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1.
PLoS One ; 19(5): e0303623, 2024.
Article in English | MEDLINE | ID: mdl-38805424

ABSTRACT

BACKGROUND: Pheochromocytoma, or paraganglioma (PPGL), is a tumor that arises from catecholamine-producing chromaffin cells of the adrenal medulla or paraganglion. Systemic therapy, such as the combination of cyclophosphamide, vincristine, and dacarbazine or therapeutic radiopharmaceuticals such as [131I] meta-iodobenzylguanidine (MIBG), may be administered in cases of locally advanced tumors or distant metastases. However, the current therapies are limited in terms of efficacy and implementation. [211At] meta-astatobenzylguanidine (MABG) is an alpha-emitting radionuclide-labeled ligand that has demonstrated remarkable tumor-reducing effects in preclinical studies, and is expected to have a high therapeutic effect on pheochromocytoma cells. METHODS: We are currently conducting an investigator-initiated first-in-human clinical trial to evaluate the pharmacokinetics, safety, and efficacy of [211At] MABG. Patients with locally unresectable or metastatic PPGL refractory to standard therapy and scintigraphically positive [123I] MIBG aggregation are being recruited, and a 3 + 3 dose escalation design was adopted. The initial dose of [211At] MABG is 0.65 MBq/kg, with a dose escalation in a 1:2:4 ratio in each cohort. Dose-limiting toxicity is observed for 6 weeks after a single bolus dose of [211At] MABG, and the patients are observed for 3 months to explore safety and efficacy profiles. The primary endpoint is dose-limiting toxicity to determine both maximum tolerated and recommended doses. The secondary endpoints include radiopharmacokinetics, urinary radioactive excretion rate, urinary catecholamine response rate, objective response rate, progression free survival, [123I] MIBG scintigraphy on reducing tumor accumulation, and quality of life. TRIALS REGISTRATION: jRCT2021220012 registered on 17 June 2022.


Subject(s)
Adrenal Gland Neoplasms , Paraganglioma , Pheochromocytoma , Radiopharmaceuticals , Adult , Aged , Female , Humans , Male , Middle Aged , Adrenal Gland Neoplasms/drug therapy , Adrenal Gland Neoplasms/diagnostic imaging , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/metabolism , Guanidines/pharmacokinetics , Guanidines/therapeutic use , Paraganglioma/drug therapy , Paraganglioma/pathology , Paraganglioma/diagnostic imaging , Paraganglioma/metabolism , Pheochromocytoma/drug therapy , Pheochromocytoma/diagnostic imaging , Pheochromocytoma/pathology , Pheochromocytoma/metabolism , Radiopharmaceuticals/pharmacokinetics , Treatment Outcome , Clinical Trials, Phase I as Topic
2.
Kaohsiung J Med Sci ; 40(6): 583-588, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38747189

ABSTRACT

Pheochromocytoma is a tumor that usually originating from adrenal medullary chromaffin cells and producing one or more catecholamines, can manifest as hereditary or sporadic. While the majority pheochromocytomas are sporadic, hereditary forms are often associated with genetic syndromes such as von Hippel-Lindau, multiple endocrine neoplasia type 2, and neurofibromatosis type 1. This study aims to analyze data from our series of surgically excited pheochromocytoma patients and compare the characteristics between hereditary and sporadic cases. We retrospectively evaluated 33 diagnosed pheochromocytoma patients, documenting clinical features, surgical complications, and tumor characteristics in both hereditary and sporadic cases. Among the patients, 21% (7 individuals) had hereditary pheochromocytoma, while 79% (26 individuals) had sporadic cases. During diagnosis, hereditary pheochromocytoma patients exhibited a significantly lower mean age compared to the sporadic group (26.4 ± 9.9 years vs. 50.4 ± 14.0 years; p < 0.001). The maximum tumor size was also lower in hereditary cases compared to sporadic cases (p = 0.004). Adrenal tumor localization analysis showed that 63.6% were right-sided, 24.2% were left-sided, and 12.1% were bilateral. Laboratory analysis revealed significantly higher urinary norepinephrine levels in hereditary pheochromocytoma patients (p = 0.021). Our findings suggest that hereditary pheochromocytoma cases are characterized by a younger age at diagnosis, smaller tumor size, and a higher prevalence of multiple bilateral adrenal adenomas. We recommend genetic testing for all pheochromocytoma patients, particularly those with early-onset disease and bilateral adrenal tumors.


