Clinical findings in 25 patients with sinonasal or nasopharyngeal extramedullary plasmacytoma in a four-decade single-centre series

Objectives: Extramedullary plasmacytoma in the sinonasal tract or nasopharynx is rare. The aim of the study was to review data on symptoms, clinical findings, treatment and follow-up of plasmacytomas in the sinonasal and nasopharyngeal regions in order to delineate the main clinical characteristics and the optimal management. Method: Twenty-five patients with sinonasal or nasopharyngeal plasmacytoma, diagnosed and treated at the Helsinki University Hospital during a 39-year period from 1975 to 2013 were retrospectively reviewed. Results: There were 18 males and 7 females with a median age of 66 years (range, 36-80). Sixty-eight percent received only radiotherapy or (chemo)radiotherapy. Forty-seven percent of them had a complete response to primary radiotherapy and one patient had a complete response after receiving additional brachytherapy. Four patients were treated primarily with surgery only. Two of them had a local recurrence, but were then successfully treated with radiotherapy. Altogether, four patients received a combination of surgery and (chemo)radiotherapy. Forty-four percent were alive with no evidence of disease after a median follow-up time of 78 months. Forty percent died of their disease and 16% died of other causes. Conclusions: Our study supports radiotherapy as a treatment of choice, but for small tumours surgery alone or in combination with radiotherapy may also be considered. Chinese abstractPeer reviewe

Nenän, nenän sivuonteloiden ja nenänielun lymfoomat : esiintyvyys ja kliininen kuva Helsingin ja Uudenmaan sairaanhoitopiirin (HUS) alueella vuosina 1975-2013

Nenän, nenän sivuonteloiden ja nenänielun lymfoomat ovat länsimaissa harvinaisia. Suomessa tämän tautiryhmän ilmaantuvuudesta tai potilaiden kliinisestä kuvasta ei ole aiemmin tehty kattavaa tutkimusta. Tutkimuksemme oli retrospektiivinen kvalitatiivinen tutkimus, joka perustui patologisanatomisiin diagnooseihin (PAD) ja sairauskertomustietoihin. Helsingin yliopiston patologian laitoksen näyterekisterin avulla löysimme 134 potilasta, joilla oli diagnoosina lymfooma tai plasmosytooma sinonasaalisella tai nenänielun alueella vuosina 1975 - 2013. Suuri osa vanhemmista sairaskertomuksista ei ollut käytettävissä ja siksi aineisto käsitti 89 potilasta vuosilta 1986 - 2013. Potilaiden tavallisimmat oireet olivat nenän tukkoisuus, nuha ja nenäverenvuodot. Kasvojen kipu ja välikorvan tulehdukset olivat myös yleisiä. Neljänneksellä oli suurentuneita imusolmukkeita. Yleisimmin tauti ilmeni nenänielun alueella ja tavallisin lymfooma-alatyyppi oli suurisoluinen B-solulymfooma. Keskimääräiseksi ilmaantuvuudeksi saimme Helsingin ja Uudenmaan sairaanhoitopiirin alueella 3,4 lymfoomatapausta vuodessa. Alue kattaa nykyisin 1,6 miljoonan väestöpohjan. Ilmaantuvuus oli nousujohteista

Clinical findings of extranodal SNT lymphoid malignancies in a four-decade single-centre series

Sinonasally located lymphoid malignancies are rare lesions with first symptoms similar to other obstructive conditions. Additionally, they often coexist with nasal inflammation and mucosal necrosis. Therefore, time from the first symptoms to diagnosis tends to be long. Awareness and early diagnosis of this disease entity could improve treatment outcome. Altogether, 142 patients with sinonasal or nasopharyngeal (i.e. sinonasal tract, SNT) lymphoid malignancies, diagnosed and treated at the Helsinki University Hospital, during a 39-year period from 1975 to 2013, were retrospectively reviewed. There were 90 males (63 %) and 52 females (37 %) with a median age of 64 years (range 26-92). Eighty-four percent of the patients had primary diseases and 16 % had relapses of lymphoid malignancies primarily diagnosed at other locations. The mean duration of symptoms prior to diagnosis was 4.8 months (range 0.5-24). The most common histological entity was diffuse large B-cell lymphoma (43 %), followed by plasmacytoma (18 %). The most common location was nasopharynx (58 %) followed by nasal cavity (44 %) and paranasal sinuses (35 %). Sixty-nine percent of the lesions were at a single anatomic location of the sinonasal tract. Fifty-two percent of the cases were of Ann Arbor Stage I. Lymphoid malignancies form an important and diverse group in the differential diagnosis of SNT tumours. They most often present with general obstructive nasal symptoms due to tumour location. Most of them are primary lesions, highlighting the importance of an accurate diagnosis as early as possible.Peer reviewe