Buffalo Hump Research Articles

7777 The Adrenal Tumor Volumes Help Predict Surgical Indications And Postoperative Recurrence Of PMAH

Abstract Disclosure: S. Suzuki: None. K. Ishiwata: None. Y. Ruike: None. K. Igarashi: None. S. Watanabe: None. M. Sugo: None. M. Fujimoto: None. H. Koide: None. K. Yokote: None. Introduction: Primary macronodular adrenal hyperplasia (PMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing's syndrome (CS). Unilateral adrenalectomy of the largest gland has been proposed as a treatment for PMAH. However, there are cases of recurrence or persistence of hypercortisolism after unilateral adrenalectomy, and remaining tumor volumes might be associated. Objective: This study aimed to analyze whether tumor volume helps select treatment and predict postoperative recurrence. Patients and methods: We conducted a retrospective study of thirteen patients (five males and eight females, mean age 59 years) with CS due to PMAH in a single center. Eight patients with PMAH underwent unilateral adrenalectomy, but five PMAH were not. Long-term clinical and biochemical outcomes were analyzed in operated and non-operated patients (112±57 months after unilateral adrenalectomy and 144±49 months after initial diagnosis, respectively). Results: Eight PMAH patients who underwent unilateral adrenalectomy had total tumor volumes greater than 1500 mm2. After unilateral adrenalectomy, when the size of the remaining adrenal tumor was greater than 1000 mm2 (1053, 1268, and 1910, respectively), recurrence occurred along with enlargement of the residual adrenal tumor in two cases, and diabetes worsened in one case. However, when the size of the remaining adrenal tumor was less than 500 mm2 (18.2, 201, 214, 327, and 446, respectively), CS resolved. Among five PMAH patients who did not receive surgery, three PMAH patients with total tumor volume less than 1500 mm2 (856, 1038, and 1496, respectively) were biochemically controlled. However, in two PMAH patients with a total volume greater than 1500 mm2 (1610 and 1650), Cushing's signs, including buffalo hump and central obesity, appeared and worsened metabolic complications during follow-up. Conclusions: Our data suggest that adrenalectomy should be considered in PMAH with a total tumor volume greater than 1500 mm2. Unilateral adrenalectomy might have a risk of recurrence if the remaining adrenal tumor is over 1000 mm2. Further large-scale examination is required. Presentation: 6/3/2024

Exophthalmos Revealing Cushing’s Syndrome: A Case Report

Exophthalmos is typically associated with Graves’ ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing’s syndrome. We present a case of a patient aged 40 years with no pathological history presenting with generalized asthenia associated with weight loss of 15kg for 2 months, epigastralgia and incoercible vomiting. Clinical examination revealed a normotensive, normocardiac patient with pain on epigastric palpation, bilateral exophthalmos, and an unremarkable cervical examination No cushingoid syndrome especially no facial erythrosis, no purple stretch marks, no buffalo hump, no amyotrophy of the lower limbs. An abdominal CT scan performed before the abdominal pain revealed an adrenal incidentaloma, a right adrenal nodule, well limited, of low density, homogeneous, very discreetly and homogeneously enhanced after injection of contrast medium, whose hemodynamic behavior was in favor of a benign adenoma. The patient underwent surgery for right adrenalectomy, with morphological and immunohistochemical findings in favour of adrenocortical carcinoma, with clinical and biological improvement. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia.

An Interesting Case of Cushing Syndrome

An Interesting Case of Cushing Syndrome A 36 year old female presented to dermatology department with Multiple ulcers present in B/L axilla, B/L inframammary fold and B/L gluteal fold (Left > Right) since 3 months. Patient was referred from dermatology department to General Medicine for opinion on puffiness of face and swelling of legs . Patient had history of topical steroid use for itchy lesions in B/L axilla and inframammary folds 4 months back . H/o weight gain present. (6 kg). H/o puffiness of face and striae present over the abdomen. Patient had no known comorbidities previously. On physical examination patient had moon face ,facial plethora + ,Buffalo hump ,Loss of axillary hair, centripetal obesity , Abdominal striae , Sparse pubic hair , Pallor +. Systemic examination was normal. Investigations revealed new onset diabetes , serum cortisol was low (<0.5 mcg/ml) , serum acth was low (1pg/ml) , HB was low (7.9gm/dl) , total count was elevated (15,600) . Patient was diagnosed to have Iatrogenic (Topical steroid induced) Cushing Syndrome with Steroid induced atrophy and ulceration of skin & subcutaneous tissue leading to secondary fungal and bacterial infection , tinea incognito with secondary bacterial infection , iron deficiency anemia and newly detected Type 2 Diabetes mellitus. Patient was appropiately treated with iv antibiotics (Inj Piperacillin Tazobactum 4.5 g iv thrice daily), daily dressing on other supportive measures along with discontinuation of steroids. Patient improved symptomatically after discontinuing topical steroids, the cushingoid features started decreasing. The ulcers also started to heal. The patient was started on Tab. Hydrocortisone 10mg - 0 – 5mg and was referred for Endocrinologist opinion

