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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

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

Mentions: Because her skin pigmentation and pathology of both adrenal glands suggested the possibility of Carney complex, heart and thyroid gland ultrasonography were performed, which were all negative. No deletion or duplication was identified in protein kinase A regulatory subunit type 1A (PRKAR1A) gene analysis. However, at the age of 8 years, she complained of left hip pain, and her physical exam suspected a bone protrusion in the left temporal area. Magnetic resonance imaging (MRI) of her hip revealed overall polyostotic bone tumors involving the bilateral femurs, ilium and sacrum. Her brain MRI revealed multiple multilocular cystic bone lesions involving the clivus, bifrontal skull base, bilateral sphenoid, and left temporal bones (Fig. 3). The right temporal bone biopsy demonstrated osteoid producing neoplasms, consistent with osteoblastoma (Fig. 4). Because the bone pain had resolved and the skull, hip and leg lesions caused no functional problems, she was on conservative management with physiologic hydrocortisone and fludrocortisone replacement.


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)

Pathologic finding of osteoblastoma (H&E, ×200). The lesion consists of multinucleated giant cell, immature bone and osteoid deposition in fibrovascular stroma.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: Pathologic finding of osteoblastoma (H&E, ×200). The lesion consists of multinucleated giant cell, immature bone and osteoid deposition in fibrovascular stroma.
Mentions: Because her skin pigmentation and pathology of both adrenal glands suggested the possibility of Carney complex, heart and thyroid gland ultrasonography were performed, which were all negative. No deletion or duplication was identified in protein kinase A regulatory subunit type 1A (PRKAR1A) gene analysis. However, at the age of 8 years, she complained of left hip pain, and her physical exam suspected a bone protrusion in the left temporal area. Magnetic resonance imaging (MRI) of her hip revealed overall polyostotic bone tumors involving the bilateral femurs, ilium and sacrum. Her brain MRI revealed multiple multilocular cystic bone lesions involving the clivus, bifrontal skull base, bilateral sphenoid, and left temporal bones (Fig. 3). The right temporal bone biopsy demonstrated osteoid producing neoplasms, consistent with osteoblastoma (Fig. 4). Because the bone pain had resolved and the skull, hip and leg lesions caused no functional problems, she was on conservative management with physiologic hydrocortisone and fludrocortisone replacement.

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