Age/sex | Clinical picture at presentation | Main lesion location | Systemic symptoms and/or abnormal blood tests at presentation | Nodal and extranodal involvement | Therapy and clinical evolution | Outcome | Ref. |
---|---|---|---|---|---|---|---|
10/M | Painless masses around parotid and submandibular glands. | Parotid and submandibular glands bilaterally. | None | Apparently both nodal and extranodal | None | Symptom-free | [21] |
9/M | Masses around submandibular glands | Submandibular glands bilaterally. | None | Apparently both nodal and extranodal | None | Symptom-free | [21] |
11/M | 1Â year history of painless bilateral neck swelling. | Submandibular and parotid glands bilaterally. | None | Both nodal and extranodal | Surgery | Not reported | [18] |
17/F | Bilateral parotid enlargement and cervical lumps localized in the submandibular region. | Mass at left common carotid artery, descending aorta down to the renal artery; MRI finding of bilateral lesions in knee and ankle. | High CRP and ESR, hypergammaglobulinemia. | Both nodal and extranodal | None | Not reported | [19] |
12/F | 1-month history of enlarging and painless submandibular lymphadenopathy. | Parotid and submandibular glands. | Recurrent fever 2Â months before presentation; high ESR. | Nodal | None | No recurrence after 28Â month of follow-up. | [28] |