Nonsecreting Tumors Research Articles

Intraoperative hemodynamic instability during laparoscopic adrenalectomy for pheochromocytoma without preoperative medical preparation compared with nonsecreting tumor

BackgroundControl of hemodynamic features during adrenalectomy for pheochromocytoma is recommended to minimize perioperative cardiovascular complications. However, episodes of intraoperative hemodynamic instability have been observed during adrenalectomies with other indications than pheochromocytoma. The objective of this study was to compare the hemodynamic instability score assessed during unilateral adrenalectomy for pheochromocytoma without preoperative medical preparation to hemodynamic instability score in nonsecreting tumor. MethodsThis was an observational study with prospective intraoperative hemodynamic data collection (every 20 seconds) and retrospective analysis. ResultsDuring the study period, 60 consecutive patients (30 pheochromocytomas vs 30 nonsecreting tumors) were included with a median number of data collections during total procedure time of 318 (interquartile range, 257–388). Mean cumulative intraoperative time outside the target blood pressure range expressed as a percentage of total procedure time was 13.3% vs 6.8% for systolic blood pressure >160 mm Hg (P = .01) and 2.4% vs 2.8% for mean arterial pressure <60 mm Hg (P = ns), respectively. The median hemodynamic instability score during total procedure time was 33 (interquartile range, 27–43) and 20 (interquartile range, 11–26) in the pheochromocytoma and nonsecreting tumor group, respectively (P < .01). Hemodynamic instability score were similar in patients with compared with without long-term antihypertensive treatment in each patient group (P = ns). The mean length of hospital stay was 2.0 ± 1.5 days, and 30-day morbidity rate was 6.6% (4/60) with no significant difference observed between both groups. ConclusionAlthough intraoperative hemodynamic instability remains greater in the pheochromocytoma group without preoperative medical preparation, both groups have similar hypotensive episodes. These data highlight the need to better understand the role of preoperative medical preparation in pheochromocytoma patients.

Risk factors associated with postoperative cerebrospinal fluid leak after endoscopic endonasal skull base surgery: Two-center retrospective cohort study.

Over the past two decades, endoscopic endonasal skull base surgery (EESBS) has revolutionized the treatment of skull base tumors by enabling minimal access to resect significant pathologies such as meningiomas, pituitary adenomas, and chordomas. Despite its efficacy, complications such as cerebrospinal fluid (CSF) leak pose significant challenges, with an average incidence of 8.9% following EESBS. Therefore, our study aims to investigate the risk factors associated with postoperative CSF leak after employing an endoscopic endonasal approach for skull base surgery, focusing on patients treated at King Abdul-Aziz Medical City in Jeddah and King Abdullah Medical City in Makkah, Saudi Arabia. A retrospective review of patients who underwent an endoscopic endonasal approach for the resection of intradural skull base pathology between January 2016 and December 2022 was performed with a total of 51 patients. Basic demographic data were collected, along with patient comorbidities, presenting symptoms, tumor pathology, tumor site, the extent of resection, and outcomes. 51 participants were analyzed, with a mean age of 43.41. Male participants comprised (58.8%), while headaches were the most common symptom (74.5%), followed by visual disturbances (51.0%). Nonsecretory tumors predominated, primarily located in the sellar and suprasellar regions (58.8%). Gross total resection was performed in the majority (56.9%), with a 9.8% incidence of postoperative CSF leak. Notably, all four patients with CSF leak experienced preoperative headaches. Hypertension was prevalent in Cases 1 and 4, with Case 4 having a history of neurological disease and radiation therapy. Anterior skull base tumors were most frequent in Cases 3 and 4. The incidence of CSF leakage after surgery in this study was similar to that previously reported. It is worth noting that repeated surgeries may increase the risk of postoperative CSF leakage. Therefore, it is important to carefully evaluate the surgical approach for tumor removal and skull base reconstruction, considering the tumor characteristics and the patient's overall condition.

Pathophysiological features of neuroendocrine tumors in cyanotic congenital heart disease: a systematic review

