Bilateral Adrenal Glands Research Articles

Unilateral primary adrenal natural killer/T-cell lymphoma: Role of fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography for staging and interim response assessment

Primary adrenal lymphoma (PAL) is a rare malignancy often involving bilateral adrenal glands. Diffuse large B-cell is the most common histological type. Unilateral presentation and T-cell/natural killer (T/NK) cell histological type is rarer. We report fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography scan findings in a case of unilateral T/NK cell PAL performed for staging and interim treatment response assessment.

An Unusual Cause of Cushing’s Syndrome and Virilization

Question: A 12-year-old girl had suffered a disease for 2 years. Physical examination showed the central obesity with a body mass index of 22.68 kg/m2, and clinical features with facial redness, thinning of the skin, growth of fat pads along the collarbone, on the back of the neck, and on the face, and purple striae on her arms and legs. Moreover, other symptoms including deepening of her voice, acne, scalp hair loss, development of pubic hair, and excessive facial and body hair were also found. Basal plasma cortisol levels (8 am, 29.16 μg/dL; normal range, 4.0–22.3) and 24-hour urinary free cortisol levels (1628.00 μg/24 h; normal range, 12.3–103.5) were elevated. The plasma adrenocorticotrophic hormone level (17.0 pg/mL; normal range, 0–46) was not increased, and there was no cortisol suppression after high dexamethasone. Plasma levels of testosterone (2541.6 ng/dL; normal range, 6.1–55), dehydroepiandrosterone sulfate (579.9 μg/dL; normal range, 17–343), and 17-hydroxyprogesterone (73.82 ng/mL; normal range, <2.9) were also elevated, although estradiol was not significantly increased (47.8 pg/mL; normal range, <460). Abdominal imaging showed a retroperitoneal mass, which was 7.9 cm in the longest axis, below the left kidney, and contrast agent and octreotide rapidly entered the mass. Bilateral adrenal glands were atrophied (Figure A–C). PET showed that the mass was below the left kidney with uneven increased metabolism (standardized uptake value max, 6.7). Surgery was performed to resect the retroperitoneal mass (Figure D, E). Postoperative plasma cortisol (0.13 μg/dL) and testosterone levels (13.6 ng/dL) decreased immediately. Did the mass cause the symptoms? What is the exact diagnosis of the girl? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Histologically, the neoplasm was diagnosed as an adrenocortical oxyphilic adenoma with myelolipoma. The histologic results are shown in Figure F. Immunohistochemistry showed that the neoplasm was positive for calretinin (Figure G), Melan-A, and Syn, but negative for adrenocorticotrophic hormone, AE1/AE3, CgA, HMB45, hepatocyte, chromogranin A, S-100, CD10, and smooth muscle actin. Ki67 was expressed in the nucleus of 1% of the tumor cells. Adrenal oncocytic adenomas are rarely found outside the adrenal gland, in sites presumed to arise from ectopic adrenal tissue. Our present case of an oncocytic adenoma was thought to be adrenocortical origin because of positive immunohistochemical staining for calretinin and Melan-A (adrenocortical markers), and negative staining for S-100 and chromogranin (adrenomedullary markers). Myelolipomas are rare tumors, with an incidence of 0.4% in extra-adrenal locations at autopsy. Only 10 cases of perirenal and 4 cases of renal myelolipomas have been described in the medical literature.1Brandler T.C. Reder I. Kahn L. Perirenal myelolipoma diagnosed on imprint: case report and review of the literature.Diagn Cytopathol. 2015; 43: 230-233Crossref PubMed Scopus (4) Google Scholar Sections of myelolipomas typically showing a variegated appearance with areas of yellow lipid-appearing tissue alternating with areas of dark brown friable tissue could be used to exclude the diagnosis of liposarcoma. Myelolipomas and ectopic oncocytic adenomas are rarely functional, but positive Syn staining in our case indicated that the mass had neuroendocrine function, which could explain the elevated levels of glucocorticoid and androgen. Some reports have described oncocytic adenomas or myelolipomas with secretory function.2Corpas Jimenez M.S. Ortega Salas R. Tenorio Jimenez C. et al.Myelolipoma associated with adrenocortical adenoma: an unusual cause of Cushing's syndrome.Endocrinol Nutr. 2014; 61: e7-9Crossref PubMed Scopus (7) Google Scholar, 3Surrey L.F. Thaker A.A. Zhang P.J. et al.Ectopic functioning adrenocortical oncocytic adenoma (oncocytoma) with myelolipoma causing virilization.Case Rep Pathol. 2012; 2012: 326418PubMed Google Scholar To the best of our knowledge, ectopic adrenocortical adenoma with myelolipoma, which causes Cushing’s syndrome and hyperandrogenism, has not been described before the current case. Therefore, the functional capacity that these tumors might sometimes have can easily be overlooked and create a diagnostic challenge. Correlation with histology, immunohistochemistry, and serum endocrine studies can be helpful in making a correct diagnosis.

