Bilateral Nodules Research Articles

Synchronous bilateral typical pulmonary carcinoid tumours diagnosed by robotic navigation bronchoscopy: A unique case.

Pulmonary carcinoids are uncommon malignant neoplasms, believed to derive from specialized neuroendocrine cells known as Kulchitsky cells. We evaluated a 69-year-old female presenting symptoms consistent with carcinoid syndrome, such as intermittent flushing and diarrhoea, along with complaints of shortness of breath and cough. Imaging revealed bilateral lung nodules, confirmed by biopsy to be carcinoid tumours. The treatment of choice for carcinoid tumours is complete surgical resection. Nonetheless, individualized management plans are crafted based on the tumour's location and the patient's respiratory function as these present challenges to anatomical resection of tumours.

Indocyanine green localization for preoperative CT-guided localization of multiple pulmonary nodules.

This study assesses the safety and efficacy of using indocyanine green (ICG) for preoperative CT-guided localization of multiple pulmonary nodules. We included patients who underwent CT-guided preoperative ICG localization followed by video-assisted thoracoscopic surgery (VATS). Four primary outcomes were evaluated: technical success, pneumothorax, pulmonary hemorrhage, and postoperative hospital stay (PHS). Patients were classified into single nodule and multiple nodules groups, with further subgroups based on the side of localization including unilateral and bilateral subgroups. Univariate and multivariate analyses were used to evaluate risk factors for PHS and pneumothorax. A total of 374 patients (54.8 ± 11.4 years, 99 with multiple nodules). The success rate in the multiple nodules group was 98.3%, similar to single nodules. Apart from PHS, no significant differences were observed in outcomes between patients with single and multiple nodules. Longer PHS was observed for patients with multiple nodules (3 [2-4] days vs. 3 [3-4] days, p = 0.022). Multivariable analysis indicated longer stays were associated with pulmonary hemorrhage during localization, surgical blood loss, postoperative complications, and non-segmentectomy procedures. Advanced age emerged as the sole independent risk factor for pneumothorax. The success rate in the unilateral subgroup and the bilateral subgroup was 97.8% and 99%, respectively, with higher pneumothorax rates in the unilateral subgroup (38.3% vs. 20%). CT-guided preoperative ICG localization of multiple pulmonary nodules is safe and effective. It can be applied to both unilateral and bilateral nodules, supporting simultaneous VATS resection.

8078 TERT Positive Thyroid Nodule in the Setting of Metastatic Melanoma

Abstract Disclosure: E. Langnas: None. S. Chao: None. K.M. Gomes: None. A. Bhan: None. Metastatic disease to the thyroid gland is uncommon with a reported incidence of up to 4% in post-surgical specimens. Lung cancer is the most common primary tumor site in autopsy series. Clinically, renal cell carcinoma is the most common primary tumor site. Metastatic melanoma to the thyroid gland is clinically rare, however, in autopsy series, it has been reported with incidence of up to 39%. A 61-year-old man presented to the endocrinology clinic for an incidentally detected thyroid nodule. He had recently presented with a chronic cough, prompting a chest x-ray that revealed pulmonary nodules. Subsequent computed tomography (CT) showed numerous bilateral lung nodules measuring up to 2.5 cm and an incidental thyroid nodule. Ultrasound of the thyroid identified a 2.7 cm mixed cystic-solid right thyroid nodule. No risk factors for thyroid cancer were identified. His TSH was 3.51uIU (0.45 - 5.33 uIU/mL). The thyroid nodule was biopsied, and pathology revealed atypical spindle cells with irregular nuclear membranes and scattered histiocytes. A further review with cytopathology suggested non-thyroidal origin of the cells. Molecular testing with AFIRMA reported a telomerase reverse transcriptase (TERT) promoter mutation at C228T. The sequencing was negative for TERT C250T, BRAF, RET, PETC1, PTC3 and MTC. Due to concerns about metastatic disease, a positron-emission tomography (PET)-CT was conducted, revealing hypermetabolic lesions in numerous bilateral lung nodules, right thyroid nodule, porta hepatis lymph node, and a soft tissue nodule within the greater curvature of the gastric body. Subsequent biopsies of the right lung and gastric body confirmed the diagnosis of metastatic melanoma. On further questioning, it was found that the patient had a history of a skin lesion on his back that was excised several years ago with pathology reporting tumoral melanosis. Malignant melanoma has a poor prognosis due to high recurrence and metastatic rates. In melanoma, TERT promoter mutations are the most common mutations in noncoding regulatory regions. TERT C228T mutations and C250T mutations are seen in malignant melanoma and studies have shown that TERT promoter mutations are associated with aggressive clinical behavior. These TERT promoter mutations can also be seen in various other cancers including primary thyroid cancers with a similar association with more aggressive thyroid tumor characteristics. Our case describes a patient with a thyroid nodule and molecular testing noting a TERT promoter mutation. Both primary thyroid cancer and metastatic disease to the thyroid should be considered in these cases. This case also highlights the importance of obtaining a thorough history of prior malignancy in patients with thyroid nodules to assess the risk of metastatic disease. Presentation: 6/1/2024