Subject(s)
Adrenal Gland Neoplasms , Pheochromocytoma , Humans , Pheochromocytoma/genetics , Pheochromocytoma/surgery , Pheochromocytoma/pathology , Female , Male , Adult , Middle Aged , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Retrospective Studies , Adolescent , Young Adult , Aged
3.
J Med Case Rep ; 18(1): 258, 2024 May 24.
Article in English | MEDLINE | ID: mdl-38783354

ABSTRACT

BACKGROUND: Angiosarcoma of the adrenal gland is a very rare malignant vascular neoplasm. The clinical symptoms are atypical or completely absent. Angiosarcomas of the adrenal gland are therefore often discovered incidentally, and the diagnosis is made histologically after resection. CASE PRESENTATION: A 46-year-old white Spanish male who was a previous smoker and nondrinker and was slightly overweight (92 kg, 176 cm, body mass index 29.7 kg/m2) with no relevant medical history presented to the internal medicine emergency department of our hospital with an unclear 12 cm tumor of the right adrenal gland. Prior to the computed tomography scan, he had had persistent evening fevers for 4 months and unintentional weight loss of 5 kg. The laboratory results showed anemia and an elevated C-reactive protein, but no hormone production. We performed an open adrenalectomy of the right adrenal gland. Finally, the histologic findings revealed an angiosarcoma of the adrenal gland. CONCLUSION: Even though angiosarcomas of the adrenal gland are rare, the differential diagnosis of an angiosarcoma should be considered if a malignant tumor of the adrenal gland is suspected. Treatment decisions should be made on an interdisciplinary basis and preferably in a specialized center. Owing to the rarity of angiosarcomas of the adrenal gland, it is necessary to continue to share clinical experience to gain a better understanding of this particular tumor entity.


Subject(s)
Adrenal Gland Neoplasms , Adrenalectomy , Fever , Hemangiosarcoma , Humans , Male , Hemangiosarcoma/diagnosis , Hemangiosarcoma/surgery , Hemangiosarcoma/pathology , Middle Aged , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/pathology , Fever/etiology , Diagnosis, Differential , Tomography, X-Ray Computed , Recurrence
4.
PLoS One ; 19(5): e0303138, 2024.
Article in English | MEDLINE | ID: mdl-38722890

ABSTRACT

Human T-cell leukemia virus type I (HTLV-I) is an oncogenic virus whose infection can cause diverse diseases, most notably adult T-cell leukemia/lymphoma (ATL or ATLL), an aggressive and fatal malignancy of CD4 T cells. The oncogenic ability of HTLV-I is mostly attributed to the viral transcriptional transactivator Tax. Tax alone is sufficient to induce specific tumors in mice depending on the promotor used to drive Tax expression, thereby being used to understand HTLV-I tumorigenesis and model the tumor types developed in Tax transgenic mice. Tax exerts its oncogenic role predominantly by activating the cellular transcription factor NF-κB. Here, we report that genetic deletion of NF-κB1, the prototypic member of the NF-κB family, promotes adrenal medullary tumors but suppresses neurofibromas in mice with transgenic Tax driven by the HTLV-I Long Terminal Repeat (LTR) promoter. The adrenal tumors are derived from macrophages. Neoplastic macrophages also infiltrate the spleen and lymph nodes, causing splenomegaly and lymphadenopathy in mice. Nevertheless, the findings could be human relevant, because macrophages are important target cells of HTLV-I infection and serve as a virus reservoir in vivo. Moreover, the spleen, lymph nodes and adrenal glands are the most common sites of tumor cell infiltration in HTLV-I-infected patients. These data provide new mechanistic insights into the complex interaction between Tax and NF-κB, therefore improving our understanding of HTLV-I oncogenic pathogenesis. They also expand our knowledge and establish a new animal model of macrophage neoplasms and adrenal tumors.


Subject(s)
Gene Products, tax , Human T-lymphotropic virus 1 , Macrophages , Animals , Humans , Mice , Adrenal Gland Neoplasms/virology , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/metabolism , Gene Products, tax/metabolism , Gene Products, tax/genetics , Human T-lymphotropic virus 1/genetics , Human T-lymphotropic virus 1/pathogenicity , Macrophages/metabolism , Macrophages/virology , Mice, Transgenic , NF-kappa B p50 Subunit/metabolism , NF-kappa B p50 Subunit/genetics , Terminal Repeat Sequences/genetics
5.
Sci Rep ; 14(1): 11209, 2024 05 16.
Article in English | MEDLINE | ID: mdl-38755394

ABSTRACT

Adrenal gland incidentaloma is frequently identified through computed tomography and poses a common clinical challenge. Only selected cases require surgical intervention. The primary aim of this study was to compare the effectiveness of selected machine learning (ML) techniques in proper qualifying patients for adrenalectomy and to identify the most accurate algorithm, providing a valuable tool for doctors to simplify their therapeutic decisions. The secondary aim was to assess the significance of attributes for classification accuracy. In total, clinical data were collected from 33 patients who underwent adrenalectomy. Histopathological assessments confirmed the proper selection of 21 patients for surgical intervention according to the guidelines, with accuracy reaching 64%. Statistical analysis showed that Supported Vector Machines (linear) were significantly better than the baseline (p < 0.05), with accuracy reaching 91%, and imaging features of the tumour were found to be the most crucial attributes. In summarise, ML methods may be helpful in qualifying patients for adrenalectomy.


