Bilateral Nodules Research Articles

ADENOCARCINOMA OF THE LUNG WITH A MILIARY PATTERN

SESSION TITLE: Medical Student/Resident Lung Cancer Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: A miliary pattern on chest imaging is often concerning for primary tuberculosis. However, such nodules may also be seen in fungal infections and autoimmune or (rarely) metastatic processes. Here, we report a case of a miliary mottling on chest imaging associated with metastatic lung adenocarcinoma. CASE PRESENTATION: A 39-year-old man with a history of obesity and tobacco use presented with worsening chest congestion and dyspnea on exertion for 2 months. He had occasional wheezing without associated symptoms, including fever, chills or night sweats, or weight loss. He worked in environmental services without any known sick contacts or recent travel history. Physical examination was unremarkable except a small, palpable right supraclavicular lymph node. The initial chest x-ray showed diffuse interstitial infiltrates. Chest CT revealed innumerable bilateral cavitary nodules with an apical gradient, a right lower lobe 6-cm mass with cavitation, and mediastinal and supraclavicular lymphadenopathy. Given concern of pulmonary tuberculosis with a miliary component, the patient remained on airborne precautions. Laboratory workup was notable for a mild leukocytosis of 12,000. Sputum cultures and AFB staining were negative. A baseline connective tissue panel, AFP, and beta-hCG levels were unmeasurable. Given his age and concern for metastatic disease, a scrotal ultrasound was performed but did not reveal any testicular mass. Subsequently, the patient underwent a fine-needle aspiration and core biopsy of the right supraclavicular lymph node, which showed metastatic adenocarcinoma. The immunohistochemical profile was suggestive of a positive CK7 but negative for CK20, TTF-1, CDX2, and Napsin A---support the diagnosis of non-small cell lung cancer with a KRAS Q61K mutation. Immuno-chemotherapy with a combination of carboplatin, pemetrexed, and pembrolizumab was initiated, given his stage IV metastatic lung adenocarcinoma. DISCUSSION: The differential diagnosis for miliary patterns on chest imaging includes tuberculosis, fungal infections, sarcoidosis, pneumoconiosis, and secondary metastasis. Miliary-pattern metastatic lung adenocarcinomas have been rarely reported. Our case is of interest given the negative TTF-1 with a KRAS Q61K mutation, the later of which is associated with resistance to the EGFR inhibitors gefitinib and erlotinib. This entity is thought to have a poorer prognosis than KRAS wild-type adenocarcinomas. CONCLUSIONS: The initial radiologic presentation of these cases can be misleading in areas with high prevalence of infections such as tuberculosis and fungal infections. The possibility of primary lung cancer should also be considered in these cases. Reference #1: A. Sekine et al., “Miliary lung metastases from non‑small cell lung cancer with Exon 20 insertion: A dismal prognostic entity: A case report,” Mol. Clin. Oncol., Oct. 2018, doi: 10.3892/mco.2018.1730. Reference #2: L. Kimmig and J. Bueno, “Miliary Nodules: Not Always Tuberculosis,” Ann. Am. Thorac. Soc., vol. 14, no. 12, pp. 1858–1860, Dec. 2017, doi: 10.1513/AnnalsATS.201706-436CC. Reference #3: P. J. Roberts, T. E. Stinchcombe, C. J. Der, and M. A. Socinski, “Personalized medicine in non-small-cell lung cancer: is KRAS a useful marker in selecting patients for epidermal growth factor receptor-targeted therapy?,” J. Clin. Oncol., vol. 28, no. 31, pp. 4769–77, Nov. 2010, doi: 10.1200/JCO.2009.27.4365. DISCLOSURES: No relevant relationships by Kamran Manzoor, source=Web Response No relevant relationships by Eric Robbins, source=Web Response No relevant relationships by Ajit Thota, source=Web Response