8788 Implications of NTRK gene fusion in Papillary thyroid cancer

Abstract Disclosure: W. Medina-Torres: None. L. El Musa Penna: None. J. Segarra-Villafane: None. L.R. Sepulveda-Garcia: None. Z. Maisonet -Feliciano: None. I.C. Arroyo: None. M. Ramirez: None. M. Alvarado: None. L.A. Gonzalez-Rodriguez: None. V.J. Carlo: None. Thyroid cancer (TC) is the most common endocrine cancer. Papillary thyroid carcinoma (PTC) is the predominant contributor of this occurrence, comprising approximately 80% of all thyroid carcinomas. PTC has a good prognosis with a 5-year survival rate of more than 90% after resection. Nonetheless, factors such as tumor size, multifocality, nodal and distant metastases, characterize more aggressive cases including tall cell variant, columnar cell variant, and hobnail variant Aside from staging parameters, PTC behavior is mostly determined by morphologic variant. This is the case of 31 y/o female without known past medical history who was evaluated at endocrinology clinic due to a fine needle thyroid aspiration (FNA) positive for PTC, tall cell variant. Total thyroidectomy with central compartment dissection was performed. Pathology report presented a main tumoral mass of 3 cm but permeating multinodular pattern involving the entire gland with focal extrathyroidal extension, positive margins and six level VI positive lymph nodes. As per report, the tumor presented both papillary and follicular patterns of growth and other findings including oncocytic change, foci of clear cell change, low mitotic rate, and most notably "reverse polarization”. This pattern has the pathological features suggestive of the novel NTRK gene fusion mutation. The patient was treated with radioactive iodine (RAI) and one year post treatment there was evidence of elevated Thyroglobulin level at 904 ng/mLand thyroglobulin Ab at 2.48 IU/mL. A WBS +SPECT was done resulting in small uptake at left posterior thyroid bed and pulmonary intake. A chest CT revealed multiple bilateral sub centimeter nodules. Based on this result, a second course of RAI was given. Follow up neck ultrasound and chest CT presented persistent left level VI lymph nodes, increased amount of bilateral sub-centimeter pulmonary nodules and new thoracic(T11) lesion. FNA of left neck lymph nodes were positive for metastatic PTC for which left neck dissection was performed. Evaluation for actionable somatic mutations was requested based on resistance behavior, remarkable for TRIM33-NTRK1 chromosomal rearrangement. Patient was evaluated by Oncology service who started Larotrectinib treatment since it was approved for NTRK driven malignancies. Six-month post therapy patient achieved clinical and biochemical improvement. In the thyroid, NTRK-driven malignancies are rare and aggressive but treatable, in the evolving genomic era. As reviewed in the literature, NTRK fusion-positive TC exhibits distinctive clinicopathological features that could aid in identifying potential patients. Thus, in such patients who have PTC with non-classic pathologic patterns, specific molecular profile of the main tumor may have significant prognostic value that must be integrated into stratification system in order to provide target therapy on time. Presentation: 6/3/2024

8219 Biochemical Disease Severity Score For Hypercortisolism Can Predict Adrenal Insufficiency After Adrenalectomy For Mild Autonomous Cortisol Secretion

Abstract Disclosure: O. Hamidi: Advisory Board Member; Self; Corcept, Neurocrine Biosciences, Amryt Pharma, Recordati Rare Diseases, Lantheus, Xeris. B.H. Salama: None. P. Dogra: None. A.P. Dackiw: None. F. Nwariaku: None. A. Islam: None. A. Mehta: None. S.C. Oltmann: None. S. Mirfakhraee: None. T. McKenzie: None. T.R. Foster: None. M. Lyden: None. B.M. Dy: None. I. Bancos: None. Introduction: Patients with mild autonomous cortisol secretion (MACS) are at risk for adrenal insufficiency (AI) after unilateral adrenalectomy. We aimed to determine the prevalence, predictors, and duration of AI after unilateral adrenalectomy for MACS, and to compare baseline and peak cortisol post cosyntropin stimulation test (CST) in diagnosing AI. Methods: We conducted a dual-center retrospective cohort study of patients with MACS who underwent unilateral adrenalectomy and were evaluated with postoperative cosyntropin stimulation test (CST). AI was diagnosed when postoperative morning cortisol was <10μg/dL and/or CST peak cortisol was <18μg/dL. Baseline and CST peak cortisol levels were considered concordant when both met corresponding cut-offs. Biochemical (BSS) and clinical (CSS) severity scores for hypercortisolism were calculated. Results: In 100 adults with MACS (77% women, median age 56 years, interquartile range [IQR] 48-63), median adenoma size was 2.8 cm (IQR, 2.2-3.7), and 25 (25%) had bilateral nodules. Median BSS was 3 (IQR, 2-5) and median CSS was 4 (IQR, 2-8). After adrenalectomy, AI was diagnosed in 56 (56%) patients. AI was associated with higher BSS, larger tumor size, but not age, sex, BMI, tumor laterality. Baseline and CST peak cortisol levels were concordant in 76% of cases. In 14% of cases morning cortisol was <10μg/dL but CST peak cortisol was ≥18μg/dL, and in 10% of cases morning cortisol was ≥10μg/dL, but CST peak cortisol was <18μg/dL. On a multivariable analysis that included age, sex, BMI, BSS, laterality, and tumor size, only higher BSS (odd ratio [OR] = 1.6 per 1 point) was associated with postoperative baseline cortisol <10μg/dL (P<0.05 for all). Independent predictors of CST peak cortisol <18μg/dL were BSS (OR 1.5 per 1 point) and unilateral adenoma (OR 3.7). During a median postoperative follow up of 13 months, 33 (59%) patients recovered from AI. Median time to recovery was 6.0 months, with AI recovery in 28% of patients by 2 months, 42% by 3 months, 53% by 6 months, and 64% by 12 months. Conclusions: Prevalence of postoperative AI in MACS was 56%. Only 76% of patients had concordant baseline and CST peak cortisol, with peak CST cortisol being more commonly abnormal in patients treated for unilateral MACS. Patients with larger adenomas and higher BSS were at higher risk for AI. Patients with MACS should be counseled about the risk and duration of AI after adrenalectomy. Presentation: 6/3/2024