HIV and adipose tissue: A long history linked to therapeutic classes of antiretrovirals

HIV infection has been controlled only since the introduction of triple therapy in 1996, combining, as antiretroviral agents, two nucleoside reverse transcriptase inhibitors (NRTIs) and one protease inhibitor (PI). However, among the NRTIs, the thymidine analogues stavudine and zidovudine led to lipoatrophy, either generalized or associated with visceral fat hypertrophy and buffalo hump. These molecules also increased insulin resistance and the prevalence of diabetes. They were replaced by other NRTIs or non-NRTIs (NNRTIs) that were considered to be free of adipose tissue (AT) toxicity. More recently, the NRTI tenofovir disoproxyfumarate (TDF) and the NNRTI efavirenz have been associated with inhibition of fat gain but not with clear lipoatrophy. Otherwise, the use of PIs led to a phenotype of trunk fat hypertrophy associated with cardiometabolic complications. To avoid their adverse effects, PIs have recently been replaced by a new class of antiretrovirals, the integrase inhibitors (INSTIs), which are well tolerated and effective in controlling HIV. However, this class has been associated with global weight gain, which may be important and concerning for some people living with HIV (PWH). Also, in the NRTI class, TDF has often been replaced by tenofovir alafenamide (TAF) due to bone and renal toxicities, and TAF has been associated with global fat gain. The cardiometabolic consequences of INTIs and TAF are primarily related to the associated weight gain. In the global obesogenic worldwide context, PWH are gaining weight as well in relation to poor health life conditions. Taking in charge obesity uses the same strategies as those used in the general population.

Awake Look-assisted Intubation in a Patient with Antiretroviral Therapy-induced Neck Lipodystrophy Posted for Liposuction Surgery

Abstract Highly active antiretroviral therapy (HAART)-induced lipodystrophy refers to a serious metabolic syndrome in human immunodeficiency virus-infected patients, characterised by the development of dyslipidaemia, insulin resistance, glucose intolerance and abnormal redistribution of body fat (peripheral lipoatrophy and lipohypertrophy). Lipohypertrophy refers to abnormal fat accumulation occurring commonly in the intra-abdominal compartment, breast, dorsocervical area (buffalo hump) or under the skin (lipomas). We report a case of HAART-induced lipodystrophy with a massive accumulation of adipose tissue in the dorsocervical region posted for liposuction.

Clinical Manifestations Before and After Surgical Treatment in Patients with Endogenous Hypercortisolism

Endogenous hypercortisolism (EH) presents varied and complex clinical manifestations (CM) but useful in follow-up. Objective to describe the CM in patients with HE before and after surgery. Material and Methods: Prospective study, cohort of 25 patients with HE, evaluated before and after surgical treatment. Medians and ranges of quantitative variables and frequency distributions of qualitative variables were determined. Cross tabulations of the presence (or not) of symptoms and signs before and after surgery were carried out, using the McNemar test, comparison of medians of quantitative variables before and after surgery using the Wilcoxon test. Results: Cushing's disease predominated (64%). The median age at diagnosis was 45 years and at the time of the study 46 years. Some symptoms that improved after surgery: fatigue (before 64% vs after 36%, p=0.039), weight gain (92% vs 44%, p=0.002), decreased concentration (64% vs 20%, p =0.001), menstrual disorders (76% vs 36% p=0.013). Signs were: bruising (72% vs 16%, p=0.000), moon face (80% vs 40%, p=0.006), buffalo hump (80% vs 48%, p=0.021), supraclavicular fat (68% vs. 40%, p=0.039). The medians of the variables decreased and the difference before and after treatment were statistically significant. Conclusions: In this cohort, all manifestations improved with weakened symptoms after surgical treatment, reaffirming the usefulness of the clinical method for early diagnosis and follow-up of HE.

O-10 Lymph node ACTH washout determined the source of ACTH secretion in a patient with Cushing's Syndrome due to medullary thyroid cancer

