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Background
Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a rare congenital abnormality. Concurrent association of pelvic mass in a patient with MRKH is even rarer and poses a diagnostic challenge regarding its origin. This case illustrates the importance of radiological imaging, especially MRI, in such circumstances, to confirm the origin of the mass, define its relationship with adjacent structures and to propose possible differential diagnosis.
Case presentation
A 43-year-old woman with a history of primary amenorrhoea presented after experiencing 20 days of vague pain in the right iliac fossa. She had been evaluated for primary amenorrhoea in the past, and diagnosed to have a hypoplastic uterus.
On clinical examination, the patient had normally developed secondary sexual characters and breast development (Tanner's stage 5). Palpation of the abdomen showed a lobulated, mobile, firm mass arising from the pelvis. Speculum examination showed a blind vagina with absent cervix, and a solid mass was palpated at the fornices. The patient was referred for an ultrasound examination.
Investigations
Transabdominal ultrasound showed two large heterogeneously hypoechoic lobulated masses in the pelvis. The uterus and right ovary were not visualised. The left ovary was normal in size. Based on ultrasound, the possibility of a mass arising from the right ovary was proposed, and contrast-enhanced MRI of the pelvis was suggested for further characterisation of the mass, and to confirm the absence of the uterus.
On MRI, two large circumscribed masses were seen in the pelvis and hypogastrium. On T2-weighted images ( figure 1 A), the masses appeared heterogeneously hypointense compared to muscles. No obvious uterus was visualised. On postcontrast imaging, the masses showed moderate enhancement with central non-enhancing necrotic areas ( figure 1 B).
Of note...