Primary pulmonary lymphangiectasia in Noonan syndrome: apropos of an extremely rare manifestation and a brief literature review.

Noonan syndrome (NS) is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, bleeding difficulties and lymphatic malformations. Although lymphatic dysplasias are present in 20% of patients with NS, however pulmonary lymphangiectasia has rarely been described. In this present paper, we report a 24-year-old male who was diagnosed with Noonan syndrome and primary pulmonary lymphangiectasia by using chest imaging modalities. A brief overview of the current literature is also provided laying emphasis on the clinical, pathogenetic and diagnostic aspects of this uncommon Noonan syndrome complication.

Pharyngeal Papilloma: a Rare Non-Pulmonary Cause of Hemoptysis.

The most common benign neoplasm of the pharynx is papilloma. It is characterized by bulging brittle lesions, which are pedicled or sessile, whitish-grey or pinkish colour. Progressive hoarseness is the main clinical feature. When the papillomata spread throughout the tracheobronchial tree symptoms such as chronic cough, stridor, dyspnea or acute respiratory distress are mostly present. Hemoptysis as a presenting symptom is exceptionally rare in patients with pharyngeal papillomatosis. Herein, we report a case of pharyngeal papillomatosis in which hemoptysis was the primary clinical manifestation. The clinical and therapeutic aspects of the disease are briefly discussed.

Bronquiolitis obliterante hipereosinofílica inducida por sulfasalazina / Sulfasalazine-induced Hypereosinophilic Obliterative Bronchiolitis

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Orange-Pigmented Sputum as a Manifestation of Smoke Grenade Inhalation Injury.

A 34-year-old man presented with scanty hemoptysis, orange-colored expectoration, and mild dyspnea. He was in an enclosed building taking part in a military training exercise inhaling an orange-colored smoke from a smoke grenade ignition. His symptoms developed immediately after the initial exposure but he sought medical assistance 20 hours later because of their persistence. Fiberoptic bronchoscopy was performed revealing diffuse inflammatory tracheobronchial tree with streaky orange-pigmented secretions in the trachea and both main-stem bronchi. Acute tracheobronchitis was diagnosed and the patient was treated with nebulized bronchodilators and intravenous corticosteroids showing complete recovery. To our knowledge, this is the first well-documented report of inhalation injury induced by a smoke bomb explosion including potassium chlorate oxidizer and Sudan I and presenting with orange-pigmented sputum production. Smoke inhalation injury is associated with significant morbidity and mortality. The heterogeneity of the smoke and the large variety of the resulting symptoms may be the reason why a definition, specific diagnostic criteria, and therapeutic guidelines are still lacking.

Paraneoplastic nephritic syndrome and concurrent solitary mediastinal lymph node metastasis from mesothelioma of testis diagnosed by endobronchial ultrasound: Unusual manifestations of an extremely rare tumor.

Malignant mesothelioma is most commonly found in the pleura, peritoneum and pericardium, whereas mesothelioma of the tunica vaginalis testis is exceedingly rare. The usual sites of metastasis are inguinal nodes, retroperitoneal lymph nodes and lung. Herein, we describe a patient with mesothelioma of testis, who presented with paraneoplastic glomerulopathy and asymptomatic solitary mediastinal lymphadenopathy on serial computed tomography imaging after radical orchiectomy. A diagnosis of metastatic lymph nodal disease was set by using convex-probe endobronchial ultrasound (EBUS) with transbronchial needle aspiration. We also briefly discuss clinical, etiological, pathological and therapeutical aspects of the disease, and highlight the paramount importance of real-time EBUS as the preferred method in the diagnostic approach of mediastinal lesions.

Primary Bronchopulmonary Actinomycosis Masquerading as Lung Cancer: Apropos of Two Cases and Literature Review.

