Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias.
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.
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Hyeoh Won
HW
Yu
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Won Im
WI
Cho
Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
Hye Rim
HR
Chung
Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
Keun Hee
KH
Choi
Department of Pathology, Seoul National University Bundang Hospital, Seongnam, Korea.
Sumi
S
Yun
Department of Orthopaedic Surgery, Seoul National University Bundang Hospital, Seongnam, Korea.
Hwan Seong
HS
Cho
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Choong Ho
CH
Shin
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Sei Won
SW
Yang
Cushing syndrome; Micronodular adrenal disease; Osteoblastoma