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Müller, Annika; Dreyling, Martin; Roeder, Falk; Baur-Melnyk, Andrea; Knösel, Thomas; Klein, Alexander; Birkenmaier, Christof; Jansson, Volkmar and Dürr, Hans Roland (2020): Primary bone lymphoma: Clinical presentation and therapeutic considerations. In: Journal of Bone Oncology, Vol. 25, 100326

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Background: Primary lymphoma of bone (PBL) is a rare entity. Due to unspecific clinical signs and equivocal radiographs diagnosis may be delayed. This retrospective report of 109 PBL cases demonstrates typical aspects of the lesion. Treatment and prognostic factors are evaluated. Methods: Retrospectively patient records were reviewed. All patients were followed for evidence of local or distant recurrence. Overall survival (OS) was used as clinical outcome. Results: The median age of the 109 patients was 62.8 years. The most common symptoms were pain (76%), swelling (29%), neurologic symptoms and pathological fracture (16% each). Mean duration of symptoms was 8 months (0-197 months). 19% of patients had indolent NHL subtypes, 72% aggressive NHL subtypes and 7% cases Hodgkin disease. Cyclophosphamid, doxorubicin, vincristine and prednisone (CHOP) or CHOP plus rituximab (RCHOP) were given in 88 (81%) of patients. Radiotherapy was delivered in 67 (61%) of cases. 51 (47%) patients received both. Surgical interventions were restricted to cases with complications as fractures. The 5-year OS was 66%. The 5-year OS was 66%. In the subgroup of 78 patients with aggressive NHL subtype there was a highly significant benefit for chemotherapy or chemotherapy and radiation in comparison to no treatment or radiation alone. Raised LDH, age, IPI and ECOG performance were prognostic factors. In multivariate analysis, age and raised LDH levels only kept significance. Conclusions: In our series of primary bone lymphoma, chemotherapy resulted in a better outcome than Radiotherapy alone. Long-term survival is based on the stage of the disease, favoring younger (< 60 years) patients with solitary bone lesions, low level of LDH and favourable ECOG performance status and IPI scores.

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