Corrigendum to "Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis".

[This corrects the article DOI: 10.1155/2020/1385978.].

Mesenteric panniculitis as a presentation of Whipple's disease: case report and review of the literature.

BACKGROUND: Whipple's disease is a rare, multi-organ disease caused by Tropheryma Whipplei. A classic presentation is characterized by arthropathy, diarrhea and weight loss but a broad spectrum of manifestations is possible. We present a case of a patient with mesenteric panniculitis as a manifestation of WD. A comprehensive review of the literature is provided. PATIENT: A 50 year old male presented at the outpatient clinic after an episode of fever and abdominal pain abroad. CT scan showed mesenteric infiltration with associated lymphadenopathies consistent with mesenteric panniculitis. After receiving 6 months of antibiotic therapy abdominal and joint pains improved. CONCLUSION: Clinicians should be aware of Whipple's disease. Mesenteric panniculitis is a rare presentation of this possible lethal infection. The golden standard for diagnosing WD is a PAS positive small bowel biopsy. Adequate antibiotic therapy is the cornerstone of treatment and usually leads to an amelioration of symptoms.

Clinical Presentation, Natural History, and Therapeutic Approach in Patients with Solitary Fibrous Tumor: A Retrospective Analysis.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare variant of soft tissue sarcoma (STS). Materials and Methods. We reviewed SFT patients (pts) treated at our institution between 12/1990 and 09/2017. RESULTS: We identified 94 pts with a median follow-up (mFU) of 4.7 years (range: 0.1-21.53). Primary sites were the chest (33%), abdomen (21.3%), brain (12.8%), and extremities (9.6%); 6.4% of pts presented with synchronous metastasis. Median overall survival (mOS) from the first diagnosis was 56.0 months (m) (0.3-258.3). Doege-Potter syndrome was seen in 2.1% of pts. Primary resection was performed in 86 pts (91.5%). Median progression-free survival was 34.1 m (1.0-157.1), and 43% of pts stayed SFT-free during FU. Local recurrence occurred in 26.7% after a mFU of 35.5 m (1.0-153.8), associated with an OS of 45.1 m (4.7-118.2). Metachronous metastasis occurred in 30.2% after a mFU of 36.0 m (0.1-157.1). OS in metastatic pts was 19.0 m (0.3-149.0). Systemic therapy was given to 26 pts (27.7%) with inoperable/metastatic disease. The most common (57.7%) upfront therapy was doxorubicin, achieving responses in 13.3% of pts with a PFS of 4.8 m (0.4-23.8). In second line, pts were treated with ifosfamide or pazopanib, the latter achieving the highest response rates. Third-line treatment was heterogeneous. CONCLUSION: SFT is an orphan malignancy with a highly variable clinical course and a considerable risk of local failure and metachronous metastasis. Surgery is the only curative option; palliative systemic therapy is used in inoperable/metastatic cases but achieves low response rates. The highest response rates are seen with pazopanib in second/third line.

Isolated local recurrence or solitary solid organ metastasis after esophagectomy for cancer is not the end of the road.

