Thoracic Mass Research Articles

A Rare Cause of Recurrent Hypoglycemia - The “Big-IGF-II” Factor

Abstract Introduction: Hormone secreting tumors are a rare cause of hypoglycemia. Here we present an interesting case of paraneoplastic phenomenon resulting in a non-islet cell tumor causing hypoglycemia (NICTH) from a solitary fibrous tumor (SFT). Clinical Case: An 83-year-old female with a history of hypertension presented with recurrent episodes of lightheadedness and falls for one month. Review of systems was negative except for insignificant weight loss. Her vital signs and physical examination were unremarkable. Only significant finding on initial blood work was a blood glucose level of 30 mg/dL (n: >70mg/dL). Additional work-up of hypoglycemia revealed a low serum free insulin level 0.6 IU/mL (n:1.5 - 14.9 IU/mL), low proinsulin <4 pmol/L (n: <18.8 pmol/L), low C-peptide level 0.14 ng/mL (n: 0.80-3.85 ng/mL), low serum insulin-like growth factor-I (IGF-I) 33 ng/ml (n:34-246 ng/ml) and elevated IGF-II levels 370 ng/mL (n:47-350 ng/mL). Blood sulfonylurea and insulin antibodies screen were negative, ACTH and morning cortisol levels were within normal limits. A CT scan of chest, abdomen and pelvis for neoplastic workup demonstrated a large heterogeneous mass within the lower lobe of the right lung. A CT-guided biopsy disclosed spindle cell neoplasm, positive for signal transducer and activator of transcription-6 (STAT6) and CD34. Reverse transcription-polymerase chain reaction (RT-PCR) confirmed the NGFI-A binding protein 2 (NAB2) and STAT6 fusion which is specific for solitary fibrous tumor (SFT). She underwent surgical resection of the thoracic mass which resulted in complete resolution of her symptoms. Clinical Lessons: NICTH is a paraneoplastic syndrome associated with various benign and malignant tumors including SFT, hemangiopericytoma and metastatic hepatocellular carcinoma to name a few. Our case is an example of NICTH associated with SFT called Doege-Potter syndrome. The prevalence of hypoglycemia is seen in less than 5% of patients with SFT. It is often secondary to ectopic overexpression of unprocessed precursor ‘big’ IGF-II molecule, which binds to IGF-I receptor, resulting in tumorigenesis. An IGF-II/IGF-1 ratio is a sensitive marker and a value of more than 10 suggests excess production of IGF-II and is likely the cause of NICTH. Physicians should be cognizant of NICTH, especially in non-diabetic, otherwise healthy individuals in whom preliminary workup for hypoglycemia is negative and should look to identify any potential tumor as the likely cause. Surgical treatment is often the only curative option. Frequent meals, dextrose infusion, glucagon and growth hormone are bridging modalities until definitive surgical treatment.

English

BACKGROUND Intrathoracic mass is a problem faced by the clinicians worldwide which is difficult to diagnose correctly. The mediastinum although a small anatomic compartment, the pathologic processes found in it are diverse. Imaging techniques are very helpful for detection and diagnosing these lesions. The current study was conducted for assessing the diagnostic accuracy and efficacy of computed tomography (CT) guided fine-needle aspiration cytology (FNAC) in the evaluation of various thoracic mass lesions and also to compare the histopathological findings of CT guided FNAC for evaluation of the type of thoracic lesions & its prognosis. METHODS Data for the study was collected from 50 patients referred to the Department of Pathology, in a tertiary care hospital for 16 months from December 2015 to March 2017. RESULTS Among 50 patients with intrathoracic mass, there were 37 men (74 %) and 13 women (26 %). The age of patients with intrathoracic masses in this study varied from 31 to 80 years. The mean age was 55 years. Thirty-two patients were found to have malignant tumours and 18 cases were of benign aetiology. The highest frequency of cases in this study were of adenocarcinoma followed by squamous cell carcinoma. CONCLUSIONS Computed tomography (CT) guided fine-needle aspiration cytology (FNAC) is a cost effective, simple, safe, highly sensitive & specific procedure with high diagnostic accuracy in the diagnosis of intrathoracic mass lesions. It has few complications, rarely requiring active management. KEY WORDS Intrathoracic Mass, Fine-Needle Aspiration Cytology, Pneumothorax, Pneumonectomy, Thoracic Mass Lesions