8194 Extramedullary Hematopoiesis Involving Bilateral Adrenal Glands In A Patient With Sickle Cell Anemia

Abstract Disclosure: V.T. Pham: None. L.N. Sendos: None. Title: Extramedullary hematopoiesis involving bilateral adrenal glands in a patient with sickle cell anemia Background: Extramedullary hematopoiesis refers to blood cell production occurring outside the bone marrow. It is a reactive process caused by inadequate production or excessive destruction of blood cells typically seen in patients with hereditary anemias or infiltrative bone marrow disorders. Extramedullary hematopoiesis usually occurs in the spleen, liver and lymph nodes, but it can also very rarely arise from the adrenal glands. Case Presentation:We present a case of a 42-year-old male with a history of sickle cell anemia who presented with chest pain and shortness of breath and had a chest CT angiography which incidentally showed a 5.6 x 3.8 cm left adrenal mass and a 2.9 x 2.1 cm right adrenal mass. Further evaluation with an MRI abdomen with and without contrast revealed a 5.5 cm lobulated left adrenal mass with patchy areas of microscopic fat and faint delayed heterogeneous enhancement as well as a 2.2 cm right adrenal nodule. The nodules were described as markedly T2 hypointense with heterogeneous T1 hyperintense/isointense signal on MRI with a broad differential diagnosis including extramedullary hematopoiesis in bilateral adrenal glands, lipid poor adenoma or neoplasm. Regarding his sickle cell disease history, the patient was diagnosed as an infant and had very rare episodes of sickle cell pain crisis as a child. However, once he entered adulthood, he began to have multiple episodes of sickle cell pain crisis requiring hospitalizations and transfusions. On further review of patient’s prior imaging scans during these previous hospitalizations, he was first noted to have bilateral sub centimeter adrenal nodules on a CT Abdomen without contrast done almost a decade prior to his current presentation. We ordered biochemical evaluation which did not show any evidence of adrenal hormonal hypersecretion. His case was discussed at our multidisciplinary conference with the radiology and surgery team and it was concluded that the imaging characteristics were most consistent with extramedullary hematopoiesis in the setting of bilateral adrenal gland involvement and patient’s history of sickle cell anemia. Therefore, no immediate surgical resection was recommended. Given the size of the left adrenal mass being greater than 4 cm in size, we advised follow-up imaging for surveillance. Discussion:There has been a rise in detection of adrenal incidentalomas due to the increased use of advanced diagnostic imaging in healthcare settings. When patients with hematologic diseases are found to have adrenal incidentalomas, it is important to consider extramedullary hematopoiesis in the differential diagnosis to avoid unnecessary invasive procedures and surgeries. Presentation: 6/2/2024

7648 Bilateral Adrenal Hemorrhage After Laminectomy: A Rare Complication

Abstract Disclosure: M. Rizk: None. S. Sharma: None. H. Qadeer: None. R. Paudel: None. C. Sherpa: None. Introduction: Bilateral Adrenal Hemorrhage (BAH) is an exceedingly uncommon complication of laminectomy, with few cases reported in the literature. In patients with history of any surgical procedure who present with unexplained symptoms of abdominal pain, back pain, hypotension, fever, confusion, or electrolyte abnormalities especially hyponatremia, acute adrenal insufficiency (AI) should be highly suspected. We herein describe a case of BAH following laminectomy. Clinical Case: 63-year-old male with past medical history of hypertriglyceridemia was admitted for post traumatic L1 burst fracture and was treated with T11-L3 fusion and T12-L3 laminectomy. The patient had normal bilateral adrenal glands after the fall on CT thoracic/lumbar spine imaging. The course was complicated by thrombocytopenia initially thought to be induced by therapeutic heparin since heparin antibody was positive. Serotonin-release assay returned negative weeks later ruling out heparin induced thrombocytopenia. Two weeks later, he developed unexplained tachycardia and CT angiogram was done which showed segmental pulmonary embolism and bilateral adrenal nodules measuring at least 3.9 cm on right side and 3.3 cm on left side. He was treated with Eliquis and was discharged home. Two weeks later, he was readmitted with weight loss of 30 lbs, weakness and altered mental status. His vital signs were significant for hypotension and tachycardia. Initial work up showed profound hyponatremia (sodium 121 mEq/L), hyperkalemia (potassium 6.1 mEq/L), hypoglycemia (Glucose 58 mg/dL), hypercalcemia (calcium 11.7 mg/dL) and acute kidney injury (creatinine 2.58 mg/dL). Repeat CT scan of chest/abdomen/pelvis without contrast showed bilateral hyperdense 4 cm adrenal mass/hematoma. Since initial CT of thorax/spine done after the fall showed normal adrenal glands, the new bilateral hyperdense adrenal masses noted post procedure is suggestive of adrenal hemorrhage/hematoma. Undetectable cortisol and high ACTH confirmed AI. Patient was treated with stress dose hydrocortisone followed by slow taper. MRI of pituitary gland showed partial empty Sella however since ACTH was high, empty sella was only an incidental finding and not the cause of AI. He is maintained on hydrocortisone 15 mg in the morning and 5 mg in the evening plus fludrocortisone 0.1 mg daily. His fatigue has improved, he is gaining weight and electrolytes are normal. Conclusions: This case report emphasizes the significance of timely diagnosis and management of BAH, which leads to primary AI. BAH is a rare complication following laminectomy. Clinicians should be aware of this possibility and consider it in the differential diagnosis of postoperative abdominal pain and electrolyte abnormalities, especially hyponatremia. Timely recognition and high index of suspicion should prompt empiric glucocorticoid replacement to decrease mortality. Presentation: 6/1/2024

