Teaching Case

A 57-year-old male presented with fatigue, giddiness and melena for 4 days. He underwent right radical nephrectomy and inferior vena cava (IVC) thrombectomy 8 years ago for conventional renal cell carcinoma (RCC). No history of adjuvant chemotherapy or recurrence after surgery. No history of nausea, vomiting, abdominal pain or use of non-steroidal anti-inflammatory drugs. Physical examination revealed tachycardia and pallor.

Upper Gastrointestinal (GI) scopy was performed which showed a polypoidal ulcerative lesion at 2^nd/3^rd part of duodenum with active bleeding. Multiple biopsies were performed and sent for histopathology. (Figure 1)

Non-contrast Magnetic Resonance Cholangiopancreatography (MRCP) was performed for periampullary tumours. It showed an ill-defined, T2 isointense, lobulated soft tissue measuring 2.5 x 2.0 cms at 3^rd part of duodenum separate from the uncinate process of pancreas. No evidence of pancreatic duct or common bile duct (CBD) dilatation. (Figure 2)

Contrast-enhanced multi-phasic Positron emission Tomography and Computed Tomography (PET-CT) study was performed to evaluate the disease status. It showed Fluro-Deoxy-Glucose (FDG) avid lobulated soft tissue density lesion at 3^rd part of duodenum region measuring 2.5 x 2.0cms and Standardized Uptake value (SUV) max of about 6.25. It shows arterial phase hyperenhancement with infiltration 2^nd/ 3^rd part of duodenum and loss of fat planes with IVC. Sub centimetre sized hypermetabolic right supraclavicular and right azygous lymph nodes are suspicious for metastases. (Figure 3)

The histopathology results were suggestive of metastatic clear cell carcinoma. (Figure 4)

Renal cell carcinoma can metastasize to any organ. The most common sites include lungs, lymph nodes, liver, bones, adrenals, kidneys, brain, heart, spleen, intestine and skin. Gastrointestinal metastases are rare but can involve the entire GI tract from oesophagus to rectum. RCC metastases constitute about 7.1 % of metastases to the small intestine. [1]

Duodenal metastasis in particular is rare with less than 40 cases reported in literature. It most frequently affects the duodenal bulb / periampullary region.  Males are more commonly affected (M: F = 1.5:1). It may present many years after primary treatment, sometimes up to 17.5 years after initial surgery. the routes of spread being direct infiltration, lymphatic or hematogenous. 

These patients present with abdominal pain, anaemia, GI bleed, obstruction, jaundice or intussusception. [2-4]

Acute or chronic upper GI bleeding can be attributed to the vascular nature of RCC and need of anticoagulation for malignancy-related thromboembolic events. [2]

On direct visualization, by endoscopy these lesions are mostly solitary polypoid or ulcerated nodules/submucosal masses. [2]

Imaging plays a secondary role. It can show heterogeneously hyperenhancing masses in the periampullary region, restaging of the tumour, active contrast leak and complications like obstruction CBD dilatation. [5,6]

No standard management exists and treatment needs to be individualized. it depends upon the extent and location of the metastases. In patients with solitary duodenal metastases, complete surgical excision should be considered. widespread disease is highly resistant to chemotherapy and radiotherapy and palliative options including surgery, embolization, radiotherapy or systemic treatment should be considered. Sunitinib an oral vascular endothelial growth factor receptor kinase inhibitor is most widely used for the initial treatment in metastatic disease. Despite advances in treatment the median survival time in metastatic RCC is 6-12 months. [1,2,3,7]

Our patient was started on Sunitinib immunotherapy.

This case highlights the importance of early recognition and high index suspicion in any nephrectomised patient with any gastrointestinal symptom for metastases. A thorough investigation, particularly endoscopy and biopsy will be diagnostic. CT and MRI can act as an adjunct for disease status evaluation, restaging, complications and response evaluation. Surgical excision in subgroups with solitary duodenal metastasis provides opportunity for potential cure and meaningful disease-free survival.

Written informed patient consent for publication has been obtained.