Abstract Introduction Ectopic ACTH syndrome constitutes 18% of Cushing's syndrome cases, and in rare cases, ACTH secretion from medullary thyroid cancer cells is the source of ectopic ACTH production. Here we report a catastrophic Cushing's syndrome case owing to medullary thyroid cancer in which the source of ACTH production was demonstrated by lymph node ACTH washout. Clinical Case A 23 year-old male was admitted to other clinics with complaints of inability to lose weight despite diet and exercise, and weight gain of approximately 40 kg, which had been going on for the last two years. At his application, his weight was 160 kg and his body mass index was 42.51 kg/m2. On physical examination, clinical stigmata suggesting Cushing's syndrome such as moon face, buffalo hump, supraclavicular fat deposition, acanthosis nigricans, centripedal obesity, purple striae on the abdomen were noted. The biochemical and hormonal evaluation confirmed ACTH-dependent Cushing's syndrome (Table 1). To establish the source of excess ACTH, first, a pituitary MRI was performed which revealed a 4×2 mm adenoma on the left side of the gland. In PET-CT, a 24×20 mm hypoechoic nodule in the inferior of the right thyroid lobe, and several pathological lymph nodes at the right side of the neck were detected. Serum calcitonin levels were high (6528 ng/L) and fine-needle aspiration biopsy from the thyroid nodule documented medullary thyroid cancer. The patient underwent bilateral total thyroidectomy and right cervical lymph node dissection. The pathology report was compatible with metastatic medullary thyroid cancer. After surgery, the remission of hypercortisolemia was not achieved and the patient was started on metyrapone treatment before the permanent treatment plan. Postoperative Gallium-68 DOTA PET-CT indicated residual disease and increased uptake in retropharyngeal, bilateral cervical level 2-4, right level 3, bilateral central, and bilateral lower cervical lymph nodes. The persistence of hypercortisolemia after surgery was most likely attributed to residual disease, however, it was considered that pituitary adenoma may also be the cause of ACTH secretion. Therefore, to confirm the source of ACTH secretion, ACTH washout samples were taken from the cervical lymph nodes and were found to be high as 958 ng/L. Reoperation was performed for recurrent mass and lymph node metastases. Conclusion Medullary thyroid cancer should definitely be considered in cases of ectopic ACTH syndrome. ACTH washing from the suspected lymph node may be an option for diagnostic purposes. In addition to the patient's primary tumor, hypercortisolism should also be treated.Table 1.Cushing's assessment tests

Launois-Bensaude Syndrome and Chronic Hepatitis B Infection in a Non-alcoholic Bosnian Woman: a Case Report

Background: Launois-Bensaude syndrome is a rare condition characterized by the accumulation of fat deposits under the skin. This disorder is most commonly found in individuals from Mediterranean countries, particularly those who are chronic alcohol consumers. The exact cause of this syndrome is still not fully understood. Objective: Launois-Bensaude syndrome, as the case of the patient with chronic hepatitis B infection we presented. Case report: A case study was conducted on a female patient admitted to the Clinic for Plastic and Reconstructive Surgery at the Clinical Centre University of Sarajevo. The patient underwent liposuction in the back of her neck after a thorough clinical examination and radiological assessment. She also had chronic hepatitis B virus infection along with other medical conditions. Discussion: Based on Schiltz's classification, the patient was diagnosed with type Ia Launois-Bensaude syndrome, which is characterized by fatty deposits in the neck region, often referred to as a "buffalo hump". Following the liposuction procedure, the patient was monitored for 2 years and did not experience a recurrence of the disease. Conclusion: Research suggests that liver disease plays a crucial role in the development of Launois-Bensaude syndrome. This highlights the importance of understanding the pathophysiological mechanisms underlying this rare disorder.

Iatrogenic cushing’s syndrome post intralesional triamcinolone acetonide in oral submucous fibrosis: 2 case reports

BackgroundOral Submucous Fibrosis (OFMF) is an oral potentially malignant disorder (OPMDs), strongly linked to betel quid chewing. It exhibits a significantly higher rate of malignant transformation compared to other OPMDs. The use of Intralesional Triamcinolone Acetonide Injection has emerged as a highly effective treatment option and has become the cornerstone of managing this condition.Case PresentationA 44-year-old female and a 40-year-old male presented with burning sensation and limited mouth opening, leading to diagnosis of OSMF. Both patients were treated with Triamcinolone Acetonide (TAC) Intralesional injections. Following a few months of treatment, a significant improvement in mouth opening was observed. However, both patients began experiencing symptoms such as facial rounding (mooning of the face), a buffalo hump, uneven hair growth, and swelling in the lower extremities. Upon recognizing these symptoms as indicative of Cushing’s Syndrome, the administration of TAC injection was discontinued. Both patients were referred to a higher-level medical facility for confirmatory tests, which revealed elevated cortisol levels in both morning (Cortisol A.M) and evening (Cortisol P.M).ConclusionTAC injection has been established as an effective treatment for OSMF. However, it is crucial to closely monitor patients for any adverse effects resulting from the treatment, which may arise from high dosage or increased frequency.