6918 A Unique Case of Oncocytic Thyroid Cancer with an NBN Gene Pathogenic Variant

Abstract Disclosure: A. Grover: None. S. Jhawar: None. S. Shekhar: None. S.A. Avadhanula: None. G. Al-Naqeeb: None. G. Thota: None. P. Veeraraghavan: None. C. Cochran: None. M.Y. Kao-Hsieh: None. A. Gharib: None. F.E. Escorcia: None. J. Del Rivero: None. J. Klubo-Gwiezdzinska: None. S. Gubbi: None. Introduction: Oncocytic thyroid carcinoma (OTC), previously known as “Hürthle cell” carcinoma, makes up less than 5% of all thyroid cancers. OTCs are often aggressive with a high risk of metastasis, and a poorer prognosis compared to other forms of differentiated thyroid carcinoma (DTC). Molecular drivers of OTC include pathogenic variants (PVs) in NRAS, DAXX, CDKN1A, the mitochondrial genome and chromosomal alterations. PVs in the NBN gene in OTC have not been previously reported. Case: A 53-year-old male was noted to have a thyroid nodule on a routine physical examination. Patient denied any hyper/hypothyroid or compressive symptoms, and family history was unremarkable. Cytopathologic analysis of the fine needle aspiration biopsy of the nodule was consistent with a thyroid follicular neoplasm (Bethesda IV). A total thyroidectomy with central compartment neck dissection was performed. Histopathologic examination demonstrated a 7 cm x 4 cm x 4 cm OTC with capsular and lymphovascular invasion, but without extra-thyroidal extension or lymph node metastasis (pT3aN0Mx). Subsequently, adjuvant radioiodine (RAI) I131 therapy with 165 mCi was administered and post-therapy scan revealed neck uptake. One-year post-therapy RAI diagnostic scan was negative. Three years following the initial thyroid surgery, elevated serum thyroglobulin (Tg) levels were noted. A computed tomogram of the chest revealed multiple bilateral lung nodules. Further imaging ruled out involvement of other distant sites. A diagnostic lower lung wedge resection demonstrated metastatic OTC. The tumor stained positive for CK7, PAX8, TG, TTF-1 and negative for CK20 on immunohistochemistry. Next-generation sequencing of the tumor tissue (Oncomine Comprehensive Assay v3) revealed a PV in the NBN gene (c.2166_2167delGCinsAT; p.Trp722Ter). A year later, the serum Tg levels continued to rise, and the lung nodules had increased in size. Due to disease progression, lenvatinib was initiated. A [F18]-fluorodeoxyglucose positron emission tomography performed one year later demonstrated persistent pulmonary tumor burden with new osseous metastasis in the right scapula, left acetabulum, and the sternum. Zoledronic acid was initiated for the treatment of the skeletal metastases, and palliative external beam radiation (2000 cGy in 5 fractions) was administered to the sternal lesion to alleviate the bone pain. Conclusion: The NBN gene encodes for the protein nibrin, a member of the MRE11/RAD50 complex that is involved in DNA repair. PVs in NBN are commonly identified in non-thyroidal malignancies such as breast and lung carcinomas. While NBN PVs are rarely reported in DTCs, as per our knowledge, this is the first described case of OTC with a PV in NBN as the molecular driver, manifesting with an aggressive disease course. Identification of this novel PV therefore adds to the current knowledge of the molecular landscape of OTC. Presentation: 6/3/2024

6966 A Rhythmic Life-Threatening Enigma Unveiled: KCNH2 Mutation-Associated Torsades de Points Unmasking Primary Hyperaldosteronism in Cardiological Realm

Abstract Disclosure: S. Dejprapasorn: None. N. Nopparatana: None. S. Soonthornpun: None. R. Leelawattana: None. N. Kietsiriroje: None. S. Limumpornpetch: None. T. Nilmoje: None. W. Lohawijarn: None. T. Wongsuttipakorn: None. P. Choochuen: None. P. Limumpornpetch: None. Introduction: Primary hyperaldosteronism (PA) is characterized by hypertension and hypokalemia. However, its ability to masquerade life-threatening Torsades de Pointes makes it an unusual and dismissed diagnosis, revealing the link between PA and serious arrythmia. Clinical Case A 30-year-old female experienced three episodes of palpitations, followed by syncope. Upon admission, her blood pressure was 147/87 mmHg. ECG showed prolonged QT intervals with potassium level was 2.6 mmol/L (3.5-5.0 mmol/L). Continuous cardiac monitoring captured Torsades de Pointes. Despite potassium replacement, long QT still persisted. Pathogenic variant, c.2592+1G>A, within KCNH2 was discovered. Aldosterone concentration showed 69.65 ng/dL (9.70-62.60 ng/dL) and plasma renin activity was 0.12 ng/mL/h (1.50-5.70 ng/mL/h). Aldosterone-to-renin ratio was 580. Aldosterone post 4-h saline infusion test was 31.71 ng/dL which were consistent with PA. Adrenal unenhanced CT scan identified bilateral adrenal nodules (right 1.3 cm, left 1.1 cm). After receiving a cardioverter-defibrillator and spironolactone, the patient has had no palpitations, and their potassium level has remained normal. Clinical lessons: PA rarely presents ventricular arrhythmia. KCNH2 mutation may link between long QT syndrome (LQTS) and PA arises. LQTS type 2 is attributable to KCNH2 mutations, impacting potassium channel subunits crucial for adjusting currents during sympathetic activation. This is essential for shortening the QT interval with increasing heart rate. KCNH2 mutations disrupt this process, prolonging the QT interval and heightening the risk of arrhythmias. Aldosterone synthesis relies on calcium signaling triggered by potassium currents. Adrenal cortex hosts approximately 90 potassium channels genes, including KCNJ5 and KCNH2. Mutations in gene can disrupt the calcium signaling cascade, leading to excessive aldosterone production. While KCNJ5 mutation is well-documented in PA, the potential correlation between KCNH2 mutations and a similar mechanism remains unexplored in clinical reports to date. This case highlights the importance of a thorough comprehension of genetic cause contributing to pathogenesis of PA with complex clinical presentations. Presentation: 6/1/2024

