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* RECENT POSTS
* Cushing’s Syndrome in the Elderly
* Complete and Sustained Remission of Hypercortisolism With Pasireotide
Treatment of an Adrenocorticotropic Hormone (Acth)-Secreting Thoracic
Neuroendocrine Tumour: an N-Of-1 Trial
* An Aggressive Case of Adrenocortical Carcinoma Complicated by
Paraneoplastic Cushing’s Syndrome
* Cushing’s Disease Patients are More Likely to Have Ocular Hypertension
* CRH Stimulation Test Boosts Cushing Disease Diagnosis
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* Cushing’s Syndrome in the Elderly: Data from the European Registry on
Cushing’s Syndrome
Evaluate whether age-related differences exist in clinical characteristics,
diagnostic approach and management strategies in patients with Cushing’s
syndrome included in the European Registry on Cushing’s Syndrome (ERCUSYN).
* Bee’s Knees: Pre-TKR, Another Setback?
So, I had the Medical Clearance Revision and it was exactly like the
Medical Clearance I did just over 30 days ago. It looks like surgery is a
go. Thursday, March 9 at 12:15. There’s extra stuff for me to do to
prepare, thanks to Cushing’s, the GH deficiency, the Adrenal Insufficiency,
the one kidney, etc but I can do it!
* In Memory: Liz Raftery, March 2012
We sadly learned that Liz died in March 2012 at the age of 45. She was an
active member of the Cushing’s Help Message Boards. She had a photo gallery
there. The photo below is from that gallery. Liz in 2002The image at left
is from 2002. Liz wrote in her bio: Hello, I’m from Hampton, just outside
London. Same old story – at least 6 years of various illnesses […]
* I t’s Kidney Cancer Awareness Month Again
Kidney Cancer Awareness is very important to me, because I learned I had it
in 2006. I’m pretty sure I had it before 2006 but in that year I picked up
my husband for a biopsy and took him to an outpatient surgical center.
While I was there waiting for the biopsy to be completed, I started
noticing blood in my urine and major abdominal cramps. I left messages […]
* Cushing's Basics: What Is Cortisol And How Is It Affecting My Energy
Levels?
You may be experiencing high cortisol levels if you’re holding onto weight
in your belly or face or have noticed fat deposits in your shoulder or
stretch marks on your stomach. Muscle weakness, blood sugar spikes, high
blood pressure and hirsutism (unwanted hair growth) may be other red flags.
* Light Up for Rare
The National Organization for Rare Disorders (NORD) asks Americans to plan
ahead to participate in the Light Up for Rare campaign to raise awareness
of rare diseases. NORD is the U.S. sponsor for Rare Disease Day on Feb.
28. The annual awareness day spotlights approximately 7,000 rare diseases
that affect more than 300 million people worldwide. More than 2 […]
* For Rare Disease Day: MaryO, Pituitary/Kidney Cancer Bio
As one can imagine, it hasn’t been all happiness and light. Most of my
journey has been documented here and on the message boards – and elsewhere
around the web.
* What I'm Doing for Rare Disease Day
Each and every day since 1987, I tell anyone who will listen about
Cushing’s...
* Today is Rare Disease Day
Today is Rare Disease Day. Learn all about Cushing's Disease at
http://www.cushings-help.com/
* Top Picks on the Message Boards updated February 27, 2023
Top content from across the message boards, hand-picked by MaryO and the
Cushing's community.
* Cushing's Support for Adults- Virtual Chat
This virtual chat, moderated by Shauna Nelson - MAGIC's Cushing's Division
Consultant, is an opportunity for adult members to share stories, discuss
treatments and successes that have worked for them.
* Update - Please Pray for the Founder of the Adrenal Alternatives Foundation
We are so happy to report Winslow is much better. Though her blood cortisol
levels are only reaching 2 despite continuous infusion, her electrolytes
have stabilized.