Mifepristone Utilization in Cushing’s From Large Bilateral Adrenal Adenoma Prior to Surgery

Utilization of mifepristone was described to mitigate hypercortisolism from bilateral adrenal Cushing’s syndrome. A 47-year-old female on abdominal pain evaluation with CT was found to have a 5.4 × 1.7 × 4.9 cm right adrenal nodule and a 4.3 × 2.9 × 3.3 cm left adrenal nodule with calcification on CT scan with each having density of -3 Housefield units. She had hypercortisolism with testing revealing high urine free cortisol, unsuppressed cortisol on 1 mg and 8 mg overnight dexamethasone suppression testing, high 11 pm salivary cortisol × 2 along with elevated random cortisol and undetectable ACTH. Her DHEA-S, plasma metanephrine, and rostenedione, renin and aldosterone levels were normal. Adrenal venous sampling for lateralization of hypercortisolism source using dexamethasone 2 mg PO Q 6 hours × 1 day, showed adrenal vein epinephrine levels step up > 100 pg/mL, indicating successful catheterization. Each side adrenal vein to vena caval cortisol ratio > 4 was consistent with autonomous cortisol secretion and left to right adrenal cortisol ratio 50 lbs weight loss, improved blood pressure, complete healing of chronic lower extremity cellulitis and independent ambulation within 4 months. Initially right adrenal adenoma and then left adrenal adenoma were resected in two separate surgeries and pathology showed bilateral enlarged adrenal glands with multiple adrenocortical nodules without adrenal carcinoma. Mifepristone, FDA approved for inoperable Cushing’s, facilitated pre-surgery optimization by effectively mitigating hypercortisolism. Mifepristone was utilized as a bridge for Cushing’s medical management prior to surgery. J Endocrinol Metab. 2015;5(3):226-228 doi: http://dx.doi.org/10.14740/jem288w

Bilateral Adrenal Masses in a Patient in Remission From Acute Myelomonocytic Leukemia

CLINICAL PRESENTATION A 51-year-old man presented in the outpatient department with a 1-month history of persistent low-grade fever. He was diagnosed with acute myelomonocytic leukemia 4 years before and was treated with induction therapy of standard-dose cytarabine with daunorubicin, followed by consolidation therapy with cytarabine. Until this time, he had been in complete remission. Workup on admission revealed no pathogen detected in blood or sputum cultures. The early morning serum cortisol was 15.89 mg/dL, and urine vanillylmandelic acid level was normal. The bone marrow aspiration revealed normal cellularity and no residual blasts. The coronal computed tomography (CT) scan of abdomen and pelvis demonstrated bilateral adrenal masses, 10.8 cm in diameter on the right side (Figure 1A, black arrow) and 10.0 cm in diameter on the left side (Figure 1A, arrowhead). Celiac trunk nodal involvement was also found (Figure 1A, white arrow). 18Fluorine-fluorodeoxyglucose (18F-FDG) positron emission tomography/CT imaging showed “H” appearance of intense 18F-FDG uptake at bilateral adrenal glands (the maximum value of standardized uptake value [SUVmax] up to 30) and an ill-defined FDG-avid tumor (about 4.5 cm, SUVmax from 18.3 to 20.9) between bilateral lesions (Figure 1B). The core biopsy of the right adrenal mass disclosed features of diffuse large B-cell lymphoma, and he was treated with rituximab plus cyclophosphamide, liposomal doxorubicin, vincristine, and prednisone (R-CLOP). Follow-up CT scan obtained 3 months later showed nearly complete resolution of bilateral adrenal mass. Although secondary involvement of the adrenal gland occurs in up to 25% of patients with non-Hodgkin’s lymphoma, its clinical manifestation is usually ambiguous. Positron emission tomography/CT has potential advantages over traditional radiological examinations for diagnosis, staging of disease and detecting recurrences of malignancies. In this case, the “H” appearance of significantly higher 18F-FDG uptake helped to differentiate secondary adrenal involvement from a primary adrenal malignancy.

