Severe allergic dysregulation due to a gain of function mutation in the transcription factor STAT6.

BACKGROUND: Inborn errors of immunity have been implicated in causing immune dysregulation, including allergic diseases. STAT6 is a key regulator of allergic responses. OBJECTIVES: This study sought to characterize a novel gain-of-function STAT6 mutation identified in a child with severe allergic manifestations. METHODS: Whole-exome and targeted gene sequencing, lymphocyte characterization, and molecular and functional analyses of mutated STAT6 were performed. RESULTS: This study reports a child with a missense mutation in the DNA binding domain of STAT6 (c.1114G>A, p.E372K) who presented with severe atopic dermatitis, eosinophilia, and elevated IgE. Naive lymphocytes from the affected patient displayed increased TH2- and suppressed TH1- and TH17-cell responses. The mutation augmented both basal and cytokine-induced STAT6 phosphorylation without affecting dephosphorylation kinetics. Treatment with the Janus kinase 1/2 inhibitor ruxolitinib reversed STAT6 hyperresponsiveness to IL-4, normalized TH1 and TH17 cells, suppressed the eosinophilia, and improved the patient's atopic dermatitis. CONCLUSIONS: This study identified a novel inborn error of immunity due to a STAT6 gain-of-function mutation that gave rise to severe allergic dysregulation. Janus kinase inhibitor therapy could represent an effective targeted treatment for this disorder.

Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency.

BACKGROUND: LPS-responsive beige-like anchor (LRBA) deficiency (LRBA-/-) and cytotoxic T-lymphocyte-associated antigen-4 (CTLA4) insufficiency (CTLA4+/-) are mechanistically overlapped diseases presenting with recurrent infections and autoimmunity. The effectiveness of different treatment regimens remains unknown. OBJECTIVE: Our aim was to determine the comparative efficacy and long-term outcome of therapy with immunosuppressants, CTLA4-immunoglobulin (abatacept), and hematopoietic stem cell transplantation (HSCT) in a single-country multicenter cohort of 98 patients with a 5-year median follow-up. METHODS: The 98 patients (63 LRBA-/- and 35 CTLA4+/-) were followed and evaluated at baseline and every 6 months for clinical manifestations and response to the respective therapies. RESULTS: The LRBA-/- patients exhibited a more severe disease course than did the CTLA4+/- patients, requiring more immunosuppressants, abatacept, and HSCT to control their symptoms. Among the 58 patients who received abatacept as either a primary or rescue therapy, sustained complete control was achieved in 46 (79.3%) without severe side effects. In contrast, most patients who received immunosuppressants as primary therapy (n = 61) showed either partial or no disease control (72.1%), necessitating additional immunosuppressants, abatacept, or transplantation. Patients with partial or no response to abatacept (n = 12) had longer disease activity before abatacept therapy, with higher organ involvement and poorer disease outcomes than those with a complete response. HSCT was performed in 14 LRBA-/- patients; 9 patients (64.2%) showed complete remission, and 3 (21.3%) continued to receive immunosuppressants after transplantation. HSCT and abatacept therapy gave rise to similar probabilities of survival. CONCLUSIONS: Abatacept is superior to immunosuppressants in controlling disease manifestations over the long term, especially when started early, and it may provide a safe and effective therapeutic alternative to transplantation.

Optimal waiting period to surgical treatment after neoadjuvant chemoradiotherapy for locally advanced rectum cancer: a retrospective observational study.