* Update - Please Pray for the Founder of the Adrenal Alternatives Foundation
Update 2/12/23 Winslow is still fighting. We are awaiting medical transport
to take her to another hospital that can better care for her. The medullary
sponge kidney is causing major complications and negatively impacting her
body's ability to retain cortisol. She's battling a lot of pain and
fighting to get back to us. Please pray and send good vi […]
* Message Board Upgrade (and new features!) Coming this Weekend
This is our January maintenance release. During the holiday period, our
focus is on providing bug fixes and improving stability.
* What Do *You* Think? Medical Gaslighting
So much in here is similar to what I went through during my years of being
diagnosed with Cushing's. The depression, the weight, the dismissal of
everything I knew to be true about what I was feeling.
* Update - Please Pray for the Founder of the Adrenal Alternatives Foundation
Update- 2/6/23 As you all know, it is a fight to get cortisol coverage.
This morning around 7:30, she went into crisis again. The nurse was alerted
that she needed cortisol. The nurse was told by the doctor that Winslow
could not have an additional dose and needed to wait until her scheduled
9:00 am dose. Her family members had to intercede to save her life. […]
* Complete and Sustained Remission of Hypercortisolism With Pasireotide
Treatment of an Adrenocorticotropic Hormone (Acth)-Secreting Thoracic
Neuroendocrine Tumour: an N-Of-1 Trial
We describe a patient presenting with a typical clinical picture of
Cushing’s Syndrome (CS). Further testing was diagnostic of ectopic
Adrenocorticotropic Hormone (ACTH) secretion, but its origin remained
occult.
* Comment added to Shelleyw, Adrenal Bio
your daughter likely has Cyclical Cushing, due to only one test positive,
and also she has EDS, I’m a Dr. and an hEDS sufferer,
* Please Pray for the Founder of the Adrenal Alternatives Foundation
The founder of Adrenal Alternatives Foundation has suffered a severe
adrenal crisis yesterday morning. She is alive but fighting for her life.
We are unsure of the status of the Foundation at this time. This is the
unfortunate reality of this disease, not even the strongest ones are exempt
from the toll it takes. Keep fighting, adrenal warriors.
* An Aggressive Case of Adrenocortical Carcinoma Complicated by
Paraneoplastic Cushing’s Syndrome
Endocrinologic evaluation revealed grossly elevated 24-hour urinary free
cortisol and elevated serum cortisol levels consistent with severe
Cushing’s syndrome, and she was started on high-dose ketoconazole.
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CUSHING’S SYNDROME IN THE ELDERLY
Posted on March 6, 2023 by MaryO
ABSTRACT
Objective
To evaluate whether age-related differences exist in clinical characteristics,
diagnostic approach and management strategies in patients with Cushing’s
syndrome included in the European Registry on Cushing’s Syndrome (ERCUSYN).
Design
Cohort study.
Methods
We analyzed 1791 patients with CS, of whom 1234 (69%) had pituitary-dependent CS
(PIT-CS), 450 (25%) adrenal-dependent CS (ADR-CS) and 107 (6%) had an ectopic
source (ECT-CS). According to the WHO criteria, 1616 patients (90.2%) were
classified as younger (<65 years) and 175 (9.8%) as older (>65 years).
Results
Older patients were more frequently males and had a lower BMI and waist
circumference as compared with the younger. Older patients also had a lower
prevalence of skin alterations, depression, hair loss, hirsutism and reduced
libido, but a higher prevalence of muscle weakness, diabetes, hypertension,
cardiovascular disease, venous thromboembolism and bone fractures than younger
patients, regardless of sex (p<0.01 for all comparisons). Measurement of UFC
supported the diagnosis of CS less frequently in older patients as compared with
the younger (p<0.05). An extra-sellar macroadenoma (macrocorticotropinoma with
extrasellar extension) was more common in older PIT-CS patients than in the
younger (p<0.01). Older PIT-CS patients more frequently received
cortisol-lowering medications and radiotherapy as a first-line treatment,
whereas surgery was the preferred approach in the younger (p<0.01 for all
comparisons). When transsphenoidal surgery was performed, the remission rate was
lower in the elderly as compared with their younger counterpart (p<0.05).
Conclusions
Older CS patients lack several typical symptoms of hypercortisolism, present
with more comorbidities regardless of sex, and are more often conservatively
treated.