PP.32.13

Objective: Adrenal venous sampling (AVS) is the preferred method to distinguish excess unilateral from bilateral aldosterone secretion in patients with primary aldosteronism. Although it is invasive and expensive conventional CT adrenal imaging is considered to be inferior to AVS. To examine whether 128-slice dual-source CT using strict standardized radiological diagnostic criteria may approach the diagnostic performance of AVS. Design and method: From May 2012 to August 2014 we performed both AVS and 128-slice dual-source CT in 50 consecutive patients with PA. AVS was pursued after correction of hypokalemia and adjustment of antihypertensive therapy, under continuous ACTH infusion. Blood samples were taken from both adrenal veins (AV) and the lower caval vein (LCV). Selective cannulation was defined as an AV/LCV ratio for cortisol of ≥3. Lateralization was defined as a dominant/non-dominant ratio for aldosterone-to-cortisol of ≥4. In addition suppression of the contralateral side was required. Computer tomography imaging was performed using 128-slice dual-source CT with a 1mm-thickness slice. As a diagnostic criterion of adrenal nodule on CT-scan a focal change > 7 mm was used. A diffuse thickening of adrenal limbs > 5 mm was thought to fit with adrenal hyperplasia. A unilateral change (adenoma/hyperplasia) was defined as a change within one single adrenal gland with normal contralateral gland. Bilateral abnormalities included bilateral adenomas and/or bilateral adrenal hyperplasia. Results: The cannulation failed in 2 (4%) subjects. No complications were observed during and after procedures. Twentythree patients (48%) demonstrated unilateral aldosterone production (14 left-sided, 9 right-sided), and 25 (48%) patients were characterized by bilateral aldosterone production. In 43,8% of the patients there was disagreement between results of AVS vs CT. In 11 subjects (22.9%) a unilateral change was found on CT-scan, while there was no lateralization on AVS. In 7 patients (14,6%) CT–scan revealed bilateral abnormalities or symmetrical normal adrenal glands whereas in AVS unilateral aldosterone secretion was observed. In 2 cases there was complete discordance between the affected sides as diagnosed by CT and AVS. Conclusions: Despite adopting strict radiological criteria to differentiate uni- from bilateral disease, the diagnostic performance of 128-slice dual-source CT-scan is inferior to that of AVS.

Disseminated histoplasmosis

Histoplasmosis is a worldwide fungal infectious disease caused by inhalation of spores of Histoplasma capsulatum. It may present as chronic pulmonary infection or in disseminated form. Disseminated histoplasmosis frequently affects the adrenal gland; however, unilateral involvement in immunosuppressed patients is the usual presentation. We report a case of an immunocompetent old male with history of weight loss, fever and bilateral adrenal masses. Computed tomography (CT)-guided biopsy of adrenal mass revealed histoplasmosis. This case report emphasizes the importance of considering fungal infection as differential diagnoses of mass lesions involving bilateral adrenals even if the patients are immunocompetent, along with the commoner lesions at these sites.

Fluoride-18 fluorodeoxyglucose positron emission tomography-computed tomography in staging and response evaluation of rare case of non-Hodgkin′s lymphoma involving adrenals, liver, and bone

Fluoride-18 fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) is increasingly applied in staging and treatment response assessment of lymphomas. Multiple isolated cases with extranodal involvement of non-Hodgkins lymphoma (NHL), detected on FDG PET-CT, have been previously reported. Here, we report a rare case of extranodal NHL involving multiple sites namely bilateral adrenals, liver and tibia in addition to supraclavicular, iliac and inguinal lymph nodes on FDG PET-CT and treatment response was assessed in follow-up FDG PET-CT.

