Congenital Adrenal Cortical Carcinoma BY Jo&
Luis Rocha Artigas,
Edgard
D’Avila
Niclewicz,
Dulcio Bastes Ribas, and Sirgio
Antonio
de Padua G. Silva,
Luiz Athayde
A
DRENAL CORTICAL CARCINOMAS in childhood are rare.‘,’ Goldstein3 in 1946 mentioned one case described by Dobbertin in 1900 of a baby born virilized who had a “congenital carcinoma” removed when 14 mo old from where the left adrenal should be. This may well have been the first case of congenital adrenal carcinoma mentioned in the literature. Burrington and Stephens4 reported one infant who was virilized at birth and they presumed this was due to excess androgen produced in utero by an adrenal cortical tumor later removed. We here report two cases of children born with adrenal cortical carcinoma. CASE
REPORTS
Case 1 A white female was admitted at age 18 days. The pregnancy was normal. At birth, she had marked abdominal distention and her clitoris was hypertrophied. An abdominal mass was felt on the left. The urinary 17-KS was 15.25 mg/24 hs. Six days later the tumor was removed through laparotomy. It was a round encapsulated mass measuring 5.5 x 4.5 x 3 cm. The capsule was not opened during surgery. The baby recovered normally and the urinary l7-KS soon returned to normal levels. She remained well until age 4 yr, when her mother noticed the appearance of pubic hair and clitoris enlargement. Radiologic examination revealed a calcified mass above the left kidney. She was readmitted with the diagnosis of left adrenal cortical carcinoma recurrence. At laparotomy a recurrent tumor was found at the same site of the previous one, above the left kidney and involving its hilus. It was necessary to remove the left kidney together with the mass. Histologic examination revealed adrenal cortical carcinoma both times. She was treated with radiotherapy course and is now well, 5 yr later her 17-KS has gone back to normal levels.
Case 2 A 5-day-old white female was admitted. As in Case I, she was born after a normal pregnancy and immediately was noted to have abdominal distention and clitoris hypertrophy. A left abdominal solid mass was easily palpated. 17-KS was 14.5 mg/24 hs. She was referred for removal of adrenal tumor. At laparotomy a large left encapsulated tumor was found. The left kidney was adherent to the mass in its inferior pole. The spleen was also adherent and both the left kidney and the spleen had to be removed in order to free the mass. The left diaphragm was firmly adherent to the mass, and after a trial at freeing it, it was felt necessary to remove a portion of the diaphragm together with the mass so as not to rupture the capsule. Immediately after the resection cardiac arrest occurred which was impossible to revert. Histology disclosed adrenal cortical carcinoma.
From the Departments of Surgery. Pediatrics, Endocrinology and Pathology. Hospital Nossa Senhora das Grafas. Curitiba, Brazil. and the Federal Universitv of Parand School of Medicine, Brazil. Address _tbr reprint requests: JosC Luis Rocha Artigas, M.D., Section of Pediatric Surgery. pital Nossa Senhora das Gracas. Rua Prof: Rosa Saporski 229, Cuririba 80000. Bra;il. ~aI976 b,v Grune & Stratton, Inc.
Journal of Pediatric Surgery, Vol. 11, No. 2 (April), 1976
Hos-
247
248
ARTIGAS
ET AL.
Fig. 1. Cose 1 when 20 days old, with clitoris enlargement and abdominal distention.
Fig. 2. IVP: Case 1 demonstrating mass left kidney and displacement of intestine.
Fig. 4. Vascular invasion first surgery.
by neoplastic cells. Hematoxylin
Fig. 5. Atypical cell with two nuclei and vacuolization eosin x 270. Case 1 at time of first surgery.
and eosin
in
its
x 160. Case 1 at time of
cytoplasm.
Hematoxylin
and
ARTIGAS
Case 2 Fig. 6. clitoris enlargement.
Fig. 7. IVP: Case 2 demo nstrating abdominal mass dir iplacing the left iliac fossa.
when
6
days
ET Al.
aJd,
, large
left kidney , to
CONCLUSION
Even though rare, childhood adrenal cortical carcinomas are often described in the literature.5-‘1 The rarity of reported cases of congenital adrenal carcinomas prompted us to report our two cases. SUMMARY
Two cases of congenital
adrenal
cortical
carcinomas
are reported.
ADRENAL
Fig. eosin
CORTICAL
8.
