A 12-year-old male patient with a long history of delayed psychomotor development and generalized tonic-clonic seizures treated with phenytoin and carbamazepine.
He was admitted to the Institute with a history of three weeks of macroscopic hematuria with clots and abdominal pain predominantly in the hypogastrium.
A heterogeneous mass of 80 x 64 x 56 mm was observed in the computerized axial tomography of the abdomen and pelvis, which compromised the upper two thirds of the left kidney.
Imaging studies were completed with a normal chest X-ray and a brain tomography in which hypodense-subcortical alterations associated with multiple thick periventricular calcifications were observed.
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Physical examination revealed an awake patient who did not obey orders, with severe impairment of upper brain functions without motor deficit.
Eye fundus showed no evidence of congenital lesions.
At the level of the nose, folds in the non-pathogen and meningeal folds showed multiple erythematous papules of up to 2 mm. A palpable mass was found throughout the left hemiabdomen
A diagnosis of left renal angiomyolipoma associated with tuberous sclerosis was proposed.
He underwent exploratory laparotomy with left radical nephrectomy and paraaortic lymphadenectomy.
A blade tumor of approximately 10 cm was found, located in the upper half of the left kidney. The regional ganglia did not show any sign of disease involvement.
She was discharged three days later.
The pathology reported 250 gr. operative area of 11.0 7.4 x 4.2. The cut of the parenchyma was found to be characterized 90% by tumor formation, location x nephrotic segment x 2.3 cm.
The ureter measured 4.6 x 0.8 cm. without significant macroscopic changes.
Surgical margins were free of neoplasia.
Paraaortic lymph nodes with congestive aspect.
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Microscopically, an in-filtrative tumoral lesion of irregular edges was observed, very cellular consisting mainly of polygonal cells of aspect
epithelioid, most with clear cytoplasm, intermingled with granular, eosinophilic cytoplasm cells and other multinucleate cells similar to ganglion cells.
These cells showed nuclear atypia, prominent vesicles, vesicles, vesicles and mitosis (0-5 mitosis / 10 CAP), as well as areas of necrosis and hemorrhage.
No vascular or perineural invasion was observed.
Ureter, perirenal fat and surgical margins were free of neoplasia.
With this histomorphology the diagnosis of epithelioid angiomyolipoma was proposed, which was confirmed with immunohistochemical tests, whose result is presented in Table I.
