Assessment score for the diagnosis of a case with pleuroparenchymal fibroelastosis.

Idiopathic pleuropulmonary fibroelastosis is an extremely rare lung disease characterized by the combination of fibrosis of the visceral pleura and the fibroelastotic changes transcending in the subpleural lung parenchyma that predominantly affects the upper lobes with accompanying volume loss. It is mostly idiopathic while infection, autoimmunity, bone marrow or lung transplantation and genetic predisposition may be associated with the development of PPFE. The disease is exceptionally rare as approximately ninety cases have been reported in the literature currently. A 35-year-old female presented with exertional dyspnea, dry cough and weight loss. Physical examination demonstrated platythorax, suprasternal notch deepening and fine rales over the upper lobes. Blood count, serum biochemistry, autoimmunity and serologic markers for collagen vascular diseases were within normal limits. Arterial blood gases demonstrated a low pO2 (48 mm Hg) and a high pCO2 (54 mm Hg) values. Chest x-ray showed bilateral parenchymal fibrotic lesions, left pneumothorax, bronchiectasis in the middle and pleural thickening in the upper lung zones while HRCT revealed bilateral apical pleural thickening, traction bronchiectasis, subpleural reticulations, ground-glass opacities and honeycombing in the upper lobes. Bronchoscopy, BAL cytology, smear and culture did not reveal any pathologic findings. Relevant with the clinical, laboratory, radiologic manifestations and the differential diagnosis with other interstitial lung diseases, PPFE was the final diagnosis. The aim of this case report was to present the clinical manifestations of our case. The second crucial objective was to establish a diagnostic scoring system relevant with the literature and the clinical manifestations of the patient.

Diagnostic utility of 68Ga-citrate and 18FDG PET/CT in sarcoidosis patients.

Sarcoidosis is a chronic granulomatous disease of unknown etiology. The disease  most commonly involves the lungs and the mediastinal lymph nodes while extrapulmonary organs such as the skin, eye, liver or spleen may also be comprised. Many imaging modalities have been used for the clinical evaluation of sarcoidosis patients but all have been found to have certain drawbacks for a reliable identification assessment due to the equivocal diagnostic results.  This case series was designed to determine the clinical trenchancy of simultaneous 68Ga citrate PET/CT [Positron emission tomography with 68Ga citrate (68Ga citrate PET/CT)] and 18F-FDG PET/CT [Positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography (18F-FDG PET/CT)] imaging in sarcoidosis patients. The main goal of the study was to evaluate sarcoidosis with respect to disease activity and organ involvement. A total of eight sarcoidosis patients with a comorbid disease suspicion were included in the study. Conventional clinical parameters used for  the diagnosis and the activity of sarcoidosis including CT [Computed tomography (CT)] were compared with the 68Ga-citrate PET/CT findings. Concurrent 18F-FDG PET/CT was performed to verify the granulomatous inflammation of sarcoidosis and to determine coexisting malignant or other inflammatory diseases. Our study results revealed that 68Ga citrate PET/CT imaging appears to be highly useful for the diagnosis, activity assessment and extrapulmonary organ involvement in sarcoidosis. Another crucial finding was the detection of extrapulmonary organ disease that are exceptionally involved, almost inaccessible by biopsy and that could not be otherwise displayed by other conventional imaging modalities. The third hallmark was the identification of a clinically asymptomatic and occult malignancy accompanying sarcoidosis that would not be revealed in any way if synchronous 18FDG PET/CT had not been performed. Simultaneous application of 68Ga citrate and 18FDG PET/CT may provide extremely useful data for the clinical evaluation of sarcoidosis patients in terms of the primary disease diagnosis, activity state, extrapulmonary organ involvement unachievable for biopsy and the clinically occult malignant disorders.

Simultaneous use of FDG-18 and 68Ga-citrate PET/CT for the differential diagnosis of sarcoidosis and malignant disease.

A 67-year-old male presented with cutaneous rash, lassitude and fatigue of three weeks. Personal history included psoriasis and sarcoidosis. Physical examination revealed macular rash on the anterior chest wall. Laboratory results were within normal limits. Chest X-ray showed normal findings. Pulmonary function tests demonstrated a mild obstructive pattern and a mild decrease in DLCO/VA. Thorax CT revealed two nodules in the right upper and middle lobe. 68Ga-citrate PET/CT did not demonstrate any active inflammatory reaction associated with sarcoidosis while 18F-FDG PET/CT revealed increased FDG uptake in the right middle lobe, upper division bronchus and in the left lower abdominal quadrant. Histopathologic examination of the colon biopsy was compatible with adenocarcinoma and bronchoscopic biopsy of the lung lesions revealed nonspecific granulomatous inflammation. BAL cytology was normal while BAL culture did not grow any pathologic organisms. Simultaneous use of 18F-FDG and 68Ga-citrate PET/CT was the hallmark for the final diagnosis in our patient. While FDG/PET has detected the pulmonary and colonic malignant foci in our patient, 68Ga-citrate PET/CT excluded the presence of active granulomatous inflammation of sarcoidosis. Simultaneous utility of these two imaging modalities in patients with sarcoidosis is of great importance in terms of guiding the clinician towards the accurate diagnostic pathway which is the hallmark for final diagnosis, especially in the presence of concomitant malignant disease.

