ABSTRACT
OBJECTIVE: We sought to evaluate how implementing a thoracic enhanced recovery after surgery (ERAS) protocol impacted surgical outcomes after elective anatomic lung resection. BACKGROUND: The effect of implementing the ERAS Society/European Society of Thoracic Surgery thoracic ERAS protocol on postoperative outcomes throughout an entire health care system has not yet been reported. METHODS: This was a prospective cohort study within one health care system (January 2019-March, 2023). A thoracic ERAS protocol was implemented on May 1, 2021 for elective anatomic lung resections, and postoperative outcomes were tracked using the electronic health record and Vizient data. The primary outcome was overall morbidity; secondary outcomes included individual complications, length of stay, opioid use, chest tube duration, and total cost. Patients were grouped into pre-ERAS and post-ERAS cohorts. Bivariable comparisons were performed using independent t -test, χ 2 , or Fisher exact tests, and multivariable logistic regression was performed to control for confounders. RESULTS: There were 1007 patients in the cohort; 450 (44.7%) were in the post-ERAS group. Mean age was 66.2 years; most patients were female (65.1%), white (83.8%), had a body mass index between 18.5 and 29.9 (69.7%), and were ASA class 3 (80.6%). Patients in the postimplementation group had lower risk-adjusted rates of any morbidity, respiratory complication, pneumonia, surgical site infection, arrhythmias, infections, opioid usage, ICU use, and shorter postoperative length of stay (all P <0.05). CONCLUSIONS: Postoperative outcomes were improved after the implementation of an evidence-based thoracic ERAS protocol throughout the health care system. This study validates the ERAS Society/European Society of Thoracic Surgery guidelines and demonstrates that simultaneous multihospital implementation can be feasible and effective.
Subject(s)
Enhanced Recovery After Surgery , Pneumonectomy , Postoperative Complications , Humans , Female , Male , Aged , Prospective Studies , Pneumonectomy/methods , Postoperative Complications/epidemiology , Postoperative Complications/prevention & control , Middle Aged , Clinical Protocols , Length of Stay/statistics & numerical dataABSTRACT
Purpose In early-stage non-small cell lung cancer (NSCLC), post-treatment mortality may influence the comparative effectiveness of surgery and stereotactic body radiotherapy (SBRT), with implications for shared decision making among high-risk surgical candidates. We analyzed early mortality after these interventions using the National Cancer Database. Patients and Methods We abstracted patients with cT1-T2a, N0, M0 NSCLC diagnosed between 2004 and 2013 undergoing either surgery or SBRT. Thirty-day and 90-day post-treatment mortality rates were calculated and compared using Cox regression and propensity score-matched analyses. Results We identified 76,623 patients who underwent surgery (78% lobectomy, 20% sublobar resection, 2% pneumonectomy) and 8,216 patients who received SBRT. In the unmatched cohort, mortality rates were moderately increased with surgery versus SBRT (30 days, 2.07% v 0.73% [absolute difference (Δ), 1.34%]; P < .001; 90 days, 3.59% v 2.93% [Δ, 0.66%]; P < .001). Among the 27,200 propensity score-matched patients, these differences increased (30 days, 2.41% v 0.79% [Δ, 1.62%]; P < .001; 90 days, 4.23% v 2.82% [Δ, 1.41%]; P < .001). Differences in mortality between surgery and SBRT increased with age, with interaction P < .001 at both 30 days and 90 days (71 to 75 years old: 30-day Δ, 1.87%; 90-day Δ, 2.02%; 76 to 80 years old: 30-day Δ, 2.80%; 90-day Δ, 2.59%; > 80 years old: 30-day Δ, 3.03%; 90-day Δ, 3.67%; all P ≤ .001). Compared with SBRT, surgical mortality rates were higher with increased extent of resection (30-day and 90-day multivariate hazard ratio for mortality: sublobar resection, 2.85 and 1.37; lobectomy, 3.65 and 1.60; pneumonectomy, 14.5 and 5.66; all P < 0.001). Conclusion Differences in 30- and 90-day post-treatment mortality between surgery and SBRT increased as a function of age, with the largest differences in favor of SBRT observed among patients older than 70 years. These representative mortality data may inform shared decision making among patients with early-stage NSCLC who are eligible for both interventions.
