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Published on: September 15, 2017
A case of adrenal myelolipoma complicated with Prader-Willi syndrome
Toru Inoue1,2, Masahiro Todaka2, Yuichi Nakazono3
1Department of Urology, Faculty of Medicine Oita University Yufu-City Oita Japan.
Insights
Prader-Willi syndrome (PWS) can be complicated by adrenal myelolipoma, a benign adrenal tumor. This report details the successful laparoscopic removal of an adrenal myelolipoma in a PWS patient.
Area of Science:
- Endocrinology
- Genetics
- Surgical Oncology
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder affecting multiple systems.
- Adrenal myelolipomas are rare, benign tumors typically asymptomatic.
Observation:
- A 24-year-old male with PWS presented with an enlarged adrenal mass.
- Imaging suggested an adrenal myelolipoma.
- The patient underwent successful laparoscopic left adrenalectomy.
Findings:
- Histopathology confirmed adrenal myelolipoma.
- The patient experienced mild postoperative pulmonary atelectasis.
- No recurrence was observed at 2-year follow-up.
Implications:
- This is the first reported case of PWS associated with adrenal myelolipoma.
- Laparoscopic adrenalectomy is a viable treatment option for adrenal myelolipoma in PWS patients.
- Highlights the importance of monitoring for adrenal pathology in PWS.
Introduction:
Prader-Willi syndrome is a congenital disorder that occurs in one in 10 000-30 000 children and is characterized by obesity, short stature, and intellectual disability.
Case Presentation:
A 24-year-old male patient with Prader-Willi syndrome presented with an enlarged adrenal tumor. Computed tomography detected a well-defined mass. Magnetic resonance imaging revealed an increased signal intensity predominantly in fatty areas, suggesting adrenal myelolipoma. Laparoscopic left adrenalectomy was performed. Postoperatively, the patient developed mild pulmonary atelectasis, myelolipoma was confirmed by histopathology, and there was no recurrence at approximately 2 years postoperatively.
Conclusion:
This is the first report of Prader-Willi syndrome complicated with adrenal myelolipoma, which was removed laparoscopically.
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