Abstract Background Patients with cyanotic congenital heart disease (CCHD) have an increased risk to develop pheochromocytomas or paragangliomas (PPGL), which are rare neuroendocrine tumours molecularly driven by the activation of the hypoxia-inducible factor 2-alpha (HIF-2α ). Purpose To define the clinical characteristics of CCHD-PPGL patients. Methods An international multicentre CCHD-PPGL consortium from 8 countries was stablished. The group performed a systematic review of clinic-pathological features of 149 CCHD-PPGL cases and compared to 1559 CCHD non-PPGL patients. Results CCHD-PPGL were predominantly women (56.4%) with univentricular heart (UVH) (55.0%), followed by tetralogy of Fallot (23.5%). Most patients remained unoperated (28.6%) or had only undergone a palliative procedure (25.8%), and the mean saturation at diagnosis was 84.0%. The median age at PPGL diagnosis was 27.0 yo [20-38]. Pheochromocytoma (PCC) occurred in 36.9% of patients, abdominal or thoracic paraganglioma (TA-PGL) in 40.3%, carotid body paraganglioma (CB-PGL) in 14.7% and a combination in 8.0%. Most CB-PGLs were non-secreting, non-symptomatic tumors while PCC and TA-PGL were mostly noradrenalin-secreting and symptomatic tumors. Metastatic disease was documented in 10.3% of non-CB and none in CB-PGL . Comparing to patients with PCC or TA-PGL, CB-PGL patients were predominantly female (81.8% vs 52.0%), older at time of diagnosis (43 yo [36.0 – 52.0] vs 25.0 yo [19.0-35.0], p&amp;lt;0.01) and with a longer period of cyanosis at diagnosis (37.0 [23-47] vs 23.0 [13-46], p&amp;lt;0.01). Patients with Fontan procedure were mostly men (63.3%) who developed a noradrenalin-secreting PCC or a TA-PGL (42.0% and 58.0% respectively) at an earlier age than non-Fontan patients (22.0yo [18.0-27.0] vs 29.0yo [21.0-41.0], p&amp;lt;0.01). Patients with Eisenmenger physiology were female patients (100%) who developed mostly CB-PGL, non-secreting tumors (54.5%) and were older at diagnosis (48.0 yo [42-58]). Compared to a cohort of 1599 CCHD non- PPGL patients, CCHD-PPGL were predominantly women (57.7% vs 44.8%, p&amp;lt;0.01), with forms of UVH (53.7% in CCHD-PPGL vs 14.1% in CCHD, p &amp;lt;0.01 ) and were mainly unoperated and palliated patients (27.1% and 26.4% respectively vs 10.0% and 5.3% in CCHD, p &amp;lt;0.01). CCHD-PPGL patients showed lower levels of oxygen saturation (80.0% [76.0-85.0] vs 84.0% [80.0-88.0], p&amp;lt;0.01) and higher haematocrit levels (60.0% [52.0-66.5] vs 53.3% [46.3-62.0], p&amp;lt;0.01) than CCHD without PPGL. Conclusions Patients with CCHD and lifelong cyanosis, and especially those with Eisenmenger physiology, probably develop CB-PGL as an adaptive response to chronic systemic hypoxemia, present a good clinical course, and usually do not require an initial surgical approach. In patients with Fontan circulation, the risk of developing a sympathetic thoracic-abdominal PPGL persists, despite correction of hypoxemia, possibly due to the initial hypoxemia during adrenal medulla post-natal development.

Long Term Post-Treatment Follow-Up of Pituitary Tumours

Aim: To study the spectrum of Pituitary tumours, their presentation, treatment and compliance with follow-up. Study design: Mixed retrospective and prospective study Place and duration of study: Medicell Institute of Diabetes, Endocrinology and Metabolism and Jinnah Postgraduate Medical Centre Karachi from 1st January 2012 to 31st July 2022. Methodology: One hundred and seventy three cases of pituitary tumours, were enrolled. Data regarding age, gender, duration of disease, symptoms, mode of treatment administered and compliance with the follow-up instructions was retrieved. Results: The mean age was 36.4±10.6 years. Female predominance was seen in 97(56.1%). Of the 136 married patients, subfertility was seen in 48(36.3%). Headache was observed in 164(94.8%) of patients. Prolactinoma was the lead tumour comprising of 76 cases (43.9%), followed by non-secretory tumours 47(27.2%), and acromegaly 43(24.9%). Other rare disorders including Cushing’s disease, craniopharyngioma and gonadotropinoma were also seen. Cabergoline was prescribed in 136 (78.6%) cases. Transsphenoidal surgery (TSS) was done in 74(42.8%) of patients. Out of 97 female patients, 38(39.2%) had persistent amenorrhea post-treatment, while this data was not available for 40(41.2%) of these women, who were lost to follow-up. The difference between mean prolactin levels prior and after the treatment (1390.048±2646.986 versus 116.360±163.369) were found to be statistically significant p&lt;0.001). Similarly, IGF-1 levels were significantly improved post treatment indicating tumour stability. Moreover, the level of mean FT4 post-treatment was 1.64, reflecting adequate replacement. Conclusion: Majority of the patients had improvement post-treatment in pituitary tumour symptoms including vision, headache and sexual function. Failure to attend for follow-up for tumour stability, control of excess hormone, and pituitary hormone replacement was seen in a large number of patients in this study. This requires careful consideration of different strategies to ensure better long term care in pituitary tumours. Keywords: Pituitary tumor, Prolactinoma, Craniopharyngioma, Gonadotropinoma, Acromegaly

PMON85 Co-occurrence of Carotid Body Tumor, Meningioma and Schwannoma in a Symptomatic Patient, Incidental Findings Vs Genetic Syndrome: A Case Report