Subject(s)
Carcinoma, Non-Small-Cell Lung/therapy , Lung Neoplasms/therapy , Radiosurgery , Adult , Aged , Aged, 80 and over , Carcinoma, Non-Small-Cell Lung/mortality , Carcinoma, Non-Small-Cell Lung/pathology , Female , Humans , Lung Neoplasms/mortality , Lung Neoplasms/pathology , Male , Middle Aged , Neoplasm Staging , Propensity Score , Proportional Hazards ModelsABSTRACT
BACKGROUND: The treatment of stage I and II non-small cell lung cancer (NSCLC) in patients with good or low surgical risk is primarily surgical resection. However, this area is undergoing many changes. With a greater prevalence of CT imaging, many lung cancers are being found that are small or constitute primarily ground-glass opacities. Treatment such as sublobar resection and nonsurgical approaches such as stereotactic body radiotherapy (SBRT) are being explored. With the advent of minimally invasive resections, the criteria to classify a patient as too ill to undergo an anatomic lung resection are being redefined. METHODS: The writing panel selected topics for review based on clinical relevance to treatment of early-stage lung cancer and the amount and quality of data available for analysis and relative controversy on best approaches in stage I and II NSCLC: general surgical care vs specialist care; sublobar vs lobar surgical approaches to stage I lung cancer; video-assisted thoracic surgery vs open resection; mediastinal lymph node sampling vs lymphadenectomy at the time of surgical resection; the use of radiation therapy, with a focus on SBRT, for primary treatment of early-stage NSCLC in high-risk or medically inoperable patients as well as adjuvant radiation therapy in the sublobar and lobar resection settings; adjuvant chemotherapy for early-stage NSCLC; and the impact of ethnicity, geography, and socioeconomic status on lung cancer survival. Recommendations by the writing committee were based on an evidence-based review of the literature and in accordance with the approach described by the Guidelines Oversight Committee of the American College of Chest Physicians. RESULTS: Surgical resection remains the primary and preferred approach to the treatment of stage I and II NSCLC. Lobectomy or greater resection remains the preferred approach to T1b and larger tumors. The use of sublobar resection for T1a tumors and the application of adjuvant radiation therapy in this group are being actively studied in large clinical trials. Every patient should have systematic mediastinal lymph node sampling at the time of curative intent surgical resection, and mediastinal lymphadenectomy can be performed without increased morbidity. Perioperative morbidity and mortality are reduced and long-term survival is improved when surgical resection is performed by a board-certified thoracic surgeon. The use of adjuvant chemotherapy for stage II NSCLC is recommended and has shown benefit. The use of adjuvant radiation or chemotherapy for stage I NSCLC is of unproven benefit. Primary radiation therapy remains the primary curative intent approach for patients who refuse surgical resection or are determined by a multidisciplinary team to be inoperable. There is growing evidence that SBRT provides greater local control than standard radiation therapy for high-risk and medically inoperable patients with NSCLC. The role of ablative therapies in the treatment of high-risk patients with stage I NSCLC is evolving. Radiofrequency ablation, the most studied of the ablative modalities, has been used effectively in medically inoperable patients with small (< 3 cm) peripheral NSCLC that are clinical stage I.
Subject(s)
Carcinoma, Non-Small-Cell Lung/therapy , Lung Neoplasms/therapy , Carcinoma, Non-Small-Cell Lung/pathology , Carcinoma, Non-Small-Cell Lung/surgery , Chemotherapy, Adjuvant , Humans , Lung Neoplasms/pathology , Lung Neoplasms/surgery , Lymph Node Excision , Neoplasm Staging , Patient Selection , Pneumonectomy , Radiosurgery , Radiotherapy, Adjuvant , Thoracic Surgery, Video-AssistedABSTRACT
Management of zone I gunshot injuries to the neck is controversial. Endovascular and endoscopic therapies are appealing minimally invasive alternatives, but they are still evolving. This case report demonstrates effective stent grafting of an arterial and an esophageal zone I neck injury after a civilian gunshot.