An atypical acute exacerbation of COPD due to Aspergillus fumigatus.

A 64-year-old male with a history of stabile chronic obstructive pulmonary disease (COPD) presented with increasing dyspnea and sputum for the last two months. Complete blood count showed WBC 14x103/ml, Hgb: 14.2 g/dL and eosinophilia. Blood biochemistry was normal. Chest x-ray showed hyperlucency while thorax computed tomography (CT) revealed obstructive lung disease and bronchiectasis. Pulmonary function tests demonstrated severe obstructive lung disease and a negative bronchoreversibility with a moderately reduced diffusing capacity/alveolar volume (DLCO/VA). ABG gases revealed significant hypoxemia. Sputum culture was negative. Total IgE was 1140 ng/ml. Aspergillus RAST, precipitins and aspergillusgalactomannan antigen were positive. CF genetic screening tests gave negative results. Allergic bronchopulmonary aspergillosis  (ABPA) is a hypersensitivity reaction that occurs due to bronchial aspergillus colonization. It is most common in patients with asthma and cystic fibrosis. We present a COPD case with an acute exacerbation due to Aspergillus fumigatus that lead to an aberrant clinical profile unresponsive to conventional treatment. Clinicians should consider Aspergillus fumigatus as an etiologic agent in an atypical and severe COPD exacerbation.

Case report of atypical endometrial stromal cells in an endometrial polyp and osteoclastic like giant cells in leiomyoma in the same patient: Is it a coincidence or is it a result of Tamoxifen treatment?

Atypical stromal cells are rarely identified in the endometrial polyps of the female lower genital system. These cells are suggested to develop due to a degenerative or reactive phenomenon. And osteoclastic like giant cells, which have been reported in many epithelial and mesenchymal tumors of uterus, may develop because of a degenerative or a reactive process. In this case report, we present a breast cancer patient who was commenced on Tamoxifen treatment presenting with formation of both atypical stromal cells in an endometrial polyp and osteoclastic like giant cells in the leiomyoma of the uterus. Patients who are treated with Tamoxifen should be followed meticulously to detect tumoral or proliferative lesions of endometrium and myometrium because of the adverse effects of tamoxifen on uterus. (www.actabiomedica.it).

Significant Neutrophilic Emperipolesis in Squamous Cell Carcinoma.

A 53-year-old man was admitted for tooth mobility. A mass was identified at the tooth base by CT. Histopathology of the excisional biopsy revealed a moderately differentiated squamous cell carcinoma. Many intact neutrophils were observed within the malignant cell cytoplasm. The patient underwent partial maxillectomy and bilateral neck dissection. Significant neutrophilic emperipolesis was detected in the resected material. Four tumor recurrences developed in the head and neck region during follow-up. Surgery and chemoradiotherapy was performed. The latest tumor recurrence occurred in the peripharyngeal and the posterior parotideal region. The patient was started on pembrolizumab therapy and nearly complete treatment response occurred. Pembrolizumab was discontinued due to the adrenal insufficiency and pulmonary tuberculosis that developed as a treatment side effect. Pembrolizumab was commenced again when tumor recurrence occurred. The patient is currently alive with ongoing pembrolizumab and antituberculous treatment. We present this case to remark the presence of a significant neutrophilic emperipolesis in the squamous cell carcinoma of the hard palate and maxilla which is rarely encountered. Emperipolesis may predict tumor behavior and the consequences of immune-modulating treatment response in squamous cell carcinomas of the head and neck in regard to the findings of our case.

A case of bleomycin-induced lung toxicity.

