My Strangest Case: A Man With Worsening Pain in Both Arms

Elizabeth E. Ginalis, MD; Arthur Carminucci, MD; Nitesh V. Patel, MD; Simon Hanft, MD


July 22, 2020


This is a rare case of a 68-year-old man with an intradural extramedullary spinal metastasis to the cervical spinal cord that presented 22 years after treatment for RCC. Ultimately, the pathology of the tumor was confirmed after histologic and immunohistochemical analysis of the surgical resection. Upon microscopic examination, tumor cells demonstrated pleomorphic epithelioid cells in a papillary pattern on hematoxylin and eosin staining (low and high power) (Figure 3a and 3b, respectively).

Figure 3.

Tumor cells contained pleomorphic nuclei with occasional pseudo-nuclear inclusions. Upon immunohistochemical staining, the tumor stained positive for PAX8 (Figure 3c), CAM5.2 (Figure 3d), vimentin, and EMA. The Ki67 index was 50%.

The tumor's profile was consistent with metastatic papillary RCC. Not only is intradural extramedullary spinal metastasis from RCC extremely rare, a latency interval of 22 years to diagnosis of metastasis is among the longest reported in the current literature.

As many as 40% of patients with RCC develop metastatic disease.[1,2] RCC most commonly metastasizes to the lungs (50%), bone (49%), lymph nodes (30%), liver (8%), and brain (3%).[1,2,3,4] However, spread to the intradural spinal cord is uncommon. In patients with a history of RCC, considering the possibility of intradural spinal metastasis is important if the patient is experiencing weakness or urinary incontinence.

Other common presenting symptoms may include bowel dysfunction, pain, and paresthesias. Physical examination findings may include neurologic deficits in motor and sensory examination or abnormal reflexes. If such signs or symptoms are present, imaging of the spine is indicated, preferably with MRI.

Owing to the rarity of intradural metastasis, treatment is often individualized, because management guidelines are not clearly defined. Moreover, intradural metastasis is associated with a poor prognosis and limited life expectancy; thus, any therapy is often palliative, with a goal to improve symptoms. Management options include surgical resection, radiation therapy, chemotherapy, or a combination of these treatments.


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