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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
CASE REPORT
Year : 2022  |  Volume : 27  |  Issue : 6  |  Page : 764-767
 

Antenatally detected functional adrenocortical adenoma: A case report and review of current literature


1 Department of Paediatric Surgery, Command Hospital (SC), Pune, Maharashtra, India
2 Department of Surgery, Armed Forces Medical College, Pune, Maharashtra, India
3 Department of Paediatric Anaesthesia, Command Hospital, Pune, Maharashtra, India

Date of Submission04-Feb-2022
Date of Decision05-Apr-2022
Date of Acceptance27-May-2022
Date of Web Publication11-Nov-2022

Correspondence Address:
Vivek Kumar Singh
Department of Paediatric Surgery, Command Hospital, Pune - 411 060, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_25_22

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   Abstract 


Pediatric adrenocortical tumors (ACTs) are rare entities with an incidence of 0.2% of all pediatric tumors. Only two cases of antenatally detected ACT have been reported in the literature. Our case is the first report of an antenatally detected suprarenal mass which manifested with postnatal virilizing features and was proven to be adrenocortical adenoma on histology.


Keywords: Adrenocortical tumors, children, virilization


How to cite this article:
Singh VK, Khanna C, Raman V S, Goel A. Antenatally detected functional adrenocortical adenoma: A case report and review of current literature. J Indian Assoc Pediatr Surg 2022;27:764-7

How to cite this URL:
Singh VK, Khanna C, Raman V S, Goel A. Antenatally detected functional adrenocortical adenoma: A case report and review of current literature. J Indian Assoc Pediatr Surg [serial online] 2022 [cited 2022 Nov 30];27:764-7. Available from: https://www.jiaps.com/text.asp?2022/27/6/764/360952





   Introduction Top


The detection of suprarenal mass is exceedingly rare in a neonate and even rarer in a fetus despite the ubiquitous antenatal screening ultrasonography (USG). Postnatally, a large number of fetal suprarenal masses turn out to be adrenal hemorrhage or congenital neuroblastomas. The other less common differential diagnosis is congenital adrenal hyperplasia, subdiaphragmatic extralobar pulmonary sequestrations, renal duplication, urinoma, gastric duplication cyst, splenic cyst, and adrenocortical tumors (ACTs). ACT in children is rare comprising only 6% of all suprarenal masses and about 0.2% of all pediatric tumors. It is even rarer in infants with only 32 cases reported in the literature so far.[1] However, only two cases in the literature are of antenatally detected ACT. We are reporting the first case of an antenatally detected suprarenal mass which manifested with virilizing features and was proven to be adrenocortical adenoma (ACA) on histology.


   Case Report Top


Our patient was a 9-month-old female infant, firstborn of a nonconsanguineous marriage, who presented with a swelling in the left flank of 4-month duration. The antenatal scan during the third trimester at gestational age of 35 weeks and 5 days was suggestive of a large predominantly cystic heteroechoic abdominal mass of size 6.8 cm × 5.8 cm in the left renal fossa with no internal vascularity with suspicion of nephroblastoma [Figure 1]. Postnatal USG on day 3 of life revealed a 5.6 cm × 4.1 cm predominantly solid heteroechoic mass in the left suprarenal region with mild internal vascularity with differential diagnosis of Wilms tumor or adrenal mass. However, the parents did not seek treatment till 9 months of life as the child was asymptomatic and reported to our center when they noticed abdominal distension and enlargement of the clitoris.
Figure 1: Grayscale and color Doppler axial sections through fetal stomach show a large predominantly solid mass in the left suprarenal region with no significant vascularity

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On presentation, the abdominal examination revealed a nontender intra-abdominal ballotable mass in the left flank with regular margins and smooth surface. Examination of external genitalia revealed clitoromegaly. Urethral and vaginal openings were visualized [Figure 2].
Figure 2: Clinical Picture of the patient showing swelling over left hypochondrium and clitoromegaly

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Imaging in the form of contrast-enhanced computed tomography (CECT) abdomen pelvis showed a heterogeneously enhancing lesion measuring 7.5 cm × 7.3 cm × 8.7 cm in the region of left adrenal gland with a few peripheral calcific foci. Superiorly, the lesion was pushing the spleen cranially, superomedially displacing the body, and tail of the pancreas anterosuperiorly, medially the lesion was reaching up to midline and laterally up to parietal peritoneum [Figure 3]. Metaiodobenzylguanidine scan did not show any abnormal uptake.
Figure 3: Axial CT scan showing a large heterogeneously enhancing lesion in the left suprarenal region, reaching till midline and causing mass effect on adjacent retroperitoneal and intraabdominal structures. CT: Computed tomography

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Hormonal workup for epinephrine and their metabolites were negative; Serum dehydroepiandrosterone (DHEA) (>1000 mcg/dl) was significantly elevated; serum cortisol, 17-OH progesterone, alpha-fetoprotein, and beta-human chorionic gonadotropin were normal.