Bilateral Subscapular Masses Diagnosed as Elastofibroma Dorsi

Elastofibroma dorsi (ED) is a benign soft tissue poorly circumscribed tumor of the chest wall. ED is characterized by hyperproliferation of fibroblastic tissue. The tumor is composed of collagen and coarse enlarged elastic fibers. The pathogenesis of the lesion is multifactorial. It clinically manifests as a palpable tumor in the infrascapular or subscapular region with occasional pain and discomfort, especially during mobilization of the shoulder. We report a case of a 70-year-old manual worker male with chronic thoracic pain and palpable bilateral symmetrical dorsal subscapular masses. Magnetic resonance showed the presence of bilateral soft tissue tumors and trucut biopsy showed ED. The lesions were completely surgically excised.

Perioperative, protective use of extracorporeal membrane oxygenation in complex thoracic surgery.

Extracorporeal membrane oxygenation (ECMO) is increasingly used in patients undergoing complex thoracic surgical procedures. However, studies reporting the clinical outcomes of these patients are limited to case reports, without real consensus. Our aim was to evaluate the perioperative use of ECMO as respiratory and/or circulatory support in thoracic surgery: indications, benefits, and perioperative management. Between May 2013 and December 2018, we reviewed the clinical data of 15 patients (11 males and 4 females; mean age: 47 years old; range, 25-73 years) undergoing ECMO-assisted thoracic surgery in our hospital. Of the 15 patients, 10 cases received peripheral veno-arterial (VA) ECMO and five cases received veno-venous (VV) ECMO. Indications for ECMO were pulmonary transplantation with hard-to-maintain oxygenation (n = 5), traumatic main bronchial rupture (n = 2), traumatic lung injury (n = 1), airway tumor leading to severe airway stenosis (n = 2), huge thoracic mass infiltrated vena cava (n = 5). The ECMO duration was 1-51 hours. All patients were successfully extubated and weaned from ECMO postoperatively. The main complications were hemorrhage (26.7%), infection (33.3%), acute hepatic dysfunction (33.3%), and venous thrombosis (26.7%). There was only one hospital death and postoperative one-year survival rate was 86%. Our experience indicates that ECMO is a feasible method for complex trachea-bronchial surgery, huge thoracic mass excision and lung transplantation, and the ECMO-related risks may be justified. With further accumulation of experience with ECMO, a more sophisticated protocol for management of critical airway or heart failure problems in thoracic surgeries can be derived.

SMARCA4-deficient thoracic sarcoma revealed by metastasis to the small intestine: a diagnostic dilemma

SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. We present the case of a 44-year-old man who underwent a surgery for a perforated small intestine. Compued tomography scan revealed a tissular mediastino–pulmonary mass.Histopathological examination of the intestinal mass shown a malignant tumour with a typical epithelioid and rhabdoid cells, numerous mitoses and large necrosis. A large panel of immunohistochemistry revealed loss of SMARCA4 and SMARCA2 and allowed the diagnosis of SMARCA4-DTS. It is important to consider SMARCA4-deficient thoracic sarcoma in the differential diagnosis of tumours showing suggestive morphologic features in patients of all ages, especially in the case of metastasis associated with thoracic mass.

Diagnostic utility of rapid on-site evaluation of endobronchial ultrasound-guided transbronchial needle aspiration samples: A study in a region of high tuberculosis burden.

Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) has emerged as a very useful tool for precise diagnosis of thoracic lesions. However, the procedure sometimes has a poor yield. Rapid on-site evaluation (ROSE) in EBUS-TBNA has been shown to be helpful to improve its diagnostic yield and minimise the need for repeat procedures. Most of the studies that have evaluated the utility of ROSE have focused on malignant lesions; however, it is important to understand its utility in benign lesions, particularly in a country with a high tuberculosis burden. This study was undertaken to evaluate the utility of ROSE in EBUS-TBNA for the diagnosis of patients presenting with mediastinal lymphadenopathy and/or thoracic mass for benign and malignant pathologies. This was a prospective study including 47 patients who underwent EBUS-TBNA for thoracic lesions (lung masses and hilar/mediastinal lymphadenopathy) over a 10-month period at a single tertiary care centre. In addition to ROSE and definitive cytology, the aspirated material was also sent for cell block and microbiological studies. Endobronchial/transthoracic biopsy was conducted for histopathological evaluation (HPE) where required, at the discretion of the clinician. ROSE diagnosis was then compared with the final diagnosis. Of the 47 patients examined using ROSE, granulomas were observed in 33 cases, non-specific inflammation in six cases, and malignancy in five cases; one case was inconclusive and the sample was found to be inadequate in two cases. The ROSE assessment matched the final diagnosis in 84.45% patients. ROSE is a simple procedure that is able to provide an immediate and accurate assessment of adequacy and has a significant yield, enabling a preliminary diagnosis to be made in both benign and malignant samples.