BACKGROUND: The optimal waiting period after neoadjuvant treatment in patients with locally advanced rectal cancers is still controversial. The literature has different results regarding the effect of waiting periods on clinical and oncological outcomes. We aimed to investigate the effects of these different waiting periods on clinical, pathological, and oncological outcomes. METHODS: Between January 2014 and December 2018, a total of 139 consecutive patients with locally advanced rectal adenocarcinoma, who were treated in the Department of General Surgery at the Marmara University Pendik Training and Research Hospital, were enrolled in the study. The patients were split into three groups according to waiting time for surgery after neoadjuvant treatment: group 1 (n = 51) included patients that have 7 weeks and less (≤ 7 weeks) time interval, group 2 (n = 45) 8 to 10 weeks (8-10 weeks), group 3 (n = 43) 11 weeks and above (11 weeks ≤). Their database records, which were entered prospectively, were analyzed retrospectively. RESULTS: There were 83 (59.7%) males and 56 (40.3%) females. The median age was 60 years, and there was no statistical difference between the groups regarding age, gender, BMI, ASA score, ECOG performance score, tumor location, and preoperative CEA values. Also, we found no significant differences regarding operation times, intraoperative bleeding, length of hospital stay, and postoperative complications. According to the Clavien-Dindo (CD) classification, severe early postoperative complications (CD 3 and above) were observed in 9 patients. The complete pathological response (pCR, ypT0N0) was observed in 21 (15.1%) patients. The groups had no significant difference regarding 3-year disease-free and 3-year overall survival (p = 0.3, p = 0.8, respectively). Local recurrence was observed in 12 of 139 (8.6%) patients and distant metastases occurred in 30 of 139 (21.5%) patients during the follow-up period. There was no significant difference between the groups in terms of both local recurrence and distant metastasis (p = 0.98, p = 0.43, respectively). CONCLUSION: The optimal time for postoperative complications and sphincter-preserving surgery in patients with locally advanced rectal cancer is 8-10 weeks. The different waiting periods do not affect disease-free and overall survival. While long-term waiting time does not make a difference in pathological complete response rates, it negatively affects the TME quality rate.

Infiltration pattern predicts metastasis and progression better than the T-stage and grade in pancreatic neuroendocrine tumors: a proposal for a novel infiltration-based morphologic grading.

The advancing edge profile is a powerful determinant of tumor behavior in many organs. In this study, a grading system assessing the tumor-host interface was developed and tested in 181 pancreatic neuroendocrine tumors (PanNETs), 63 of which were <=2 cm. Three tumor slides representative of the spectrum (least, medium, and most) of invasiveness at the advancing edge of the tumor were selected, and then each slide was scored as follows. Well-demarcated/encapsulated, 1 point; Mildly irregular borders and/or minimal infiltration into adjacent tissue, 2 points; Infiltrative edges with several clusters beyond the main tumor but still relatively close, and/or satellite demarcated nodules, 3 points; No demarcation, several cellular clusters away from the tumor, 4 points; Exuberantly infiltrative pattern, scirrhous growth, dissecting the normal parenchymal elements, 5 points. The sum of the rankings on the three slides was obtained. Cases with scores of 3-6 were defined as "non/minimally infiltrative" (NI; n = 77), 7-9 as "moderately infiltrative" (MI; n = 68), and 10-15 as "highly infiltrative" (HI; n = 36). In addition to showing a statistically significant correlation with all the established signs of aggressiveness (grade, size, T-stage), this grading system was found to be the most significant predictor of adverse outcomes (metastasis, progression, and death) on multivariate analysis, more strongly than T-stage, while Ki-67 index did not stand the multivariate test. As importantly, cases <=2 cm were also stratified by this grading system rendering it applicable also to this group that is currently placed in "watchful waiting" protocols. In conclusion, the proposed grading system has a strong, independent prognostic value and therefore should be considered for integration into routine pathology practice after being evaluated in validation studies with larger series.

Pulmonary artery sarcoma treated with pulmonary endarterectomy and leaflet reconstruction using Ozaki technique.

Pulmonary arterial intimal sarcomas (PAIS) are rare malignancies with a poor prognosis. Sarcomas present with signs and symptoms mimicking pulmonary thromboembolic disease, delaying the diagnosis. We present a 29-year-old male patient diagnosed with PAIS in the right and main pulmonary arteries extending to the left pulmonary leaflet. The patient was treated with pulmonary endarterectomy and pulmonary leaflet reconstruction using the Ozaki technique.

Outcome of solid and cavitary pulmonary nodules in rheumatoid arthritis patients-case series.

