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Frontiers in Endocrinology 2024Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%-10%...
Ectopic ACTH syndrome (EAS) remains one of the most demanding diagnostic and therapeutic challenges for endocrinologists. Thymic neuroendocrine tumors account for 5%-10% of all EAS cases. We report a unique case of a 31-year-old woman with severe EAS caused by primary metastatic combined large-cell neuroendocrine carcinoma and atypical carcinoid of the thymus. The patient presented with severe hypercortisolemia, which was successfully controlled with continuous etomidate infusion. Complex imaging initially failed to detect thymic lesion; however, it revealed a large, inhomogeneous, metabolically active left adrenal mass infiltrating the diaphragm, suspected of primary disease origin. The patient underwent unilateral adrenalectomy, which resulted in hypercortisolemia resolve. The pathology report showed an adenoma with adrenal infarction and necrosis. The thymic tumor was eventually revealed a few weeks later on follow-up imaging studies. Due to local invasion and rapid progression, only partial resection of the thymic tumor was possible, and the patient was started on radio- and chemotherapy.
PubMed: 38948516
DOI: 10.3389/fendo.2024.1399930 -
American Journal of Surgery Jun 2024Secondary adrenal tumors (SATs) are uncommon, and the benefits of adrenalectomy for SATs have not been well-established. A systematic review and meta-analysis were... (Review)
Review
BACKGROUND
Secondary adrenal tumors (SATs) are uncommon, and the benefits of adrenalectomy for SATs have not been well-established. A systematic review and meta-analysis were conducted to assess the survival benefits of adrenalectomy for SATs.
METHOD
ology: A systematic literature search was performed (1990-2022). The inclusion criteria included a known primary tumor with confirmed adrenal metastasis in patients who underwent adrenalectomy. The primary outcome was the overall survival (OS).
RESULTS
A total of 26 studies were included, with 2279 patients. The average age at the time of diagnosis was 61.1 years. Lung cancer was the most common primary tumor. The average time from primary tumor diagnosis to identification of adrenal metastasis was 17 months. The median OS was 35.2 months. One, three, and five-year OS were 79.7 %, 49.1 %, and 37.9 %, respectively.
CONCLUSION
The results of this review provide insight into the long-term survival of patients with SATs who underwent adrenalectomy. The study highlights the need for further research to identify the risk factors that play a role in the outcome of adrenalectomy in patients with SATs.
PubMed: 38945726
DOI: 10.1016/j.amjsurg.2024.115809 -
International Journal of Surgery Case... Jun 2024Adrenal Cavernous Hemangioma is an extremely rare histological type of adrenal tumors, typically asymptomatic and occasionally revealed by a symptom or complication....
INTRODUCTION AND IMPORTANCE
Adrenal Cavernous Hemangioma is an extremely rare histological type of adrenal tumors, typically asymptomatic and occasionally revealed by a symptom or complication. Here, we report an atypical symptomatic case to enrich the limited international case series.
CASE PRESENTATION
We present the case of an 80-year-old woman who underwent laparoscopic left adrenalectomy for a painful and potentially malignant left adrenal neoplasm, leading to the discovery of a five-centimeter adrenal cavernous hemangioma. The post-operative course was uneventful. The postoperative course was uneventful, and the chronic lumbar pain described initially vanished at the six-month follow-up.
CLINICAL DISCUSSION
Adrenal cavernous hemangioma is typically silent and incidentally discovered on cross-sectional imaging. Symptomatic or complicated forms are extremely rare. Clinical, biological, radiological and histology assessment are crucial for management. Therapeutic decisions depend on the malignancy probability and the functional nature of the adrenal neoplasm, considering surgery versus conservative approaches. Patient's point-of-view and background are also determining factors in the decision-making process. Mini-invasive adrenalectomy is superior to open approach, when feasible and safe.
CONCLUSION
Adrenal cavernous hemangioma is a rare benign vascular tumor often discovered on adrenalectomy specimen. This case illustrates a rare cause of chronic lumbar pain. It also underscores the importance of a multidisciplinary medical decision for this kind of tumors.
PubMed: 38945012
DOI: 10.1016/j.ijscr.2024.109936 -
Asian Journal of Surgery Jun 2024
PubMed: 38944603
DOI: 10.1016/j.asjsur.2024.06.094 -
The Surgical Clinics of North America Aug 2024Pheochromocytomas and paragangliomas are distinctive neuroendocrine tumors which frequently produce excess catecholamines with resultant cardiovascular morbidity. These... (Review)
Review
Pheochromocytomas and paragangliomas are distinctive neuroendocrine tumors which frequently produce excess catecholamines with resultant cardiovascular morbidity. These tumors have a strong genetic component, with up to 40% linked to hereditary pathogenic variants; therefore, germline genetic testing is recommended for all patients. Surgical resection offers the only potential cure in the case of localized disease. Given the potential for catecholaminergic crises, appropriate perioperative management is crucial, and all patients should undergo alpha-adrenergic blockade before resection. Therapeutic options for metastatic disease are limited and include surgical debulking, radiopharmaceutical therapies, and conventional chemotherapy.
Topics: Humans; Pheochromocytoma; Adrenal Gland Neoplasms; Paraganglioma; Adrenalectomy
PubMed: 38944505
DOI: 10.1016/j.suc.2024.02.014 -
The Surgical Clinics of North America Aug 2024Hypertension leads to multiple comorbidities and increased risk for mortality. Endocrine disorders contribute to the development of hypertension, including primary... (Review)
Review
Hypertension leads to multiple comorbidities and increased risk for mortality. Endocrine disorders contribute to the development of hypertension, including primary aldosteronism (PA). This article discusses the evaluation and management of PA.
