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Cureus Feb 2024Gastrointestinal stromal tumors (GISTs) represent a rare form of gastrointestinal neoplasm. This report details a medical case involving a 44-year-old woman who...
Gastrointestinal stromal tumors (GISTs) represent a rare form of gastrointestinal neoplasm. This report details a medical case involving a 44-year-old woman who underwent bilateral pheochromocytoma resection, GIST gastrectomy, and laparoscopic adrenalectomy with intestinal resection. Despite an initially positive response to oral imatinib, treatment was delayed due to economic constraints. This delay resulted in a critical event marked by abdominal GIST metastasis to the abdominal wall, subsequent rupture leading to hemoperitoneum, and emergency surgery. Following an adequate postsurgical recovery, she was successfully discharged prior to medication adjustments.
PubMed: 38516470
DOI: 10.7759/cureus.54532 -
Reports of Practical Oncology and... 2023The objective was to retrospectively evaluate the contribution of fluorodeoxyglucose [F] positron emission tomography/computed tomography (FDG-PET/CT) to the re-staging...
BACKGROUND
The objective was to retrospectively evaluate the contribution of fluorodeoxyglucose [F] positron emission tomography/computed tomography (FDG-PET/CT) to the re-staging of adrenocortical carcinoma (ACC).
MATERIALS AND METHODS
A total of 16 patients (10 males and 6 females), who underwent adrenalectomy due to adrenocortical carcinoma and FDG-PET/CT scan to re-stage the tumor between July 2007 and April 2013, were included in the present study. The mean age was 53.37 ± 13.91 years (min: 30, max: 74) The patients were required to fast for six hours prior to scanning, and whole-body PET scanning from the skull base to the upper thighs was performed approximately 1 h after the intravenous injection of 555 MBq of F-18 FDG. Whole body CT scanning was performed in the cranio-caudal direction. FDG-PET images were reconstructed using CT data for attenuation correction. Suspicious recurrent or metastatic lesions were confirmed by histopathology or clinical follow-up.
RESULTS
Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of FDG-PET/CT were 100%, 83.3%, 90.9%, 83.3%, and 93.7%, respectively.
CONCLUSION
FDG-PET/CT detects local recurrence and/or distant metastases with high accuracy in the re-staging of operated adrenocortical carcinoma. It is considered that the procedure could play an important role in treatment decision after the operation and post-operative follow-up and could influence the entire decision-making process.
PubMed: 38515818
DOI: 10.5603/rpor.98727 -
Frontiers in Endocrinology 2024Double somatic mutations in and have recently been identified in a small subset of aldosterone-producing adenomas (APAs). As a possible pathogenesis of APA due to...
Double somatic mutations in and have recently been identified in a small subset of aldosterone-producing adenomas (APAs). As a possible pathogenesis of APA due to these mutations, an association with pregnancy, menopause, or puberty has been proposed. However, because of its rarity, characteristics of APA with these mutations have not been well characterized. A 46-year-old Japanese woman presented with hypertension and hypokalemia. She had two pregnancies in the past but had no history of pregnancy-induced hypertension. She had regular menstrual cycle at presentation and was diagnosed as having primary aldosteronism after endocrinologic examinations. Computed tomography revealed a 2 cm right adrenal mass. Adrenal venous sampling demonstrated excess aldosterone production from the right adrenal gland. She underwent right laparoscopic adrenalectomy. The resected right adrenal tumor was histologically diagnosed as adrenocortical adenoma and subsequent immunohistochemistry (IHC) revealed diffuse immunoreactivity of aldosterone synthase (CYP11B2) and visinin like 1, a marker of the zona glomerulosa (ZG), whereas 11β-hydroxylase, a steroidogenic enzyme for cortisol biosynthesis, was mostly negative. CYP11B2 IHC-guided targeted next-generation sequencing identified somatic (p.D32Y) and (p.Q209H) mutations. Immunofluorescence staining of the tumor also revealed the presence of activated β-catenin, consistent with features of the normal ZG. The expression patterns of steroidogenic enzymes and related proteins indicated ZG features of the tumor cells. PA was clinically and biochemically cured after surgery. In conclusion, our study indicated that and -mutated APA has characteristics of the ZG. The disease could occur in adults with no clear association with pregnancy or menopause.