Subject(s)
Blood Vessel Prosthesis Implantation/instrumentation , Blood Vessel Prosthesis , Carotid Artery Injuries/surgery , Carotid Artery, Common/surgery , Esophagoscopy/instrumentation , Esophagus/surgery , Neck Injuries/surgery , Stents , Wounds, Gunshot/surgery , Carotid Artery Injuries/diagnostic imaging , Carotid Artery, Common/diagnostic imaging , Esophagus/diagnostic imaging , Esophagus/injuries , Humans , Male , Neck Injuries/diagnostic imaging , Tomography, X-Ray Computed , Treatment Outcome , Wounds, Gunshot/diagnostic imaging , Young AdultSubject(s)
Carcinoma, Non-Small-Cell Lung/therapy , Lung Neoplasms/therapy , Practice Guidelines as Topic , Carcinoma, Non-Small-Cell Lung/diagnosis , Carcinoma, Non-Small-Cell Lung/genetics , Carcinoma, Non-Small-Cell Lung/pathology , Humans , Lung Neoplasms/diagnosis , Lung Neoplasms/genetics , Lung Neoplasms/pathology , Neoplasm Staging , PrognosisABSTRACT
BACKGROUND: The definitive treatment of esophageal cancer remains surgical resection. Morbidity and mortality are highly influenced by the success of the anastomosis created in the reconstruction of the resected esophagus. The results of an anastomotic technique that creates an esophageal mucosal tube are analyzed. METHODS: The medical records of all patients undergoing esophagectomy at a single institution by 3 surgeons between January 2002 and July 2008 were reviewed. Patients who underwent a 2-layer, hand-sewn, esophageal anastomosis using a mucosal tube were included. The unique aspect of the anastomosis was the creation of an esophageal mucosal tube that facilitates a tension-free, precise mucosal approximation. RESULTS: Of the 61 patients who underwent esophageal reconstructions (60 gastric, 1 colonic), 49 (80%) had a diagnosis of esophageal neoplasm. Of those with cancer, 20 (41%) had neoadjuvant therapy before the resection. Two patients presented with perforation. The anastomoses were intrathoracic in 57 of 61 (93%) and cervical in 4 cervical. There were no operative deaths. All patients underwent contrast study at an average of 5 days postoperatively. The anastomotic leak rate was 2% (1 of 61). Postoperative dilations (mean, 1.3 dilations) were done in 12 of 61 patients (20%), using a low symptom threshold for endoscopy and dilation. CONCLUSIONS: The use of the esophageal mucosal tube and 2-layer anastomosis is a robust technique that results in a low leak rate. Strictures are minimal and easily dilated if they occur. Use of a gastrotomy larger than 2.5 cm may decrease stricture rates.
Subject(s)
Esophageal Neoplasms/surgery , Esophagectomy , Esophagus/surgery , Anastomosis, Surgical/methods , Female , Humans , Male , Middle Aged , Mucous Membrane , Suture TechniquesABSTRACT
PURPOSE: This study evaluated the efficacy and safety of pemetrexed, carboplatin, and bevacizumab followed by maintenance pemetrexed and bevacizumab in patients with chemotherapy-naive stage IIIB (effusion) or stage IV nonsquamous non-small-cell lung cancer (NSCLC). PATIENTS AND METHODS: Patients received pemetrexed 500 mg/m(2), carboplatin area under the concentration-time curve of 6, and bevacizumab 15 mg/kg every 3 weeks for six cycles. For patients with response or stable disease, pemetrexed and bevacizumab were continued until disease progression or unacceptable toxicity. RESULTS: Fifty patients were enrolled and received treatment. The median follow-up was 13.0 months, and the median number of treatment cycles was seven (range, one to 51). Thirty patients (60%) completed > or = six treatment cycles, and nine (18%) completed > or = 18 treatment cycles. Among the 49 patients assessable for response, the objective response rate was 55% (95% CI, 41% to 69%). Median progression-free and overall survival rates were 7.8 months (95% CI, 5.2 to 11.5 months) and 14.1 months (95% CI, 10.8 to 19.6 months), respectively. Grade 3/4 hematologic toxicity was modest-anemia (6%; 0), neutropenia (4%; 0), and thrombocytopenia (0; 8%). Grade 3/4 nonhematologic toxicities were proteinuria (2%; 0), venous thrombosis (4%; 2%), arterial thrombosis (2%; 0), fatigue (8%; 0), infection (8%; 2%), nephrotoxicity (2%; 0), and diverticulitis (6%; 2%). There were no grade 3 or greater hemorrhagic events or hypertension cases. CONCLUSION: This regimen, involving a maintenance component, was associated with acceptable toxicity and relatively long survival in patients with advanced nonsquamous NSCLC. These results justify a phase III comparison against the standard-of-care in this patient population.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma, Non-Small-Cell Lung/drug therapy , Lung Neoplasms/drug therapy , Adult , Aged , Aged, 80 and over , Anemia/chemically induced , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal, Humanized , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Bevacizumab , Carboplatin/administration & dosage , Carboplatin/adverse effects , Carcinoma, Non-Small-Cell Lung/pathology , Disease-Free Survival , Drug Administration Schedule , Fatigue/chemically induced , Female , Follow-Up Studies , Glutamates/administration & dosage , Glutamates/adverse effects , Guanine/administration & dosage , Guanine/adverse effects , Guanine/analogs & derivatives , Humans , Kaplan-Meier Estimate , Lung Neoplasms/pathology , Male , Middle Aged , Neoplasm Staging , Neutropenia/chemically induced , Pemetrexed , Thrombocytopenia/chemically induced , Treatment OutcomeABSTRACT
Acute development of superior vena cava syndrome is unusual. This report describes a patient who suddenly presented with a superior vena cava obstruction after esophageal and tracheal stenting for a malignant tracheoesophageal fistula. Stenting of the superior vena cava rapidly alleviated the obstruction and resulted in resolution of symptoms.