BACKGROUND: Rheumatoid pulmonary nodule can be detected in up to 32% of rheumatoid arthritis (RA) patients and approximately one-third of nodules may cavitate. We aimed to evaluate characteristics of patients with RA developing cavitary pulmonary nodular (CPN) lesions under disease-modifying antirheumatic drugs (DMARDs), follow-up of both cavitary and solid nodules, and their outcome with the treatment. METHODS: RA patients who presented with CPN lesions during follow-up were recruited retrospectively in this case series analysis. Total numbers and mean diameters of cavitary and solid nodules in each thorax computed tomography (CT) have been determined and followed up by two experienced pulmonary physicians. Moreover, changes in treatment after the development of the CPN lesions and characteristics of cavitary nodules were collected. RESULTS: Eleven patients with CPN lesions were reported. At the time of CPN diagnosis, more patients were taking leflunomide than methotrexate (81% vs 19%). Half of the patients were receiving biologic therapy and only 18% were taking anti-TNF drugs. After a median of 24 (3-65) months of follow-up, the regression of CPN lesions was determined in 45% (5/11) of patients. Four of these 5 (80%) patients were switched to a treatment regimen without leflunomide and three of them to nonanti-TNF biologic treatment or targeted synthetic DMARDs (tocilizumab, tofacitinib, and rituximab). DISCUSSION: CPN lesions seen in RA patients are often pulmonary manifestations of the underlying disease; however, one must rule out malignancies or infections. If lesions progress under DMARDs, it is advised to discontinue synthetic DMARDs (LEF/MTX) and switch to another biological DMARD with different modes of action.

Endobronchial ultrasound transbronchial needle aspiration in elderly patients: safety and performance outcomes EBUS-TBNA in elderly.

AIM: Complication rates are low and endobronchial ultrasound guided needle aspiration (EBUS-TBNA) is generally regarded as a safe procedure, but there is a very limited number of studies evaluating the efficacy and safety of the procedure in advanced ages. The aim of this study is to assess the safety and performance outcomes of EBUS-TBNA in elderly. METHODS: It was a retrospective observational study; patients who received EBUS-TBNA between September 2016 and January 2018 were evaluated. We analyzed patient's characteristics, doses of midazolam, and lidocaine used, regions of lymph node biopsies, and complications. Also, functionality and general physical status of patients over 65 years of age were evaluated. RESULTS: During study period 132 cases of EBUS-TBNA were evaluated. 39 (29.5%) cases were aged 70 years, and over. There were more comorbidities in older group. Performance status of older group was worse. Furthermore, when evaluated according to American College of Cardiology (ACC)/American Heart Association (AHA) and American Society of Anesthesiologists (ASA), the older group was found to be composed of the riskier patients. When patients aged between 65 and 69, and over 70 compared, older patient's Barthel, EQ 5-D, SGA, and G8 scores were found to be worse. Despite that, there was no difference in the frequency, and types of complications between both groups. Diagnostic performance was not different between age groups. CONCLUSIONS: Independent from comorbidities, general health status, and functionality EBUS-TBNA procedure in 70-year-old and over patients is a safe minimally invasive procedure.

Problems in the reproducibility of classification of small lung adenocarcinoma: an international interobserver study.

AIMS: The 2015 WHO classification for lung adenocarcinoma (ACA) provides criteria for adenocarcinoma in situ (AIS), minimally invasive adenocarcinoma (MIA) and invasive adenocarcinoma (INV), but differentiating these entities can be difficult. As our understanding of prognostic significance increases, inconsistent classification is problematic. This study assesses agreement within an international panel of lung pathologists and identifies factors contributing to inconsistent classification. METHODS AND RESULTS: Sixty slides of small lung ACAs were reviewed digitally by six lung pathologists in three rounds, with consensus conferences and examination of elastic stains in round 3. The panel independently reviewed each case to assess final diagnosis, invasive component size and predominant pattern. The kappa value for AIS and MIA versus INV decreased from 0.44 (round 1) to 0.30 and 0.34 (rounds 2 and 3). Interobserver agreement for invasion (AIS versus other) decreased from 0.34 (round 1) to 0.29 and 0.29 (rounds 2 and 3). The range of the measured invasive component in a single case was up to 19.2 mm among observers. Agreement was excellent in tumours with high-grade cytology and fair with low-grade cytology. CONCLUSIONS: Interobserver agreement in small lung ACAs was fair to moderate, and improved minimally with elastic stains. Poor agreement is primarily attributable to subjectivity in pattern recognition, but high-grade cytology increases agreement. More reliable methods to differentiate histological patterns may be necessary, including refinement of the definitions as well as recognition of other features (such as high-grade cytology) as a formal part of routine assessment.

