Ann Pediatr Endocrinol Metab.  2016 Mar;21(1):47-50. 10.6065/apem.2016.21.1.47.

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

• KMID: 2161307
• DOI: http://doi.org/10.6065/apem.2016.21.1.47

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.

Keyword

Cushing syndrome; Micronodular adrenal disease; Osteoblastoma

MeSH Terms

Biopsy
Child*
Child, Preschool
Conjunctiva
Cushing Syndrome*
Female
Femur
Follow-Up Studies
Humans
Hyperplasia*
Lip
Mass Screening
Mouth Mucosa
Osteoblastoma*
Pelvis
Pigmentation
Rare Diseases
Skull Base

Figure

• 

  Fig. 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).

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  Fig. 2 Pathology of both adrenal glands. (A) The right adrenal gland measured 4 cm×3 cm×0.7 cm, and the left adrenal gland measured 5.5 cm×3.2 cm×1.5 cm. The adrenal glands are enlarged and have multiple micronodules on the cut surface (white arrows). Microscopically, circumscribed small nodules are observed in the adrenal cortex, and the nodule diameter usually does not exceed 0.5 cm. No pigment granules are observed. H&E staining (B: ×40; C: ×100).

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  Fig. 3 Magnetic resonance imaging of brain and hip. (A) Horizontal view of the brain demonstrates multiple multilocular cystic bone lesions with internal hemorrhage and prominent wall enhancements involving the clivus, bifrontal skull base, bilateral sphenoid and left temporal bones (white arrows). (B) Coronal view of the hip with section at the femur head level revealed overall polyostotic bone tumors involving bilateral femurs as well as the ilium and sacrum (white arrows)

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  Fig. 4 Pathologic finding of osteoblastoma (H&E, ×200). The lesion consists of multinucleated giant cell, immature bone and osteoid deposition in fibrovascular stroma.

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