Publication: Primary extranodal vaginal non-hodgkin lymphoma: Diagnostic pitfalls and therapeutic challenges
Authors
Petrillo, Marco ; Fara, Antonella Maria ; Fedeli, Maria Antonietta ; Fozza, Claudio ; Cossu, Antonio ; Gulotta, Alessandra ; Dessole, Francesco ; Piana, Andrea ; Capobianco, Giampiero ; Tanda, Francesco ; Dessole, Salvatore
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Publisher
Universidad de Murcia. Departamento de Biología Celular e Histología
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DOI
DOI: 10.14670/HH-18-085
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info:eu-repo/semantics/article
Description
Abstract
Introduction. Primary extranodal nonhodgkin 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.
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