Background: Minimally invasive adrenalectomy and advances in anaesthetic techniques have transformed surgery for phaeochromocytoma. This 17-year review describes the evolution of phaeochromocytoma care in our unit.
Methods: We performed a retrospective cohort review of all patients who underwent adrenalectomy for phaeochromocytoma from 2000 to 2016. Patients were divided into three time periods, early: 2000-2005 (n = 17), middle: 2006-2010 (n = 15) and late: 2011-2016 (n = 24). The posterior retroperitoneoscopic adrenalectomy was introduced in 2011. Demographics and clinicopathological details were extracted. Median values for nominal data were compared using Mann-Whitney U-test. A chi-squared test was used to compare categorical data.
Results: Sixty-one adrenalectomies were performed on 56 patients: 19 open, 17 laparoscopic and 20 posterior retroperitoneoscopic adrenalectomies. The median length of operation decreased from 135 to 90 min from the early to the late time period (P > 0.05). Length of stay decreased from a median of 5 days in the early group to 1 day in the late group (P = 0.01). A total of 94.1% of the early period patients were admitted to the intensive care unit compared to 30.4% of the late group (P = <0.01). Need for post-operative vasopressors and blood transfusions was significantly reduced.
Conclusion: Over the 17-year period, the choice of operative technique has transitioned towards posterior retroperitoneoscopic adrenalectomy. Operative time, rate of intensive care unit admission, and admission length have all decreased without any increase in rates of complications.
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http://dx.doi.org/10.1111/ans.16847 | DOI Listing |
Int J Surg Pathol
January 2025
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Metastasis of alveolar soft part sarcoma (ASPS) to the adrenal gland is infrequent, with only eight patients reported in the literature. Here we present an ASPS in an adolescent girl presented as a hypervascular adrenal incidentaloma along with a review of the available literature. This study aims to serve as a reference to aid in the pre-operative radiological and histopathological diagnosis of this rare entity.
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September 2024
Nephrology, The Children's Hospital at Westmead, Westmead, Australia.
A 17-year-old boy with Von Hippel-Lindau syndrome presented with hypertension, raised plasma catecholamines, and MRI findings of a new pancreatic tail lesion and 2 stable right adrenal lesions concerning for functional neuroendocrine tumors. A 68 Ga-DOTATATE PET/CT demonstrated intense tracer avidity within the pancreatic lesion with minimal uptake in the adrenal lesions. Conversely, a 123 I-MIBG SPECT/CT study demonstrated high-grade tracer uptake within the adrenal lesions, with no significant uptake appreciated in the pancreatic lesion.
View Article and Find Full Text PDFJCO Precis Oncol
May 2024
Department of Internal Medicine and Oncology, Semmelweis University, Faculty of Medicine, Budapest, Hungary.
Purpose: Medullary thyroid carcinoma (MTC) in MEN2B syndrome is associated with germline mutation. Patients harboring de novo mutations are usually diagnosed at more advanced disease stages. We present a young woman with Met918Th mutation diagnosed with stage IV MTC at age 10 years.
View Article and Find Full Text PDFCureus
March 2024
Surgical Oncology, Marshfield Medical Center, Wisconsin, USA.
Autosomal dominant hereditary paraganglioma-pheochromocytoma syndrome (HPPS) is a rare genetic disorder characterized by neuroendocrine tumor development associated with pathogenic variants in succinate dehydrogenase (SDH) enzyme complex genes. Particularly, HPPS linked to SDHB mutation poses a significant clinical challenge due to its association with aggressive tumor features and a high risk of malignancy. Our report underscores the diversity in the presentation of patients with SDHB-mutated paraganglioma and the feasibility of managing it with a minimally invasive surgical approach.
View Article and Find Full Text PDFAnn Surg Oncol
July 2024
Surgical Oncology, Marshfield Medical Center, Marshfield, WI, USA.
Background: Up to 41% of intra- and extra-adrenal paragangliomas are linked to germline mutations with autosomal dominant transmission, which necessitates genetic testing for patients and their relatives. Certain alterations, such as the succinate dehydrogenase (SDH) subunit B gene mutation, are associated with a significant risk of extra-adrenal, malignant, and metastatic disease forms. This highlights the need for routine genetic counseling and diligent surveillance, as well as surgeon awareness of hereditary paraganglioma-pheochromocytoma syndrome (HPPS).
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