From https://academic.oup.com/ejendo/advance-article-abstract/doi/10.1093/ejendo/lvad008/7030701?redirectedFrom=fulltext&login=false
Thoughts? Discussion on the Cushing’s Help Message Boards
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Filed under: adrenal, Cushing's, pituitary, symptoms | Tagged: adrenal, ectopic,
elderly, ERCUSYN, European Registry on Cushing’s Syndrome, pituitary | Leave a
comment »
COMPLETE AND SUSTAINED REMISSION OF HYPERCORTISOLISM WITH PASIREOTIDE TREATMENT
OF AN ADRENOCORTICOTROPIC HORMONE (ACTH)-SECRETING THORACIC NEUROENDOCRINE
TUMOUR: AN N-OF-1 TRIAL
Posted on February 7, 2023 by MaryO
Abstract
N-of-1 trials can serve as useful tools in managing rare disease. We describe a
patient presenting with a typical clinical picture of Cushing’s Syndrome (CS).
Further testing was diagnostic of ectopic Adrenocorticotropic Hormone (ACTH)
secretion, but its origin remained occult. The patient was offered treatment
with daily pasireotide at very low doses (300 mg bid), which resulted in
clinical and biochemical control for a period of 5 years, when a pulmonary
typical carcinoid was diagnosed and dissected. During the pharmacological
treatment period, pasireotide was tentatively discontinued twice, with immediate
flare of symptoms and biochemical markers, followed by remission after drug
reinitiation.
This is the first report of clinical and biochemical remission of an ectopic CS
(ECS) with pasireotide used as first line treatment, in a low-grade lung
carcinoid, for a prolonged period of 5 years. In conclusion, the burden of high
morbidity caused by hypercortisolism can be effectively mitigated with
appropriate pharmacological treatment, in patients with occult tumors.
Pasireotide may lead to complete and sustained remission of hypercortisolism,
until surgical therapy is feasible. The expression of SSTR2 from typical
carcinoids may be critical in allowing the use of very low drug doses for
achieving disease control, while minimizing the risk of adverse events.
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Filed under: adrenal, Cancer, Clinical trials, Cushing's, Rare Diseases,
Treatments | Tagged: ACTH, carcinoid, ectopic, Ectopic Cushing’s syndrome, Lung
carcinoid, pasireotide, SSTR2, Thoracic neuroendocrine tumor | Leave a comment »
AN AGGRESSIVE CASE OF ADRENOCORTICAL CARCINOMA COMPLICATED BY PARANEOPLASTIC
CUSHING’S SYNDROME
Posted on February 4, 2023 by MaryO
ABSTRACT
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with a poor
prognosis. Surgical resection may be curative if localized disease is
identified, although recurrence is common. Research shows that the use of
adjuvant therapeutic regimens such as EDP-M (combination of mitotane, etoposide,
doxorubicin, and cisplatin) in high-risk patients has survival benefits.
A 75-year-old female was incidentally found to have a right adrenal
heterogeneous internal enhancement measuring 5.0 x 3.7cm. The workup confirmed
autonomous adrenal production of corticosteroids and she was referred to surgery
for an adrenalectomy. A T2 ACC with positive margins and lympho-vascular
invasion was resected, following which she was started on external beam
radiation followed by four cycles of carboplatin and etoposide. Despite initial
treatments, she was diagnosed with refractory metastatic disease at subsequent
follow-ups. Pembrolizumab immunotherapy was started, but disease progression
continued, and she was eventually transitioned to mitotane 1g twice daily. She
continued to worsen and was eventually transitioned to hospice care.
The management of ACC remains diagnostically challenging, especially because
most patients do not present until an advanced stage of disease. Surgery is
commonly employed with a curative intent, and opinions regarding adjuvant
cytotoxic therapy and/or radiotherapy remain mixed.
INTRODUCTION
Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy
with an annual incidence of 0.5-2.0 cases per million persons [1]. ACC is
associated with an unsatisfactory prognosis with an estimated median survival of
about three to four years. The five-year survival is 60-80% for tumors confined
to the adrenal space, 35-50% for locally advanced disease, and 0% to 28% in
cases of metastatic disease [2].
