Clinico-pathological characteristics of congenital pulmonary lymphangiectasis: report of two cases

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.     

Congenital pulmonary lymphangiectasis: Report of an autopsy case masquerading as pulmonary interstitial emphysema

We describe the clinicopathologic features of a case of congenital pulmonary lymphangiectasis (CPL). A male Japanese infant born prematurely at 34 weeks of gestation developed a severe moaning sound, dyspnea, and prominent respiratory acidosis about 10 min after delivery. A chest X-ray film showed bilateral frosted glass-like infiltrates with an air bronchogram and an air leak around the cardiac shadow, suggesting pneumomediastinum. The patient died of hypoxemic respiratory failure 13 h after birth. The death was complicated by bilateral pneumothorax, despite the initiation of artificial ventilation and administration of a surfactant. At autopsy, small cystic lesions were noted in the visceral pleura, interlobular septa, and hilum of both lungs. A histologic examination of the lungs showed diffuse and marked dilation of the lymphatic channels in the subpleural, peribronchial, interlobular, and hilar areas. The channels were lined with flattened endothelium, which was immunohistochemically positive for D2-40. In addition, lymphangiectasis was found around the thymus and intra-abdominal organs, but no cardiovascular anomalies were seen. The findings conformed to a primary form of CPL, Noonan Group 3. Although pulmonary interstitial emphysema (PIE) was considered an important differential diagnosis because of the overlapping clinicopathologic features, a giant cell reaction surrounding the interstitial cystic lesions, a histologic hallmark of PIE, was absent in the present case.     

Pneumonectomy Case in a Newborn with Congenital Pulmonary Lymphangiectasia

Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks'' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.

Graphical Abstract

相似文献   

Pulmonary vascular involvement in sarcoidosis: a report of 40 autopsy cases.

We examined pulmonary vascular involvement in 40 autopsy cases of sarcoidosis. In these cases granulomatous involvement was observed at all levels from large elastic pulmonary arteries to venules, and venous involvement was more prominent than arterial involvement. The extent of granulomatous vascular involvement was related to that of parenchymal granuloma. No significant difference was found between upper and lower lobes in the incidence of granulomatous vascular involvement. The distribution of granulomata in the blood vessels was segmental and adventitial, and medial involvement was prominent in the larger vessels. Healed lesions of granulomatous vascular involvement also were observed at various levels in blood vessels. Prominent granulomatous involvement was found in the lymphatic capillaries and collecting lymphatic vessels in lungs with sarcoidosis. Serial sections of the lungs demonstrated interstitial granuloma directly connecting the lymphatic capillaries around small blood vessels. Granulomatous involvement in vasa vasorum and lymphatic capillaries is likely to be an important factor in the pathogenesis of granulomatous vascular involvement in lungs with sarcoidosis. The present study suggests that granulomatous vascular involvement and its sequelae may contribute to the development of pulmonary sarcoidosis.     

Extrapulmonary manifestations of sarcoidosis as the cause of primary symptoms--4 case reports

Sarcoidosis is a multisystem disorder most frequently presenting with hilar lymphadenopathy, pulmonary infiltrations, ocular and skin lesions. However pulmonary manifestations typically dominate, any organ can be affected. Sometimes leading symptoms are caused by extrapulmonary manifestation of the disease, and together with the absence of typical picture in chest radiographs may be confusing for the physicians. We present 4 cases of proven sarcoidosis in which leading symptoms were caused by sarcoidal involvement of different organs (liver, spleen, heart and skin) without typical changes in the lung. In all cases multiorgan involvement was documented and disease was successfully treated.     

Pneumocystis carinii infection of the small intestine in a patient with acquired immune deficiency syndrome

The authors describe a patient with acquired immune deficiency syndrome (AIDS) who presented with an acute abdomen. A plaque-like tumor of the small intestine was resected and found to consist of masses of Pneumocystis carinii organisms. The organisms also exhibited a perivascular and intravascular distribution. Identical changes were found in regional lymph nodes. In addition to silver stains and electron microscopy, an immunohistochemical method for the demonstration of P. carinii was employed. The technique may have advantages over silver staining, as it identifies trophozoites in addition to cysts. A review of the literature concerning extrapulmonary pneumocystosis indicates that affected patients nearly always have concurrent pulmonary infection. The pattern of organ involvement and the finding of perivascular and intravascular organisms are consistent with lymphatic or hematogenous dissemination from the pulmonary focus. Pulmonary pneumocystosis was not documented in the patient described herein, although there were radiographic densities in one pulmonary lobe.     