Is perineural invasion (PN) a determinant of disease free survival in early stage colorectal cancer?

BACKGROUND/AIMS: The prognostic importance of perineural invasion (PN) in colorectal cancer (CRC) is unclear. The aim of this study to find out whether the PN was an independent stratification factor of postoperative relapse in curatively resected high-risk stage II & III CRC patients who were treated with adjuvant therapy. METHODOLOGY: Data of patients with high risk stage II & all stage III CRCs treated with adjuvant chemotherapy were retrospectively analyzed. Pathological features of final surgical specimen were noted. Disease-free survival was determined by Kaplan-Meier estimator, with differences determined by multivariate analysis using the Cox multiple hazards model. Results were compared using the log-rank test. RESULTS: PN was found to be positive in 26% in the files of 593 eligible patients. In 21% of the reports PN status was not reported. Presence of PN in the resected primary tumors did not have independent effect on DFS. Further analyses for importance of PN on DFS of colon or rectal cancers did not show any effect. CONCLUSIONS: This study had failed to demonstrate any prognostic effect of PN for DFS in surgically resected stage II and III CRC patients who received adjuvant treatments.

p95-HER2 and trastuzumab resistance in metastatic breast cancer; is immunohistochemistry appropriate?

PURPOSE: Unraveling the mechanisms underlying the resistance to trastuzumab is important for amending the prognosis of patients with human epidermal growth factor receptor 2 (HER2) positive metastatic breast cancer. Experimentally, it has been shown that p95-HER2 positive breast tumors are resistant to trastuzumab. The aim of this study was to investigate the predictive and prognostic importance of p95-HER2 expression by immunohistochemistry in HER2-positive metastatic breast cancer patients treated with trastuzumab. METHODS: Only patients who had a histological diagnosis of HER2-positive metastatic breast cancer and who had received first line therapy containing trastuzumab were enrolled in the study. Immunohistochemistry was used to analyze p95-HER2 expression in the tissue blocks of the patients. RESULTS: The study was performed on 38 patients aged between 30 and 84 years. In 14 patients (36.8%), p95-HER2 was positive, whereas it was negative in the remaining 24 patients (63.2%). There was no significant correlation between p95-HER2 expression and overall survival, response to trastuzumab, and progression-free survival (PFS). CONCLUSION: Unlike previous reports, there was no correlation between the p95-HER2 expression and resistance to trastuzumab. It may be argued that an analysis using immunohistochemistry is inadequate for determining p95- HER2. In order to ascertain whether immunohistochemistry is an appropriate method, studies with larger patient groups are needed.

PTEN loss is not associated with trastuzumab resistance in metastatic breast cancer.

PURPOSE: Although the clinical benefits of trastuzumab are well known, intrinsic or acquired resistance is a commonly encountered clinical condition. A potential resistance mechanism is aberrant downstream signal transmission due to loss of phosphatase and tensine homologue (PTEN). This study investigated the relationship between trastuzumab response and loss of PTEN in metastatic breast cancer patients. METHODS: Patients with histologically confirmed human epidermal growth factor receptor 2 (HER2) positive metastatic breast cancer, who were treated with trastuzumab were enrolled into the study. PTEN expression was immunohistochemically evaluated. RESULTS: The patient median age was 50 years. Of 38 patients, 6 (15.8%) showed PTEN loss. No statistically significant difference was found between trastuzumab response, overall survival (OS) and progression-free survival (PFS) and PTEN loss (p=0.538). CONCLUSION: The activation of phosphatidylinositol 3-kinase (PI3K) pathway resulting from PTEN loss was not found to be correlated with trastuzumab response and survival. PTEN loss should not lead to exclusion of patients from the potential to benefit from trastuzumab administration.