Thoracic Multidetector Computed Tomography Evaluation of Inflammatory Myofibroblastic Tumor of the Lung in Pediatric Patients in the Era of Modern Diagnosis.

The purpose of this study was to investigate the characteristic thoracic multidetector computed tomography (MDCT) findings of pathologically proven inflammatory myofibroblastic tumor (IMT) of the lung in children in the era of modern understanding based on refined pathologic diagnosis. All pediatric patients (age 18 y and above) with a known pathologic diagnosis of IMT of the lung who underwent thoracic MDCT studies from May 2008 to December 2020 were included. Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of abnormalities in the lung (nodule, mass, cyst, ground-glass opacity, consolidation), pleura (pleural effusion, pneumothorax), and mediastinum and hilum (lymphadenopathy). When a lung abnormality was present, the number, size, composition (solid, cystic, or combination of both), location (laterality, lobar distribution, and intraparenchymal vs. pleural-based), borders (well-circumscribed vs. ill-defined), the presence and type of associated calcification (punctate, dense, curvilinear, or flocculent), the presence of associated cavitation, contrast enhancement pattern (homogeneous, heterogenous, central, or peripheral), and other associated findings (neural foramen involvement, anomalous vessels, mass effect, and invasion of adjacent thoracic structures) were also evaluated. Interobserver agreement between 2 independent reviewers was evaluated with κ statistics. In all, 12 thoracic MDCT studies from 12 individual pediatric patients (5 males [42%] and 7 females [58%]; mean age: 9.9 y; SD: 4.4 y; range: 2 to 16 y) comprised the final study population. All 12 thoracic MDCT studies (100%) were performed with intravenous contrast. The most frequent MDCT finding of IMT of the lung in children is a solitary (92%), pleural-based (83%), well-circumscribed (100%), solid (92%) mass with heterogenous contrast enhancement (100%), often with dense calcification (50%), which occurred in both lungs and all lobes with similar frequency. No pleural abnormality (pleural effusion, pneumothorax) or mediastinal abnormality (lymphadenopathy) was detected. In addition, although mass effect on adjacent thoracic structures was frequently seen (42%), no invasion, neural foramen involvement, or associated anomalous vessels was identified. There was excellent interobserver κ agreement between 2 independent reviewers for detecting abnormalities on thoracic MDCT studies (κ>0.95). IMT of the lung in children typically presents as a solitary, pleural-based, well-circumscribed, solid mass with heterogenous contrast enhancement, often with dense calcification, without significant laterality or lobar preference. In addition, pleural or mediastinal abnormalities are characteristically absent. These notable MDCT attributes of IMT of the lung are an important and novel finding, with great potential to help differentiate pediatric IMT of the lung from other thoracic masses in children.

A Large Intrathoracic Mass in A Patient With Neurofibromatosis Type 1

Background and Importance: Dural ectasia is circumferential extension or dilatation of the dural sac, it is commonly associated with Neurofibromatosis type 1 (NF1). Because NF1 is associated with a high likelihood of tumor formation, it is critical to distinguish it from other posterior mediastinal cancers such as neurofibroma, neuroblastoma, and ganglioneuroma. Scoliosis is the most frequently observed feature in NF1 patients with spinal deformities. Moreover, there are several distinctive radiographic features, including dural ectasia, defective pedicles, and spondylolisthesis, which are relatively less common in these patients. Surgery may be performed for stabilization of the spine. Case Presentation: The present study reports on a 62-year-old female with neurofibromatosis type 1 who was referred for an unusual chest X-ray and chest Computed Tomography (CT) revealing a thoracic mass while she was hospitalized and under observation for dyspnea. The chest X-ray film showed homogenous opacity with a well-defined margin in the right apex. The chest CT showed an enlarged intervertebral foramen and defected vertebral arch around the mass and scoliosis. Although the pre-operative diagnosis was dumbbell type neurinoma, the tumor was found to be a protrusion of dura mater with spinal fluid out of the spinal canal. Part of the wall was excised, the residual opening was repaired, and surgical stabilization was performed. Conclusion: Retrospectively, magnetic resonance imaging showed the characteristics of thoracic dural ectasia and anterior meningocele, which, in an asymptomatic case, require regular radiographic follow-ups. Surgical intervention is an alternative for patients with spinal deformities or symptomatic patients due to the adverse effects of spinal cord compression and mediastinal structures.

