YO7 - Diagnostic Dilemma : A case of Metastatic Tubulocystic Carcinoma of the Kidney

Date 17 December 2016
Event ESMO Asia 2016 Congress
Session Poster lunch
Topics Renal Cell Cancer
Presenter Nisha Shariff
Authors N. Shariff1, M. Saad1, R. Pathmanathan2, A. Alip1
  • 1Clinical Oncology, University of Malaya Faculty of Medicine, 50603 - Kuala Lumpur/MY
  • 2Pathology, Sime Darby Medical Centre Subang Jaya, 47500 - Subang Jaya/MY

Abstract

Case Summary

A 66 year old man presented with haematuria in November 2012. An initial Computer Tomography (CT) scan showed a left renal subcapsular haematoma. As there was no suggestion of malignancy, he was observed and serial imaging showed a resolving haematoma. In August 2013, he complained of weight loss and abdominal pain. A repeated CT scan revealed a left renal mass, multiple liver lesions and extensive lymphadenopathy. Biopsy of his liver lesions was reported as combined hepatocellular carcinoma-cholangiocarcinoma. Repeated renal and liver biopsy confirmed the final diagnosis of metastatic tubulocystic renal carcinoma(TCRC) - as immunohistochemistry stains were negative for Hepar-1 and AFP. He was started on weekly temsirolimus which improved his symptoms by week 4. Repeat CT scan showed stable disease. He experienced clinical progression 19 weeks after starting treatment and succumbed to his disease at week 38.

TCRC is a rare renal neoplasm. Initially thought to be a subtype of collecting duct carcinoma, it is now related to papillary renal cell carcinoma(RCC). The 2010 AJCC/TNM classification for RCC recognizes TCRC as a distinct group.

TCRC follow an indolent course with good prognosis. Only 10% were reported to metastasise and were associated with papillary RCC. Our patient was reported to have papillary differentiation on biopsy which may explain the aggressive nature of his disease. Histologically, tubules are lined by a single layer of low nuclear grade cuboidal epithelial cells with eosinophilic cytoplasm – commonly with areas of hobnail appearance.

Despite clear treatment choices in the management of metastatic clear cell RCC, treatment of non-clear cell histologies are less defined. mTOR-targeted therapy can benefit patients with non-clear cell RCC and is recommended in guidelines. For patients with non-clear cell RCC treated with mTOR therapy, median progression-free survival was 2.9 months and overall survival(OS) was 8.7 months. However, median OS for MSKCC poor risk group was 6.4 months. Our patient had 4.8 months on temsirolimus before clinical progression and survival of 9.5 months.

Rare tumours with atypical presentation are a diagnostic dilemma and require multidisciplinary approach to best manage these patients.