Subject(s)
Adenocarcinoma/complications , Lung Neoplasms/complications , Stents , Superior Vena Cava Syndrome/therapy , Tracheoesophageal Fistula/therapy , Humans , Male , Middle Aged , Superior Vena Cava Syndrome/etiology , Tracheoesophageal Fistula/etiologyABSTRACT
BACKGROUND: The development of upper gastrointestinal malignancies after bariatric surgery has not been well characterized. Our objective was to review the experience of patients with distal esophageal cancer that was diagnosed after bariatric surgery. METHODS: A retrospective review was conducted to identify patients who had undergone bariatric surgery (1999 to 2006) and who later developed high-grade dysplasia or adenocarcinoma of the distal esophagus. RESULTS: Three patients (of 2,875 [0.1%]) developed esophageal cancer: 2 after Roux-en-Y gastric bypass and 1 after vertical banded gastroplasty. All three patients had complaints of reflux, and two were treated with esophagectomy. The third patient presented with invasive carcinoma and died 2 years after diagnosis. CONCLUSIONS: Our findings emphasize the importance of precise endoscopic evaluation before bariatric surgery in patients with gastroesophageal reflux disease (GERD), of the necessity for continuing postsurgical surveillance in patients with known Barrett's esophagitis, and of early evaluation in patients who develop new symptoms of GERD after bariatric surgery.
Subject(s)
Adenocarcinoma/etiology , Bariatric Surgery , Esophageal Neoplasms/etiology , Esophagogastric Junction , Postoperative Complications , Aged , Barrett Esophagus/etiology , Gastroesophageal Reflux/etiology , Humans , Male , Middle Aged , Obesity, Morbid/complications , Obesity, Morbid/surgery , Retrospective StudiesABSTRACT
BACKGROUND: Gastrointestinal stromal tumors (GISTs) are the most common sarcoma of the gastrointestinal tract, but occur rarely in the esophagus. Use of tyrosine kinase inhibitors has revolutionized therapy for GISTs but complete resection remains the treatment of choice. Esophageal GISTs require special consideration regarding perioperative treatment, evaluation, and conduct of operation. METHODS: We report our experience (from 2000 to 2003) with four patients who underwent resection of esophageal GIST, reviewed consensus management guidelines, and performed a literature review for reported cases. Query of the National Cancer Database was made to assess national experience with esophageal GISTs. RESULTS: All four patients had esophageal GISTs successfully differentiated from leiomyomata by immunohistochemistry on fine needle aspirates obtained during endoscopic ultrasound. At the time of resection all tumors were noted to be adherent to tissues other than submucosa that required resection of esophageal muscularis, periesophageal tissue, or mucosa. All patients were thought to have complete resection and were alive at last contact (median, 33 months). Two of four patients recurred, one of whom underwent esophagectomy; the other patient is alive on imatinib. Seven case reports with median follow-up of three years reported no recurrence with either enucleation or esophagectomy. The National Cancer Database contains 33 cases of esophageal GIST reported since 1999. Nine of 24 patients died within six months of diagnosis. CONCLUSIONS: Esophagectomy is the treatment of choice for esophageal GISTs but requires attention to operative details that emphasize complete en bloc excision of surrounding tissue. Preoperative imatinib may improve resectability.