WHEN LIGHTNING STRIKES TWICE: A CROSSOVER SYNDROME WITH LIGHT CHAIN DEPOSITION AND LYMPHOID INTERSTITIAL PNEUMONIA

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Light chain deposition disease (LCDD) is a rare cystic lung disease, involving monoclonal proliferation and deposition of light chains. Lymphoid Interstitial Pneumonia (LIP) is an uncommon interstitial lung disease (ILD) usually seen with connective tissue disorders (CTD). We present a challenging case involving the co-existence of LCDD and LIP. CASE PRESENTATION: We present the 25-year long clinical course of a 47-year-old male who was initially diagnosed with a mucosa-associated lymphoid tissue (MALT) lymphoma of the parotid gland, treated with excision. Over the subsequent 5-10 years, he was diagnosed with rheumatoid arthritis and Sjogren’s syndrome. Treatment with methotrexate was complicated by the development of human anti-murine antibodies. He also developed progressive cough and dyspnea. Computed Tomography (CT) imaging of the chest revealed bilateral nodules and interstitial infiltrate, suggestive of LIP. Right lower lobe wedge resection was performed; histopathology demonstrated lymphoplasmacytic infiltrate and eosinophilic proteinaceous deposits which were negative on Congo red staining. Immunohistochemistry revealed predominantly kappa light chains within the eosinophilic material. Serial follow-up imaging was notable for the development of diffuse cystic lung disease. He was diagnosed with LCDD and was treated with bortezomib, but developed Steven-Johnson syndrome. This was followed by treatment with lenalidomide and then cyclophosphamide. A follow up positron emission tomography (PET) CT scan showed no change in the size of pulmonary nodules. The patient was also trialed on bendamustine and ofatumumab, an anti-CD20 monoclonal antibody, with no significant improvement. A recent chest CT, obtained 15 years following the onset of lung disease, demonstrated wide-spread thin walled cysts and nodular disease (Fig 1,2). His symptoms have stabilized with oxygen supplementation and treatment of exacerbations with steroids and antibiotics, and he is currently on surveillance. DISCUSSION: LCDD, an entity caused by plasma cell proliferation and monoclonal light chain deposits in various organs often found in association with lymphoproliferative conditions, can manifest as diffuse cystic lung disease. (1) It has a poor prognosis. Treatment options include immunomodulators and chemotherapeutic agents, including bortezomib, daratumumab, and bendamustine. LIP, an ILD, is usually seen in patients with CTD and HIV. (2) Therapeutic options include immunosuppressants such as rituximab and cyclophosphamide. Our case was challenging, given the confluence of two syndromes- LIP with underlying rheumatic conditions, and LCDD due to a lymphoproliferative disorder. Multiple avenues of treatment were attempted but failed to produce an objective improvement in disease course. CONCLUSIONS: LIP and LCDD can occur in tandem; enrollment in study trials should be considered for these patients given poor prognosis. Reference #1: Arrossi AV, Merzianu M, Farver C, et al. Nodular pulmonary light chain deposition disease: an entity associated with Sjögren syndrome or marginal zone lymphoma. J Clin Pathol. 2016 Jun;69(6):490-6. Reference #2: Swigris JJ, Berry GJ, Raffin TA, et al. Lymphoid interstitial pneumonia: a narrative review. Chest. 2002 Dec;122(6):2150-64. DISCLOSURES: No relevant relationships by Pius Ochieng, source=Web Response No relevant relationships by NISHANT SHARMA, source=Web Response No relevant relationships by Pandi Todhe, source=Web Response

Asymptomatic plantar nodules in a 1-year-old healthy girl

A healthy 1-year-old girl presented to our dermatology clinic with bilateral asymptomatic planter nodules that were noticed by her parents at 1 month of age. There was no family history of similar lesions. On examination, there were bilateral, soft, skin-colored, nontender, mobile nodules over the medial plantar surface of both feet measuring around 1.5 × 0.5 cm (Fig 1). Ultrasound examination of the plantar surfaces found increased subcutaneous focal fatty deposition at the site of skin nodules.

Importance of The Medical History in The Diagnosis of Hypersensitivity Pneumonitis: A Case Report

Introduction: Hypersensitivity pneumonitis, most frequent types are those related to farming and bird breeding. In this paper, a patient with occupational exposure to metal dusts and welding fumes but diagnosed with HP thanks to the guidance of detailed anamnesis revealing inhalational exposure to pigeon antigens both at home and in workplace Case: 32-year-old male. He applied with shortness of breath, dry cough. He has been working production of kitchen equipment made from stainless steel. When environmental exposures questioned, he declared that he bred up to ten pigeons at the basement of his house and up to fifteen pigeons at a coop next to workplace building. Spirometry revealed restrictive pattern. In patient’s chest X-ray, bilateral increased reticular densities were observed. Computed tomography of thorax revealed radiological findings supporting subacute hypersensitivity pneumonitis such as bilateral diffuse millimetric centrilobular ground-glass nodules which are coalescing in some areas, mosaic perfusion and air trapping in basal areas. Bronchoalveolar lavage cytology revealed percentages of lymphocytes as 85%. Discussion: Detailed anamnesis revealing environmental and occupational inhalational exposures guides the physician in diagnosis of HP. After initial suspicion, supporting laboratory and radiologic findings confirm the diagnosis to provide appropriate management.