Novel ALK immunohistochemistry assay (clone OTI1A4, Dako) is a sensitive, reliable marker for identifying ALK rearrangements in lung adenocarcinomas: A validation study.

OBJECTIVES: We present the first study validating the recent Dako ALK assay (clone OTI1A4, in vitro diagnostic) for detecting ALK rearrangements in lung adenocarcinoma. METHODS: Lung adenocarcinoma cases between 2011 and 2023 were retrospectively collected to create a cohort of 203 samples. Cases were stained with Dako ALK OTI1A4 and Ventana ALK D5F3 and reviewed by 3 pathologists independently. Correlation between assays, including their sensitivity and specificity, was evaluated. RESULTS: The cohort (n = 203) consisted of resections, core needle biopsies, and cell blocks. Agreement between Dako ALK OTI1A4 and Ventana ALK D5F3 assays was "almost perfect," with κ = 0.89. The sensitivity and specificity of the Dako ALK OTI1A4 assay were 93.3% and 96%, respectively, in a subgroup of 55 molecularly confirmed cases (n = 30 with and n = 25 without ALK rearrangement). CONCLUSIONS: Immunohistochemistry-based assays provide a valid and reasonably priced alternative, especially in settings where molecular confirmatory tests are neither offered nor accessible. Given high interassay and molecular concordance, we propose that the novel Dako OTI1A4 assay can be reliably used to identify cases with ALK rearrangement.

Intra-ampullary Papillary Tubular Neoplasm (IAPN): Clinicopathologic Analysis of 72 Cases Highlights the Distinctive Characteristics of a Poorly Recognized Entity.

The guidelines recently recognized the intra-ampullary papillary tubular neoplasm (IAPN) as a distinct tumor entity. However, the data on IAPN and its distinction from other ampullary tumors remain limited. A detailed clinicopathologic analysis of 72 previously unpublished IAPNs was performed. The patients were: male/female=1.8; mean age=67 years (range: 42 to 86 y); mean size=2.3 cm. Gross-microscopic correlation was crucial. From the duodenal perspective, the ampulla was typically raised symmetrically, with a patulous orifice, and was otherwise covered by stretched normal duodenal mucosa. However, in 6 cases, the protrusion of the intra-ampullary tumor to the duodenal surface gave the impression of an "ampullary-duodenal tumor," with the accurate diagnosis of IAPN established only by microscopic correlation illustrating the abrupt ending of the lesion at the edge of the ampulla. Microscopically, the preinvasive component often revealed mixed phenotypes (44.4% predominantly nonintestinal). The invasion was common (94%), typically small (mean=1.2 cm), primarily pancreatobiliary-type (75%), and showed aggressive features (lymphovascular invasion in 66%, perineural invasion in 41%, high budding in 30%). In 6 cases, the preinvasive component was pure intestinal, but the invasive component was pancreatobiliary. LN metastasis was identified in 42% (32% in those with ≤1 cm invasion). The prognosis was significantly better than ampullary-ductal carcinomas (median: 69 vs. 41 months; 3-year: 68% vs. 55%; and 5-year: 51% vs. 35%, P =0.047). In conclusion, unlike ampullary-duodenal carcinomas, IAPNs are often (44.4%) predominantly nonintestinal and commonly (94%) invasive, displaying aggressive features and LN metastasis even when minimally invasive, all of which render them less amenable to ampullectomy. However, their prognosis is still better than that of the "ampullary-ductal" carcinomas, with which IAPNs are currently grouped in CAP protocols (while IAPNs are kindreds of intraductal tumors of the pancreatobiliary tract, the latter represents the ampullary counterpart of pancreatic adenocarcinoma/cholangiocarcinoma).

Syndrome of inappropriate antidiuretic hormone secretion associated with olfactory neuroblastoma.

