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Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias.

Yu HW, Cho WI, Chung HR, Choi KH, Yun S, Cho HS, Shin CH, Yang SW - Ann Pediatr Endocrinol Metab (2016)

Bottom Line: She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years.Her bone tumor biopsies were compatible with osteoblastoma.This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.

ABSTRACT
Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

No MeSH data available.


Related in: MedlinePlus

Computed tomography of the adrenal gland. Horizontal view of the abdomen revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (black arrow).
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Figure 1: Computed tomography of the adrenal gland. Horizontal view of the abdomen revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (black arrow).

Mentions: A 44-month-old girl was admitted who complained of excessive weight gain with growth failure and mood change. Her blood pressure was 150/80 mmHg, height was 96 cm (25th percentile), weighed 19.7 kg (>97th percentile) and her body mass index was 21.4 kg/m2 (>97th percentile). Plethoric moon face, hypertrichosis, and spotty pigmentation on her conjunctivae, lip and oral mucosa were observed. There was no specific medical history, including tumor or adrenal disease, in her family. Her urinary cortisol level was 966.2 µg/m2/24 hr (reference range [RR], <70 µg/m2/24 hr7)), and her serum cortisol level after a high dose dexamethasone suppression test was basal 34.6 µg/dL (RR, 5–23 µg/dL), peak 32.9 µg/dL (RR, <5 µg/dL). The 24-hour urine cortisol levels of six-day Liddle's dexamethasone suppression test revealed following values; 1,380.0; 1,357.2; 1,064.4; 897.0; 944.0; 755.6 µg/24 hr from day 1 to 6, respectively. The result was compatible to adrenal CS and no paradoxical increase was observed. Whereas her symptoms and signs of cortisol excess were very prominent, and her blood and urine cortisol levels were increased significantly, her computed tomography of the adrenal gland revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (Fig. 1). She underwent laparoscopic left adrenalectomy at the age of 47 months. Because her symptoms of excess cortisol and increased urinary cortisol levels remained after left adrenalectomy, right adrenalectomy was performed at 58 months. The pathology of both adrenal glands revealed adrenocortical micronodular hyperplasia without apparent pigmentation, which was more compatible with micronodular adrenocortical disease (MAD) than primary pigmented adrenocortical disease (PPNAD) (Fig. 2).


Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias.

Yu HW, Cho WI, Chung HR, Choi KH, Yun S, Cho HS, Shin CH, Yang SW - Ann Pediatr Endocrinol Metab (2016)

Computed tomography of the adrenal gland. Horizontal view of the abdomen revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (black arrow).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4835562&req=5

Figure 1: Computed tomography of the adrenal gland. Horizontal view of the abdomen revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (black arrow).
Mentions: A 44-month-old girl was admitted who complained of excessive weight gain with growth failure and mood change. Her blood pressure was 150/80 mmHg, height was 96 cm (25th percentile), weighed 19.7 kg (>97th percentile) and her body mass index was 21.4 kg/m2 (>97th percentile). Plethoric moon face, hypertrichosis, and spotty pigmentation on her conjunctivae, lip and oral mucosa were observed. There was no specific medical history, including tumor or adrenal disease, in her family. Her urinary cortisol level was 966.2 µg/m2/24 hr (reference range [RR], <70 µg/m2/24 hr7)), and her serum cortisol level after a high dose dexamethasone suppression test was basal 34.6 µg/dL (RR, 5–23 µg/dL), peak 32.9 µg/dL (RR, <5 µg/dL). The 24-hour urine cortisol levels of six-day Liddle's dexamethasone suppression test revealed following values; 1,380.0; 1,357.2; 1,064.4; 897.0; 944.0; 755.6 µg/24 hr from day 1 to 6, respectively. The result was compatible to adrenal CS and no paradoxical increase was observed. Whereas her symptoms and signs of cortisol excess were very prominent, and her blood and urine cortisol levels were increased significantly, her computed tomography of the adrenal gland revealed 5-mm nodular thickening of the left adrenal gland and a nearly normal right adrenal gland (Fig. 1). She underwent laparoscopic left adrenalectomy at the age of 47 months. Because her symptoms of excess cortisol and increased urinary cortisol levels remained after left adrenalectomy, right adrenalectomy was performed at 58 months. The pathology of both adrenal glands revealed adrenocortical micronodular hyperplasia without apparent pigmentation, which was more compatible with micronodular adrenocortical disease (MAD) than primary pigmented adrenocortical disease (PPNAD) (Fig. 2).

Bottom Line: She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years.Her bone tumor biopsies were compatible with osteoblastoma.This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.

ABSTRACT
Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases.

No MeSH data available.


Related in: MedlinePlus