Mifepristone Utilization in Cushing’s From Large Bilateral Adrenal Adenoma Prior to Surgery

Utilization of mifepristone was described to mitigate hypercortisolism from bilateral adrenal Cushing’s syndrome. A 47-year-old female on abdominal pain evaluation with CT was found to have a 5.4 × 1.7 × 4.9 cm right adrenal nodule and a 4.3 × 2.9 × 3.3 cm left adrenal nodule with calcification on CT scan with each having density of -3 Housefield units. She had hypercortisolism with testing revealing high urine free cortisol, unsuppressed cortisol on 1 mg and 8 mg overnight dexamethasone suppression testing, high 11 pm salivary cortisol × 2 along with elevated random cortisol and undetectable ACTH. Her DHEA-S, plasma metanephrine, and rostenedione, renin and aldosterone levels were normal. Adrenal venous sampling for lateralization of hypercortisolism source using dexamethasone 2 mg PO Q 6 hours × 1 day, showed adrenal vein epinephrine levels step up &gt; 100 pg/mL, indicating successful catheterization. Each side adrenal vein to vena caval cortisol ratio &gt; 4 was consistent with autonomous cortisol secretion and left to right adrenal cortisol ratio &lt; 2 suggested bilateral adrenal Cushing’s syndrome. She had multiple co-morbidities including uncontrolled hypertension, poor functional status requiring walker and cane, chronic non-healing cellulitis, glucose intolerance and hypokalemia. Mifepristone was initiated for medical management of hypercortisolism prior to surgery, resulting in &gt; 50 lbs weight loss, improved blood pressure, complete healing of chronic lower extremity cellulitis and independent ambulation within 4 months. Initially right adrenal adenoma and then left adrenal adenoma were resected in two separate surgeries and pathology showed bilateral enlarged adrenal glands with multiple adrenocortical nodules without adrenal carcinoma. Mifepristone, FDA approved for inoperable Cushing’s, facilitated pre-surgery optimization by effectively mitigating hypercortisolism. Mifepristone was utilized as a bridge for Cushing’s medical management prior to surgery. J Endocrinol Metab. 2015;5(3):226-228 doi: http://dx.doi.org/10.14740/jem288w

Autopsy report of disseminated histoplasmosis: An important differential of adrenal enlargement

A 57-year-old diabetic male, presented with altered sensorium since 2 days. He had a history of weight loss, loss of appetite, intermittent low-grade fever of 6 months duration. He had been administered empirical antituberculous therapy some months ago with no alleviation of symptoms. On examination, he was asthenic, normotensive, and anemic with hepatosplenomegaly. Ultra-sonography abdomen revealed bilateral enlarged adrenals with hepatosplenomegaly. Clinically impression was of tuberculosis disseminated malignancy. He expired within 2 days of admission. At autopsy, both the adrenals were markedly enlarged showing extensive areas of necrosis on the cut surface. Histology showed them to be brimming with colonies of yeast-forms of Histoplasma capsulatum. These organisms were also seen in the spleen, lungs and kidneys. The patient had died of septicemia following disseminated histoplasmosis that was, unfortunately, not diagnosed during his life. Histoplasmosis is amenable to treatment with Amphotericin B. Prompt diagnosis and treatment could have helped in salvaging this patient.

Diagnosis and treatment of adrenocorticotrophic hormone-independent macronodular adrenocortical hyperplasia: A report of 23 cases in a single center.

The aim of the present study was to present the treatment observations of patients with adrenocorticotrophic hormone (ACTH)-independent macronodular adrenal hyperplasia (AIMAH) from a single medical center. A total of 23 patients with AIMAH were retrospectively reviewed according to the clinical features of the patient. Physical symptoms and biochemical evidence of Cushing’s syndrome (CS) were present in all the cases. High and low dose dexamethasone failed to suppress cortisone secretion on the suppression tests and ACTH levels were low in all the cases. Bilateral enlarged adrenal glands were observed with computed tomography scans in the 23 cases, which were determined to be bilateral adrenal macronodular hyperplasia by pathological analysis. Normal blood pressure was observed in eight patients in the 2–8 years following the bilateral adrenalectomy. A total of 15 cases underwent single side adrenalectomy and their blood pressures markedly decreased three years following surgery; medical treatment was administered to 12 of the 15 patients, while three patients underwent a contralateral adrenalectomy. Nelson’s syndrome was not diagnosed for any of the cases. AIMAH has unique endocrinological, radiological and pathological features, presenting as an independent etiological factor of CS. Diagnosis of AIMAH is usually derived from pathological examination and long term remission may be achieved by unilateral adrenalectomy. Contralateral adrenalectomy should be performed in cases of recurrence, when followed with periodical examination of the symptoms and the serum concentration of cortisol.