A 64-year-old female was admitted for dry cough, dyspnea, fever, loss of appetite, and weight loss. Past medical history revealed scoliosis, cholecystectomy, and Hodgkin lymphoma. ABG values were: pH: 7.42, pCO2: 40.2 mm Hg, pO2: 61.4 mm Hg. Chest CT showed cystic lesions, emphysema, ground glass, and reticular opacities. ABG values worsened under 8L/min nasal oxygen. The patient underwent bilevel positive airway pressure (BiPAP) and methylprednisolone 60 mg/day bid was commenced. The final diagnosis was respiratory insufficiency due to bleomycin toxicity. The patient deceased on the sixth day after transfer to the intensive care unit. Bleomycin is an effective chemotherapeutic agent used for Hodgkin lymphoma treatment. It causes significant lung toxicity in half of the patients. Clinicians should always remember that bleomycin toxicity may lead to fatal complications in patients with comorbid conditions. We present this case to remark the possible consequences of bleomycin toxicity and the precautions taken to preclude bleomycin-induced pulmonary complications are discussed.

The value of exfoliative cell cytology in the diagnosis of exudative pleural effusions.

The sensitivity and specificity of exfoliative cell cytology for the diagnosis of exudative pleural effusions varies widely according to the etiologic causes. The aim of this study is to assess the diagnostic value of exfoliative cell cytology for the identification of exudative pleural effusions. This is a retrospective study of the patients with an exudative pleural effusion admitted at our clinic in the last twenty years. We have conducted the clinical, the cytological findings, and the diagnostic results of six hundred patients from hospital records.  Male to female ratio was 2.2:1 with a mean age of 42.8 years (range 18-78 years) among the patients. Samples were processed and evaluated according to the standard methods. Cytology results were reviewed and the patients were stratified according to the final diagnosis of their disease. Of the six hundred exudative effusions, 240 were malignant on exfoliative cytology pleural fluid alone. Adenocarcinoma was the most common type of malignancy. Tuberculosis was the second most frequent etiology for the exudative effusions followed by infection and collagen vascular diseases. Diagnostic accuracy of cytology showed a good correlation with the final diagnosis with an overall 70.1% sensitivity, 62.5% specificity, and a 95.9% positive predictive value for all exudative pleural effusions. Cytologic examination of the pleural fluid is a simple non-invasive procedure as the initial step for the diagnostic work up of patients with a pleural effusion.  Exfoliative cytology provides high a final diagnostic yield for the identification of an exudative pleural effusion etiology. Furthermore, cytologic analysis leads the clinician into the correct diagnostic pathway as the most informative laboratory tool even when it was not diagnostic by itself for equivocal cases.

Coexistence of adenomyosis, adenocarcinoma, endometrial and myometrial lesions in resected uterine specimens.

The present study was conducted to identify endometrial and myometrial lesions coexisting with adenomyosis, and to evaluate the clinicopathological characteristics of endometrial adenocarcinomas associated with adenomyosis. A retrospective analysis of the resected uterine specimens of 319 patients with adenomyosis admitted between January 1, 2014 and August 1, 2017 was performed. The endometrial and myometrial lesions coexisting with adenomyosis were evaluated. The clinicopathological prognostic factors, including tumor grade, myometrial invasion, lymphovascular space involvement, lymph node invasion, pathological stage and recurrence, were analysed. For data analysis, the Chi-squared test was used and a P-value of <0.05 was considered to indicate statistically significant differences. The mean age of the patients was 52.1 years. A total of 32 patients had endometrial carcinoma associated with adenomyosis. In addition to endometrioid adenocarcinoma of different grades, rare clear cell carcinoma cases were also observed. Two cases of malignant mesenchymal tumors (one low-grade endometrial stromal sarcoma and one leiomyosarcoma) were also diagnosed. Therefore, patients presenting with abnormal uterine bleeding should undergo thorough evaluation for the presence of adenomyosis and/or leiomyoma(s). Although the cases of endometrial adenocarcinoma associated with adenomyosis generally had a good prognostic outcome, there were also rare cases of patients with agressive tumor morphology. The inflammatory and tissue response arising around the foci of adenomyosis generate a preventive mechanism against the invasion of adenocarcinomas coexisting with adenomyosis. This response is likely the primary mechanism underlying the good clinical course of these tumors. Therefore, the presence of adenomyosis may be an important factor for the determination of prognosis.

Microcystic, elongated, and fragmented pattern of invasion in relation to histopathologic and clinical prognostic factors in endometrioid endometrial adenocarcinoma.

