• Rare case of lymphoplasmacytic lymphoma/ Waldenström macroglobulinaemia with predominant myometrial and cervical involvement. • Examination of histologic sections showed atypical perivascular lymphoid aggregates and additional testing identified an IgM kappa paraproteinemia with MYD88 p.L265P mutation. • To our knowledge, there have been only two cases previously described in the literature of LPL/WM involvement in the female genital tract. Lymphoplasmacytic lymphoma / Waldenström macroglobulinemia (LPL/WM) is a rare and indolent low-grade B-cell lymphoproliferative neoplasm that often presents with vague symptoms or asymptomatically. While it most commonly involves the bone marrow, LPL/WM can sometimes involve the lymph nodes and spleen, and rarely the central nervous system, skin, and pleural cavities. We report a rare case of lymphoplasmacytic lymphoma/ Waldenström macroglobulinaemia presenting with predominant myometrial and cervical involvement. A 57-year-old G2P2 postmenopausal female with a history of HPV + HSIL presented following a single episode of abnormal uterine bleeding with associated urinary urgency and pelvic pressure. Transvaginal ultrasound examination was unremarkable and endometrial biopsy via hysteroscopy was unsuccessful due to stenotic cervical os. The patient subsequently underwent a total robotic hysterectomy with bilateral salpingo-oophorectomy. Examination of histologic sections showed atypical perivascular lymphoid aggregates consistent with involvement by a low grade B cell lymphoma with predominant myometrial and cervical involvement. Differential diagnosis at the time included marginal zone lymphoma (MZL) and lymphoplasmacytic lymphoma (LPL). Additional testing identified an IgM kappa paraproteinemia with MYD88 p.L265P mutation. Bone marrow biopsy and aspirate confirmed the diagnosis of lymphoplasmacytic lymphoma / Waldenström macroglobulinaemia (LPL/WM). To our knowledge, there have been only two cases previously described in the literature of LPL/WM involvement in the female genital tract; both of which had prominent involvement of the ovaries. Although exceedingly rare, LPL/WM involvement of the female genital tract should be considered on the differential diagnosis if atypical lymphoid cells or dense lymphoid aggregates are observed.
Dent et al. (Sun,) studied this question.