Hilar Mass Research Articles

Splenic Hilar Mass With Pain, Diarrhoea and Sweating: Can It Be Malignant?

Splenic Hilar Mass With Pain, Diarrhoea and Sweating: Can It Be Malignant?

TO DO OR NOT TO DO? THE CASE FOR PDL1 TESTING IN LOCALIZED HEAD AND NECK SQUAMOUS CELL CARCINOMA (HNSCC)

SESSION TITLE: Lung Cancer SESSION TYPE: Fellow Case Reports PRESENTED ON: 10/23/2019 8:45 AM - 9:45 AM INTRODUCTION: HNSCC is the 6th most common cancer with recurrence rate of over 50%. Multiple mechanisms have been identified by which tumor cells evade T-cell directed immunity. Expression of PDL1 is one of these mechanisms (1). The clinical role of PDL1 testing in localized HNSCC has not been defined in the literature given rising interest in use of immunomodulating agents to treat advanced cancer/metastasis. We present a case of localized HNSCC post excision which presented as metastasized recurrence in the lung (leading to demise of the patient). CASE PRESENTATION: 73-year-old female nonsmoker, asthmatic presented with chest pain for 3 days. PMH was significant for progressive shortness of breath and cough for 1 month treated as pneumonia, and moderately well differentiated P16 negative HNSCC of tongue (T2N0MO) s/p left partial glossectomy and ipsilateral neck dissection with clear margins and negative lymph nodes 1 year ago. On admission, patient was hemodynamically stable with SpO2 of 90% on room air. Physical exam findings revealed decreased right sided air entry with dullness to percussion. CXR was read as atelectasis of the right lung with right sided mediastinal shift. Chest CT revealed bilateral PE and a large right lower lobe and hilar mass with right paratracheal lymphadenopathy. Additional bilateral pulmonary nodules demonstrated cavitation. Bronchoscopy and EBUS yielded keratinizing, P16 negative squamous cell cancer (SqCC) involving right upper lobe, bronchus intermedius, hilar mass and Station 7 lymph node. Immunohistochemistry (IHC) showed high expression of PDL1 i.e. Tumor Proportion Score (TPS) of 91%. Given the heavy burden of SqCC in the lung, metastatic disease from the tongue was suspected. IHC testing of the tongue specimen was requested and revealed high expression of PDL1 with TPS of 75%. Therapy with paclitaxel, carboplatin and pembrolizumab was initiated and planned for every 3 weeks, however the patient soon expired. DISCUSSION: FDA has approved two immunomodulating agents for use in PDL1 positive recurrent or metastatic HNSCC. There is no clear guideline on the utility of PDL1 testing in localized HNSCC. This case highlights the need for future research in PDL1 testing in the subset of localized HNSCC to prevent similar fatal outcomes due to recurrence. CONCLUSIONS: Incorporation of testing PDL1 in localized HNSCC might lead to improved immunotherapy and recurrence rates. Reference #1: Forster MD, Devlin MJ. Immune Checkpoint Inhibition in Head and Neck Cancer. Front Oncol. 2018;8:310. Published 2018 Aug 29. https://doi.org/10.3389/fonc.2018.00310 DISCLOSURES: No relevant relationships by Muhammad Ali, source=Web Response No relevant relationships by Mary Jo Farmer, source=Web Response

1273 IgG4 Related Cholangiopathy Masquerading as Cholangiocarcinoma

INTRODUCTION: IgG4 related diseases can involve multiple organs, best described in cases of autoimmune pancreatitis (AIP). IgG4-related cholangiopathy is difficult to distinguish from other forms of biliary ductal disorders including primary biliary cholangitis, primary sclerosing cholangitis (PSC), and cholangiocarcinoma (ChCA). Specifically, IgG4 cholangiopathy and ChCA share many clinical and imaging findings which make it difficult to distinguish these entities. We report a rare case of IgG4 cholangiopathy mimicking a ChCA. CASE DESCRIPTION/METHODS: A 69-year-old male presented with nausea/vomiting, weight loss, painless jaundice, and CT suggestion of a malignant hilar mass. ERCP with cytology and EUS with FNA from the hilar mass were negative for malignancy. Two further ERCPs with Spyscope hepatic duct bifurcation biopsies showed severe stenosis of the right hepatic ducts and diffuse biliary stricturing suggestive of PSC. Brushings for cytology and fluorescence in situ hybridization (FISH) were obtained, balloon dilation and stenting performed. MRCP noted multifocal intrahepatic biliary strictures, suggestive of PSC; a hilar mass, as seen on CT, was not seen. The MR suggested a solid pancreatic uncinate process mass. A repeat EUS showed changes of chronic pancreatitis but no mass. A comprehensive review of the combination of intrahepatic strictures, pancreatic atrophy and uncinate mass suggested a diagnosis of IgG4 disease; an ANA was positive at 1:160 and IgG4 was elevated at 1,250 mg/dL (normal < 123). The patient was started on prednisone and had complete resolution of abnormal liver tests, clinical symptoms, MR biliary ductal dilatation, and MR pancreatic mass within 3 weeks. DISCUSSION: While described in AIP, IgG-4 related diseases can affect multiple organs, including kidneys, liver, salivary gland, orbit, breast, pericardium, aorta, skin, lungs, prostate, meninges, and the pituitary gland. IgG4-related cholangiopathy is a distinct yet rare etiology, characterized by elevated serum IgG4 levels and IgG4-positive plasma cell infiltration with storiform fibrosis/obliterative phlebitis of the bile duct wall. It has been identified in patients with concomitant AIP and is steroid responsive. Early diagnosis of IgG4 cholangiopathy avoids prolonged hospitalizations, unnecessary repeated procedures, and morbidity/mortality due to treatment delay We describe a rare case of IgG4 autoimmune cholangiopathy with delayed diagnosis due to concern for ChCA.