Abstract Carotid body tumors, also known as chemodectomas/paraganglionomas; are rare neuroendocrine tumors that arise from parasympathetic ganglionic cells. They are usually benign with incidence of malignancy is &amp;lt;10%. In majority of cases, tumors are asymptomatic and found incidentally on clinical exam or radiological imagining. Their association with other non-neuroendocrine tumors as seen in present case; has not been well documented in literature. Case presentation 43 y/o female with PMH of GERD, obesity, asthma and anxiety disorder who came to endocrine clinic for left middle thyroid lobe nodule(0.9×0.6×0.6cm) evaluation; incidentally noted to have a multilobulated heterogeneous hypo echoic and iso-echoic mildly vascularized solid nodular mass (3.46×1.83×1.93 cm) in the left carotid bifurcation area; splaying the common and the internal carotid arteries on US. Review of systems was positive for left sided neck pain, intermittent palpitations, headaches, anxiety, and sleep disturbances. Clinical exam was remarkable for thyromegaly and a palpable non-tender 3cm left neck mass with no cervical lymphadenopathy. Initial labs were notable for mild elevation of chromogranin A (108.4 ng/ml) with normal levels of TSH, free T3, free T4, plasma metanephrines and 24-hour urinary metanephrines. Considering patient's persistent symptoms; plasma catecholamines levels were done and were negative. MRI soft tissue neck confirmed 2.8 cm lobular mass suggestive of paraganglioma. Metastatic work up was negative. Patient was referred for genetic testing considering young age and to rule out hereditary disorders. Interim, patient presented to the ED with complain of sudden onset lumbar backache without sphincteric involvement; neurological exam was unrevealing but on MRI lumbar spine found to have a 5.7×4.8×4cm lobulated well-circumscribed homogeneous soft tissue mass suggestive of schwannoma. Cervical and thoracic spine imaging was unremarkable. MRI brain showed a 1.1cm enhancing mass in right Meckel cave suggestive of meningioma. PET-DOTATATE scan was performed that revealed left neck mass as intensely avid, Right petrous apex mass as moderately avid and lumbar mass as not avid at all, consistent with the diagnosis of paraganglionoma, meningioma and schwannoma respectively. No evidence of DOTATATE avid nodal/ distant metastatic disease was found. Initially a hereditary etiology was suspected but genetic testing including (VHL, NF1/2, SDHD, SDHB) came back negative. Case was discussed with multidisciplinary teams; Neurosurgery service recommends conservative management of both meningioma and schwannoma at the moment considering benign course. Regarding paraganglioma, as tumor is non-secretory on repeated work up; sympathetic blockade is not warranted and patient is scheduled for pre-op embolization and surgical resection by Head and Neck surgery. Conclusion Paraganglionomas are generally benign non-secretory tumors that usually occur with other neuroendocrine tumors both as hereditary syndromes and sporadic cases. But their association with non-neuroendocrine tumors is mere incidental or has a genetic causation; is still unclear and warrants further research. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

PMON85 Co-occurrence of Carotid Body Tumor, Meningioma and Schwannoma in a Symptomatic Patient, Incidental Findings Vs Genetic Syndrome; a case report.

Abstract Carotid body tumors, also known as chemodectomas/paraganglionomas; are rare neuroendocrine tumors that arise from parasympathetic ganglionic cells. They are usually benign with incidence of malignancy is &amp;lt;10%. In majority of cases, tumors are asymptomatic and found incidentally on clinical exam or radiological imagining. Their association with other non-neuroendocrine tumors as seen in present case; has not been well documented in literature. Case presentation 43 y/o female with PMH of GERD, obesity, asthma and anxiety disorder who came to endocrine clinic for left middle thyroid lobe nodule(0.9×0.6×0.6cm) evaluation; incidentally noted to have a multilobulated heterogeneous hypo echoic and iso-echoic mildly vascularized solid nodular mass (3.46×1.83×1.93 cm) in the left carotid bifurcation area; splaying the common and the internal carotid arteries on US. Review of systems was positive for left sided neck pain, intermittent palpitations, headaches, anxiety, and sleep disturbances. Clinical exam was remarkable for thyromegaly and a palpable non-tender 3cm left neck mass with no cervical lymphadenopathy. Initial labs were notable for mild elevation of chromogranin A (108.4 ng/ml) with normal levels of TSH, free T3, free T4, plasma metanephrines and 24-hour urinary metanephrines. Considering patient's persistent symptoms; plasma catecholamines levels were done and were negative. MRI soft tissue neck confirmed 2.8 cm lobular mass suggestive of paraganglioma. Metastatic work up was negative. Patient was referred for genetic testing considering young age and to rule out hereditary disorders. Interim, patient presented to the ED with complain of sudden onset lumbar backache without sphincteric involvement; neurological exam was unrevealing but on MRI lumbar spine found to have a 5.7×4.8×4cm lobulated well-circumscribed homogeneous soft tissue mass suggestive of schwannoma. Cervical and thoracic spine imaging was unremarkable. MRI brain showed a 1.1cm enhancing mass in right Meckel cave suggestive of meningioma.PET-DOTATATE scan was performed that revealed left neck mass as intensely avid, Right petrous apex mass as moderately avid and lumbar mass as not avid at all, consistent with the diagnosis of paraganglionoma, meningioma and schwannoma respectively. No evidence of DOTATATE avid nodal/ distant metastatic disease was found. Initially a hereditary etiology was suspected but genetic testing including (VHL, NF1/2, SDHD, SDHB) came back negative. Case was discussed with multidisciplinary teams; Neurosurgery service recommends conservative management of both meningioma and schwannoma at the moment considering benign course. Regarding paraganglioma, as tumor is non-secretory on repeated work up; sympathetic blockade is not warranted and patient is scheduled for pre-op embolization and surgical resection by Head and Neck surgery. Conclusion Paraganglionomas are generally benign non-secretory tumors that usually occur with other neuroendocrine tumors both as hereditary syndromes and sporadic cases. But their association with non-neuroendocrine tumors is mere incidental or has a genetic causation; is still unclear and warrants further research. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Distinct clinical phenotypes in a family with a novel truncating MEN1 frameshift mutation