Congenital pulmonary lymphangiectasis with chylothorax: A heterogeneous lymphatic vessel abnormality

We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defects is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich-Turner, and Down syndrome. © 1993 Wiley-Liss, Inc.     

Localized retroperitoneal lymphangioleiomyomatosis mimicking malignancy. A case report and review of the literature

Lymphangioleiomyomatosis (lymphangiomyomatosis [LAM]), a rare disease of unknown etiology that is seen only in women usually in the reproductive period, generally presents with features of pulmonary involvement. Extrapulmonary involvement, such as angiomyolipomas and retroperitoneal adenopathy, can occur in up to 75% of cases. It is very rare, however, for patients to present with features of extrapulmonary LAM. We present an unusual, localized case of LAM presenting with neurologic symptoms related to a retroperitoneal mass in a 51-year-old woman. Magnetic resonance imaging showed that the mass involved retroperitoneal lymph nodes, and a clinical diagnosis of atypical sarcoma (possibly from a uterine primary) was made. The mass was resected, and a total abdominal hysterectomy was performed. On pathologic examination, the mass showed classic histologic features of LAM with spread along lymphatic channels in the lymph nodes. Intralymphatic projections simulated lymphatic metastasis; however, the cytologic features were benign. Immunostains revealed the tumor to be positive for smooth muscle actin and desmin, but negative for HMB-45. The uterus was unremarkable, except for a subserosal leiomyoma. Although intratumoral variability for HMB-45 has recently been described, to the best of our knowledge, this is the first documented case of HMB-45-negative, histologically classic LAM. Because of the presence of several atypical features in this case, such as age, location, compressive neurologic presentation, radiologic impression of atypical sarcoma, and HMB-45 negativity, we feel that this case may represent a distinct, as yet uncharacterized variant of LAM.     

Responses of C fiber afferents of the rabbit airways and lungs to changes in extra-vascular fluid volume

Effects of changes in extra-vascular fluid volume produced by pulmonary lymphatic obstruction and plasmapheresis on the activities of bronchial and pulmonary C fiber receptors and rapidly adapting receptors (RARs) were investigated in New Zealand White rabbits. In intact rabbits, pulmonary lymphatic obstruction either alone or in combination with plasmapheresis did not stimulate pulmonary C fiber receptors. Only the combined stimulus activated the bronchial C fiber receptors. Bronchial C fiber receptors were also stimulated by graded increases in left atrial pressure (+5 and +10 mmHg). In contrast, RARs were activated by lymphatic obstruction either alone or in combination with plasmapheresis. These procedures increase the extra-vascular fluid volume in the carina and bronchi but not in the lungs (alveoli). In rabbits with chronic pulmonary venous congestion secondary to mitral valve damage, bronchial C fiber receptors were not stimulated by these increments in left atrial pressure which were insufficient to increase the extra vascular fluid content of the airways. However, both pulmonary and bronchial C fiber receptors were stimulated when the left atrial pressure was raised to 25 mmHg in these animals to cause pulmonary edema.     

Prevalence and Its Predictors of Extrapulmonary Involvement in Patients with Pulmonary Tuberculosis

Extrapulmonary organ involvement in human immunodefiaency virus (HIV)-infected patients with pulmonary tuberculosis (TB) is reported to be 26%, however, the clinical predictors of extrapulmonary involvement in pulmonary TB patients has not been reported yet. We tried to determine the clinical predictors of presence of extrapulmonary involvement in patients with pulmonary TB. Cross-sectional study was performed including all adult patients with culture-proven pulmonary TB diagnosed between January 1, 2004 and July 30, 2006, at a tertiary referral hospital in South Korea. The presence of extra-pulmonary TB involvement was diagnosed based on bacteriological, pathological, or clinical evidence. Among 320 patients with a culture-proven pulmonary TB, 40 had extrapulmonary involvement. Patients with bilateral lung involvement were more likely to have extrapulmonary involvement, with an adjusted odds ratio (OR) of 4.21 (95% confidence interval [CI], 1.82-9.72), while patients older than 60 yr (adjusted OR, 0.27; 95% CI, 0.08-0.89), patients with cavitary lesions (adjusted OR, 0.37; 95% CI, 0.16-0.84), and with higher levels of serum albumin (adjusted OR, 0.45; 95% CI, 0.25-0.78) had less frequent involvement. Clinicians should be aware of the possibility of extrapulmonary involvement in TB patients with bilateral lung involvement without cavity formation or lower levels of serum albumin.     

Histochemical reactivities of lectins and surfactant apoprotein in pulmonary adenocarcinomas and their metastases.