Recurrent disease after esophagectomy bears an infaust prognosis, especially when multiple recurrences are present. But little is known about survival in patients with limited recurrence (solitary locoregional recurrence or solid organ metastasis). Herein, we report our experience with these subgroups. We analyzed 1754 consecutive patients surgically treated with curative resection for esophageal cancer and cancer of the gastroesophageal junction between 1990 and 2012. Seven subgroups were defined according to the recurrence type (locoregional vs. organ metastasis), the site of recurrence (abdominal, thoracic, cervical for lymph nodes and lung, liver, adrenals and others for organ metastasis) and also the number of lesions (one vs. multiple lymph node stations or organ metastasis) Of these groups; clinical isolated locoregional recurrence (ciLR) was defined as solitary lymph-node recurrence confined to one compartment (cervical, thoracic or abdominal, within or outside surgical dissection-field) at clinical staging. Clinical solitary solid organ metastasis (csSOM) was defined as metastasis in a resectable solid organ, i.e. liver, lung, brain or adrenal. Salvage therapies were grouped in five categories. Kaplan-Meier curves were used to calculate survival. Recurrent disease was observed in 766 patients (43.7%) with overall 5-year survival of 4.5% after diagnosis of recurrence. Fifty-seven patients (7.4%) showed ciLR and 110 (14.4%) csSOM. Median time-to-recurrence was 16.8 months in ciLR and 9.9 months in csSOM (P = 0.0074). Survival is significantly improved compared to supportive therapy when local therapy is possible (P < 0.0001). In 25 (15%) of ciLR or csSOM patients, surgical therapy with or without systemic therapy, yielded a 5-year survival of 49.9% (median 54.8 months) after diagnosis of recurrence. When surgery was impossible or contraindicated, the combination of chemoradiotherapy appeared to be superior to chemotherapy alone (respectively 27.0% vs. 4.6% 5-year survival) or radiotherapy alone (no 5-year survival). Recurrent disease after esophagectomy is a common problem with poor overall survival. However prolonged survival could be obtained in selected patients if the recurrent disease is limited to ciLR or csSOM, if surgery (+/- systemic therapy) can be performed. If not a combination of chemoradiotherapy seems to offer the second best option. Patients presenting with a ciLR or csSOM should be discussed in a dedicated multidisciplinary team meeting as to evaluate and define the place of salvage treatment which in well selected cases could offer a perspective of prolonged survival.

The Development of an Electronic Portfolio for Postgraduate Surgical Training in Flanders.

BACKGROUND: Contemporary surgical postgraduate training is characterized by clear outcomes for the profession and an assessment program that shows that trainees master these outcomes. The tool used to collect assessment and feedback instruments is the portfolio, nowadays used in many countries worldwide. METHODS: The four Flemish surgical coordinators, together with experts from different universities, devised an electronic portfolio. This portfolio holds both the logbook, as imposed by the evaluation committee and assessment instruments used for the Master in Specialized Medicine. RESULTS: The e-portfolio is now used by a number of surgical trainees and has been approved by the evaluation committee. In 2015, all Flemish surgical trainees will be using one and the same e-portfolio. CONCLUSIONS: Although the e-portfolio for surgical training has now been devised and accepted by all major parties involved, a lot of work has to be done to implement the instrument. As resident duty hours show no improvement on education in surgery (but rather a perception of worsened education) surgery training is fazing huge challenges.

2nd ESMO Consensus Conference in Lung Cancer: locally advanced stage III non-small-cell lung cancer.

To complement the existing treatment guidelines for all tumour types, ESMO organises consensus conferences to focus on specific issues in each type of tumour. The 2nd ESMO Consensus Conference on Lung Cancer was held on 11-12 May 2013 in Lugano. A total of 35 experts met to address several questions on non-small-cell lung cancer (NSCLC) in each of four areas: pathology and molecular biomarkers, first-line/second and further lines of treatment in advanced disease, early-stage disease and locally advanced disease. For each question, recommendations were made including reference to the grade of recommendation and level of evidence. This consensus paper focuses on locally advanced disease.

The Development of an Electronic Portfolio for Postgraduate Surgical Training in Flanders.

BACKGROUND: Contemporary surgical postgraduate training is characterized by clear outcomes for the profession and an assessment program that shows that trainees master these outcomes. The tool used to collect assessment and feedback instruments is the portfolio, nowadays used in many countries worldwide. METHODS: The four Flemish surgical coordinators, together with experts from different universities, devised an electronic portfolio. This portfolio holds both the logbook, as imposed by the evaluation committee and assessment instruments used for the Master in Specialized Medicine. RESULTS: The e-portfolio is now used by a number of surgical trainees and has been approved by the evaluation committee. In 1015, all Flemish surgical trainees will be using one and the same e-portfolio. CONCLUSION: Although the e-portfolio for surgical training has now been devised and accepted by all major parties involved, a lot of work has to be done to implement the instrument. As resident duty hours show no improvement on education in surgery (but rather a perception of worsened education) surgery training is fazing huge challenges.

Single-Centre 20-Year Experience with Surgical Treatment of Thymic Tumours.

