PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV

Poster Tumore der Haut
P 47
Extranodal nasal-type natural killer/T-cell lymphoma mimicking refractory
sinusitis in a 60-year-old man
Werner Nindl 1
Hansgeorg Müller 1
Bernhard Zelger 1
Wolfgang Willenbacher 2
Lorenzo Cerroni 3
Matthias Schmuth 1
1 Universitätsklinik für Dermatologie Innsbruck, Anichstraße 35, 6020 Innsbruck
2 Department of Hematology and Oncology, Innsbruck Medical University, Innsbruck
3 Research Unit of Dermatopathology, Department of Dermatology, Medical University
of Graz, Graz
Introduction: Extranodal NK/T-cell lymphoma, nasal type, is an aggressive
non-Hodgkin's lymphoma subtype originating from peripheral T-cells or natural killer
(NK) cells. Due to its rarity in European countries, the diagnosis is often missed at initial
presentation, with its nonspecific symptoms misattributed to more common chronic
diseases.
Methods and Results: We report a 60-year-old white man who presented with
uncontrollable acute exacerbation of chronic sinusitis despite antibiotic therapy for 3
months. Physical examination showed left-sided nasal deviation and obstruction, and
a diffuse swelling of the paranasal soft tissue. Imaging (CT, MRI) revealed no evidence
of bone damage. The patient underwent endoscopic sinus surgery along with
septoplasty that was followed by a revision surgery within one month due to
persistence of symptoms. Histological examination of the resected tissue showed nonspecific
features of chronic inflammation. MRI and a repeated intranasal biopsy showed
no evidence of malignancy. No long-term clinical improvement but the occurrence of
nasal skin necrosis was observed under immunosuppressive therapy (Anakinra, Tocilizumab,
methylprednisolone). Histological assessment of a cutaneous excisional biopsy
demonstrated a lymphoid infiltrate (CD3Îμ+, CD56+, TIA-1+, Granzyme-B+, EBER-ISH+)
comprising small- to medium-sized cells with irregular nuclei. 18F-FDG PET scan and
bone marrow examination showed no further involvement (stage IE). The International
Prognostic Index (IPI) was low risk (1 point; LDH 381 U/L). Initial therapy for this locally
advanced nasal-type NK/T-cell lymphoma consisted of alemtuzumab plus CHOP
chemotherapy and palliative plastic surgery of the destructed nasal tissue. Since no
clinical response was observed after 5 cycles of chemotherapy, a first salvage
chemotherapy with DHAP and radiotherapy were done. After a local recurrence
involving the left orbita, two cycles of SMILE chemotherapy induced a complete
remission on PET/CT. The patient subsequently underwent autologous stem cell
transplantation.
Conclusion: Recalcitrant chronic sinusitis may be the initial presentation of nasal-type
NK/T-cell lymphoma. A high index of suspicion, performance of repeat biopsies, and
careful histological examination are crucial to prevent a delay in diagnosis.
104