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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 -
The British Journal of Surgery Jun 2024
Development of an ontology for laparoscopic transabdominal adrenalectomy via a comprehensive modified Delphi survey and its validation on a multicentric pilot data set for surgical training and future video analysis with machine learning algorithms.
Topics: Humans; Adrenalectomy; Laparoscopy; Delphi Technique; Machine Learning; Pilot Projects; Video Recording
PubMed: 38916133
DOI: 10.1093/bjs/znae148 -
JCEM Case Reports Jun 2024Pheochromocytomas predominantly produce catecholamines, and rarely also produce ACTH, causing Cushing syndrome (CS). Cyclic CS, an uncommon presentation of...
Pheochromocytomas predominantly produce catecholamines, and rarely also produce ACTH, causing Cushing syndrome (CS). Cyclic CS, an uncommon presentation of hypercortisolism, poses a diagnostic challenge. We report a 71-year-old woman who developed cyclic ectopic ACTH secretion from a pheochromocytoma. Previous evaluations showed intermittent elevations in cortisol and ACTH levels, normal pituitary magnetic resonance imaging, and an adrenal nodule. On admission, she was hypertensive and had cushingoid features. Bilateral inferior petrosal sinus sampling with desmopressin stimulation and an 8-mg dexamethasone suppression test suggested ectopic ACTH secretion, but ACTH increased during the peripheral desmopressin stimulation test. Plasma normetanephrines were about 2-fold above the upper reference limit. F-fluoro-dopa and Gallium-DOTATATE positron emission tomography/computed tomography scans, computed tomography, and magnetic resonance imaging identified an adrenal mass. After doxazosin adrenoceptor blockade, she underwent right adrenalectomy; histopathology and immunohistochemistry confirmed an ACTH-secreting pheochromocytoma. Postoperative blood pressure normalized and serum cortisol and plasma ACTH levels were suppressed, requiring physiologic hydrocortisone replacement. This case underscores the importance of considering pheochromocytoma in ACTH-dependent hypercortisolism with elevated metanephrines and an adrenal mass. Timely diagnosis and treatment can reduce morbidity and improve quality of life.
PubMed: 38915761
DOI: 10.1210/jcemcr/luae094 -
Frontiers in Surgery 2024Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, probably of fibroblastic origin, mainly in the extremities and pleura. Primary SFT of the adrenal gland is...
Solitary fibrous tumor (SFT) is a rare mesenchymal tumor, probably of fibroblastic origin, mainly in the extremities and pleura. Primary SFT of the adrenal gland is clinically more rare. Here, we report the case of a 47-year-old woman who detected a left adrenal mass on physical examination, without any symptoms, and no laboratory abnormalities. A computed tomography (CT) examination of the adrenal gland suggested a round-like soft tissue density shadow in the left adrenal area. An unenhanced scan showed uneven density of the mass, with a scattered circular-like cystic low-density shadow inside, and an enhanced scan showed obvious uneven enhancement. We considered it to be adrenal pheochromocytoma. Ultimately, the patient was treated with laparoscopic left adrenalectomy. A pathological examination suggested an adrenal SFT. We reviewed previous case reports of adrenal SFTs and summarized the clinical characteristics of adrenal SFT combined with the relevant literature. For adrenal tumors with uneven low-density shadow and uneven CT enhancement features, we should consider the differential diagnosis of adrenal SFT.
PubMed: 38903862
DOI: 10.3389/fsurg.2024.1363807 -
SAGE Open Medical Case Reports 2024Adrenal gland cysts are rare and uncommon manifestations. Mostly asymptomatic, discovered incidentally during radiological studies or at autopsy, or without...
Adrenal gland cysts are rare and uncommon manifestations. Mostly asymptomatic, discovered incidentally during radiological studies or at autopsy, or without characteristic symptoms. The spectrum of these entities may include benign cysts or malignant cystic neoplasms. They are classified into four types: pseudocysts, endothelial cysts, epithelial cysts, and parasitic cysts. Though pseudocysts are reported to be the most frequently clinically recognized adrenal cysts in surgical series, endothelial cysts are more frequent in autopsy series. Even with advanced imaging modalities, it is still difficult to differentiate a benign adrenal neoplasm from a malignant one. As a result, getting a definitive diagnosis and starting treatment is challenging. In both symptomatic and asymptomatic cases with a large diameter or increasing sizes during follow-up, or with any abnormality of adrenal hormones, surgery is the treatment of choice in symptomatic. Herein, we present a 47-year-old female with a nonfunctional left adrenal endothelial cyst, who was incidentally found during a computerized tomography scan. The patient presented with left-sided nephritic colic due to renal lithiasis. The objective of this paper is to recall the clinical characteristics and to specify the diagnostic contribution of imaging as well as the therapeutic modalities of this entity.