Spontaneous rupture of adrenal incidentaloma in a patient with subclinical Cushing’s syndrome, whose clinical expression is metabolic syndrome

A 68-year-old female with enlarged bilateral adrenal glands who was being monitored for metabolic syndrome (MetS) and subclinical Cushing’s syndrome was admitted to our hospital because of rupture of the left adrenal gland. We performed abdominal aortography and embolization of the left middle adrenal artery, and the patient’s pain resolved soon afterwards. After the left adrenal rupture, endocrinological studies still showed subclinical Cushing’s syndrome, and she exhibited no changes in the metabolic disorders. Because she refused removal of the left adrenal gland, we observed her clinical course carefully. One year after the adrenal gland rupture, abdominal computed tomography revealed complete absorption of the retroperitoneal hemorrhage and 131I-adosterol scintigraphy showed a similar result to that previously found. Although adrenal rupture is a rare condition, we should be aware of its possibility during the follow-up of incidental adrenal tumors. Subclinical Cushing’s syndrome has many common features with lifestyle diseases, such as excessive visceral fat, hypertension, metabolic disorders of glucose and lipids. Careful examinations are needed to follow up many patients with lifestyle-related diseases or MetS for a long period.

Cushing syndrome.

Cushing syndrome.

Early postmortem volume reduction of adrenal gland: initial longitudinal computed tomographic study.

We aimed to confirm whether postmortem adrenal volumetric changes occur by measuring adrenal volumes on computed tomography (CT). Fifty-five adrenal glands from 28 subjects who died were included. All subjects underwent antemortem CT (AMCT) and postmortem CT (PMCT) within 94-1,191min after death, followed by conventional autopsy. CT volumetry was performed using freely-available software. Differences between AMCT and PMCT adrenal volumes were evaluated statistically along with differences in the degree of volume change, elapsed time to PMCT, and presence of underlying malignant disease. The mean volume of the right adrenal gland decreased from 3.8cm(3) on AMCT to 2.6cm(3) on PMCT (P<0.001); the left adrenal gland decreased from 4.2cm(3) on AMCT to 3.1cm(3) on PMCT (P<0.001). Conventional autopsy revealed decreased intracellular lipid components in portions of the adrenal glands. No correlation between the adrenal gland reduction rate and the elapsed time from AMCT to death or from death to PMCT was observed (P=0.99 and 0.79; P=0.28 and 0.59 for the right and left adrenal glands, respectively). Significant differences in both the bilateral adrenal gland reduction rates and underlying malignant disease were found for the left adrenal gland (P=0.015), but not for the right (P=0.74). Adrenal volume reduction was observed on PMCT compared to AMCT. This highlights the need to further elucidate the mechanism of adrenal shrinkage during the agonal stage and after death. This may be explained by pathological findings of intracellular lipid depletion.

Bilateral adrenal gland haemorrhage: an unusual cause.

SummaryOur patient had drainage of a large amoebic liver abscess. This got complicated by a severe degree of hypotension, which required aggressive fluid resuscitation and hydrocortisone support. Computerised tomography (CT) of the abdomen revealed bilateral adrenal gland haemorrhage (BAH) resulting in primary adrenal gland failure, which was the cause for hypotension. Patient was on long-term warfarin for provoked deep vein thrombosis of lower limb, which was discontinued before the procedure. Thrombophilia profile indicated the presence of lupus anticoagulant factor with prolonged activated partial thromboplastin time (aPTT). Patient was discharged on lifelong warfarin. This case emphasises the need for strong clinical suspicion for diagnosing BAH, rare but life-threatening condition, and its association with amoebic liver abscess and anti-phospholipid antibody syndrome (APLS).Learning points Recognition of BAH as a rare complication of sepsis.APLS can rarely cause BAH.

