Adult Multicystic Nephroma: Case Report and Review of the Literature

Abstract

A 53-year-old female patient admitted to the urology clinic with left flank pain without renal colic or macroscopic haematuria. No mass was detected on bimanual examination on left costovertebral triangle. Abdominal CT scan showed decreased renal parenchymal thickness of upper pole of the left kidney. Complex cystic mass with coarse calcifications and pathological contrast involvement, and a mass of 4x3 cm in size was detected on lateral aspect of the upper pole of the left kidney (Bosniak category III) [ As the presented lesion was located away from the collecting system and vascular structures, open nephron sparing surgery was performed successfully. On gross pathological examination the specimen was 4x4x1 cm in size and macroscopically encapsulated. ABSTRACTMulticystic nephroma (MCN) is relatively a rare, nongenetic and benign lesion of the kidney which has a bimodal distribution. The congenital form is commonly seen under the age of 2. Adult-onset MCN is more often seen in women, especially in post menopausal females. The aetiopathogenesis of MCN is still unknown. Ovarian-like stroma of the tumour indicates the tumour originates from the mullerian tissue of the kidney. Hormonal theory is suggestive as the stroma of the tumour is positive for estrogen and progesteron. Preoperative diagnosis of MCN is difficult. Surgical approach is recommended both for the histological diagnosis and the treatment of the disease. We here present a 53-year-old female patient with MCN admitted to the urology clinic with left flank pain treated successfully with partial nephrectomy. No recurrence was noted after six months of clinical and radiological follow-up.On cut section, multicystic white lesion with calcified areas was seen [Table/ Fig-1f]. The content of the cysts was serum-like and lucid. No macroscopic haemorrhage and necrosis was seen in the specimen. Microscopically, the specimen showed enlarged cystic structures. Cysts were lined with flattened epithelial cells arranged in a single row and had a hobnail appearance. They were surrounded by the cellular and fibroblastic stroma, histologically similar to ovarian stroma and the homogenous and eosinophilic areas resembling to corpus luteum. Enlarged tubules were surrounded by mature adipocytes and calcified areas. No blastemal cells or immature epithelial elements were seen. No epithelial cells with clear cytoplasm were seen. Both ovarian like stroma and corpus luteum like areas were positive for estrogen receptor (ER) and negative for progesterone receptor (PR). They were also positive for cytoceratine (CK19), epithelial membranous antigen (EMA) and CD15 and negative for CD10 [

Twenty children with non-Wilms renal tumors from a reference center in Central Anatolia, Turkey

“…Multicystic nephroma and cystic partially differentiated nephroblastoma are two histological subtypes [22][23][24]. These are nonheritable, benign lesions and are rare both in children and adults [23].…”

“…Most children present with a painless abdominal mass. The sonographic appearance of the multilocular cystic renal tumor includes multiple anechoic spaces traversed by thin septa and no solid elements [22][23][24]. No cases of aggressive behavior have been documented, but surgery is required for the diagnosis and differential diagnosis from cystic Wilms tumor, RCC, or mesoblastic nephroma [4].…”

Multicystic nephroma: a rare benign renal tumor with diagnostic predicament

“…5 MCN has many synonyms such as multilocular cystic renal tumor, cystic nephroma, multilocular benign cystic neoplasm, multilocular cyst and polycystic nephroblastoma. 3,6 Etiology of MCN is controversial and multiple theories proposed include Mullerian origin because of ovarian like stroma and thought to be a developmental lesion having malignant potential. 1,2,4 Scanning electron microscopy shows epithelial cells with short microvilli and long cilia resembling collecting duct epithelium, suggesting the origin from collecting duct.…”

“…The 2 nd peak occurs in adults in the age group of 40 to 60 years with female predominance with M:F=1:8. 1,3 MCN has a very good prognosis and over 200 cases have been reported in literature. 4 Its histology and etiopathogenesis is controversial but according to WHO it is categorised under Mixed Epithelial Stromal Tumors (MEST), although Renal Epithelial Stromal Tumors (REST) can be used to encompass both MCN and MEST.…”