THU011 Cushing’s Disease Diagnosis Challenge With Standard Pituitary MRI: A Clinical Case Report

Abstract Disclosure: E. Kasiri: None. N. Shah: None. Introduction: Cushing’s disease (CD); the most common cause of endogenous Cushing’s syndrome (CS) is mainly caused by an adrenocorticotropin (ACTH)-secreting pituitary adenomas. Although MRI remains the preferred imaging to detect these adenomas, still about 50% of microadenomas may be missed using standard 1.5-Tesla MRI and on the other hand higher resolution MRI with 3T magnets is not widely used. Here we present a case of Cushing’s disease with equivocal 1.5T MRI which made a next step of management rather challenging. Case presentation: A 57-year-old female with history of hypertension, type 2 diabetes mellitus and obesity (BMI: 34.41 kg/m2) presented to our endocrinology clinic to establish care. Her main complaint was difficulty losing weight. She denied taking any steroids or oral contraceptive pill. Vitals showed BP: 124/85 mm Hg, HR: 100 bpm and T: 98.3 °F. Physical exam was significant for moon facies, buffalo hump and abdominal obesity. She was screened for Cushing syndrome with 1mg overnight low dose dexamethasone test which resulted in abnormal cortisol response with a level of 2.1mcg/dl (<1.8 mcg/dl). A 24hr urine free cortisol: 7.3 mcg/dl (<45 mcg/dl) and two late night salivary cortisol measurements resulted in 0.388 and 0.229 mcg/dl (> 3 times upper limit of normal). Other labs showed elevated baseline ACTH :67.4 pg/ ml (7.2 - 63.3 pg/mL), cortisol :19.4 mcg/dl (4.5-23 mcg/dl) and normal DHEAS:1864.1ng/ml (650-3400 ng/mL). We decided to distinguish ectopic vs pituitary source of hypercortisolism by an 8 mg (high dose) dexamethasone suppression test which showed suppressed ACTH <1.5 pg/ml, Cortisol :2.6 mcg/dl (>50% suppression from baseline) suggesting pituitary origin. The standard pituitary 1.5T MRI with and without contrast as next step resulted in questionable microadenoma in the right aspect of pituitary gland and was equivocal. Within the multidisciplinary team decision was made to perform 3T pituitary MRI rather than invasive inferior petrosal sinus sampling (IPSS) which confirmed the pituitary microadenoma of 8.1 mm size with no mass effect and patient is scheduled for transsphenoidal pituitary surgery without needing further testing. Discussion: 3T MRI, with higher magnetic field than 1.5T allows to improve image quality and help with tumor detection in Cushing’s disease. It reportedly has higher sensitivity (80%) and specificity (∼100%) compared to 1.5T MRI which has overall sensitivity and specificity of 71% and 87% respectively. However, it is not the first to order by the clinicians when evaluating the ACTH secreting pituitary adenoma. In the case of equivocal 1.5 T MRI usually the next step is IPSS which is invasive. With availability of Corticotropin Releasing Hormone in the USA being difficult, limited studies of using DDAVP and limitations in finding tertiary centers to perform IPSS, a 3T MRI would be more feasible as a next step to identify a pituitary adenoma. Presentation: Thursday, June 15, 2023

FRI054 Partial Lipodystrophy As A Manifestation Of Autoimmune Polyglandular Syndrome 1

Abstract Disclosure: S. Agarwal: None. C. Xing: None. M.S. Anderson: Stock Owner; Self; Medtronic Diabetes, Merck. A. Garg: None. Background: Autoimmune polyglandular syndrome 1 (APS1) is a rare autosomal recessive disorder due to mutations in AIRE gene that manifests mainly with chronic mucocutaneous candidiasis, primary hypoparathyroidism and adrenal insufficiency. We report an extremely rare APS1 patient who developed partial lipodystrophy during adulthood. Clinical Case: This 39-year-old white female developed frequent diaper rashes, oral thrush and tetany at the age of one year due to candidiasis and hypoparathyroidism. At 6 years, she developed hypothyroidism. At 11 years, she developed fatigue, excessive thirst, and anorexia due to primary adrenal insufficiency. At 13 years of age, she had thelarche and adrenarche but never attained menarche. At 14 years, she underwent a sibling matched allogenic bone marrow transplant due to multiple antibiotic-refractory fungal infections. At 35 years, her serum triglycerides level was 904 mg/dL and loss of subcutaneous fat from the upper and lower extremities and hips was noted but she had increased subcutaneous fat on the chest and abdomen as well as buffalo hump. Thigh skinfold thickness was 8 mm (<10th percentile of normal). A whole-body dual-energy X-ray absorptiometry revealed a total body fat of 38% with lower extremity fat of 25%-27% (<1st percentile of normal). Whole exome sequencing on DNA extracted from saliva revealed two pathogenic variants in AIRE gene; c.83T>C; p.Leu28Pro and c.769C>T; p.Arg257* confirming the diagnosis of APS1. No other pathogenic variants were noted in the known lipodystrophy genes. Serum was negative for anti-perilipin 1 autoantibody. Discussion: Biallelic mutations in the AIRE hinder the process of selective apoptosis of T-cells in the thymic medulla that have a strong affinity to self-antigens leading to autoimmunity manifesting as APS1. Previously, a 5-year-old boy with APS1 was reported to develop generalized lipodystrophy and his serum was positive for anti-perilipin 1 autoantibody. Recently, serum anti-perilipin-1 autoantibodies have also been reported in the Aire gene knock out mice. In contrast, our patient has partial lipodystrophy and was negative for anti-perilipin 1 autoantibody. This suggests that patients with APS1 may harbor many different autoantibodies against adipocyte expressed proteins and develop different patterns of autoimmune lipodystrophies. Presentation: Friday, June 16, 2023

