A 48-year-old female smoker of 20 cigarettes/day, with hepatorenal polycystosis diagnosed 12 years earlier, and secondary chronic kidney disease stage 3 (with creatinine clearance of 50 ml/min and without proteinuria).
No other pathological history of interest.
The current disease begins with severe left lumbar pain requiring admission.
Initially it is oriented as cystic crisis.
A renal vesical echography is performed in which, in the context of hepatorenal polycystosis, there is a heterogeneous disorganization renal parenchyma due to cysts. There is a heterogeneous echogenic effect in the left pelvis.
Due to the lack of specificity of the findings and consolidation, the CT study again shows bilateral renal degeneration due to polycystosis, with some hyperdense cysts in the basal study performed without intravenous contrast.
The involvement is asymmetric, more intense on the left side, with the kidney being larger.
The left pyelic region is occupied by a soft tissue density tissue that shows moderate enhancement after intravenous contrast administration, with no clear definition of its limits.
Functionally, there is a clear delay in the left side and absence of elimination of intravenous contrast in the sections performed in a delayed manner.
The set of findings suggest tumoral occupation of the renal pelvis in the context of polycystosis with secondary functional alteration.
Retroperitoneal lymph nodes not significantly increased in size in interaortocaval and retroaortic situation are observed.
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With the clinical diagnosis of a renal mass compatible with a neoformative process, a left radical nephrectomy was performed, with left adrenalectomy plus lymphadenectomy, without incidents or immediate complications.
There were no postoperative complications, with stable renal function.
One month after nephrectomy, creatinine clearance was 52 ml/min and proteinuria was 0.16 g/24 h.
In the anatomopathological study of the specimen, macroscopically highlights a firm, whitish renal mass of 9 x 8.5 x 5 cm, with poorly defined borders, which presents a central area of necrosis.
It is located in the central renal portion, affecting almost the entire parenchyma, reaching the perirenal adipose tissue and surrounding the adrenal gland.
Four lymph nodes are identified in the renal hilium.
Non-tumoral renal parenchyma is completely destructured and replaced by multiple cysts up to 2 cm in diameter, affecting both the cortex and the renal medulla.
Histologically, the tumor consists of large and eosinophilic cytoplasm cells with rounded nuclei with marked atypia, which are organized into a solid, squamous pattern, characteristic of a carcinoma.
Tumor necrosis areas are extensive.
The tumor affects the proximal ureter, renal pelvis, renal parenchyma and adipose tissue both perirenal and perihilar.
The tumor is localized in the renal vein and extensive tumoral mass perineural with involvement of renal hilium ganglia.
The tumor mass consists of squamous cell carcinoma in which an area of intraureteral papillary urothelial carcinoma is observed.
Metastasis of the carcinoma was identified in two of the four hiliar lymph nodes identified in the specimen.
Surgical resection margins of the specimen are negative.
Paraaortic lymphadenectomy identifies 6 lymph nodes free of recurrence.
In summary, the pathological diagnosis of the specimen is poorly differentiated squamous cell carcinoma arising in the renal pelvis and ureter, pT4pN2 category, in the context of autosomal polycystic kidney disease.
Currently, the patient is under oncological treatment.
