PRO-Angoff method for remote standard setting: establishing clinical thresholds for the upper digestive disease tool.

BACKGROUND: The Upper Digestive Disease (UDD) Tool™ is used to monitor symptom frequency, intensity, and interference across nine symptom domains and includes two Patient-Reported Outcome Measurement Information System (PROMIS) domains assessing physical and mental health. This study aimed to establish cut scores for updated symptom domains through standard setting exercises and evaluate the effectiveness and acceptability of virtual standard setting. METHODS: The extended Angoff method was employed to determine cut scores. Subject matter experts refined performance descriptions for symptom control categories and achieved consensus. Domains were categorized into good, moderate, and poor symptom control. Two cut scores were established, differentiating good vs. moderate and moderate vs. poor. Panelists estimated average scores for 100 borderline patients per item. Cut scores were computed based on the sum of the average ratings for individual questions, converted to 0-100 scale. RESULTS: Performance descriptions were refined. Panelists discussed that interpretation of the scores should take into account the timing of symptoms after surgery and patient populations, and the importance of items asking symptom frequency, severity, and interference with daily life. The good/moderate cut scores ranged from 21.3 to 35.0 (mean 28.6, SD 3.6) across domains, and moderate/poor ranged from 47.5 to 71.3 (mean 54.5, SD 7.0). CONCLUSIONS: Panelists were confident in the virtual standard setting process, expecting valid cut scores. Future studies can further validate the cut scores using patient perspectives and collect patient and physician preferences for displaying contextual items on patient- and physician-facing dashboard.

Esophagectomy Enhanced Recovery After Surgery Initiative Results in Improved Outcomes.

BACKGROUND: Esophagectomy for esophageal cancer is a procedure with high morbidity and mortality. This study developed a Multidisciplinary Esophagectomy Enhanced Recovery Initiative (MERIT) pathway and analyzed implementation outcomes in a single institution. METHODS: The MERIT pathway was developed as a practice optimization and quality improvement initiative. Patients were studied from November 1, 2021 to June 20, 2022 and were compared with historical control subjects. The Wilcoxon rank sum test and the Fisher exact test were used for statistical analysis. RESULTS: The study compared 238 historical patients (January 17, 2017 to December 30, 2020) with 58 consecutive MERIT patients. There were no significant differences between patient characteristics in the 2 groups. In the MERIT group, 49 (85%) of the patients were male, and their mean age was 65 years (range, 59-71 years). Most cases were performed for esophageal cancer after neoadjuvant therapy. Length of stay improved by 27% from 11 to 8 days (P = .27). There was a 12% (P = .05) atrial arrhythmia rate reduction, as well as a 9% (P = .01) decrease in postoperative ileus. Overall complications were reduced from 54% to 35% (-19%; P = .01). CONCLUSIONS: This study successfully developed and implemented an enhanced recovery after surgery pathway for esophagectomy. In the first year, study investigators were able to reduce overall complications, specifically atrial arrhythmias, and postoperative ileus.

Maintaining quality of life after major lung resection for carcinoid tumor.

BACKGROUND: Pulmonary carcinoid is a rare diagnosis with surgery remaining the standard treatment of choice. However, resection may impact patients' daily activities due to decreased lung volume reserve and postoperative pain. Our study aims to compare the impact of different types of surgical resection on the post-operative quality of life with the application of a strict peri-operative pulmonary care program. METHODS: Patients who underwent surgery for bronchopulmonary carcinoid tumors in a tertiary cancer center between August, 2017 and March, 2020 were identified and demographic data was collected. Patients were contacted via phone for the qualitative and quantitative assessment of pain and quality of life, utilizing the Arabic version of Short-form McGill Pain Questionnaire and Activity of Daily Living (ADL) instrument respectively. Lung reserve was assessed before and after surgery. Statistical analysis used Chi-Square for categorical variables and ANOVA for continuous variables. RESULTS: A total of 16 patients underwent different type of resection. The majority were male (n = 10; 63%) with a mean age of 44 years (19-81). Most common clinical stage was stage I (n = 12, 75%) with typical carcinoid features recorded in more than half of the cases (n = 11, 69%). Almost all patients underwent surgical excision (n = 15, 94%) with negative resection margin and no major post-operative complications. Bilobectomy was the most frequent procedure (n = 6, 40%) and video-assisted thoracoscopic surgery (VATS) was utilized in 8 patients (50%). Expected changes were recorded in pre- and postoperative pulmonary function test with an average drop of 10 in FEV1 and 14 mL/min/mmHg in DLCO. The majority of patients (n = 15, 94%) were totally independent doing daily activities. Mild intermittent pain was found in 7 patients (44%) who scored an average intensity of 1.6 out of 10. CONCLUSIONS: Excellent long-term outcomes can be achieved following surgical resection of pulmonary carcinoid tumors with little to no effect on patients' lung function and quality of life in regard to performance status and post-operative pain when a good peri-operative pulmonary, physical rehabilitation, and pain management programs are adopted and strictly implemented.

