Splenohepatic sarcoidosis 12 years after breast cancer curative surgery: a diagnostic dilemma in imaging

A 52-year-old woman was referred to the internal medicine department due to a persistently elevated alkaline phosphatase (ALP, 175 U/L) with an increasing trend. The gamma-glutamyl transferase level was 99 U/L, whereas the transaminases, bilirubin, erythrocyte sedimentation rate and white blood cell count were normal. The abdominal ultrasound did not reveal any abnormal findings. The patient was otherwise asymptomatic without any significant comorbidity. There was no fever or weight loss. Her regular medication included escitalopram 10 mg once daily, omega-3 fatty acids and vitamin D, which were stopped after the detection of increased ALP. Her habits included social drinking (three alcohol doses per week). There was no previous exposure to environmental, occupational or infectious elements. Twelve years earlier, she underwent right breast cancer resection and axillary lymph node dissection for grade II intraductal breast cancer (oestrogen receptor positive and progesterone receptor negative). Breast reconstruction was performed 2 years afterwards. There were no signs of recurrence during a 5-year follow-up. There was no abdominal surgery in her anamnesis. The patient underwent magnetic resonance imaging of the upper abdomen, which depicted multiple hypointense splenic lesions in the T2-weighted scan and smaller heterogeneous areas in the liver. The magnetic resonance cholangiography showed normal biliary anatomy. The case was discussed at the oncological multidisciplinary meeting for the decisions regarding the progression of the diagnostic pathway. A computed tomography (CT) scan of the abdomen and the chest showed multiple low-attenuation, nonenhancing splenic and hepatic lesions (Fig. 1) and a few small nonspecific nodules in the lungs. Tumour markers in the blood (carcinoembryonic antigen, alpha-fetoprotein, carbohydrate antigen 12-5 and 19-9) were within the normal range, except the carbohydrate antigen 15-3 which was slightly elevated (33 kU/L). Fine needle aspiration biopsies that were obtained percutaneously from splenic lesions were non-diagnostic; however, there was no sign of malignancy. An exploratory laparoscopy was undertaken (Fig. 2) which confirmed the presence of numerous light grey areas on the spleen and liver that were rather soft on palpation with the laparoscopic instrument. Operative biopsies from both the liver and spleen revealed granulomatous inflammation. Ziehl–Neelsen staining was negative and the diagnosis was competent with sarcoidosis. A positron emission tomography-CT (PET-CT) was subsequently performed. There were hypermetabolic foci in the neck, mediastinum, bones and groins, and in the para-aortic and para-iliac regions. Prednisolone 40 mg daily was started due to the extension of sarcoidosis. One month later, ALP level regressed to normal range (75 U/L). Sarcoidosis is a chronic inflammatory disease of unknown aetiology and it may affect multiple organs. It has been proposed that breast cancer may constitute a risk or triggering factor for developing sarcoidosis which most commonly occurs in the thorax or lymph nodes, whereas hepatosplenic manifestation is infrequent. In a sarcoidosis registry, 18% of patients had a history of breast cancer, however, this potential association remains still unproven. Interestingly, the mean age of sarcoidosis onset after breast cancer is 61 years which is much higher than that of the regular population with no cancer history. The interval between breast cancer and sarcoidosis is rather short, typically around 2–4 years. Later occurrence is exceptional with reported extremes reaching up to 8 years. From the clinical point of view, differential diagnosis between metachronous or metastatic cancer and sarcoidosis is challenging, particularly in patients with a history of breast cancer. Sarcoidosis is a granulomatous disease and the nodular lesions that develop in the affected organ may mimic cancer in imaging. PET-CT is more effective than CT in the determination of the extension of the