BackgroundMEN1 mutations can inactivate or disrupt menin function and are leading to multiple endocrine neoplasia type 1, a rare heritable tumor syndrome. Case presentationWe report on a MEN1 family with a novel heterozygous germline mutation, c.674delG; p.Gly225Aspfs*56 in exon 4 of the MEN1 gene. Diagnosis and clinical phenotyping of MEN1 was established by laboratory tests, ultrasound, biopsy, MRI imaging and endosonography. The clinical course of the disease was followed in the index patient and her family members for eight years. The mutation was associated with distinct clinical phenotypes in the index patient and three family members harboring p.Gly225Aspfs*56. Family members affected showed primary hyperparathyroidism but variable patterns of associated endocrine tumors, adrenal cortical adenomas, prolactinoma, multifocal pancreatic neuroendocrine tumors, insulinoma and nonsecretory neuroendocrine tumors of the pancreas. The mutation c.674delG; p.Gly225Aspfs*56 leads to a frameshift from codon 225 with early truncation of the menin protein. In silico analysis predicts loss of multiple protein-menin interactions in p.Gly225Aspfs*56, potentially rendering menin insufficient to control cell division and replication. However, no aggressive neuroendocrine tumors were observed in the follow-up of this family.ConclusionsWe report a novel heterozygous MEN1 frameshift mutation, potentially causing (at least partial) inactivation of menin tumor suppression potential but lacking a genotype–phenotype correlation. Our study highlights the importance of personalized care with appropriate testing and counseling in MEN1 families.

Abstract P3-09-10: NTRK1/2/3 fusions are observed in both secretory and non-secretory breast cancers

Abstract Background NTRK1/2/3 kinase fusions are rare oncogenic drivers found in &amp;lt;1% of solid tumors. NTRK fusion-positive (NTRK+) solid tumors have shown meaningful clinical response to small molecule NTRK inhibitors such as entrectinib and larotrectinib, and broad genomic testing will help identify patients (pts) with these rare alterations. NTRK fusions occur more frequently in some cancers than others. For example, NTRK fusions are characteristic of secretory breast cancers (BCa), and it has been posited that non-secretory BCa lack druggable NTRK fusions (PMID: 30707464). However, these conclusions were drawn on limited patient populations. Here we show evidence of NTRK fusions in both secretory and non-secretory breast tumors in the largest real-world cohort of NTRK+ breast tumors reported to date. Methods Comprehensive genomic profiling of at least 324 cancer-related genes, including NTRK1/2/3, was done at Foundation Medicine on de-identified all-comer BCa pts during the course of routine clinical care. Pathology reports and hematoxylin and eosin (H&amp;E) images of NTRK+ breast tumor tissue were examined by a board-certified pathologist for designation of secretory vs. non-secretory phenotype. Results NTRK fusions were identified in 23 BCa specimens. H&amp;E images were available for review in 22 cases, and another was confirmed as secretory via the pathology report. Pathologist review of those 23 cases revealed that NTRK fusions were present in 11 with secretory phenotypes, 11 with non-secretory phenotypes, and 1 with a mixed secretory phenotype. Secretory cases most often harbored a ETV6-NTRK3 fusion (n=7) with additional recurrent fusions seen in TPM3-NTRK1 (n=2) and LMNA-NTRK1 (n=2). Non-secretory breast cancers also harbored ETV6-NTRK3 (n=2), TPM3-NTRK1 (n=1), and LMNA-NTRK1 (n=1) fusions, as well as several unique fusion events that retain the NTRK kinase domain. Conclusions While rare, NTRK fusions occur in a wide variety of tumor types. Within BCa, this includes both secretory and non-secretory phenotypes, with half of the identified alterations occurring in non-secretory BCa. Recurrent events, including ETV6-NTRK3, were observed in both populations. These data support NTRK fusion testing for all BCa pts with both secretory and non-secretory histologic subtypes. Citation Format: Ethan Sokol, Sophia Maund, Jeffrey Ross, Timothy Wilson. NTRK1/2/3 fusions are observed in both secretory and non-secretory breast cancers [abstract]. In: Proceedings of the 2021 San Antonio Breast Cancer Symposium; 2021 Dec 7-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2022;82(4 Suppl):Abstract nr P3-09-10.