Topics: Hyperaldosteronism; Humans; Adrenalectomy; Hypertension
PubMed: 38944504
DOI: 10.1016/j.suc.2024.02.013 -
The Surgical Clinics of North America Aug 2024Adrenal incidentalomas have increased due to advanced and more sensitive imaging modalities, their increased utilization, and the aging population. Most adrenal... (Review)
Review
Adrenal incidentalomas have increased due to advanced and more sensitive imaging modalities, their increased utilization, and the aging population. Most adrenal incidentalomas are nonfunctional and benign, but a subset of patients has functional and/or malignant tumors. The evaluation of patients with an adrenal incidentaloma involves addressing 2 clinical questions: (1) Is the tumor functional? (2) Is the tumor malignant? A careful history and physical examination focused on signs and symptoms of adrenal functional tumors, biochemical testing, and imaging features are the cornerstone in the evaluation of patients with an adrenal incidentaloma.
Topics: Humans; Adrenal Gland Neoplasms; Incidental Findings; Diagnosis, Differential; Tomography, X-Ray Computed
PubMed: 38944503
DOI: 10.1016/j.suc.2024.02.012 -
European Journal of Endocrinology Jun 2024To assess whether clinical and imaging characteristics predict hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACA).
OBJECTIVE
To assess whether clinical and imaging characteristics predict hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACA).
DESIGN
Single center cohort study.
METHODS
Consecutive adult patients with incidental ACA diagnosed between 2000 and 2016.
RESULTS
Of 1516 patients with incidental ACA (median age 59 years, 62% women), 699 (46%) had nonfunctioning adenomas (NFA), 482 (31%) had mild autonomous cortisol secretion (MACS), 62 (4%) had primary aldosteronism (PA), 39 (3%) had Cushing syndrome (CS), 18 (1%) had PA and MACS (PA-MACS), and 226 (15%) had incomplete workup. Age, sex, tumor size, and tumor laterality, but not unenhanced computed tomography Hounsfield units (HU), were associated with hormonal subtypes. In a multivariable analysis, ≥1cm growth was associated with younger age (odds ratio per 5-year increase, OR=0.8, P=0.0047) and longer imaging follow-up (OR=1.2 per year, P<.0001). Adrenalectomy was performed in 355 (23%) patients, including 38% of MACS and 15% of NFA. Adrenalectomy for NFA and MACS was more common in younger patients (OR=0.79 per 5-year increase, P=0.002), larger initial tumor size (OR=2.3 per 1-cm increase, P<.0001), ≥1cm growth (OR=15.3, P<.0001), and higher post-dexamethasone cortisol (OR=6.6 for >5 vs <1.8 mcg/dL, P=0.002).
CONCLUSIONS
Age, sex, tumor size, and laterality were associated with ACA hormonal subtype and can guide diagnosis and management. Tumor growth was more common with younger age and longer follow-up. Unenhanced HU did not predict hormonal subtype or growth. Adrenalectomy for MACS and NFA was mainly performed in younger patients with larger tumor size, growth, and elevated post-dexamethasone cortisol.
PubMed: 38941271
DOI: 10.1093/ejendo/lvae078 -
World Journal of Surgery Jun 2024
Retraction: Validation of the aldosteronoma resolution score as a predictive resolution score of hypertension after unilateral adrenalectomy for primary aldosteronism in a North-African population.
PubMed: 38938051
DOI: 10.1002/wjs.12266 -
Journal of Clinical Medicine Jun 2024: Leiomyosarcoma (LMS) originating from the adrenal gland is exceedingly rare, constituting a minute fraction of soft tissue sarcomas. Due to its rarity, with less than... (Review)
Review
: Leiomyosarcoma (LMS) originating from the adrenal gland is exceedingly rare, constituting a minute fraction of soft tissue sarcomas. Due to its rarity, with less than 50 documented cases in English medical literature, the diagnosis and management of adrenal LMS remain challenging. The aim of this study was to perform a review of the literature, in order to evaluate the prognosis of these rare cancers and report our specific case. : A systematic review of the literature was conducted using PubMed, Web of Science, Google Scholar, and Scopus databases, up to December 2020. The search utilized MeSH terms such as "Adrenal Gland Neoplasms," "Leiomyosarcoma," "Adrenalectomy," and "Smooth Muscle Tumor." The inclusion criteria focused on studies reporting patients with a histopathological diagnosis of adrenal leiomyosarcoma. The PRISMA guidelines were followed to ensure a comprehensive analysis. : Out of 63 identified studies, 43 met the inclusion criteria and were reviewed. These studies highlighted the rarity and aggressive behavior of adrenal leiomyosarcoma. Surgical excision remains the cornerstone of treatment, often complemented by adjuvant therapies. The reviewed case involved a 52-year-old woman who underwent a right laparoscopic adrenalectomy for a 9 × 7 × 6 cm grade 3 leiomyosarcoma. Despite subsequent adjuvant chemotherapy, hepatic metastases were detected, illustrating the aggressive nature of the disease. The literature underscores the importance of histopathological analysis and long-term surveillance for managing disease progression. : Optimal management of adrenal leiomyosarcoma requires a multidisciplinary approach and meticulous follow-up. The rarity of the disease poses challenges for standardizing treatment, but surgical excision and tailored adjuvant therapies show promise. Further research is essential to refine treatment strategies and improve prognosis for this rare malignancy.
PubMed: 38930027
DOI: 10.3390/jcm13123499