Inflammatory Myofibroblastic Tumor of the Lung: Unusual Presentation

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare entity considered to be a benign neoplastic lesion. It often mimics spindle cell sarcoma, fibrous histiocytoma, or fibrosarcoma in histologic appearance. Because of its variable cellular composition, it seldom allows diagnosis preoperatively either through transthoracic fine-needle aspiration or bronchoscopic biopsy. In this report we describe a unique occurrence of a rarely described large inflammatory myofibroblastic tumor of the lung that mimics a posterior mediastinal tumor in a 45-year-old female, detailing clinical presentation, imaging, and management.     

Immediate causes of death in thyroid carcinoma: clinicopathological analysis of 161 fatal cases

Most patients with thyroid carcinoma have a good prognosis. Due to the small number of fatal cases, it has not been clarified what conditions result in death for patients with thyroid carcinoma. To provide appropriate management for advanced thyroid carcinoma patients, we analyzed causes of death in 161 fatal cases. Clinical characteristics and immediate (final) causes of death based on pathological conditions were analyzed in 62 anaplastic carcinomas and 99 fatal differentiated carcinomas. Single fatal conditions could not be specified in 55 patients. In the remaining 106 patients, respiratory insufficiency (43%) was the most common specific fatal condition, followed by circulatory failure (15%), hemorrhage (15%), and airway obstruction (13%). Respiratory insufficiency due to remarkable pulmonary metastasis replacing lung tissue, massive hemorrhage and airway obstruction due to uncontrolled local tumors, and circulatory failure resulting from compression of the vena cava by extensive mediastinal or sternal metastases were found to be the most important immediate causes of death. Based on this knowledge, several palliative procedures may be worth considering to improve survival and quality of life in patients with advanced thyroid carcinoma.     

Multicentric malignant fibrous histiocytomas of the colon

Malignant fibrous histiocytoma is a primitive mesenchymal tumour usually found in the soft tissues of the extremity or torso of adults. Only two previous reports of this lesion in the abdominal viscera are present in the world medical literature. A case is reported of three synchronous tumors in the rectum, colon, and omentum with the pathologic appearance of malignant fibrous histiocytoma. Although resected completely with a transverse colocolostomy and a low-anterior resection, the expected behavior of this tumor is one of aggressive local recurrence followed by distant metastasis. The clinical and pathologic characteristics of malignant fibrous histiocytoma are reviewed.     

Malignant fibrous histiocytoma of the ileum

Malignant fibrous histiocytoma occurs most commonly in the extremities and trunk, but rarely in visceral organs. This report documents a case of malignant fibrous histiocytoma arising in the terminal ileum. Following surgical resection, there is no evidence of recurrence or metastasis in this patient after one-year followup studies     

Deep Benign Fibrous Histiocytoma of the Anterior Mediastinum Mimicking Malignancy

The following report describes the case of a 43-year-old male smoker that was referred to the rapid access lung clinic with haemoptysis, chest pain, and axillary lymphadenopathy—a clinical picture that raised concern for a possible underlying malignancy. Preliminary investigations revealed elevated D-dimers, low-volume haemoptysis, and a normal chest X-ray, which lowered the index of suspicion. However, computed tomography (CT) pulmonary angiogram identified a right hilar mass, several parenchymal cysts, and a large mediastinal mass. In addition, a left-sided adrenal lesion was also discovered following CT abdomen pelvis, potentially indicating metastatic disease. Fortunately, a positron emission tomography scan failed to detect any metabolic activity in either the right hilar mass, left adrenal lesion or the anterior mediastinal mass. CT-guided biopsy identified the mediastinal mass as a low-grade spindle cell tumour. Due to its large size, the mass was surgically resected and confirmed to be a deep benign fibrous histiocytoma. The significance of this report is to highlight a clinical presentation suggestive of malignancy but actually resulting from a rare variant of a benign tumour. The constellation of regional lymphadenopathy, respiratory and gastrointestinal symptoms, lung cysts, an adrenal tumour, and a mediastinal mass appeared to suggest a progressive disease pattern more commonly associated with malignancy.     

Primary malignant fibrous histiocytoma of the jejunum metastatic to the spine: report of a case

We report a case of malignant fibrous histiocytoma (MFH) metastatic to the spine. A 41-yr-old male was admitted to our hospital for radiation treatment of MFH of the spine. He began to show signs suggestive of partial small bowel obstruction. Computed tomography demonstrated jejuno-jejunal intussusception. The patient was taken to the operating room, where the diagnosis was confirmed. Partial jejunal resection was performed. The lead point of the intussusception was histologically diagnosed to be a high-grade malignant fibrous histiocytoma. We believe that the spinal lesion was the metastatic lesion and that metastasis occurred via the vessels of Adamciewicz. To our knowledge this is the first case thus reported.     

