Metastatic neuroendocrine tumor

Case contributed by Dr. Khawaja Bilal Waheed

Presentation

A young woman presented with a 3-month history of inter-menstrual bleeding, and a 1.5-month history of epigastric pain, nausea, and abdominal distension with 2-kg weight loss. Deranged liver function tests were noted.

Patient Data

Age: 35 years
Gender: Female

Computed tomography shows multiple mostly hypodense liver lesions embedded in both lobes. An ill-defined heterogeneously enhancing ileocecal mass is seen with adjacent large lymph nodal mass. Several small rounded solid left renal hypodense lesions are also seen. A large heterogeneously enhancing pelvic solid ovarian mass is also noted. Mild upper abdominal lymphadenopathy and mild ascites. No evidence of bowel obstruction, pulmonary nodules, or focal bony lesions. 

Contrast enhanced magnetic resonance imaging showed heterogeneous ileocecal mass with adjacent lymph nodal mass, besides a large predominantly solid heterogeneously enhancing ovarian mass in the pelvis.

Radiologists should be aware of the possibility of a metastatic neuroendocrine tumor in a young patient with the widespread and rapidly progressive multiorgan disease. Advanced scintigraphy studies are available to evaluate further that may avoid needle biopsies.  

Case Discussion

In our case, a biopsy of the liver lesion was initially performed that revealed metastatic neuroendocrine carcinoma likely from the ileocolic region, which was later confirmed by colonoscopic biopsy. Excision of ovarian mass also revealed metastatic disease from the neuroendocrine tumor.

Imaging-wise, primary (colonic, ovarian) or secondary/ metastatic disease and lymphoma were initially considered and information shared with the clinicians. Ill-defined heterogeneously enhancing ileocecal mass with an adjacent large mesenteric nodal mass also favored the epicenter of disease to be at this site in our case.

Neuroendocrine tumors (NET) represent a wide spectrum of disease or malignancy derived from neural crest tissue found in the pituitary gland, adrenal and gastrointestinal tract. 68Ga-DOTATATE PET is becoming a popular imaging technique for detecting NETs. Although surgery remains the option for localized tumors however systemic treatment options (including peptide receptor radionuclide therapy) for metastatic NET have been promising 1.

Hepatic metastases are more common than primary liver tumors, usually are asymptomatic and found during work-up of malignancy presenting in another way. They appear mostly as hypodense on CT imaging, with a few primaries (renal cell carcinoma, islet cell tumors, NET) that can have hyperenhancing metastases. Gastric and appendiceal cancers have a high rate of ovarian metastatic disease 2,3. However, sometimes a biopsy of ovarian mass remains the option to exclude synchronous ovarian malignancy as well.

Clinicians and radiologists should be aware of NET in the differential possibility of widespread metastatic disease in a young patient.

Acknowledgement: Special thanks to Dr. Emad Fouad M Said (Consultant Radiologist, KFMMC, Dhahran) and Dr. Saad Mohammed Alsubaie (Consultant Histopathology, KFMMC, Dhahran) for contributing to this case.   

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