An USG-guided biopsy of the mass showed pathological evidence of an adrenocortical neoplasm with immunohistochemistry positive for synaptophysin, chromogranin, NSE, vimentin, and inhibin, and Ki-67 proliferation index of 10% with no comment of malignant potential.

In view of the large functional adrenal lesion with unknown malignant potential, the patient was offered surgical intervention. After proper informed consent, the child underwent an open left adrenalectomy. Intraoperative findings were consistent with an 8 cm × 6 cm left adrenal mass abutting splenic vein superomedially, renal vein inferomedially, and tail of pancreas posteriorly [Figure 4]. Steroid replacement was provided for 3 days in the postoperative period, and the child was discharged on the 4th day. Histopathology was conclusive of an ACA with a Modified Weiss score: 2/7. On follow-up at 6 months, the child is asymptomatic with normal DHEA levels and no further increase in clitoromegaly.
Figure 4: Intraoperative picture showing adrenocortical tumor with large adrenal vein

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   Discussion Top


Adrenal lesions on antenatal scans are generally picked up in the late third trimester in view of late development of the adrenal gland. Adrenal hemorrhage is the most common etiology of an adrenal mass in the neonatal period, usually occurring in the first few days of life and is four times more common than neuroblastoma.[2] Although a USG with color Doppler may help in the differentiation by showing blood flow signals with usually a feeding vessel in case of neuroblastoma or other adrenal tumors and lack of it in case of adrenal hemorrhage, a postnatal computerized tomography (CT) is the investigation of choice to help differentiate between adrenal hemorrhage and neuroblastoma; adrenal hemorrhage may initially look like a hypoechoic mass which gradually shrinks and becomes more echogenic, unlike neuroblastoma.

The correct diagnosis of neonatal adrenal mass depends on antenatal USG, postnatal clinical manifestations, urine VMA test, and radiological imaging, but dynamic observation of adrenal mass by CT and ultrasound is important to reach an accurate diagnosis. Moreover, adrenal mass which is difficult to diagnose can safely be followed up for around a month without any adverse effects on the therapy and prognosis of the tumor.[3]

Adrenal adenoma on antenatal USG appears as a semisolid, semicystic heterogeneous mass, which is quite similar to those observed in neuroblastoma. However, on plain CT images, lipid-rich adenomas characteristically have low attenuation of <10 HU while lipid-poor adenomas show higher attenuation values, and calcifications are seen less commonly in adrenal adenomas than in adrenocortical carcinomas (ACC). On CECT images, adrenal adenomas show moderate homogeneous contrast enhancement with rapid washout on delayed imaging. In magnetic resonance imaging (MRI), because of the presence of microscopic fat, the lipid-rich adenomas show a signal intensity drop on opposed-phase chemical shift images.[4]

The incidence of ACT is 0.2% of all pediatric tumors, out of which only 25% is ACA. ACT is more common in females with a female-to-male ratio of 2:1, however, there is no gender preponderance in the case of ACT detected in the fetal/newborn period.[5] Only 10% of ACT are functional, secreting mineralocorticoids, glucocorticoids, and sex hormones. In the case of functional tumors, signs of virilization are the most common symptom in the case of ACA while Cushing's syndrome is the most common symptom in the case of ACC.[6]

The most common presentation of these tumors is a palpable abdominal mass. USG is the first investigation of choice followed by cross-sectional imaging by CT scan to better characterize the lesion and to know its exact extent and size. A large non homogeneously enhancing mass that contains areas of hemorrhage, necrosis, and calcification are classic features of malignant ACT. In addition, evaluation of the abdomen, chest, and bone to assess the common sites of metastasis and evaluation of the inferior vena cava and right atrium, to look for tumor thrombus should also be done. MRI is not very helpful in differentiating between adenoma and carcinoma because of similar signal intensity characteristics.

