71yo fairly healthy M (ECOG 1) who presented with unintentional weight loss, fatigue, and progressive swelling of left neck and shoulder.
PET with extensive bulky conglomerate FDG-avidity throughout right neck, supraclavicular region, right shoulder including scapula with a pathological fracture, upper arm, with additional hypermetabolic adenopathy of left neck. Spleen enlarged to 15.5cm but without hypermetabolism.
Biopsy of R neck mass with CD5-/CD10- small B-cell lymphoma. No large cell component seen. Cyclin D1 and BCL 6 negative. LPL vs MZL. MYD88 negative.
MRI brain with extensive dural thickening and enhancements in frontal regions bilaterally, with similar signal changes and enhancement in supraorbital and frontal scalp soft tissues. No intracranial parenchymal mass.
Would your working diagnosis be MZL with dural involvement in this scenario and how would you approach treatment? Would you recommend RT followed by systemic therapy or systemic therapy alone? I was thinking of ritux/len or R-CHOP. Would you treat this as CNS lymphoma with high-dose MTX based regimens?
I agree, this is suspicious for MZL (assuming no IgM MGUS). How high does the pet suv looks like (data showed suv of 15 associated with dlbcl transformation). I am a bit surprised for such an aggressive presentation of MZL, thus trying to make sure this is not transformed. Plain MZL very unlikely to go to the CNS. I would consider LP confirm no CNS disease, sometimes you see large cells on LP.If this is MZL with no evidence of transformation then I would consider BR. Data showed non inferiority and possibly even superiority in phase 3 trials. It would also be easier on the patient. If this dural thickening is direct extension from skull MZL, BR should work there as this is not considered CNS disease.
SUV of 27-32 and given the aggressive presentation, I do wonder if there are areas of DLBCL. Shall proceed with LP and attempt a re-biopsy to evaluate for transformation and proceed accordingly.
Given how high the suv, I would consider this dlbcl transformation clinically and treat accordingly. I would treat as dlbcl, options include rchop or pola rchp (which I prefer based on the polarix trial). I would use prophylactic IT mtx once with every cycle given the high risk for CNS disease because of the direct extension. This does not penetrate the CNS but I would assume your LP would be negative. If the LP is positive then I would treat with rchop alternating with hdmtx.