Locally advanced thymoma; does neoadjuvant chemotherapy make a difference?

BACKGROUND: Regardless of its rare occurrence, Thymoma remains the most frequently encountered primary tumor of the anterior mediastinum comprising about 50% of all masses in the region. Surgical resection, via thymectomy, remains the mainstay treatment modality. In locally advanced and borderline resectable tumors, neoadjuvant chemotherapy (NACT) may be utilized to increase the chance of R0 resection, raising the question of its efficacy and safety. METHODS: Demographic and clinical data from patients who presented to a tertiary cancer center between January 2015-October 2021 with a diagnosis of thymoma and underwent curative surgical resection was collected. Computed tomography scan was used to delineate clinical staging, tumor size and to detect post-therapeutic variations in tumor burden. The response evaluation criteria in solid tumors (RECIST) was used to classify the effect of NACT on tumor burden. The pathological response was determined by measuring the percentage of necrotic tissue. RESULTS: A total of 23 patients were diagnosed with thymoma. Most patients were male with a mean age 46 (± 15) years at diagnosis. The most common clinical stage was stage II with 5 patients (22%). A total of 12 patients had NACT as compared to 11 patients who had upfront surgery. The mean change in tumor volume was 165 cm3 (p = 0.079) and the change in and maximum diameter was 1.53 ± 1.49 cm (p < 0.01). The effect of NACT on tumor burden based on RECIST criteria was minimal as 8 patients had stable disease. Based on pathological findings, the average necrotic portion of the tumor was 39.5% (p = 0.152). The overall survival rate is 95.65%, mean survival was 115 months (4-125). Recurrence occurred in 5 patients. The NACT group had a higher risk for recurrence (4; 33.3%) with a mean survival of 43.8 months compared to 59.6 months in those who did not receive induction therapy. CONCLUSIONS: The exact role of induction chemotherapy in locally advanced thymoma patients remains controversial. NACT effect after utilizing radiological and pathological assessment tools was not found to significantly improve oncological outcomes compared to upfront surgery in locally advanced disease, with minimal radiologic and pathologic effect. To further demonstrate the impact of induction chemotherapy, we recommend multicentric collaborative studies.

Determinants of Long-term Survival Decades After Esophagectomy for Esophageal Cancer.

BACKGROUND: Long-term survival in esophagectomy patients with esophageal cancer is low due to tumor-related characteristics, with few reports of modifiable variables influencing outcome. We identified determinants of overall survival, time to recurrence, and disease-free survival in this patient cohort. METHODS: Adult patients who underwent esophagectomy for primary esophageal cancer from January 5, 2000, through December 30, 2010, at our institution were identified. Univariate Cox models and multivariable logistic regression analyses were used to identify associations between modifiable and unmodifiable patient and clinical variables and outcome of survival for the total cohort and a subgroup with locally advanced disease. RESULTS: We identified 870 patients with esophageal cancer who underwent esophagectomy. The median follow-up time was 15 years, and the 15-year overall survival rate was 25.2%, survival free of recurrence was 57.96%, and disease-free survival was 24.21%. Decreased overall survival was associated with the following unmodifiable variables: older age, male sex, active smoking status, history of coronary artery disease, advanced clinical stage, and tumor location. Decreased overall survival was associated with the following modifiable variables: use of neoadjuvant therapy, advanced pathologic stage, resection margin positivity, surgical reintervention, and blood transfusion requirement. The overall survival probability 6 years after esophagectomy was 0.920 (95% CI, 0.895-0.947), and time-to-recurrence probability was 0.988 (95% CI, 0.976-1.000), with a total of 17 recurrences and 201 deaths. CONCLUSIONS: Once patients survive 5 years, recurrence is rare. Long-term survival can be achieved in high-volume centers adhering to National Comprehensive Cancer Network guidelines using multidisciplinary care teams that is double what has been previously reported in the literature from national databases.