Transdiaphragmatic Intercostal Hernia-An Unusual Hepatic Injury After a Car Accident: A Case Report and Review of the Literature.

Transdiaphragmatic intercostal hernia, in which the abdominal contents of the hernia protrude through the diaphragm and the thoracic wall defect. is a very rare type of hernia with only a few cases having been reported in the literature. That type of hernia is usually manifested in male patients after trauma, penetrating or blunt. It is frequently presented with a palpable thoracic mass and pain. The indicated treatment is surgery. We present the case of a 60-year-old female admitted to the hospital after a car accident and suffered multiple rib fractures (6th, 7th, 8th right ribs / 7th, 8th, 9th left ribs), as well as flail thorax, hemothorax bilaterally, left subcutaneous emphysema and swelling of soft tissues of the right lateral thoracoabdominal wall. CT scan revealed herniation of hepatic parenchyma and intestinal loops into the thorax. The patient was treated surgically, and his postoperative course was uneventful. We also review the relevant literature concerning this transdiaphragmatic, intercostal hernia and identify 42 cases. Transdiaphragmatic intercostal hernia is a rare condition, usually manifested in male patients after trauma, penetrating or blunt. It is frequently presented with a palpable thoracic mass and pain. The indicated treatment is surgery.

Clinical Value of CT-Guided Fine Needle Aspiration and Tissue-Core Biopsy of Thoracic Masses in the Dog and Cat.

Simple SummaryDiagnostic imaging is of paramount importance in the diagnosis of thoracic lesions. Radiology has traditionally been considered the diagnostic procedure of choice for these diseases in addition to a correct cytological and histopathologic diagnosis. In human medicine, Computed Tomography (CT) and CT-guided biopsy are used in the presence of lesions which are not adequately diagnosed with other procedures. In the present study, thoracic lesions from 52 dogs and 10 cats of different sex, breed and size underwent both CT-guided fine-needle aspiration (FNAB) and tissue-core biopsy (TCB). In this study, 59 of 62 histopathological samples were diagnostic (95.2%). Cytology was diagnostic in 43 of 62 samples (69.4%). General accuracy for FNAB and TCB were 67.7% and 95.2%, respectively. Combining the two techniques, the overall mean accuracy for diagnosis was 98.4%. CT-guided FNAB cytology can be considered a useful and reliable technique, especially for small lesions or lesions located close to vital organs and therefore dangerous to biopsy in any other way.Diagnosis of thoracic lesions on the basis of history and physical examination is often challenging. Diagnostic imaging is therefore of paramount importance in this field. Radiology has traditionally been considered the diagnostic procedure of choice for these diseases. Nevertheless, it is often not possible to differentiate inflammatory/infectious lesions from neoplastic diseases. A correct cytological and histopathologic diagnosis is therefore needed for an accurate diagnosis and subsequent prognostic and therapeutic approach. In human medicine, Computed Tomography (CT) and CT-guided biopsy are used in the presence of lesions which are not adequately diagnosed with other procedures. In the present study, thoracic lesions from 52 dogs and 10 cats of different sex, breed and size underwent both CT-guided fine-needle aspiration (FNAB) and tissue-core biopsy (TCB). Clinical examination, hematobiochemical analysis and chest radiography were performed on all animals. In this study, 59 of 62 histopathological samples were diagnostic (95.2%). Cytology was diagnostic in 43 of 62 samples (69.4%). General sensitivity, accuracy and PPV for FNAB and TCB were 67.7%, 67.7% and 100% and 96.7%, 95.2% and 98.3%, respectively. Combining the two techniques, the overall mean accuracy for diagnosis was 98.4%. Nineteen of 62 cases showed complications (30.6%). Mild pneumothorax was seen in 16 cases, whereas mild hemorrhage occurred in three cases. No major complications were encountered. CT-guided FNAB cytology can be considered a useful and reliable technique, especially for small lesions or lesions located close to vital organs and therefore dangerous to biopsy in other way.

