Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia Initially Presenting as Postmenopausal Pelvic Pain and Bleeding: A Rare Case Report and Literature Review

Purpose: To report a rare case of lymphoplasmacytic lymphoma/ Waldenström macroglobulinemia with predominant myometrial and cervical involvement. Methods: Case report. Results: A 57-year-old G2P2 postmenopausal female with a history of tubal ligation and HPV+ HSIL on pap smear presented with an eight-month history of pelvic pain with associated urinary urgency and pelvic pressure as well as fatigue and a single episode of abnormal uterine bleeding and unremarkable transvaginal ultrasound. Endometrial sampling via hysteroscopy was attempted but unable to be completed due to stenotic cervical os. The patient subsequently underwent definitive surgical management with a total robotic hysterectomy with bilateral salpingo-oophorectomy. Pathology showed atypical perivascular lymphoid aggregates suspicious for involvement by low grade B cell lymphoma involving the cervical stroma, myometrium, and bilateral fallopian tubes and ovaries, with predominant myometrial and cervical involvement. Differential diagnosis at the time included marginal zone lymphoma (MZL) and lymphoplasmacytic lymphoma (LPL). Additional testing identified a MYD88 p.L265P mutation and an IgM kappa paraproteinemia. A PET scan revealed hypermetabolic bilateral axillary and upper abdominal lymph nodes. The patient underwent a bone marrow biopsy and aspirate that solidified the diagnosis of IgM-type lymphoplasmacytic lymphoma / Waldenström macroglobulinemia (LPL/WM). Conclusion: Lymphoplasmacytic lymphoma / Waldenström macroglobulinemia (LPL/WM) is an extremely 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. 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.