Food Deserts Increase Readmission After Esophagectomy for Cancer: A Multi-institutional Study.

BACKGROUND: Food deserts are low-income census tracts with poor access to supermarkets and are associated with worse outcomes in breast, colon, and a small number of esophageal cancer patients. This study investigated residency in food deserts on readmission rates in a multi-institutional cohort of esophageal cancer patients undergoing trimodality therapy. METHODS: A retrospective review of patients who underwent trimodality therapy at 6 high-volume institutions from January 2015 to July 2019 was performed. Food desert status was defined by the United States Department of Agriculture by patient ZIP Code. The primary outcome was 30-day readmission after esophagectomy. Multilevel, multivariable logistic regression was used to model readmission on food desert status adjusted for diabetes, insurance type, length of stay, and any complication, treating the institution as a random factor. RESULTS: Of the 453 records evaluated, 425 were included in the analysis. Seventy-three patients (17.4%) resided in a food desert. Univariate analysis demonstrated food desert patients had significantly increased 30-day readmission. No differences were seen in length of stay, complications, or 30-day mortality. In the adjusted logistic regression model, residing in a food desert remained a significant risk factor for readmission (odds ratio, 2.11; 95% CI, 1.07-4.15). There were no differences in 30-day, 90-day, or 1-year mortality based on food desert status, although readmission was associated with worse 90-day and 1-year mortality. CONCLUSIONS: Food desert residence was associated with 30-day readmission after esophagectomy in patients undergoing trimodality treatment for esophageal cancer in this multi-institutional population. Identification of patients residing in a food desert may allow surgeons to focus preventative interventions during treatment and postoperatively to improve outcomes.

Atypical perigraft seroma masquerading as a forearm tumor in a dialysis patient.

BACKGROUND: An extremely rare manifestation of perigraft seroma (PGS), in which a dense, semisolid jelly-like mass had formed around the shunt instead of the standard fluid-like form of the usual seroma, leading to misdiagnosis with other entities, such as tumors around the synthetic arterio-venous shunt (AVS) was presented. CASE REPORT: A 64-year-old male with multiple myeloma post autologous bone marrow transplant with a renal impairment, presented with a rare form of PGS, which was noticed 2 months after placing a synthetic AVS vascular graft. The mass increased in size, and multiple attempts for excision failed due to recurrence, which led to tumor misdiagnosis. The mass reoccurrence stopped completely only after the radical shunt removal. CONCLUSION: This case report revealed a rare form of PGS, in which the seroma was represented as a firm, semisolid jelly-like mass rather than the typical fluid type transudate seroma. Despite its rarity, it was associated with a high recurrence rate because unlike the standard perishunt seroma, this semisolid jelly-like material could neither be aspirated, nor could it be resected en-bloc, leading to shunting dysfunction. Its management included advanced imaging and a high probability of shunt removal or replacement.

Early surgical and oncological outcomes during adoption of a single port VATS lung resection in a tertiary cancer center: a retrospective analysis.

