QUESTION-i want ask for you help concerning a case of adrenocortical carcinoma in a 4 years old girls who's hospitalized actually in our hospital . This is the case

Patient B D.She is  4 years and 9 months, and does not have any  particular history of personal or familiar pathology.
• A precocious puberty consisting of a development of pubic hair, and no mammalian or   axillary hair developpement  (A1 S1 P3), with  clitoromegaly •  associated with abdominal pain
• the diagnosis of an adrenal mass measuring 80 *  72 mm on MRI (mass with clear limits, regular contours, arriving in contact with the bowel loops, sheathing the vein and renal artery) without lymphadenopathy
Laboratory parameters  revealed: 18/05/2014 (the patient was 3 years and 9 months)

  • DHEAS:> 1500 microgr / dl (8.5-317.3)
    • Testosterone: 6.50ng / ml
    • 17 OH P: 9.60ng / ml (0070-1530)
    • FSH: 2.2 mui / ml
    • LH: 0.19 mui / ml
    • E2: 31pg / ml
    • The serum potassium, serum sodium and blood pressure were normal
  • cortisol before surgery   is Unknown. The measurement of metanephrines before surgery was not done.The patient has undergone a surgery in June 4/ 2014
  • total mass excision (surgery was without complications) has been done
    The pathologycal result was :
    • adrenocortical carcinoma  with a weiss’s score 6
    • Immunohistochemistry: positive for synaptophysin   ,   negative for chromogranin
    • Ki: 35 p100
    • P53: not done since not available

    After the patient has undergone chemotherapy with carboplatin and etoposide (4 cure with a 21-day interval) between June 2014 and October 2014

    At its orientation to us  (20/01/2015):
    The patient presented:
    • A TANNER stage  S1 A1 P3
    • A weight: 19 kg (+ 1DS)
    • Height: 107 cm (+0.5 DS)
    • A 4 cm growth rate during the last 6 months
    • The rest of the clinical exam was unremarkable
    • DHEAS: 19.63micrg / dL (32.7-276)
    • Testosterone: 0.34ng / ml (0-0.9)
    • 17OHP: 0.7ng / ml (inf à0.4)
    • FT3: 3 pmol/l (2.62-5.7), FT4: 17.6pmol / l (9-19), TSH: 1.39 mIU / ml (0.35 to 4.94)
    • FSH: 7.92mUI / l (standard: 0.2-3.3)
    • LH: 2.70m IU / l (standard: 0.23-1.8)
    • Estradiol: 42pg / ml (inf to 5 pg / ml)
    • Test at 0.1mg Decapeptyl:

  FSH (mUI/ml) LH (mUI/ml) E2 (pg/ml)
base 7.1   42
T+3hours 26.73   49
T+24hours 15.01 6.13 144

We found a progressive central precocious puberty
Radiological assessment:
Abdominal pelvic CT:
• No signs of tumor recurrence
• left adrenal lodge is empty
• right  adrenal is without abnormalities

Hypothalamic pituitary MRI:IS • normal
PET SCAN:  two or three hypermetabolic pathological  lesions with little intensity at the Fuller parieto coloc (SUV MAX = 2.4)
No pathological  hypermetabolic lesion in the adrenal lodges
one hypermetabolic lesion  at the  right gluteal muscle  (SUV max = 3.2)
No other pathological lesion, apart from a homogeneous fixation in  the liver and spleen

so , a lot of questions are persisting concerning this patient. please if you can help us answering these questions for a best caring
This puberty, it is secondary to:
• persistent adrenocortical carcinoma
• Or to progressive central precocious puberty
• Or another origin

This adrenocortical carcinoma:
• Is it scalable, given the results  of  PET SCAN ,  normal biology
• is there  indication for further investigations to confirm  the evolutionary character of this adrenocortical carcinoma
• Are there indications for therapeutic  mitotane in this child. Thank you very much.
Nadjib Kaoauche , fellow in training - -universitary hospital of constantine
RESPONSE- What is the bone age of your patient? If far advanced, she could have central precocious puberty.
From the blood tests and the PET scans it seems that the tumor has recurred.  You could add mitotane in her treatment. Best regards!. GP Chrousos, MD