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Published on: September 15, 2017
Cystic adrenal lesions: focus on pediatric population (a review)
Mara Carsote1, Adina Ghemigian1, Dana Terzea2
1Endocrinology Department, Carol Davila University of Medicine and Pharmacy & C.I. Parhon National Institute of Endocrinology, Bucharest, Romania.
Insights
Adrenal cysts in children and young adults range from benign to malignant, requiring careful diagnosis. Management involves observation or surgery, with a focus on preserving the adrenal gland.
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Diagnostic Imaging
Background:
- Adrenal cysts are rare but can affect any organ, with specific considerations in pediatric populations.
- Classification includes pure cysts, parasitic cysts, and cystic tumors like neuroblastoma.
- Adrenal hemorrhage, a distinct entity, presents unique diagnostic challenges and associations.
Purpose of the Study:
- To review the literature on adrenal cysts in children and young adults.
- To highlight diagnostic criteria and differential diagnoses, particularly cystic neuroblastoma.
- To discuss current management strategies and prognostic implications.
Main Methods:
- Literature review of pediatric adrenal cysts.
- Analysis of cyst types, complications, and associated conditions.
- Comparison of diagnostic features and management approaches.
Main Results:
- Adrenal cysts encompass a spectrum from benign to neoplastic lesions.
- Cystic neuroblastoma is a critical differential diagnosis for adrenal hemorrhage.
- Hemorrhage is often self-limiting, while tumors require specific interventions.
Conclusions:
- Pediatric adrenal cysts necessitate a multidisciplinary approach for accurate diagnosis and management.
- Management strategies range from conservative follow-up to surgical intervention, prioritizing gland preservation.
- Laparoscopic surgery is emerging as a viable option, though open procedures remain common.
Background And Aim:
The cysts may potentially affect any organ; adrenals cysts are rare. This is a review of the literature regarding adrenal cysts, focusing on children and young adults.
General Data:
Three major types have been described: pure cysts (endothelial, epithelial, and hemorrhagic or pseudocyst), parasitic (as hydatid) cysts and cystic part of a tumour (most frequent are neuroblastoma, ganglioneuroma, pheocromocytoma, and teratoma). The complications are: bleeding, local pressure effects; infection; rupture (including post-traumatic); arterial hypertension due to renal vessels compression. Adrenal hemorrhage represents a particular condition associating precipitating factors such as: coagulation defects as Factor IX or X deficiency, von Willebrand disease, thrombocytopenia; antiphospholipid syndrome; previous therapy with clopidogrel or corticosteroids; the rupture of a prior tumour. At birth, the most suggestive features are abdominal palpable mass, anemia, and persistent jaundice. Adrenal insufficiency may be found especially in premature delivery. The hemorrhage is mostly self-limiting. Antenatal ultrasound diagnosis of a cyst does not always predict the exact pathology result. The most important differential diagnosis of adrenal hemorrhage/hemorrhagic cyst is cystic neuroblastoma which is highly suggestive in the presence of distant metastases and abnormal catecholamine profile. The major clue to differentiate the two conditions is the fact that the tumor is stable or increases over time while the adrenal hemorrhage is expected to remit within one to two weeks.
Conclusion:
Pediatric adrenal cysts vary from simple cysts with a benign behavior to neoplasia- related lesions displaying severe prognosis as seen in cystic neuroblastoma. A multidisciplinary team is required for their management which is conservative as close follow-up or it makes necessary different surgical procedures in cases with large masses or if a malignancy suspicion is presented. Recently, laparoscopic approach is regarded as a safe procedure by some authors but generally, open surgery is more frequent used compare to adults; in most cases the preservation of normal gland is advisable.
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