PULMONARY VEIN THROMBOSIS VISUALIZED BY ENDOBRONCHIAL ULTRASOUND (EBUS): A RARE FINDING

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Endobronchial ultrasound (EBUS) offers a minimally invasive tool to allow for visualization and histopathological diagnosis of mediastinal and hilar lymph nodes or masses. Simultaneously, it allows for visualization of major vasculature surrounding the tracheobronchial tree such as the pulmonary veins and arteries. Limited report exists regarding pulmonary vein thrombosis (PVT) visualization with EBUS with only one case being found after conducting a PubMed search. We are presenting an additional case of PVT incidentally diagnosed by chest computed tomography (CT) and later visualized by EBUS. CASE PRESENTATION: A 66-year-old female with a history of 30 pack-year smoking and COPD stage IVB, was found to have a sub-centimeter left lower lobe (LLL) nodule on lung cancer screening. CT chest with contrast was performed revealing new right and left hilar lymphadenopathy without extra-thoracic involvement. Due to claustrophobia PET scan was not tolerated. Repeat CT with contrast revealed that the presumed left hilar mass was actually an occlusive pulmonary vein thrombosis in the superior pulmonary vein. Few weeks later patient was admitted to the hospital with worsening fatigue and right side pleuritic chest pain. Exam was unremarkable. She was managed with therapeutic anticoagulation and antibiotics for possible infectious thrombosis. EBUS-transbronchial needle aspiration of station R10 revealed small cell lung carcinoma. Incidentally the pulmonary vein thrombosis visualized by EBUS (Figure1&2) and confirmed with Doppler mode was comparable with CT images. Chest CT repeated after 6 weeks showed minimal change in the PVT and the patient was continued on therapeutic enoxaparin without complication. DISCUSSION: PVT is a rare and fatal condition with non-specific presenting symptoms making diagnosis a challenge. There have been several case reports on PE diagnosis by EBUS, but to the best of our knowledge there has been only one other case report of PVT visualized on EBUS. No guidelines exist for the diagnosis of PE and PVT with EBUS and much of literature are the case reports only. We are presenting this case to demonstrate that EBUS could be an option to diagnose or confirm PVT when other modalities are contraindicated (e.g. CT pulmonary angiography in the presence of renal impairment or contrast allergy). Although EBUS visualization of PVT may not be applicable to the general population due to cost and invasiveness it could be considered as an alternative diagnostic modality in the right sub-population. CONCLUSIONS: PVT is a rare condition which could prove fatal if diagnosed late. Due to its nebulous presentation, diagnosis can be challenging. EBUS could be considered for the diagnosis or confirmation of PVT in the right sub-population. Reference #1: MacEachern P, Dang B, Stather D, Tremblay A. Tumor invasion into pulmonary vessels viewed by endobronchial ultrasound. J Bronchol 2008; 15:206–207. Reference #2: Swartz MA, Gillespie CT. Pulmonary emboli detected by endobronchial ultrasound. Am J Respir Crit Care Med 2011; 183:1569. Reference #3: Mohammad R. Al-Ajam et al. Endobronchial Ultrasound. When to Venture into the Vasculature. ll AnnalsATS Issues. Vol. 10, No. 4 | Aug 01, 2013. DISCLOSURES: No relevant relationships by Muhammad Bilal, source=Web Response No relevant relationships by Cynthia Callahan, source=Web Response No relevant relationships by Ameet Kumar, source=Web Response No relevant relationships by Benedicta Nnodum, source=Web Response