Mediastinal granuloma and mediastinal fibrosis

Mediastinal granuloma/mediastinal fibrosis is a chronic inflammatory disease of the mediastinum. Mediastinal granuloma is the abnormal enlargment of mediastinal lymph nodes by granulomatous inflammation, is usually asyptomatic or minimally symptomatic, and is often detected on chest radiographs taken for other reasons. In contrast, mediastinal fibrosis is extensive fibrous tissue throughout the middle mediastinum causing compression, encasement, or invasion of the large bronchi, superior vena cava, pulmonary veins, or esophagus, often with serious clinical consequences. Some patients may exhibit a clinical entity characterized by enlarged fibrotic and/or calicified lymph nodes with a variable amount of fibrosis that may be asymptomatic, or may cause symptoms by compression or invasion of structures in the mediastinum. Clinical manifestations may be due to superior vena cava (SVC) obstruction, esophageal compression, large airway involvement, pulmonary artery or pulmonary vein narrowing, or laryngeal or phrenic nerve impingement. Definitive diagnosis is traditionally made on the basis of a surgical exploration and biopsy, either a mediastinoscopy or thoracotomy; however, characteristic findings on computed tomography (CT) of the chest may be sufficient for a diagnosis in a certain number of cases. The best therapy is unknown. Antifungal therapy and corticosteroids have been reported effective in some cases. Surgical resection of localized mediastinal granuloma or fibrosis causing symptoms is often effective. However, surgical resection of extensive mediastinal fibrosis usually is not feasible.     

A case of cutaneous malignant fibrous histiocytoma with multiple organ metastases

Malignant fibrous histiocytoma is a soft tissue sarcoma that most commonly occurs in the extremities and rarely metastasizes cutaneously. A 79-year-old male patient consulted a dermatologist 11 months after recognizing an intractable ulcer on the right mandible. Punch biopsy revealed eosinophilic tumor cells in the dermal area and proliferation of rich spindle cells. Malignant fibrous histiocytoma or atypical fibroxanthoma was suspected and he was referred to our hospital. Red plaque tumors on the right mandible and right temple were 30 mm and 15 mm in size, respectively. The right mandible lesion was ulcerated. Immunohistochemically, the lesions were positive for CD10, CD74 and alpha-smooth muscle actin. Radiological analysis revealed multiple organ metastases, including bone, liver, lung and skin on the right temple. The patient was diagnosed with malignant fibrous histiocytoma, stage IV and died 8 weeks after the first visit due to respiratory failure. Cutaneous malignant fibrous histiocytoma has a poor prognosis resulting in death.     

Solitary pancreatic metastasis of malignant fibrous histiocytoma treated by distal pancreatectomy.

A tumor in the body of the pancreas was detected in a 31-year-old man who had undergone a resection of a malignant fibrous histiocytoma (MFH) of the left distal femur 2 years before. The patient underwent a distal pancreatectomy with regional lymph node dissection. The surgical specimen revealed MFH metastatic to the pancreas. He is alive without recurrence or metastasis at 1 year after pancreatectomy. This case seems to be the first report of successfully resected pancreatic metastasis of a malignant fibrous histiocytoma in the published literature, as far as we can determine.     

Primary hepatic malignant fibrous histiocytoma mimicking cystadenocarcinoma: a case report

Introduction Malignant fibrous histiocytoma (MFH) is one of the common soft tissue sarcomas in adults, involving the extremities and less commonly the retroperitoneal space or other sites of the body.[1-3] It is thought to be originated from undifferentiated mesenchymal cells capable of multidirectional differentiation.[4] This is why it can be found in all organs.[5] MFH of the liver is extremely rare, and only 28 cases have been reported to date.[6-14] In this report we present a case of …     