FRI324 Pituitary Macroadenoma With Carcinomatous Transformation And Multiple Cranial Masses And Spinal Leptomeningeal Carcinomatosis: A Case Report

Abstract Disclosure: C. Cristancho: None. G. Szabo: None. Background: Aggressive pituitary tumors are invasive tumors not responding to standard therapies and presenting with multiple local recurrences; if metastases occur, the tumors are defined as pituitary carcinoma (PC) Clinical Case: 51- year-old Hispanic woman with history of pituitary macroadenoma diagnosed at the age of 41, when presented with weight gain and decrease in visual acuity. Per records initial Brain MRI reported hyperintense sellar lesion of 2.1x2.3x2.6 cm with supra-sella extension and shift of optic chiasm. Patient had 4 pituitary surgeries between 2014-2018 due to growth and had radiation and Temozolomide between 2018-2020. Pathology was diffusely positive for ACTH, PRL, and Ki-67 was 10-20%, P 55 positive staining was 25-30%. As a result from pituitary surgeries patient developed central hypothyroidism, and central AI. Subsequent mornings cortisol were nl between 16-18. No UFC or LNSC were available. MEN1 negative. Brain MRIs done between 2019 and 2020 showed stable 13x8 mm residual pituitary mass with postoperative changes. Patient denied history of diabetes, fragility fractures, HTN, DVTs. She had regular menstrual periods until age of 42. Had left adrenal mass resection in 2019 with benign pathology results. During her initial endocrinology visit her ROS was positive for lower back pain and mild proximal muscle weakness. Patient denied visual changes, headaches, skin changes, polydipsia or polyuria. Physical exam was remarkable for small buffalo hump, and 5-/5 proximal muscle weakness. Negative for moon face, purple striae, acanthosis nigricans, hirsutism, acne. Initial tests were consistent with ACTH-dependent CS: elevated cortisol PM: 18.2 (n <10ug/dL) and LNSC 0.58 and 0.64 mcg/dl (n <0.09), 24-hr UFC 264 (n <50 mcg/dl), abnormal ACTH 150 (n <50 pg/ml).Brain MRI revealing extra-axial mass of 1.6 x1.1 cm overlying posterior aspect of right cerebellar hemisphere and focal lesion in left prepontine cistern of 0.8 cm. Leptomeningeal Enhancement. No sellar mass. Spine MRI showed scattered nodules lining the cord throughout the cervical and upper thoracic spine concerning for metastatic disease. Right cerebellar mass biopsy positive for metastatic pituitary neuroendocrine tumor corticotroph cell subtype, ACTH, chromogranin A, synaptophysin positive; PRL, TSH, GnRH, alpha subunit staining was negative; Ki-67 of 2%. Temozolomide, Capecitabin, and Ketoconazole 200 mg TID were started. Subsequent LNSC <0.03 and <0.03 mcg/dl, UFC at 3 different times were 12.8, 11.2 and 8.1 mcg/dl showing rapid decrease of cortisol levels in a 4-week period. A block-replace approach was decided with Dexamethasone 1 mg daily and a lower dose of Ketoconazole 200 mg BID. Patient reports no side effects. Presentation: Friday, June 16, 2023

PAEDIATRIC ADRENOCORTICAL CARCINOMA: A CASE REPORT

Adrenocortical carcinoma (ACC) is a relatively rare but an aggressive neoplasm that can have variety of manifes­tations. It can manifest in the form of virilization, hyperaldosteronism, cushingoid features or combination of that. Due to rapid appearance of signs and symptoms in children, these tumours can be diagnosed early however if delayed can bear worse prognosis as they tend to metastasize early in course of disease progression. We report a case of 4 years old female patient from Chitral, Pakistan who was referred to Endocrinology Department from Paediatric Department of Hayatabad Medical Complex, Peshawar Patient presented at paediatric OPD with com­plaints of weight gain, hirsutism, rashes over the face and acne. Patient had typical moon facies, buffalo hump and distended abdomen without striae as well as mass in the left hypochondrium on examination. On further evaluation clitoromegaly and pubic hair tanner stage 4 were found. Both Ultrasound and CT-Scan showed left supra-renal soft tissue mass invading the inferior venacava (IVC). She was further evaluated and her overnight dexamethasone suppression test (ODST) and serum testosterone levels were high although serum sodium and potassium were normal ruling out hyperaldosteronism. Next, biopsy was performed which was consistent with adrenocortical carcinoma. ACC although rare is highly aggressive tumor that requires early detection and diagnosis specially in children. If diagnosis is delayed prognosis may not be favourable. It therefore requires avid clinical suspicion to proceed onto diagnostic lines for patient’s benefit as well as physician’s satisfaction.