Surgical en-bloc resection is commonly employed and is recommended for
locoregional disease. There is no standard of care for the management of ACC
although cytotoxic cisplatin-based regimens such as EDP-M (a combination of
mitotane, etoposide, doxorubicin, and cisplatin) may be employed as adjuvant
therapy in those with very high recurrence risk. Mitotane is recommended for
patients with a high risk of recurrence (stage III disease, R1 resection
margins, or Ki67 >10%) although its routine use for low/moderate risk disease is
controversial [3]. Despite complete resection of early-stage disease, recurrence
rates in ACC are still very high and appropriate management remains a challenge.
We demonstrate a patient with a limited-stage T2 ACC who, despite receiving
primary surgery, adjuvant chemotherapy and radiotherapy, progressed to
metastatic disease.
CASE PRESENTATION
A 75-year-old female was evaluated by endocrinology for an incidentally
discovered adrenal mass. A week prior, she was hospitalized for chest pain. A CT
angiogram to exclude aortic dissection revealed a large right adrenal lesion
with foci of heterogeneous internal enhancement measuring 5.0 cm x 3.7 cm
(Figure 1).
FIGURE 1: COMPUTED TOMOGRAPHY (CT) SCAN OF THE ABDOMEN DEMONSTRATING
INCIDENTALLY NOTED ADRENAL MASS.
White circle: Large irregular right-sided adrenal mass with foci of heterogenous
internal enhancement noted
She was initially asymptomatic, and denied constitutional symptoms such as
fatigue or unexplained loss of weight. However, she had a history of
hypertension and anxiety, which raised concern for a pheochromocytoma. She
otherwise denied unexplained bruising, palpitations, muscle aches, tremors, and
heat/cold intolerance.
Aside from hypertension and anxiety, she had a history of type II diabetes
mellitus managed on metformin alone. Her family history was remarkable for a
brother who also had a left adrenal lesion which was found to be a
non-functional adenoma following adrenalectomy.
Her vitals were normal except for a blood pressure of 150/90. Examination showed
a well-nourished female with no obvious Cushingoid features, such as increased
dorsocervical fat pad, axillary or abdominal striations, or unexplained
extremity bruising. Cardiac and respiratory exams were within normal limits, and
no lymphadenopathy was appreciated.
She was scheduled for further workup of her adrenal incidentaloma and was found
to have an elevated serum cortisol level. An overnight low-dose dexamethasone
suppression test was non-suppressed, and adrenocorticotropic hormone (ACTH)
level was found to be low (Table 1). These findings confirmed autonomous adrenal
production of corticosteroids, and she was referred to surgery for
adrenalectomy.
Investigation (units) Value (initial) Value (repeat) Reference range 24-hour
urinary epinephrine (mcg/24hr) <1.4 – <21 24-hour urinary norepinephrine
(mcg/24hr) 28 – 15-80 24-hour urinary metanephrines (mcg/24hr) <29 – 30-180
24-hour urinary normetanephrines (mcg/24hr) 211 – 148-560 Plasma renin activity
(ng/mL/hr) 0.2 – 0.2-1.6 Serum aldosterone (ng/dL) 4.1 – 2-9 Serum cortisol
(ug/dL) 22.2 54.1 2.7-10.5 (for 6-8PM) 24-hour urinary cortisol (mcg/day) 22.9
1347 <45 ACTH level (pg/mL) 3.2 – 7.2-63.3
TABLE 1: INVESTIGATIONS PERFORMED IN THE WORKUP OF THE PATIENT’S INCIDENTALOMA.
REPEAT VALUES FOR SELECT INVESTIGATIONS ARE PRESENTED A YEAR LATER AFTER SHE
PRESENTED WITH METASTATIC DISEASE.
ACTH: adrenocorticotropic hormone
She successfully underwent surgery without complications. A surgical pathology
report showed a high-grade adrenocortical carcinoma with positive surgical
margins. Small vessel lymphovascular invasion was noted, but regional lymph
nodes could not be assessed. The primary tumor was staged T2, with a mitotic
rate of 22/50 high power fields that marked it as high grade histologically
(Figure 2).