251
CARCINOMA
Capsular
invasion
demonstrated
from
tumor
taken
from
Case
2.
Hematoxylin
x 65.
Fig.
9.
Vascular
invasion
by neoplastic
cells
in Case
2. Hematoxylin
and
eosin
x 65.
and
252
ARTIGAS
Fig. 10.
Atypical mitosis
in tumor from Case 2. Hematoxylin
ET AL.
and eosin x 270.
REFERENCES 1. Hayles AB, Hahn al: Hormone-secreting
HB Jr, Sprague tumors of the
RG, et adrenal
cortex in children. Pediatrics 37: 19, 1966 2. Rapaport E, Goldberg MB, Gordan GS, et al. Mortality in surgically treated adrenocortical tumors: Review of cases reported for 20 year period (1930-49). Postgrad Med 11:325, 1952 3. Goldstein AE, Rubin SW, Askin 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. Am J Dis Child 72: 563, 1946 4. Burrington JD, Stephens CA: Virilizing tumors of the adrenal gland in childhood: Report of eight cases. J Pediatr Surg 4:291, 1969 5. Bennett AH, Harrison JH, Thorn GW: Neoplasms of the adrenal gland. J Urol 106: 607, 1971
6. Fontaine G, Lacheretz M, DuPont A, et al: Tumeur de la cortico-surr6nale avec pubertt pr6coce et gyntcomastie chez un garcon de 3 ans l/2. Ann Ptdiat 17:463, 1970 7. Gershanik JJ, Elmore M, Levkoff AH: Congenital concurrence of adrenal cortical tumor, ganglioneuroma and toxoplasmosis. Pediatrics 51:705, 1973 8. Huvos AG, Hajdn SI, Brasfield RD, et al: Adrenal cortical carcinoma (Clinicopathologic study of 34 cases). Cancer 25:354, 1970 9. Kenny FM, Hashida Y, Askari HA, et al: Virilizing tumors of the adrenal cortex. Am J Dis Child 115:445, 1968 IO. Stewart DR, Jones PM, Jolleys A: Carcinoma of the adrenal gland in children. J Pediatr Surg 19:59. 1974 11. Vaisman S, Pizzi T, Ribera G, et al: Adenocarcinoma suprarrenal. Rev Chilena de Ped 3:175, 1971
Luis Rocha Artigas,
Edgard
D’Avila
Niclewicz,
Dulcio Bastes Ribas, and Sirgio
Antonio
de Padua G. Silva,
Luiz Athayde
A
DRENAL CORTICAL CARCINOMAS in childhood are rare.‘,’ Goldstein3 in 1946 mentioned one case described by Dobbertin in 1900 of a baby born virilized who had a “congenital carcinoma” removed when 14 mo old from where the left adrenal should be. This may well have been the first case of congenital adrenal carcinoma mentioned in the literature. Burrington and Stephens4 reported one infant who was virilized at birth and they presumed this was due to excess androgen produced in utero by an adrenal cortical tumor later removed. We here report two cases of children born with adrenal cortical carcinoma. CASE
REPORTS
Case 1 A white female was admitted at age 18 days. The pregnancy was normal. At birth, she had marked abdominal distention and her clitoris was hypertrophied. An abdominal mass was felt on the left. The urinary 17-KS was 15.25 mg/24 hs. Six days later the tumor was removed through laparotomy. It was a round encapsulated mass measuring 5.5 x 4.5 x 3 cm. The capsule was not opened during surgery. The baby recovered normally and the urinary l7-KS soon returned to normal levels. She remained well until age 4 yr, when her mother noticed the appearance of pubic hair and clitoris enlargement. Radiologic examination revealed a calcified mass above the left kidney. She was readmitted with the diagnosis of left adrenal cortical carcinoma recurrence. At laparotomy a recurrent tumor was found at the same site of the previous one, above the left kidney and involving its hilus. It was necessary to remove the left kidney together with the mass. Histologic examination revealed adrenal cortical carcinoma both times. She was treated with radiotherapy course and is now well, 5 yr later her 17-KS has gone back to normal levels.
Case 2 A 5-day-old white female was admitted. As in Case I, she was born after a normal pregnancy and immediately was noted to have abdominal distention and clitoris hypertrophy. A left abdominal solid mass was easily palpated. 17-KS was 14.5 mg/24 hs. She was referred for removal of adrenal tumor. At laparotomy a large left encapsulated tumor was found. The left kidney was adherent to the mass in its inferior pole. The spleen was also adherent and both the left kidney and the spleen had to be removed in order to free the mass. The left diaphragm was firmly adherent to the mass, and after a trial at freeing it, it was felt necessary to remove a portion of the diaphragm together with the mass so as not to rupture the capsule. Immediately after the resection cardiac arrest occurred which was impossible to revert. Histology disclosed adrenal cortical carcinoma.
