0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Pathological Case of the Month FREE

[+] Author Affiliations

Section Editor: Enid Gilbert-barness, MD

More Author Information
Arch Pediatr Adolesc Med. 2000;154(12):1268. doi:.
Text Size: A A A
Published online

DIAGNOSIS AND DISCUSSION: CONGENITAL ADRENOCORTICAL CARCINOMA

Figure 1. Well-encapsulated tumor measuring 6 × 4 × 3 cm pushing the right kidney downward. The photograph has been taken from the posterior aspect.

Figure 2. Tumor cells arranged in nests with intervening blood vessels (hematoxylin-eosin, original magnification ×140).

Figure 3. Capsular invasion by the tumor (hematoxylin-eosin, original magnification ×140).

Figure 4. Metastatic deposits in the liver (hematoxylin-eosin, original magnification ×280).

This child presented with a rare entity: congenital adrenocortical carcinoma with microscopic metastasis to the liver. The tumor revealed 6 of 9 histologic criteria laid out by Medeiros and Weiss,1 which are found to be associated with adrenal cortical neoplasms that metastasized or recurred. These are capsular invasion, necrosis, sinusoidal invasion, mitosis (>5/50 high-power field), diffuse architecture (>33% of tumor), and eosinophilic tumor cell cytoplasm (>75% of tumor). Above all, the sine qua non of malignant tumors (metastasis to the liver) was also seen.

Adrenocortical carcinoma in children is rare24 and comprises only 0.2% of all childhood malignant tumors.5 They comprise 6% of childhood malignant adrenal tumors, most being neuroblastomas.6 Congenital adrenocortical carcinomas are even more rare, and few cases have been published in the world literature.79 In contrast to adults, most of these tumors in children are hormonally active, and perhaps early detection is the reason for their so-called better survival. Clinically, they manifest by virilization, Cushing syndrome, and occasionally with aldosteronism and feminization (in decreasing order of frequency). A mixture of syndromes is the most common mode of presentation. There is greater incidence of functional adrenal tumors in females than males for unexplained reasons. The occurrence of Cushing syndrome resulting from an adrenal cortex tumor in infants in association with hemihypertrophy and urinary tract anomalies as well as various other anomalies suggests oncogenic factors occurring during embryonic development.2,9,10 Carcinomas outnumber adenomas in childhood adenocortical tumors.

Most children with adrenal tumors have signs of androgen excess,3,11 including acne, deepening of voice, muscular appearance, pubic hair, rapid statural growth, and enlarged penis or clitoris. The differential diagnoses in a virilized boy are quite limited and include late-onset congenital adrenal hyperplasia, Leydig cell tumor of the testis, and true isosexual precocity. Virilization associated with features of hypercortisolism or feminization occurs twice as frequently as "pure" virilization.12 Virilizing tumors tend to be small (≈50 g). Endogenous Cushing syndrome in children younger than age 10 years is usually due to an adrenal tumor,13 whereas in older children, hyperplasia may exceed tumors in frequency.14 Cushing syndrome without virilization is rarely observed. The classic features of Cushing syndrome include obesity, muscle wasting, plethora, round facies, striae, short stature, and hypertension. Linear growth may be normal in children with associated virilization. Truncal obesity may be observed in older children, but infants tend to demonstrate generalized obesity. Primary aldosteronism is rare in children and is usually due to adrenocortical hyperplasia, but few cases are reported in the literature in which primary aldosteronism is due to an adrenocortical tumor.15,16 Pure feminizing tumors are rarely encountered in children. Only 7 cases of feminizing adenomas in males have been recorded.17 About 5% of childhood adrenocortical tumors produce no clinical evidence of endocrine dysfunction. This does not mean that they are incapable of steroidogenesis but only that they do not produce an excess of active hormones. Because these tumors do not produce symptoms, they are extremely large at the time of diagnosis and have poor prognosis.

Accepted for publication May 28, 1999.

Reprints: Nandita Kakkar, MD, Department of Histopathology, PGIMER, Chandigarh-160012, India.