Thoracic mass lesion in a 14-year-old girl: Questions

Thoracic mass lesion in a 14-year-old girl: Questions

Thoracic mass lesion in a 14-year-old girl: Answers.

Thoracic mass lesion in a 14-year-old girl: Answers.

A refined method to monitor arousal from hibernation in the European hamster

BackgroundHibernation is a physiological and behavioural adaptation that permits survival during periods of reduced food availability and extreme environmental temperatures. This is achieved through cycles of metabolic depression and reduced body temperature (torpor) and rewarming (arousal). Rewarming from torpor is achieved through the activation of brown adipose tissue (BAT) associated with a rapid increase in ventilation frequency. Here, we studied the rate of rewarming in the European hamster (Cricetus cricetus) by measuring both BAT temperature, core body temperature and ventilation frequency.ResultsTemperature was monitored in parallel in the BAT (IPTT tags) and peritoneal cavity (iButtons) during hibernation torpor-arousal cycling. We found that increases in brown fat temperature preceded core body temperature rises by approximately 48 min, with a maximum re-warming rate of 20.9℃*h-1. Re-warming was accompanied by a significant increase in ventilation frequency. The rate of rewarming was slowed by the presence of a spontaneous thoracic mass in one of our animals. Core body temperature re-warming was reduced by 6.2℃*h-1 and BAT rewarming by 12℃*h-1. Ventilation frequency was increased by 77% during re-warming in the affected animal compared to a healthy animal. Inspection of the position and size of the mass indicated it was obstructing the lungs and heart.ConclusionsWe have used a minimally invasive method to monitor BAT temperature during arousal from hibernation illustrating BAT re-warming significantly precedes core body temperature re-warming, informing future study design on arousal from hibernation. We also showed compromised re-warming from hibernation in an animal with a mass obstructing the lungs and heart, likely leading to inefficient ventilation and circulation.

CT guided biopsy of lung lesions

Introduction: Computed Tomography (CT) Biopsy is a corrigible coruscate procedure that corrected the discrepancy of the results between final biopsy and unguided Biopsy. CT guidance permits biopsy of nearly all lesions regardless of size and position. Recognition of the accuracy of biopsy and simple methods of treating pneumothorax has brought this method within the reach of most radiologist and pathologist. Infections and other neoplastic process may be proven by this method. In cases of malignancy of the lungs, cytopathological examination of material obtained by CT guided biopsy offers a quick and specific diagnosis which help clinicians implement appropriate anticancer measures like chemotherapy and radiotherapy. The present study is undertaken to determine the accuracy and efficacy of percutaneous biopsy in diagnosis of various lung lesions, and correlate the concordance with available results and available other methods. An interest to analyse the age, gender, topographic distribution and cytopathological diagnosis of thoracic mass lesions using CT guided biopsy is specially stepped in the current study. Aim: To assess the role of Computed tomography (CT) guided biopsy in the diagnosis of thoracic mass lesion and to analyse and compare the results with other studies. To evaluate the diagnostic accuracy and complication of Computed tomography (CT) guided biopsy in thoracic masses. To know the pathological spectrum of thoracic mass lesions and to correlate CT findings with cytopathological report. Methods: This Prospective study was done at Nizam’s Institute of medical sciences, Hyderabad, surrounded by thickly populated districts which provide surplus material for doing research work and is also fully equipped with all the required modes and means for researches and health care. This study was conducted totally at indoor site including both OPD and IPD males and females from both urban and rural exclusively adults. Fifty one cases having Lung masses both malignant and benign were selected and subjected to CT guided biopsy. Statistical analysis was carried out by Using SPSS soft ware version 12. Results: Majority of the patients with lung masses in present study were males who constituted 66.6% and the females were 33.4%. Minimum age was of 22yrs and maximum age was of 92 yrs from the patients of this study. The mean age is 57 yrs. The chief etiological factor for the initiation of the pathogenesis of lung masses was found to be smoking. Out of the total cases included for the study 49% were malignant and 37% were benign lung masses. The complications observed were very few only in 8 cases (15.8%) in total of 51 cases selected for the study. The pneumothorax was noticed in 13.7% and haemoptysis in 1.9%. Conclusion: This CT guided biopsy stands as fruitful topic for study will be to elucidate the diagnostic and prognostic procedures in chest. This procedure of diagnosis with prosaic complications like Pneumothorax or Haemothorax or Haemoptysis and with its mosaic results outstands and extends its scope of study.

