Case ReportFirst Report of Renal Cell Carcinoma Metastasizing to the Clivus in a Pediatric Patient
Introduction
Renal cell carcinoma (RCC) is a relatively rare malignancy, occurring in <5% of adult malignancies.1 It is even rarer in the pediatric population.1
At the time of discovery, up to one quarter have metastasized already.1 The classic demographic is an elderly male with a history of smoking, who usually presents with hematuria, flank pain, and an abdominal mass.1 The usual sites of spread are to the lungs in half of the cases; bones, lymph nodes, and the brain account for the rest.1
The clivus is infrequently involved in metastatic malignancy in general.1 Reports in the literature of this phenomenon are few and far between.1, 2, 3, 4
From 1980 to present, only 20 cases of clival metastases have been reported in the literature. They came from diverse primary malignancies, including renal cell, lung, breast. and prostate.1
Involvement of the clivus by metastatic RCC is even rarer, only 3 cases having been described in the literature; all were in adults in their sixth decade of life.1
When it does get involved, owing to the close anatomic relationship between the clivus and the sixth cranial nerve, diplopia on the basis of an abducens palsy is usually present.1, 2, 4 On exiting from the brainstem, the sixth cranial nerve travels in the subarachnoid space along the posterior border of the clivus, before entering Dorello's canal on its way to the cavernous sinus.
The nerve can get stretched between the 2 rigid points of its exit from the brainstem and its entry into Dorello's canal by pathologies displacing the brainstem, hence abducens palsy is not infrequently a false localizing sign in conditions of raised intracranial pressure.
In clival tumors, however, there may be direct contact with and compression of the nerve, hence its palsy becomes a true localizing sign. Involvement of the cavernous sinus laterally may result in further cranial nerve deficits.3
Section snippets
Case Report
We present an 11-year-old girl who presented with headaches and diplopia of relatively short history. She had no other complaints. On examination, she had a left-sided sixth nerve palsy. A magnetic resonance imaging showed a clival lesion (Figure 1). Her parents were reluctant to consent for a biopsy and therefore she was lost to us for several months. Upon return, she looked far sicker, had lost weight, developed nausea and dysphagia and a repeat scan now showed a much larger lesion of the
Discussion
This case is the first ever report in the literature of renal cell carcinoma metastasizing to the clivus in a pediatric patient.
A PubMed search of the English medical literature for “Paediatric RCC metastasizing to the skull or spine” did not reveal any published data.
A recent study showed that renal cell carcinoma occurs more commonly in black females in the pediatric population, with a mean age of 15.4 years. Metastatic disease was recorded in 25.6% of patients and these demonstrated a poor
Conclusion
Our case illustrates several important lessons.
First, metastatic carcinoma should not be discounted purely on the basis of age. Second, primary translocation RCC may be clinically asymptomatic and painless, hence escaping early detection, and third, these tumors may have the propensity to metastazise to unusual sites.
This article serves to remind practitioners of this possibility when faced with this clinical scenario.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.