Medeiros  LJWeiss  LM New developments in the pathologic diagnosis of adrenal cortical neoplasms. Am J Clin Pathol. 1992;9773- 83
Miller  RW Peculiarities in the occurrence of adrenal cortical carcinoma [editorial]. AJDC. 1978;132235
Hayles  ABHahn  HBSprague  RG  et al.  Hormone-secreting tumours of the adrenal cortex in children [editorial]. Pediatrics. 1966;3719
Rapaport  EGoldberg  MBGordan  GS  et al.  Mortality in surgically treated adreno-cortical tumours: review of cases reported for a 20-year period (1930-1949). Postgrad Med. 1952;11325- 332
Young  JL  JrMiller  RW Incidence of malignant tumours in US children. J Pediatr. 1975;86254- 258
Link to Article
Stewart  DRJones  PAJolly  SA Carcinoma of adrenal gland in children. J Pediatr Surg. 1974;959- 67
Link to Article
Burrington  JDStephens  CA Virilizing tumours of the adrenal gland in childhood: report of eight cases. J Pediatr Surg. 1969;4291- 302
Link to Article
Goldstein  AERubin  SWAskin  JA Carcinoma of adrenal cortex with adrenogenital syndrome in children: complete review of the literature and report of a case with recovery in a child 8 months of age. AJDC. 1946;72563
Artigas  JLRNiclewicz  EDSilva  AdPGRibas  DBAthayde  SL Congenital adrenal cortical carcinoma. J Pediatr Surg. 1976;11247- 252
Link to Article
Fraumeni  JFMiller  RW Adrenal cortical neoplasms and hemihypertrophy, brain tumour and other disorders. J Pediatr. 1967;70129- 138
Link to Article
Kenny  FMHashide  IAskri  ASiebver  WHFetterman  GH Virilizing tumour of the adrenal cortex. AJDC. 1968;115445- 459
Humphrey  GBed Adrenal and Endocrine Tumors in Children.  Boston, Mass Kluwer Academic Publishers1984;240
Guin  GHGilbert  EF Cushing's syndrome in children associated with adrenal cortical carcinoma: a case report with review of literature. AJDC. 1956;92297- 307
McArthur  RGCloutier  MDHayles  ABSprague  RB Cushing disease in children: findings in 13 cases. Mayo Clin Proc. 1972;47318- 336
Canell  BSandegard  EHokfelt  B Primary aldosteronism due to an adrenal adenoma in a 3-year-old child. Acta Pediatr Scand. 1964;53205- 212
Link to Article
Kelch  RPConnors  MHKaplan  SI  et al.  A calcified aldosterone producing tumor in a hypertensive, normokalemic prepubertal girl. J Pediatr. 1973;83432- 437
Link to Article
Sultan  CDescomp  SBGarandeau  PBressot  NJean  R Pubertal gynecomastia due to an estrogen producing adrenal adenoma. J Pediatr. 1976;95744- 746
Link to Article

Tables

References

Medeiros  LJWeiss  LM New developments in the pathologic diagnosis of adrenal cortical neoplasms. Am J Clin Pathol. 1992;9773- 83
Miller  RW Peculiarities in the occurrence of adrenal cortical carcinoma [editorial]. AJDC. 1978;132235
Hayles  ABHahn  HBSprague  RG  et al.  Hormone-secreting tumours of the adrenal cortex in children [editorial]. Pediatrics. 1966;3719
Rapaport  EGoldberg  MBGordan  GS  et al.  Mortality in surgically treated adreno-cortical tumours: review of cases reported for a 20-year period (1930-1949). Postgrad Med. 1952;11325- 332
Young  JL  JrMiller  RW Incidence of malignant tumours in US children. J Pediatr. 1975;86254- 258
Link to Article
Stewart  DRJones  PAJolly  SA Carcinoma of adrenal gland in children. J Pediatr Surg. 1974;959- 67
Link to Article
Burrington  JDStephens  CA Virilizing tumours of the adrenal gland in childhood: report of eight cases. J Pediatr Surg. 1969;4291- 302
Link to Article
Goldstein  AERubin  SWAskin  JA Carcinoma of adrenal cortex with adrenogenital syndrome in children: complete review of the literature and report of a case with recovery in a child 8 months of age. AJDC. 1946;72563
Artigas  JLRNiclewicz  EDSilva  AdPGRibas  DBAthayde  SL Congenital adrenal cortical carcinoma. J Pediatr Surg. 1976;11247- 252
Link to Article
Fraumeni  JFMiller  RW Adrenal cortical neoplasms and hemihypertrophy, brain tumour and other disorders. J Pediatr. 1967;70129- 138
Link to Article
Kenny  FMHashide  IAskri  ASiebver  WHFetterman  GH Virilizing tumour of the adrenal cortex. AJDC. 1968;115445- 459
Humphrey  GBed Adrenal and Endocrine Tumors in Children.  Boston, Mass Kluwer Academic Publishers1984;240
Guin  GHGilbert  EF Cushing's syndrome in children associated with adrenal cortical carcinoma: a case report with review of literature. AJDC. 1956;92297- 307
McArthur  RGCloutier  MDHayles  ABSprague  RB Cushing disease in children: findings in 13 cases. Mayo Clin Proc. 1972;47318- 336
Canell  BSandegard  EHokfelt  B Primary aldosteronism due to an adrenal adenoma in a 3-year-old child. Acta Pediatr Scand. 1964;53205- 212
Link to Article
Kelch  RPConnors  MHKaplan  SI  et al.  A calcified aldosterone producing tumor in a hypertensive, normokalemic prepubertal girl. J Pediatr. 1973;83432- 437
Link to Article
Sultan  CDescomp  SBGarandeau  PBressot  NJean  R Pubertal gynecomastia due to an estrogen producing adrenal adenoma. J Pediatr. 1976;95744- 746
Link to Article

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections