scholarly journals Myxoid adrenal cortical adenoma and isolated adrenal myelolipoma

2020 ◽  
Vol 5 (4) ◽  
pp. 272-277
Author(s):  
A. A. Lisitsyn ◽  
V. P. Zemlyanoi ◽  
L. I. Velikanova ◽  
I. A. Bekhtereva ◽  
Z. R. Shafigullina
Keyword(s):  
Adrenal Gland ◽  
High Performance ◽  
Reductase Activity ◽  
Adrenal Adenoma ◽  
Gas Chromatography Mass Spectrometry ◽  
Syndrome Type ◽  
Hormonal Activity ◽  
Hydroxylase Deficiency ◽  
Adrenal Myelolipoma

Objectives to reveal the clinical, morphological and hormonal characteristics of patients operated on for isolated adrenal myelolipoma and myxoid adenomas with adrenal myelolipoma. Material and methods.The study was based on the results of examination of patients operated on for adrenal myelolipoma in the period from 2000 to 2015. During the study, the hormonal activity in patients with isolated and myxoid adrenal myelolipomas was analyzed. A pathomorphological and immunohistochemical study of the removed formations of the adrenal gland was performed. The article presents rare clinical observations of patients undergoing treatment in the clinic of E.E. Eichwald, North-Western State Medical University named after I.I. Mechnikov, with accidentally detected neoplasms, which were a combination of adenoma and myelolipoma of the adrenal gland. Results.The occurrence of isolated myelolipomas and myxoid adenomas with myelolipoma among incidentalomas was 6.3% and 4.9%, respectively. The average age of patients with adrenal myelolipoma is 54.5 years. The ratio of women to men was 2:1. According to our data, four types of hormonal activity have been identified among myxoid formations of the adrenal gland. Type 1 is associated with Cushing's syndrome, type 2 is associated with Conn's syndrome, type 3 is associated with autonomic cortisol secretion, type 4 has no hormonal activity. Based on the results obtained by high-performance liquid chromatography (HPLC), 11-hydroxylase deficiency was noted, and according to the study of the urine steroid profile by gas chromatography-mass spectrometry (GCMS), the signs of type 2 11-hydroxysteroid dehydrogenase deficiency and an increase in 5-reductase activity in patients with isolated adrenal myelolipoma were obtained. Conclusion.The combination of adenoma with myelolipoma, as well as of adenoma, myelolipoma and cavernous hemangioma is a rare pathology, the diagnosis of which is based on the pathomorphological examination of the removed tumor. Myelolipoma as a part of adrenal adenoma is an incidentaloma, which can be of different sizes and is accompanied by hormonal dysfunction of the cells of the adrenal cortex. An uncertain intrascopic phenotype and hormonal activity of myxoid formations are the decisive factors in favor of surgical intervention.

scholarly journals Giant adrenal myelolipoma associated with 21-hydroxylase deficiency: unusual association mimicking an androgen-secreting adrenocortical carcinoma

2010 ◽  
Vol 54 (4) ◽  
pp. 419-424 ◽  
Author(s):  
Lívia Mara Mermejo ◽  
Jorge Elias Junior ◽  
Fabiano Pinto Saggioro ◽  
Silvio Tucci Junior ◽  
Margaret de Castro ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Adrenal Gland ◽  
Adrenocortical Carcinoma ◽  
Adrenal Mass ◽  
Surgical Excision ◽  
Abdominal Ultrasound ◽  
Hydroxylase Deficiency ◽  
Adrenal Myelolipoma ◽  
The Right ◽  
21 Hydroxylase Deficiency

The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.

scholarly journals SUN-197 Giant Adrenal Myelolipoma Associated with Prolonged ACTH Elevation in a Patient with Congenital Adrenal Hyperplasia (CAH)

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Vidumini Kaluarachchi ◽  
Muhammad Fahad Arshad ◽  
Sabapathy Balasubramanian ◽  
Chern Beverly Brenda Lim ◽  
Matthew Bull ◽  
...  
Keyword(s):  
Abdominal Pain ◽  
Adrenal Gland ◽  
Ct Scan ◽  
Uneventful Recovery ◽  
Left Adrenal Gland ◽  
Hydroxylase Deficiency ◽  
Cushing Disease ◽  
Adrenal Myelolipoma ◽  
21 Hydroxylase Deficiency

Abstract Background: Myelolipomas are rare benign tumours which consist of adipose tissue and mature hematopoietic tissue. The incidence ranges from 0.08 to 0.4% and they constitute 15% of adrenal incidentalomas. We report the case of a massive adrenal myelolipoma in a patient with CAH. Clinical Case: A 51year old man with salt losing CAH due to 21 hydroxylase deficiency presented to the emergency department with abdominal pain. CT scan of the abdomen revealed a 15 x 16 x 19cm mass in the left adrenal gland. There were concerns about malignancy, however expert radiology review diagnosed a massive adrenal myelolipoma. The patient was diagnosed to have salt losing CAH during infancy and managed with cortisone acetate and fludrocortisone during childhood, then dexamethasone 0.5mg nocte, 0,25mg mane as an adult. At 27 years he was reviewed and found to be Cushingoid with abdominal striae, obesity and hypertension. A CT scan of the abdomen done at that time showed a normal left adrenal gland with nodular right adrenal gland. While he was on dexamethasone, his 17-OHP was 14 nmol/l (NR: 0–6.0 nmol/l). His dexamethasone was reduced, then he was transferred on to prednisolone and then to hydrocortisone. 17-OHP level and ACTH levels have risen with each change in medication. At the time of referral with abdominal pain, he was on hydrocortisone 10 mg mane, 10 mg midday and 5mg at 1700h and his biochemistry showed: ACTH 486ng/l (NR 7.2–63.3), 17-OHP 101.1nmol/l (0–6.0nmol/l), renin 5.5 nmol/l/hr (NR 0.2–3.3) and androstenedione 26.6 nmol/l (NR 0–7.8). He underwent laparoscopic hand-assisted left adrenalectomy with the removal of a 2030 gram adrenal myelolipoma. He made an uneventful recovery with remarkable clinical improvement in general wellbeing. Histology confirmed adrenal myelolipoma. Conclusion: Chronic exposure to high ACTH levels causes metaplasia of adrenocortical cells, but a direct causal relationship for myelolipomas is not established. The role of ACTH in development of myelolipomas is supported by the increased incidence in patients with Addison’s disease, Nelson’s syndrome and ACTH dependent Cushing Disease. Our case supports the role of ACTH as the myelipoma developed after the reduction of patient’s glucocorticoid treatment and ACTH levels were high for years. Myelolipomas are usually asymptomatic but can present with abdominal pain, spontaneous rupture, and spontaneous retroperitoneal haemorrhage. There are no set guidelines but for large and rapidly growing tumours, surgery may be considered..

