Intravascular Lymphoma Associated with the Female Genital Tract—Diagnostic Considerations, Therapeutic Approaches, and Outcomes

TL;DR

This paper reviews intravascular lymphoma cases affecting the female genital tract, focusing on diagnosis, treatment, and outcomes.

Contribution

The study provides a systematic review of IVL cases in the female genital tract, highlighting diagnostic and therapeutic approaches.

Findings

Abnormal vaginal bleeding and pelvic pain are common symptoms in IVL cases involving the female genital tract.

FDG PET/CT and elevated LDH are key diagnostic tools for identifying hematologic involvement in this region.

B-cell IVL is predominantly treated with CHOPR-CHOP chemotherapy and often hysterectomy.

Abstract

Intravascular lymphoma (IVL) is an uncommon subtype of non-Hodgkin’s extranodal lymphoma, distinguished by the proliferation of neoplastic cells within the lumen of small- to medium-sized arteries, with various organs recorded as impacted. The objective of this study was to evaluate the current literature about IVL and its involvement in the female genital tract, including an overview of diagnostic methods, imaging, and pathological features, selected therapy modalities, and outcomes in patients afflicted by this malignancy. We performed a narrative review with a systematic identification and presentation of published cases of IVL affecting the female genital tract. A literature search was carried out across PubMed, Scopus, and Web of Science for relevant studies presenting data on IVL affecting the female genital tract. Case reports and series that met predefined inclusion and exclusion criteria specified by the modified PECOS (“Population,” “Exposure,” “Comparison,” “Outcomes,” and “Study design”) framework were included. Patients most commonly presented with abnormal vaginal bleeding, pelvic pain, and B symptoms. Fluorodeoxyglucose positron emission tomography computed tomography (FDG PET/CT), often performed alongside abnormal laboratory findings such as elevated lactate dehydrogenase (LDH), played a key role in raising suspicion for hematologic involvement of the female genital tract and guiding biopsy. Most cases represented B-cell intravascular lymphoma and were treated with Rituximab plus (CHOPR-CHOP) based chemotherapy, frequently combined with hysterectomy.