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. |