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Table 1 Details of previously reported MCMA carcinoma along with a current case in the medical literature

From: Mixed cortico-medullary adrenal carcinoma in children: looks are deceptive!!

Year Age Sex Clinical features Biochemical Urinary findings Cytology Gross Histopathology Prognosis/survival
2012 78 F Dizziness
Hypertension
Elevated serum DHEA 571 mg/dL (N < 200) Elevated urine metanephrines 457 μg/24 h (N 24–96 μg/24 h) Not done Not available IHC staining for chromogranin A, synaptophysin, Melan-A and inhibin positive.
S-100 - sustentacular cells.
121 days follow-up was uneventful later developed 8.5 cm sized mass adjacent to the adrenalectomy site.
Liver mass measuring 1.7 cm +.
2013 63 M Mass effect
Abdominal pain
Weight loss
Morning cortisol, 661 nmol/L (n = 171–536)
NSE (ng/mL), 20.33 (< 12.5)
Chromogranin A, 21 ng/mL (n = 2–18)
Morning cortisol after 1 mg overnight dexamethasone suppression test, 100 nmol/L (n < 50)
Urine catecholamines, 150 g/24 h (10–280) Not done Not available The neoplastic cells were positive for chromogranin A, synaptophysin, NSE, vimentin and S-100 and were focally positive for calretinin and CKAE1/AE3 Radiology and laboratory follow-up in 1 year. Then, the patient died 18 months post-surgery—respiratory failure due to diffuse pulmonary metastases.
2016 50 M Mass effect
Abdominal pain
Weight loss
Morning cortisol, 24.34 μg/dL (n = 6.2–19.4). Urine free cortisol, 151.28 μg/24 h (n = 36–137)
ACTH < 1.00 pg/mL (n = 7.2–63.3).
VMA in 24 h urine, 4.3 mg/24 h (n = 1.9–9.8).
Not done Well-defined encapsulated, solid with a little fat weight 2100 g and measured 22 cm Undifferentiated high-grade carcinoma.
Tumour necrosis, multinucleated and bizarre cells+.
Mitoses 50/hpf.
Focal vascular invasion.
No capsular invasion.
IHC - Inhibin, Calretinin, Chromogranin A and S-100 markers are positive.
Melan-A—negative.
18 months after the operation, the patient developed liver metastasis followed by lung metastasis.
Present case (2021 ) 2 F Abdominal pain
Weight loss
Morning cortisol, 35.2 μg/dL Urinary VMA 2.76 mg/24 h (N < 2.20 mg/24 h), urinary ketones positive Dual population of cells Specimen weighed 280 g and measures 14 × 10 × 9 cm
Solid
Haemorrhage and necrosis+
Malignant tumour with geographical areas of necrosis with the presence of viable tumour in between. Died in post-operative period due to acute respiratory distress.