This study reports a patient having olfactory neuroblastoma complicated by syndrome of inappropriate antidiuretic hormone secretion. Olfactory neuroblastoma is a rare tumor that begins in the olfactory membrane. Only 10 cases have been reported previously. Because of having nonspecific symptoms, most patients manifest at an advanced stage at the time of diagnosis. Olfactory neuroblastoma may show local invasion and/or distant metastasis. We demonstrated preoperatively clinical and biochemical parameters consistent with antidiuretic hormone syndrome turned to normal ranges after the treatment. Surgery, chemotherapy, and radiotherapy are the choices of treatment; among these, surgery is an indispensible treatment.

Results of surgical treatment of pulmonary artery sarcomas: Does histology affect survival?

Background: In this study, we aimed to describe our experience with primary pulmonary artery sarcoma in patients who underwent pulmonary endarterectomy and to evaluate clinical features, treatment, outcomes, and survival rates according to the histological subtypes of this malignant disease. Methods: Between March 2011 and May 2022, a total of 13 patients (7 males, 6 females; mean age: 52.6±13.0 years; range, 30 to 69 years) who underwent pulmonary endarterectomy and diagnosed with a pulmonary artery sarcoma were retrospectively analyzed. The diagnosis was confirmed histopathologically in all patients. Data including demographics, clinical characteristics, intra- and postoperative complications, length of hospital stay, morbidity, mortality, and short-term and long-term outcomes were recorded. Operative mortality was defined as death in the hospital or within 30 days of surgery. Results: Mortality was observed in one patient due to massive hemoptysis. Morbidity developed in two patients due to acute respiratory distress. Pulmonary vascular resistance improved significantly from 508 dyn/s/cm-5 to 191 dyn/s/cm-5 (p<0.004). All patients received chemotherapy following surgery. Median followup was 14 months. Median survival for the entire series was 18 months. One-year and three-year survival rates were 60.6% and 30.3%, respectively. Median survival for leiomyosarcomas (n=6) was seven months, while it was 44 months for intimal sarcomas (p=0.004). Three-year survival was 66.7% for intimal sarcomas and 0% for leiomyosarcomas. Conclusion: Pulmonary artery sarcoma may mimic chronic thromboembolic pulmonary hypertension. Patients with a suspected diagnosis of pulmonary artery sarcoma should be referred to expert pulmonary endarterectomy centers for surgery where a multidisciplinary team is available. Pulmonary endarterectomy has both diagnostic and therapeutic value and may improve survival and quality of life. Patients with intimal sarcoma have longer survival compared to those with leiomyosarcoma.

FDG PET/CT Features of Polysaccharide-Based Hemostatic Agent: Chronic Inflammatory Changes Can Mimic Metastatic Lesions.

PURPOSE: To prevent hemorrhagic complications, hemostatic agents (HAs) have been widely used in recent years. The use of HAs can lead to false-positive results on postoperative imaging. There exists only 1 study in the literature evaluating these applications during surgical procedures. Therefore, we aimed to evaluate the postoperative imaging features of polysaccharide-based HAs in thoracic surgery patients who have had 18F-FDG PET/CT scans. PATIENTS AND METHODS: Two hundred nine consecutive patients who underwent thoracic surgery were enrolled in this study. A topical polysaccharide-based HA was applied to the surgical bed for all of the patients. The patients diagnosed with cancer were followed up with subsequent thoracic CT scans, and 42 of these patients were also imaged with 18F-FDG PET/CT, which then comprised the main study group. Due to suspicion of metastasis, 19/42 patients were reoperated or rebiopsied. The latest histopathological findings were accepted as criterion standard, and previous FDG PET/CT images were retrospectively reevaluated. RESULTS: Polysaccharide-based HAs that appear as amorphous basophilic material were identified in histopathological samples of 11/19 patients. Lymphocytes, plasma cells, and histiocytes, which formed foreign body reaction and/or foreign body granuloma, indicating the presence of chronic inflammation, were seen in all of the samples. 18F-FDG PET/CT showed increased FDG uptake in all of these lesions. CONCLUSIONS: Despite the inconsistency of the literature, polysaccharide-based HAs can be demonstrated in human surgical specimens as amorphous basophilic materials even after a long time from the initial surgical procedure. These agents almost always cause chronic inflammatory changes. In addition, these agents may mimic "false-positive" findings on postoperative FDG PET/CT scans.