Actinomycosis is a rare and slowly progressive infectious disease that can affect a variety of organ systems including the lung. It is caused by filamentous Gram-positive anaerobic bacteria of the genus Actinomyces. Despite its rarity, pulmonary actinomycosis can involve lung parenchyma, bronchial structures, and chest wall. The disease can mimic lung malignancy given its nonspecific clinical and radiological presentation, thus posing a diagnostic dilemma to the attending physician. In this paper, we describe two patients with pulmonary actinomycosis mimicking bronchogenic carcinoma; the former presented with peripheral infiltrate and associated hilar/mediastinal lymphadenopathy and the latter presented with a foreign body-induced endobronchial mass. Clinical, imaging, diagnostic, and therapeutical aspects of the disease are discussed, demonstrating the paramount importance of the histological examination of lung tissue specimens in the confirmation of the infection given either its low culture yield or the limited use of new molecular diagnostic tools in routine clinical practice.

Bronchoscopic drainage of a malignant lung abscess.

Bronchoscopic drainage of a pyogenic lung abscess is an established therapeutic approach in selected patients in whom conventional antibiotic therapy fails. This intervention has also been undertaken in patients with abscess owing to underlying lung cancer and prior combined radiochemotherapy. However, this procedure has rarely been performed in cavitary lesions of advanced tumor origin before initiating any chemotherapy/radiotherapy scheme. Herein, we describe a case of a 68-year-old woman with lung adenocarcinoma stage IIIB, who underwent bronchoscopic drainage of necrotizing tumor lesion, thus improving her initial poor clinical condition and rendering other treatment modalities, such as radiotherapy, more effective and beneficial. Bronchoscopic drainage of a symptomatic cancerous lung abscess should be considered as an alternative and palliative treatment approach in patients with advanced inoperable non-small cell lung cancer.

Haemoptysis as a primary manifestation of cryptogenic organizing pneumonia (COP).

Cryptogenic organizing pneumonia (COP), previously called bronchiolitis obliterans organizing pneumonia (BOOP) is a clinicopathological disorder of unknown aetiology but increasingly reported. It usually presents with symptoms of dyspnea, cough, fever, weight loss accompanied by the presence of alveolar opacities on chest radiograph. Haemoptysis, described as blood streaking has only rarely been reported as primary presentation of COP. Herein, we report a case of COP in which submassive haemoptysis was the main clinical manifestation. The clinical, radiological, pathological, and therapeutic aspects of the disease are briefly discussed. Cryptogenic organizing pneumonia should be taken into consideration in the differential diagnosis of severe haemoptysis.

Unilateral primary lung hypoplasia diagnosed in adulthood.

Unilateral primary pulmonary hypoplasia is rare in adulthood. It is usually present in the neonatal period or in early childhood, and is characterized by a decreased number of bronchial segments and decreased/absent alveolar air space. Most patients have recurrent episodes of wheezing or pneumonia and severe respiratory distress leading to chronic respiratory failure, whereas some patients may occasionally be asymptomatic. Herein, we present a case of left lung hypoplasia in an asymptomatic 28-year-old male, who was admitted to the hospital for further investigation of an abnormal result on a chest radiograph that was obtained as part of a health evaluation for military service. A thorough workup, including imaging modalities and bronchoscopy, disclosed a hypoplastic left lung, which had gone undiagnosed for 25 years. Embryological, clinical, and diagnostic aspects are briefly discussed.

Solitary gastric metastasis from primary lung adenocarcinoma: a rare site of extra-thoracic metastatic disease.

Lung cancer metastasizing to gastrointestinal (GI)-tract is a rare event. Gastric metastasis is usually asymptomatic but when mucosal in location it may cause symptoms as demonstrated in the current case. This report describes a 60-year old male who was admitted for evaluation of a left upper lobe lung mass with associated bilateral nodular opacities and mediastinal lymphadenopathy. After thorough work-up the diagnosis of advanced lung adenocarcinoma was made. During hospitalization period and prior to starting chemotherapy, he exhibited upper gastrointentinal bleeding. Esophagogastroduodenoscopy revealed an ulcerative lesion in the gastric corpus representing metastasis of the primary lung carcinoma that ensued from immunohistochemical analysis. Clinical, pathological and therapeutic aspects of this uncommon site of extrathoracic metastatic disease are discussed, emphasizing the importance of the immunohistochemistry in the differential diagnosis of lung carcinomas whether primary or secondary to the lung.

Tracheal pseudo-tumor caused by herpes simplex virus.