BACKGROUND: Video-assisted thoracoscopic surgery (VATS) is a safe and effective surgical approach for pulmonary resection. VATS can be accomplished with only a single incision, resulting in less postoperative pain and paresthesia, better cosmetic results, and greater patient satisfaction. Single-port VATS (spVATS) has become increasingly common for lung resection. We assess the early surgical and oncological outcomes after adopting this new technique at our tertiary cancer center as the first institution to do so in the country. METHOD: Medical records for 257 patients in a tertiary cancer center, with a diagnosis of non-small cell lung cancer, pulmonary metastasis, or other chest-confined pathology, were accessed to obtain perioperative outcomes, pathologic results, post-operative follow-up data, and early surgical and oncological outcomes. All patients underwent spVATS for limited or major lung resection. Simple descriptive analysis was utilized. RESULTS: spVATS was either performed with curative intent (79.8%, N = 205), or as a diagnostic procedure (20.2%, N = 52). Resection types were subcategorized for curative intent group as limited (73.6%, N = 151), lobectomy (16.6%, N = 34), and complex (9.7%, N = 20). Resection with a negative margin (R0) rate was 100% for the primary lung cancer (PLC) patients and 97% for the pulmonary metastasectomy (PM) group. The complication rate was 5%. Three-year disease-free survival was 87% and 68.5% for PLC and PM group, respectively. The 3-year overall-survival was 91.3% for the PLC and 82.8% for PM. Operation duration showed a downtrend over the study period in each curative subcategory with a borderline difference in the limited resection (P value = 0.05). CONCLUSION: All the spVATS procedures were successfully performed without perioperative severe complications or mortality, regardless of complexity. R0 resection was excellent. Middle- and long-term efficacies of spVATS for lung cancer require further follow-up. With proper training, appropriate indication and meticulous application, adopting spVATS is safe and feasible technique that does not compromise surgical and oncological outcomes.

Salvaging the Digit in Invasive Subungual Malignancies Using a Triple Technique under Awake Local Anesthesia.

INTRODUCTION: Amputation for subungual malignancy (SUM) was thought to be the gold standard in preventing recurrence and metastasis. The rationale behind this aggressive treatment was never based on scientific evidence. Even though multiple recent studies supported more conservative management by illustrating successful results of the digit salvage technique, especially for "in situ" SUM, this salvage approach is not well supported for the more aggressive type of the "invasive" SUM; herein, we salvaged two cases of "invasive" SUM. Case Presentation. We present two cases of invasive SUM without radiographic evidence of intraosseous involvement, where we avoided digit amputation for both invasive subungual squamous cell carcinoma of the thumb and invasive subungual melanoma of the ring finger. Both were salvaged by using a triple technique under awake local anesthesia which included (I) radical excision of the nail bed unit including both eponychium and periosteum, (II) dorsal cortical bone shaving using a high-speed burr for the distal phalanx, and (III) flap coverage. Brunelli flap was used for the thumb in the first case, and V-Y plasty combined with proximal nail fold advancement flap was used for the ring finger in the second case. There was no evidence of local or distant recurrence, with a good functional outcome after 2.5 years in the first case and 2 years in the second. CONCLUSION: Ensuring complete resection with negative margins while preserving the functionality of the affected digit is considered to be the optimal challenge in treating "invasive" subungual malignancies. These two case reports contribute by reporting a successful digit salvage. The safety of this procedure could be confirmed by larger series and longer follow-up periods.

Chest wall and diaphragm reconstruction; a technique not well established in literature - case report.

INTRODUCTION: Regardless of its rarity, and indolent clinical course, chest wall tumor places high morbidity and burden on patients especially when invasion to a neighboring structure is found. Once detected, surgery is the cornerstone for treatment of such etiology combined with chemo-radiotherapy. In order to maintain intact respiratory function, chest wall reconstruction must be performed whenever resection is done. Herein, we present a case of chest wall tumor that necessitated three ribs and part of hemidiaphragm resection and reconstruction with optimal post-operative results. CASE PRESENTATION: A 27-year-old male patient who had chest wall and diaphragm reconstruction for a chest wall Ewing sarcoma, using a single patch of expanded polytetrafluoroethylene (ePTFE) mesh with diaphragm implanted into the middle of the mesh. There were no immediate nor post-operative complications. The patient received post-operative radiotherapy with good functional and cosmetic results. CONCLUSION: We present a novel and safe technique for combined chest wall and diaphragmatic resection following excision of an invading tumor while ensuring cosmesis and functionality of the ribcage as well as the diaphragm.

Transdiaphragmatic single-port video-assisted thoracoscopic surgery; a novel approach for pulmonary metastasectomy through laparotomy incision - case series.