PubMed: 38881974
DOI: 10.1177/2050313X241261510 -
Cureus May 2024Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic...
Pheochromocytomas are tumors that develop from the chromaffin cells of the adrenal medulla. More than 40% of cases of pheochromocytomas are associated with genetic conditions such as neurofibromatosis type 1 (NF1) or von Hippel-Lindau syndrome. Cystic pheochromocytomas are rare, generally asymptomatic, and thus of bigger size at the time of diagnosis. Surgical treatment is necessary to prevent cardiovascular morbidity and malignancy risk. We report the case of a 27-year-old patient admitted for further examination of a left adrenal mass that was discovered by an abdominal CT scan in the context of abdominal pain associated with hypertension evolving for three years. The clinical examination showed the presence of multiple café au lait spots, axillary and inguinal freckling with two dermal neurofibromas diagnosed clinically, as well as Lisch nodules on bilateral ophthalmic examination, thus meeting the clinical criteria for the diagnosis of NF1. The clinical laboratory investigation showed elevated urinary metanephrine and normetanephrine levels. CT scan examination showed a 10 cm left adrenal cystic mass on abdominal CT. This mass uptake of the radioligand in metaiodobenzylguanidine (MIBG) scintigraphy without secondary extra-adrenal localization allowed the diagnosis of a seemingly benign cystic pheochromocytoma to be made. The patient was put on presurgical drug preparation with volume expansion and then underwent left unilateral adrenalectomy. The histopathological study was in favor of a rather aggressive cystic pheochromocytoma with a pheochromocytoma of the adrenal gland scaled (PASS) score of 9. Blood pressure and urine catecholamines at seven days, three months, six months, and one year after surgery were normalized. Cystic pheochromocytoma is a rare tumor with a potentially poor prognosis. It is characterized by a more insidious evolution and a larger volume at diagnosis. It should be considered a diagnosis in patients with a cystic adrenal mass or an extra-adrenal mass with fluctuating blood pressure during surgery. This case illustrates the importance of both presurgical preparation and screening for pheochromocytoma in neurofibromatosis type 1.
PubMed: 38864044
DOI: 10.7759/cureus.60151 -
International Journal of Surgery Case... Jul 2024Adrenocortical tumors (ACTs) are rare endocrine neoplasms in children, with functional ACTs being more prevalent than non-functional types. Clinical manifestations...
INTRODUCTION
Adrenocortical tumors (ACTs) are rare endocrine neoplasms in children, with functional ACTs being more prevalent than non-functional types. Clinical manifestations typically include virilization, Cushing's syndrome, and hyperaldosteronism. Surgical intervention is the primary treatment for ACTs, with a significant risk of recurrence in adrenocortical carcinoma even after complete resection.
PRESENTATION OF CASE
This case presentation describes a 3.5-year-old female with generalized hirsutism and clitoral hypertrophy, leading to the discovery of a left adrenal tumor. The child underwent adrenalectomy, revealing a benign adrenal cortical adenoma. Unfortunately, due to loss of follow-up, the child later presented with pulmonary metastases and passed away, preventing further investigation into the source of metastases.
DISCUSSION
Adrenocortical tumors are uncommon in children, with the classification of ACTs into adenomas and carcinomas. To our knowledge, this is the third case of an adrenocortical tumor in a child in Syria. We highlight the challenges in managing pediatric ACTs and emphasize the importance of timely intervention and close monitoring to improve outcomes. Regular follow-up is crucial to detect complications early and optimize treatment strategies, especially considering the unpredictable behavior of these tumors.
CONCLUSION
This case confirms that distinguishing between adrenocortical adenoma and carcinoma can be challenging even histologically. Therefore, it is necessary to follow up after treating each case of adenoma in a child to prevent major complications.
PubMed: 38852562
DOI: 10.1016/j.ijscr.2024.109878 -
Cureus May 2024Renal cell carcinoma (RCC) has a high metastatic potential. While metastasis to common sites like the lungs, liver, bones, and brain is well-documented, metastasis to...