FIGURE 2: HEMATOXYLIN & EOSIN STAIN OF A SECTION OF TISSUE FROM PATHOLOGIC
BIOPSY UNDER HIGH POWER MICROSCOPY. NOTED ARE THE INCREASED NUMBER OF MITOTIC
FIGURES, INCREASED NUCLEAR:CYTOPLASMIC RATIO, AND ABNORMAL MITOTIC FIGURES
TYPICAL FOR A HIGH-GRADE MALIGNANCY,
She was subsequently referred to oncology for further evaluation, and proceeded
with external beam radiation therapy for a total dose of 4500 cGy over 25
fractions, followed by adjuvant therapy with four cycles of carboplatin and
etoposide. Dose reduction was needed after cycle two for worsening fatigue and
neuropathy, but she otherwise tolerated the treatments well.
Nearly a year later, a regular surveillance CT demonstrated multiple
sub-centimeter pulmonary nodules with patchy ground-glass abnormalities
concerning for metastatic disease. In view of her disease progression, she
started pembrolizumab immunotherapy.
Repeat imaging, in the setting of worsening fatigue and anorexia, confirmed
enlargement of her multiple lung nodules with a new soft tissue mediastinal mass
also being found (Figure 3). She developed worsening lower extremity edema and
required hospitalizations for recurrent hypokalemia with hypertension.
Endocrinologic evaluation revealed grossly elevated 24-hour urinary free
cortisol and elevated serum cortisol levels consistent with severe Cushing’s
syndrome, and she was started on high-dose ketoconazole.
FIGURE 3: CT OF THE CHEST DEMONSTRATING MULTIPLE NODULES IN THE LUNGS CONSISTENT
WITH METASTATIC DISEASE PROGRESSION.
Green lines: Identified lung parenchymal nodules measuring 2.60 cm (panel 1) and
2.24 cm (panel 2) in greatest diameter.
Despite six months of immunotherapy, repeat imaging showed substantial increase
in size of both her multiple bilateral lung nodules. Extensive mediastinal and
hilar adenopathy was also noted. Her treatment regimen was switched once more to
mitotane 1g twice daily. She also had multiple subsequent hospitalizations for
severe hypokalemia complicated by atrial fibrillation with rapid ventricular
response.
She continued to clinically deteriorate, with increasing shortness of breath,
fatigue, and chest pain. A goals of care discussion was held in view of her
aggressive disease course and multiple lines of failed therapy. She was then
transitioned to hospice care, and her mitotane was stopped.
DISCUSSION
Although overall adrenal tumors are common in the population, affecting about
3-10% of people, most of these are benign. ACC on the other hand is rare, and
approximately 40-60% of ACCs are found to be functional tumors that produce
hormones. Fifty to 80% of these functional ACCs secrete cortisol [4]. A
surprising percentage of these may even be picked up incidentally, with one
multicentric and retrospective evaluation of 1096 cases demonstrating that 12%
of adrenal incidentalomas are ACCs [2]. Despite improved detection rates,
however, this has not translated to earlier detection and treatment of ACC [5].
The first proposed TNM staging classification scheme for ACC in 2003 by the
International Union Against Cancer (UICC) had notable shortcomings, including
similar outcomes for both stage II and III disease [6]. A study of 492 patients
in a German ACC registry found that disease-specific survival (DSS) did not
significantly differ between stage II and stage III ACC (hazard ratio, 1.38; 95%
confidence interval, 0.89-2.16) and furthermore, patients who had stage IV ACC
without distant metastases had an improved DSS compared with patients who had
metastatic disease (P = .004) [7]. The American Joint Committee of Cancer
(AJCC), and the European Network for the Study of Adrenal Tumors (ENSAT)
consequently developed revised staging systems that better reflect patient
prognosis.
The most important predictors of survival in patients with ACC are tumor grade,
tumor stage, and surgical treatment. For patients after surgical resection, the
administration of adjunctive therapy is guided by the risk of recurrence.