1368 Hilar Inflammation Mimicking a Klatskin Tumor

INTRODUCTION: Hilar cholangiocarcinomas or Klatskin tumors (KT) pose significant diagnostic challenges; 15% of resections for KT prove to be non-malignant. Inflammatory, infectious, and other etiologies mimic radiological features of KT. A high index of suspicion is needed to avoid hepatic resection and associated morbidities while aggressively excluding KT. CASE DESCRIPTION/METHODS: A 64-year-old male who presented with jaundice, unintentional 50 lb weight loss, and fatigue was found to have bile duct dilation on ultrasound. A CT confirmed severe right and left hepatic duct dilatation with abrupt cutoff due to a 4 cm infiltrative hilar mass involving the right and left hepatic ducts, and extending into the common hepatic duct. Work-up for causes of jaundice (autoimmune hepatitis, hemochromatosis, alpha-1 antitrypsin deficiency, viral hepatitis, celiac sprue, autoimmune cholangiopathy) via ANA, AMA, F-actin, IgG, IgG4, HFE mutation, TIBC, ferritin, A1AT, tTG, hepatitis serologies was negative. Over 12 weeks, 3 ERCPs failed to confirm KT via biopsies, cytology, and fluorescence in situ hybridization (FISH). A severe proximal common hepatic duct stricture was found on initial ERCP, which was dilated and stented. ERCP with Spyscope showed hepatic bifurcation stricture improvement. This stricture was biopsied and again brushed, dilated and stented. A third ERCP showed further improvement in stricture and otherwise normal intra- and extra-hepatic ducts. Dilation to 8 mm was performed without further stenting. Cytology and FISH were negative for KT. Two CT guided biopsies of the mass were also negative for KT and failed to show biliary ductal thickening or clear evidence of the prior noted hilar mass. Two MRCPs, 7 months apart, showed significant improvement of right and left bile duct confluence stricturing, felt to represent fibrous tissue and scarring. DISCUSSION: Despite improvement in cross sectional imaging, advances in interventional radiologic and endoscopic modalities, differentiation between benign and malignant hilar biliary stenoses remains a major challenge. Up to 20% of hilar resection show chronic fibrosing inflammation without evidence of KT. We aggressively pursued this concern with 3 ERCPs (cytology, FISH, biopsy with Spyscope), and 2 CT guided biopsies. CT and MR showed radiologic improvement of the mass and lack of evidence of KT. Exclusion of autoimmune disease or other etiologies allowed a diagnosis of idiopathic inflammation, thus avoiding surgery.

2261 GIST or MPNST or Both? An Enigmatic Mass

INTRODUCTION: Neurofibromatosis (NF) has been frequently associated with multiple cancers and the expansive growth of these tumors often is one of the reasons for their short life expectancy. The incidence of gastrointestinal stromal tumors (GIST) and malignant peripheral nerve sheath tumors (MPNST) in patients with NF tends to be higher than the general population. The literature describes patients with only one of these tumors. The patient presented in our report likely had both tumors concomitantly, which makes our case rare. CASE DESCRIPTION/METHODS: A 39-year-old male with neurofibromatosis type 2 affecting the spine presented to the ED with RUQ abdominal pain, nausea, vomiting and jaundice. Abdominal CT and MRCP showed intrahepatic bile duct dilation with obstruction of the common duct at the gallbladder level. ERCP showed a localized biliary stricture with dilation of the common hepatic, left and right hepatic, and intrahepatic ducts. A stent was placed, but brush cytology was non-diagnostic. EUS with FNA was performed, which was also non-diagnostic. Repeat MRI a month later revealed interval growth, compressing the porta hepatis. Exploratory laparotomy demonstrated a perihilar mass (5 cm × 5 cm) involving the entire CBD and part of the left lobe of the liver. A second small bowel tumor (2 cm × 2 cm) was also present. He underwent a radical resection of the peri-hilar mass, CBD, Roux-en-Y hepatico-jejunostomy to the intra-hepatic bile ducts, retroperitoneal and peri-portal lymphadenectomy, left partial hepatectomy and small bowel resection. The small bowel mass showed a GIST with typical morphology, a low mitotic rate and a conventional immunophenotype, with expression of CD117 and DOG-1, negative for epithelial membrane antigen, rare expression of SOX10 and retention of H3K27me3. The hilar mass was mitotically active, malignant-appearing spindle cell neoplasm with necrosis. Its immunophenotype was not concordant with its morphologic features since it was found to be positive for both CD117 and DOG-1, scattered expression of SOX, diagnostic for GIST. The hilar mass showed complete loss of expression of H3K27me3, an immunohistochemical finding typically associated with malignant peripheral nerve sheath tumors. DISCUSSION: GIST and MPNSTs are reported in patients with NF. Our patient presented with a mass that had features of both in the same tumor making the diagnosis difficult and its management uncertain. Reporting of other such cases may help establish a pattern regarding this entity.