12573 Management Challenges In A Young Male With Bilateral Macronodular Adrenal Hyperplasia

Abstract Disclosure: C. Nguyen: None. V. Ghernautan: None. L.Y. Melendez-Ramirez: None. M.C. Chiang: None. D. Lovre: None. Bilateral adrenal hyperplasia (BAH) may present in isolation or as part of a syndrome. It is classified according to nodule size as Macronodular: primary bilateral macronodular adrenal hyperplasia (PBMAH), or Micronodular: primary pigmented micronodular adrenal hyperplasia (PPNAD) and isolated micronodular adrenal hyperplasia (iMAD). High variability in cortisol secretion and disease progression is seen in both forms subclinical and overt Cushing’s Syndrome (CS). In PBMAH, bilateral adrenalectomy is advised for remission of CS, but benefits must be weighed against risks of lifelong adrenal insufficiency. A 45-year-old male with hypertension, depression, prediabetes, obesity presented to clinic for second opinion regarding incidentally discovered adrenal nodules five years prior with negative initial hormonal workup. Over the previous year he had gained 20 lbs. and developed proximal muscle weakness and sleep apnea. Physical exam showed uncontrolled hypertension and increased abdominal and supraclavicular fat. Hormonal testing revealed hypercortisolism. Adrenal CT confirmed bilateral adrenal nodules up to 4.9 cm with absolute washout ≥60% consistent with benign etiology. CS secondary to PBMAH was diagnosed. Prior to surgical evaluation. Ketoconazole 200 mg twice daily was started and titrated to 400 mg twice daily over the next three months. ACTH became detectable and symptoms of hypercortisolism improved. Right adrenalectomy was performed. Surgical pathology showed golden yellow-orange nodules with minimal grey adrenal parenchyma without necrosis or hemorrhage. The gland was 9.5 x 5.8 x 2.6 cm and weighed 85 g. Microscopic examination showed macronodular hyperplasia. Following surgery, he experienced a 17-pounds weight loss, improvement in blood pressure and quality of life. Three months later, hypercortisolism recurred. He elected to be monitored clinically and contemplating option of left adrenalectomy.Our case highlights the challenges in the care of patients with PBMAH. Bilateral adrenalectomy (BA) is the classical treatment. Its disadvantages include life-long hormone replacement with risk for adrenal crisis, increased morbidity, and mortality. Surgical alternatives, such as unilateral adrenalectomy (UA) and UA with subtotal contralateral adrenal resection, have shown promising results with up to 90% remission rates. The role of adjuvant novel medical therapy is unknown. Studies to compare cost, quality of life, cardiovascular risk, comorbidities, and life span between BA and UA are lacking. New biomarkers are needed to allow closer monitoring of disease progression. Additional research is necessary on medical management of hypercortisolism recurrence after UA and its long-term safety. Finally, given the presence of germline defects associated with adrenal hyperplasia, genetic testing should be offered to all patients with BAH. Presentation: 6/1/2024

12205 Challenging Case Of Anaplastic Thyroid Cancer With Unusual Histological Features: A Diagnostic Dilemma With High Grade Sarcoma

Abstract Disclosure: B. Gautam: None. B. Tiwari: None. M.S. Hossain: None. S. Hossain: None. S.C. Kumar: None. H. liao: None. Introduction: Anaplastic thyroid carcinoma (ATC) is a rare but highly aggressive thyroid cancer accounting for < 2% of cases, characterized by its rapid growth, extensive local invasion, and poor prognosis. Distinguishing ATC from other high-grade sarcomas, such as leiomyosarcoma, is challenging due to shared morphological features and overlapping immunoprofiles. We present a case involving a 73-year-old male presenting with rapidly growing neck mass, leading to diagnostic challenges differentiating between ATC and high-grade sarcomas. Case presentation: A 73-year-old male presented to ED with a rapidly growing painful neck mass for last 3 months, associated with dysphagia, dyspnea & dysphonia. He denied any fever, chills, weight loss and symptoms of hyperthyroidism or hypothyroidism. No personal or family history of thyroid cancer. On physical exam: vitals were normal, Neck exam revealed a left sided neck mass, mobile with deglutition, hard in consistency associated with hoarseness of voice. No stridor or wheeze noted on auscultation. Physical exam was otherwise normal. Laboratory tests showed normal CBC, CMP and TSH 1.45 mIU/L, T4 9.7 ug/dl. CT neck with contrast showed 5.4 x 5.6 x 8.2 cm large lobulated left thyroid mass with rightward airway deviation & substernal extension. Ultrasonogram of thyroid showed 9.6 x 5.3 x 3.3 cm left thyroid lobe with internal calcifications & extrathyroidal extension with several abnormal appearing cervical lymph node, largest measuring 4.9 x 2.4 x 3.3 cm. CT chest/abdomen/pelvis with contrast showed multiple bilateral lung nodules measuring up to 1.5 cm and 1.4 x 1.3 cm left gluteal soft tissue mass. IR-guided left neck mass biopsy was suggestive of primary spindle cell neoplasm with initial immunoprofiles consistent with a high-grade sarcoma, favoring leiomyosarcoma. Given the rapid growth of the neck mass with associated dyspnea, left thyroidectomy was performed and surgical pathology was suggestive of a high-grade sarcoma. However, subsequent pathology review made a final diagnosis of ATC, based on morphologic & immunohistochemical profile showing tumor cells strongly positive for p53 and Desmin with few cells positive for PAX-8, Cam 5.2 and CD68, and negative thyroglobulin, TTF-1, CK7, CK20, CK MNF116, pancytokeratin AE1/AE3 and CK5/6. Thyroseq revealed a distinctive molecular profile specifically positive for TERT and TP53 supporting diagnosis of ATC. Discussion: This case highlights the diagnostic dilemma posed by the resemblance between ATC and high-grade sarcomas due to overlapping clinical, morphological and immunohistology features. While both malignancies may exhibit similar morphological features, specific immunohistochemistry profiles along with molecular testing aid in distinguishing between them and guiding appropriate treatment strategies. Presentation: 6/1/2024

