Table 1.
Demographic details and clinical presentation in 10 children and adolescents with adrenocortical tumors initially mistaken as neuroblastoma.
| Patient | Presentation | Lab test | Imaging | Management | Finaldiagnosis | Outcome | |
|---|---|---|---|---|---|---|---|
| Tumor biopsy/resection | Consolidation of diagnosis | ||||||
| Female, 0.4 y | - Finding in surveillance for Beckwith–Wiedemann syndrome | - Blood: not done - Urine: catecholamines negative |
- Sonography: suprarenal mass 20 ml | - Tumor resection (R0) | Histology by reference pathologist | ACA | Alive |
| Male, 0.8 y | - Finding in surveillance for hemihypertrophy | - Not done | - Sonography: suprarenal mass 11 ml - mIBG: negative |
- Tumor resection (R0) | Histology by reference pathologist | ACA | Alive |
| Male, 1.0 y | - Finding in surveillance for hemihypertrophy | - Not done | - Sonography: suprarenal mass 3 ml | - Tumor resection (R0) | Histology by reference pathologist | ACA | Lost to follow-up |
| Male, 0.2 y | - Incidental finding in sonography | - Blood: normal levels of endocrine hormones - Urine: not done |
- Sonography: suprarenal mass 18 ml - mIBG: negative - Bone scan: negative |
- Tumor resection (R0) | Histology by reference pathologist | ACC | Death of disease |
| Male, 0.4 y | - Incidental finding in echocardiography - Perioral paleness |
- Not done | - Sonography: suprarenal mass 7 ml | - Tumor resection (R0) | Histology and reference pathology | ACA | Alive |
| Female, 8.3 y | - Abdominal and back pain for 2 months - Finding in diagnostic sonography |
- Blood: normal levels of endocrine hormones - Urine: not done |
- Sonography: suprarenal mass 168 ml | - Biopsy | Histology and reference pathology | ACC | Alive |
| Male, 3.2 y | - Fever - Painless abdominal mass - Hypertension |
- Blood: androgens, testosterone, estrogen, progesterone levels increased - Urine: not done |
- Sonography: abdominal mass 480 ml | - Biopsy | Histology and reference pathology | ACC | Alive |
| Female, 0.4 y | - Incidental clinical finding—virilization - Hypertrophy of clitoris - Hypertension - Sweating - Reduced performance - Tiredness - Weakness - Headache - Incidental clinical finding during presentation for another reason |
- Blood: androgens, testosterone, DHEAS, androstenedione, estrogen, estriol, estradiol, progesterone, 17-OHP, glucocorticoids, cortisol, renin, aldosterone levels increased - Urine: not done |
- Sonography: abdominal mass 304 ml | - Tumor resection - Intraoperative tumor spillage |
Histology and reference pathology | Adreno- cortical tumor of unknown dignity | Alive |
| Female, 8.4 y | - Symptomatic therapy of endocrine signs for one year by non-specialist—virilization - Premature pubarche - Hypertrophy of clitoris - Acne - Sweating - Smelling perspiration - Greasy hair - Vertigo - Nausea and vomiting - Symptomatic therapy of endocrine signs for one year by non-specialist |
- Blood: androgens, testosterone, DHEAS, progesterone, 17-OHP, glucocorticoids, cortisol levels increased - Urine: not done |
- Sonography: abdominal mass 293 ml | - Tumor resection - Intraoperative tumor spillage |
Histology by reference pathologist | ACC | Death of disease |
| Female, 15.8 y | - Patient from foreign country, no data available | No data | No data | - Tumor resection (R0) - 8 cycles of chemotherapy |
Histology by reference pathologist | ACC | Alive |
ACA, adrenocortical adenoma; ACC, adrenocortical carcinoma; DHEAS, dehydroepiandrosterone sulphate; mIBG, meta-iodobenzylguanidine; NBL, neuroblastoma; NSE, neuron-specific enolase; R0, microscopic complete tumor resection; y, years; 17-OHP, 17-hydroxyprogesterone.