Topics: Adult; Female; Pregnancy; Humans; Middle Aged; Adrenocortical Adenoma; Aldosterone; Cytochrome P-450 CYP11B2; beta Catenin; Hyperaldosteronism; Adenoma; Hypertension; Mutation; GTP-Binding Protein alpha Subunits
PubMed: 38505749
DOI: 10.3389/fendo.2024.1286297 -
International Journal of Surgery Case... Apr 2024Pheochromocytomas are rare catecholamine-secreting tumors with a high potential for recurrence post-surgery, necessitating prolonged follow-up. This case highlights the...
INTRODUCTION
Pheochromocytomas are rare catecholamine-secreting tumors with a high potential for recurrence post-surgery, necessitating prolonged follow-up. This case highlights the diagnostic and therapeutic challenges in managing recurrent pheochromocytoma.
CASE PRESENTATION
A 25-year-old female, with a history of left pheochromocytoma treated with adrenalectomy a decade earlier, presented with a right adrenal mass. Despite controlled hypertension, elevated urine metanephrines suggested recurrence. Imaging showed a right adrenal mass and suspicious left paraaortic lymph nodes, confirming the diagnosis of recurrent malignant pheochromocytoma in the left adrenal bed and right adrenal gland, with metastasis to the paraaortic lymph nodes. The patient underwent right adrenalectomy coupled with cytoreductive surgery (CRS) in the form of excision of left-sided adrenal bed recurrence and left paraaortic lymph node dissection, intraoperative radiation therapy (IORT), and hyperthermic intraperitoneal chemotherapy (HIPEC). On follow-up six years later, the patient remains free from recurrence.
DISCUSSION
This case illustrates the importance of continued surveillance in pheochromocytoma patients, even those with a low-risk profile. The recurrence in this case, despite a smaller initial tumor size and no genetic predispositions, underscores the unpredictable nature of pheochromocytomas. The successful management with CRS, IORT, and HIPEC emphasizes the need for a personalized and multifaceted treatment approach.
CONCLUSION
Pheochromocytoma patients, including those initially considered low risk, require long-term monitoring due to the risk of recurrence. The utilization of CRS, IORT, and HIPEC in this case was pivotal in managing the recurrent and metastatic malignant disease effectively, demonstrating the significance of a comprehensive, multidisciplinary treatment strategy in such complex cases.
PubMed: 38503158
DOI: 10.1016/j.ijscr.2024.109504 -
Frontiers in Endocrinology 2024The modified three-level technique for retroperitoneal laparoscopic adrenalectomy (RLA) has proven beneficial in the treatment of adrenal lesions in patients with...
OBJECTIVES
The modified three-level technique for retroperitoneal laparoscopic adrenalectomy (RLA) has proven beneficial in the treatment of adrenal lesions in patients with BMI≥25 Kg/m. This paper aims to summarize our institution's seven-year experience using this technique for all patients with Adrenal Lesions ≤ 6cm.
PATIENTS AND METHODS
Between January 2016 and December 2022. The patients underwent laparoscopic adrenal surgery were categorized into Zhang's technique (ZT) (Three-level Technique) group and modified technique (MT) group. The fundamental characteristics and perioperative data were analyzed, with statistical significance set at p<0.05.
RESULTS
In total, 731 patients were stratified into two groups: ZT (n=448) and MT (n=283). Statistically significant distinctions were not detected between the two groups regarding sex, BMI, tumor location, tumor size, tumor type, or American Society of Anesthesiologists (ASA) score (p>0.05). The MT group demonstrated superior outcomes compared to the ZT group in terms of operative time, estimated blood loss, drainage volume, diet recovery time, complication rates, and postoperative hospitalization duration (p<0.05). 17 (4.34%) in the ZT group required unplanned adrenalectomy, while there was none in MT group (P<0.05).
CONCLUSION
MT retroperitoneal laparoscopic adrenalectomy has demonstrated its benefits in the treatment of adrenal lesions across all patients with adrenal lesions ≤ 6cm, serving as a valuable point of reference for the surgical management of adrenal diseases.
