Perianal pseudolymphoma: A case report and review of the literature.

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.

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].

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, 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 haematology-oncology 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 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.

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.

Learning points

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

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.

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

Tahleesa Cuda.