Twenty Eight Days in the Life of a Vocal Pathology: A Case Study of Videolaryngostroboscopy, Acoustic, and Perceptual Variability

The purpose of this investigation was to observe laryngeal tissue and vocal function changes over the course of 28 days in a single participant diagnosed by a laryngologist with bilateral nodules. Laryngeal imaging, acoustic variables and perceptual assessments of voice quality, and perceived vocal effort were obtained every morning for 28 consecutive days. A daily journal of occupational and recreational voice use as well as menstruation and alcohol consumption was maintained each day. It was hypothesized that the laryngeal pathology would appear more extensive and the vocal function measures obtained would be worse following extensive voice use. Laryngeal imaging, acoustic variables, and perceptual measures quantified provided evidence to support the study hypotheses. The size, extent, and asymmetry of the bilateral vocal pathologies observed were more extensive on days following occupational and recreational vocal loading. Acoustic and perceptual measures obtained correlated with the laryngeal tissue changes observed. Study findings support a more holistic approach to laryngeal pathology diagnosis that is based on a more thorough understanding of vocal loading considerations up to 48 hours prior to laryngeal endoscopy to better understand the pathophysiology of the observed lesion(s) for most accurate clinical diagnosis.

Primary lung neoplasms presenting as multiple synchronous lung nodules.

Multiple synchronous lung nodules are frequently encountered on computed tomography (CT) scanning of the chest and are most commonly either non-neoplastic or metastases from a known primary malignancy. The finding may initiate a search for primary malignancy elsewhere in the body. An exception to this rule, however, is a class of rare primary lung neoplasms that originate from epithelial (pneumocytes and neuroendocrine), mesenchymal (vascular and meningothelial) and lymphoid tissues of the lung. While these rare neoplasms also present as multiple synchronous unilateral or bilateral lung nodules on chest CT, they are often overlooked in favour of more common causes of multiple lung nodules. The correct diagnosis may be suggested by a multidisciplinary team and established on biopsy, performed either as part of routine diagnostic work-up or staging for malignancy. In this review, we discuss clinical presentations, imaging features, pathology findings and subsequent management of these rare primary neoplasms of the lung.

Co- infectious Cytomegalovirus and Pneumocystis Jiroveci Pneumonia in a Polyarteritis Nodosa Patient: A Case Report

Our report discusses a patient diagnosed with PAN since 3 years ago. He presented with fever, chills and nonproductive cough. He was a long time receiver of immunosuppressant drugs for his underlying condition. Upon examination he was febrile, had cushingoid appearance and cackles in both lungs. Lung CT scan showed opacities in right upper lobe lung and multiple bilateral nodules and ground glass opacity along with mild thickening of pleura. A bronchoscopy was ordered to asses PCP, and without hesitation empirical therapy was started. However, his clinical condition did not improve as expected. At this time, suspecting another infection at play, a PCR and BAL specimen was ordered for CMV. After receiving the result of BAL analysis, our suspicion was confirmed for both PCP and CMV pneumonia. CMV is an important opportunistic infection in immunocompromised individuals. This case highlights this importance in immunocompromising conditions. In this setting, presence of respiratory signs and symptoms point out to PCP as the first differential diagnosis; but at the same time it’s crucial for clinicians to consider the possibility of CMV as a co-infective agent.

Desmoplastic Small Round Cell Tumor with an Unusual Site and Age Presentation: A Case Report and Literature Review

Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive sarcoma usually arising in the abdomen or pelvis of young males under 30 years of age. We present a case of a 32-year-old male with a palpable axillary mass and multiple bilateral lung nodules. Excisional biopsy of the axillary mass showed sheets and nests of small round cells with numerous mitoses, areas of necrosis, and desmoplastic stroma. Initial immunohistochemical stains performed at an outside institution demonstrated immunoreactivity for epithelial markers and focal reactivity for breast markers. The tumor was initially diagnosed as poorly differentiated carcinoma, with consideration of a possible primary breast cancer. Additional workup demonstrated strong, diffuse positivity for desmin in tumor cells, leading to the final diagnosis of DSRCT. Subsequent molecular testing confirmed DSRCT with EWSR1 gene rearrangement. This case illustrates the importance of recognizing the morphologic features of DSRCT in the setting of uncommon location or patient age.