Renal cell carcinoma (RCC) has a high metastatic potential. While metastasis to common sites like the lungs, liver, bones, and brain is well-documented, metastasis to the colon, particularly the descending colon, remains an uncommon occurrence. When RCC does metastasize to the gastrointestinal tract, it commonly spreads to the small bowel and stomach. There are few cases reported in literature involving RCC metastasis to the colon. The commonly affected areas within the colon include the rectosigmoid colon, splenic flexure, and transverse colon. We describe an 87-year-old male with a history of stage III RCC diagnosed three years ago, followed by left-sided nephroureterectomy, partial adrenalectomy, and perinephric lymph node dissection. He presented to the emergency department (ED) with melena and generalized abdominal pain for one week. Stool occult blood was positive. Computed tomography (CT) of the abdomen was significant for stable postsurgical changes related to prior left nephrectomy and colonic mass at the proximal descending colon. A colonoscopy revealed a necrotic appearing friable mass in the descending colon. The pathology of the mass revealed proliferated atypical cells positive for paired box 8 (PAX8), a cluster of differentiation 10 (CD10), RCC, and pan-cytokeratin and negative for caudal-type homeobox 2 (CDX2), thyroid transcription factor-1 (TTF-1), and a cluster of differentiation 68 (CD68), consistent with metastatic RCC.
PubMed: 38841042
DOI: 10.7759/cureus.59756 -
Cureus Jun 2024We report a case of a 22-year-old female with pedal edema, hypokalemia, and hypertension. On suspicion of hyperaldosteronism, further workup was pursued, which only...
We report a case of a 22-year-old female with pedal edema, hypokalemia, and hypertension. On suspicion of hyperaldosteronism, further workup was pursued, which only revealed a low serum adrenocorticotropic hormone (ACTH) and an inappropriately normal cortisol level after a 1-mg dexamethasone suppression test, suggestive of primary hypercortisolism. CT of the chest, abdomen, and pelvis revealed a left adrenal mass. Based on the clinical findings and biochemical abnormalities, we were expecting this tumor to be aldosterone-secreting, but both serum aldosterone and renin levels were normal in our patient. Eventual surgical resection confirmed initial suspicions of malignancy, as it was found to be adrenal cortical carcinoma. This case highlights the unusual presentation of this rare but aggressive endocrinologic neoplasm and the importance of its prompt diagnosis and treatment.
PubMed: 38827415
DOI: 10.7759/cureus.61481 -
Innovative Surgical Sciences Mar 2024Since its introduction as a clinical technique, robotic surgery has been extended to different fields of surgery. However, the indications as well as the number of...
OBJECTIVES
Since its introduction as a clinical technique, robotic surgery has been extended to different fields of surgery. However, the indications as well as the number of robotic procedures varied in different institutions. The aim of this investigation was to evaluate the current use of robotic surgery in general and digestive surgery in Switzerland.
METHODS
All Swiss surgical departments that are recognized training institutes for postgraduate training in surgery by the Swiss Institute of Medical Education (SIWF) were queried with a detailed questionnaire regarding the use of robotic surgery techniques and were analyzed regarding hospital size and type of hospital.
RESULTS
Ninety-three departments were queried, and 67 % (n=63) answered the survey. Fifty-eight were public, and five were private institutions. Seventeen (26 %) of the queried departments used robotic surgery in digestive surgery. Four out of 17 (23 %) of the departments that performed robotic surgery were private hospitals, while 13 (77 %) were public institutions. In the majority of departments, robotic surgery of the rectum (n=12; 70.6 %) and colon (n=11; 64.7 %) was performed, followed by hernia procedures (n=8; 47.1 %) and fundoplication (n=7; 41.2 %). Less frequently, pancreatic resections (n=5; 29.4 %), cholecystectomy (n=4; 23.5 %), adrenalectomy (n=4; 23.5 %), gastric bypass (n=3; 17.7 %), gastric sleeve (n=3; 17.7 %), hepatic procedures (n=2; 11.7 %), or small bowel resections (n=1; 5.9 %) were performed as robotic procedures. More than 25 procedures per year per department were performed for hernia surgery (n=5 departments), gastric bypass (n=2 departments), cholecystectomy, fundoplication, and colon surgery (each n=1 department).
CONCLUSIONS
The number and range of robotic procedures performed in Switzerland varied widely. Higher accreditation for general surgery or subspecialization of visceral surgery of the department was positively associated with the use of robotic techniques, reflecting an unequal availability of robotic surgery.
PubMed: 38826632
DOI: 10.1515/iss-2023-0052