BACKGROUND: Large single-centre institutional series on thymic tumours are rare. Complete resection remains the mainstay of successful treatment. Characteristics and survival were reviewed in all patients treated between 19932013. METHODS: Hospital databases revealed 134 patients with pathologically-proven thymic tumour. Follow-up (median 63 months) was through patient notes and telephone contact with general practitioner. RESULTS: Patients were classified in Masaoka-Koga stages: I: 50 (37%); Ila: 14 (10%); lib: 14 (10%); III: 27 (20%); IVa: 19 (14%); IVb: 4 (3%); unknown: 6 (5%). According to WHO classification, pathological subtypes were A: 19 (14%); AB: 25 (19%); B1: 21 (16%); B2: 31 (23%); B3: 15 (11%); thymic carcinoma: 23 (17%). Parathymic syndromes were diagnosed in 45 patients: myasthenia gravis (84%); pure red-cell aplasia (4%); hypogammaglobulinemia (2%); and others. 124 patients (93%) underwent surgery with complete resection in 104 (84%). Surgical approach was: sternotomy: 79; thoracotomy: 35; cervicotomy: 2; other/unknown: 8. In 73 patients (59%) no biopsy was taken prior to surgical resection, 25 were treated with induction chemotherapy, 36 received adjuvant radiotherapy. Hospital mortality was 0.81%. 35 patients died during follow-up (13 of tumour or treatment-related causes). Overall and recurrence-free survival at 5, 10, and 15 years were 86%; 64%; 47% and 67%; 49%; and 31%, respectively and were significantly (p < 0.01) different according to Masaoka-Koga stage. There was a significant association between WHO classification and Masaoka-Koga stages I-IIa-IIb versus III-IVa-IVb (p < 0.01). CONCLUSIONS: Operability and complete resectability of thymic tumours in our experience is high resulting in prolonged overall and recurrence-free survival. Masaoka-Koga stage is an important predictor for survival and shows a significant association with WHO classification.

Lobar lung transplantation from deceased donors: a valid option for small-sized patients with cystic fibrosis.

BACKGROUND: Small-sized patients with cystic fibrosis usually face long waiting times for a suitable lung donor. Reduced-size lung transplantation (LTx) was promoted to shorten waiting times. We compared donor and recipient characteristics and outcome in lobar ([L]) versus full-size ([FS]) lung recipients. METHODS: Between July 1, 1991, and February 28, 2011, 535 isolated LTx were performed, including 74 in cystic fibrosis patients (8 L, 66 FS). Patients were followed up until September 2012. RESULTS: [L] recipients were younger, smaller, and lighter. Sex, waiting times, and donor data (age, sex, height, weight, PaO2/FiO2, and ventilation time) were comparable. Cardiopulmonary bypass was used more often in [L]; cold ischemia was comparable for first lung but longer in [L] for second lung; implantation times were comparable. In-hospital mortality rate was 0% in [L] versus 3% in [FS]. Both intensive care unit and hospital stay were longer in [L]. Grade 3 primary graft dysfunction was more pronounced in [L] at T0 and at T48. FEV1 increased significantly in both groups from preoperative value. Bronchiolitis obliterans syndrome was absent in [L] and diagnosed in 18 patients in [FS], accounting for 6 of 15 late deaths. All [L] are still alive. No differences in survival were found between the groups. CONCLUSIONS: Although hindered by a higher incidence of primary graft dysfunction, L-LTx is a viable option with excellent survival and pulmonary function comparable to FS-LTx.

Combined liver and lung transplantation with extended normothermic lung preservation in a patient with end-stage emphysema complicated by drug-induced acute liver failure.

Isolated lung transplantation (LuTx) and liver transplantation are established treatments for irreversible lung and liver failure. Combined liver and lung transplantation (cLiLuTx) is a less common, but approved therapy of combined organ failure, mostly applied in patients suffering from progressive cystic fibrosis and advanced liver disease. We report a patient who was listed for LuTx due to end-stage chronic obstructive pulmonary disease and who developed drug-induced acute hepatic failure. The only therapeutic option was hyper-urgent cLiLuTx. To correct the poor coagulation in order to reduce the per-operative risk of bleeding, the liver was transplanted first. In anticipation of the longer lung preservation time, cold flushed lungs were preserved on a portable lung perfusion device for ex vivo normothermic perfusion for 11 h 15 min, transplanted sequentially off-pump, and reperfused after a total ex vivo time of 13 h 32 min and 16 h for the first and second lung, respectively. Ten months later, the patient is doing well and no rejection occurred. Normothermic ex vivo lung perfusion may help to prolong preservation time, facilitating long-distance transport and combined organ transplantation.