All patients suspected of ACT should undergo hormonal evaluation to ascertain their functional status. Urinary 17-ketosteroids levels are elevated in the majority of the functional tumors irrespective of whether it causes Cushing's syndrome or virilization and plasma DHEA sulfate levels are high in approximately 90% of the cases.[7] Patients with signs of excessive glucocorticoids have elevated levels of urinary 17-hydroxycorticosteroid, and dexamethasone suppression test is done to confirm Cushing's syndrome. These tests not only help us in reaching the diagnosis but also provide useful markers for the detection of tumor recurrence.

On biopsy, the distinction between benign and malignant ACT may be difficult even in the hands of an experienced pathologist. Distant metastasis or the presence of local invasion is the only definitive diagnostic criterion of malignant ACT. In the absence of these findings, a simple and reliable pathological method, the Weiss criteria are used for ascertaining the malignant potential of the lesion. There are a total of five criteria which are used in the updated Weiss system and include: >6 mitoses/50 high-power fields and ≤25% clear tumor cells in the cytoplasm, abnormal mitoses, necrosis, and capsular invasion.[8] The first two criteria are scored 0 when absent or 2 when present and the last three are scored 1 when present. The threshold for malignancy is a total score ≥3. Our patient had a total score of 2, and hence labeled as adenoma.

Manifestations such as clitoromegaly, early puberty, and hirsutism can create an immense impact on the early psychosocial development of the child and need to be tackled early. Complete surgical excision remains the mainstay of treatment for the functional or malignant ACT. Most adrenal lesions >4 cm should be removed regardless of imaging findings due to increased risk of adrenocortical carcinoma.[9] Clinical observation and follow-up is recommended for lesions <4 cm and nonfunctional lesions with benign features on imaging.[10] These lesions are followed up annually by clinical, hormonal, and radiological testing to look for features pointing toward surgical intervention. Patients with ACC are followed up in the standardized manner of all malignant tumors with 3 monthly follow-ups in the 1st year, 6 monthly in the second, and yearly for the next 5 years.[11]

In conclusion, antenatally detected functional adrenocortical tumors are extremely rare pediatric tumors that require a thorough diagnostic evaluation, early and complete surgical excision for optimal outcomes.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Quintos JB, Salas M. Virilizing adrenal adenoma in a 20 month-old girl. J Pediatr Endocrinol Metab 2009;22:779-80.  Back to cited text no. 1
    
2.
Goodman SN. Neuroblastoma screening data. An epidemiologic analysis. Am J Dis Child 1991;145:1415-22.  Back to cited text no. 2
    
3.
Yao W, Li K, Xiao X, Zheng S, Chen L. Neonatal suprarenal mass: Differential diagnosis and treatment. J Cancer Res Clin Oncol 2013;139:281-6.  Back to cited text no. 3
    
4.
Sargar KM, Khanna G, Hulett Bowling R. Imaging of nonmalignant adrenal lesions in children. Radiographics 2017;37:1648-64.  Back to cited text no. 4
    
5.
Isaacs H Jr. Fetal and newborn adrenocortical tumors. Fetal Pediatr Pathol 2010;29:99-107.  Back to cited text no. 5
    
6.
Ribeiro RC, Michalkiewicz EL, Figueiredo BC, DeLacerda L, Sandrini F, Pianovsky MD, et al. Adrenocortical tumors in children. Braz J Med Biol Res 2000;33:1225-34.  Back to cited text no. 6
    
7.
Satge D, Philippe E, Ruppe M, Levy JM, Lutz P, Walter P. Neonatal carcinoma. Review of the literature apropos of a case. Bull Cancer 1988;75:373-84.  Back to cited text no. 7
    
8.
Musella M, Conzo G, Milone M, Corcione F, Belli G, De Palma M, et al. Preoperative workup in the assessment of adrenal incidentalomas: Outcome from 282 consecutive laparoscopic adrenalectomies. BMC Surg 2013;13:57.  Back to cited text no. 8
    
9.
Angeli A, Osella G, Alì A, Terzolo M. Adrenal incidentaloma: An overview of clinical and epidemiological data from the National Italian Study Group. Horm Res 1997;47:279-83.  Back to cited text no. 9
    
10.
Kapoor A, Morris T, Rebello R. Guidelines for the management of the incidentally discovered adrenal mass. Can Urol Assoc J 2011;5:241-7.  Back to cited text no. 10
    
11.
Libé R. Adrenocortical carcinoma (ACC): Diagnosis, prognosis, and treatment. Front Cell Dev Biol 2015;3:45.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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