A case report and literature review of Carney complex with atrial adenomyxoma

BackgroundCarney complex (CNC) is a rare multiple endocrine neoplasia syndrome characterized by mucocutaneous lentigines/ blue nevi, cardiac myxoma and endocrine overactivity. Here, we report a CNC case with PRKAR1A gene mutation characterized by left atrial adenomyxoma to explore the diagnosis and treatment of CNC.Case presentationA 42-year-old woman with a history of cardiac tumour surgery presented with typical features of Cushing syndrome, including central obesity, buffalo hump, mild facial plethora, purple striae on the lower abdomen, and spotty skin pigmentation. Left atrial adenomyxoma and thyroid papillary carcinoma were identified by postoperative histologic assays. Genetic screening revealed a pathogenic germline heterozygous mutation of c.682C > T (p.R228X) in exon 7 of the PRKAR1A gene. The clinical features and normal ACTH levels suggest this patient suffered the ACTH-independent primary pigmented nodular adrenocortical disease (PPNAD) with cyclic hypercortisolism or ACTH-dependent Cushing syndrome.ConclusionCNC is uncommon, however, if a patient develops clinical features involving multiple endocrine and non-endocrine tumors, especially Cushing syndrome and cardiac myxoma, CNC should be considered. Genetic analysis is recommended in patients with suspected CNC.

The Superficial Fascia System: Anatomical Guideline for Zoning in Liposuction-Assisted Back Contouring.

Dorsal contour deformity presents with different manifestations in each part of the back, such as back rolls, iliac crest deposit, and buffalo hump. However, scant current literature exists on the anatomical basis of dorsal contour deformity. The aim of this study was to better understand the anatomical characteristics of the back, and to propose evidence-based zoning principles for liposuction-assisted back contouring. A total of 12 fresh cadavers were dissected for observation of each hierarchy in the vertical order (skin to deep fascia) and transverse comparison of the superficial fascial system (SFS) in the scapular-infrascapular-lumbar triangle region. Full-dorsum vectorial sections were used for the study of suprascapular fat deposits. The SFS acts as a bridge connecting all levels of the dorsal subcutaneous tissue. Macroscopically, it is denser in the scapular and lumbar triangle regions and looser in the infrascapular region; microscopically, the ultrastructure of the retinaculum cutis consists of loose interlobular fascia and stiff functional fascia. The regional variation pattern of the SFS in the back was consistent with observed back contour deformities in Asian female patients. A better understanding of the topographic anatomy of the back applied to evidenced zoning is the basis for improving surgical precision and avoiding dorsal contour deformity.

A Case Report on Multiple Adverse Events Associated with Systemic Usage of Dexamethasone

Dexamethasone is a corticosteroid used in the management of various allergic conditions, inflammatory and autoimmune disorders such as rheumatoid arthritis, nausea and vomiting associated with cancer chemotherapy, multiple sclerosis, cerebral edema. ADRs associated usage of corticosteroids includes steroid-induced hyperglycemia, osteoporosis, dyslipidemia, steroid-induced Cushing syndrome, cataracts, increased risk of infections, and thinning of the skin. Cushing's syndrome is a hormonal disorder triggered due to chronic exposure of body tissues to high levels of cortisol. As corticosteroids are widely used by physicians for the management of various inflammatory disorders, patients must be warned about the possible ADRs associated with chronic use. In summary, this is a case of a patient who developed multiple adverse events after usage of dexamethasone that is evident by clinical features and laboratory investigations for which the patient was successfully provided with symptomatic and supportive care.
 Keywords: Adverse Drug Reaction (ADR), Buffalo hump, Hyperglycaemia, Osteoporosis.

