Treatment Guidelines and Outcome
Extranodal non-Hodgkin lymphomas with primary sinonasal localization are characterized by an aggressive behavior and a dismal prognosis (Li et al. 1998). Radiotherapy and chemotherapy are the mainstay of lymphoproliferative neoplasms treatment, alone or as a part of a combined protocol. Nevertheless, sinonasal non-Hodgkin lymphoma appears less responsive to chemotherapy than lesions with different anatomic localization (Li et al. 1998).
In the early stages or in presence of small lesions (limited IE), radiotherapy is the only treatment modality required. In extensive IE lesions, chemotherapy may be added, even though a single modality treatment (radiotherapy or chemotherapy) is also possible (Li et al. 1998). In case of advanced lesions, combined protocols including concomitant chemotherapy and radiotherapy (IIE) or chemotherapy followed by adjuvant radiotherapy are advised (Li et al. 1998). Radiotherapy dose varies from 40 to 70 Gy (Li et al. 1998; QuraisHi et al. 2000). Palliation may be ensured by chemotherapy alone. Chemotherapeutic regimen, according to different protocols, is based upon agents such as vincristine, cyclophosphamide, bleomycin, adriamycin, etoposide (Yu et al. 1997).
Sinonasal lymphomas are characterized by a poor prognosis, with a 5-year overall survival ranging from 24% (Yu et al. 1997) to 65% (Li et al. 1998). Poor prognostic factors are advanced age (over 60 years), advanced Ann Arbor stage, and presence of B symptoms (Yu et al. 1997). According to the results of a very recent report, complete remission after treatment was the only significant positive prognostic factor in a multivariate analysis (Li et al. 2004).
Patterns of failure for non-Hodgkin lymphomas of the sinonasal tract include local, regional and distant progression or recurrence (Li et al. 1998). Local and/ or regional failures account for about 43%, whereas systemic recurrences, which mainly occur in the skin, lung and liver (Li et al. 1998), are detected in 30% of patients (Li et al. 2004). Early stage lesions have a higher propensity for local relapse (Yu et al. 1997).
Extramedullary plasmacytoma is a highly radiosensitive tumor (MajuMdar et al. 2002), with a 94% local control rate for localized disease if more than 40 Gy in 4 weeks are delivered (MendenHALL et al. 1980). For low-grade solitary extramedullary plasmacytoma, a dose of 35-40 Gy in 3 weeks is recommended (SusnerwALA et al. 1997). Elective treatment on the neck is instead considered unnecessary (SusnerwaLa et al. 1997), since nodal metastases at presentation or during the course of the disease appear not to affect the overall prognosis or the possibility of conversion to multiple myeloma (SusnerwALA et al. 1997). Chemotherapy should be reserved for invasive (Soesan et al. 1992) and/or poorly differentiated lesions (MajuMdar et al. 2002). Since late recurrences are possible, local failure may be effectively re-treated by irradiation. Surgery instead should be reserved for resectable recurrences after radiotherapy (SusnerwALA et al. 1997).
The rarity of extramedullary plasmacytoma makes identification of prognostic factors and evaluation of treatment outcomes quite difficult (SusnerwALA et al. 1997). However, the high grade of the lesion seems to be the most important negative prognostic factor (MajuMdar et al. 2002). The risk of recurrence or conversion to a multiple myeloma, even 15 years after treatment, supports the need for a prolonged, even life-long follow up (SusnerwALA et al. 1997). Periodic examinations should include nasal endos-copy and MR for the primary site and ultrasonogra-phy for the neck. Also blood and urine analysis are recommended in order to detect monoclonal paraprotein and Bence Jones protein, respectively.
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