3161 A Rare Cause of Duodenal Perforation From Migrated Biliary Stent Managed Endoscopically

INTRODUCTION: Biliary stent migration is not uncommon (6-10%). A resultant duodenal perforation is however a rare complication (<1%) often associated with significant morbidity and can require surgery. Here we present a rare case of a duodenal perforation from plastic stent migration which was successfully managed endoscopically with through-the-scope (TTS) clips. CASE DESCRIPTION/METHODS: A 75 years old lady with history of painless obstructive jaundice, CT imaging with a hilar soft tissue mass and intrahepatic biliary dilation underwent endoscopic ultrasound (EUS) and endoscopic retrograde cholangiography (ERCP). A 7 Fr × 15 cm plastic biliary stent was placed into the right system after sphincterotomy and stricture dilation. The left system could not be accessed. Post procedure liver chemistries improved and she was discharged on antibiotics with close follow up. Patient developed lower abdominal pain and presented to the emergency department approximately 48 hours after ERCP. CT abdomen revealed biliary stent migration with concern for duodenal perforation and extraluminal air with heterogenous free fluid. Emergent upper endoscopy (EGD) showed migration of plastic biliary stent with distal tip protruding through the lateral wall of the second portion of the duodenum. The stent was removed using rat-tooth forceps revealing a 3 mm mucosal defect which was closed with TTS clips. Hospital course was complicated by fevers and repeat imaging showed increasing size of air and fluid collection. Repeat EGD showed a visible area of leakage on one edge of the previously repaired perforation. This was closed with hemostatic clips. Small bowel follow through studies at day 3 and 7 post procedure confirmed no contrast extravasation to suggest a persistent duodenal leak. Her diet was advanced and she was discharged with clinic follow up. DISCUSSION: Duodenal perforation from biliary stent migration is rare and limited case reports present the use of TTS clips in endoscopic management. In our patient the plastic biliary stent was endoscopically visualized protruding through the opposite wall of the duodenum while the proximal end was still in the biliary tree, likely creating enough force to cause a perforation. Although the initial attempted closure with TTS clips revealed no leakage immediately post procedurally, there was persistent leakage requiring repeat endoscopy and further clips for closure. A high morbidity surgical procedure can be avoided by this endoscopic approach even if there is a recurrence of leakage.

UNUSUAL PRESENTATION OF PNEUMOMEDIASTINUM AND SUBCUTANEOUS EMPHYSEMA MIMICKING ANGIOEDEMA

SESSION TITLE: Case Report Semifinalists 2 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a relatively safe technique associated with a very low complication rate for the sampling of mediastinal and hilar lymph nodes. Here we report a rare case of pneumomediastinum and subcutaneous emphysema following EBUS-TBNA. CASE PRESENTATION: 72 years old female, past smoker with remote history of right breast cancer s/p lumpectomy, radiation and chemotherapy (20 years ago), referred for evaluation of a 2.2 cm left hilar mass. She underwent elective EBUS under deep sedation after uneventful placement of a laryngeal mask airway, with transbronchial needle aspiration in the subcarinal (7) and left hilar stations (10L). There were no immediate complication. Two hours post procedure she developed rapidly progressive facial and neck swelling without any stridor. She was initially thought to have angioedema, and was intubated for airway compromise after she received IV Methylprednisolone 125 mg, IV epinephrine 10 mcg and 30 mcg subcutaneous epinephrine without improvement. No laryngeal edema was seen during intubation. Chest X ray subsequently showed extensive pneumomediastinum and subcutaneous emphysema. The decision was made to extubate patient since there was a risk of developing pneumothorax because of positive pressure. Patient was found to have air leak prior to extubation and was extubated to nasal cannula. Patient was discharged the next day to follow up as an outpatient. DISCUSSION: EBUS-TBNA is a safe procedure and complications are minimal. There are only a few case reports of pneumomediastinum associated with airway trauma from EBUS-TBNA so far. Subcutaneous emphysema may be observed in association with pneumomediastinum once pressure gradients allow air to spread via the fascial planes to the surrounding soft tissues [1]. Subcutaneous emphysema can mimic angioedema, an often a relatively benign disorder. Clinical presentations may include dramatic swelling of the neck associated with chest pain, wheezing, dyspnea, and dysphagia. Sparing of lips and presence of crepitus can be an important clue to differentiate it from angioedema [2]. Management of pneumomediastinum and sub cutaneous emphysema depends upon the clinical severity. Most patients respond well to the conservative management,however surgery may be required in rare cases. Treatment should be aimed at decreasing intrathoracic pressure spikes by reducing coughing and straining. CONCLUSIONS: Although EBUS-TBNA is considered a safe procedure with rare complications, it is necessary to remember that serious complications such as pneumomediastinum can occur. Our patient’s unusual presentation led to initial misdiagnosis. Although rare, clinicians must remain mindful of the complication to avoid delay in treatment. Reference #1: Ortiz, R., Hayes, M., Arias, S., Lee, H., Feller-Kopman, D. and Yarmus, L. (2014). Pneumomediastinum and Pneumopericardium after Endobronchial Ultrasound–Guided Transbronchial Needle Aspiration. Annals of the American Thoracic Society, 11(4), pp.680-681. Reference #2: Dhawan, A., Singal, A., Bisherwal, K., & Pandhi, D. (2016). Subcutaneous emphysema mimicking angioedema. Indian Dermatology Online Journal, 7(1), 55. https://doi.org/10.4103/2229-5178.174304 DISCLOSURES: No relevant relationships by Kulothungan Gunasekaran, source=Web Response No relevant relationships by Gini Priyadharshini Jeyashanmugaraja, source=Web Response No relevant relationships by Min Qiao, source=Web Response No relevant relationships by Daniel Rudolph, source=Web Response No relevant relationships by Christopher Winterbottom, source=Web Response

