A 14- month old male infant presents with an enlarging abdominal mass.

[bharat]

A 14- month old male infant presents with an enlarging abdominal mass.
Laboratory examination revels increased urinary leval of metanephrine and vanillymandelic acid.
A histological section from the mass revels a tumor composed of small,primitive appearing cells with the hyperchromatic nuclei and little or no cytoplasm .
Occasional focal groups of tumor cells are arranged in a ring around the central space. What is the correct diagnosis of this tumor?
a. Adrenal cortical carcinoma.
b. Ganglioneuroma.
c. Nephroblastoma.
d. Neuroblastoma.
e. Pheochromocytoma.

[Oak]

although patient present with the abdominal mass in the Adrenal cortical carcinoma but there is no elevation of urinary metanephrine and vanillymandelic acid so its excluded.

Others do not correlate with the clinical finidngs

Clinical presentation of abdominal mass and the histological finding inidates that the child is suffering from the Neuroblastoma but biochemical finding seems controversial and indicates towards pheochromocytoma.

Because urinary metanephrine and vanillymandelic acid are elevated in the pheochromocytoma but in Neuroblastoma its homovanillic acid (HVA) and/or vanillylmandelic acid (VMA).

If I go with the biochemical findings I would go for Pheochromocytoma but there is no BP symtops and If I have to go throgh clinical presentation and the histological finding eventhough biochemcial findings seems controviersial I would go for Neuroblastoma.

[bharat]

yep. correct answer is D. Neruroblastoma. Because they are malignant tumor of the adrenal medulla that occur in very young patients who presents with ababdominal mass.
Histologically , these tumors are composed of small cells forming Homer-Wright rosettes which are group of cells aranged in a ring around a central mass of pink neural filaments. EM shows neurosecratery granules with in the cytoplasm of tumor mass, while immunohistological stains are positive for neuron specific enolase.
These highly aggresive tumor are unique ,some spontaneously regress and some dediffrentiate in to benign tumor such as ganglioneuromas.
Three distinct chromosome abnormality is associated with this disease as:-
near terminal delation of short part of chromosome no 1, Homogenously staining resion of chromosome no 2 & multiple double minute chromosome bodies which are related with N-myc oncogene.(no. of N-myc determinr the aggresiveness of this disease)