Abnormal flow volume loops in patients with intrathoracic Hodgkin's disease

STUDY OBJECTIVES: To study the incidence of upper airway obstruction, as measured on the flow volume loop (FVL), in patients with bulky mediastinal Hodgkin's disease; to correlate the FVL with CT of the chest; and to follow the changes in the FVL after treatment of the tumor. DESIGN: Retrospective study of pulmonary function tests (PFTs) and chest CTs performed as part of a clinical trial for Hodgkin's disease. SETTING: Memorial Sloan-Kettering Cancer Center, a comprehensive cancer care center. PATIENTS: Twenty-five patients (15 men and 10 women; age range, 20 to 57 years) with bulky mediastinal Hodgkin's disease enrolled in a clinical trial of chemotherapy followed by external beam radiation therapy. MEASUREMENTS AND RESULTS: Fourteen of 25 patients (56%) had an abnormal FVL prior to therapy; after chemotherapy, only 7 of 25 patients (28%) had an abnormal FVL. The abnormal patterns seen were either those typical of fixed obstruction or variable extrathoracic obstruction. No patient had a pattern typical of variable intrathoracic obstruction. On chest CT scan, 16 patients had grade-I tracheal deformity; 6 had grade-II deformity, and 3 had grade-III deformity. All three patients with grade-III deformity had a fixed obstruction pattern, as did three patients with a grade-I pattern. Patients with a fixed pattern on FVL had significant decreases in inspiratory and expiratory flow rates. CONCLUSION: FVL abnormalities suggesting upper airway obstruction occurred in > 50% of patients with bulky mediastinal Hodgkin's disease. A fixed pattern of obstruction was associated with the lower flow rates and severe tracheal distortion on CT; these patients may warrant special attention prior to general anesthesia or invasive procedures. Asymptomatic patients with abnormal FVLs but normal tracheal profiles need not undergo extensive evaluation. No patients showed the expected pattern typical of intrathoracic obstruction, but rather the major effect was on the inspiratory loop. The authors speculate on the mechanism for this unexpected finding.     

Malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception

A case of malignant fibrous histiocytoma metastases to the small intestine and colon presenting as an intussusception is described. Although malignant fibrous histiocytoma is the most common soft tissue sarcoma in late adult life, GI involvement has rarely been reported. The review of both our case and eight cases in the English-language literature suggests that GI involvement from malignant fibrous histiocytoma occurs most frequently in the small intestine (six of nine) and that two major clinical manifestations of GI involvement are GI bleeding (five of nine) from ulcerated tumors and intussusception (two of nine) led by polypoid tumors.     

Early Recurrence of Malignant Fibrous Histiocytoma of the Heart

Malignant fibrous histiocytoma is a pleomorphic soft tissue sarcoma, which constitutes only 2% of all cardiac malignancies and is typically located in the left atrium. We report a young male patient with malignant fibrous histiocytoma located on the right side of the heart. Early recurrence was observed after extensive surgical resection to relieve symptoms of outflow tract obstruction. Noninvasive evaluation and management with regard to the literature are discussed.     

Giant solitary fibrous tumors of the pleura: two case reports

Solitary fibrous tumors of the pleura (SFTP) are rare neoplasms.We report two cases of giant SFTP treated by surgical resection. One of these was enormous (30 x 19 x 18 cm, weight: 4,050 g) and occupied the entire left pleural cavity with complete lung collapse and contralateral mediastinal shift. Both tumors were removed en bloc. The patients had no postoperative complications.Surgical treatment is curative in most patients; resection must be complete because of the possibility of recurrence and metastasis.     

肺鳞癌结肠黏膜转移一例报告和文献复习

肺癌患者发生结肠转移非常罕见，患者一般无典型症状，晚期可出现腹痛、消化道出血、梗阻、穿孔等症状，并发症和死亡率较高，短期预后差。本中心报道一位63岁男性患者，以"间断咳嗽和右下腹隐痛"就诊，诊断肺鳞状细胞癌并纵隔淋巴结转移和结肠寡转移，经过PD-1免疫治疗，症状改善明显，目前仍在治疗和随访中。     

Endoscopic ultrasound-guided fine-needle aspiration for non-small cell lung cancer staging: A systematic review and metaanalysis

BACKGROUND: Endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA) is a minimally invasive alternative technique for mediastinal staging of non-small cell lung cancer. A metaanalysis was performed to estimate the diagnostic accuracy of EUS-FNA for staging mediastinal lymph nodes (N2/N3 disease) in patients with lung cancer. METHODS: Relevant studies were identified using Medline (1966 to November 2005), CINAHL, and citation indexing. Included studies used histology or adequate clinical follow-up (> 6 months) as the "gold standard," and provided sufficient data for calculating sensitivity and specificity. Summary receiver operating characteristic curves metaanalysis was performed to estimate the pooled sensitivity and specificity. RESULTS: In 18 eligible studies, EUS-FNA identified 83% of patients (95% confidence interval [CI], 78 to 87%) with positive mediastinal lymph nodes (pooled sensitivity) and 97% of patients (95% CI, 96 to 98%) with negative mediastinal lymph nodes (pooled specificity). In eight studies that were limited to patients who had abnormal mediastinal lymph nodes seen on CT scans, the sensitivity was 90% (95% CI, 84 to 94%) and the specificity was 97% (95% CI, 95 to 98%). In patients without abnormal mediastinal lymph nodes seen on CT scans (four studies), the pooled sensitivity was 58% (95% CI, 39 to 75%). Minor complications were reported in 10 cases (0.8%). There were no major complications. CONCLUSIONS: EUS-FNA is a safe modality for the invasive staging of lung cancer that is highly sensitive when used to confirm metastasis to mediastinal lymph nodes seen on CT scans. In addition, among lung cancer patients with normal mediastinal adenopathy seen on CT scans, despite lower sensitivity, it has the potential to prevent unnecessary surgery in a large proportion of cases missed by CT scanning.     