Subject(s)
Adrenal Gland Neoplasms , Adrenalectomy , Machine Learning , Humans , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/diagnostic imaging , Male , Adrenalectomy/methods , Female , Middle Aged , Aged , Tomography, X-Ray Computed , Adult , Algorithms
6.
Sci Rep ; 14(1): 12174, 2024 05 28.
Article in English | MEDLINE | ID: mdl-38806567

ABSTRACT

Robotic adrenalectomy (RA) has gained significant popularity in the management of adrenal gland diseases. We report our experience at a single tertiary institution and evaluate the safety and surgical outcomes of RA. The data of 122 consecutive patients who underwent RA from October 2009 to December 2022 at Korea University Anam Hospital (Seoul, Korea) were reviewed. There were no perioperative complications. Clinicopathological features and surgical outcomes were retrospectively analyzed through complete chart reviews. Noteworthy findings include the influence of sex, tumor size, and body mass index on operation time, with the female and small tumor groups exhibiting shorter operation times (P = 0.018 and P = 0.009, respectively). Pheochromocytoma was identified as a significant independent risk factor for a longer operation time in the multivariate analysis [odds ratio (OR), 3.709; 95% confidence interval (CI), 1.127-12.205; P = 0.031]. A temporal analysis revealed a decreasing trend in mean operation times across consecutive groups, reflecting a learning curve associated with RA adoption. RA is a safe and effective operative technique alternative to laparoscopic adrenalectomy that has favorable surgical outcomes and enhances the convenience of the operation.


Subject(s)
Adrenal Gland Neoplasms , Adrenalectomy , Operative Time , Robotic Surgical Procedures , Humans , Female , Adrenalectomy/methods , Adrenalectomy/adverse effects , Male , Robotic Surgical Procedures/methods , Robotic Surgical Procedures/adverse effects , Middle Aged , Retrospective Studies , Treatment Outcome , Adult , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Aged , Pheochromocytoma/surgery , Pheochromocytoma/pathology , Risk Factors , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Laparoscopy/methods , Laparoscopy/adverse effects
7.
Int J Mol Sci ; 25(10)2024 May 15.
Article in English | MEDLINE | ID: mdl-38791437

ABSTRACT

Gap junctions (GJs) are important in the regulation of cell growth, morphology, differentiation and migration. However, recently, more attention has been paid to their role in the pathogenesis of different diseases as well as tumorigenesis, invasion and metastases. The expression pattern and possible role of connexins (Cxs), as major GJ proteins, under both physiological and pathological conditions in the adrenal gland, were evaluated in this review. The databases Web of Science, PubMed and Scopus were searched. Studies were evaluated if they provided data regarding the connexin expression pattern in the adrenal gland, despite current knowledge of this topic not being widely investigated. Connexin expression in the adrenal gland differs according to different parts of the gland and depends on ACTH release. Cx43 is the most studied connexin expressed in the adrenal gland cortex. In addition, Cx26, Cx32 and Cx50 were also investigated in the human adrenal gland. Cx50 as the most widespread connexin, along with Cx26, Cx29, Cx32, Cx36 and Cx43, has been expressed in the adrenal medulla with distinct cellular distribution. Considerable effort has recently been directed toward connexins as therapeutically targeted molecules. At present, there exist several viable strategies in the development of potential connexin-based therapeutics. The differential and hormone-dependent distribution of gap junctions within adrenal glands, the relatively large gap junction within this gland and the increase in the gap junction size and number following hormonal treatment would indicate that gap junctions play a pivotal role in cell functioning in the adrenal gland.