Unusual Thoracic Tumours- A Concise Imaging Approach

Introduction: There are certain uncommon large thoracic masses sharing overlapping radiological features with commoner ones except few salient characteristic features. Diagnosing these uncommon tumours has practical implications for patient management and prognosis including obviating unnecessary surgeries. Aim: To demonstrate characteristic Computed Tomography (CT) features of large common and certain uncommon intra-thoracic masses Materials and Methods: A retrospective cross-sectional search was done in January 2020, from the archives of patients record in Radiology Department from December 2017 to January 2019 for intra-thoracic tumours. Those tumors or masses meeting inclusion criteria were analysed by two radiologists who were blinded about the histopathologic reports. A probable radiological diagnosis was made and it was later matched-up with the histopathologic reports to reach to a confirmed diagnosis. The unique CT features were separately tabulated and their p-value for a particular diagnosis was calculated using Fisher’s-exact test. Results: Out of total 23 cases studied, 12 bronchogenic carcinomas and 11 uncommon diagnoses including Solitary fibrous tumour, Synovial sarcoma, Pulmonary blastoma, Askin’s tumour, Schwannoma and Metastasis were found. Subtle rib erosion in Askin’s tumour (p-value=0.004), intra-tumoural vessels in Synovial sarcomas (p-value=0.0006) and intense enhancement in some non-bronchogenic tumours (p-value=0.0137) were found to be significantly associated features. Conclusion: When encountering an opaque hemithorax on chest radiograph and a large mass on CT, one should look for certain peculiar features of these unusual lung masses to suggest them as a differential diagnosis, as “Our eyes see only what our mind knows”.

Intrinsic sources of tachykinin-related peptide in the thoracic ganglion mass of the crab, Cancer borealis

Neuropeptides comprise the largest class of neural and neuroendocrine signaling molecules. Vertebrate tachykinins (TKs) and the structurally-related invertebrate tachykinin-related peptides (TRPs) together form the largest neuropeptide superfamily, with a number of conserved neural and neuroendocrine functions across species. Arthropods, including crustaceans, have provided many insights into neuropeptide signaling and function. Crustacean tachykinin-related peptide occurs in endocrine organs and cells and in two of the major crustacean CNS components, the supraoesophageal ganglion (“brain”) and the stomatogastric nervous system. However, little is known about TRP sources in the remaining major CNS component, the thoracic ganglion mass (TGM). To gain further insight into the function of this peptide, we aimed to identify intrinsic TRP sources in the TGM of the Jonah crab, Cancer borealis. We first adapted a clearing protocol to improve TRP immunoreactivity specifically in the TGM, which is a dense, fused mass of multiple ganglia in short-bodied crustaceans such as Cancer species of crabs. We verified that the clearing protocol avoided distortion of cell body morphology yet increased visibility of TRP immunoreactivity. Using confocal microscopy, we found TRP-immunoreactive (TRP-IR) axon tracts running the length of the TGM, TRP-IR neuropil in all ganglia, and approximately 110 TRP-IR somata distributed throughout the TGM, within and between ganglia. These somata likely represent both neural and neuroendocrine sources of TRP. Thus, there are many potential intrinsic sources of TRP in the TGM that are positioned to regulate behaviors such as food intake, locomotion, respiration, and reproduction.

Giant thoracic ganglioneuroma with lymph node infiltration of a child: a case report

BackgroundGanglioneuroma is a rare neurogenic tumor which could grow into massive size in the thorax with minimal or no symptoms. Resection of these tumors will be challenging in view of large size and vascular encasement. Here, we report resection of a thoracic ganglioneuroma in a child with infiltration of spinal canal and lymph node metastasis, which is a very rare occurrence.Case presentationA 3-year and 10-month-old girl presented with a left side thoracic mass with minimal symptoms and signs. After the initial imaging with ultrasound scan and cross-sectional imaging, she had a true cut biopsy of the lesion confirming the diagnosis of ganglioneuroma. On exploration, she had a paraspinal thoracic mass extending from level of arch of aorta down to diaphragm with evidence of tumor infiltration of the intercostal spaces and spinal canal in multiple vertebral levels. Pathologically, she had infiltrated lymph nodes which were encircling the lower thoracic aorta. The tumor was resected successfully, and she recovered with a good functional status.ConclusionThoracic ganglioneuroma has a good prognosis after complete resection even with spinal canal and lymph node infiltration. The resection could be arduous in case of massive size and vascular encasement; hence, benefit versus risk of complete resection should be carefully weighed.