Central airway obstruction

Central airway obstruction is a problem facing all medical and surgical subspecialists caring for patients with chest diseases. The incidence of this disorder appears to be rising because of the epidemic of lung cancer; however, benign causes of central airway obstruction are being seen more frequently as well. The morbidity is significant and if left untreated, death from suffocation is a frequent outcome. Management of these patients is difficult, but therapeutic and diagnostic tools are now available that are beneficial to most patients and almost all airway obstruction can be relieved expeditiously. This review examines current approaches in the workup and treatment of patients suffering from airway impairment. Although large, randomized, comparative studies are not available, data show significant improvement in patient outcomes and quality of life with treatment of central airway obstruction. Clearly, more studies assessing the relative utility of specific airway interventions and their impact on morbidity and mortality are needed. Currently, the most comprehensive approach can be offered at centers with expertise in the management of complex airway disorders and availability of all endoscopic and surgical options.     

Ventilation inhomogeneity assessed by nitrogen washout and ventilation-perfusion mismatch by capnography in stable and induced airway obstruction

Few studies have been published on gas distribution in the lung during acute and stable airway obstruction in children. Multiple breath nitrogen (N(2)) washout is an established method for assessing ventilation inhomogeneity, while the tidal breathing capnogram may be used as an indicator of ventilation-perfusion (V(')(A)/Q) mismatch. We hypothesized that significant V(')(A)/Q mismatch is not seen in stable airway obstruction unless obstruction is severe, and that stable and induced airway obstruction of similar severity would result in different degrees of V(')(A)/Q mismatch. To test this hypothesis, we performed spirometry measurements of forced expiratory volume in 1 sec (FEV(1)), multiple breath N(2) washout, and tidal breathing capnography in 11 young patients (9-30 years) with cystic fibrosis, 37 asthmatic patients (8-18 years), and 34 healthy subjects (7-20 years). Lung function was measured at rest, after airway obstruction induced by cold dry air hyperventilation or methacholine challenge, and after beta(2)-agonist treatment. V(')(A)/Q mismatch was assessed from the slopes of the phases II and III of the capnogram. We observed a normal capnogram during stable obstruction of moderate severity despite significant ventilation inhomogeneity. In patients with severe stable obstruction and in those with induced airway obstruction significant ventilation inhomogeneity and pathological capnograms were seen. Induced airway obstruction, resulted in a more pathological capnogram than stable obstruction of similar severity. beta(2)-agonist treatment reduced ventilation inhomogeneity, but did not improve the capnogram. Our findings are compatible with the presence of an efficient pulmonary blood flow regulatory mechanism that adequately compensates for chronic ventilation inhomogeneity of moderate severity, but not for severe or sudden airway obstruction.     

Clinical relevance of airway remodelling in airway diseases.

Asthma and chronic obstructive pulmonary disease (COPD) are characterised by airflow obstruction, airway remodelling (measurable structural change) and inflammation. The present review will examine the relationship between airway remodelling in these two conditions with respect to symptoms, abnormal lung function, airway hyperresponsiveness and decline in lung function. The potential for remodelling to be a protective response will also be discussed. Asthma is associated with variable symptoms and changes in lung function and also fixed abnormalities of lung function and an increased rate of decline in lung function with age. There is a relative preservation of the relaxed airway lumen dimensions, prominent thickening of the smooth muscle layer and reduced airway distensibility. The severity of asthma is related to the degree of airway remodelling, which is most marked in cases of fatal asthma. In COPD, symptoms are persistent and predictable but also progressive and are related to fixed abnormalities of lung function. Remodelling is associated with narrowing of the airway lumen and an increased thickness of the airway wall, although not usually to the extent seen in asthma. COPD is most often due to smoking where there is also remodelling of the parenchyma that may contribute to symptoms.     

Wheezing and stridor

Wheezes are defined as high-pitched, continuous, adventitious lung sounds. They are produced by oscillation of opposing airway walls whose lumen is narrowed. Although asthma is the most common cause of wheezing, a wide variety of disease processes may result in wheezing due to airway obstruction. This obstruction may be caused by airway edema, smooth muscle constriction, increased secretions, vascular congestion, mass lesions, scarring, or foreign bodies. Stridor is a special kind of wheeze described as a loud musical sound of constant pitch, which is heard in patients with tracheal or laryngeal obstruction. The full differential diagnosis of airway obstruction should be carefully considered in any patient with wheezing or stridor.