Despite early-stage resection, disease recurrence rates in ACC are very high.
Besides the EDP-M regimen, no others have been successfully evaluated in large,
randomized trials [4]. Whenever possible, it is still recommended that patients
be referred to a clinical trial on an individual basis.
The ADJUVO clinical trial consisted of 91 low-recurrence-risk ACC patients who
were randomly assigned to either observation or adjuvant mitotane therapy after
surgical resection. Low recurrence risk is defined as Ki67<10%, stage I-III
according to ENSAT classification, and microscopically complete resection.
Adjuvant mitotane treatment failed to demonstrate statistically significant
differences in disease-free survival, recurrence-free survival and overall
survival between these patient groups [8]. Our case seems to suggest that even
limited-stage disease may need to be managed aggressively not just with primary
surgery, but also adjuvant chemoradiotherapy, especially with a high histologic
grade.
PD-1 blockade in adrenocortical carcinoma was evaluated in a phase II study of
39 participants, with a progression-free survival of 2.1 months independent of
mismatch repair deficiency status being reported [9]. Despite switching to
pembrolizumab in our patient, disease progression continued unabated, calling
into question the clinical benefit of PD-1 blockade in ACC.
A small study on the use of metyrapone with EDP-M in three advanced ACC patients
with Cushing’s syndrome displayed a good safety profile with minor drug-drug
interactions and appears to be a good option in combination with mitotane and
other cytotoxic chemotherapies [10]. Ketoconazole is often less effective than
metyrapone and requires regular monitoring of liver function tests, although it
also inhibits androgen production.
CONCLUSIONS
This case demonstrates the unfortunate prognosis of many patients with ACC.
Although patients may present with classical symptoms of hypercortisolism or
hyperandrogenism, many patients do not present with symptoms until the disease
has advanced. Surgery may be employed with curative intent, although the
evidence for adjuvant radiotherapy is mixed. The management for patients with
ACC continues to remain a challenge due to the lack of evidence for optimal
therapeutic management. In view of the aggressive nature of ACC, patients with
high-grade histology despite limited-stage disease require adjuvant
chemoradiation in addition to primary surgery to maximize the chances of
progression-free survival. Also, although the use of PD-1 blockade has
revolutionized cancer care in several other tumor types, evidence of clear
benefit in ACC is lacking, as our case demonstrates.
REFERENCES
1. Kerkhofs TM, Verhoeven RH, Van der Zwan JM, et al.: Adrenocortical
carcinoma: a population-based study on incidence and survival in the
Netherlands since 1993. Eur J Cancer. 2013,
49:2579-86. 10.1016/j.ejca.2013.02.034
2. Else T, Kim AC, Sabolch A, et al.: Adrenocortical carcinoma. Endocr Rev.
2014, 35:282-326. 10.1210/er.2013-1029
3. Survival Rates for Adrenal Cancer.
(2022). https://www.cancer.org/cancer/adrenal-cancer/detection-diagnosis-staging/survival-by-stage.html.
4. Fassnacht M, Dekkers OM, Else T, et al.: European Society of Endocrinology
Clinical Practice Guidelines on the management of adrenocortical carcinoma
in adults, in collaboration with the European Network for the Study of
Adrenal Tumors. Eur J Endocrinol. 2018, 179:G1-G46. 10.1530/EJE-18-0608
5. Kebebew E, Reiff E, Duh QY, Clark OH, McMillan A: Extent of disease at
presentation and outcome for adrenocortical carcinoma: have we made
progress?. World J Surg. 2006, 30:872-8. 10.1007/s00268-005-0329-x
6. Fassnacht M, Wittekind C, Allolio B: [Current TNM classification systems
for adrenocortical carcinoma]. Pathologe. 2010,
31:374-8. 10.1007/s00292-010-1306-1
7. Fassnacht M, Johanssen S, Quinkler M, et al.: Limited prognostic value of
the 2004 International Union Against Cancer staging classification for
adrenocortical carcinoma: proposal for a Revised TNM Classification.