PATIENT SUMMARY
We have made modifications to the classic retroperitoneal laparoscopic adrenalectomy and achieved superior surgical outcomes, resulting in a procedure known as modified retroperitoneal laparoscopic adrenalectomy. This technique is suitable for both obese individuals and the general population with adrenal lesions ≤ 6cm.
Topics: Humans; Retrospective Studies; Adrenal Gland Neoplasms; Adrenalectomy; Retroperitoneal Space; Laparoscopy
PubMed: 38501101
DOI: 10.3389/fendo.2024.1342240 -
Journal of Medical Case Reports Mar 2024This report presents a case of cannabinoid-induced hyperemesis syndrome causing repeated violent retching in a patient with a large (8 cm) adrenal pheochromocytoma...
BACKGROUND
This report presents a case of cannabinoid-induced hyperemesis syndrome causing repeated violent retching in a patient with a large (8 cm) adrenal pheochromocytoma resulting in hypertensive urgency.
CASE PRESENTATION
A 69-year-old white male patient with a previously diagnosed pheochromocytoma presented to the emergency department with nausea and vomiting and was found to have hypertensive urgency. Computed tomography scan did not show any acute abdominal pathology and history was inconsistent with a gastrointestinal etiology. Patient had a history of daily cannabinoid use for many years and repeated self-limited hyperemesis episodes, and thus a diagnosis of cannabinoid-induced hyperemesis syndrome was made. It was concluded that the likely explanation for the hypertensive urgency was from physical compression of his adrenal tumor during the episodes of retching resulting in a catecholamine surge. The patient was given antiemetics and admitted to the intensive care unit for blood pressure management. Blood pressure was initially controlled with phentolamine and a clevidipine infusion, then transitioned to oral doxazosin and phenoxybenzamine. Hyperemesis and abdominal pain resolved after 24 hours, and his blood pressure returned to baseline. The patient was discharged with the recommendation to stop all cannabis use. On follow-up, his blood pressure remained well controlled, and he subsequently underwent adrenalectomy for tumor removal.
CONCLUSION
Hyperemesis can cause hypertensive events in patients with pheochromocytoma by increasing abdominal pressure, leading to catecholamine release.
Topics: Aged; Humans; Male; Adrenal Gland Neoplasms; Cannabinoid Hyperemesis Syndrome; Cannabinoids; Catecholamines; Hypertensive Crisis; Pheochromocytoma; Vomiting
PubMed: 38500192
DOI: 10.1186/s13256-024-04497-0 -
The Journal of International Medical... Mar 2024Primary adrenal epithelioid sarcoma is a rare lesion of the adrenal gland, and only seven cases have been reported in the domestic and international literature to date.... (Review)
Review
Primary adrenal epithelioid sarcoma is a rare lesion of the adrenal gland, and only seven cases have been reported in the domestic and international literature to date. We herein report a case involving a 65-year-old man with primary adrenal epithelioid sarcoma. After being admitted to the hospital with an adrenal mass found on physical examination, the patient underwent laparoscopic right adrenalectomy. Postoperative pathological findings indicated an epithelioid sarcoma (proximal type). Primary adrenal epithelioid sarcoma is a rare malignancy. Diagnosis is challenging and relies on histopathology and immunohistochemical staining.
Topics: Male; Humans; Aged; Biomarkers, Tumor; Immunohistochemistry; Sarcoma
PubMed: 38497120
DOI: 10.1177/03000605241233145 -
Current Problems in Cardiology Jun 2024Fulminant myocarditis (FM) is a rare illness characterized by abrupt and severe widespread cardiac inflammation, which frequently results in mortality due to cardiogenic... (Review)
Review
A multidisciplinary approach of a patient with pheochromocytoma presenting as recurrent acute fulminant myocarditis: A rare case report and comprehensive review of literature.
