Isolated renal haemangioma in children: presentation and management

Abstract

A 28-day-old baby boy presented with progressive jaundice and pallor of 3 weeks' duration. On examination he had moderate abdominal distension with a large rightsided firm immobile abdominal mass reaching the right iliac fossa. Laboratory studies showed severe anaemia (haemoglobin 52 mg/L), thrombocytopenia (platelet count 26 000/ µ L) and unconjugated hyperbilirubinaemia (total/direct bilirubin 193/7mg/L); Coombs test was negative. CT of the abdomen revealed a 10 × 7.5 × 5.5 cm heterogeneous mass arising from the right kidney, with areas of increased density suggestive of acute haemorrhage (Fig. 1). This large tumour was consistent with Wilms' tumour although mesoblastic nephroma was another possibility. The patient underwent emergency right nephrectomy. The kidney contained a 6.5 × 6.0 × 4.5 cm mass, the inner edge of which abutted the renal pelvis without invading it. The outer aspect of the mass compressed the surrounding renal cortex. The overlying renal capsule was focally ruptured and there was extensive haemorrhage into the perirenal fat. The cut surface of the mass was dark brown and friable, and the bulk of it appeared to be composed of clotted blood (Fig. 2). Microscopy of the mass revealed a cavernous haemangioma with extensive intratumoral and perirenal haemorrhage. Identifiable features of haemangioma were limited to a thin rim along the interface between the mass and normal renal tissue. They consisted of anastomosing vascular spaces separated by a scant connective tissue stroma, as well as large cystic spaces containing clotted blood (Fig. 3a). Entrapped normal tubules were occasionally seen within the stroma (Fig. 3b). The cells lining the vascular spaces were benign and expressed endothelial markers (factor VIII and CD34). Extensive sampling of the mass showed no evidence of malignancy. The patient had an uneventful postoperative course, and normal haematological and biochemical profiles upon discharge from hospital.

Pediatric Anastomosing Hemangioma: Case Report and Review of Renal Vascular Tumors in Children

“…A summary of the 50-year literature of pediatric renal vascular tumor cases (exclusive of malformations) is provided in Table 1. [8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] The list includes recent histologic diagnoses which are either provisionally or not yet incorporated in the International Society for the Study of Vascular Anomalies (ISSVA) classification system. 23 One of the uncategorized lesions is anastomosing hemangioma, which was recently described and has since been increasingly reported in the literature.…”

Association of capillary haemangioma with bilateral hydronephrosis in an infant

“…Isolated cavernous haemangioma was reported in the right kidney of a newborn, who presented with moderate abdominal distension and a large abdominal mass reaching to right iliac fossa 5 . Rastogi R et al reported a vesical haemangioma at VUJ in a patient with abdominal pain secondary to hydroureteronephrosis 6 .…”

Cavernous hemangioma of the kidney: A report of two cases and review of the literature

“…Organs like kidney are rarely involved and bilaterality is still rarer. [1–4] Multiple hemangiomas in the same kidney occur in upto 12 % cases. [4] Renal hemangiomas may vary in size from few millimeters to several centimeters in diameter.…”

“…[14] Sometimes renal hemangioma is a manifestation of a generalized hemangiomatosis that is they are associated with hemangiomas in liver, bowel, bone, or brain. [5] Patients usually present with profuse or relapsing painless gross hematuria, sometimes pain may be present, which mimics renal colic.…”

“…In a small localized tumor, a nephron sparing surgery may be tried but when malignancy cannot be ruled out, like in our cases, radical nephrectomy is considered. [1–48] Radiation therapy and recently laser-assisted ablation have been successful in small lesions diagnosed before surgery. [8–10]…”