Case Report: A case study and literature review of diffuse large B-cell lymphoma presenting as a primary uterine mass with appendiceal involvement and an 11q gain
Year: 2026
Type: article
Abstract: Context Primary lymphoma of the uterine corpus is an exceptionally uncommon form of extranodal lymphoma that is often mistaken for uterine sarcoma or carcinoma. We present a case of primary diffuse large B-cell lymphoma (DLBCL) of the uterus, with appendiceal involvement and a gain at 11q23.3/11q24.3 without MYC rearrangement. The characteristic distal 11q loss required for the diagnosis of high-grade B-cell lymphoma with 11q aberration was not demonstrated; the tumor was therefore classified as DLBCL, NOS with an 11q gain. In addition, we review 34 documented cases from 1965 to 2025 that include comprehensive clinicopathological information. Findings A 56-year-old female patient presented with a rapidly growing abdominal mass and compressive symptoms. Imaging revealed marked uterine enlargement (>15 cm) and a separate appendiceal mass. Histological analysis identified a germinal center B-cell-like DLBCL (CD20 + , CD10 + , Bcl-6 + , MUM-1 − , Ki-67 90%), and FISH analysis demonstrated gains at 11q23.3/11q24.3 without MYC rearrangement. After surgery, the patient began R-CHOP chemotherapy. Among the 34 reviewed cases, the median age was 56 years (range 3.5–84); common symptoms included abnormal vaginal bleeding (56%), pelvic mass (47%), and abdominal pain (41%). DLBCL accounted for 74% of cases (GCB subtype 15%, non-GCB 41%). Uncommon subtypes included NK/T-cell lymphoma (6%), peripheral T-cell lymphoma (6%), myeloid sarcoma (3%), indolent T-cell lymphoproliferative disorder (3%), and others. Treatment approaches have shifted from surgery alone to surgery plus R-CHOP, which was used in 50% of recent cases; however, comparative survival could not be assessed from these descriptive data. The combination of 11q alteration with appendiceal involvement appears not to have been previously reported; however, this observation is descriptive and does not by itself establish a distinct diagnostic entity. In summary Diagnosing primary uterine lymphoma presents considerable challenges. Our case draws attention to a gain at 11q23.3/11q24.3 as a molecular feature of uncertain significance and highlights the value of a multidisciplinary care approach. Systematic chemotherapy, particularly R-CHOP, is the mainstay of current treatment for DLBCL, although comparative efficacy could not be assessed from the reviewed cases.
Source: Frontiers in Medicine
Institutions: Wenzhou Medical University, Ningbo No. 2 Hospital
Language: English
Subfield: Pathology and Forensic Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $3,801
OpenAlex ID: W7225434201