Subject(s)
Connexins , Gap Junctions , Humans , Connexins/metabolism , Gap Junctions/metabolism , Adrenal Gland Neoplasms/metabolism , Adrenal Gland Neoplasms/pathology , Carcinogenesis/metabolism , Carcinogenesis/pathology , Adrenal Glands/metabolism , Adrenal Glands/pathology , Animals , Gene Expression Regulation, Neoplastic
8.
BMC Urol ; 24(1): 90, 2024 Apr 18.
Article in English | MEDLINE | ID: mdl-38637748

ABSTRACT

BACKGROUND: Laparoscopic adrenalectomy is widely performed for a number of hormone-producing tumors and postoperative management depends on the hormones produced. In the present study, we conducted a retrospective analysis to clarify the risk factors for postoperative complications, particularly postoperative fever after laparoscopic adrenalectomy. METHODS: We analyzed 406 patients who underwent laparoscopic adrenalectomy at our hospital between 2003 and 2019. Postoperative fever was defined as a fever of 38 °C or higher within 72 h after surgery. We investigated the risk factors for postoperative fever after laparoscopic adrenalectomy. RESULTS: There were 188 males (46%) and 218 females (54%) with a median age of 52 years. Among these patients, tumor pathologies included 188 primary aldosteronism (46%), 75 Cushing syndrome (18%), and 80 pheochromocytoma (20%). Postoperative fever developed in 124 of all patients (31%), 30% of those with primary aldosteronism, 53% of those with pheochromocytoma, and 8% of those with Cushing syndrome. A multivariate logistic regression analysis identified pheochromocytoma and non-Cushing syndrome as independent predictors of postoperative fever. Postoperative fever was observed in 42 out of 80 cases of pheochromocytoma (53%), which was significantly higher than in cases of non-pheochromocytoma (82/326, 25%, p < 0.01). In contrast, postoperative fever developed in 6 out of 75 cases of Cushing syndrome (8%), which was significantly lower than in cases of non-Cushing syndrome (118/331, 35.6%, p < 0.01). CONCLUSION: Since postoperative fever after laparoscopic adrenalectomy is markedly affected by the hormone produced by pheochromocytoma and Cushing syndrome, it is important to carefully consider the need for treatment.


Subject(s)
Adrenal Gland Neoplasms , Cushing Syndrome , Hyperaldosteronism , Laparoscopy , Pheochromocytoma , Male , Female , Humans , Middle Aged , Adrenalectomy/adverse effects , Cushing Syndrome/surgery , Pheochromocytoma/surgery , Retrospective Studies , Case-Control Studies , Laparoscopy/adverse effects , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Risk Factors , Hyperaldosteronism/surgery , Hormones
9.
J Pediatr Endocrinol Metab ; 37(5): 482-485, 2024 May 27.
Article in English | MEDLINE | ID: mdl-38618883

ABSTRACT

OBJECTIVES: Childhood cancer survivors are at risk for premature ovarian insufficiency, especially after treatment with alkylating agents. The objective of this report is to highlight a case in which this phenomenon caused a false-positive pregnancy test. CASE PRESENTATION: A workup was performed in a 14-year-old girl with a positive pregnancy test. She was diagnosed with stage IV neuroblastoma of the left adrenal gland at the age of 4 years. She received extensive treatment, including alkylating agents, and had been diagnosed with premature ovarian insufficiency. An LH/hCG suppression test was performed using high dose 17 bèta-estradiol: hCG levels normalized. CONCLUSIONS: The pregnancy test was false-positive due to production of low amounts of hCG by the pituitary gland as a result of high LH concentrations following premature ovarian insufficiency. It may be helpful to perform the LH/hCG suppression test to prove pituitary origin of the hCG overproduction.


Subject(s)
Primary Ovarian Insufficiency , Humans , Female , Primary Ovarian Insufficiency/diagnosis , Primary Ovarian Insufficiency/pathology , Adolescent , Pregnancy , Pregnancy Tests , Neuroblastoma/complications , Neuroblastoma/pathology , Neuroblastoma/drug therapy , Adrenal Gland Neoplasms/complications , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/diagnosis , False Positive Reactions , Luteinizing Hormone/blood , Prognosis
10.
BMJ Case Rep ; 17(4)2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38670570

ABSTRACT

With only 15 reported cases, anastomosing haemangioma of adrenal is a rare entity and usually presents as adrenal incidentaloma. A hypertensive, diabetic, non-smoker man in his late 60s presented with irritative voiding symptoms. On evaluation, he was found to have a urinary bladder mass and left adrenal incidentaloma measuring 8 cm. Metabolic evaluation confirmed it to be non-functional.The patient underwent transurethral resection of bladder tumour with left laparoscopic adrenalectomy. Intraoperatively, the adrenal tumour was highly vascular with multiple feeder vessels. Grossly it was soft, encapsulated with focal grey-brown areas. Microscopically, most of adrenal gland was replaced by anastomosing proliferating capillary vessels within framework of non-endothelial supporting cells reminiscent of splenic sinusoids. The tumour was positive for CD-31, CD-34, Glut-1 and SMA.Anastomosing haemangioma is a benign entity but it must be differentiated from angiosarcoma. Characteristic imaging features are not yet defined and is, therefore, difficult to diagnose preoperatively.