7618 Thyroid Nodules, Normal Thyroid Levels, and Chronic Anxiety: A Case for Sub-Clinical Toxic Multinodular Goiter

Abstract Disclosure: C.M. Barrera: None. Introduction: Chronic anxiety and panic disorders, as described in the Diagnostic and Statistical Manual of Mental Disorders (DSM-V), are the most common psychiatric conditions. Hyperthyroidism is a known cause of both chronic anxiety and panic disorder. Case 1: A 36-year-old overweight Latin female is referred for evaluation of multiple thyroid nodules. The thyroid ultrasound was heterogeneous, with multiple bilateral nodules. In her history, the patient has chronic anxiety with panic attacks resulting in frequent emergency room visits. The TSH and T4 levels were TSH 0.59 mIU/L (0.40-4.5) and T4 10.7 mcg/dL (5.1-11.9). An I[1]23- Thyroid Scan showed a hot nodule with mild suppression and an uptake of 31.8% (The top limit is 35%). The patient started methimazole 2.5 mg daily, and after two months, her chronic anxiety and panic attacks resolved. Post-methimazole TSH rose to 1.18 mIU/L, and T4 10.0 mcg/dL and she has weaned off anxiety medications. Case 2: A 49-year-old overweight Latin female is referred for increased thyroid nodules. She complained of six months of tachycardia and anxiety. The TSH and T4 levels were TSH of 0.828 mIU/L (0.40-4.5) and T4 of 7.9 mcg/dl (4.5-12.0). An I[1]23-Thyroid Scan showed no dominant hot or cold nodules, with an uptake of 36.4% (The top limit is 35%, within normal error limits). She started methimazole 5 mg daily, resolving her tachycardia and anxiety within five weeks. Post-methimazole TSH was 1.64 mIU/L (0.45-4.5), and T4 6.7 mcg/dL (4.5-12.0). Case 3: A 60-year-old overweight Latin female is referred for evaluation of multiple thyroid nodules of ten years. She complained of chronic anxiety and panic attacks for two years. The TSH and T4 were normal at TSH 0.36 mIU/L (0.35- 5.50) and T4 9.7 mcg/dL (4.5-10.9). The thyroid ultrasound showed multiple bilateral nodules. An I[1]23-Thyroid Scan showed a suspected hot nodule without appreciable suppression and an uptake of 33% (The top limit is 35%). The patient started methimazole 5 mg daily. After eight weeks, she reported the resolution of most of her chronic anxiety with post-methimazole TSH 2.39 mIU/L and T4 7.1 mcg/dL (4.5-12.0). Discussion: The normal range of TSH, typically from 0.4-4.5 mIU/L, is calculated by measuring the TSH levels among a normal population. The group range then determines the laboratory reference range. This contrasts with individual values, which have a narrower range, suggesting that each person has a unique thyroid level. This suggests that TSH levels at the extremum, associated with hyperfunctioning nodules contributing to a rise in uptake, may not be normal for that patient and may explain the anxiety and other symptoms. Learning Points: Evolving multiple autonomous thyroid nodules lowers TSH levels while raising thyroid uptake. Symptoms of chronic anxiety and panic attacks can predate abnormal thyroid levels and resolve with low-dose methimazole. Presentation: 6/1/2024

7314 The Thyroid Tetralogy: Hypothyroidism, Graves' Disease, AFTN, and Thyroid Cancer in a Single Patient

Abstract Disclosure: F. Sajid: None. F. Mohammadrezaei: None. K. Tiwari: None. J. Shapiro: None. F. Mohsin: None. T. Lin: None. Introduction: In this case, we explore a rare and complex situation involving a patient's shift from hypothyroidism to hyperthyroidism. This transition led to the diagnosis of Marine Lenhart Syndrome (MLS), an uncommon condition characterized by the coexistence of Graves' disease and autonomously functioning thyroid nodules (AFTNs). Adding to the complexity of the case, the patient was also diagnosed with papillary thyroid carcinoma (PTC). Case Presentation: A 47-year-old woman with a history of hypothyroidism, treated with levothyroxine for 14 years, presented with palpitations, weight loss, and insomnia for a few months. Her levothyroxine dose was gradually reduced and eventually discontinued by her primary care physician due to low TSH and high free T4 levels. Post-discontinuation, her symptoms were improving. Laboratory tests showed TSH 0.01 mIU/mL (0.39-4.08), Thyroglobulin (TG) 72 ng/mL (3-40), Free T4 2.5 ng/dL (0.8-1.8), Free T3 11.5 ng/dL (0.2-0.5ng/dL). Furthermore, Thyrotropin Receptor Antibody (TRAb), and Thyroid Peroxidase Antibody (TPO Ab) were positive leading to a diagnosis of Graves' Disease and initiation of Methimazole. After three months on Methimazole, her TSH remained suppressed with normalized free T4 and T3 levels. A thyroid ultrasound revealed bilateral nodules, with a calcified well-circumscribed nodule in the right mid-pole. A Radioactive iodine uptake (RAIU) scan showed a hyperfunctioning right mid-pole thyroid nodule. Fine-needle aspiration biopsy was consistent with Bethesda V, suggestive of possible malignancy. Consequently, she underwent a total thyroidectomy, with pathology confirming multifocal classic PTC, stage pT1bN0. Post-surgery, she developed hypothyroidism and was started on levothyroxine. Four weeks later, her TSH remain suppressed, other thyroid function tests and calcium levels were within normal range: TSH 0.01 mIU/mL, Free T4 1.0 ng/dL, TG 4.6 ng/mL, Thyroglobulin antibodies 1 IU/mL (less than or equal to 1), PTH intact 43 pg/mL (16-77), and Calcium 8.9 mg/dL (8.6-10.2). Conclusion: This case represents a complex and unusual medical scenario where a patient transitioned from hypothyroidism to hyperthyroidism, ultimately leading to the diagnosis of MLS in conjunction with PTC. The exact mechanism behind this transition is not fully understood. In the management of hypothyroidism, adjustments in thyroxine dosage are common, but significant tapering of the dose should prompt further investigation. The presence of PTC in AFTNs is considered rare, as AFTNs are typically characterized as hot nodules, implying they are hyperfunctioning and generally considered to be less likely malignant compared to cold nodules. This case underscores the need for comprehensive evaluation and follow-up in patients with thyroid disorders, especially when there is a change in thyroid function or the presence of nodules. Presentation: 6/3/2024

