Adrenal cortical adenoma/carcinoma

Clinical features

  • The adrenal cortical cell is part of endocrine epithelia and has the capacity of hormone production: steroids, glucocorticoids and mineralocorticoids.
    • Adenomas-rarely functional- incidental detection more frequent
    • Carcinomas- 50% are hormone secreting - Cushing syndrome or virilisation of women patients (androgen secretion).
  • 0.2% of all paediatric malignancies.
  • Presentation and prognosis differ from those of adults
  • More common at ages of 0-5 years, but presents bimodal occurrence: In infancy and in adolescence
  • Abdominal mass with pain and fever
  • Associations with Li-Fraumeni, Beckwith-Wiedemann, Ver-Morrison, cancer family syndrome, SBLA (S- sarcoma; B- breast and brain neoplasm; L- leukaemia, laryngeal and lung carcinoma; A- adrenal cortical carcinoma) and foetal alcohol syndrome
  • Associations with genitourinary anomalies, neurofibromatosis, pigmented nevi, brain tumours and hemi hypertrophy
  • Associations with endocrine abnormalities (virilisation and Cushing’s syndrome) is common in children, although feminization and Conn’s syndrome are rare

 

Fig 97 - Adrenal cortical adenoma/ carcinoma – Fine needle aspiration from an adrenal adenoma. Cellular smear with discohesive neoplastic cells with bland nuclei (H&E)
Fig 98 - Adrenal cortical adenoma/ carcinoma – Fine needle aspiration from an adrenal adenoma. Neoplastic groups of cells with, eosinophilic cytoplasm with hyaline globules (H&E)
Fig 99 - Adrenal cortical adenoma/ carcinoma – Fine needle aspiration from an adrenal carcinoma - pleomorphic population of neoplastic cells with eccentric nuclei with irregular contour. Numerous multinucleated neoplastic cells with nuclear pseudoinclusions, coarse chromatin and evident nucleoli (H&E)
Fig 100 - Adrenal cortical adenoma/ carcinoma – Fine needle aspiration from an adrenal carcinoma. Multinucleate neoplastic cells are evident (Giemsa)

 

 

  • Cellular smears
  • Lipidic background-Giemsa stains
  • Nude nuclei (single or in morular pattern)
  • Sheets and discohesive neoplastic cells separated by delicate vessels
  • Monomorphic cells with bland nuclei and pleomorphic cells, with irregular outline, coarse chromatin and evident nucleoli
  • Neoplastic cells are polygonal or plasmacytoid
  • Pleomorphism can be exuberant- not associated with malignancy
  • Nucleoli can be prominent
  • Eccentric nuclei
  • Nuclear pseudo inclusions
  • Cytoplasm ranging from eosinophilic (more frequent in carcinomas) to clear and vacuolated (more frequent in adenomas)
  • Cytoplasmic hyaline globules
  • Mitoses are variable
  • Sarcomatoid appearance generally seen in poorly differentiated carcinomas
  • There are no cytological criteria for distinguishing benign from malignant cases, although a bubbly lipid background has been reported by some authors to correlate with benign nature(7)

 

Immunocytochemistry

 

  • D11: positive (nuclear) in 80%
  • Ad4BP (nuclear adrenal 4 binding protein): positive (100% in adrenal cortical carcinomas)
  • A103 (melan A): positive (100% in adrenal cortical carcinomas )
  • Cytokeratin: decreased expression relative to the degree of malignancy
  • Vimentin: increased expression relative to the degree of malignancy
  • Inhibin A: positive
  •  NSE: positive
  •  Synaptophysin: positive
  • Chromogranin: negative
  • CAM 5.2:positive
  • S100 protein: positive
  • Hep Par1: positive
  • CEA: negative
  • EMA: negative
  • CD10: negative
  • CK7: negative
  • CK20: negative
  • HMB45:negative
  • Bcl-2: positive

 

Differential diagnosis

 

  • Renal cell carcinoma
    • More discohesive cellular smears
    • Well-defined polygonal cells with centrally placed nuclei
    • Prominent nucleoli
    • Pleomorphism, when seen, is more uniform in the cell population
    • Low molecular weight cytokeratin: positive
    • EMA: positive
    • CD10:positive
    • D11: positive (occasionally)
    • Ad4BP: negative
    • A103 (melan A): negative
    • Inhibin A: negative
    • Neuroendocrine markers: negative
    • CEA: negative
  • Hepatocellular carcinoma
    • Low molecular weight cytokeratin: positive
    • D11: positive in 100%
    • Ad4BP: negative
    • A103 (melan A): negative
    • Inhibin A: negative
    • Neuroendocrine markers: negative
  • Pheochromocytoma
    • Chromogranin: positive
    • Bcl-2: negative
    • Inhibin A: negative
    • EMA: negative

 

Main points

  • Functional tumours do not carry worse prognosis, unlike in adults
  • A poor outcome is more common in the adolescent age group
  • Survival, in malignant cases, depends on the stage and the completeness of surgical resection
  • Children with Beckwith-Wiedemann Syndrome have better prognosis (6) There are no cytological criteria for distinguishing benign from malignant cases, although a bubbly lipid background has been correlated by some authors with benign nature
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