ATRIAL FIBRILLATION AS INITIAL PRESENTATION OF SMALL CELL LUNG CANCER

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia characterized by irregular, disorganized and rapid atrial activation leading to an irregular ventricular rhythm. The left atrium (LA) is highly susceptible to compression by extra-cardiac structures. Almost any cardiovascular condition can predispose to AF, common causes being hypertension, coronary artery disease, heart failure, valvular heart disease, cardiomyopathy, pericarditis. Extra-cardiac causes include thyrotoxicosis, chest trauma, thoracic surgery, obstructive sleep apnea, pulmonary embolism, medication use, alcohol excess, and withdrawal etc. We present a unique case of atrial fibrillation leading to a diagnosis of small cell lung cancer (SCLC). CASE PRESENTATION: We present a 74-year-old Caucasian female with significant past medical history of chronic obstructive pulmonary disease, coronary artery disease, sick sinus syndrome, hypertension, hyperlipidemia, and 70 pack-year tobacco abuse who presented with dyspnea and intermittent chest pain for last 3 days. Initial workup revealed a negative troponin trend, EKGs with non-ischemic changes. On the night of admission, patient's telemetry monitor revealed episodes of ventricular tachycardia. Subsequently, her pacemaker was interrogated and revealed runs of AF. Transthoracic echo was ordered which showed possible left atrial thrombus or mass in the LA. Interestingly enough, her previous echo just three months did not reveal any mass. A transesophageal echo was done for further evaluation which showed a large mass impinging on the LA and pulmonary veins. Computer Tomography (CT) scan of the heart revealed a 5.6 x 5.8 x 5.1cm subcarinal mass impinging on the left atrium and compressing the right pulmonary vein. The patient was taken for an endo-bronchial ultrasound with fine needle aspiration of the mass, and the pathology revealed SCLC. DISCUSSION: Though rare and often difficult to establish, there are several case reports that show an association between external compression of the LA and AF. Our patient interestingly had a benign echo just 3 months prior not showing any structurally normal heart. Previous imaging with CT Chest one year prior did not reveal subcarinal mass compressing the LA. As so, this highlights the aggressive nature of SCLC. SCLC is commonly seen in about 10-15% of lung cancers. It is a rapidly growing cancer most often seen in smokers. It typically presents as a large hilar mass with lymphadenopathy or in central airways narrowing the bronchial lumen. CONCLUSIONS: In patients with high-risk factors of lung malignancy, it is important to maintain a broad differential of their presenting symptoms. It is important to arrange a close follow up with the care team, especially in those patients with chronic diseases that may mask an underlying malignancy. Reference #1: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5129693/ Reference #2: National Cancer Institute. Scientific Framework for Small Cell Lung Cancer (SCLC). June 2014. Available online: http://deainfo.nci.nih.gov/advisory/ctac/workgroup/SCLC/SCLC%20Congressional%20Response.pdf. Accessed April 29 2016. Reference #3: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5627048/ DISCLOSURES: No relevant relationships by Machaiah Madhrira, source=Web Response no disclosure on file for Ranjit Nair; No relevant relationships by Harsh Patel, source=Web Response no disclosure on file for Muhammad Tola

PARESTHESIA, PAIN, AND ANOSMIA: AN UNCOMMON PRESENTATION OF LUNG CANCER

SESSION TITLE: Case Report Semifinalists 11 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: While paraneoplastic neurological disorders (PND) remain underreported in small cell lung cancer (SCLC), they have been most commonly associated with this tumor as compared with other forms of lung malignancy CASE PRESENTATION: An 84 year-old female with history of prior tobacco abuse presented following a fall, causing right hip fracture. She reported a 1 year history of debilitating symptoms of numbness and pain beginning in her feet, which gradually progressed to involve her distal lower extremities, and similar symptoms subsequently developing in her hands and progressing to involve both upper extremities and left shoulder accompanied by weakness in both upper and lower extremities, with subsequent loss of balance contributing to her fall. Additionally she reported total loss of smell, altered taste, early satiety, and an unintentional weight loss of 30 lbs. She had been evaluated in the outpatient setting and underwent an unremarkable upper endoscopy, colonoscopy, chest X-ray, and CT-scan of the abdomen/pelvis. An EMG demonstrated diffuse sensory polyneuropathy involving both upper and lower extremities. Prior to her presentation to the ED, serologic testing for a suspected paraneoplastic process was performed. On presentation, neurologic examination was notable for fluent speech, normal orientation and cognition, anosmia, normal cranial nerve II-XII testing, and diffuse bilateral upper and lower extremity weakness. Laboratory evaluation demonstrated normocytic anemia (HCT 31), otherwise unremarkable. During hospitalization, the paraneoplastic workup ordered by her neurologist returned as positive for ANNA-1 and CRMP-5-IgG titers. A CXR was repeated and demonstrated a well-circumscribed density in the superior left hilar region, new compared to prior imaging. CT chest demonstrated a 3.2 x 4.8 x 4.8 cm mass in the left hilar region, adjacent to the left mainstem bronchus with narrowing the left pulmonary artery, and a filling density within the left inferior pulmonary vein extending to the left atrium concerning for thrombosis or direct tumor extension. A PET/CT demonstrated a hypermetabolic left hilar mass and hypermetabolic pretracheal and bilateral hilar adenopathy. Biopsy demonstrated small cell lung cancer. DISCUSSION: We add to the literature a case of a diffuse sensory, motor and cranial neuropathy attributable to paraneoplastic neurologic syndrome secondary to SCLC. Lambert Eaton myasthenic syndrome and limbic encephalitis are the most commonly described PND associated with SCLC. Although peripheral sensory neuropathy is well described with SCLC, especially in the presence of positive onconeural antibodies (mainly ANNA-1 and CRMP-5), such extensive neurological involvement has rarely been described. CONCLUSIONS: This case highlights the need to consider early evaluation for underlying malignancy in patients with otherwise unexplained diffuse neurologic symptoms Reference #1: Gozzard, P., Woodhall, M., Chapman, C., Nibber, A., Waters, P., Vincent, A., Lang, B., Maddison, P. (2015). Paraneoplastic neurologic disorders in small cell lung carcinoma: A prospective study. Neurology, 85(3), 235-9 Reference #2: Raspotnig M., Vedeler C., Storstein A. (2015) Paraneoplastic neurological syndromes in lung cancer patients with or without onconeural antibodies. Journal of the Neurological Sciences, 348 (1-2) , pp. 41-45. Reference #3: Kanaji, N., Watanabe, N., Kita, N., Bandoh, S., Tadokoro, A., Ishii, T., Dobashi, H., Matsunaga, T. (2014). Paraneoplastic syndromes associated with lung cancer. World journal of clinical oncology, 5(3), 197-223 DISCLOSURES: No relevant relationships by Matthew Ferrantino, source=Web Response No relevant relationships by Sandrine Hanna, source=Web Response No relevant relationships by Tala Salem, source=Web Response

