Unusual adrenal cortical tumor of unknown biologic potential: a nodule in a nodule in a nodule.
Adrenocortical tumors are uncommon neoplasms in childhood. Most pediatric adrenal tumors are virilizing and carcinomas are more common than adenomas. Recent molecular data suggest an adenoma-to-carcinoma progression sequence in adrenal cortical neoplasms. We report a case of a 5-year-old boy who presented with virilizing symptoms secondary to an adrenal tumor that was resected laparoscopically. The bulk of the tumor was a large, yellow mass with typical features of an adrenal cortical adenoma. In the center was a well-circumscribed tan-brown nodule that was distinct from the adenoma and had oncocytic features. A third minute focus (3.0 mm) was noted that was not circumscribed or encapsulated, but showed marked pleomorphism and abundant mitoses, including atypical forms and increased Ki67 compared with the outer 2 nodules. Molecular analysis to assess the clonality and mutation rates of the 3 distinct areas showed only 2 genetic loci with allelic imbalances.
Ranganathan
Sarangarajan
S
Department of Pathology, Children's Hospital of Pittsburgh, PA 15213, USA.
Lynshue
Kecha
K
Hunt
Jennifer L
JL
Kane
Timothy
T
Jaffe
Ronald
R
D000306:Adrenal Cortex Neoplasms / Q000150:complications / Q000235:genetics / Q000473:pathology*; D000315:Adrenalectomy; D018246:Adrenocortical Adenoma / Q000150:complications / Q000235:genetics / Q000473:pathology*; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000209:etiology / Q000473:pathology; D004273:DNA, Neoplasm / Q000032:analysis; D006628:Hirsutism / Q000209:etiology / Q000473:pathology; D006801:Humans; D019656:Loss of Heterozygosity; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications / Q000235:genetics / Q000473:pathology; D016133:Polymerase Chain Reaction; D016896:Treatment Outcome
D004273:DNA, Neoplasm