We discuss an unusual presentation, a vulval-urethral mass, of a rare condition, primary bladder lymphoma. We discuss the history, the investigations (with images) and the treatment. We then review this rare disease.
A 78 year old woman (with mild dementia) was investigated for non visible haematuria. On flexible cystoscopy a large soft irregular mass was seen extruding from the urethra. It was continuous with a mass involving the neck and trigone with a diffusely reddened bladder. Urine cytology revealed hyperchromatic nuclei but no obvious malignant cells. She underwent TURBT of the internal component and a biopsy of the external component. Both specimens showed diffuse infiltration by large malignant lymphoid cells with frequent mitotic figures and apoptotic bodies. High Ki67 95%. These features were consistent with diffuse large B cell lymphoma.
On CT urogram, there was a lobulated lesion at the level of the trigone measuring approximately 3 x 2 cm in size. It appears to further extend superiorly along the posterior wall to the level of the left VUJ. A right inguinal node measured 27mm.
The patient was referred after TURBT, to the haematology team who commenced prednisolone 40mg and 4 cycles of multi-agent low dose chemotherapy R-Pmit CEBO in 6 week cycles. There was shrinkage of the tumour. She then received 30 grays of external beam radiotherapy over 15 sessions. She achieved an excellent response.
The lymphoma arises in the sub-mucosal follicles. Peak age is 40-60 with women being affected more commonly. It more usually forms part of a systemic picture
There is a spectrum of variants, with T cell, Burkitts, diffuse large B and Hodgkins.
The most frequent variant is the low grade mucosa associated lymphoid tissue variant.
Diagnois is by microscopy and appropriate immunohistochemistry staining. These are done to exclude lympho-epithelial carcinoma and small cell neuro-endocrine carcinoma which can mimic this.
Pictures will be provided