creating angst for both patient and clinician [1]. Currently, only limited and mostly low-level evidence exists on the enigmatic condition. The condition occurs at sites of cutaneous trauma or within haemangiomas [2,3]. Symptoms described include rectal bleeding, rectal or perianal polyps, prolapse, constipation and anal pain and tenderness [3]. Disease resolution follows cessation of causative stimulus and with non-invasive treatment [2]. Herein, we present such a case of a patient that was referred to our acute surgical unit. The purpose of this paper is to highlight perianal pseudolymphoma, although rare, as a potential cause for acute perianal pain. The following case report was written in keeping with the SCARE criteria [4]. 2. Case report A 28-year-old female presented with first episode, worsening severe perianal pain. Her symptoms developed acutely in the days prior to her presentation. She denied a history of perianal trauma,
∗ Corresponding author at: Australia/2703/141 Campbell Street, Bowen Hills, Queensland, 4006, Australia. E-mail address: [email protected] (T. Cuda).
anal intercourse and high-risk activities. Relevant past medical history included a one year history of altered bowel habit with diarrhea, low dose opioid use for chronic lower back pain and current smoking status. She denied a family history of inflammatory bowel disease or other gastrointestinal disorders. The degree of perianal tenderness limited bedside examination. Examination under anaesthesia found multiple acute ulcerations of the anal verge, suggestive of Herpes Simplex. There were no abscesses, fistulae or haemorrhoids. Intra-operative biopsies demonstrated ulceration with active chronic inflammatory cells and atypical lymphoid proliferation within lymphatics. Features favoured benign intralymphatic proliferation of T-cell lymphoblasts, known commonly as pseudolymphoma. Immunohistochemistry confirmed cells limited to lymphatics. The histology result raised concerns about intravascular anaplastic large cell lymphoma, prompting clinical haematologyoncology review. PET imaging and gastroscopy were unremarkable. Colonoscopy demonstrated ongoing superficial ulceration of the anus. Investigations for Inflammatory Bowel Disease. Helicobacter pylori, Varicella zoster virus, herpes simplex virus, treponemal antibody, chlamydia trachomatis and Neisseria gonorrhoeae were negative. Erythrocyte sedimentation rate, full blood count, liver function tests and renal function were within normal ranges. Perianal swabs cultured streptococcus pyogenes. The patient was treated with clindamycin and acyclovir followed by cephalexin. Her symptoms subsequently resolved. Our
http://dx.doi.org/10.1016/j.ijscr.2017.08.014 2210-2612/© 2017 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
CASE REPORT – OPEN ACCESS 272
T. Cuda, P. Prinsloo / International Journal of Surgery Case Reports 39 (2017) 271–272
haematology-oncology colleague felt symptoms were almost certainly a result of benign lymphoma, but would monitor the patient in following months to ensure complete resolution. 3. Discussion Although a worrisome time for both patient and clinician with concerns of a malignant lymphoma looming, a much less ominous cause was found. Perianal pseudolymphoma is an uncommon condition, which poses a diagnostic dilemma for treating clinicians. With its ability to mimic an acute surgical presentation, this is a condition and scenario that general surgeons may encounter during their career.
Consent Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Authors contribution Study concepts: T. Cuda; P. Prinsloo. Study design: T. Cuda. Data acquisition: T. Cuda; P. Prinsloo. Quality control of data and algorithms: T. Cuda; P. Prinsloo. Guarantor
Learning points Tahleesa Cuda. • • • • •
Perianal pseudolymphoma is extremely rare; Rare cause for acute, severe perianal pain; Cause is unknown; Associated with perianal trauma and haemangiomas; Resolve by treating the cause.
