CASE 6230 Published on 26.09.2007

MR findings of primary uterine body and cervix lymphoma.

Section

Genital (female) imaging

Case Type

Clinical Cases

Authors

Anastasiou Ath., Geranou Chr., Michailidou G., Georgitziki K., Vekiou R. Dept of Radiology, "Hippokrateion" General Regional Hospital 54644 - Thessaloniki / GREECE

Patient

17 years, female

Clinical History
A 17-year-old female presented to our hospital with a 4-month history of menometrorrhagia and lower abdominal pain.
Imaging Findings
A 17-year-old female was admitted to our hospital because she had been experiencing abnormal vaginal bleeding for 4 months and increasing pelvic discomfort 3 weeks prior to admission. Her medical and family history was unremarkable. Physical examination showed no sign of palpable abdominal mass, peripheral lymphadenopathy or lower extremity edema. Bimanual pelvic palpation was painful and revealed a fixed bulky cervix. Cervical smear was performed repeatedly and it was negative. Blood evaluation was also normal. Magnetic Resonance Imaging was performed in a 1 Tesla Unit using a pelvic phased array coil. MR demonstrated enlargement and infiltration of the uterine corpus and cervix. Clear margination of the lesion appeared, showing an abrupt line transition to the healthy uterine fundus. Despite the extensive involvement of the myometrium and cervical stroma, the endometrium and the cervical epithelium were preserved. The lesion exhibited a homogeneous hypointense signal on T1 WI and trufi images and was uniformly enhanced with gadolinium. The urinary bladder was pushed by the enlarged cervix but it remained intact. Bilateral hydronephrosis and dilatation of the ureters were also present. Colposcopic biopsy established the diagnosis of primary lymphoma, MALT (Mucosa Associated Lymphoid Tissue) type. Bone marrow biopsy was negative. The patient was successfully treated with chemotherapy thus resulting in marked remission.
Discussion
Uterine cervical lymphoma is rare, comprising less than 1% of cervical malignancies, although it is the most common site of lymphoma (primary or secondary) in the female genital tract. Cervical involvement in multiorgan disease is more common than primary lymphoma. Uterine corpus lymphomas are even rarer. The age at presentation ranges from 20 years to 80 years, with the median age varying from 40 years to 59 years. Intermittent vaginal bleeding or spotting is the usual presentation although symptomatic pelvic mass and/or urinary symptoms can be other modes of presentation. Cervical cytology is often normal as these tumours arise from cervical stroma, and squamous epithelial lining is preserved initially. A deep cervical biopsy is essential for diagnosis. Diffuse uterine enlargement is the most common appearance. Less commonly, there may be a polypoidal or multinodular mass or a submucosal mass mimicking leiomyoma. CT features of uterine lymphoma are similar to those of other primary pelvic neoplasms with diffuse uterine enlargement and lobular contour alteration, often mimicking fibroids. It has been reported that cervical lymphoma is best defined on T2 weighted images or contrast-enhanced T1 weighted images. Uterine lymphoma on MRI is usually homogeneous in signal, lacks clear margination and shows moderate, uniform enhancement unlike degenerative leiomyoma, endometrial carcinoma or large cervical tumours. Architectural preservation and an intact endometrium have been reported to be characteristic features of uterine lymphoma. Cross-sectional imaging (CT, MRI) is required for staging the extent of disease. In patients with multiorgan disease the pattern of change may be typical of lymphoma, allowing a confident pre-biopsy diagnosis, although, for treatment and prognostic purposes, biopsy is mandatory to determine the precise histological subtype. MRI is superior to CT as an adjunct to clinical evaluation of invasive cervical cancer, providing more complete assessment of morphological risk factors and staging, important in patient prognosis and treatment planning. Both techniques are comparable in terms of assessing lymph node size, though lymph node specific iron oxide contrast agents promise an improvement in lymph node characterization on MR imaging .The morphologic differential diagnosis includes chronic lymphocytic cervicitis, chronic PID, undifferentiated adenocarcinoma of the endometrium, anaplastic squamous cell carcinoma, mixed mesodermal tumour, poorly differentiated stromal sarcoma, cervical small cell carcinoma, tumour with neuroendocrine differentiation and also metastatic tumours. The proper use of an adequate immunohistochemical panel of antibodies including epithelial, haematological and mesenchymal markers can lead to the correct diagnosis.
Differential Diagnosis List
Primary lymphoma of the uterine body and cervix, MALT type.
Final Diagnosis
Primary lymphoma of the uterine body and cervix, MALT type.
Case information
URL: https://www.eurorad.org/case/6230
DOI: 10.1594/EURORAD/CASE.6230
ISSN: 1563-4086