Cancer. 2009, 115:243-50. 10.1002/cncr.24030
8. Berruti A, Fassnacht M, Libè R, et al.: First randomized trial on adjuvant
mitotane in adrenocortical carcinoma patients: the Adjuvo Study. J Clin
Oncol. 2022, 40:1. 10.1200/JCO.2022.40.6_suppl.001
9. Raj N, Zheng Y, Kelly V, et al.: PD-1 blockade in advanced adrenocortical
carcinoma. J Clin Oncol. 2020, 38:71-80. 10.1200/JCO.19.01586
10. Claps M, Cerri S, Grisanti S, et al.: Adding metyrapone to chemotherapy
plus mitotane for Cushing’s syndrome due to advanced adrenocortical
carcinoma. Endocrine. 2018, 61:169-72. 10.1007/s12020-017-1428-9
From https://www.cureus.com/articles/135058-an-aggressive-case-of-adrenocortical-carcinoma-complicated-by-paraneoplastic-cushings-syndrome#!/
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Filed under: adrenal, Cancer, Cushing's, symptoms | Tagged: abs, adrenocortical
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CUSHING’S DISEASE PATIENTS ARE MORE LIKELY TO HAVE OCULAR HYPERTENSION
Posted on December 29, 2022 by MaryO
The following is the summary of “Increased Risk of Ocular Hypertension in
Patients With Cushing’s Disease” published in the December 2022 issue
of Glaucoma by Ma, et al.
--------------------------------------------------------------------------------
Ocular hypertension was more common in people with Cushing’s illness. The usage
of steroids in the body is a major contributor to high intraocular pressure
(IOP). Topical or systemic glucocorticoid use may increase the prevalence of
ocular hypertension in the general population from 30–40%. The prevalence of
ocular hypertension in endogenous hypercortisolemia and the ophthalmological
consequences following endocrine remission after surgical resection are unknown.
During the period of January 2019 through July 2019, all patients with Cushing’s
disease (CD) who were hospitalized at a tertiary pituitary facility for surgical
intervention had their intraocular pressure (IOP), vision field, and
peripapillary retinal nerve fiber layer thickness recorded.
Nonfunctioning pituitary adenoma (NFPA) patients and acromegaly patients from
the same time period were used as comparison groups. Researchers showed
postoperative changes in IOP, estimated the odds ratio (OR), and identified risk
variables for the development of ocular hypertension. About 52 patients with CD
were included in the study (mean age 38.4±12.4 years). Patients with CD had an
IOP that was 19.4±5.4 mm Hg in the left eye and 20.0±7.1 mm Hg in the right eye,
which was significantly higher than that of patients with acromegaly (17.5±2.3
mm Hg in the left eye and 18.6±7.0 mm Hg in the right eye, P=0.033) and NFPA
(17.8±2.6 mm Hg in the left eye and 17.4±2.4 mm Hg in the right eye, Ocular
hypertension was diagnosed in 21 eyes (20.2%) of CD patients, but only 4 eyes
(4.7%) of acromegaly patients and 4 eyes (4.5%) of NFPA patients. Patients with
CD had an odds ratio (OR) of 5.1 [95% CI, 1.3-25.1, P=0.029] and 6.6 [95% CI,
1.8-30.3, P=0.007] for developing ocular hypertension compared with the 2
control groups.
Higher levels of urine-free cortisol were associated with an increased risk of
ocular hypertension in CD patients (OR=19.4, 95% CI, 1.7-72.6). Patients with CD
saw a decrease in IOP at 1 month following surgery, and this improvement was
maintained for another 2 months. Researchers conclude that endogenous
hypercortisolemia should be included as part of the glaucoma assessment due to
the increased risk of ocular hypertension in CD. Ophthalmologists and
neuroendocrinologists should use their judgment in light of this finding.
Source: journals.lww.com/glaucomajournal/Fulltext/2022/12000/Increased_Risk_of_Ocular_Hypertension_in_Patients.3.aspx
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Filed under: Cushing's, symptoms | Tagged: Cushing Disease, ocular hypertension,
steroids, UFC | Leave a comment »
CRH STIMULATION TEST BOOSTS CUSHING DISEASE DIAGNOSIS
Posted on December 27, 2022 by MaryO
The study covered in this summary was published on Research Square as a preprint
and has not yet been peer reviewed.