OBJECTIVE: To investigate the association of microcystic, elongated, and fragmented (MELF) pattern of invasion with prognostic factors in endometrioid endometrial adenocarcinoma (EEA). MATERIAL AND METHODS: Stained tissue sections from 83 cases of EEA operated by the same gynecologic oncologist were reviewed to identify cases showing MELF-type invasion in this retrospective study. The association of MELF pattern with age, tumor grade, depth of myometrial invasion, and presence of lymphovascular space invasion (LVSI) was analyzed. RESULTS: FIGO grade 2 and grade 1 tumors were evident in 53.0% and 38.6% of patients, respectively. Depth of myometrial invasion was <50% in 72.0% of patients, and LVSI was absent in 77.1%. MELF pattern was confirmed in 35 (42.2%) patients. Presence of MELF pattern was associated with significantly higher mean ± standard deviation age (62.9±6.9) years vs. 58.9±9.1 years, p=0.033), and found to be more likely in patients with high-grade tumor (FIGO grade III; 85.7% vs. 14.3%, p<0.001), deep (≥50%) myometrial invasion (78.3% vs. 21.7%, p<0.001), and presence of LVSI (94.7% vs. 5.3%, p<0.001) as compared with absence of MELF pattern. CONCLUSION: In conclusion, our findings revealed a high rate of MELF pattern among patients with EEA alongside the association of MELF pattern with poor prognostic factors such as high grade tumor, deep myometrial invasion, and LVSI.

Propylthiouracil-induced lupus-like or vasculitis syndrome.

A 27 year old female with Graves' disease presented with fever, exertional dyspnea and polyarthralgia. Erythema nodosum had occured three months earlier. The patient declared irregular use of propylthiouracil (PTU) for the last 8 months. Neutropenia and microscopic hematuria developed in the second week of admission. Chest X-ray showed inhomogenous pulmonary opacities, left pleural effusion and cardiomegaly. Computed tomography (CT) revealed multiple subpleural nodules, left pleural effusion, pericardial effusion, enlarged mediastinal and axillary lymph nodes. Bronchoalveolar lavage (BAL) cytology demonstrated hemosiderin laden macrophages. Histopathologic examination of the transbronchial biopsy specimen revealed a nonspecific inflammation. Serum was positive for ANA, P-ANCA, MPO-ANCA, PR3-ANCA and negative for anti-ds-DNA, C-ANCA, C3, C4 and anti-histone antibody. All symptoms resolved in two months after PTU withdrawal and starting steroid treatment. The same clinical manifestations recurred when the patient used PTU erronously one month after discharge.This is a case of PTU induced-autoimmune disease in whom the accurate distinction between drug-induced-lupus (DIL) and vasculitis was not possible due to the significant overlap of clinical and laboratory findings causing a significant diagnostic challenge for the chest physician.

Round atelectasis in sarcoidosis.

Round atelectasis is a benign inflammatory condition most frequently observed in patients with asbestos exposure but it can also result from a variety of chronic pleural diseases like infection. It has not previously been described in sarcoidosis. We report the occurrence of round atelectasis in four previously diagnosed sarcoidosis patients who were under follow up at our outpatient clinic. Three patients had symptoms consisting of thoracic pain, dry cough and sensation of fullness at the posterior thorax, respectively. Chest roentgenogram showed subpleural or pleural based opacity with diameters ranging from 2 to 3 cm in each of the patients. Chest computerized tomography (CT) revealed features of round atelectasis. Fiberoptic bronchoscopy with transbronchial lung biopsy was performed. Diagnosis was confirmed by the histopathologic examination of the biopsy samples. The mechanical influence of a prior pleural effusion due to sarcoidosis may be the predominant mechanism underlying the onset of round atelectasis in these patients. Clinicians should bear in mind the possibility of sarcoidosis as an etiologic factor for round atelectasis.

Anxiety and depression in COPD patients and correlation with sputum and BAL cytology.

BACKGROUND AND AIMS: Anxiety and depression are common in patients with chronic obstructive pulmonary disease (COPD). The degree of lung function may not explain anxiety and depression. The aim of our study was to assess the psychological aspects of COPD, to test the BODE index (a composite score of body mass, obstruction, dyspnea and exercise capacity), and to evaluate the association between atypical cytologic findings of sputum, bronchoalveolar lavage (BAL) and the pyschological components of the disease. METHODS: COPD was classsified according to the GOLD stages based on forced expiratory volume in 1 second (FEV1) in 60 stable patients. The BODE index was calculated for grading COPD. The Hospital anxiety and depression (HAD) scale was used to appraise the anxiety and depression symptoms. Cytologic examination of sputum and BAL samples were performed in each patient. The cytologic findings were classified as normal, mild, moderate or severe atypia. RESULTS: The overall prevalance of anxiety and depression symptoms was 41.7% and 46.7% respectively. The prevalance of these symptoms increased with increasing BODE stages and correlated well with the severity of atypical BAL cytology results (p < 0.001). Dyspnea and reduced exercise capacity were the predominant mechanisms leading to anxiety and depression symptoms associated with COPD. CONCLUSIONS: We conclude that the BODE index is superior to GOLD stratification for explaining anxiety and depression symptoms in COPD. BAL cytologic findings, which reflect the distal parenchymal lung structure, correlated significantly with the presence of the anxiety and depression symptoms.