Evaluation of Adrenal Vein Sampling Use and Outcomes in Patients With Primary Aldosteronism

BackgroundPrimary aldosteronism (PA) occurs in 10%-20% of patients with resistant hypertension. Guidelines recommend adrenal vein sampling (AVS) to identify patients for surgical management. We evaluate the use of AVS in managing PA to better understand the selection and outcomes of medical versus surgical treatment. MethodsA retrospective review was performed, and patients were divided into those who did (AVS) and did not have AVS (non-AVS). Demographics, aldosterone and renin levels, blood pressure, comorbidities, and antihypertensive medications were recorded. Reasons to defer AVS and medical versus surgical decision-making were examined and groups were compared. ResultsWe included 113 patients; 39.8% (45/113) had AVS, whereas 60.2% (68/113) did not. Groups were similar in age, body mass index, and initial systolic blood pressure (SBP).In patients who underwent AVS, 31 of 45 (68.9%) had unilateral secretion and were referred for surgery, whereas 13 of 45 (28.9%) had bilateral secretion. Of the 31 referred for surgery, 26 underwent laparoscopic adrenalectomy, all cured; four refused surgery; and one counseled toward medical management by their physician.In 68 non-AVS patients, 6 (8.8%) underwent adrenalectomy without sampling and 2 with no clinical improvement. The remaining deferrals were because of normal or bilateral adrenal nodules on imaging (8/68, 11.8%); medical management due to poor surgical candidacy (12/68, 17.6%); patient refusal of intervention (13/68, 19.1%); or reasons not stated (28/68, 41.1%). At the follow-up, patients who underwent AVS had lower median SBP (135.4 mmHg versus 144.7 mmHg, P = 0.0241) and shorter follow-up (17.7 mo versus 54.0 mo, P < 0.0001). Surgically managed patients had biochemical resolution of PA with normalization of potassium levels (3.6 to 4.7mEq/L, P < 0.00001). ConclusionsAVS correctly selects patients for surgical management avoiding unnecessary surgery. However, despite guidelines, AVS is not always pursued as part of PA treatment, potentially excluding surgical candidates.

Bilateral lung nodules resection by image-guided video-assisted thoracoscopic surgery: a case series

BackgroundWe demonstrated the safety and feasibility of image-guided video-assisted thoracoscopic surgery (iVATS) of bilateral lung lesions in a hybrid operating room.MethodsThis study was a retrospective analysis of a case series. A total of 7 patients with 15 small lung nodules underwent bilateral iVATS between July 2018 and May 2019. All procedures were completed within a single anesthesia procedure and performed in a hybrid operating room that had a cone-beam computed tomography (CT) apparatus equipped with a laser navigation system. The lesion characteristics, operation methods, and peri-operative clinical outcomes were summarized.ResultsA total of 7 patients with 15 resected lung nodules were analyzed. The most common pathological result of our bilateral iVATS was metastasis. The median length of hospital stay was 5 days (range from 3 to 10 days). The median right chest tube duration was 2 days (range from 1 to 8 days), and the median left chest tube duration was 3 days (range from 2 to 5 days). Only one patient had a complication during his hospitalization period. There was no surgery-related mortality observed.ConclusionsFor bilateral pulmonary nodules, the iVATS procedure seems to be a feasible and cost-effective approach.

A New MEN2 Syndrome with Clinical Features of Both MEN2A and MEN2B Associated with a New RET Germline Deletion

Background Multiple endocrine neoplasia type 2 (MEN2) is a hereditary cancer syndrome caused by RET proto-oncogene mutation. Two different clinical variants of MEN2 are known (MEN2A and MEN2B): medullary thyroid carcinoma (MTC) almost always present and associated with pheochromocytoma (Pheo), and primary hyperparathyroidism (HPTH) in MEN2A and with Pheo and other nonendocrine diseases in MEN2B. Case Report. A 7-year-old girl, previously treated for a pelvic plexiform neurofibroma, arrived at our observation with a peculiar MEN2B syndrome and with HPTH. The neck ultrasound showed bilateral thyroid nodules, local lymph node lesions, and a suspicious left hyperplastic parathyroid. The CT scan showed a megacolon and described the persistence of the pelvic tumor. A new RET germline deletion in exon 11 (c.1892_1899delCGAGCT; p.Glu632_Leu633del) was found. She underwent total thyroidectomy, central compartment and latero-cervical lymph node dissection, and neck exploration for primary HPTH. The histology confirmed bilateral MTC, multiple lymph node metastases, a hyperplastic parathyroid, and a parathyroid adenoma. Conclusions This is the first case of a complex syndrome characterized by peculiar features of MEN2B, without Pheo but with a pelvic plexiform neurofibroma and with HPTH, which is typical of MEN2A. A “de novo” new germline RET deletion located in exon 11 was found.