KEY TAKEAWAYS
* Adding a corticotropin-releasing hormone (CRH) stimulation test immediately
following a 2-day low-dose dexamethasone suppression test (LDDST) ― what’s
known as a Dex-CRH test and was first introduced in 1993 ― identified Cushing
disease in 5 of 65 people (7.7%) with a confirmed diagnosis but who had
previously shown normal cortisol levels on a conventional LDDST.
* However, the Dex-CRH test also resulted in one (2.5%) false positive case
compared with an LDDST alone.
* Measuring serum dexamethasone levels further improved the diagnostic accuracy
of the Dex-CRH test.
WHY THIS MATTERS
* It can be challenging to diagnose Cushing syndrome and to differentiate
Cushing disease from nonneoplastic physiologic hypercortisolism caused by
conditions that can present with Cushing syndrome–like clinical features,
such as diabetes and obesity.
* The Dex-CRH test, first described in 1993, initially appeared superior to an
LDDST alone for ruling out nonneoplastic hypercortisolism, with a report of
100% sensitivity, specificity, and diagnostic accuracy. However, subsequent
studies that used different protocols and in which dexamethasone was not
measured had results that called into question the accuracy, sensitivity, and
specificity of the Dex-CRH test.
* This study reports the accuracy, sensitivity, and specificity of the Dex-CRH
test for diagnosing Cushing disease, performed as first described, in 107
patients, including 74 for whom dexamethasone was also measured.
STUDY DESIGN
* The researchers analyzed data from 107 patients with suspected Cushing
disease who underwent a Dex-CRH test during 2002–2014 at the Cleveland
Clinic.
KEY RESULTS
* Sixty-five people received a confirmed diagnosis of Cushing disease and
underwent follow-up for a median of 66 months. Cushing disease was not
confirmed in 42 patients who were followed for a median of 52 months.
* The median age of the 107 patients was 40 years, and 82% to 88% were women.
The median body mass index for these patients was 34–37 kg/m2.
* Among the 65 patients with confirmed Cushing disease, five patients (7.7%)
had a suppressed cortisol level no greater than 1.4 μg/dL after the LDDST but
were appropriately classified as having Cushing disease with a cortisol level
that surpassed 1.4 μg/dL by 15 minutes after CRH stimulation.
* In contrast, 3 of 42 patients (7.1%) in the group without confirmed Cushing
disease had an abnormal Dex-CRH test result. For one of these three patients,
the LDDST result was borderline normal, with a cortisol level post-DEX of 1.4
μg/dL that increased to 3.1 μg/dL by 15 minutes after CRH stimulation, which
resulted in this patient receiving a false positive diagnosis.
* A cortisol threshold value of more than 1.4 μg/dL during the Dex-CRH test was
diagnostic of Cushing disease with sensitivity of 100%, specificity of 93%,
and diagnostic accuracy of 97%.
* Among the 74 patients with dexamethasone measurements, the sensitivity of the
Dex-CRH test was unchanged, but the specificity and diagnostic accuracy
increased to 97% and 99%, respectively.
LIMITATIONS
* The study was retrospective.
* Not all patients underwent measurement of dexamethasone level.
* No uniform protocol existed for the diagnostic work-up and follow-up of
patients suspected of having Cushing disease.
DISCLOSURES
* The study did not receive commercial funding.
* The authors had no financial disclosures.
This is a summary of a preprint research study , “The Addition of
Corticotropin-Releasing Hormone to 2-Day Low Dose Dexamethasone,” written by
researchers primarily from the Cleveland Clinic and Johns Hopkins University
School of Medicine, published on Research Square, and provided to you by
Medscape. This study has not yet been peer reviewed. The full text of the study
can be found on research square.com.
From https://www.medscape.com/viewarticle/985984
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Filed under: Cushing's, Diagnostic Testing | Tagged: dexamethasone suppression
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