Subject(s)
Adrenal Gland Neoplasms , Adrenalectomy , Hemangioma , Humans , Male , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Hemangioma/surgery , Hemangioma/diagnosis , Hemangioma/complications , Adrenalectomy/methods , Diagnosis, Differential , Middle Aged , Urinary Bladder Neoplasms/surgery , Urinary Bladder Neoplasms/diagnosis , Urinary Bladder Neoplasms/pathology
11.
J Surg Res ; 298: 88-93, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38593602

ABSTRACT

INTRODUCTION: Elevated metanephrine and catecholamine levels 3-fold upper limit of normal (ULN) are diagnostic for pheochromocytoma. We sought to determine whether size correlates with biochemical activity or symptoms which could guide timing of surgery. METHODS: Data from consecutive patients undergoing adrenalectomy for pheochromocytoma at our institution over a 10-year period were retrospectively collected. These included maximal lesion diameter on preoperative imaging, plasma/urine metanephrine and/or catecholamine levels, demographic variables and presence of typical paroxysmal symptoms. Receiver operating characteristic curves were used to assess predictive accuracy. RESULTS: Sixty-three patients were included in the analysis (41 females and 22 males). Median age was 56 (43, 69) years. Due to various referring practices, 31 patients had documented 24-h urine metanephrine, 26 had 24-h urine catecholamine, and 52 had fractionated plasma metanephrine levels available for review. Values were converted to fold change compared to ULN and the maximum of all measured values was used for logistic regression. Median tumor size was 3.40 (2.25, 4.55) cm in greatest dimension. Tumor size at which pheochromocytoma produced > 3-fold ULN was ≥2.3 cm (AUC of 0.84). Biochemical activity increased with doubling tumor size (odds ratio = 8, P = 0.0004) or ≥ 1 cm increase in tumor size (odds ratio = 3.03, P = 0.001). 40 patients had paroxysmal symptoms, but there was no significant correlation between tumor size/biochemical activity and symptoms. CONCLUSIONS: In our study, tumor size directly correlated with the degree of biochemical activity and pheochromocytomas ≥2.3 cm produced levels 3 times ULN. These findings may allow clinicians to adjust timing of operative intervention.


Subject(s)
Adrenal Gland Neoplasms , Adrenalectomy , Metanephrine , Pheochromocytoma , Humans , Pheochromocytoma/surgery , Pheochromocytoma/pathology , Pheochromocytoma/blood , Female , Male , Middle Aged , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/blood , Retrospective Studies , Adult , Aged , Metanephrine/urine , Metanephrine/blood , Catecholamines/urine , Catecholamines/blood , Tumor Burden , Clinical Relevance
12.
Elife ; 122024 Apr 30.
Article in English | MEDLINE | ID: mdl-38687678

ABSTRACT

Internalization from the cell membrane and endosomal trafficking of receptor tyrosine kinases (RTKs) are important regulators of signaling in normal cells that can frequently be disrupted in cancer. The adrenal tumor pheochromocytoma (PCC) can be caused by activating mutations of the rearranged during transfection (RET) receptor tyrosine kinase, or inactivation of TMEM127, a transmembrane tumor suppressor implicated in trafficking of endosomal cargos. However, the role of aberrant receptor trafficking in PCC is not well understood. Here, we show that loss of TMEM127 causes wildtype RET protein accumulation on the cell surface, where increased receptor density facilitates constitutive ligand-independent activity and downstream signaling, driving cell proliferation. Loss of TMEM127 altered normal cell membrane organization and recruitment and stabilization of membrane protein complexes, impaired assembly, and maturation of clathrin-coated pits, and reduced internalization and degradation of cell surface RET. In addition to RTKs, TMEM127 depletion also promoted surface accumulation of several other transmembrane proteins, suggesting it may cause global defects in surface protein activity and function. Together, our data identify TMEM127 as an important determinant of membrane organization including membrane protein diffusability and protein complex assembly and provide a novel paradigm for oncogenesis in PCC where altered membrane dynamics promotes cell surface accumulation and constitutive activity of growth factor receptors to drive aberrant signaling and promote transformation.


