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1134 Marginal zone lymphoma with terminal plasmacytoid differentiation of the uterus treated with hysterectomy for long-term control
  1. Marija Batkoska1,
  2. Maja Krajec2,
  3. Aleš Christian Mihelac2 and
  4. Nina Kovacevic2
  1. 1University of Ljubljana, Medical faculty, Ljubljana, Slovenia
  2. 2Oncology Institute Ljubljana, Ljubljana, Slovenia

Abstract

Introduction/Background Lymphomas originating from the mucosa-associated lymphoid tissue (MALT) of the uterus are extremely rare. Histology is key since treatment and prognosis depend on the lymphoma subtype and stage of the disease.

Methodology A 57-year-old otherwise healthy patient had endometrial abrasion due to postmenopausal bleeding. The histology report showed a monoclonal B-cell lymphoid proliferation in the endocervical mucosa with distinct plasma cellular differentiation. She was immediately appointed to the Institute of Oncology Ljubljana where she was presented at the lymphoma and gynaecological tumour board. Vaginal ultrasound showed a 10 mm hyperechoic endometrium. CT scans of the neck, thorax and abdomen were performed and showed isolated lymphoma infiltrate in the uterus without signs of tumour growth elsewhere. Laboratory findings were unremarkable, there was no molecular spike in the proteinogram. Bone marrow histopathology was suspicious for up to 10% bone infiltration with low-grade small-cell B-cell lymphoma. Three weeks post-diagnosis, due to the gynaecological problems, hysterectomy with bilateral adnexectomy was performed as a first-line treatment, since radiotherapy of the involved site might yield risk for further malignant transformation of tissues. The histological report showed a solitary 2 mm focus of atypical lymphoid proliferation of small B lymphocytes in the anterior cervical wall, positive for PAX-5 and CD20. Based on morphology and immunophenotype, the exact type of lymphoma could not be determined. A molecular genetic examination was not possible. DNA genotyping did not demonstrate a mutation in the MYD88 gene. The findings speak more in favour of MALT, grade IVA.

Results The patient is currently in remission with regular check-ups every 3 months.

Conclusion A multidisciplinary approach is pivotal in addressing such rare disease promptly, ensuring accurate diagnosis and timely treatment. When a long survival period is expected and the late consequences of radiotherapy are taken into consideration, sometimes surgery is the preferred treatment for haematological malignancy.

Disclosures The authors have no conflict of interest. Before writing the abstract, a written consent was obtained from the patient.

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