BACKGROUND: Pulmonary metastasectomy was performed in the early twentieth century and ever since, it has evolved to be one of the main treatment options for certain metastatic malignancies. The advancement of minimally invasive procedures enabled new techniques to minimize morbidity and improve patient quality of care and overall outcome. CASES PRESENTATION: Herein we present three patients, aged 53, 48, and 27 years, known to have sigmoid, rectal, and non-seminomatous germ cell tumors respectively. All patients were diagnosed to have metastatic lung nodules and underwent laparotomy to excise abdominal tumors followed by trans-diaphragmatic single-port video-assisted thoracoscopic pulmonary metastasectomy. All patients achieved complete surgical tumor excision, and none had pulmonary related complications on follow-up. CONCLUSION: Our prescribed novel trans-diaphragmatic single-port video-assisted thoracoscopic surgery (VATS) technique for synchronous pulmonary metastasectomy and intra-abdominal tumor resection is safe and can achieve complete resection with negative margins.

The impact of a perioperative pulmonary care bundle implementation on postoperative outcomes in 1,665 surgical cancer patients: experience from a tertiary referral cancer center in Jordan.

BACKGROUND: Postoperative pulmonary complications can be a major catastrophic consequence of major surgeries and can lead to increased morbidity, mortality, hospital stay, and cost. Many protocols have been tried to reduce serious adverse outcomes with effective strategies including a bundle of preoperative, intraoperative and postoperative techniques. Using these techniques maybe challenging in developing countries with limited resources even in specialized centers. METHODS: A before-and-after trial comparing our data from the national surgical quality improvement program (NSQIP) based on their reports. Data was collected prospectively for the patients who underwent major surgeries at King Hussein Cancer Center during the year 2017 when the use of the perioperative pulmonary care bundle was mandatory to all surgery teams and compared it with the data collected retrospectively for the patients who underwent the same type of surgeries in the year 2016 when the use of such a bundle was optional. The primary end point is the decrease in incidence of postoperative pulmonary complications. Simple descriptive statistical analysis was used to obtain results. RESULTS: The potential risk factors for postoperative pulmonary complications for 1665 patients divided into 2 groups (2016 vs. 2017); 764 (45.9%) vs. 901 (54.1%), respectively. There were no significant differences regarding gender (male 46.7% vs. 46.4%, P value = 0.891, female 53.3% vs. 53.6%, P value = 0.39), mean of age (53.5 year vs. 5.28 year, P value = 0.296), mean of body mass index (BMI) (28.6 vs. 6%, 28.6, P value = 0.95), smoking status; (69.6% vs. 65.1%, P value = 0.052), ventilator use (0.3% vs. 0.4% P value = 0.693) and chronic obstructive pulmonary disease (1.4% vs. 1.4 with, P value = 0.996).The data showed a significant reduction in postoperative pneumonia between the 2 groups (2016 vs. 2017) (2.7% vs. 0.9%, P value = 0.004) and showed a significant reduction in unplanned intubation, 1.4% in 2016 vs. 0.7% in 2017. CONCLUSIONS: The standardization of perioperative pulmonary care bundle, including smoking cessation, perioperative pulmonary interventions and early mobilization, significantly reduces the incidence of postoperative pulmonary complications in cancer patients. This technique's implementation was feasible easily even with limited resources in a developing country like Jordan.

"Whoops" fixation of proximal humerus pathological fracture ended with forequarter amputation - Case report.

INTRODUCTION: Even with the advancement of limb salvage surgery techniques, forequarter amputation (FQA) is still used in orthopedic oncology. Even though it might pose catastrophic sequelae on the patient's lifestyle, debilitating one's ability to perform regular tasks, FQA is still considered as a treatment of last resort for huge fungating tumors of the upper extremity. CASE PRESENTATION: We present a case of an 18-year-old male patient, who was diagnosed in Libya with left proximal humerus fracture after a trivial trauma and underwent open reduction and internal fixation using k-wires as it was thought to be a simple fracture. Soon after, pain and swelling progressed severely and an open biopsy confirmed a diagnosis of osteosarcoma and imaging suggested metastatic disease to the lungs for which he was started on chemoradiotherapy. He was referred to our cancer center to continue his management and due to the aggressive nature of the tumor, the patient underwent palliative forequarter amputation followed by multiple lines of chemotherapy and radiotherapy, all of which failed to halt the progression of the disease. The patient was lost to follow up due to his decision to go back to Libya. CONCLUSION: "Whoops" surgeries are fixated upon repairing fractures without looking for the alarming signs on radiographs to exclude pathological entity. As in our case, the procedure done escalated the osteosarcoma into such a massive fungating tumor due to the violation of the osteosarcoma pseudo capsule, in which the only available option is to do a palliative forequarter amputation.