Subject(s)
Cell Membrane , Membrane Proteins , Proto-Oncogene Proteins c-ret , Proto-Oncogene Proteins c-ret/metabolism , Proto-Oncogene Proteins c-ret/genetics , Humans , Membrane Proteins/metabolism , Membrane Proteins/genetics , Cell Membrane/metabolism , Signal Transduction , Protein Transport , Cell Transformation, Neoplastic/genetics , Cell Transformation, Neoplastic/metabolism , Cell Proliferation , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/metabolism , Adrenal Gland Neoplasms/pathology
13.
BMC Pediatr ; 24(1): 186, 2024 Mar 16.
Article in English | MEDLINE | ID: mdl-38491421

ABSTRACT

BACKGROUND: The aim of this study was to investigate the clinical features and treatment options for pediatric adrenal incidentalomas(AIs) to guide the diagnosis and treatment of these tumors. METHODS: The clinical data of AI patients admitted to our hospital between December 2016 and December 2022 were collected and retrospectively analyzed. All patients were divided into neonatal and nonneonatal groups according to their age at the time of the initial consultation. RESULTS: In the neonatal group, 13 patients were observed and followed up, and the masses completely disappeared in 8 patients and were significantly reduced in size in 5 patients compared with the previous findings. Four patients ultimately underwent surgery, and the postoperative pathological diagnosis was neuroblastoma in three patients and teratoma in one patient. In the nonneonatal group, there were 18 cases of benign tumors, including 9 cases of ganglioneuroma, 2 cases of adrenocortical adenoma, 2 cases of adrenal cyst, 2 cases of teratoma, 1 case of pheochromocytoma, 1 case of nerve sheath tumor, and 1 case of adrenal hemorrhage; and 20 cases of malignant tumors, including 10 cases of neuroblastoma, 9 cases of ganglioneuroblastoma, and 1 case of adrenocortical carcinoma. CONCLUSIONS: Neuroblastoma is the most common type of nonneonatal AI, and detailed laboratory investigations and imaging studies are recommended for aggressive evaluation and treatment in this population. The rate of spontaneous regression of AI is high in neonates, and close observation is feasible if the tumor is small, confined to the adrenal gland and has no distant metastasis.


Subject(s)
Adrenal Gland Neoplasms , Neuroblastoma , Teratoma , Humans , Infant, Newborn , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/therapy , Adrenal Gland Neoplasms/pathology , Neuroblastoma/diagnosis , Neuroblastoma/therapy , Retrospective Studies , Teratoma/diagnosis , Teratoma/surgery
14.
Medicine (Baltimore) ; 103(12): e37452, 2024 Mar 22.
Article in English | MEDLINE | ID: mdl-38518040

ABSTRACT

BACKGROUND: Adrenal cellular schwannomas are exceptionally rare stromal tumors that are often misdiagnosed due to the lack of specific radiological, serological, or clinical features. In this report, we describe the differential diagnosis of a rare adrenal cellular schwannoma. METHODS: A 69-year-old man with a history of persistent hypertension, chronic kidney disease, hypertensive heart disease, and cardiac insufficiency was hospitalized due to bilateral lower extremity edema lasting for 3 months. Plain computed tomography at that time revealed a space-occupying lesion in the right adrenal gland. As serum levels of catecholamines, cortisol, and adrenocorticotropic hormone were within normal ranges, the edema was attributed to the chronic kidney disease and cardiac insufficiency, and the patient was referred to our hospital for surgical treatment. Contrast-enhanced computed tomography revealed heterogeneous enhancement in the adrenal mass indicating pheochromocytoma. An irregularly shaped 5 cm mass with a complete capsule in the right adrenal gland was laparoscopically resected. The postoperative histopathological diagnosis was adrenal cellular schwannoma. RESULTS: The postoperative course was unremarkable and the tumor did not recur during 5 years of follow-up. CONCLUSION: Adrenal cellular schwannoma is a very rare tumor that is extremely difficult to preoperatively diagnose. Histological and immunohistochemical analyses are required for differential diagnosis and confirmation. Cellular schwannomas can transform into malignant peripheral nerve sheath tumors, but not often. Consequently, regular postoperative follow-up is required for such patients, especially imaging.


Subject(s)
Adrenal Gland Neoplasms , Hypertension , Neurilemmoma , Renal Insufficiency, Chronic , Male , Humans , Aged , Diagnosis, Differential , Neoplasm Recurrence, Local/diagnosis , Neurilemmoma/diagnosis , Neurilemmoma/surgery , Neurilemmoma/pathology , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Hypertension/diagnosis , Edema/diagnosis , Renal Insufficiency, Chronic/diagnosis
15.
J Clin Pathol ; 77(6): 378-382, 2024 May 17.
Article in English | MEDLINE | ID: mdl-38458747