Atypical thymoma in a horse: Diagnostic approach and application of an alternative histological classification system

SummaryThymoma is a rare condition in horses. This case of a 24‐year‐old Warmblood gelding that was presented with oedema and signs of vascular congestion of the left forelimb caused by a thoracic mass is described. The diagnostic work‐up included blood chemistry, cytology of pleural effusion, ultrasonography, radiography and the visualisation and collection of a tissue sample by thoracoscopy. Finally, post‐mortem findings and histopathology revealed thymic epithelial neoplasia with histomorphologic features previously unreported in horses. The unique mixture of spindle‐shaped and epithelioid tumour cells in combination with clear features of malignancy suggested the application of the Moran and Suster histological classification system for thymomas in humans (Moran and Suster, 2008, Curr. Treat. Options Oncol., 9, 288), which has not been reported in an equine case before and fits well to the present case.

EPCO-03. REPORT OF NTRK FUSION IN A PEDIATRIC PATIENT WITH SPINAL GANGLIOCYTOMA

Abstract BACKGROUND Gangliocytomas are rare WHO grade 1 central nervous system tumors of neuronal/ mixed neuronal-glial classification that most commonly occur in the cerebrum, favoring the temporal lobe. They are also reported in cerebellum in the setting of Lhermite-Duclos disease. Spinal gangliocytomas are exceedingly rare, constituting 3–10% of reported pediatric cases. Maximal safe surgical resection is the mainstay of therapy, as response to irradiation or traditional chemotherapy is variable. Given the rarity of these tumors, little is known about the molecular makeup or the role of targeted therapy in this population. CASE Our patient presented as a 7-year-old male with thorocolumbar scoliosis. A magnetic resonance imaging (MRI) of his spine incidentally demonstrated a heterogeneously enhancing, expansile solid and cystic intramedullary thoracic mass from T6 through T11. MRI brain did not show any intracranial mass. He subsequently underwent a laminectomy with laminoplasty for the intramedullary mass. Histopathologic analysis confirmed a WHO grade I gangliocytoma with moderately cellular abnormal central neural tissue composed of irregular clusters and cords of mature-appearing neurons in a coarse neuropil background. Because of the unusual location and presentation of his disease, molecular analysis including DNA and RNA sequencing was performed which revealed a somatic BCR-NTRK2 fusion. DISCUSSION NTRK gene fusions are known oncogenic drivers, reported with varying frequency in many pediatric and adult tumors. They are reported in < 1% of adult head and neck cancers but greater than 90% of congenital infantile fibrosarcomas. NTRK fusions are seen in pediatric brain tumors like infantile high grade gliomas, however they have not been previously reported in gangliocytoma. As TRK inhibitors are efficacious in treating a number of NTRK-rearranged malignancies, this novel finding provides a therapeutic option for NTRK fusion positive gangliocytoma where complete surgical resection is not safely possible.

Posterior Spinal Surgery for Extradural Spinal Metastasis of Round Cell Tumor in the Lower Thoracal Region

Spinal cord dysfunction caused by a spinal tumor is not always easy to treat since the tumor itself possesses variably etiology and low ability of nerve impairment to recover. The most common extradural tumor is derived from metastases, followed by other pathology such as plasmacytoma, lymphoma, etc. Since histopathologically they may present as round cell tumors, pre-operative data and intraoperative findings may help the clinician to establish appropriate diagnosis. This case report aims to describe the role of posterior spinal surgery and the reasoning of the selected approach in the treatment of spinal metastasis mimicking plasmacytoma in the lower thoracal region. We report 56 years old male who has myelopathy symptoms caused by lower thoracal extradural mass lesion, which histopathological study shows round cell tumor findings. Laminectomy and posterior stabilization were undertaken, and additional rotational flap was made to close sacral decubitus ulcer defect. Axial pain was improved and the patient discharged from the hospital with improved quality of life.