Subject(s)
Esophageal Neoplasms/surgery , Esophagectomy/methods , Gastrointestinal Stromal Tumors/surgery , Aged , Esophageal Neoplasms/pathology , Female , Gastrointestinal Stromal Tumors/pathology , Humans , Male , Middle Aged , Positron-Emission TomographySubject(s)
Actinomyces/isolation & purification , Actinomycosis/complications , Actinomycosis/surgery , Bronchial Fistula/microbiology , Bronchial Fistula/surgery , Esophageal Fistula/microbiology , Esophageal Fistula/surgery , Stents , Female , Humans , Middle Aged , Recurrence , Surgical Flaps , ThoracotomyABSTRACT
BACKGROUND: Acute lung injury (ALI) is a life-threatening complication of talc pleurodesis. This study defines characteristics that predispose patients to ALI and severe hypoxemia in patients after video-assisted thoracoscopic surgery (VATS) talc pleurodesis. METHODS: Charts of patients who underwent talc pleurodesis at Northwestern Memorial Hospital between January 1, 1997 and December 31, 2003 were retrospectively reviewed. We sought variables associated with the development of postoperative ALI or severe hypoxemia, defined as an increase in fraction of the inspired oxygen by more than 0.15 within 24 hours after the surgery. The analysis included 84 patients (58 women, 26 men) who underwent VATS talc pleurodesis for malignant (n = 74) or benign (n = 10) indications. RESULTS: ALI developed in 5 patients (5.9%), severe hypoxemia developed in 25 (29.8%), and 54 (64.3%) did not have postoperative complications. In multivariate analysis, the presence of peripheral edema before pleurodesis (p = 0.005), any preoperative requirement for supplemental oxygen (p = 0.032), and chemotherapy within 14 days before pleurodesis (p = 0.04) were identified as predictors of ALI or severe postoperative hypoxemia. CONCLUSIONS: Oxygen supplementation, recent chemotherapy, and presence of peripheral edema were independent predictors of a combined outcome of ALI or severe hypoxemia after VATS talc pleurodesis. Patients with these characteristics might be at risk for adverse outcomes of talc pleurodesis and should be considered for alternative therapy for their effusions.
Subject(s)
Hypoxia/etiology , Pleurodesis/adverse effects , Respiratory Distress Syndrome/etiology , Sclerosing Solutions/adverse effects , Talc/adverse effects , Aged , Antineoplastic Agents , Edema , Female , Humans , Male , Middle Aged , Oxygen Inhalation Therapy , Pleural Effusion/surgery , Pleurodesis/methods , Retrospective Studies , Risk Factors , Thoracic Surgery, Video-AssistedABSTRACT
Gastric volvulus is a potentially lethal condition. Pneumonectomy patients have decreased physiologic reserve, and thus they are more susceptible to morbidity and mortality from postoperative complications. We report successful management of a patient with hiatal hernia that resulted in acute gastric volvulus after left pneumonectomy.
Subject(s)
Hernia, Hiatal/complications , Pneumonectomy/adverse effects , Stomach Volvulus/etiology , Adenocarcinoma/complications , Adenocarcinoma/surgery , Hernia, Hiatal/pathology , Humans , Lung Neoplasms/complications , Lung Neoplasms/surgery , Male , Middle AgedABSTRACT
Retrograde gastroesophageal intussusception has been rarely reported in the literature. Risk factors include poor fixation of the stomach due to either long or loose mesenteric attachments; high intraabdominal pressure due to retching, physical exertion, or ascites; and hiatal hernia, which can lead to the development of a large gastroesophageal opening. An attempt at endoscopic reduction is reasonable, but laparotomy and manual reduction is usually required. We report a case of retrograde gastroesophageal intussusception in a patient with long-standing achalasia and two previous Heller myotomies.
Subject(s)
Esophageal Achalasia/surgery , Esophageal Diseases/etiology , Intussusception/etiology , Stomach Diseases/etiology , Female , Humans , Intussusception/diagnostic imaging , Middle Aged , Postoperative Complications , Tomography, X-Ray ComputedABSTRACT
Acquired inhibitors to clotting factors most commonly involve factor VIII and are associated with autoimmune disease. Factor VIII inhibitors can cause severe spontaneous and iatrogenic bleeding that is difficult to manage. Factor VIII inhibitors are rarely associated with solid tumors and only three cases of adenocarcinoma of the lung have been reported. This report describes the multidisciplinary management of a factor VIII inhibitor-producing stage Ia lung adenocarcinoma that ultimately resulted in complete resectability.