ABSTRACT

Paired-like homeobox 2B (PHOX2B) is a gene essential in the development of the autonomic nervous system. PHOX2B mutations are associated with neurocristopathies-Hirschsprung disease (HSCR) and congenital central hypoventilation syndrome (CCHS)-and peripheral neuroblastic tumours. PHOXB2 plays an important role in the diagnostics of these conditions.Genotyping of a PHOX2B pathogenic variant is required to establish a diagnosis of CCHS. In HSCR patients, PHOX2B immunohistochemical staining has proven to be a valuable tool in identifying this disease. Furthermore, PHOXB2 is a predisposition gene for neuroblastoma, in which PHOX2B immunohistochemical staining can be used as a highly sensitive and specific diagnostic marker. The utility of PHOX2B immunohistochemistry in pheochromocytoma and paraganglioma has also been studied but yields conflicting results.In this review, an overview is given of PHOX2B, its associated diseases and the usefulness of PHOX2B immunohistochemistry as a diagnostic tool.


Subject(s)
Homeodomain Proteins , Hypoventilation , Immunohistochemistry , Neuroblastoma , Transcription Factors , Humans , Homeodomain Proteins/genetics , Transcription Factors/genetics , Hypoventilation/congenital , Hypoventilation/diagnosis , Hypoventilation/genetics , Neuroblastoma/diagnosis , Neuroblastoma/genetics , Neuroblastoma/pathology , Sleep Apnea, Central/diagnosis , Sleep Apnea, Central/genetics , Biomarkers, Tumor/genetics , Biomarkers, Tumor/analysis , Hirschsprung Disease/diagnosis , Hirschsprung Disease/genetics , Hirschsprung Disease/pathology , Mutation , Adrenal Gland Neoplasms/genetics , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/pathology , Genetic Predisposition to Disease
16.
Pathologie (Heidelb) ; 45(3): 218-222, 2024 May.
Article in German | MEDLINE | ID: mdl-38472383

ABSTRACT

A radiologically diagnosed tumor in a 29-year-old woman with a fever of around 39 °C was operated on under the suspicion of cholecystitis or a liver abscess. A solid tumor was found in the adrenal gland and resected. The frozen section findings did not reveal a clear diagnosis of entity and assignment. Histologically, the tumor was found to consist of densely clustered large histiocyte-like cells with expression of vimentin, CD68, and CD163 as well as negativity for keratin, langerin, and SMA. We diagnosed xanthogranulomatous adrenalitis and discussed the differential diagnoses (Langerhans cell histiocytosis, Rosai-Dorfman disease, malakoplakia, Erdheim-Chester disease).


Subject(s)
Adrenal Gland Neoplasms , Xanthomatosis , Humans , Adult , Female , Diagnosis, Differential , Adrenal Gland Neoplasms/diagnosis , Adrenal Gland Neoplasms/pathology , Adrenal Gland Neoplasms/surgery , Xanthomatosis/diagnosis , Xanthomatosis/pathology , Xanthomatosis/surgery , Granuloma/diagnosis , Granuloma/pathology , Granuloma/surgery , Adrenal Gland Diseases/diagnosis , Adrenal Gland Diseases/pathology , Erdheim-Chester Disease/diagnosis , Erdheim-Chester Disease/pathology , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/pathology , Histiocytosis, Sinus/surgery
17.
Front Endocrinol (Lausanne) ; 15: 1342240, 2024.
Article in English | MEDLINE | ID: mdl-38501101

ABSTRACT

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/m2. 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.


Subject(s)
Adrenal Gland Neoplasms , Laparoscopy , Humans , Retrospective Studies , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Adrenalectomy/methods , Retroperitoneal Space/surgery , Retroperitoneal Space/pathology , Laparoscopy/methods
18.
Abdom Radiol (NY) ; 49(4): 1231-1240, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38430264

ABSTRACT

PURPOSE: To assess indications, safety, and effectiveness of percutaneous adrenal mass biopsy in contemporary practice. METHODS: This institutional review board-approved, retrospective study included all patients undergoing percutaneous image-guided adrenal mass biopsies at an academic health system from January 6, 2015, to January 6, 2023. Patient demographics, biopsy indications, mass size, laboratory data, pathology results, and complications were recorded. Final diagnoses were based on pathology or ≥ 1 year of imaging follow-up when biopsy specimens did not yield malignant tissue. Test performance calculations excluded repeat biopsies. Continuous variables were compared with Student's t test, dichotomous variables with chi-squared test. RESULTS: A total of 160 patients underwent 186 biopsies. Biopsies were indicated to diagnose metastatic disease (139/186; 74.7%), for oncologic research only (27/186; 14.5%), diagnose metastatic disease and oncologic research (15/186; 8%), and diagnose an incidental adrenal mass (5/186; 2.7%). Biopsy specimens were diagnostic in 154 patients (96.3%) and non-diagnostic in 6 (3.8%). Diagnostic biopsies yielded malignant tissue (n = 136), benign adrenal tissue (n = 12), and benign adrenal neoplasms (n = 6) with sensitivity = 98.6% (136/138), specificity = 100% (16/16), positive predictive value = 100% (136/136), and negative predictive value = 88.9% (16/18). Adverse events followed 11/186 procedures (5.9%) and most minor (7/11, 63.6%). The adverse event rate was similar whether tissue was obtained for clinical or research purposes (10/144; 6.9% vs. 1/42; 2.4%, p = 0.27), despite more specimens obtained for research (5.8 vs. 3.7, p < 0.001). CONCLUSION: Percutaneous adrenal mass biopsy is safe, accurate, and utilized almost exclusively to diagnose metastatic disease or for oncologic research. The negative predictive value is high when diagnostic tissue samples are obtained. Obtaining specimens for research does not increase adverse event risk.