From the Departments of Surgery. Pediatrics, Endocrinology and Pathology. Hospital Nossa Senhora das Grafas. Curitiba, Brazil. and the Federal Universitv of Parand School of Medicine, Brazil. Address _tbr reprint requests: JosC Luis Rocha Artigas, M.D., Section of Pediatric Surgery. pital Nossa Senhora das Gracas. Rua Prof: Rosa Saporski 229, Cuririba 80000. Bra;il. ~aI976 b,v Grune & Stratton, Inc.
Journal of Pediatric Surgery, Vol. 11, No. 2 (April), 1976
Hos-
247
248
ARTIGAS
ET AL.
Fig. 1. Cose 1 when 20 days old, with clitoris enlargement and abdominal distention.
Fig. 2. IVP: Case 1 demonstrating mass left kidney and displacement of intestine.
Fig. 4. Vascular invasion first surgery.
by neoplastic cells. Hematoxylin
Fig. 5. Atypical cell with two nuclei and vacuolization eosin x 270. Case 1 at time of first surgery.
and eosin
in
its
x 160. Case 1 at time of
cytoplasm.
Hematoxylin
and
ARTIGAS
Case 2 Fig. 6. clitoris enlargement.
Fig. 7. IVP: Case 2 demo nstrating abdominal mass dir iplacing the left iliac fossa.
when
6
days
ET Al.
aJd,
, large
left kidney , to
CONCLUSION
Even though rare, childhood adrenal cortical carcinomas are often described in the literature.5-‘1 The rarity of reported cases of congenital adrenal carcinomas prompted us to report our two cases. SUMMARY
Two cases of congenital
adrenal
cortical
carcinomas
are reported.
ADRENAL
Fig. eosin
CORTICAL
8.
251
CARCINOMA
Capsular
invasion
demonstrated
from
tumor
taken
from
Case
2.
Hematoxylin
x 65.
Fig.
9.
Vascular
invasion
by neoplastic
cells
in Case
2. Hematoxylin
and
eosin
x 65.
and
252
ARTIGAS
Fig. 10.
Atypical mitosis
in tumor from Case 2. Hematoxylin
ET AL.
and eosin x 270.
REFERENCES 1. Hayles AB, Hahn al: Hormone-secreting
HB Jr, Sprague tumors of the
RG, et adrenal
cortex in children. Pediatrics 37: 19, 1966 2. Rapaport E, Goldberg MB, Gordan GS, et al. Mortality in surgically treated adrenocortical tumors: Review of cases reported for 20 year period (1930-49). Postgrad Med 11:325, 1952 3. Goldstein AE, Rubin SW, Askin 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. Am J Dis Child 72: 563, 1946 4. Burrington JD, Stephens CA: Virilizing tumors of the adrenal gland in childhood: Report of eight cases. J Pediatr Surg 4:291, 1969 5. Bennett AH, Harrison JH, Thorn GW: Neoplasms of the adrenal gland. J Urol 106: 607, 1971
6. Fontaine G, Lacheretz M, DuPont A, et al: Tumeur de la cortico-surr6nale avec pubertt pr6coce et gyntcomastie chez un garcon de 3 ans l/2. Ann Ptdiat 17:463, 1970 7. Gershanik JJ, Elmore M, Levkoff AH: Congenital concurrence of adrenal cortical tumor, ganglioneuroma and toxoplasmosis. Pediatrics 51:705, 1973 8. Huvos AG, Hajdn SI, Brasfield RD, et al: Adrenal cortical carcinoma (Clinicopathologic study of 34 cases). Cancer 25:354, 1970 9. Kenny FM, Hashida Y, Askari HA, et al: Virilizing tumors of the adrenal cortex. Am J Dis Child 115:445, 1968 IO. Stewart DR, Jones PM, Jolleys A: Carcinoma of the adrenal gland in children. J Pediatr Surg 19:59. 1974 11. Vaisman S, Pizzi T, Ribera G, et al: Adenocarcinoma suprarrenal. Rev Chilena de Ped 3:175, 1971
