Primary Extranodal Non-Hodgkin's Lymphoma in Adults: Clinicopathological and Survival Characteristics

Abstract

Among 318 cases of non-Hodgkin's lymphoma (NHL) treated in our unit, 145 (45.6%) had primary extranodal NHL (PE-NHL). The stomach was the most common site (42.1%), followed by the PE-NHL of the head and neck region. Histologically aggressive histologies (65.5% intermediate and 20.7% high grade) predominated. 89.6% of the cases were localized (stage IE, 51% and stage II, 38.6%) but 28% had B symptoms. CR was achieved in 82.1% of the cases. 5-years disease free survival and overall survival were both 65%. Factors that influence prognosis were stage and high grade histology. Among various primary sites the Waldeyer's ring, small intestine and testes had the worse prognosis. Compared to nodal NHL, the PE-NHL were more frequently localized, belonged more often to aggressive histologies and had more often distal extranodal relapses. CR rates and disease free and overall survival were significantly better for PE-NHL. The survival rates, however, listed according to stage and histology for nodal and PE-NHL were not different. We conclude that although PE-NHL differed from nodal NHL in several respects, prognosis is mainly a factor of stage and histology rather than of the primary localization per se.

Extensive Extranodal Involvement of Rare Sites in Non Hodgkin’s Lymphoma Detected on 18F- FDG PET-CT: A Case Report

“…The involvement of extranodal sites is a common feature in the course of NHL. Moreover, some NHLs are considered to originate at sites other than the lymph nodes or the spleen, and are referred to as primary extranodal NHL (PE-NHL) [12,13]. When NHL has extranodal presence, the involved sites can be the gastrointestinal tract, bone, brain, testis, ovary, lung, nasopharynx, soft tissue, thyroid, kidney, liver, breast, skin, etc.…”

Multifocal Extranodal Non-Hodgkin Lymphoma: A Clinicopathologic Study of 37 Cases in Greece, a Hellenic Cooperative Oncology Group Study

“…Other common sites are skin and bone. Less common primary localizations of PE-NHL have also been reported [1,2,14].…”

“…Moreover, some NHLs are considered to originate at sites other than the lymph nodes or spleen and are referred to as primary extranodal lymphoma (PE-NHL) [1,2]. The origin of most PE-NHLs can be ascribed to one given organ system or site.…”

Lymphome non hodgkinien à localisation mandibulaire : à propos de deux observations

“…Ces deux observations illustrent le polymorphisme de l'expression clinique des LNH dans la cavité buccale : dans le premier cas, il simule un processus infectieux odontogène, dans le deuxième cas une tumeur odontogène bénigne. Les atteintes révélatrices des LNH ont dans 30 % des cas un siège extra-ganglionnaire [1] et constituent les LNH extra-ganglionnaires (LNHE) qui s'observent en premier lieu dans la région cervico-faciale (30 à 40 % des cas) [5 ], puis dans l'estomac [1] . Les LNH associés à l'anneau de Waldeyer sont bien connus et souvent classés dans le groupe de LNH associés aux tissus lymphoïdes muqueux ou lymphomes MALT (Mucosa Associated Lymphoïd Tissue) [13] .…”