ODP387 Hemihypertrophy: A Rare Clinical Presentation of Cushing's disease

Abstract Background Hemihypertrophy is a rare clinical presentation of Cushing's disease. It has been described in three newborns with massive enlargement of adrenal glands in presence of other congenital abnormalities and in a 17-year-old female with pituitary adenoma with a genetic diagnosis of Beckwith Weidman syndrome. We describe a case of Cushing's disease with lower extremity hemihypertrophy in the absence of features suggestive of an underlying syndrome. 1,2 Clinical Case: A 12-year-old male with no significant past medical history presented with poor growth and rapid weight gain. In addition, he had hemihypertrophy of left lower extremity interfering with his physical activity. Physical exam showed Tanner Stage 1 for pubic hair and testicular volume of 4 ml bilaterally, with no signs of hirsutism, bruising or purplish striae. Initial work up showed normal thyroid hormone and growth factor levels. Midnight salivary cortisol levels were 238 ng/dl and 51 ng/dl respectively (range <100 ng/dl). One mg dexamethasone suppression test showed suppressed cortisol level of 1 mcg/dl. At the subsequent visit seven months later, he had gained 13.8 kg and grown 0.9 cm in height. Physical exam showed facial plethora and buffalo hump, which had not been present at the initial visit. Left and right leg circumference above the knee were 22 inches and 20 inches respectively. Repeat midnight cortisol levels on two separate occasions were 251 ng/dl and 441 ng/dl respectively. Repeat 1 mg dexamethasone suppression test showed suppressed cortisol levels of 1.2 mcg/dl. Because of clinical picture consistent with Cushing syndrome and elevated salivary cortisol levels, 24-hour urine cortisol levels were ordered that were 145 mcg and 276 mcg/24h respectively (range 1-45 mcg/24hours). ACTH was elevated at 65 pg/ml. CT scan of abdomen and pelvis was normal. Left leg ultrasound was normal. MRI of brain showed a 4 mm hypoenhancing lesion in the pituitary gland to the left of midline. A peripheral DDAVP stimulation test showed elevated ACTH and cortisol levels suggestive of pituitary origin. MRI of legs showed asymmetric fat deposition causing hemihypertrophy. Patient underwent transsphenoidal resection of pituitary adenoma with postoperative remission (postop cortisol nadir <1 mcg/dl). At follow up visit after surgery, patient had lost 0.2 kg, buffalo hump had disappeared, and left leg circumference had slightly improved. Genetic testing ispending. Conclusion This case demonstrates that hemihypertrophy can be a presenting symptom of Cushing's disease.

PMON315 Hyperglycemia Dilemma: Concomitant Type 1 Diabetes Mellitus and Cushing's disease

Abstract Introduction Type 1 diabetes (T1DM) is an autoimmune condition causing destruction of insulin-producing pancreatic beta cells. About a third of patients present with DKA at diagnosis. Insulin is mainstay therapy to achieve glucose control. Glucose hemostasis is affected by several factors. Conditions including acute illness or endocrinopathy such as Cushing's disease can cause hyperglycemia. We present a case of a pediatric patient with T1DM and difficult to control hyperglycemia due to concomitant hypercortisolism. Case The patient is an eleven-year-old male, who first presented in DKA at the age of 10. HgA1c at diagnosis was 17.9%. GAD65 and IA2 antibodies were positive. Compliance to medications as well as post-hospital follow-up has been an issue. At his initial follow-up visit, 8 months after diagnosis, they reported adherence to prescribed insulin regimen although his A1c was 11.3%. Insulin dose was adjusted based on current A1c level and documented glucose monitor readings. 3 months after, improvement of A1c was documented at 10.7%. He however complained of eye swelling and noted to have elevated blood pressure. Due to concerns of nephrotic syndrome he was referred to nephrology. His renal function test and urine micro-albumin came back normal. He was started on Lisinopril 5mg daily but eventually switched to amlodipine 5mg daily to control his blood pressure. In the following 14 months, nephrology noted that his hypertension was difficult to control in spite of optimizing medications. He was eventually admitted for severe headache. Significant exam on admission revealed moon facies, buffalo hump, striae on his extremities. A CT scan revealed pituitary macroadenoma 1.1×2cm. TSH was slightly low, FT4 normal. Growth hormone was also elevated. ACTH was 38 pg/ml (n 7.2-63 pg/ml)and serum cortisol was 7.1mcg/dl (n 3-22 mcg/dl). 24hr urine cortisol sent but was still pending. The rest of the pituitary work up was normal. Patient was scheduled for transsphenoidal surgery. A repeat 24-hour urine cortisol was again sent prior to surgery assuming initial test was lost. Interestingly, the initial 24-hour Urine free cortisol came back significantly elevated at 1,304 mcg (normal 2.3-37mcg) and urine free cortisol is . Pathology came back as pituitary apoplexy. 24-hour urine cortisol right before surgery came back undetectable confirming this. Conclusion Non-adherence to medication is a common reason for persistent hyperglycemia in T1DM but providers should be vigilant of other possible etiologies. Continued follow-up and detailed physical exam to check for clues such as hypertension, striae, buffalo hump, and bruising can lead to unmasking a secondary etiology. We present the first reported case of type 1 diabetes and Cushing's disease from a functional pituitary macro-adenoma that subsequently develop pituitary apoplexy. It is important to note that 24 urine cortisol remains the gold standard for diagnosis regardless of normal serum cortisol levels. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PMON80 Case of Cushing's Disease That Presented as Diabetic Ketoacidosis and Successfully Treated With Stereotactic Radiosurgery During the COVID-19 Pandemic