Fulminant myocarditis (FM) is a rare illness characterized by abrupt and severe widespread cardiac inflammation, which frequently results in mortality due to cardiogenic shock, ventricular arrhythmias, or multiorgan system failure. Pheochromocytoma is an uncommon and difficult-to-diagnose cause of FM, and it is associated with a significant risk of recurrent acute myocarditis. There is, however, little information on reoccurring acute FM. Herein, we report a rare case of recurrent acute FM due to pheochromocytoma. We present the case of a 22-year-old woman who was admitted to our hospital three days previously with acute dyspnea. Five months prior, the patient was diagnosed with post-acute myocarditis, and a massive tumor on the right adrenal gland was discovered, which lead to pheochromocytoma diagnosis. In this present admission, following the exclusion of infection, autoimmune, and metabolic derangements, pheochromocytoma was presumed to be the reason for the recurrence and more severe acute FM during the current hospitalization. The patient responded favorably to high-dose steroids combined with heart failure therapy regimens. To detect recurrent acute myocarditis related to pheochromocytoma, a multidisciplinary approach was used, including several laboratory biomarkers and imaging findings. Following pheochromocytoma removal and biopsy, the patient recovered satisfactorily. Our findings may provide beneficial contributions to the literature as pheochromocytoma is an uncommon but important cause of recurrent acute myocarditis. A multidisciplinary approach is essential in identifying acute FM and determining the underlying causes of this malady.
Topics: Humans; Pheochromocytoma; Myocarditis; Female; Adrenal Gland Neoplasms; Young Adult; Recurrence; Acute Disease; Tomography, X-Ray Computed; Adrenalectomy
PubMed: 38492617
DOI: 10.1016/j.cpcardiol.2024.102521 -
Endocrinology, Diabetes & Metabolism Mar 2024To present a case series of Cushing's syndrome (CS) during pregnancy caused by adrenocortical adenomas, highlighting clinical features, hormonal assessments and outcomes.
PURPOSE
To present a case series of Cushing's syndrome (CS) during pregnancy caused by adrenocortical adenomas, highlighting clinical features, hormonal assessments and outcomes.
METHODS
We describe five pregnant women with CS, detailing clinical presentations and laboratory findings.
RESULTS
Common clinical features included a full moon face, buffalo back and severe hypertension. Elevated blood cortisol levels with circadian rhythm disruption and suppressed adrenocorticotrophic hormone (ACTH) levels were observed. Imaging revealed unilateral adrenal tumours. Two cases underwent laparoscopic adrenalectomies during the second trimester, while three had postpartum surgery. All required hormone replacement therapy, with postoperative pathological confirmation of adrenocortical adenomas.
CONCLUSION
Diagnosis of CS during pregnancy is challenging due to overlapping features with normal pregnancy: elevated blood cortisol levels and abnormal diurnal rhythm of blood cortisol, suppressed aid diagnosis. Treatment should be individualised due to a lack of explicit optimum therapeutic approaches. Laparoscopic adrenalectomy may be an optimal choice, along with multidisciplinary management including hormone replacement therapy.
Topics: Female; Humans; Pregnancy; Cushing Syndrome; Adrenocortical Adenoma; Hydrocortisone; Adrenalectomy
PubMed: 38475883
DOI: 10.1002/edm2.474 -
Open Life Sciences 2024The pheochromocytoma is an uncommon endocrine neoplasm that originates from chromaffin cells and causes significant cardiovascular effects through the intermittent or...
The pheochromocytoma is an uncommon endocrine neoplasm that originates from chromaffin cells and causes significant cardiovascular effects through the intermittent or sustained release of catecholamines. In this report, we present a rare case of myocardial infarction (MI) induced by pheochromocytoma. A 53-year-old female presented to the emergency department with a history of intermittent palpitations, back pain, and sweating for over 10 years, which had worsened over the past 2 days. The patient's cardiac enzymes and troponin levels were significantly elevated, and the electrocardiogram (ECG) showed ST-segment elevation, leading to an initial diagnosis of acute myocardial infarction. Echocardiography revealed apical ballooning, indicative of stress cardiomyopathy. Emergency coronary angiography revealed no significant stenosis, and the patient's blood pressure was fluctuating. Computerized tomography (CT) scan of the adrenal gland revealed a bilateral adrenal mass, with the left adrenal mass being larger in size after contrast-enhanced CT scan. The patient's left adrenal gland was successfully removed through laparoscopic adrenalectomy, and histopathology results confirmed the presence of adrenal pheochromocytoma. Follow-up for 3 months after discharge showed the patient had no symptoms and good prognosis. The abnormal findings on echocardiography and ECG resolved. Prompt diagnosis and management of pheochromocytoma are crucial for a favorable prognosis.
PubMed: 38465332
DOI: 10.1515/biol-2022-0830