Fungating malignant peripheral nerve sheath tumor arising from a slow-growing mass in the forearm: a case report and review of the literature.

BACKGROUND: Malignant peripheral nerve sheath tumor is a rare and aggressive form of sarcoma that arises from a peripheral nerve, mostly in association with neurofibromatosis type 1. Half of the cases were reported in the extremities, with the lungs being the most common site of metastasis. We report a rare case of successful limb salvage surgery performed for a large exophytic malignant peripheral nerve sheath tumor of the right forearm with wide surgical margins followed by split-thickness skin graft and later a flexor carpi radialis tendon transfer to extensor digitorum communis tendon. CASE PRESENTATION: A 51-year-old Bedouin Arabic man presented to our institution with an incompletely excised, large, fungating, malignant peripheral nerve sheath tumor occupying most of his right forearm. Staging imaging showed multiple lung nodules. He underwent wide local excision followed by skin graft and tendon transfer as a palliative measure to preserve the function of his dominant limb. The operation was performed without any complications, and the patient had an excellent postoperative result. Afterward, he was started on multiple lines of chemotherapy that failed because of disease progression, and the patient died 7 months after the operation. CONCLUSION: Clinicians must consider the possibility of soft tissue sarcoma even in a patient with a small, slow-growing, superficial mass. Furthermore, a wrong open biopsy or nononcological surgical procedure may lead to possible contamination and ultimately a more radical procedure than would have originally been necessary, where this can be prevented by an early referral to a highly specialized sarcoma center.

Heart rupture repair during huge mediastinal mass resection - case report.

BACKGROUND: Ventricular rupture is rarely described in the literature outside the context of myocardial infarction, infection or neoplasm. It is associated with a high mortality rate due to late presentation and delayed surgical intervention, which involves sutureless or sutured techniques. Comprehensive literature review failed to identify any case of intra-operative right ventricular heart rupture followed by myocardial repair and a complete recovery after a prolonged intensive care unit (ICU) stay. CASE PRESENTATION: A 57-year-old previously healthy gentleman presented complaining of a new onset shortness of breath for 2 months. A large mediastinal mass was found on chest imaging and biopsy revealed a thymoma. Patient received a neoadjuvant Cisplatin/Doxorubicin/Cyclophosphamide (CAP) regimen chemotherapy then sternotomy and thymectomy en bloc with anterior pericardium. Post-thymectomy, the patient continued to be hypotensive in recovery despite aggressive fluid resuscitation. He was sent back to theatre, aggressive fluid resuscitation continued, surgical site exploration was done by reopening the sternum, and the bleeding source was identified and controlled, but intraoperative asystole developed. During internal cardiac massage, the right ventricle ruptured with a 3 cm defect which was successfully repaired using a pericardial patch without a bypass machine due to unavailability at our cancer center. The patient remained dependent on mechanical ventilation through tracheostomy for a total of 2 months due to bilateral phrenic nerve paralysis, was discharged from ICU to the surgical floor 66 days after the operation and weaned off ventilator support after 85 days, adequate respiratory and physical rehabilitation followed. Patient is doing very well now with excellent performance, and free of tumor recurrence 30 months after surgery. CONCLUSION: Right ventricular rupture is rarely described outside the context of myocardial infarction and valvular heart disease. Tumor proximity to the heart and neoadjuvant cardiotoxic chemotherapy are the proposed causes for precipitating the cardiac rupture in our case. Post-surgical patients who receive early physical rehabilitation and respiratory physiotherapy have improved survival and outcome.