Alveolar proteinosis in Behçet's disease.

A 51-year-old man with Behçet's disease complained of fever, dry cough and dyspnea during exertion. Chest CT showed ground glass opacities with interstitial septal thickening in both lungs. Bronchoalveolar lavage (BAL) revealed amorphous and lipoproteinaceous material that was periodic acid-Schiff (PAS) stain positive. Transbronchial biopsy specimen demonstrated PAS positive alveolar eosinophilic material consistent with pulmonary alveolar proteinosis. Serum anti-granulocyte-macrophage colony stimulating factor (GM-CSF) antibody was negative. Recent studies have reported anti-GMCSF not present in the the serum of patients with secondary pulmonary alveolar proteinosis (PAP) but they have not reported so in patients with idiopathic PAP. We report a case of alveolar proteinosis in the setting of Behçet's disease with spontaneous remission.

Diagnosis of elastofibroma.

Elastofibroma is a relatively rare soft tissue mass. The lesion is probably reactive and occurs most commonly in the periscapular region. It is a degenerative benign neoplasm with the clinical appearence of a malignant tumour. The present report describes the case of a 70-year-old man with bilateral elastofibroma. The diagnosis was established with needle aspiration biopsy and positron emission tomography/computed tomography. The present case suggests that needle aspiration biopsy and positron emission tomography/computed tomography are highly useful in the diagnosis of this rare, benign tumour.

[The therapeutic effect of normobaric oxygen in experimental peritonitis and the efficiency of rectal fever, WBC, CRP and procalcitonin in monitoring response of the therapy]. / Normobarik oksijenin deneysel peritonitin tedavisindeki yeri ve tedavinin izlenmesinde rektal ates, lökosit, CRP ve prokalsitoninin etkinligi.

BACKGROUND: It was investigated the effect of using normobaric oxygen (NO) in addition to antibiotherapy in experimental peritonitis and the changes of rectal fever (RF), WBC, CRP and procalcitonin levels were evaluated. METHODS: After the preliminary research of the normal values, rats were infected by E. coli intraperitoneally. Four groups were assigned into "no therapy", "given NO", "given antibiotic", "given antibiotic + NO" groups. RESULTS: The decline of RF and WBC levels on 3rd and 5th days was recorded in antibiotic + NO group versus the other groups. It was observed that group 4 was superior to the others. The positivity of periton cultures and the inflammation in the muscle were found to be less in antibiotic + NO group. No correlation was found between pathological and microbiological recovery and blood CRP level in all groups. But a significant decrease in blood procalcitonin level was determined in group 4 compared to the other groups. On day 3, procalcitonin and CRP levels increased with increasing WBC levels. On day 5, procalcitonin levels also decreased in groups with decreased WBC levels, but no significant correlation was found between CRP and WBC levels. CONCLUSION: It was concluded that using of NO in addition to antibiotherapy could increase the success rate of experimental intraabdominal sepsis therapy and blood procalcitonin and WBC levels could be more beneficial than CRP levels in monitoring of the severity of the sepsis.

Computed tomography and bronchoscopy in endobronchial tuberculosis.

BACKGROUND: The therapeutic response to endobronchial tuberculosis is usually evaluated by bronchoscopy. Currently, there are no published studies investigating the use of computed tomography for the evaluation of therapeutic response in endobronchial tuberculosis. OBJECTIVE: A retrospective study was performed to evaluate the bronchoscopic and computed tomographic features of endobronchial tuberculosis before and after treatment. The aim of this study was to investigate the usefulness of computed tomography for the assessment of treatment. METHODS: The clinical, pathological and bronchoscopic features of endobronchial tuberculosis were evaluated in 55 patients. The age range of the patients was 21 to 52 years. Computed tomography and bronchoscopy were performed before and after treatment. RESULTS: Diagnosis of tuberculosis was confirmed by culture and histopathological examination. Bronchoscopic examination revealed 89 endobronchial lesions of various types in 55 patients. The exudative type was the most common. Follow-up bronchoscopy revealed that exudative-, ulcerative- and granular-type lesions healed completely. Computed tomography performed after treatment correlated well with the follow-up bronchoscopic findings. CONCLUSION: The results suggest that follow-up computed tomography is useful for the evaluation of therapeutic response and complications associated with endobronchial tuberculosis, and may replace bronchoscopy.