BACKGROUND: Herpes simplex virus (HSV) has been shown to cause respiratory tract infections mostly in severely immunocompromised patients. Endobronchial tumor-like lesions have been described very rarely as HSV pulmonary manifestations in critically ill patients or in immunosuppressed individuals. CASE PRESENTATION: This case study describes a 75-yr-old male who presented with persistent hoarseness. Fiberoptic bronchoscopy showed marked mucosal thickening protruding in mid and distal trachea causing stenosis. Biopsy specimens demonstrated cytopathological changes consistent with HSV type 1 and 2 infection. CONCLUSIONS: To the best of the authors' knowledge, this is the first reported case of HSV presenting as an endotracheal tumor in an immunocompetent person.

Thyroid gland metastasis from small cell lung cancer: an unusual site of metastatic spread.

Metastasis to the thyroid gland is uncommon compared with the frequency of primary thyroid tumors. The primary sites of metastatic thyroid tumors usually include the breast, lung, kidney and stomach. Among lung cancer types metastasizing to the thyroid, adenocarcinomas are the commonest followed by squamous and large cell carcinomas. Small cell lung carcinoma has not been frequently reported to cause thyroid metastatic deposits. Herein, we describe a patient with small cell lung cancer who developed metastatic lesions to the thyroid and brain simultaneously. Thyroid ultrasonography-guided fine-needle aspiration cytology (US-FNAC) and particularlyimmunocytochemistry documented metastasis from primary lung cancer. Clinical, cytopathological and therapeutic aspects of this unusual site of extrathoracic metastatic disease are discussed laying special emphasis on the paramount importance of the immunocytochemistry in distinguishing primary thyroid tumors from thyroid metastasis due to lung cancer.

Subpleural lipoma: Management of a rare intrathoracic tumor.

INTRODUCTION: Subpleural lipomas are rare intrathoracic benign tumors. They are often discovered incidentally on plain chest radiographs and the diagnosis is usually established by computed tomography. PRESENTATION OF CASE: We report a case of subpleural lipoma, with enlargement during a period of two years. Pathology examination of the specimen confirmed the diagnosis of lipoma. DISCUSSION: For non-invasive diagnostic investigation, computed tomography enables the identification and quantification of subpleural lipoma due to their characteristic fat attenuation. Surgical resection with thoracotomy or VATS provides more accurate and firm diagnosis, and complete excision. CONCLUSION: This clinical entity needs attention due to difficult preoperative differentiation. Complete surgical excision of these lesions with the appropriate surgical approach is mandatory, for both diagnosis and treatment.

Osteoblastoma of the rib: A rare benign tumor with an unusual location.

INTRODUCTION: Osteoblastoma is a rare benign bone tumor commonly located at spine and long bones. However, rib involvement has been reported less frequently. PRESENTATION OF CASE: In this report, we describe a young adult male presenting with left posterolateral chest wall pain. Chest computed tomography showed a calcified tumor in the left third posterior segment of the rib. Radical mass resection was performed and histopathology confirmed a benign osteoblastoma. At last follow-up, 10 months postoperatively, the patient has eventually relieved of the unbearable chest pain. Imaging evaluation revealed no evidence of recurrent tumor. DISCUSSION: Osteoblastoma is an uncommon primary bone tumor accounting for only 1% of all bone tumors. Ribs are involved in less than 5% of patients. The disease has usually good prognosis with a tendency for local destruction and recurrence. CONCLUSION: Radical surgery remains the treatment of choice to prevent recurrences and to provide a definite diagnosis differentiating it from osteoblastoma-like osteosarcoma.

Endobronchial ultrasound: a useful tool in the diagnosis of bronchogenic cyst.

Diagnosis of bronchogenic cysts is possible with computed tomography, where the cysts are seen usually as well-circumscribed lesions of water density. However, many of the cysts have a soft-tissue density thus rendering them indistinguishable from neoplasms. In this article, we describe a case of bronchogenic cyst presenting as soft-tissue mass that was evaluated and diagnosed by endobronchial ultrasound (EBUS). We discuss the ultrasound image characteristics of the cyst and its histopathology findings. EBUS seems to be a valuable tool in the diagnosis of bronchogenic cysts and also enables their complete aspiration.