Renal Medullary Carcinoma; A Rare Entity

Abstract

Renal medullary carcinoma (RMC) is an uncommon aggressive neoplasm of the kidney. RMC is biologically aggressive with a very poor prognosis, and metastasis is seen in up to 95% of the patients at diagnosis or shortly thereafter. The common sites of metastasis are respectively lymph nodes, lungs, livers, and adrenal glands in order of frequency. The presence of poorly differentiated eosinophilic cells in a characteristic fibro-inflammatory stroma is seen in histological examination. The origin and pathogenesis of RMC are unclear. The radiographical and pathological findings suggest that RMC probably originates in the calyceal epithelium in or near the renal papillae, which could be the result of chronic ischemic damage in the renal papillae epithelium by sickled erythrocytes. Positivity of VEGF and HIF-1α supports the chronic hypoxia that may be caused in the pathogenesis of RMC. Other factors such as genetic or environmental factors are important. Although hemoglobinopathy is very common, RMC is very rare. An understanding of the molecular and genetic factors of this rare disease is important for its prevention and treatment. We herein describe an adult Turkish patient, who presented with hematuria. The diagnosis was RMC after pathological examination.

Keywords: Case reports; Kidney neoplasms; Medullary carcinoma.

Figure 1

Computed tomography shows the mass in the central area of the kidney.

Figure 2

It shows Gross-section of the excised kidney.

Figure 3

Tumor cells have infiltrated into the renal pelvis (HE&400).

Figure 4

Tumor cells have a loss of INI-1 expression (×200).

Figure 5

Cytoplasmic area is depicted with positive staining of vimentin (×200).

Figure 6

Positive staining of the tumor cells with panCK is illustrated (×200).

Figure 7

Positive staining of the tumor cells with CK19 is depicted (×100).

Figure 8

Cells are negative for the expression of uroplakin, P63, CK7, CK20, and 334βE12 (×100)