RESUMEN
BACKGROUND: Metanephric adenoma (MA) is a rare benign renal tumor that resembles renal cell carcinoma and Wilms' tumor in radiological as well as pathological appearance. It can present at any age or gender, and it is extremely rare in the pediatric age group with less than 50 reported cases. Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder of carbohydrate metabolism. Herein, we report a rare incidence of MA in a boy with a genetically confirmed FBS who underwent a nephron-sparing surgery. CASE PRESENTATION: A 21-month-old boy was referred to the pediatric urology clinic for further evaluation of an incidentally discovered left renal mass. His laboratory investigations showed normal renal function, hypophosphatemia, high blood glucose level, markedly elevated serum alkaline phosphatase, and low serum vitamin D. Blood picture showed signs of polycythemia and urinalysis showed glucosuria and aminoaciduria. Genetic testing was positive for Fanconi-Bickel syndrome. Radiological investigations were carried out with abdominal ultrasound and computerized tomography (CT) with intravenous contrast documented a sharply marginated peripheral hypoechoic hypovascular homogeneously enhancing mass at the upper pole of the left kidney measuring 2.0 × 1.8 × 2.0 cm. The child was admitted and started on supportive treatment until his medical condition was stabilized, then underwent elective open left partial nephrectomy via a left upper transverse abdominal transperitoneal incision. The excised renal mass was sent for histopathological assessment and was found to be a tumor composed of tightly packed tubules with no mitotic figures or necrosis and scanty cytoplasm consistent with MA. After good hydration and tumor resection, his polycythemia gradually improved. The patient was discharged home in a good condition with his proper replacement therapies. His follow-up abdominal ultrasound after 12 months showed no signs of recurrence. CONCLUSIONS: Metanephric adenoma is extremely rare in the pediatric age group, especially in those who have a FBS. The only way to diagnose and treat this tumor is by surgical resection as most patients are asymptomatic. A nephron-sparing surgery is better for this age group in which the future renal function is considered.
RESUMEN
The wild Prunus korshinskyi has a restricted distribution in small scattered areas of Jabal Al-Lauz (2580â¯m above sea level), a mountain in northwestern Saudi Arabia. Major objective of current study is to provide information about P. korshinskyi by analyzing its ecology in terms of floristic diversity, plant associations, environmental features, and its size structure and nutritional value. For this purpose, 12 stands of 20â¯m2 were selected along the Jabal Al-Lauz mountain ridge. Three plant associations and their environmental variables were identified and characterized after application of a two-way indicator species analysis (TWINSPAN), detrended correspondence analysis (DCA). And canonical correspondence analysis (CCA) as follows: VGI: P. korshinskyi-Astracantha echinus, VGII: Artemisia siberi-P. korshinskyi, and VGIII: Retama raetam-Artemisia sieberi. The edaphic factor affecting the distribution of the associations were pH, potassium and manganese content. The size class frequency distribution of P. korshinskyi shows a J-shape in spring and fall for the whole population; there was no regeneration via seed. Nutritional evaluation showed the fruits had a higher content of total carbohydrate, fats, crude protein, phosphorus, and potassium, and lower content of ash, Fe, Ca, Mn, Mg, and Zn than that in stems and leaves. This ecological knowledge gained through this study would be beneficial for managing and conserving of P. korshinskyi in a Jabal Al-Lauz area with its distinct and unique vegetation.