Endocr Pathol (2014) 25:397–403 DOI 10.1007/s12022-014-9325-8
Adrenocortical Oncocytoma Presenting as Cushing’s Syndrome: An Additional Report of a Paediatric Case Bernardo Dias Pereira & Elisabete Silva Rios & Rafael Adame Cabrera & Jorge Portugal & Luísa Raimundo
Published online: 31 July 2014 # Springer Science+Business Media New York 2014
Abstract Oncocytomas are tumours predominantly or exclusively composed of oncocytes, cells with granular and eosinophilic cytoplasm filled with mitochondria. Although they can occur in every organ, they are rare in adrenal glands, and in paediatric patients they are even rarer, with only three case reports previously published. We present a preschool child developing Cushing’s syndrome due to an adrenocortical oncocytoma, which was confirmed immunohistochemically with antibodies to the mitochondrial electron complex 2. A 5.8-year-old girl presented with clinical features of Cushing’s syndrome. ACTH-independent hypercortisolism was confirmed biochemically and a left adrenal mass was detected by imaging and removed by laparotomy. Histopathological analysis revealed a tumour composed of more than 95 % of oncocytes, confirmed immunohistochemically with antibodies to subunits A and B of the mitochondrial enzyme succinate dehydrogenase. Using the Lin–Weiss–Bisceglia score system and the reticulin algorithm, this tumour was categorized as a benign adrenocortical oncocytoma. The patient currently has 64 months of follow-up, without any evidence of relapse of B. D. Pereira (*) : J. Portugal : L. Raimundo Serviço de Endocrinologia e Diabetes, Hospital Garcia de Orta, E.P.E., Avenida Torrado da Silva, 2801-951 Almada-Setúbal, Portugal e-mail: [email protected] E. S. Rios Serviço de Anatomia Patológica, Centro Hospitalar São João, E.P.E., Alameda Prof. Hernani Monteiro, 4440-563 Porto, Portugal E. S. Rios Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), Rua Dr. Roberto Frias, 4200-465 Porto, Portugal R. A. Cabrera Serviço de Anatomia Patológica, Instituto Português de Oncologia de Lisboa, Francisco Gentil, E.P.E., Rua Prof. Lima Basto, 1099-023 Lisbon, Portugal
symptoms. To our knowledge, we herein present the youngest patient developing an adrenocortical oncocytoma and the first manifestation of Cushing’s syndrome due to this rare neoplasm in paediatric patients. We also emphasize the clinical usefulness of immunohistochemistry to the mitochondrial enzyme succinate dehydrogenase to confirm the oxyphilic nature of adrenocortical oncocytomas. Keywords Adrenal gland . Oncocytoma . Cushing’s syndrome . Preschool child . Succinate dehydrogenase
Introduction Oncocytomas are neoplasms predominantly or exclusively composed of oncocytes, histologically recognized as epithelial cells with granular eosinophilic cytoplasm confirmed to be rich in mitochondria with immunohistochemistry (IHC) and/ or electron microscopy. It has been reported that they can develop in every organ, most commonly the kidney, the thyroid and the salivary glands [1]. In the adrenal glands, they are exceedingly rare, occurring mainly in adults [2]. So far, there have been only three published cases of adrenocortical oncocytoma (AO) in paediatric age [3–5]. Herein, we present the clinical and pathological findings of an AO occurring in a preschool child with Cushing’s syndrome. Clinical Case A 5.8-year-old (yo) girl was referred to our department due to suspected precocious puberty. She had a 2 months history of sudden onset weight gain, breast development and pubic hair. Her past medical history and family history were unremarkable.
