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Introduction: An extremely rare pediatric renal tumor, ossifying renal tumor of infancy (ORTI) usually presents in male infants with intermittent episodes of painless gross hematuria. The tumor is benign and surgically treatable. Imaging typically shows a preserved renal outline with a calcified intrapelvic mass. Microscopically, these lesion shows varying components of osteoid, osteoblastic cells, and spindle cells. However, the etiology is unclear. Surgical excision is curative and recurrence or malignant disease is unheard of.We report a case of ORTI and review the literature to assess the clinicopathological features of this unusual neoplasm. Case presentation: A 5-month-old boy was admitted with isolated gross hematuria. Ultrasound showed a well-defined solid mass in the lower pole of right kidney that was heterogenous on CT (Computed Tomography) but without calcifications. Nephrectomy was done and histopathology showed an ossified core surrounded by a spindle cell component confirming the diagnosis of an ORTI. Conclusion: ORTI is a rare benign pediatric renal tumor with some typical clinical, radiological and pathological features. A male infant with painless intermittent macroscopic hematuria with an endophytic calcified renal mass involving the calyceal system, points towards an ORTI as the possible diagnosis.