Primary lymphomas of the female genital tract: imaging findings

Maeda²,

J Clin Exp Hematopathol

et al. 2020

The relapse of acute lymphoblastic leukemia (ALL) usually involves the bone marrow, with the central nervous system being the most frequent extramedullary site. The relapse of ALL in the female genital organs, particularly the uterus, is markedly rare. We report such a patient who developed relapse in the bone marrow and uterus. The uterine lesion, which presented as abnormal uterine bleeding, consisted of a mass on MRI and proliferation of ALL cells on histology. MRI revealed a heterogeneous high-intensity mass (T2-WI/D-WI) with a diameter of 6.8 cm, a notable decrease in the apparent diffusion coefficient (ADC), and mild enhancement by contrast enhancement study. Histological findings of the uterine cervix demonstrated the infiltration of ALL. The patient achieved remission by allogeneic haplo-identical hematopoietic stem-cell transplantation, but died of complications of the transplantation. This case suggested that attention should be paid to the uterus as a site of extramedullary relapse. In addition, abnormal uterine bleeding, which is a common sign of hormonal imbalance and hormone replacement therapy after chemotherapy, may be an initial sign of extramedullary recurrence. To confirm uterine relapse as an intractable disease, the accumulation of more cases is required.

Section: Discussionmentioning

confidence: 99%

Uterine relapse of Philadelphia chromosome-negative acute lymphoblastic leukemia

Maeda²,

J Clin Exp Hematopathol

et al. 2020

The British Journal of Radiology

“…11 Cases of FL and endometrial primary marginal zone lymphoma have also been recorded. 12 Both the cervix and uterine body are usually involved with diffuse uterine enlargement and FDG avidity ( Figure 11), a large lobulated ( Figure 12)/exophytic mass or nodules. 12 While submucosal leiomyomas are similar in appearance on conventional imaging 12 the hypermetabolism in lymphoma on PET is a distinguishing feature.…”

Section: Female Genitourinary Organs Cervix/uterusmentioning

confidence: 99%

“…Patients can be asymptomatic or experience pelvic pain. 12 Secondary involvement is described in cases of ovarian lymphoma. 13 Primary fallopian tube lymphoma can be unilateral or bilateral, with variable presentations-associated with chronic inflammation (tubo-ovarian abscess/endometriosis), a pelvic mass or an incidental finding during surgery.…”

Section: Fallopian Tubementioning

confidence: 99%

“…8,14 Involvement can be unilateral or bilateral and ascites is infrequent despite the larger size of the lesion when compared with other ovarian malignancies. 12 Less than 50% of cases demonstrate peritoneal involvement and extension often occurs through the pelvic and para-aortic lymph nodes. 12 In premenopausal women, benign physiological FDG uptake of the ovaries and endometrium varies cyclically and occurs at VAGINA (a-c) homogenous, intense, focal tracer uptake is seen in a markedly thickened vaginal wall (green arrows) with a maximum standardised uptake value of 32.8 g ml -1 consistent with DLBCL that was treated with chemotherapy and radiotherapy.…”

Section: Ovarianmentioning

confidence: 99%

“…8 Vaginal Primary vaginal lymphoma ( Figure 14) is exceedingly rare with a mean age of diagnosis in the fifth decade, typically presenting with perineal discomfort or vaginal discharge/bleeding. 12 Approximately, 50% are diagnosed at autopsy due to the lack of solid lesions that could otherwise be identified by conventional imaging. FDG-PET can potentially prevent a delay in diagnosis and treatment as the mean 5-year survival is 80% for early stages and 30% in advanced stages.…”

Section: Ovarianmentioning

confidence: 99%

See 2 more Smart Citations

Genitourinary involvement of lymphomas on FDG-PET

Naik

Lin

2018

Primary extranodal lymphomatous involvement of the genitourinary tract is rare and secondary extranodal involvement in disseminated disease occurs more frequently. Imaging of metabolic activity with 2-(fluorine-18) fluoro-2-deoxy-d-glucose (FDG) used in PET facilitates the identification of these extranodal sites of disease, particularly in the absence of structural lesions on conventional imaging modalities. Primary extranodal lymphoma affecting the genitourinary system is often caused by high-grade Non-Hodgkin's Lymphoma (NHL) with the most common subtype being diffuse large B-cell lymphoma (DLBCL). Although rare, the incidence of extranodal lymphoproliferative disease is increasing and a delay in diagnosis holds a poor prognosis. Familiarity with benign and physiological causes of FDG uptake, particularly due to the urinary tracer excretion is crucial in identifying sites of lymphomatous involvement in the genitourinary system. Additionally, non-lymphomatous malignancies are usually treated surgically, whereas lymphoma is primarily treated with chemotherapy and/or radiotherapy. Therefore, accurate identification and staging together with histological confirmation significantly impacts management of these patients. This article serves to review and illustrate the imaging findings on FDG-PET/CT of primary extranodal lymphoma affecting the genitourinary system.

American Journal of Surgical Pathology

Follicle Center Lymphoma (FCL) of the Lower Female Genital Tract (LFGT)

Saksena

Jain

Pack

et al. 2022

Primary cutaneous follicle center lymphoma has been distinguished from nodal follicular lymphoma (FL) based on genomic and clinical features. The nature of other extranodal FLs is not well defined. We report 15 cases of follicle center lymphoma involving the lower female genital tract. Cases were evaluated using an immunohistochemical panel for B-cell lymphoma, B-cell clonality, fluorescence in situ hybridization for BCL2 gene rearrangement, and next-generation sequencing. All patients had localized disease with no evidence of bone marrow involvement. Most cases (12/15, 80%) had a follicular pattern, at least focally. Large centrocytes were a prominent feature leading to concern for diffuse large B-cell lymphoma by referring pathologists. Neoplastic cells were positive for CD20 and BCL-6, while BCL-2 was positive in 2/15 (13%) cases. Fluorescence in situ hybridization for BCL2 gene rearrangement was negative in 10/11 (91%) cases. Next-generation sequencing performed in 10 cases revealed TNFRSF14 as the most frequently mutated gene in 6/10 (60%) cases. No case had CREBBP or KMT2D mutations as seen in nodal FL. None of the patients had progressive disease with durable complete remission achieved in 10/12 (83%) cases. The median follow-up period was 7.8 years (range: 0.2 to 20.5 y) with a 5-year overall survival of 100%. We conclude that follicle center lymphoma of the lower female genital tract is a novel variant of primary cutaneous follicle center lymphoma. Despite a frequent component of large cells, it is characterized by localized disease and low risk for dissemination. Awareness and recognition are important to distinguish these lesions from aggressive B-cell lymphomas.