Surgery for liver tumors that are close to major vascular structures of future liver remnant

Introduction: In patients with just enough future liver remnant (FLR) parenchymal transection preserving all the inflow or venous outflow of FLR is very important. Tumors close to the major hilar vessels or to the outflow of FLR pose unique challenges for assessment of resectability. In both benign and malignant conditions which are close to major vessels of FLR meticulous surgery is required to obtain good outcomes. Hence, we looked at the data of such tumors which are close to the inflow and outflow of FLR and studied the surgical feasibility and perioperative outcomes. Aim: The aim of the present study was to study the perioperative results of resection of tumors close to the inflow or outflow of future liver remnant. Material and methods: The study was a retrospective study conducted in the Department of Surgical Gastroenterology, Nizam’s Institute of Medical Sciences, Hyderabad between 2015 to 2018.All tumors of the liver which were close (9cm). One patient underwent 2 stage hepatectomy because of hemodynamic instability. The final histopathology was HCC in 4, Hepatoblastoma 1, Adenoma 1 and Tuberculosis in 1. Seven patients with hilar mass underwent surgery (3 had hilar cholangiocarcinoma, 1 intrahepatic cholangiocarcinoma and 3 benign). Segmentectomy was done in 3 (1 colorectal liver metastasis, 2 HCC with CLD) The final biopsy was malignancy in 12 patients. Two (2/12, 16.6%) of them had a positive resection margin). Five patients (5/ 17, 29%) with preoperative suspicion of malignancy had a benign etiology (1 adenoma, 2 tuberculosis, 2 granular cell tumor) on final histopathology. There was 1(1/23, 4.3%) mortality in our series due to bile leak and fungal sepsis. Two patients had transient liver failure (Grade II). Six (5 Grade II, 1 Grade V) patients had transient bile leak. Conclusion: Tumors close to inflow or outflow of FLR can be resected with acceptable morbidity and mortality.