scholarly journals CHARACTERISTICS OF METABOLISM OF GLUCOCORTICOID HORMONESIN PATIENTS WITH PHEOCHROMOCYTOMA

2017 ◽  
Vol 9 (3) ◽  
pp. 59-64
Author(s):  
M A Ivanushko ◽  
N V Vorokhobina ◽  
L I Velikanova ◽  
Z R Shafigullina
Keyword(s):  
Mass Spectrometry ◽  
Gas Chromatography ◽  
High Performance Liquid Chromatography ◽  
High Performance ◽  
Biologically Active ◽  
Gas Chromatography Mass Spectrometry ◽  
Blood Levels ◽  
Glucocorticoid Hormones ◽  
Urine Excretion

The article performs analysis of glucocorticoid hormones data and their metabolites in- vestigated by methods of high-performance liquid chromatography and gas chromatography - mass- spectrometry in 47 patients with pheochromocytoma (РНЕО) and 28 patients with hormone-inactive adenoma of the adrenal cortex. We revealed increased blood levels оf biologically active glucocorticoids and elevation of urine excretion of their tetrahydrometabolites in patients with PHEO. 6 patients with PHEO showed biochemical signs of the autonomous cortisol production. A positive correlation of free normetanephrine excretion with blood cortisol, corticosterone and 11-deoxycortisol and urine excretion of tetrahydrometabolites and cortols and a negative correlation with the urine excretion of tetrahydro-11-dehydrocorticosterone were detected. An increase of the THF/THE and (THF+alloTHF+cortol)/ (THE+allo-THE+ cortolone) ratios may indicate a decrease of 11β-hydroxysteroiddehydrogenase type 2 activity in patients with РНЕО.

Oro facial digital syndrome type 2- An Indian case report

2016 ◽  
Vol 3 (4) ◽  
pp. 115
Author(s):  
Naresh Bansal ◽  
Ashok Gupta ◽  
Manisha Goyal ◽  
Manish Sharma ◽  
Priyanshu Mathur ◽  
...  
Keyword(s):  
Case Report ◽  
Syndrome Type

Application of a fractionation technique for better understanding of the removal of natural organic matter by alum coagulation

2002 ◽  
Vol 2 (5-6) ◽  
pp. 427-433 ◽  
Author(s):  
J. van Leeuwen ◽  
C. Chow ◽  
R. Fabris ◽  
N. Withers ◽  
D. Page ◽  
...  
Keyword(s):  
Organic Matter ◽  
Natural Organic Matter ◽  
High Performance ◽  
Relative Proportion ◽  
Water Source ◽  
Size Exclusion ◽  
Gas Chromatography Mass Spectrometry ◽  
Pyrolysis Gas ◽  
Alum Treatment ◽  
Hydrophobic Acids

To gain an improved understanding of the types of organic compounds that are recalcitrant to water treatment, natural organic matter (NOM) isolates from two drinking water sources (Mt. Zero and Moorabool reservoirs, Victoria, Australia) were separated into fractions of distinct chemical behaviour using resins. Four fractions were obtained from each water source and were organics absorbed to: (1) XAD-8 (very hydrophobic acids, VHA); (2) DAX-4 (slightly hydrophobic acids, SHA); (3) bound to an anion exchange resin (charged organics, CHAR); and (4) not absorbed or bound to resins (neutrals, NEUT). These fractions were then tested to determine the capacity of alum to remove them from water and to correlate this with the character of each isolate. The fractions were characterised by the application of high performance size exclusion chromatography (HPSEC), bacterial regrowth potential (BRP), trihalomethane formation potential (THMFP), pyrolysis gas-chromatography mass spectrometry (Py-GC-MS) and thermochemolysis. The highest removals of dissolved organic carbon (DOC) by alum treatment were in waters spiked with the CHAR fractions while the NEUT fractions were the most recalcitrant. The number average molecular weights (Mn) of DOC of the CHAR fractions before treatment were the highest, whilst those of the NEUT fractions were the lowest. After alum treatment, the Mn of the NEUT fractions were only slightly reduced. Results from Py-GC-MS and thermochemolysis indicate that the NEUT fractions had the highest relative proportion of saccharide derived organic material. Nonetheless, the BRP of waters spiked with the NEUT fractions differed markedly, indicating that organics recalcitrant to alum treatment can vary substantially in their chemical composition and capacity to support microbial growth.

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