Surgical Reconstruction Methods following Radical Excision of Distal Ulna Osteosarcoma in Both Skeletally Mature and Immature Patients.

The distal ulna has always been considered to be expendable and its removal has been advocated for a variety of post-traumatic degenerative and oncological conditions but recent studies showed that the distal radioulnar joint allows supination and protonation of the forearm and is important to one's grip strength and lifting ability. Several prosthesis models have already been made to replace the mechanical functionality of the distal radioulnar joint. We present two cases of females aged 22 and 12 years, respectively, who presented with wrist pain and swelling without any history of trauma and with terminal degree limitation in wrist movements due to tenderness and swelling. Both of them did not have any distant metastasis upon radiographic staging. The skeletally mature patient underwent radical excision of the distal ulnar osteosarcoma and received a distal radioulnar joint replacement prosthesis (Scheker prosthesis). The other skeletally immature patient underwent radical excision of the involved distal ulnar osteosarcoma with stabilization of the residual ulnar stump using the extensor carpi ulnaris sling in a modified version of the Goldner and Hayes technique. Both of our patients were treated according to the protocols of our multidisciplinary clinic sarcoma team by starting with neoadjuvant chemotherapy, followed by surgery and adjuvant chemotherapy. Both registered an almost complete restoration of the normal wrist and hand function and were in complete remission for 26 and 24 months, respectively. Based on our literature review, these are some of the extremely rare cases in which the osteosarcoma affected an unusual site (the distal ulna where they underwent a rare type of reconstruction status following radical excision of a malignant tumor).

Metastatic non-functional paraganglioma to the lung.

INTRODUCTION: Paragangliomas are rare endocrine tumors that arise from the extra-adrenal autonomic paraganglia and sympathetic paragangliomas usually secret catecholamines and are located in the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis. In contrast, most parasympathetic paragangliomas are nonfunctional and located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull. Such neoplasms, although rare, are clinically important because they may recur after surgical resection and 10% of them give rise to metastases causing death with the lymphatic nodes, bones, liver, and lungs being the most common locations. CASE PRESENTATION: We present a case of a 26-year-old male patient that was diagnosed with paraganglioma of the right-frontal lobe infiltrating the falx and frontal bone which was diagnosed after suffering from a headache and abnormal vision. On initial work-up he was found to have right pulmonary nodules that increased in size after follow up and other nodules appeared in the contralateral lung. He underwent subtotal resection of the brain tumor and complete resection of the bilateral pulmonary nodules. CONCLUSION: To our knowledge, paraganglioma is considered to be a rare entity in the central nervous system with very few cases being reported in the supratentorial region and no cases were reported of metastatic such paraganglioma to the lung.

Novel Double Central Ray Amputation of the Third and Fourth Digits: Case Report and Literature Review.

Whenever partial hand amputations for soft tissue sarcomas are attempted, special consideration should be given to achieve a balance between complete resection associated with negative margins and preservation of functionality to the patient so that the hand can support the contralateral intact hand for bimanual activities. This difficult decision is even more challenging within the limited anatomical confines of the hand. Based on our literature review, this is the first case of double central 3rd and 4th ray amputation, as far as we know with good hand function, evaluated by the Musculoskeletal Tumor Rating Scale.

Spina Bifida: Pathogenesis, Mechanisms, and Genes in Mice and Humans.

Spina bifida is among the phenotypes of the larger condition known as neural tube defects (NTDs). It is the most common central nervous system malformation compatible with life and the second leading cause of birth defects after congenital heart defects. In this review paper, we define spina bifida and discuss the phenotypes seen in humans as described by both surgeons and embryologists in order to compare and ultimately contrast it to the leading animal model, the mouse. Our understanding of spina bifida is currently limited to the observations we make in mouse models, which reflect complete or targeted knockouts of genes, which perturb the whole gene(s) without taking into account the issue of haploinsufficiency, which is most prominent in the human spina bifida condition. We thus conclude that the need to study spina bifida in all its forms, both aperta and occulta, is more indicative of the spina bifida in surviving humans and that the measure of deterioration arising from caudal neural tube defects, more commonly known as spina bifida, must be determined by the level of the lesion both in mouse and in man.