Subject(s)
Adrenal Gland Neoplasms , Humans , Retrospective Studies , Sensitivity and Specificity , Predictive Value of Tests , Adrenal Gland Neoplasms/diagnostic imaging , Adrenal Gland Neoplasms/pathology , Image-Guided Biopsy/methods
19.
J Laparoendosc Adv Surg Tech A ; 34(4): 359-364, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38301125

ABSTRACT

Introduction: Obesity is associated with numerous chronic conditions and an increased risk for surgical complications. Laparoscopic and robotic adrenalectomy have proven effective in the resection of adrenal tumors. This study analyzes the outcomes of severely obese patients (body-mass index [BMI] ≥35 kg/m2) following minimally invasive adrenalectomy. Materials and Methods: A retrospective analysis of patients who underwent minimally invasive adrenalectomy at our institution between 2010 and 2023 was conducted. Two matching analyses were performed. The first analysis compared patients with BMI greater versus lower than 35 kg/m2. The second analysis compared outcomes between robotic and laparoscopic adrenalectomy in patients with a BMI ≥35 kg/m2. Results: A total of 278 patients were included in the study. The median tumor size was 29 mm. Adrenal tumors had similar laterality, and most were hormonally active (66.2%). The most common pathological diagnosis was pheochromocytoma (25.5%). No statistical difference was found in peri- and postoperative outcomes between patients with BMI ≥35 and <35 kg/m2 who underwent minimally invasive adrenalectomy. When the surgical approach was compared in severely obese patients, robotic adrenalectomy was associated with shorter hospital length of stay with similar operative time as the laparoscopic approach. Conclusions: Minimally invasive adrenalectomy is safe and feasible in patients with BMI ≥35 kg/m2. Robotic and laparoscopic approaches are both safe and efficient for the resection of adrenal tumors in severely obese patients.


Subject(s)
Adrenal Gland Neoplasms , Laparoscopy , Humans , Adrenalectomy/adverse effects , Body Mass Index , Retrospective Studies , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Laparoscopy/adverse effects , Obesity/surgery , Length of Stay
20.
Updates Surg ; 76(2): 565-571, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38316738

ABSTRACT

Textbook outcome is a multidimensional quality management tool that uses a set of traditional surgical measures to reflect an "ideal" surgical result for a particular pathology. Retrospective study of all patients undergoing scheduled for adrenal tumors surgery at an endocrine surgery unit from January 2010-December 2022. The definition of Textbook Outcome were: R0 resection, no Clavien-Dindo ≥ IIIa complications, no prolonged stay (< P75), no readmissions, and no mortality in the first 30 days. The main objective was to analyze the rate of Textbook Outcome obtained. One hundred and five patients were included in the study. Textbook Outcome was achieved in 71.4%. Surgical approach (p < 0.001), Charlson scale (p = 0.031), American Society of Anesthesiologists Classification (p = 0.047) and surgical time (p < 0.001) were all significantly associated with the achievement of Textbook Outcome. The laparoscopic approach was associated as an independent factor with obtaining Textbook Outcome (OR:5.394; p = 0.016), as was surgical time (OR:0.986; p = 0.004). Textbook Outcome is a novel, useful, easy-to-interpret tool for measuring results in adrenal surgery. The laparoscopic approach is associated with a higher rate of "ideal" surgical results. The study was registered in the public accessible database clinicaltrials.gov with the ClinicalTrials.gov ID: NCT05888753.


Subject(s)
Adrenal Gland Neoplasms , Laparoscopy , Humans , Adrenalectomy/methods , Retrospective Studies , Adrenal Gland Neoplasms/surgery , Adrenal Gland Neoplasms/pathology , Postoperative Complications/etiology , Laparoscopy/methods , Treatment Outcome
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