Primary extranodal vaginal non-hodgkin lymphoma: Diagnostic pitfalls and therapeutic challenges.

Introduction: Primary extranodal non-hodgkin vaginal lymphoma (PeNHVL) represents a rare entity, with few data published until now. We present here a series of patients with PeNHVL, analyzing our data as part of a detailed review of the available literature.

Methods: The study included a consecutive series of 6 patients with final diagnosis of PeNHVL admitted at our Institution between January 2000 and December 2017. The systematic review was conducted according to PRISMA guidelines. A literature search of the PubMed, MEDLINE and EMBASE electronic databases was performed using the following terms: 'vaginal lymphoma'. Relevant data were collected and analyzed for the purposes of this study, reporting results through a narrative approach.

Results: In our series discomfort and vaginal pain, refractory to medical treatments represent the symptoms of disease presentation, and the presence of localized/diffused anelastic area in the vaginal wall with tactile sensation of cork emerges as diagnostic sign (Cork Wall sign). The literature revision included 41 studies, with an overall population of 74 patients. The vast majority of women were diagnosed as early stage disease (93.6%) and received chemotherapy (74.6%) with a very high response rate (96%). Death from disease occurred in 5 women (6.7%).

Conclusions: Localized or diffused hard-ligneous vaginal areas with Cork Wall sign represent the typical sign of disease presentation. PeNHVL is characterized by a very high sensitivity to chemotherapy and very favourable prognosis; therefore, radical surgery is not indicated. Histotype characterization is crucial to identify those uncommon variants associated with a less favorable clinical outcome.