Modern Linear Accelerator–Based Radiotherapy Is Safe and Effective in the Treatment of Secretory and Nonsecretory Pituitary Adenomas

Adjuvant radiotherapy (RT) can help achieve local control (LC) and reduce hormonal overexpression for pituitary adenomas (PAs). Prior reports involved Gamma Knife or older linear accelerator (LINAC) techniques. The aim of this study was to report long-term outcomes for modern LINAC RT. Institutional retrospective review of LINAC RT for PAs with minimum 3 years of magnetic resonance imaging follow-up was performed. Hormonal control was defined as biochemical remission in absence of medications targeting hormone excess. LC defined using Response Evaluation Criteria in Solid Tumors on surveillance magnetic resonance imaging. Progression-free survival defined as time alive with LC without return of or worsening hormonal excess from secretory PA. Kaplan-Meier and Cox proportional hazard models used. From 2003 to 2017, 140 patients with PAs (94 nonsecretory, 46 secretory) were treated with LINAC RT (105 fractionated RT, 35 radiosurgery) with median follow-up of 5.35 years. Techniques included fixed gantry intensity-modulated radiotherapy (51.4%), dynamic conformal arcs (9.3%), and volumetric modulated arc therapy (39.3%). Progression-free survival at 5 years was 95.3% for secretory tumors and 94.8% for nonsecretory tumors. Worse progression-free survival was associated with larger planning target volume on multivariable analysis (hazard ratio 2.87, 95% confidence interval 1.01-8.21, P= 0.049). Hormonal control at 5 years was 50.0% and associated with higher dose to tumor (hazard ratio 1.05, 95% confidence interval 1.02-1.09, P= 0.005) and number of surgeries (hazard ratio 1.74, 95% confidence interval 1.05-2.89, P= 0.032). Patients requiring any pituitary hormone replacement increased from 57.9% to 70.0% after RT. Modern LINAC RT for patients with PAs was safe and effective for hormonal control and LC. No difference in LC was noted for functional versus nonfunctional tumors, possibly owing to higher total dose and daily image guidance.

Head and Neck Paragangliomas in the Czech Republic: Management at the Otorhinolaryngology Department

Head and neck paragangliomas (HNPGLs) are rare neuroendocrine tumors, comprising only 3% of all head and neck tumors. Early diagnosis forms an integral part of the management of these tumors. The two main aims of any treatment approach are long-term tumor control and minimal cranial nerve morbidity. The scope of this article is to present our case series of HNPGLs to stress most important clinical aspects of their presentation as well as critical issues of their complex management. Thirty patients with suspected HNPGLs were referred to our otorhinolaryngology clinic for surgical consultation between 2016–2020. We assessed the demographical pattern, clinicoradiological correlation, as well as type and outcome of treatment. A total of 42 non-secretory tumors were diagnosed—16.7% were incidental findings and 97% patients had benign tumors. Six patients had multiple tumors. Jugular paragangliomas were the most commonly treated tumors. Tumor control was achieved in nearly 96% of operated patients with minimal cranial nerve morbidity. Surgery is curative in most cases and should be considered as frontline treatment modality in experienced hands for younger patients, hereditary and secretory tumors. Cranial nerve dysfunction associated with tumor encasement is a negative prognostic factor for both surgery and radiotherapy. Multifocal tumors and metastasis are difficult to treat, even with early detection using genetic analysis. Detecting malignancy in HNPGLs is challenging due to the lack of histomorphological criteria; therefore, limited lymph node dissection should be considered, even in the absence of clinical and radiological signs of metastasis in carotid body, vagal, and jugular paragangliomas.

DESCRIPTION OF CLINICAL AND PARACLINICAL RESULTS OF LAPAROSCOPIC RETROPERITONEAL RESECTION OF BENIGN ADRENAL ADENOMA AT VIET DUC FRIENDSHIP HOSPITAL IN 2018

The study was conducted with objectives: Describe the clinical and paraclinical results of laparoscopic retroperitoneal resection of benign adrenal tumors at Viet Duc Friendship Hospital in 2018 on 67 patients diagnosed as benign adrenal tumor and was treated with retroperitoneal endoscopic surgery at Viet Duc hospital in 2018. The study design was retrospective. Results: The clinical syndromes of the patients were mainly Cushing's syndrome (3%), Conn's syndrome (29,8%), Pheochromocytome (17,9%) and 49,3% of patients with nonsecretory tumors. . Specific biochemical tests: hypercortisolemia (4,48%); increased blood catecholamines (16,42%) and increased urinary catecholamines (14,93%). Ultrasound: The average tumor size was 20.37 ± 16.05mm; The diagnostic sensitivity was 74,6%. CT or MRI: The mean tumor size was 26.63 ± 12.7mm; highly sensitive and is the gold standard on diagnostic imaging. Conclusion: The clinical and laboratory results of patients undergoing laparoscopic resection of benign adrenal tumors are highly sensitive.