8042 A Rare Case of Papillary Thyroid Carcinoma in a Patient with Li-Fraumeni Syndrome

Abstract Disclosure: K.M. Bakhtawar: None. G. Jaiswal: None. Li-Fraumeni syndrome (LFS) is an autosomal dominant syndrome with a high lifetime probability of cancer at an early age. The spectrum of cancers primarily includes osteosarcoma, soft tissue sarcoma, breast cancer, brain tumors, leukemia, and Adenoid cystic carcinoma. Prior studies have estimated the cumulative cancer risk to be about 50% by age 40 and as high as 90% by age 60. As screening for TP53 mutations has improved and more patients are identified, the LFS cancer spectrum has expanded to include thyroid cancer. However, the occurrence of thyroid cancer in LFS has not been extensively assessed in the literature. A recent study examined cancer patients carrying the Brazilian p.R337H TP53 mutation which demonstrated that out of 101 patients, 11 had thyroid cancer (10.9%). In our case, a 45-year-old female with a past medical history of Li-Fraumeni syndrome, Von Willebrand disease, Pulmonary embolism, HER-2 positive invasive ductal carcinoma of the left breast status post chemotherapy, and bilateral mastectomy presented for evaluation of papillary thyroid carcinoma (PTC). An enlarged thyroid was initially identified on physical exam by the PCP with a follow-up thyroid ultrasound showing bilateral nodules. The right upper pole 1.9 x 1.3 x 1.6 cm nodule was biopsied revealing PCT. Following neck ultrasound demonstrated mildly enlarged lymph nodes on the right side, level 2A 1.5 cm and level 3 1.6 cm. The patient subsequently underwent total thyroidectomy and lymph node dissection. The pathology report revealed a 1.4 cm PTC in the upper right pole, a 0.9 cm PCT in the isthmus, and a 0.8 cm PTC in the left midpole. 4/62 lymph nodes were positive with the largest deposit of 1 cm. All margins were negative for carcinoma with no angioinvasion or lymphatic invasion. She was staged as T1bN1bM0. We are awaiting post-operative Thyroglobulin level to assess the need for Radioactive iodine. BRAFV600E and P53 mutation was identified. The patient carries known heterozygous mutation p.R1755H in the TP53 gene with an estimated lifetime cancer risk of 93%. This particular mutation has not been associated with thyroid cancer in prior studies. Although thyroid cancer is an uncommon manifestation of LFS, this case begs the importance of further studies to explore this association which may then lead to early screening and treatment of thyroid cancer in individuals with founder TP53 mutations. Presentation: 6/1/2024

Surgical approach selection and prognosis analysis of papillary thyroid microcarcinoma in the isthmus

Objective:To compare the prognosis of papillary thyroid microcarcinoma of the isthmus（PTMCI） after different surgical methods, and to investigate the most appropriate surgical plan for it, so as to provide reference for the selection of clinical surgical plan. Methods:The clinical data of 106 PTMCI patients diagnosed with postoperative pathology after surgical treatment in Department of Otolaryngology Head and Neck Surgery, Ningbo Medical Center Lihuili Hospital from January 2016 to June 2023 were retrospectively analyzed. The patients were divided into 3 groups according to whether there were nodules in the lateral lobe of the thyroid gland, namely, isolated PTMCI group, PTMCI group with unilateral lobe nodules, and PTMCI group with bilateral lobe nodules. Combined with follow-up information, the differences of recurrence rate, survival rate and postoperative complications after different surgical methods were compared among all groups. The surgical procedures included isthmic thyroidectomy, isthmic + unilateral lobectomy, and total thyroidectomy. All patients underwent central lymph node dissection at the same time. Results:There were no significant difference in recurrence rate, survival rate and postoperative complications among all groups and between groups. Postoperative recurrence occurred in 2 patients, among which 1 patient was PTMCI with multiple focal tumors in unilateral lobe undergoing isthmus + unilateral lobectomy + ipsilateral central lymph node dissection. The recurrence was manifested as contralateral cervical lymph node metastasis. Another case of PTMCI with bilateral benign nodules underwent isthmus + unilateral lobe（larger nodule） resection + ipsilateral central lymph node dissection, and the recurrence was manifested as residual glandular recurrence. One patient developed permanent hoarseness after surgery. The postoperative pathology of 31 patients（29.2%） indicated multiple focal thyroid carcinoma. Postoperative pathology of 41 patients（38.7%） suggested lymph node metastasis in the central region of neck. The disease-specific survival rate was 100%. Conclusion:Isthmic thyroidectomy is recommended for isolated PTMCI. Isthmus of thyroid+ unilateral lobectomy is feasible for PTMCI with unilateral lobectomy. If multiple suspicious malignant nodules（≥3） occur in unilateral lobectomy, total thyroidectomy is recommended. Total thyroidectomy is feasible for PTMCI with bilateral nodules. All PTMCI patients should undergo prophylactic central cervical lymph node dissection at the same time.

