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

**Authors:** Aleksandar Ristic, Marija Rovcanin, Ana Tomic, Aleksandar Rakic, Nebojsa Zecevic, Svetlana Jankovic

PMC · DOI: 10.3390/diseases14030109 · 2026-03-17

## 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.

## Key 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.

## Linked entities

- **Chemicals:** Fluorodeoxyglucose (PubChem CID 53716604)

## Full-text entities

- **Genes:** MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, CD34 (CD34 molecule) [NCBI Gene 947], IVL (involucrin) [NCBI Gene 3713], KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, IL2RA (interleukin 2 receptor subunit alpha) [NCBI Gene 3559] {aka CD25, IDDM10, IL2R, IMD41, TCGFR, p55}, PWWP3A (PWWP domain containing 3A, DNA repair factor) [NCBI Gene 84939] {aka EXPAND1, HSPC211, MUM-1, MUM1}, MYC (MYC proto-oncogene, bHLH transcription factor) [NCBI Gene 4609] {aka MRTL, MYCC, bHLHe39, c-Myc}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}, PAX5 (paired box 5) [NCBI Gene 5079] {aka ALL3, BSAP, PAX-5}, CD79A (CD79a molecule) [NCBI Gene 973] {aka IGA, IGAlpha, MB-1, MB1}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}
- **Diseases:** HLH (MESH:D051359), immunological dysregulation (MESH:D007154), fever (MESH:D005334), uterine artery weakness (MESH:D018908), NHL (MESH:D008228), endometrial polyp (MESH:D014591), vascular blockage (MESH:D015508), gynecologic malignancies (MESH:D005833), uterine leiomyomas (OMIM:150699), Anemia (MESH:D000740), necrotic (MESH:D009336), uterine and/or ovarian mass (MESH:D010049), lymphadenopathy (MESH:D008206), Intravascular Lymphoma (MESH:D008223), Hyperplastic bone marrow (MESH:D001855), ischemia (MESH:D007511), neurological impairments (MESH:D009422), ovarian serous carcinoma (MESH:D010051), NK (natural killer)/T-cell lymphoma (MESH:D000077428), skin lesions (MESH:D012871), leiomyoma (MESH:D007889), hepatosplenomegaly (MESH:C535727), thrombocytopenia (MESH:D013921), diffuse large B-cell lymphoma (MESH:D016403), chronic pelvic pain (MESH:D011472), EBV infection (MESH:D020031), blood count (MESH:D006402), lymphoproliferative disorders (MESH:D008232), hematological malignancies (MESH:D019337), B-Cell IVL (MESH:D016393), Hodgkin lymphoma (MESH:D006689), lymphomatous obstruction (MESH:D013967), uterine mass (MESH:C536030), pelvic pain (MESH:D017699), leiomyomatous lesion (MESH:D009059), T-cell lymphoma (MESH:D016399), injury to (MESH:D014947), leukocytosis (MESH:D007964), (endometrial) carcinoma (MESH:D016889), B (MESH:D006509), uterine sarcomas (MESH:D012509), bleeding (MESH:D006470), B-cell IVLs (MESH:D015448), uterine cancer (MESH:D014594), purpura (MESH:D011693), irregular uterine hemorrhage (MESH:D014592), neutropenic sepsis (MESH:D018805), Death (MESH:D003643), hematopoietic cancer (MESH:D009369), weight loss (MESH:D015431)
- **Chemicals:** cyclophosphamide (MESH:D003520), R (MESH:D001120), etoposide (MESH:D005047), Rituximab (MESH:D000069283), CVAD (MESH:C064396), CHOPR (-), EPOCH (MESH:C079446), CVP (MESH:C034588), -FDG (MESH:D019788), methotrexate (MESH:D008727), mitoxantrone (MESH:D008942)
- **Species:** Homo sapiens (human, species) [taxon 9606], human gammaherpesvirus 4 (Epstein Barr virus, no rank) [taxon 10376]

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Source: https://tomesphere.com/paper/PMC13025139