Adrenocortical Carcinoma

ADRENOCORTICAL CARCINOMA


ADRENO-CORTICAL CARCINOMA

  • Adrenocortical carcinoma is a rare tumor.
  • Most cortical carcinoma secreting one of the adrenocortical hormones excessive (cortisol).
  • Invades locally and distant sites
  • >50%- functional

ETIOLOGY-

  • Cause is unknown
  •  Li–Fraumeni syndrome, caused by an inherited inactivation mutation in TP53.

MORPHOLOGICAL FEATURES-

  • Adrenocortical carcinoma is well demarcated
  • It is predominantly yellow mixed with intermixed areas of hemorrhage, necrosis and calcification.

CLINICAL FEATURES-

  • Seen in 4th – 5th decade
  • Slightly more in females
  • Abdominal fullness, back pain, anorexia, features of metastases
  • Size- 9- 12 cm (<5cm)
  • Weight- 40- 60 gm
  • Most commonly Cushing syndrome is seen > virilisation (mostly occurs in hormone producing tumours)

INVESTIGATIONS-

  • CECT- IOC

TREATMENT-

  • Radical open surgery
  • Drugs- ketoconazole, aminoglutethimide, metyrapone
  • Mitotane is the most common chemotherapeutic agent in the traetment of ACC.

Exam Important

  • Adrenocortical carcinoma is a rare tumor.
  • Most cortical carcinoma secreting one of the adrenocortical hormones excessive (cortisol).
  • Invades locally and distant sites
  • >50%- functional

ETIOLOGY-

  • Cause is unknown
  •  Li–Fraumeni syndrome, caused by an inherited inactivation mutation in TP53.

CLINICAL FEATURES-

  • Seen in 4th – 5th decade
  • Slightly more in females
  • Abdominal fullness, back pain, anorexia, features of metastases
  • Size- 9- 12 cm (<5cm)
  • Weight- 40- 60 gm
  • Most commonly Cushing syndrome is seen > virilisation (mostly occurs in hormone producing tumours)

TREATMENT-

  • Radical open surgery
  • Drugs- ketoconazole, aminoglutethimide, metyrapone
  • Mitotane is the most common chemotherapeutic agent in the traetment of ACC.
Don’t Forget to Solve all the previous Year Question asked on ADRENOCORTICAL CARCINOMA

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