A RARE CASE OF PARANEOPLASTIC EDEMATOUS DERMATOMYOSITIS ASSOCIATED WITH SMALL CELL LUNG CANCER

SESSION TITLE: Lung Cancer: Unusual Presentations SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/21/2019 3:15 PM - 4:15 PM INTRODUCTION: Paraneoplastic edematous dermatomyositis (EDM) as a manifestation of Small Cell Lung Cancer (SCLC) is a rare process with one reported case in the literature. CASE PRESENTATION: We present a 51-year-old, previously healthy woman with a nine pack-year smoking history, who presented with a one-month history of progressive facial edema, facial erythema, lip swelling, and a violaceous atypical rash with crusting lesions involving her extremities, anterior neck, chest wall (V sign), and periungual area. The erythematous rash with blistering initially developed on the undersurface of both forearms after exposure to a wheelchair which the patient attributed to a nickel allergy. The patient failed low-dose outpatient steroids and was admitted with progressive lip swelling and facial edema. Over the course of her hospitalization, the patient developed significant proximal muscle weakness, proximal muscle tenderness, and was found to have an elevated creatine kinase level of 5,912 [IU]/L, antinuclear antibody titer was positive at 1:640 dilution, and a dermal biopsy consistent with dermatomyositis. Chest CT revealed a hilar mass with precarinal adenopathy. The patient underwent endobronchial ultrasound with transbronchial aspirate demonstrating SCLC. The patient’s facial edema and atypical rash responded to high-dose oral prednisone although proximal weakness persisted at discharge from the hospital. Upon follow up with hematology and oncology, the patient was formally diagnosed with limited stage SCLC and initiated on etoposide and carboplatin with radiation therapy. Additional evaluation with CT Abdomen demonstrated no evidence of distant metastasis. After completion of her chemoradiotherapy, her subcutaneous edematous rash resolved. DISCUSSION: A review of the literature suggests that EDM is rare with only 23 cases reported to date. EDM may be associated with a higher mortality and a more fulminant course. CONCLUSIONS: Familiarity with this paraneoplastic phenomenon may lead to early diagnosis of treatable malignancies and decrease short term mortality. Reference #1: Zarrabi, K., et al., Paraneoplastic edematous dermatomyositis: A rare syndromeobserved in a case of small cell lung cancer. Clinics and practice, 2017. 7(4). Reference #2: Milisenda JC, Doti PI, Prieto-González S, Grau JM. Dermatomyositis presenting withsevere subcutaneous edema: five additional cases and review of the literature. SeminArthritis Rheum 2014; 44:228. DISCLOSURES: No relevant relationships by James Davidson, source=Web Response No relevant relationships by Jeremy Harvey, source=Web Response No relevant relationships by Rahul Sampath, source=Web Response No relevant relationships by Mathew Thomas, source=Web Response No relevant relationships by Ly Tran, source=Web Response

Splenic Hilar Mass With Pain, Diarrhoea and Sweating: Can It Be Malignant?

A 37-year-old lady with no known comorbidity complained of left hypochondrial pain for 6 months duration. It was associated with loss of weight and appetite with occasional carcinoid symptoms such as diarrhoea and sweating. She had no blood transfusion before. Clinically, she has no features of thalassemia and the abdomen was soft with a palpable vague mass on the left hypochondrial region. A computed tomography (CT) of the abdomen revealed a soft tissue mass measuring 8.9 x 10.3 x 9.0 cm in the enlarged spleen

Re: Diagnostic Yield and Complication Rate in Percutaneous Needle Biopsy of Renal Hilar Masses with Comparison with Renal Cortical Mass Biopsies in a Cohort of 195 Patients.

No AccessJournal of UrologyUrological Survey1 Jul 2019Re: Diagnostic Yield and Complication Rate in Percutaneous Needle Biopsy of Renal Hilar Masses with Comparison with Renal Cortical Mass Biopsies in a Cohort of 195 Patients Cary SiegelMD Cary SiegelCary Siegel More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000000255AboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "Re: Diagnostic Yield and Complication Rate in Percutaneous Needle Biopsy of Renal Hilar Masses with Comparison with Renal Cortical Mass Biopsies in a Cohort of 195 Patients." The Journal of Urology, 202(1), p. 11 © 2019 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 202Issue 1July 2019Page: 11-11 Advertisement Copyright & Permissions© 2019 by American Urological Association Education and Research, Inc.MetricsAuthor Information Cary Siegel More articles by this author Expand All Advertisement Loading ...

SIGNET-RING CELL CARCINOMA OF UNKNOWN PRIMARY: A CASE REPORT AND REVIEW OF RELATED LITERATURE OF A RARE TYPE OF CANCER WITH A RARE PRESENTATION

PURPOSE: We report a case of metastatic signet-ring cell carcinoma (SRRC) of unknown primary presenting with superior vena cava syndrome and discuss our approach to diagnosis and management. METHODS: Case report and review of literature. RESULTS: A 53-year-old man, non-smoker, presented with a three-month history of worsening non-productive cough, upper back pain and facial and bilateral upper extremity swelling. On admission, he had a blood pressure of 180/100 mmHg, facial puffiness, neck vein engorgement, absent breath sounds with decreased vocal fremitus over the right lower lung and swelling of bilateral upper extremities. Work-up done revealed the following: (a) chest CT scan:right para-tracheal and hilar mass compressing the superior vena cava and bilateral pleural effusion; (b) fiberoptic bronchoscopy:extrinsic compression of the right lower lobe - superior lung segment; (c) pleural fluid and bronchoalveolar lavage histopathology:consistent with metastatic adenocarcinoma (SRCC subtype – positive for CK7, negative for CK 20, TTF-1, and calretinin), favoring a gastric or pancreaticobiliary primary; (d) abdominopelvic CT scan:slight fundic wall thickening and normal pancreas and bile duct; multiple scattered metastatic foci were noted. CONCLUSIONS: He gradually improved with radiotherapy and intravenous steroids. Out-patient chemotherapy was then planned. CLINICAL IMPLICATIONS: SRCC is an aggressive type of poorly differentiated adenocarcinoma with superior vena cava syndrome as a rare initial manifestation. Determining the primary site remains priority and is paramount in guiding further management. However, current evidences have mainly focused on palliative chemotherapy as treatment of choice in most cases.

