MON-LB040 Challenge in Diagnosing and Treating of Mediastinal Paraganglioma

Paraganglioma is a rare type of neuroendocrine tumor of the autonomicnervous system.1 It is extremely rarely present in mediastinum (less than2%).2Case report We report a 43 years-old woman, she was referred to our clinic with ahistory of high blood pressure (BP) for two years, accompanied by spellsof sweating, headache, anxiety and palpitation. She was provisionaldiagnosed with essential hypertension and was on four medications onmaximum dose.The patient declined any previous surgical history, and review of systemswas unremarkable. On examination, she was conscious and alert. Her vitalsigns were within normal limit. She reported to our clinic with homereadings of BP ranging from 150-180 mmHg systolic and 90-100 mmHgdiastolic.Initial investigations of basic chemistry, renal profile, hormonal profile,aldosterone, and renin were within the normal range.Serum Normetanephrine was 800 ng/L (normal < 180 ng/L), 24 hoursurine of Normetanephrine was 5205 microgram/24 hours (normal < 600microgram/24 hours), and 24 hours urine Metanephrine was withinnormal. CT scan of the adrenal and MRI abdomen showed normal adrenalglands and no mass in the abdomen. MIBG scan was normal for the wholebody. An unusual location of the Paraganglioma was suspected, andfurther images were carried on. A CT chest showed 4x4 cm posteriormediastinal mass in area of Aortopulmonary window, adherent toposterior wall of Aorta and pulmonary artery. Positron EmissionTomography (PET) scan was done prior to the surgery.Surgical resection of the mass with reconstruction of both pulmonaryartery and pericardium was done without any complications. Moreover,histopathology confirmed the diagnosis of Paraganglioma.The patient was followed up with a CT scan six months post-operativelyas an outpatient, along with 24-hour urine Metanephrine and Normetanephrine. All labs and imaging were normal. The patient hadanother Normetanephrine measurement twelve months later and it wasnormal. Now, she has been followed for seven years with no moresymptoms and normal BP readings. All of her antihypertensive agentswere discontinued. ConclusionWe experience an unusual location of Paraganglioma at the mediastinum,which is representing less than 2% of all Paraganglioma. However, in thepresence of characteristic of Paraganglioma symptoms, a thoroughassessment should be carried out and such location of mediastinumshould be suspected and investigated.References:1. Institute NC. Pheochromocytoma and Paraganglioma Treatment(PDQ®)-Health Professional Version. https://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq. Published 2019. Accessed February 1, 2020.2. Paraganglioma: An Uncommon Cause of Mediastinal Mass. -PubMed - NCBI. https://www.ncbi.nlm.nih.gov/pubmed/32000513.Accessed February 1, 2020.

Clinical significance of vanillylmandelic acid in neuroblastoma

Background: The second most common solid tumor in children is Neuroblastoma (NB). In about 90% of cases of NB, elevated levels of catecholamines or its metabolites are found in the urine or blood which includes Vanillylmandelic Acid (VMA) and Homovanillic Acid (HVA). Ferritin, Neuron-Specific Enolase (NSE) and Lactate Dehydrogenase (LDH) are commonly assessed in children suspected to have NB, and the levels of these markers are commonly used for differential diagnosis. Multiple clinical and imaging tests are needed for accurate patient assessment. Iodine 123(123I) Metaiodobenzylguanidine (MIBG) is the first-line functional imaging agent used in neuroblastoma imaging. To evaluate the utility of these marker present study was undertaken with 91 NB patients and 40 normal healthy control.Methods: The study comprised of blood samples and 24 hour’s urine sample from 40 normal healthy subjects and 91 untreated patients with histologically proven Stage III and IV NB cases referred to our institute. Method used for NSE was Enzyme Immunoassay (Elisa), serum Ferritin was MIA, LDH-photometry and VMA by Column Chromatography.Results: Amongst the parameters studied VMA showed highest sensitivity (91%), specificity (94.4%) positive predictive value (97.8%) and 85% negative predictive value at the cut off levels of 7mg/ ml of creatinine as compared to other studied parameters.Conclusions: This study suggests that the detection of VMA in combination with routine histological examination, MIBG scan, serum NSE and LDH may improve the diagnosis of Neuroblastoma.

Simple adrenal cyst masquerading clinically silent giant cystic pheochromocytoma

Pheochromocytoma (PCC) may present as a cystic or solid tumour. Cystic PCCs are difficult to differentiate from simple cysts in the absence of classic symptoms of PCCs. Cystic nature develops due to intralesional bleeding and necrosis. We present a case of young man without any comorbidity who was diagnosed as a simple adrenal cyst and planned for laparoscopic excision but found to be functional PCC during the time of surgery only. The patient was managed with extensive monitoring and use of multiple drugs to control blood pressure. Surgery was completed without any complication laparoscopically and postoperative period was also uneventful. Histopathology confirmed the diagnosis of cystic PCC. Our case also shows the importance of functional imaging like metaiodobenzylguanidine (MIBG) scan in doubtful cases of adrenal cysts when other biochemical markers are unremarkable to diagnosis. We emphasise the importance of meticulous preparation for any intraoperative disasters even for apparently simple adrenal cyst.

Successful treatment of refractory metastatic neuroblastoma with panobinostat in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine therapy

Introduction Neuroblastoma commonly required multimodal therapy containing surgery, chemotherapy, radiotherapy, and immunotherapy. Case report In our case, who had refractory metastatic neuroblastoma, we use histone deacetylase inhibitor (panobinostat) in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine (MIBG) therapy. Management and outcome This approach leads to successfully treat the patient. MIBG scan and bone marrow examination after therapy revealed no evidence of tumor. Now, she underwent autologous transplantation six months ago and free of tumor. Conclusion Panobinostat can cause apoptosis induction in refractory metastatic neuroblastoma in combination with MIBG therapy and chemotherapy.