Subject(s)
Adenocarcinoma/blood , Autoantibodies/immunology , Autoimmune Diseases/etiology , Blood Coagulation Factors/therapeutic use , Blood Loss, Surgical/prevention & control , Factor VIII/immunology , Hemorrhage/etiology , Lung Neoplasms/blood , Paraneoplastic Syndromes/etiology , Pneumonectomy , Adenocarcinoma/complications , Adenocarcinoma/drug therapy , Adenocarcinoma/immunology , Adenocarcinoma/pathology , Adenocarcinoma/surgery , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Autoimmune Diseases/immunology , Carboplatin/administration & dosage , Combined Modality Therapy , Cyclophosphamide/therapeutic use , Diabetes Mellitus, Type 2/complications , Factor VIII/antagonists & inhibitors , Hemorrhage/immunology , Humans , Hyperglycemia/chemically induced , Hyperglycemia/etiology , Immunoglobulins, Intravenous/therapeutic use , Immunosuppressive Agents/therapeutic use , Lung Neoplasms/complications , Lung Neoplasms/drug therapy , Lung Neoplasms/immunology , Lung Neoplasms/pathology , Lung Neoplasms/surgery , Male , Neoplasm Staging , Paclitaxel/administration & dosage , Paraneoplastic Syndromes/immunology , Partial Thromboplastin Time , Prednisone/adverse effects , Prednisone/therapeutic useABSTRACT
Benign metastasizing leiomyoma is a rare condition affecting women with a history of uterine leiomyomata and is characterized by multiple histologically benign pulmonary smooth muscle tumors. Speculations on its pathogenesis include a benign uterine leiomyoma colonizing the lung, a metastatic low-grade uterine leiomyosarcoma, and primary pulmonary leiomyomatosis. To elucidate its pathogenesis, we analyzed the clinical, pathological and immunohistochemical features, clonality, and telomere length of multiple lung and uterine tumors in three patients with benign metastasizing leiomyoma. In all cases, pulmonary tumors had benign histology and immunohistochemical profiles (estrogen receptor positive, progesterone receptor positive, and very low proliferative index) identical to uterine leiomyoma. In eight tumors from three patients, clonality was assessed by analyzing the variable length of the polymorphic CAG repeat sequence within the human androgen receptor gene. In the two informative patients pulmonary and uterine tumors showed identical patterns of androgen receptor allelic inactivation, indicating that they were clonal. The telomere length measured by fluorescence in situ hybridization in pulmonary leiomyomas of all three patients were either long or very long and were identical to the uterine counterparts, indicating significant telomere shortening is not a crucial step for developing metastases. Our evidence supports the notion that benign metastasizing leiomyoma is clonally derived from benign-appearing uterine leiomyomas.
Subject(s)
Leiomyoma/pathology , Lung Neoplasms/secondary , Uterine Neoplasms/pathology , Adult , Clone Cells/chemistry , Clone Cells/metabolism , Clone Cells/pathology , Female , Humans , Immunohistochemistry , In Situ Hybridization, Fluorescence , Leiomyoma/genetics , Leiomyoma/metabolism , Lung/diagnostic imaging , Lung/pathology , Lung Neoplasms/genetics , Lung Neoplasms/metabolism , Receptors, Androgen/genetics , Receptors, Estrogen/analysis , Receptors, Progesterone/analysis , Telomere/genetics , Tomography, X-Ray Computed , Trinucleotide Repeats/genetics , Uterine Neoplasms/genetics , Uterine Neoplasms/metabolism , X Chromosome Inactivation/geneticsABSTRACT
Gangliocytic paraganglioma (GP) is a rare, typically benign tumor that shows neuroectodermal (neurosustentacular or Schwannian and neuronal) and neuroendocrine differentiation. Once thought to arise exclusively in the periampullary region as a solitary lesion, recent reports have documented both origin of GP in a variety of extra-duodenal sites as well as synchronous multifocal presentation of the tumor. Herein, we describe the first reported case of simultaneous occurrence of GP in the superior mediastinum and esophagus. A mass in the mid-distal esophagus and a separate mass in the superior mediastinum at the thoracic inlet were found in a 58-year-old woman by computed tomography scan. Subsequent biopsy of the superior mediastinal mass showed nests of epithelioid tumor cells coexisting with ganglioneuromatous elements, whereas biopsy of the esophageal mass showed nests of epithelioid cells with interspersed ganglion cells. The epithelioid tumor cells showed diffuse immunohistochemical expression of keratin (CAM 5.2), chromogranin, and synaptophysin supporting true neuroendocrine differentiation; ganglion cells expressed S-100 protein and neurofilament protein; and the spindled elements expressed S-100 protein, neurofilament protein, and glial fibrillary acidic protein indicating Schwannian differentiation. The finding of another GP occurring outside the periampullary region bolsters the argument for a stem cell origin of this unusual tumor.