Pulmonary Mineralization Secondary to Iatrogenic Hyperadrenocorticism in a Dog

An 11-year-old, intact male Shih Tzu presented with obesity, thin hair coat, and abdominal distention. The patient had previously received corticosteroid therapy for dermatitis. Thoracic radiographs showed incidental findings of a generally diffuse bronchointerstitial pattern and markedly radiolucent pleural lines between the middle and caudal lung lobes, but clinical signs related to the thoracic radiographic findings were absent. Echogenicity of the hepatic parenchyma was increased and bilateral adrenal glands were not enlarged on ultrasonography. On computed tomography, the central regions of the lung lobes showed ground-glass attenuation, the peripheral regions of the lung lobes were relatively hypoattenuated compared with the central regions, and hyperattenuated nodules were distributed throughout the pulmonary parenchyma. Iatrogenic hyperadrenocorticism and secondary pulmonary mineralization was diagnosed on the basis of diagnostic imaging and adrenocorticotropic hormone stimulation test.

Anterior panhypopituitarism in diffuse large B-cell stage IV lymphoma

The most common presentation of metastases to the pituitary gland in systemic lymphoma is diabetes insipidus resulting from infiltration of the infundibulum/posterior lobe. We describe a 69-year-old man with diffuse large B-cell stage IV lymphoma who presented with anterior pituitary hypofunction, without features of posterior pituitary involvement. He presented with a few months of postural dizziness and hypotension, weight loss, fever, strabismus of right eye and a superficial abdominal wall mass. At this time he had no history of malignancy. Biochemically he had hypovolemic hyponatremia, secondary hypothyroidism and adrenal insufficiency. Further hormonal work-up revealed panhypopituitarism but no diabetes insipidus. Imaging of the brain, thorax and abdomen demonstrated diffuse intracranial pachymeningeal thickening and enhancement, multiple lymphadenopathies, a bulky right adrenal gland and a large left suprarenal mass, which were indicative of an infiltrative disease. Imaging of the pituitary showed heterogeneous enhancement of the anterior lobe with an unremarkable pituitary stalk and posterior lobe. Biopsy of the superficial abdominal wall mass revealed diffuse large B-cell lymphoma confirmed by bone marrow aspiration biopsy. Positron emission tomography (PET) scan confirmed diffuse systemic disease involving the right orbital apex, bilateral adrenal glands, bone and bone marrow, retroperitoneum and subcutaneous tissues; however, the pituitary gland, infundibulum and hypothalamus did not show any lesions on the PET scan. The patient was commenced on two cycles of chemotherapy but unfortunately died, thus recovery of pituitary function was not tested. Pure anterior pituitary hypofunction can uncommonly present in individuals with metastases to the pituitary gland, in contrast to the more common posterior pituitary/infundibulum involvement.

Clinical characteristics of adrenocorticotropic hormone independent macronodular adrenal hyperplasia: a report of 30 cases

To explore the clinical characteristics of adrenocorticotropic hormone (ACTH) independent macronodular adrenal hyperplasia (AIMAH). A total of 30 AIMAH patients from January 2001 to December 2011 at our hospital were reviewed retrospectively and their clinical data collected. AIMAH was equally distributed between genders. Their mean age was 44 ± 9 years and median course of disease 5 years. Hypertension was the most common clinical manifestation. Circadian rhythm of plasma cortisol disappeared in all patients, and the level of 24 hour urinary free cortisol (24 hUFC) was normal only in 4 (13.3%) patients. Both low and high dose dexamethasone suppression tests were not suppressed in 30 (100.0%) and 28 (93.3%) patients respectively. The stimulation tests for detecting aberrant expression of hormone receptors were performed in 14 patients. At least one aberrant cortisol response was identified in 12 patients. Twenty-five patients underwent adrenalectomy. Among 7 patients of bilateral adrenalectomy, 6 achieved remission while 8 patients did so among 14 patients of unilateral adrenalectomy. AIMAH should be considered in patients with massively enlarged bilateral adrenal glands. Treatment modalities should be decided according to clinical manifestations and cortisol level so as to relieve symptoms and improve prognosis.