Ultrasonography and laryngeoscopic findings are similar in detecting laryngeal lesions

Aims: There is a growing need for alternative maneuvers for diagnosing laryngeal diseases. The objective of this study is to evaluate laryngeal ultrasound as an alternative to laryngeal endoscopy in diagnosis and assessment of various laryngeal lesions.Materials and Methods: This prospective study was conducted at the E.N.T. outpatient clinic of Minia University Hospital. The study included 50 patients (28 males and 22 females) with laryngeal lesions. Patients were examined by direct laryngoscopy and then were examined by using high resolution ultrasonography.Results: No significant differences between direct laryngoscopy (DL) and ultrasonography diagnosis results. By DL, bilateral vocal fold nodule was diagnosed in 14 cases. However, ultrasound diagnosed only 11 cases of them. 16 cases were diagnosed with left or right vocal fold polyp and the ultrasound diagnosed 15 cases of them. 10 patients were diagnosed as glottal mass and the ultrasound diagnosed only 8 cases of them. For vocal fold cyst, four cases were diagnosed by both DL and ultrasound. Also, for Reinke's edema, four cases were diagnosed by both DL and ultrasound. One case was diagnosed as Laryngeocele by both maneuvers. One case was diagnosed as Laryngeoscleroma by DL while it was not diagnosed by ultrasound.Conclusion: Ultrasonography could be used effectively as an alternative technique to direct endoscopy in diagnosis of different laryngeal lesions.

Clinicopathological features of pulmonary epithelioid hemangioendothelioma: a study of 18 cases

Objective: To investigate the clinicopathological features of pulmonary epithelioid hemangioendothelioma (PEHE). Methods: Eighteen cases of PEHE were collected from August 2011 to December 2018 at the First Affiliated Hospital of Zhengzhou University. All cases were retrospectively studied by hematoxylin and eosin staining and immunohistochemistry (IHC). The clinicopathological features were reviewed; the status of CAMTA1 and TFE3 gene was analyzed and patients' outcome was followed up. Results: Of the 18 cases, there were 11 males and 7 females with a male to female ratio of 1.6 to 1.0. The patients' age ranged from 36 to 68 years (mean 52 years). Twelve cases (12/18) showed a single nodule and six cases (6/18) showed multiple bilateral nodules. Seven cases (7/18) involved other organs besides lung. Seventeen (17/18) patients presented with respiratory symptoms and one patient (1/18) presented with abdominal pain. Grossly, the tumors were greyish-white nodules with indistinct borders. Microscopically the tumor cells were epithelioid and arranged in strands and nests, and cytoplasmic vacuoles were commonly noted. The stroma was myxochondroid or hyaline. By IHC, the tumor cells were positive for CD31(18/18), CD34 (16/18), ERG (18/18) and Fli-1 (18/18); CKpan was focally positive in 5 cases (5/18). TFE3 was positive in 3 cases (3/18), and Ki-67 index ranged from 5% to 30%. FISH analysis showed seventeen cases (17/18) had CAMAT1 rearrangement, one case had TFE3 rearrangement displaying a split signal. Eight patients (8/18) had surgical excision, three patients (3/18) had surgery and chemotherapy, and seven patients (7/18) had chemotherapy only. Four patients (4/18) died of the disease. Conclusions: Patients with PEHE have non-specific symptoms, and correct diagnosis depends on pathologic biopsy and the exclusion of other tumors with epithelioid morphology. Some patients with PEHE have poor prognosis, particularly in those who have multiple nodules, peripheral invasion or metastasis.

Auricular Multiple Tophi Presenting as Painful Bilateral Nodules: A Case Report and Literature Review

Gout tophus is most commonly found in the lower extremities such as toes and ankles. The auricle is a rare site for gout tophi. Although auricular tophus (AT) is not life threatening, it is important because it can be misdiag-nosed as other diseases including malignancy. ATs are firm deposits of crystallized uric acid, which may gradually grow at the subcutaneous layer of the auricle. ATs can be treated with dietary modification and medication for a long time. When pain persists, cosmetic deformity is concerned, or prompt resolution of those symptoms are required, surgical excision is worth considering. Here, we report the case of a patient presenting with multiple bilateral auricular painful nodules. Thorough questioning regarding medical history revealed that the patient had a history of untreated gout. Histopathological examination confirmed the diagnosis of AT. So, we report this case with review of literatures. (J Clinical Otolaryngol 2020;31:57-62)

An Unusual Variant of Lemierre’s Syndrome with Multiple Abscesses and Hepatic Vein Thrombosis