Randomized phase 2 trial on refinement of early-stage NSCLC adjuvant chemotherapy with cisplatin and pemetrexed versus cisplatin and vinorelbine: the TREAT study.

BACKGROUND: Adjuvant chemotherapy is beneficial in non-small-cell lung cancer (NSCLC). However, balancing toxicity and efficacy mandates improvement. PATIENTS AND METHODS: Patients with completely resected stages IB-pT3N1 NSCLC were randomly assigned to either four cycles cisplatin (C: 50 mg/m(2) day (d)1 + 8) and vinorelbine (V: 25 mg/m(2) d1, 8, 15, 22) q4 weeks or four cycles cisplatin (75 mg/m(2) d1) and pemetrexed (Px: 500 mg/m(2) d1) q3 weeks. Primary objective was the clinical feasibility rate (no grade (G)4 neutropenia/thrombocytopenia or thrombocytopenia with bleeding, no G3/4 febrile neutropenia or non-hematological toxicity; no premature withdrawal/death). Secondary objectives were drug delivery and efficacy. RESULTS: One hundred and thirty two patients were randomized (stages: 38% IB, 10% IIA, 47% IIB, 5% pT3pN1; histology: 43% squamous, 57% non-squamous). The feasibility rates were 95.5% (cisplatin and pemetrexed, CPx) and 75.4% (cisplatin and vinorelbine, CVb) (P = 0.001); hematological G3/4 toxic effects were 10% (CPx) and 74% (CVb) (P < 0.001), non-hematological toxic effects were comparable (33% and 31%, P = 0.798). Delivery of total mean doses was 90% of planned with CPx, but 66% (cisplatin) and 64% (vinorelbine) with CVb (P < 0.0001). The median number of cycles [treatment time (weeks)] was 4 for CPx (11.2) and 3 for CVb (9.9). Time to withdrawal from therapy differed significantly between arms favoring CPx (P < 0.001). CONCLUSION: Adjuvant chemotherapy with CPx is safe and feasible with less toxicity and superior dose delivery compared with CVb.

Learning curve in tracheal allotransplantation.

The first vascularized tracheal allotransplantation was performed in 2008. Immunosuppression was stopped after forearm implantation and grafting of the recipient mucosa to the internal site of the transplant. Nine months after forearm implantation, the allograft was transplanted to the tracheal defect on the radial blood vessels. Since then, four additional patients have undergone tracheal allotransplantation, three (patients 2-4) for long-segment stenosis and one (patient 5) for a low-grade chondrosarcoma. Our goal was to reduce the time between forearm implantation and orthotopic transplantation and to determine a protocol for safe withdrawal of immunosuppressive therapy. Following forearm implantation, all transplants became fully revascularized over 2 months. Withdrawal of immunosuppression began 4 months after graft implantation and was completed within 6 weeks in cases 2-4. Repopulation of the mucosal lining by recipient cells, to compensate for the necrosis of the donor mucosa, was not complete. This resulted in partial loss of the allotransplant in patients 2-4. In patient 5, additional measures promoting recipient cell repopulation were made. The trachea may be used as a composite tissue allotransplant after heterotopic revascularization in the forearm. Measures to maximize recipient cell repopulation may be important in maintaining the viability of the transplant after cessation of immunosuppression.

The TERT-CLPTM1L locus for lung cancer predisposes to bronchial obstruction and emphysema.

