Enlarged uterus

A 48-year-old woman was admitted with abdominal pain and distension for a week, with no analgesic improvement, and vaginal bleeding. On physical exploration, cervix hypertrophy was found.

Ultrasound demonstrated an enlarged uterus with myomas and polyps, previously known.

Abdominopelvic contrast-enhanced CT scan showed diffuse uterus enlargement, at the expense of the cervix. It associated multiple retroperitoneal adenopathies, which enveloped the aorta and the  Inferior Vena Cava; and common, internal and external iliac bilateral adenopathies with locoregional fat stranding. In addition, pathologic inguinal adenopathies were seen.

MRI was performed, it demonstrated on the uterine cervix a solid and heterogeneous mass of 7,5 x 7 cm, which involved the vagina and impinged the posterior bladder wall. In the right wall of the uterus body, a solid mass of 7 x 6 cm was seen, in contact with another right mass of 4 x 2 cm. These masses had low signal intensity on T1-weighted images (WI), high signal on T2-WI and diffusion-WI (DWI), low values on ADC map and important heterogeneous enhancement on contrast-enhanced T1-WI. Furthermore, pathologic lymph nodes, described on CT scan, were seen.  

A biopsy was made and confirmed uterine lymphoma.

Only 1% of extranodal lymphoma affects the female genital tract. [1, 2] Although malignant lymphoma frequently infiltrates the uterus in disseminated disease, primary involvement of the uterine cervix and corpus is uncommon. [3, 4] Uterine involvement usually affects the cervix, with uterine body involvement being extremely rare. [2, 3] Most of these tumours are non-Hodgkin lymphoma, being the most common cell type diffuse large B-cell non-Hodgkin lymphoma (70%), followed by follicular lymphoma (30%). [5]

Uterine lymphoma affects women over a broad age range, being more common in the fourth and fifth decades. [1, 5]

Abdominal pain and genital bleeding are the most common presenting symptoms. Hydronephrosis may be found, due to bladder or ureter involvement. [2]

On imaging tests, uterine lymphoma may be manifested as diffuse enlargement of the uterus or as a localized mass (multinodular mass, endocervical polyp or submucosal mass). [1]

MR imaging is the technique of choice. The most common finding is extensive diffuse symmetrical enlargement of the uterus with homogeneous low signal intensity on T1-weighted images (WI) and intermediate to high signal intensity on T2-WI. Contrast-enhanced T1-WI shows heterogeneous enhancement of the uterus. [1, 3, 4]

Characteristic features are the preservation of the endometrial epithelium, junctional zone and uterine architecture.  It is rare to found tumoral necrosis and destruction of the endometrial lining. [1]

Imaging tests (CT and MRI) are important in the evaluation of the disease´s extent, the involvement of adjacent organs (vagina, urethra, bladder and ureters) and lymph nodes. [1]

Cervical cytology is normal due to tumour cells developing in the cervical stroma and causing rare necrosis and mucosal ulceration. The definitive diagnosis is made with tissue biopsy. [1, 2]Differential diagnosis is broad and it is difficult to distinguish from other uterine malignancies. [4]

Uterine lymphoma has a good prognosis with an appropriate treatment (radiation therapy and chemotherapy), although it has not been established a standard treatment. Hysterectomy is avoided because it does not improve the prognosis and it has a high risk of bleeding. [1, 2, 4] The most important factor for prediction of the patient´s prognosis is the stage of the disease. [1]

In conclusion, uterine lymphoma should be considered in a patient with an enlarged uterus and characteristic features as preservation of the uterine architecture, endometrial enhancement and homogeneous signal intensity. [1, 5]