Conflicts of interest All authors disclose that there are no conflicts of interest related to this work. Funding
References [1] E. Levy, J. Godet, B. Cribier, D. Lipsker, Pseudolymphoma of the skin: ambiguous terminology: a survey among dermatologists and pathologists, Annales de dermatologie et de venereologie 140 (2013) 105–111. [2] T. Terada, Cutaneous pseudolymphoma: a case report with an immunohistochemical study, Int. J. Clin. Exp. Pathol. 6 (2013) 966–972. [3] J.S. Cornes, M.H. Wallace, B.C. Morson, A summary of the clinical features of 100 cases of benign lymphoma of the rectum and anal canal, Proc. R. Soc. Med. 54 (1961) 729. [4] R.A. Agha, A.J. Fowler, A. Saeta, I. Barai, S. Rajmohan, D.P. Orgill, The SCARE Statement, Consensus-based surgical case report guidelines, Int. J. Surg. (London, England) 34 (2016) 180–186.
No funding resources to declare. Ethical approval Ethics approval from Greenslopes Private Hospital – Greenslopes Research and Ethics Committee, approved on 10th April 2017, protocol 17/21.
Open Access This article is published Open Access at sciencedirect.com. It is distributed under the IJSCR Supplemental terms and conditions, which permits unrestricted non commercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Contents lists available at ScienceDirect
International Journal of Surgery Case Reports journal homepage: www.casereports.com
Perianal pseudolymphoma: A case report and review of the literature Tahleesa Cuda a,b,∗ , Pieter Prinsloo b a b
Royal Brisbane and Women’s Hospital, Brisbane, Australia Department of Surgery, Cairns Private Hospital, Cairns, Queensland, Australia
a r t i c l e
i n f o
Article history: Received 7 May 2017 Received in revised form 11 August 2017 Accepted 11 August 2017 Available online 17 August 2017 Keywords: Perianal pseudolymphoma Benign intralymphatic lymphoma Case report
a b s t r a c t INTRODUCTION: Perianal pseudolymphoma is a rare lymphoproliferative disorder that can mimic cutaneous lymphoma. The condition can present acutely with concerns of perianal sepsis. The purpose of this case review is to review the current literature on perianal pseudolymphoma and emphasise to the surgical community its presentation, suggested aetiology and management. CASE PRESENTATION: In this case report, we detail the case of a 28-year-old female who presented with perianal pseudolymphoma. Malignant lymphoproliferative diseases required exclusion. Conservative management of this rare condition was successful. DISCUSSION: We highlight the differential diagnoses, investigations and treatment options for a rare condition. A review of the literature on the limited published data on perianal pseudolymphoma is briefly discussed. CONCLUSION: The aim of this case report is to highlight the existence of such a rare condition that can imitate an acute surgical presentation and its successful conservative management. © 2017 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Perianal pseudolymphoma is a rare, polyclonal lymphoproliferative disorder of B- or T-cell origin. It is a benign condition, which clinically and histologically mimics cutaneous lymphoma. It is difficult to discern one condition from the other, creating angst for both patient and clinician [1]. Currently, only limited and mostly low-level evidence exists on the enigmatic condition. The condition occurs at sites of cutaneous trauma or within haemangiomas [2,3]. Symptoms described include rectal bleeding, rectal or perianal polyps, prolapse, constipation and anal pain and tenderness [3]. Disease resolution follows cessation of causative stimulus and with non-invasive treatment [2]. Herein, we present such a case of a patient that was referred to our acute surgical unit. The purpose of this paper is to highlight perianal pseudolymphoma, although rare, as a potential cause for acute perianal pain. The following case report was written in keeping with the SCARE criteria [4]. 2. Case report A 28-year-old female presented with first episode, worsening severe perianal pain. Her symptoms developed acutely in the days prior to her presentation. She denied a history of perianal trauma,
∗ Corresponding author at: Australia/2703/141 Campbell Street, Bowen Hills, Queensland, 4006, Australia. E-mail address: [email protected] (T. Cuda).