Clinical studies suggest that bronchial obstruction and emphysema increase susceptibility to lung cancer. We assessed the possibility of a common genetic origin and investigated whether the lung cancer susceptibility locus on chromosome 5p15.33 increases the risk for bronchial obstruction and emphysema. Three variants in the 5p15.33 locus encompassing the TERT and CLPTM1L genes were genotyped in 777 heavy smokers and 212 lung cancer patients. Participants underwent pulmonary function tests and computed tomography of the chest, and completed questionnaires assessing smoking behaviour. The rs31489 C-allele correlated with reduced forced expiratory volume in 1 s (p=0.006). Homozygous carriers of the rs31489 C-allele exhibited increased susceptibility to bronchial obstruction (OR 1.82, 95% CI 1.24-2.69; p=0.002). A similar association was observed for diffusing capacity of the lung for carbon monoxide (p=0.004). Consistent with this, CC-carriers had an increased risk of emphysema (OR 2.04, 95% CI 1.41-2.94; p=1.73 × 10(-4)) and displayed greater alveolar destruction. Finally, CC-carriers also had an increased risk for lung cancer (OR 1.90, 95% CI 1.21-2.99; p=0.005), and were more susceptible to developing both lung cancer and bronchial obstruction than lung cancer alone (OR 2.11, 95% CI 1.04-4.26; p=0.038). The rs31489 variant on 5p15.33 is associated with bronchial obstruction, presence and severity of emphysema, and lung cancer.

Endoscopic thoracic sympathectomy for posttraumatic complex regional pain syndrome.

INTRODUCTION: Posttraumatic complex regional pain syndrome (CRPS) has a strongly negative impact on rehabilitation and activities of daily living. Treatment is most often unrewarding. AIM: To analyze the efficacy of endoscopic thoracic sympathectomy (ETS) in reducing pain and disability associated with CRPS prospectively. PATIENT AND METHODS: Over a 5-year period, 12 patients (7 females and 5 males; median age 46.5 [range 34-60 years]) with posttraumatic CRPS underwent unilateral ETS. The median duration of CRPS symptoms before ETS was 3.8 months (range 1.2-19.9). The sympathetic chain was resected from the 2nd to the 5th rib, and the nerve of Kuntz was severed. Median postoperative 16 months (range 12-40). Pain was assessed, at rest (passive) and during movement (active), using a visual analogue scale (VAS) from 0 to 10. RESULTS: One patient (8%) suffered a hydrothorax and 3 patients (25%) complained of contralateral compensatory hyperhydrosis. At 1 month (n = 12), 2 months (n = 7), 6 months (n = 12), and 1 year (n = 12) after ETS, there was a significant decrease in passive and active VAS (P < 0.05). Ten out of the 12 patients (83%) needed fewer analgesics after surgery, and eight (67%) did not need analgesics at all. The median sleep duration improved significantly from a preoperative value of 2 h (range 1-7) to a postoperative value of 6.25 h (range 3.5-8) (P < 0.001). Overall, patient satisfaction was 83%. CONCLUSION: ETS is effective at decreasing pain and improving quality of life, and should therefore be considered in the treatment of CRPS.

Early stage non-small-cell lung cancer: challenges in staging and adjuvant treatment: evidence-based staging.

Staging of non-small-cell lung cancer is a multidisciplinary process involving imaging, endoscopic and surgical techniques. Accuracy is vital in order to avoid false-positive interpretations leading to a false stage III or IV diagnosis in early stage patients, or false-negative findings leading to a false early stage diagnosis in patients with mediastinal lymph node disease. CT scan offers great anatomical detail of tumour spread, but radiological imaging lacks information on the biological nature of the lesions. The latter is brought in by 2-[fluorine-18] fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET) scan as a metabolic imaging tool, which, however, has clearly lower spatial resolution. Therefore, contemporary staging relies on the combination of both, preferably in a fusion PET-CT scan. Absence of suspected lymph node metastasis on both CT and PET has a high negative predictive value, and these patients may in general proceed to surgery. In most others, tissue confirmation of the locoregional lymph node status is needed. The historical standard of mediastinoscopy is nowadays complemented by endoscopic techniques by the bronchial or esophageal approach. Each of these techniques remains important in modern staging algorithms. A practical scheme for rational staging in clinical practice is discussed.

Cervical videomediastinoscopy.

Cervical mediastinoscopy is a frequently used technique to assess the mediastinum, in particular the mediastinal lymph nodes in patients presenting with non-small cell lung cancer (NSCLC). The senior author of this article is credited for developing in 1989 the concept of what is now called videomediastinoscopy. The introduction of videomediastinoscopy has proven to be superior to conventional mediastinoscopy and has made teaching of this operation much easier. However, imaging modalities, in particular positron emission tomography, have substantially decreased the need for mediastinoscopy in early stage NSCLC, while in more advanced stages the indication for primary staging and/or restaging after induction therapy is now challenged by the increasing experience with endobronchial ultrasound, endoesophageal ultrasound, and fine-needle aspiration. This article discusses the current deployment of videomediastinoscopy in the diagnosis and management of NSCLC.