S2960 Incidental Diagnosis of a Rare Metastatic Neuroendocrine Breast Cancer During Routine Screening Mammogram Which Eventually Revealed Primary Small Intestine Carcinoid Tumor

Introduction: Neuroendocrine neoplasms (NENs), defined as epithelial neoplasms with predominant neuroendocrine differentiation, typically occur in the pancreas, tubular gastrointestinal tract, or lung with or without secretory syndromes. Neuroendocrine breast carcinoma (NEBC) is a rare tumor which accounts for 1-2% of all invasive breast carcinoma. When NENs are found on a biopsy of a breast nodule it is considered either primary NEBC or metastatic if another primary organ is discovered. NEBC usually presents as a breast lump on exam without neuroendocrine symptoms. Therefore, making a diagnosis of NEBC prior to biopsy is difficult. We present a case of an incidentaloma discovered on routine mammography that turned out to be NEBC on biopsy and further evaluation discovered an asymptomatic primary small bowel NEN, thus confirming the breast as metastatic NEBC. Case Description/Methods: A routine mammogram in 60 yo. female revealed an abnormal 4 mm solid nodule in the right breast. A core needle biopsy found a low grade, well differentiated NEN, with positive staining for synaptophysin and chromogranin. She felt well and denied any symptoms. Medical history was notable for immune hepatitis with liver cirrhosis managed by her hepatologist. An abdominal MRI/MRCP revealed thickening in the small bowel wall consistent with a primary carcinoid tumor and demonstrated hepatic surface metastases. Plasma and 24 hr. urine 5 HIAA and chromogranin A plasma levels were normal. A lumpectomy was performed and therapy with lanreotide i.m. monthly injections begun. At 6 months follow-up, she remains asymptomatic, with stable disease. Discussion: Metastasis to the breast from a primary NEN is infrequently reported in the literature. If discovered in a patient without a history of known primary NEN in another organ, imaging is warranted to seek asymptomatic and non-secreting tumors elsewhere, as in the GI tract of our patient. No proven effective chemotherapy or RT for either primary or metastatic NEBC exists. No proven effective chemotherapy regimen exists for NENs, but therapy with long-acting somatostatin analogs are the preferred first-line option for NENs associated with several hormonal syndromes as well as for growth control in well-differentiated unresectable or metastatic gastroenteropancreatic NENs as in our case.

Adrenal ganglioneuromas: a retrospective multicentric study of 104 cases from the COMETE network.

Adrenal ganglioneuromas are rare, differentiated, neuroblastic tumors that originate from the peripheral sympathetic nervous system. Because of their rarity, information is limited, derived from small cases series. Our objective was to characterize this tumor and provide help for its management. A retrospective multicenter analysis of adrenal ganglioneuromas from 20 French centers belonging to the COMETE network and one Belgian center. Among the 104 cases identified, 59.6% were women (n = 62/104), median age at diagnosis was 29 years, with 24 pediatric cases. 60.6% (n = 63/104) were incidentalomas. Ganglioneuromas were non-secreting tumors in 90.8% of cases (n = 89/98), whereas the preoperative hormonal evaluation was indeterminate for 9.2% of patients (n = 9/98). CT imaging, performed on 96 patients, revealed large tumors (median diameter of 50 mm) with a non-contrast density > 10 Hounsfield units in 98.1% (n = 52/53) and calcifications in 64.6% of cases (n = 31/48). Increased uptake on 123I-MIBG scintigraphy and 18F-FDG-PET/CT was observed in 26.7% (n = 8/30) and 42.2% (n = 19/45) of the tumors, respectively. All 104 patients underwent surgery. No recurrence was observed among the 42 patients who had an imaging follow-up (mean 29.6 months, median 18 months (4-156)). Adrenal ganglioneuromas are large tumors, mostly nonfunctioning, without benign imaging features. Although the duration of follow-up was limited in our series, no recurrence was identified. A review of the literature confirms the absence of postoperative recurrence. Based on all available data, in the absence of special circumstances (genetic form, uncertain histological diagnosis), long-term follow-up is not necessary after complete surgery for patients with an adrenal ganglioneuroma.

EVALUATING THE RESULTS OF LAPAROSCOPIC SURGERY CUT BENIGN ADRENAL TUMOR RETROPERITONEAL ROAD IN VIETNAM GERMAN FRIENDSHIP HOSPITAL IN 2018

A descriptive regression study evaluating surgical outcomes of 67 patients diagnosed with benign adrenal tumors and treated with retroperitoneal laparoscopic surgery at Viet Duc Hospital in 2018.Results: For Surgical subjects mainly belonged to ASA group I or II, the average surgery time of the study subjects was 71.04 ± 23.50. In the group of pathological tumors, the surgical time of the group of patients with non-secretory tumours was the longest (74.2 ± 23.2 minutes); The operative time of the group of patients with Conn’s disease was the shortest (67.5 ± 26.1 minutes). The averageoperating time of the group of tumors from 1-10mm in size was 60 minutes. 66/67 patients with total thoracic resection due to tumor accounted for 98.5%. Only 1/67 patients with selective resectionof TTT. Out of a total of 67 patients studied, there were 2 patients with subcutaneous emphysema, accounting for 3.0%.

A Prospective Evaluation of Quality of Life in Patients Undergoing Extended Endoscopic Endonasal Surgery for Benign Pituitary Gland Lesion.

