App.Genital Mâle

cas 0808401

Liens et agrandissements sur : images et texte en bleu. Links - Zoom: pictures and highlighted text.
  Tumeur testiculaire, enfant de 5 ans, marqueurs tumoraux négatifs,   Testicular tumor, 5 yo. Serologic tumour markers negative.  
  Orchidectomie, aprés examen extemporané en faveur d'une tumeur maligne faiblement différenciée.   Frozen section: Poorly differenciated malignant tumour. Orchidectomy  
     
     
     
     
     
       
     
  CK (AE1-AE3)   EMA  
     
  Desmine   Desmin  
 

Diagnostic proposé

 

 

 

 

 

 

 

 

Metastase d'un néphroblastome.

 

Proposed diagnosis

 

 

 

 

 

 

 

 

Nephroblastoma, metastatic.

 
 

Arguments

Le chirurgien a "oublié" de préciser à l'íntervention, l'antériorité d'un nephroblastome traité il y a 2 ans. Cependant il a communiqué le résultat des sérologies de tumeur testiculaire: négatif.

Le type histologique du nephroblastome primitif (Non favorable?) n'est pas disponible.

Liens dans le texte (en bleu) ci-dessous

 

 

The surgeon ommited to mention that the boy was treated for a nephroblastoma 2 years previously. He stated that serology for testicular tumors was negative.

Histological typing of the primary nephroblastoma (non favorable histology?) is not available.

Links to images in the highlighted text below

 

Case report article (Click below):

Wilms’ tumour metastasis to the testis

     

Microscopy of Wilm's tumor(in Ackerman's Surgicla Pathology 7ed.)

     

.....Microscopically, three major components are identified: undifferentiated blastema, mesenchymal (stromal) tissue, and epithelial tissue.Most Wilms' tumors show a representation of all three components, but the proportions vary widely. Some tumors are biphasic, and still others are monophasic (monomorphous). The blastematous areas are extremely cellular and composed of small round-to-oval primitive cells; the cytoplasm is usually very scanty, but sometimes it exhibits an oncocytoid appearance. The pattern of growth may be diffuse, nodular, cord-like (serpentine), or basaloid (with peripheral palisading). Wilms' tumors in which the blastematous component predominates can be confused with any of the small round cell tumors, including neuroblastoma. The mesenchymal elements usually have a spindle-cell fibroblast-like configuration but may also exhibit differentiation toward various cell types, particularly smooth muscle and skeletal muscle. Sometimes this mesenchymal component predominates almost to the exclusion of others. Wilms' tumors with an extensive skeletal muscle component are invariably seen in young children and are bilateral in over half of the cases. Predominantly rhabdomyosarcomatous Wilms' tumors involving the renal pelvis acquire morphologic features very similar to those of botryoid rhabdomyosarcoma. In some instances, these largely mesenchymal neoplasms are seen in the opposite kidney of patients with typical Wilms' tumor.

The epithelial component is characterized by the formation of embryonic tubular (and sometimes glomerular) structures that closely recapitulate the appearance of normal developing metanephric tubules (and glomeruli) at the light microscopic, ultrastructural, and lectin histochemistry levels. The differentiation can be so pronounced that tumor analogs of nearly all segments of the normal nephron can be formed. These tubular structures can be small and round, thus simulating the rosettes of neuroblastoma. Features favoring tubules over rosettes are the presence of a lumen, single cell layer, distinct basal lamina, and surrounding fibromyxoid stroma. The differential diagnosis of predominantly epithelial Wilms' tumors also includes multicystic nephroma and renal cell carcinoma. Exceptionally, marked hydropic changes are seen in the tubular epithelium...

The blastematous elements show only focal positivity for vimentin; the epithelial elements react for keratin, EMA, various lectins, and the various components of the basement membrane; the mesenchymal elements show a reactivity pattern consonant with their morphologic appearance (such as positivity for myoglobin and desmin in rhabdomyoblastomatous foci); and the neural elements—when present—exhibit reactivity for neuron-specific enolase, glial fibrillary acidic protein, and S-100 protein....