Hepatic hilar mass in an adolescent: a rare case of hepatobiliary tuberculosis

BackgroundHepatobiliary tuberculosis is a rare manifestation of Mycobacterium tuberculosis infection, especially in younger patients. The non-specific symptoms and signs as well as the lack of definite imaging characteristics often impedes diagnosis. Definite diagnosis of tuberculosiscan be obtained through histopathological examination; conventional anti-tuberculosis drugs and surgery are the most commonly recommended treatments.Case presentationA previously healthy 15-year-old rural adolescent male presented with a 2-month history of weight loss and fatigue. We strongly suspected a Klatskin tumor; therefore, exploratory laparotomy was performed. However, the microscopical findings revealed a granuloma consisting of epithelioid cells, caseous necrosis, and lymphocytic infiltration, indicating caseating granulomatous inflammation and yielding a final diagnosis of hepatic hilar tuberculosis.ConclusionHepatic hilar tuberculosis is an extremely rare case; few physicians may have actually treated a case. This report therefore aims to improve the overall understanding of lymphatic tuberculosis of the hepatic hilum.

A young male with left hilar mass.

A young male with left hilar mass.

Multiple Synchronous Tuberculous Aneurysms

AbstractThe authors report a case of a 35-year-old woman with a medical history of recently diagnosed pulmonary tuberculosis presenting with chest and back discomfort. Chest radiograph demonstrated a bulky hilar mass, and computed tomography revealed multiple saccular aneurysms in the thoracic aorta, multiple fusiform aneurysms in the coronary arteries, left renal and splenic arteries, and right internal iliac pseudoaneurysms. Surgical management of the mycotic aneurysms was indicated in view of symptomatology. Thoracic endovascular aortic repair (TEVAR) and ascending aortic replacement with aortic valve suspension and coil embolization of right iliac artery pseudoaneurysm were performed. The coronary artery aneurysms were managed with surveillance.

Portal Cavernoma Cholangiopathy

Portal cavernoma cholangiopathy (formerly portal biliopathy) is a type of biliary injury that occurs in association with a portal vein thrombus or cavernoma. Although the radiographic features of portal cavernoma cholangiopathy have been enumerated in the literature, its histologic features have not been described in detail.We describe the histologic findings in liver specimens from three patients with radiologically confirmed portal cavernoma cholangiopathy.Of the three patients, one underwent surgical resection due to a clinical suspicion for cholangiocarcinoma, one had a liver biopsy sample obtained for evaluation of possible cirrhosis, and one had a clinically suspicious "hilar mass" at the time of orthotopic liver transplant. Histologic features common among the three liver specimens included portal venous abnormalities, where the portal veins were obliterated or small relative to the portal tract size, and obstructive biliary changes, such as ductular reaction and reactive epithelial atypia accompanied by a mixed inflammatory cell infiltrate with neutrophils.This case series provides clinicopathologic characteristics of portal cavernoma cholangiopathy. Histologic changes are reminiscent of hepatoportal sclerosis and/or bile duct obstruction. Attention to portal veins can provide helpful diagnostic clues, especially when biopsy samples are obtained from patients with a known portal vein thrombus or cavernoma.

Superior vena cava syndrome in a patient with locally advanced lung cancer with good response to definitive chemoradiation: a case report

BackgroundThe incidence of superior vena cava syndrome within the United States is roughly 15,000 cases per year. Superior vena cava syndrome is a potentially life-threatening medical condition; however, superior vena cava syndrome is not fatal in the majority of cases. Superior vena cava syndrome encompasses a collection of signs and symptoms resulting from obstruction of the superior vena cava, including swelling of the upper body of the head, neck, arms, and/or breast. It is also associated with cyanosis, plethora, and distended subcutaneous vessels. Lung cancer, including both non-small cell lung cancer and small cell lung cancer, is the most common extrinsic cause of superior vena cava syndrome. Intrinsic disruption of superior vena cava flow can also precipitate superior vena cava syndrome. This case report describes an unusual presentation and potential etiology of superior vena cava syndrome.Case presentationOur patient was a 51-year-old black woman with locally advanced, stage IIIB non-small cell lung cancer who had no clinical symptoms of superior vena cava syndrome at the time of diagnosis. However, she did have radiographic evidence of superior vena cava stenosis caused by extrinsic compression from her large right hilar primary tumor. She was treated with definitive chemoradiation, receiving 60 Gy of external beam radiation therapy given concurrently with chemotherapy. Three months after completion of radiotherapy, she developed signs of superior vena cava syndrome, including breast and supraclavicular swelling. She had a chest computed tomography scan showing over 50% reduction in the size of a right hilar mass; however, she had continued radiographic stenosis of the superior vena cava. The distribution of stenosis appeared to be inferior to the caudal extent of pretreatment tumor volume. She had no other radiographic indications for superior vena cava syndrome.ConclusionsGenerally, superior vena cava syndrome is the result of extrinsic compression of the superior vena cava by tumor. Our patient’s case represents the development of superior vena cava syndrome after an excellent response of tumor with near-complete tumor response. We suspect chemoradiation therapy as a potential etiology for the precipitation of the superior vena cava syndrome, which is currently not well reported in the medical literature.