Introduction Endoscopic endonasal surgery (EES) has become the preferred approach for pituitary tumor resection. Nevertheless, research on quality of life related to pituitary adenoma surgery is scarce. Objective The aim of the study is to evaluate short-term quality of life in patients after endoscopic endonasal resection of pituitary tumors and to find predictors for poor quality of life (QOL) outcome. Materials and Methods A prospective cohort study was conducted, including all patients who underwent EES for pituitary tumors in a tertiary medical referral center. Recruited patients completed the Anterior Skull Base Disease-Specific QOL (ASBS-Q) questionnaire and the Sinonasal Outcome Test 22 (SNOT-22) questionnaire before surgery, 2 and 4 to 6 months after surgery. Demographic and clinical data was collected. Results Our study included 49 patients. The overall ASBS-Q scores significantly improved 4 to 6 months after surgery (4.46 vs. 4.2, p < 0.05). We found a significant improvement in QOL related to emotional state 2 months post surgery (4.41 vs. 3.87, p < 0.05), which became borderline significant 4 to 6 months post surgery. There was a significant improvement in pain (4.5 vs. 4.08, p < 0.05) and vitality (4.43 vs. 4.16, p < 0.05) domains 4 to 6 months post surgery. SNOT-22 scores did not change significantly postoperatively. Factors such as secreting and non-secreting tumors, tumor size, intraoperative cerebrospinal fluid leak, gross tumor resection, endocrine remission, and the use of nasoseptal flap reconstruction did not have a significant effect on QOL. Conclusion We found that patients after EES reported improved QOL 4 to 6 months post surgery. Specific improvement was noted in the QOL related to pain and vitality.

A Rare Case of Metastatic Transformation to Pituitary Carcinoma

Background: Pituitary carcinomas are rare and comprise less than 0.5% of pituitary tumors and frequently arise from previously resected and/or radiated infiltrating adenomas.1 Radiation therapy is used to prevent regrowth but does not have significant data supporting improved prognosis.2 True pituitary carcinomas requires the presence of craniospinal and/or systemic metastases. Here, we present a case of benign pituitary adenoma that had progressed to a metastatic tumor and responded to radiation therapy. Clinical Case: Our patient was first diagnosed in 1982 with a pituitary mass after undergoing an MRI due to right eye blindness. He ultimately had a transcranial resection of the tumor and post-operative external radiation to residual tumor. Final pathology was consistent with a nonsecretory tumor. Additionally, he was diagnosed with panhypopituitarism and he was treated accordingly. Due to barriers of patient adherence, only one post-operative MRI was obtained, and he was lost to endocrine follow up. In 2007, he was again referred to endocrinology for new vision loss in the left eye. An MRI revealed a 3.0 x 3.8 x 4.4 cm tumor that compressed the optic chiasm. Additionally, multiple lesions were seen within the dural and leptomeningeal extra-axial areas including the largest left frontal lesion of 2.4 cm and a 1.7 cm lesion posterior to the cord within the foramen magnum. The patient underwent endonasal transsphenoidal resection of the sellar mass and biopsy of the frontal lesion in October 2007. Pathology showed pituitary adenocarcinoma in both the sellar and frontal lesions and immunoperoxidase staining was negative for all hormones. He completed salvage radiation to the sellar and craniospinal lesions in December 2007. MRI scans completed 7 months later showed stable residual pituitary and metastatic lesions. The patient received 6-month MRI screening exams for the first 2 years and then annually thereafter, as well as PET scanning, without significant progression or new metastatic lesions. Conclusion: We present a case of benign pituitary adenoma which transformed following resection to a malignant tumor with metastatic lesions in the brain and spinal cord that was treated with radiation with relative success. Despite the often-aggressive nature of these metastatic tumors, the patient has experienced a successful treatment course with minimal side effects and without progression 13 years after initial treatment of the metastatic pituitary carcinoma.

Clinical prognostic factors in pediatric adrenocortical tumors: A meta-analysis.

Pediatric adrenocortical tumors (ACT) are rare and sometimes aggressive malignancies, but there is no consensus on the outcome predictors in children. A systematic search of MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies from 1994 to 2020 about pediatric ACT was performed. In 42 studies, 1006 patients, aged 0-18years, were included. The meta-analyses resulted in the following predictors of better outcome: age <4years (P<.00001), nonsecreting tumors (P=.004), complete surgical resection (P<.00001), tumor volume (P<.0001), tumor weight (P<.00001), tumor maximum diameter (P=.0009), and Stage I disease (P<.00001). Moreover, patients affected by Cushing syndrome showed a worse outcome (P<.0001). International prospective studies should be implemented to standardize clinical prognostic factors evaluation, together with pathological scores, in the stratification of pediatric ACT.