Abstract Background Although Cushing's disease is known to impair glucose metabolism, diabetic ketoacidosis (DKA) as its initial presentation has been reported in only a few case reports. The first-line treatment for Cushing's disease is transsphenoidal surgery, but safety concerns during the COVID-19 pandemic has led to the utilization of other treatment options. We present a case of Cushing's disease that presented initially as DKA and was successfully treated with stereotactic radiosurgery. Case Presentation Our patient was a 36-year-old female known to have diabetes mellitus and hypertriglyceridemia for one year. She presented with one-week history of generalized weakness, blurring of vision, and elevated blood pressure. Her blood glucose level was high at 523 mg/dL, associated with ketosis and metabolic acidosis. She was diagnosed with diabetic ketoacidosis which resolved after hydration and intravenous insulin administration. Further history revealed that she has been experiencing progressive weight gain and infertility for the past five years. She had cushingoid features including obesity, scalp hair thinning, moon facies, acne, hirsutism, and buffalo hump. Midnight salivary cortisol was high at 9.34 ng/mL (0.20-4. 00 ng/mL). Low dose dexamethasone suppression test showed an unsuppressed serum cortisol level at 31.2 mcg/dL (<1.8 mcg/dL). Adrenocorticotropic hormone (ACTH) level was high at 88.5 pg/mL (<46 pg/mL) and pituitary magnetic resonance imaging (MRI) showed an enlarged pituitary gland measuring 13.4 mm in the craniocaudal dimension with leftward displacement of the pituitary stalk. She was diagnosed with Cushing's disease and was initially advised transsphenoidal surgery. Due to concerns of COVID-19 transmission, the team and the patient decided to do stereotactic radiosurgery (SRS). The procedure was performed without complication. At 2 months follow-up, there was noted improvement of blurring of vision, normalization of cortisol level to 20.72 mcg/dL (4.30-22.40 mcg/dL), and a decreased ACTH level at 62.19 pg/mL. There was a 4 mm decrease in the craniocaudal dimension of the pituitary gland on repeat pituitary MRI. Thyroid stimulating hormone (TSH) level normalized from 0.209 to 0.647 uIU/mL (0.55-4.78 uIU/mL), and the glycohemoglobin decreased from 11.5% to 6.3%. Hypertension was controlled on one medication. Conclusion Stereotactic radiosurgery is usually reserved as a secondary option to transsphenoidal surgery. On average, normalization of cortisol levels after SRS takes 14 months. In our patient, the serum cortisol decreased to within normal levels at only 2 months after SRS. While a longer follow-up is desirable in order to assess the durability of control of hypercortisolemia, our case demonstrates that stereotactic radiosurgery is a safe and effective treatment option for Cushing's disease during the COVID-19 pandemic, when safety protocols and decreased hospital and patient resources might hinder the performance of transsphenoidal surgery. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Age-related trend of spine changes

Introduction. The increase in the number of elderly and senile people is recognized as a global problem, therefore, the development of new indicators that objectively reflect the aging of the human body is an urgent and significant task. However, the available quantitative indicators and methods used in the field of aging, according to WHO estimates, are limited and do not provide a deep understanding of the key aspects of healthy aging. This article offers information about the possibilities of studying the panoramic X-ray image of the entire spine for developing some new indicators and evaluating the age-related changes observed.The purpose of the work: to investigate the age-related spine changes on the basis of previously developed criteria for quantitative and qualitative assessment of the shape and position of the spine structures.Materials and methods. X-ray images of all spine parts were examined in the sagittal projection for 141 patients, 57 men and 84 women with dorsopathies. The selection of patients was carried out randomly. The cohort of patients was divided into 4 groups: 1st — 21–44 years (average age 32,7 years) — 31 people, 2st — 45–59 years (average age 52,6 years) — 39 people, 3st — 60–74 years (average age 66,8 years) — 50 people and 4st — 75–88 years (average age — 81,1 years) — 21 people. The study was carried out on a personal computer screen, without the participation and additional irradiation of patients. A digital X-ray image of the whole spine in the sagittal plane was obtained for each patient. On the combined digital radiograph the occipital vertical was drown starting from the outer hillock of the occipital bone along all spine parts. The anteroposterior axes of CV–TV vertebrae (r axes) were drawn and the tangent line segments were applied on the contours of the base and dorsal planes of the sacral image. The angles were measured between the occipital vertical and the perpendiculars restored to these lines at the points of their intersection with the occipital vertical. Statistical processing of the data obtained was carried out.Results. In the course of statistical processing on the basis of the data obtained, some distinctive features of the studied structures were identified, methods of their quantitative assessment were proposed and boundaries of the proposed indicator registration were determined. The peculiarities of changes in the position of the spine depending on age are revealed: for people over 70 years, the occipital vertical most often passes anteriorly from the anterior contours of the bodies or crosses the bodies of the thoracic vertebrae; for people over 60 years, the cervical-thoracic transition at the CVII–TIII level more often has the form of enhanced kyphosis («Bear withers» or «Buffalo hump» type), and the head is in a forward position (FHP); for people over 70 years, the sacrum more often has an upright position.Conclusion. The proposed diagnostic method made it possible to determine position type of the spine structures and to characterize the age-related spine changes.