Morbidity and mortality after induction chemotherapy followed by surgery in IIIa-N2 non small cell lung cancer.

OBJECTIVE: To evaluate the frequency and risk of postoperative complications and mortality in patients with IIIa-N2 non small cell lung cancer after induction chemotherapy and surgery. METHODS: In a surgical database records from ninety two patients, operated between January 1, 2000 and December 31, 2006 were reviewed. Univariate analysis was used to identify predictors of postoperative complications and in-hospital mortality. RESULTS: All cases were histologically confirmed stage IIIa-N2. All patients received preoperative platinum based chemotherapy without radiotherapy. Pneumonectomy was performed in 20 cases (23.5%), from which 9 right sided. (Bi)lobectomy was performed in 53 cases (62.4%) and sleeve lobectomy in 11 cases (17.2%). One wedge resection was performed (1.2%). In 7 cases (7.6%) only an exploration was done. Complications developed in 35 patients (38%). Major complications in 15 patients (16%). No bronchopleural fistulae were observed. Analysis identified increased age and high physiological and operative severity score for the enumeration of mortality and morbidity (POSSUM) as a risk factor to develop complications, and a high simplified comorbidity score as a risk factor to develop a major complication. Higher age, Charlson comorbidity index, simplified comorbidity score and POSSUM were a risk factor for developing pneumonia. CONCLUSION: Although surgery after induction therapy for IIIa-N2 NSCLC can be done with a morbidity and mortality comparable to surgery alone, it remains a high risk operation. It should therefore be performed in a center with experience. Bronchial stump protection should be used whenever there is an increased risk for developing a bronchopleural fistula. In deciding whether to do surgery or radiotherapy one should keep in mind the feasibility of performing a complete resection together with a preoperative assessment to predict complications and mortality. For the preoperative assessment several scoring systems can be used from which we find the simplified comorbidity score most useful.

Internal herniation after Roux-en-Y gastric bypass: case reports and a review of the literature.

Internal herniation, defined as a protrusion of the bowel through a peritoneal or mesenteric aperture into a compartment of the abdominal cavity, is a potentially serious complication following Roux-en-Y gastric bypass. In view of the high risk of strangulation and volvulus with possible progression to necrotic bowel, sepsis and multiple organ failure, early diagnosis and prompt surgical intervention are crucial to minimise morbidity and mortality rates. As little is known about internal herniation, pathogenesis, consequences, clinical presentation and diagnostic approach are discussed in this article. A review of the literature and the consideration of three cases make it clear that diagnosis is often delayed due to a vague, non-specific clinical presentation and the frequently negative radiological investigations. Displacement of the superior mesenteric artery (SMA) with crowding, stretching and engorgement of its visceral branches, mesenteric swirl and a clustered appearance of small bowel loops are CT findings with a high specificity but low sensitivity. A high degree of vigilance, communication between radiologists and surgeons and a low threshold for surgical exploration is therefore necessary.

Bilateral lobar lung transplantation--the first two cases in Belgium.

In the last twenty years lung transplantation has become an established treatment for end-stage lung failure refractory to medical management. Over this time, better short and long-term results have been achieved due to improvements in organ procurement, perfusion and preservation strategies, newer immunosuppressant regimes and better post-transplant care. The limiting factor for the number of lung transplantation procedures performed is the shortage of available donor organs. This results in longer waiting times for listed patients, with a substantially increased risk of dying prior to transplantation, especially in the paediatric population. Several surgical strategies have evolved to overcome the donor shortage, with lobar transplantation becoming a viable alternative. We describe our initial experience with two young patients with end-stage cystic fibrosis (CF) who required lung transplantation. Given their small size it was not possible to transplant an entire lung from an adult donor in each hemithorax. We describe lobar transplantation as a technique used to overcome this, in the first such operation in Belgium.