Diagnosis of Pulmonary Artery Sarcoma with Multi-modality Imaging.

Pulmonary artery sarcoma is an extremely uncommon malignancy with a poor prognosis. It is often difficult to distinguish it from pulmonary thromboembolic disease because of nonspe cific signs and symptoms as well as similar imaging findings. We present a 46-year-old man who had initially been diagnosed with presumed asthma that later proved to be pulmonary artery sarcoma. The patient was evaluated with multi-modality imaging studies which showed a mass in the pulmonary artery, its extension, mobility and invasion, and attachment to the artery wall. Pulmonary artery mass was excised and pulmonary artery endarterectomy was performed. The histopathological diagnosis was undifferentiated sarcoma with pleomorphic morphology.

Is There Any Role of Pulmonary Endarterectomy in Pulmonary Arterial Hydatidosis?

BACKGROUND: Hydatid cyst is a zoonosis caused by Echinococcus granulosis. Pulmonary artery involvement is a rare condition. The aim of this study was to review the investigators' experience with the surgical treatment of pulmonary arterial hydatidosis. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy (PEA) and who had a diagnosis of hydatidosis at or after PEA. RESULTS: A total of 8 patients (2 male and 6 female; mean age, 31.25 ± 13.68 years) with hydatidosis were defined. Only 1 patient presented with hemoptysis, whereas the rest of the patients reported exertional dyspnea as their main symptom. Cardiac hydatidosis associated with pulmonary arterial involvement was noted in 1 patient. The mean time interval for duration of disease was 12 ± 24.29 months before PEA. Mortality was observed in 2 patients as a result of massive hemoptysis in 1 patient and right-sided heart failure in the other. No anaphylactic reaction was observed. Significant difference was detected in mean pulmonary vascular resistance as a decline from 442.38 ± 474.20 dyn/s/cm-5 to 357.25 ± 285.34 dyn/s/cm-5 after PEA (P = .011). Two patients had recurrence of the disease after a median follow-up of 9.1 months All survivors improved to New York Heart Association functional classes I and II. CONCLUSION: Pulmonary arterial hydatidosis may mimic chronic thromboembolic pulmonary hypertension, and in these patients the diagnosis can be made with PEA. Pulmonary endarterectomy may be a therapeutic option for patients who do not respond to medical therapy if the cystic lesions are surgically accessible. PEA should be performed only in expert centers because of the high risk of perioperative morbidity, mortality, and postoperative recurrence.

Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience.

BACKGROUND: Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. METHODS: Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. RESULTS: We identified 9 patients (6 women; median age, 48 years [range, 23-55]) with IPV. The diagnosis was confirmed after histopathologic examination of all surgical materials. The mean duration of disease before surgery was 88.0 ± 70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in 6 patients and unilateral in 3. No deaths occurred; however 1 patient had pulmonary artery stenosis, and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30 mm Hg (range, 19-67) to 21 mm Hg (range, 15-49) after surgery (P < .05). Pulmonary vascular resistance also improved significantly from 270 dyn/s/cm-5 (range, 160-1600) to 153 dyn/s/cm-5 (range, 94-548; P < .05). After a median follow-up of 41 months, all but 1 patient had improved to the New York Heart Association functional class I. CONCLUSIONS: IPV can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.

FDG-Avid Lung Nodule Formation by the Use of Hemostatic Powder: A Potential Cause of False-Positive FDG PET/CT.

ABSTRACT: FDG PET/CT is used in the diagnosis and follow-up of various malignant tumors and changes patient management in routine clinical practice. However, inflammatory, infectious, physiological, and technical causes and benign pathological conditions may lead to false-positive FDG uptake. We report a case of a 59-year-old man who was considered having a false-positive FDG-PET pulmonary nodular lesion in favor of metastasis, which was formed by the use of hemostatic powder.