Introduction: Lemierre’s syndrome is an uncommon, life-threatening disease that develops as a severe complication of oropharyngeal infection. However, this syndrome has also been reported during anaerobic septicemia, originating from diverse sources of infection, such as the gastrointestinal system. The diagnosis of this syndrome remains challenging, and is often prompted by the detection of Fusobacterium necrophorum in blood culture, rather than with clinical signs or symptoms. Case Presentation: A previously healthy 38-year-old woman was hospitalized for a 6-day history of fever, watery diarrhea, and vomiting. Physical examination of the patient showed fever, pharyngitis, and diffuse abdominal tenderness. Laboratory investigations revealed elevated inflammatory markers, and two blood cultures revealed Fusobacterium necrophorum. An abdominal ultrasound showed a liver abscess and hepatic vein thrombosis. The thoracoabdominal and cerebral computed tomography scan confirmed the results of the abdominal ultrasound. It additionally revealed bilateral lung nodules and a parietal brain abscess. The patient’s general condition and her laboratory test results, improved after receiving treatment with ceftriaxone and metronidazole. She received antibiotics for three months, without anticoagulation therapy. Repeated computed tomography scans showed a resolution of the liver, pulmonary, and brain abscesses, as well as the recanalization of the hepatic vein. Conclusions: Lemierre’s syndrome emerges as a result of septic illness and liver abscesses in case of gastrointestinal symptoms despite the lack of common presentation.

SAT-231 A Case of Metastatic Pheochromocytoma Associated with Beckwith-Wiedemann Syndrome

Introduction:Beckwith-Wiedemann Syndrome (BWS) is an autosomal dominant disorder of chromosome 11p15 that results in increased IGF-2 and CDK1NC. This leads to excessive cell proliferation and tumor formation. The following highlights a case of metastatic pheochromocytoma in a patient with BWS.Clinical Case:A 30-year-old male presented with sudden onset blurry vision without any associated complaints. His past medical history was significant for BWS. His family history was negative for uncontrolled hypertension, sudden death, thyroid cancer or hyperparathyroidism. Physical examination was notable for an elevated systolic blood pressure of 200/160 mm of hg and fundoscopy revealed features of hypertensive emergency. Laboratory investigations revealed an elevated plasma normetanephrine [10445 pg/ml (normal: <148)], metanephrine [93 pg/ml (normal: <57)], total metanephrine [10538 pg/ml (normal: <205)], epinephrine [134 pg/ml (normal:<50)], norepinephrine [23526 pg/ml (normal: 112-658), total catecholamine level [23660 (normal: 123-671pg/ml)] and dopamine [403 pg/ml (normal<30)] levels. His PTH, corrected serum calcium, gastrin, insulin, carcinoembryonic antigen, calcitonin levels and basal metabolic panel were all normal. MRI of the abdomen demonstrated bilateral adrenal nodules with a large mass encasing the celiac axis along with evidence of hepatic lesions. I-123 MIBG scan showed mild radioactive tracer uptake in the adrenal nodules and mass near the celiac axis but not in the hepatic lesions. PET scan confirmed MRI findings and was negative for any evidence of malignancy in the chest, pelvis and skeleton. MRI of the brain was negative for metastasis as well as pituitary abnormalities. Ultrasound-guided liver biopsy was positive for malignant cells that stained positive for chromogranin and synaptophysin confirming the diagnosis of metastatic pheochromocytoma. He was treated with phenoxybenzamine, diltiazem and lisinopril. He underwent cycles of cyclophosphamide, vincristine and dacarbazine. Genetic testing revealed a variant in SDHD gene which was of uncertain significance. Repeat biochemical testing on follow up after a year and a half showed a decreased plasma normetanephrine [487pg/ml] and metanephrine levels [110 pg/ml] in comparison to his levels on presentation. Repeat imaging revealed a decrease in tumor burden including bilateral adrenal nodules, celiac axis mass and hepatic metastases.Conclusion:This is an unusual case of malignant pheochromocytoma in the absence of SDHB mutation in a patient with BWS. Genetic causes in these patients are yet to be determined. However, genes H19 and KCNQ1OT1 have been implicated in addition to IGF-2 and CDK1NC

MON-451 An Unusual Case of Follicular Thyroid Cancer with Pulmonary Metastasis and Concurrent Hyperthyroidism