Vocal dysfunction following thyroid surgery: a multidimensional subjective and objective study

Background and objectiveFollowing thyroid surgery, vocal changes are a common complication and well-known morbidity that may be linked to neuronal and non-neuronal voice breakdown. Nevertheless, their effects on different voice characteristics are not fully understood, and their bases are still poorly characterized. In order to determine the diagnostic indicators that address the nature of such post-thyroidectomy voice alternations, this study was designed to provide a multidimensional assessment of vocal function after thyroid surgery.MethodsThis research was a 1-year prospective cohort study conducted on 100 adult patients aged 40.19 (± 12.82) years who were recruited from the outpatient clinic of Phoniatric Unit, Assiut University Hospital, and scheduled to undergo thyroid surgery during the period from November 2020 to November 2021. All subjects underwent vocal assessment preoperatively and 15 days, 1 month, and 2 months postoperatively by filled in subjective evaluation of voice complaints via voice handicap index (VHI-30), auditory perceptual assessment (APA) of the voice, and videolaryngoscopy in addition to acoustic analysis using computerized speech lab (CSL). Statistical analysis was performed to compare multi-parameter voice assessment tools across different assessment time points.ResultsThe voice changes were significantly decreased from 51.0% after 15 days postoperatively to 33.0% after 2 months of follow-up. Among these cases, 35.0% cases developed vocal fold paralysis and complained of a breathy voice (27% developed unilateral vocal fold paralysis, and 8% developed bilateral focal fold lesions), and the remaining 16.0% cases had no paralytic manifestations. Also, only one case developed gross lesion “bilateral vocal fold nodules.” The subjective evaluation of voice outcome after thyroidectomy showed significant improvement in VHI subscales and total score from 15 days postoperatively to 2 months of follow-up (P < 0.001). All of the acoustic parameters except HNR showed a significant difference across the different assessment settings (P < 0.001).ConclusionThyroidectomy can result in significant vocal alterations, even in cases where the laryngeal nerve is unharmed. These changes should be taken into consideration in patient having thyroid surgery, especially a total thyroidectomy because of malignant lesions. More efforts are needed in order to determine the extent and pathophysiological reasons for the vocal alterations following thyroid surgery in order to reduce the morbidity associated with one of the most popular surgical procedures performed globally.

Bilateral Internal Auditory Canal Metastasis From Lung Cancer on FDG PET/CT.

The occurrence of metastatic tumor in the internal auditory canal is very rare. Thus, we report FDG PET/CT findings of bilateral Internal auditory canal metastasis from lung cancer in a 67-year-old man. FDG PET/CT and MRI showed nodules in the bilateral internal auditory canal with an SUVmax of 5.2. This case hints us that bilateral metastasis should be considered as differential diagnosis when we encounter bilateral nodule in the internal auditory.

Immunoglobulin G subclass 4-related disease of the lacrimal sac presenting as bilateral lacrimal sac nodules: A rare case report

Immunoglobulin G subclass 4-related disease of the lacrimal sac presenting as bilateral lacrimal sac nodules: A rare case report

Trochlear nerve schwannoma with concomitant osimertinib-responsive stage IV lung adenocarcinoma: illustrative case.

The prognosis for cancer patients has been improved because of the development of molecularly targeted drugs. Treatment of intracranial tumors must be personalized while prioritizing the treatment of comorbid cancers. A 38-year-old man presented with bloody sputum, bilateral multiple nodules, and a mass in the lower lobe of his right lung. Bronchoscopy revealed stage IV lung adenocarcinoma with an epidermal growth factor receptor (EGFR) mutation. Screening head magnetic resonance imaging revealed a 38-mm-diameter mass in the left petroclival area. Because the patient was neurologically intact, the treatment of lung adenocarcinoma was prioritized, and the third-generation EGFR-tyrosine kinase inhibitor osimertinib was used. Although nodules in the lung began to shrink, the intracranial lesion expanded and caused hydrocephalus, necessitating a ventriculoperitoneal shunt. The tumor also caused diplopia, dysarthria, and gait abnormalities. A left anterior transpetrosal approach was used to remove the tumor derived from the trochlear nerve. The pathological examination revealed schwannoma. Neurological symptoms improved following surgery. Osimertinib was continued during the perioperative period. Osimertinib was effective for lung adenocarcinoma but not for trochlear nerve schwannoma, which required surgical intervention. It is necessary to tailor the treatment of benign brain tumors in patients with concurrent malignant cancers. https://thejns.org/doi/10.3171/CASE24144.

Obstructive Bilateral Renal Fungal Bezoars in an Extremely Premature Neonate Treated With Antifungals and Urokinase Irrigation: A Case Report and Review of the Literature.

An ex-27-week gestation female infant developed bilateral forearm nodules at 4 weeks of life during treatment for methicillin-sensitive Staphylococcus aureus bacteremia. A pure growth of Candida albicans was isolated on culture of both sterile aspiration of the forearm abscess and urine without evidence of methicillin-sensitive Staphylococcus aureus. The patient went on to develop bilateral obstructive renal fungal bezoars at 11 weeks of life. Bilateral nephrostomies were required to alleviate obstruction with the addition of local irrigation with amphotericin B deoxycholate. Two weeks later, urokinase via the nephrostomy tubes was added due to an unchanged appearance on ultrasound (US) and ongoing candiduria. A significant reduction in the size of bezoars was seen on US after 3 days. Sterilization of urine culture was achieved 7 weeks into treatment, and resolution of bezoars on US was seen 9 weeks after treatment began. No adverse events occurred from the use of local urokinase. Urokinase irrigation via nephrostomy is an effective and safe adjunctive treatment in refractory obstructive renal candidiasis in neonates.