anal intercourse and high-risk activities. Relevant past medical history included a one year history of altered bowel habit with diarrhea, low dose opioid use for chronic lower back pain and current smoking status. She denied a family history of inflammatory bowel disease or other gastrointestinal disorders. The degree of perianal tenderness limited bedside examination. Examination under anaesthesia found multiple acute ulcerations of the anal verge, suggestive of Herpes Simplex. There were no abscesses, fistulae or haemorrhoids. Intra-operative biopsies demonstrated ulceration with active chronic inflammatory cells and atypical lymphoid proliferation within lymphatics. Features favoured benign intralymphatic proliferation of T-cell lymphoblasts, known commonly as pseudolymphoma. Immunohistochemistry confirmed cells limited to lymphatics. The histology result raised concerns about intravascular anaplastic large cell lymphoma, prompting clinical haematologyoncology review. PET imaging and gastroscopy were unremarkable. Colonoscopy demonstrated ongoing superficial ulceration of the anus. Investigations for Inflammatory Bowel Disease. Helicobacter pylori, Varicella zoster virus, herpes simplex virus, treponemal antibody, chlamydia trachomatis and Neisseria gonorrhoeae were negative. Erythrocyte sedimentation rate, full blood count, liver function tests and renal function were within normal ranges. Perianal swabs cultured streptococcus pyogenes. The patient was treated with clindamycin and acyclovir followed by cephalexin. Her symptoms subsequently resolved. Our
http://dx.doi.org/10.1016/j.ijscr.2017.08.014 2210-2612/© 2017 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
CASE REPORT – OPEN ACCESS 272
T. Cuda, P. Prinsloo / International Journal of Surgery Case Reports 39 (2017) 271–272
haematology-oncology colleague felt symptoms were almost certainly a result of benign lymphoma, but would monitor the patient in following months to ensure complete resolution. 3. Discussion Although a worrisome time for both patient and clinician with concerns of a malignant lymphoma looming, a much less ominous cause was found. Perianal pseudolymphoma is an uncommon condition, which poses a diagnostic dilemma for treating clinicians. With its ability to mimic an acute surgical presentation, this is a condition and scenario that general surgeons may encounter during their career.
Consent Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. Authors contribution Study concepts: T. Cuda; P. Prinsloo. Study design: T. Cuda. Data acquisition: T. Cuda; P. Prinsloo. Quality control of data and algorithms: T. Cuda; P. Prinsloo. Guarantor
Learning points Tahleesa Cuda. • • • • •
Perianal pseudolymphoma is extremely rare; Rare cause for acute, severe perianal pain; Cause is unknown; Associated with perianal trauma and haemangiomas; Resolve by treating the cause.
Conflicts of interest All authors disclose that there are no conflicts of interest related to this work. Funding
References [1] E. Levy, J. Godet, B. Cribier, D. Lipsker, Pseudolymphoma of the skin: ambiguous terminology: a survey among dermatologists and pathologists, Annales de dermatologie et de venereologie 140 (2013) 105–111. [2] T. Terada, Cutaneous pseudolymphoma: a case report with an immunohistochemical study, Int. J. Clin. Exp. Pathol. 6 (2013) 966–972. [3] J.S. Cornes, M.H. Wallace, B.C. Morson, A summary of the clinical features of 100 cases of benign lymphoma of the rectum and anal canal, Proc. R. Soc. Med. 54 (1961) 729. [4] R.A. Agha, A.J. Fowler, A. Saeta, I. Barai, S. Rajmohan, D.P. Orgill, The SCARE Statement, Consensus-based surgical case report guidelines, Int. J. Surg. (London, England) 34 (2016) 180–186.
No funding resources to declare. Ethical approval Ethics approval from Greenslopes Private Hospital – Greenslopes Research and Ethics Committee, approved on 10th April 2017, protocol 17/21.
Open Access This article is published Open Access at sciencedirect.com. It is distributed under the IJSCR Supplemental terms and conditions, which permits unrestricted non commercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