Introduction: Follicular Thyroid Cancer (FTC) accounts for approximately 12 % of all thyroid cancers and is the second most common cause of differentiated thyroid cancer. However, coexisting hyperthyroidism and thyroid malignancy is considered a rare event and is thought to have a prevalence of 0.21–9.0 %. We describe a compelling case of metastatic FTC initially presenting with subclinical hyperthyroidism progressing to overt hyperthyroidism.Clinical Case: An 88-year-old man with a history of prostate cancer, previous tobacco dependence, and hypertension initially presented with complaints of a chronic non-productive cough associated with unintentional weight loss and fatigue. Initial imaging included a chest CT that showed several bilateral pulmonary nodules suspicious for metastatic disease, as well as an enlarged left-sided thyroid mass, which extended from the inferior pole into the mediastinum. FDG PET/CT showed avid disease identified in the lung. CT-guided lung biopsy was performed, and histopathology revealed FTC. Thyroid function tests (TFTs) revealed a Tg level of 1427 (0.00–33.70), TgAb <1.0 (< 13.6), TSH 0.05 (0.40–4.50), FT4 1.1 (0.8–1.8) and FT3 3.4 (2.3–4.2). Biochemical testing was consistent with subclinical hyperthyroidism. Neck ultrasound showed bilateral thyroid nodules, the largest located on the left mid-pole measuring 3.4 x 3.2 x 3.7 cm. He underwent a whole-body I-131 diagnostic scan, which showed intense uptake of the radionuclide within the thyroid gland, more evident on the left, but additional metastatic lesions elsewhere in the visualized body were not possible. TFTs were repeated, which showed a TSH of 0.01, FT4 of 2.0, and FT3 of 6.4. He was subsequently started on methimazole daily and eventually became euthyroid. Due to advanced age, significant mediastinal extension, and lung metastasis, the patient was not a candidate for surgical resection and treatment with a targeted tyrosine kinase inhibitor. He ultimately opted for an observational approach.Conclusion: Although metastatic FTC carries a poor prognosis, concurrent hyperthyroidism is thought to be associated with a more aggressive disease course and higher mortality compared to differentiated thyroid cancers in euthyroid patients.Sources: 1. Pazaitou-Panayiotou, K., Perros, P., Boudina, M., Siardos, G., Drimonitis, A., Patakiouta, F., & Vainas, I. (2008). Mortality from thyroid cancer in patients with hyperthyroidism: the Theagenion Cancer Hospital experience, European Journal of Endocrinology, 159(6), 799–803.2. Benjamin Momo Kadia, Christian Akem Dimala, Ndemazie Nkafu Bechem, Desmond Aroke. BMC Res Notes. 2016; 9: 369.3. Parameswaran R, Shulin Hu J, Min En N, Tan WB, Yuan NK. Patterns of metastasis in follicular thyroid carcinoma and the difference between early and delayed presentation. Ann R Coll Surg Engl. 2017;99(2):151–154.

SUN-483 A Retrospective Diagnosis of Malignant Struma Ovarii After Discovery of Pulmonary Metastases

Background: Malignant struma ovarii is a rare ovarian tumor that is histologically identical to differentiated thyroid carcinoma.1 We present a case of a struma ovarii that was recognized as being malignant only after the discovery of pulmonary metastases. Clinical Case: A 29 year old female presented to the hospital with acute right lower abdominal pain, suspicious for ovarian torsion. She underwent urgent right salpingoopherectomy and pathology demonstrated a mature cystic teratoma with benign struma ovarii. Two years later, a CT of the abdomen incidentally revealed bilateral pulmonary nodules. Review of the imaging showed that these pulmonary nodules were also present two years prior, and had since become larger. Video-assisted thoracoscopic surgery was performed and lung biopsy was positive for well-differentiated thyroid carcinoma. The patient then underwent total thyroidectomy which revealed a 0.3 x 0.3 cm infiltrative papillary thyroid cancer, follicular variant, without lymphovascular invasion. Thyroglobulin level decreased from 169 ng/mL pre-operatively to 80 ng/mL post-operatively, but then continued to be variable ranging from 56 to 252 ng/mL (1.6-50 ng/mL). Thyroglobulin antibodies remained negative.Pathology from right ovary was re-reviewed at a second institution and found to be consistent with highly differentiated thyroid carcinoma with characteristic nuclear features of papillary thyroid carcinoma.A diagnostic whole body I-131 scan showed uptake within the thyroid bed, bilateral lung nodules, left distal thigh and right mid thigh. These thigh lesions were not visualized on lower extremity ultrasound. After dosimetry was performed, the patient received radioactive iodine-131 200 mCI. Post-therapy scan six days later demonstrated uptake in the thyroid bed, bilateral lungs and bilateral thighs. About five months later, thyroglobulin level had decreased to 0.4 ng/mL with a suppressed TSH. A repeat CT chest demonstrated that the lung nodules had all decreased in size, largest from 0.5 cm to 0.3 cm. Conclusion: Careful examination of struma ovarii pathology should be performed to evaluate for malignant features since benign appearing histology can present diagnostic difficulty.2 In this case, thyroglobulin level was lower than reported in previous cases; however, sites of metastases were responsive to radioactive iodine therapy indicating well differentiated disease and a favorable prognosis.