398
The physical examination revealed diastolic hypertension (106/73 mmHg, centile 95), obesity (BMI 20.7 Kg/m2, +2.84 SD) and decreasing height velocity (−0.76 SD at 4.3 years old; −4.1 SD at 5.3 years old—Fig. 1). She had facial fullness and
Endocr Pathol (2014) 25:397–403
plethora (Fig. 2a). Tanner stages for breast and pubic hair development were 2 and 4, respectively. The hormonal evaluation revealed a fourfold to fivefold increase in urinary free cortisol in two instances (668.7 and
Fig. 1 Typical auxologic profile of paediatric Cushing’s syndrome: Increasing weight gain (inferior curve) with flattening of height curve (superior curve)
Endocr Pathol (2014) 25:397–403
399
Fig. 2 Facial fullness and plethora at the first paediatric endocrinology consultation (a) and 1 year postoperatively (b)
967.7 nmol/day, reference 27.6–165.6), failure of suppression of cortisol levels on overnight low dose dexamethasone test (659.9 nmol/L; normal,
Adrenocortical Oncocytoma Presenting as Cushing’s Syndrome: An Additional Report of a Paediatric Case Bernardo Dias Pereira & Elisabete Silva Rios & Rafael Adame Cabrera & Jorge Portugal & Luísa Raimundo
Published online: 31 July 2014 # Springer Science+Business Media New York 2014
Abstract Oncocytomas are tumours predominantly or exclusively composed of oncocytes, cells with granular and eosinophilic cytoplasm filled with mitochondria. Although they can occur in every organ, they are rare in adrenal glands, and in paediatric patients they are even rarer, with only three case reports previously published. We present a preschool child developing Cushing’s syndrome due to an adrenocortical oncocytoma, which was confirmed immunohistochemically with antibodies to the mitochondrial electron complex 2. A 5.8-year-old girl presented with clinical features of Cushing’s syndrome. ACTH-independent hypercortisolism was confirmed biochemically and a left adrenal mass was detected by imaging and removed by laparotomy. Histopathological analysis revealed a tumour composed of more than 95 % of oncocytes, confirmed immunohistochemically with antibodies to subunits A and B of the mitochondrial enzyme succinate dehydrogenase. Using the Lin–Weiss–Bisceglia score system and the reticulin algorithm, this tumour was categorized as a benign adrenocortical oncocytoma. The patient currently has 64 months of follow-up, without any evidence of relapse of B. D. Pereira (*) : J. Portugal : L. Raimundo Serviço de Endocrinologia e Diabetes, Hospital Garcia de Orta, E.P.E., Avenida Torrado da Silva, 2801-951 Almada-Setúbal, Portugal e-mail: [email protected] E. S. Rios Serviço de Anatomia Patológica, Centro Hospitalar São João, E.P.E., Alameda Prof. Hernani Monteiro, 4440-563 Porto, Portugal E. S. Rios Instituto de Patologia e Imunologia Molecular da Universidade do Porto (IPATIMUP), Rua Dr. Roberto Frias, 4200-465 Porto, Portugal R. A. Cabrera Serviço de Anatomia Patológica, Instituto Português de Oncologia de Lisboa, Francisco Gentil, E.P.E., Rua Prof. Lima Basto, 1099-023 Lisbon, Portugal
symptoms. To our knowledge, we herein present the youngest patient developing an adrenocortical oncocytoma and the first manifestation of Cushing’s syndrome due to this rare neoplasm in paediatric patients. We also emphasize the clinical usefulness of immunohistochemistry to the mitochondrial enzyme succinate dehydrogenase to confirm the oxyphilic nature of adrenocortical oncocytomas. Keywords Adrenal gland . Oncocytoma . Cushing’s syndrome . Preschool child . Succinate dehydrogenase
Introduction Oncocytomas are neoplasms predominantly or exclusively composed of oncocytes, histologically recognized as epithelial cells with granular eosinophilic cytoplasm confirmed to be rich in mitochondria with immunohistochemistry (IHC) and/ or electron microscopy. It has been reported that they can develop in every organ, most commonly the kidney, the thyroid and the salivary glands [1]. In the adrenal glands, they are exceedingly rare, occurring mainly in adults [2]. So far, there have been only three published cases of adrenocortical oncocytoma (AO) in paediatric age [3–5]. Herein, we present the clinical and pathological findings of an AO occurring in a preschool child with Cushing’s syndrome. Clinical Case A 5.8-year-old (yo) girl was referred to our department due to suspected precocious puberty. She had a 2 months history of sudden onset weight gain, breast development and pubic hair. Her past medical history and family history were unremarkable.
398
The physical examination revealed diastolic hypertension (106/73 mmHg, centile 95), obesity (BMI 20.7 Kg/m2, +2.84 SD) and decreasing height velocity (−0.76 SD at 4.3 years old; −4.1 SD at 5.3 years old—Fig. 1). She had facial fullness and
Endocr Pathol (2014) 25:397–403
plethora (Fig. 2a). Tanner stages for breast and pubic hair development were 2 and 4, respectively. The hormonal evaluation revealed a fourfold to fivefold increase in urinary free cortisol in two instances (668.7 and
Fig. 1 Typical auxologic profile of paediatric Cushing’s syndrome: Increasing weight gain (inferior curve) with flattening of height curve (superior curve)
Endocr Pathol (2014) 25:397–403
399
Fig. 2 Facial fullness and plethora at the first paediatric endocrinology consultation (a) and 1 year postoperatively (b)
967.7 nmol/day, reference 27.6–165.6), failure of suppression of cortisol levels on overnight low dose dexamethasone test (659.9 nmol/L; normal,