Prognostic value of semiquantification NP-59 SPECT/CT in primary aldosteronism patients after adrenalectomy

Primary aldosteronism (PA), characterized by an excessive production of aldosterone, affects 5-13 % of patients with hypertension. Accurate strategies are needed for the timely diagnosis of PA to allow curability and prevention of excessive cardiovascular events and related damage. This study aimed to evaluate the usefulness of semiquantification of (131)I-6β-iodomethyl-norcholesterol (NP-59) single photon emission computed tomography (SPECT)/CT in differentiating aldosterone-producing adenoma (APA) from idiopathic adrenal hyperplasia (IAH) and in predicting clinical outcomes after adrenalectomy. We retrospectively reviewed 49 PA patients who had undergone adrenalectomy after NP-59 SPECT/CT within 1 year. A conventional visual scale (VS) and two semiquantitative parameters generated from SPECT/CT, adrenal to liver ratio (ALR) and lesion to contralateral ratio of bilateral adrenal glands (CON), with cutoff values calculated by receiver-operating characteristic (ROC) analysis, were compared with pathology results and postsurgical outcomes to determine the accuracy. An ALR cutoff of 1.84 and a CON cutoff of 1.15 showed an ability to distinguish adenoma from hyperplasia similar to VS (p = 0.2592 and 0.1908, respectively). An ALR cutoff of 2.28 and a CON cutoff of 1.11 yielded the highest sensitivity and specificity to predict postsurgical outcomes, and an ALR of 2.28 had an ability superior to VS (p = 0.0215), while a CON of 1.11 did not (p = 0.1015). Patients with either ALR or CON greater than the cutoff had a high probability of positive postsurgical outcomes (n = 36/38), while patients with both ALR and CON less than the cutoff had a low probability of positive postsurgical outcomes (n = 2/11). Semiquantification of NP-59 scintigraphy has an ability similar to VS in differentiating APA from IAH, but an excellent ability to predict postsurgical outcomes of adrenalectomy. An ALR or CON greater than the cutoff strongly suggests benefits from adrenalectomy, and both ALR and CON less than the cutoff implies a reduced chance of improvement in postsurgical outcome.

WITHDRAWN: Healthy pregnancy outcome with aggressive emergency management of neuroendocrine tumor in early pregnancy

Abstract Purpose Neuroendocrine tumors adversely affect the pregnancy outcome, if remain undiagnosed or untreated. Management of such a patient is challenging, especially while weighing the advantages and disadvantages of surgical and medical treatment during early pregnancy. The Case We present the case of a pregnant woman who presented to us with hypertension, episodes of headache, sweating and palpitations. On investigation, she was found to have bilateral heterogenous adrenal masses, a hypervascular lesion inferior to the aortic bifurcation, multiple hypervascular masses with calcifications in the pancreas, and thrombosis of the splenic vein. Twenty-four hour urinary metanephrine measured 350μg. Since she was in first trimester of pregnancy and waiting with such extensive disease was not favorable, she was aggressively treated along with urgent surgery in the form of bilateral adrenalectomy, distal pancreatectomy and splenectomy soon after the diagnosis. Histopathological examination of bilateral adrenal glands confirmed pheochromocytoma, tumour from aortic bifurcation showed paraganglioma, and pancreatic mass showed nodules having neuroendocrine morphology and positivity for chromogranin and synaptophysin. Biochemical studies revealed non- functioning nature of pancreatic tumour. She had a normal labor and delivery of a healthy baby. Genetic analysis revealed missense c.499C>T (p.ARG167TRP) mutation in von Hippel Lindau's gene. Principal conclusions Any advantage of delaying the surgery in a pregnant woman diagnosed of neuroendocrine tumours in first trimester should be weighed against potential hazards of long term medical therapy and the disease itself. We emphasize the importance of timely treatment, particularly in multifocal and extensive neuroendocrine tumours disease, for a favorable pregnancy outcome.

Large bilateral adrenal leiomyomas presenting as calcified adrenal masses: a rare case report.

We report the case of a 55-year-old woman with bilateral, large, calcified adrenal tumors who was treated by laparoscopic adrenalectomy. The patient presented with upper abdominal discomfort for the past 5 years. Her imaging showed bilateral enlarged adrenal glands up to 10-cm size with punctate calcifications. Positron emission tomography scan demonstrated moderate fluorodeoxyglucose avidity in the left adrenal mass. Bilateral laparoscopic adrenalectomy was performed through a transperitoneal approach. The postoperative period was uneventful, and the patient was discharged on the third postoperative day. Histology findings were consistent with adrenal leiomyomatosis.