SAT-193 Clinical Case of ARMC5 Tumor Syndrome: A Rare Case of Cushing’s Syndrome from Primary Bilateral Macronodular Adrenal Hyperplasia Caused by ARMC5 Mutation with Concomitant Presence of Meningiomas and Primary Hyperparathyroidism

BACKGROUND: Primary Bilateral Macronodular Adrenal Hyperplasia (PBMAH) is a known rare cause of Cushing’s syndrome (CS). A mutation in the armadillo repeat containing 5 (ARMC5) sequence is associated with up to 55% of PBMAH cases. Recent studies have linked ARMC5 mutations to presence of other benign neoplasias suggesting that ARMC5 could be a tumor suppressor gene. Case: 72-year-old female with a history of obesity, HTN, DM2, osteoporosis, multiple meningiomas, and hypercalcemia with recurrent kidney stones was incidentally found to have bilateral adrenal nodules on CT imaging. She had mild cushingoid features with truncal obesity and moon facies. She had multiple low dose dexamethasone suppression tests with AM cortisol levels in 17-21 ug/dL range (<1.8 ug/dL). She had late night salivary cortisols of 0.885ug/dL and 1.935ug/dL (0.022-0.254 ug/dl in PM). The overall clinical picture of obesity, HTN and DM2 in combination with biochemical testing and presence of bilateral adrenal adenomas was suggestive of CS secondary to PBMAH. On her evaluation for recurrent kidney stones she had a PTH of 107.2 pg/mL (15-65 pg/mL), and a calcium of 10.8 mg/dL (8.3-10.5 mg/dL). She was diagnosed with primary hyperparathyroidism. Imaging studies found a 1.1 cm ectopic parathyroid adenoma situated at the aortic pulmonary window. Surgical evaluation was performed and surgery was not offered given the precarious location of the parathyroid tumor. She had a known history of four meningiomas, two of which were resected and two considered uncresectable. PBMAH in presence of all her other medical comorbidities prompted genetic evaluation for the patient. Analysis revealed a heterozygous ARMC5 mutation. Given the familial pattern of inheritance associated with ARMC5 mutations, patient’s daughter also underwent genetic testing. Daughter tested positive for the mutation as well. Patient was offered surgical and medical therapy options for her PBMAH. She is currently being evaluated for an unilateral adrenalectomy. Discussion: The pathophysiology of CS from PBMAH remains poorly understood leading to an insidious delay in diagnosis and treatment. Inactivating ARMC5 mutations of familial origins are known genetic triggers for development of PBMAH. ARMC5 is also a proposed tumor suppressor gene whose proteins are found in endocrine tissues all over body. Mutation of ARMC5 gene potentially can lead to multi-glandular tumor syndromes. Screening PBMAH patients and their family members for ARMC5 mutations may lead to earlier CS diagnosis/treatment times as well as better understanding of the gene’s neoplastic potential.

SAT-229 Bilateral Pheochromocytoma as Incidental Finding in a Patient with Neurofibromatosis Type 1

Background.In contrast to other genetic syndromes associated with Pheochromocytoma and Paraganglioma (PPGL) in which yearly biochemical screening is recommended, in neurofibromatosis due to the low penetrance of these tumors, screening is only recommended when there is clinical suspicion1. Given that signs and symptoms of catecholamine excess are vague and non-specific, clinicians should keep a low threshold for biochemical testing in these patients.Clinical Case.70 year old female with Neurofibromatosis type 1 (NF-1) diagnosed at age 24, hypertension and atrial fibrillation, was referred to our institution after being found incidentally with bilateral adrenal nodules. She had a 2 year history of poorly controlled, labile hypertension. Treatment at the time of presentation included doxazosin, metoprolol, valsartan and hydralazine. She denied other symptoms of catecholamine excess.Further workup revealed elevated 24 hour urine normetanephrine at 2,530 mcg (ULN 500 mcg) and metanephrine at 1,325 mcg (ULN 290 mcg). Serum metanephrines were also elevated with free metanephrine of 1.6 nmol/L (ULN 0.5 nmol/L) and free normetanephrine of 4.1 nmol/L (ULN 0.9 nmol/L). Her biochemical metanephrine profile was consistent with pheochromocytomas in NF-1. MIBG uptake confirmed the presence of bilateral pheochromocytomas. She underwent a complete right adrenalectomy and a left cortical sparing adrenalectomy. Her adrenocortical function remains adequate. Post operatively while her blood pressures were no longer labile, she continued to require anti-hypertensive therapy.Conclusion.Patients who are at risk of developing pheochromocytomas and paragangliomas due to genetic syndromes or known germline mutations need ongoing clinical follow and biochemical testing for timely case detection. While in NF-1 pheochromocytomas are usually unilateral and present during early adulthood, they can present with bilateral disease and later in life2. During surgical treatment, even with unilateral disease, adrenocortical function should be preserved when possible3. Hypertension may persist despite complete surgical resection.