Endonasal endoscopic versus microscopic transsphenoidal surgery in pituitary tumors among the young: A comparative study & meta-analysis

PurposeThe transsphenoidal approach presents unique challenges in young, with scanty literature. This study compares the outcome of pituitary tumors among young in our center between endoscopic(EES) and microscopic(MTS) transsphenoidal surgery, with a meta-analysis. MethodsPatients within 20 years were studied for their surgical approach to a favorable outcome of endocrine remission (ER) (functioning) or Gross/Near-Total resection (nonfunctioning), besides the need for retreatment. Relevant studies were pooled and analyzed according to PRISMA guidelines. ResultsOut of 64 young patients with pituitary tumors, 48 underwent transsphenoidal surgery using MTS(33) or EES(15). Of these, 21, 14, 5, and 8 had Cushing's, somatotropinomas, prolactinomas, and non-secreting tumors, respectively. Mean symptom duration was 28months, with weight gain(50 %) and visual complaints(29 %) most prevalent. Hypogonadism(21 %) was the most frequent endocrinopathy. The mean tumor volume was 3.8 cm3. Over mean follow-up of 4.4years, favorable outcome was significantly higher after EES than MTS(78.6 % vs. 46.7 %)(odds ratio 4.18, p = 0.05). EES's better outcome was homogeneous across subgroups of age and tumor type, with no significant subgroup difference. Symptom duration was significantly higher among those who required retreatment(p = 0.05), while ER had a non-significant association with tumor volume(p = 0.07). Overall, 40 %, 27 %, 17 %, and 8% were on hydrocortisone, thyroxine, sex hormone, and desmopressin, respectively, at follow-up with no significant difference between EES and MTS. In pooled analysis of literature, both favorable outcome(74 % vs. 48 %,p = 0.02) and retreatment rate(8% vs. 37 %,p = 0.004) were significantly better with EES than MTS. ConclusionAmong young patients with pituitary tumors, the favorable outcome and retreatment rates are better with endonasal endoscopy and associated with symptom duration and tumor volume.

GCT-30. TREATMENT OF PRIMARY INTRACRANIAL GERM CELL TUMORS: SINGLE INSTITUTION EXPERIENCE OF 74 CASES WITHOUT HISTOLOGICAL CONFIRMATION

BACKGROUND AND OBJECTIVEPrimary intracranial germ cell tumors (PIGCTs) are a group of heterogeneous tumors. It is very difficult to treat those patients without pathological diagnosis. This study retrospectively analyzed the clinical data and outcomes of patients with clinically diagnosed (without histologically confirmed) PIGCTs in SunYat-sen University Cancer Center.METHODSPatients who were clinically diagnosed as PIGCTs without histological diagnosis through surgical resection or biopsy were included in this study. Patients were analyzed for clinical characteristics, treatment patterns, outcomes and adverse effects.RESULTSFrom May 2002 to July 2014, 74 patients clinically diagnosed with PIGCTs received chemotherapy and/or radiotherapy at the Sun Yat-sen University Cancer Center. The median age was 16.5 years old (4–45 years old, majority was teenagers). The most of tumors were found in male, and located in the pineal and suprasellar regions. When the patients were grouped into diagnostic chemotherapy group (57 cases), diagnostic radiotherapy group (5 cases) and gamma knife radiosurgery group (12 cases) based on their initial anti-tumor therapy. The 5-year survival rates were 84.3%, 75.0% and 75.0%, respectively. There was a trend that the chemotherapy group got a better survival. Patients were allocated to secretory tumor group (49 cases) and non-secretory tumor group (25 cases) based on their levels of tumor makers (α-FP and β-hCG). The 5- year survival rates were 80% and 77.8% (P value = 0.966), respectively.CONCLUSIONClinical diagnosed PIGCT (without histological confirmation) patients may obtain good responses when receiving comprehensive treatments of chemotherapy combined with radiotherapy.

Prevalence and Incidence of Atrial Fibrillation in a Large Cohort of Adrenal Incidentalomas: A Long-Term Study.

Chronic glucocorticoids excess leads to morphological and functional cardiac alterations, a substrate for arrhythmias. Autonomous cortisol secretion (ACS) in adrenal incidentalomas is a model of chronic endogenous hypercortisolism. To investigate prevalence and incidence of atrial fibrillation (AF) in a large cohort of patients with ACS. Retrospective study. University hospital. Patients evaluated between 1990 and 2018 for adrenal incidentalomas (n = 632), without pheochromocytoma, primary aldosteronism, Cushing syndrome, congenital adrenal hyperplasia, and adrenal malignancy. Cortisol after 1-mg dexamethasone suppression test < or > 50 nmol/L defined nonsecreting tumors (NST) (n = 420) and ACS (n = 212), respectively. Assessment of AF at baseline (n = 632) and during a median follow-up of 7.7 years retrospectively (NST, n = 249; ACS, n = 108). Comparison with general population. Prevalence and incidence of AF. AF prevalence was higher in patients with ACS (8.5%) than NST (3.1%, P = 0.003) and the general population (1.7%; P < 0.001 vs ACS, P = 0.034 vs NST). The age-adjusted rate ratio to the general population was 1.0 for NST and 2.6 for ACS. AF was associated with ACS (odds ratio, 2.40; 95% confidence interval [CI], 1.07-5.39; P = 0.035). The proportion of patients with AF at last evaluation was higher in ACS (20.0%) than NST (11.9%; P = 0.026). ACS showed a higher risk of incident AF than NST (hazard ratio, 2.95; 95% CI, 1.27-6.86; P = 0.012), which was associated with post-dexamethasone cortisol (hazard ratio, 1.15; 95% CI, 1.07-1.24; P < 0.001), independently of known contributing factors. Patients with adrenal incidentalomas and ACS are at risk of AF. Electrocardiogram monitoring may be recommended during follow-up.