PG Corner- Case 16

Written by | HISTOPATHOLOGY, PG CORNER

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Background

A 51-year-old hypertensive lady with recently diagnosed Diabetes mellitus, presented with complaints of weight gain and hair loss. CT whole abdomen showed a large soft tissue lesion in right supra renal region measuring 8 x 6cm. Serum cortisol: 39.26 mcg/dl. Surgical excision was performed.

Gross specimen

Fig. 16a; Gross 1: A circumscribed bright yellow to orange coloured tumor

Fig. 16b; Gross 2: Tumor with myxoid areas

Microscopy

Fig 16c; H&E; 4x

Fig 16d; H&E; 10x

Fig 16e; H&E; 20x

Fig 16f; H&E; 40x

Fig 16g; H&E; 10x

Click here to reveal the description

Immunohistochemistry

Fig 16h; CK

Fig 16i; Chromogranin A

Fig 16j; PAX-8

Fig. 16k; Melan-A

Fig 16l; Synaptophysin

Click here to reveal the IHC findings

Questions & Answers:

  1. What is your diagnosis?
    • Adrenal cortical adenoma
    • Adrenal cortical carcinoma –low grade
    • Adrenal cortical carcinoma –high grade
    • Pheochromocytoma
  2. Which of the following is/are associated with this tumor?
    • Li Fraumeni syndrome
    • Beckwith-Wiedemann syndrome
    • Congenital adrenal hyperplasia
    • All of the above
  3. Definitive criteria for malignancy in this tumor is:
    • Distant metastases
    • Presence of atypical mitotic figures
    • Necrosis
    • Vascular invasion
  4. Which of the following IHC marker is usually not expressed in this tumor?
    • Synaptophysin
    • Chromogranin A
    • Melan A
    • Inhibin A

Click here to reveal the answers

  • Malignant epithelial tumor of adrenal cortical cells.
  • Median age of presentation: Bimodal, with peaks in 1st  and 5th decade of life.
  • Female predilection
  • Mostly involves single adrenal gland
    • If bilateral adrenal enlargement is seen :  Think of something else
  • Clinical features:
    • Symptoms of steroid hormone excess
    • Abdominal pain and fullness
    • Incidental
    • Both androgen and estrogen production in an adrenal tumor:  suspicious for adrenocortical carcinoma
  • Modified Weiss criteria:
    • Score:  2x mitotic rate criterion  + 2x clear cytoplasm criterion + abnormal mitosis criterion + necrosis criterion + capsular invasion criterion
    • Score ≥3-  suggests malignancy
Morphological
Characteristics
Score
 Present
Absent
Mitosis. 5/50 hpf
1
0
Clear cells  ≤ 25%
1
0
Abnormal mitosis
1
0
Necrosis
1
0
Capsular invasion
1
0
  • Histological variants:
    • Oncocytic
    • Myxoid
    • Sarcomatoid
  • Differential diagnosis:
    • Adrenal cortical adenoma
    • Oncocytoma
    • Pheochromocytoma
    • Renal cell carcinoma(primary and metastatic)
    • Hepatocellular carcinoma(primary and metastatic)
    • Metastatic melanoma
  • Immunohistochemistry markers :  
    • Positive markers:  SF-1, Inhibin A , Melan A, Synaptophysin,  Calretinin
    • Negative markers: CK, EMA  & CEA,  Chromogranin A(usually negative)
  • Reticulin stain:  highlights loss of nested architecture
  • Genetic alterations: 
    • Overexpression of IGF2,  DLGAP5 & PINK1
    • TP53 mutation
    • WNT pathway defects (CTNNB1 mutation)
  • Associated hereditary syndromes:
    • Li-Fraumeni syndrome
    • Lynch syndrome
    • Multiple endocrine neoplasia type 1
    • Familial adenomatous polyposis
    • Carney complex
    • Beckwith-Wiedemann syndrome
    • Neurofibromatosis type 1
  • Treatment:
    • Surgical excision
    • Locoregional lymph node dissection for localized tumor
    • Radiation therapy for metastatic tumor
  • Adverse prognostic factors:
    • Hypercortisolism
    • Age>50 years
    • Ki67 index-high
    • Increased expression of SF-1

Contributed by: Dr. Sunil Pasricha

Compiled by: Dr. Vidya Menon


In case of queries, email us at: info.pathbliss@rgcirc.org

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Last modified: 17/08/2021

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