Sarcomatoid and rhabdoid dedifferentiation in clear cell renal cell carcinoma, a rare combo: Does that have a dismal prognosis?

Sarcomatoid and rhabdoid dedifferentiation of Renal Cell Carcinoma (RCC) has long been recognized as subtypes with extremely poor prognosis and reduced progression-free survival. The overall incidence ranges from 5% to 20%. These subtypes present at an advanced stage at diagnosis and are associated with aggressive behaviour with a high recurrence rate. Promising results including complete response were observed in patients who tolerate the multi-modal treatment well. We report a case of a 75-year-old lady who underwent radical nephrectomy with renal vein thrombectomy for RCC. Histopathology confirmed clear cell carcinoma with rhabdoid and sarcomatoid differentiation. She was started on Sunitinib and followed up for 1 year. She is doing well at present with no recurrence. This case report stresses that though rhabdoid and sarcomatoid carcinoma have a dismal prognosis, the outcome is promising if detected early and offered multimodal treatment. The sRCC (sarcomatoid RCC) and rRCC (rhabdoid RCC) portend a poorer prognosis. Overall survival is slightly better with rRCC compared to sRCC.  IHC (immunohistochemistry) plays an important role in diagnosing and treating these variants. The combination of the two variants further worsens the prognosis.  Results are much better if the diagnosis is made early and prompt multimodal treatment is initiated.