In an attempt to better understand the biologic behavior of neoplastic cell metastasis, a histochemical study with the use of six different lectins and a monoclonal antibody against human pulmonary surfactant apoprotein (PE-10) was carried out on primary adenocarcinomas of the lungs and their regional (usually lymphatic to lymph nodes or contralateral lung) and distant (usually hematogenous to extrathoracic organs) metastatic lesions of 54 postmortem cases. Primary pulmonary adenocarcinomas were classified further into acinar, papillary, and solid types according to WHO histological typing. Acinar type primary adenocarcinoma of the lungs showed significantly higher (p less than 0.05) binding reactions to Ricinus communis-I (RCA-I) and Ulex europaeus-I (UEA-I) lectins than solid type adenocarcinoma. With six different lectins, concordantly positive reactions between primary pulmonary adenocarcinomas and their lymphatic and hematogenous metastases were seen in 67% or more cases, and with soybean agglutinin (SBA) and UEA-I the concordance rates between primary and lymphatic metastases (lymph nodes and contralateral lungs, respectively) were significantly higher (p less than 0.05) than those between primary and hematogenous metastases. With PE-10 immunohistochemistry, concordantly positive reactions between primary and metastases were low, especially in cases of distant hematogenous metastases (25%), but the statistical significance of differences was missed by narrow margins. With alcian blue PAS-stain, concordantly positive reactions of mucin production between primary adenocarcinomas and both lymphatic and hematogenous metastases were high (92%), but there was no evidence of correlation between lectin bindings and alcian blue-PAS reactions to either primary or metastatic lesions of pulmonary adenocarcinomas.     

Pulmonary pathology in Gaucher's disease.

Gaucher's disease is a familial storage disease caused by a deficiency of the enzyme glucocerebrosidase. Pulmonary involvement is considered rare in Gaucher's disease, especially type I. Sporadic case reports have shown various types of lung involvement, but the spectrum of pulmonary pathology in Gaucher's disease has not been described. Nine cases of Gaucher's disease were retrieved from the autopsy file of Hadassah Medical Center, Jerusalem, Israel. There were six cases with type I Gaucher's disease and three cases with type II. Lung sections were evaluated, and special stains were employed, including immunohistochemical stains for CD68, cytokeratin, and CD34. Gaucher cells were found in the lungs in all nine cases. The involvement was considered pathologically significant in five of nine cases and clinically significant in three of nine cases. Four distinct patterns of pulmonary involvement by Gaucher cells emerged: intracapillary (9 of 9), patchy interstitial infiltrates in a lymphatic distribution (2 of 9), massive interstitial thickening of alveolar septa (1 of 9), and intra-alveolar infiltrates (2 of 9). The universal involvement of pulmonary capillaries indicates that this is probably systemic in nature and not intrinsic to the lungs. Pulmonary involvement in Gaucher's disease is commoner than previously recognized. Immunocytochemical stains help to identify isolated Gaucher cells and distinguish them from native alveolar macrophages.     

Pulmonary manifestations in Proteus syndrome: pulmonary varicosities and bullous lung disease

We report on two patients with Proteus syndrome (PS), with emphasis on its pulmonary manifestations. The first patient was a 6‐year‐old girl diagnosed with PS at 5 years of age. The pulmonary abnormalities first observed at age 3 years and included streaky densities with accentuated vascular markings detected by chest radiography. The patient had persistent abnormalities on follow‐up chest radiographs. Chest computed tomography (CT) scans showed diffuse pulmonary venous dilatations. The second patient was a 10‐year‐old boy diagnosed with PS at age 4 years. Chest radiography and CT scans showed patchy and streaky densities intermixed with small bullae, which were interpreted as pneumonia with post‐inflammatory pneumatoceles. The patient developed diffuse enlargement of air spaces of the lungs at age 10 years with severe respiratory compromise. Although pulmonary manifestations in PS are uncommon, recognition of pulmonary vein malformation and the presentation of enlarged air spaces in the lungs at an earlier age are important for accurate diagnosis. The plain radiograph findings of accentuated vascular markings seen in patients with PS may appear similar to interstitial or chronic pneumonia. This report emphasizes the features of lung involvement in children with PS and suggests that specific attention be paid to pulmonary manifestations using chest CT scans. © 2011 Wiley‐Liss, Inc.     

Severe granulomatous giant cell myocarditis in Wegener's granulomatosis

Summary A 28-year-old male patient suffering from Wegener's granulomatosis died suddenly with signs of cardiac failure after clinical symptoms had basically subsided under chemotherapy. Autopsy revealed pulmonary granulomata, necrotizing vasculitis of the lungs and kidneys, focal and segmental necrotizing glomerulonephritis, and diffuse granulomatous and necrotizing giant cell myocarditis.Histological confirmation of inflammation of the heart in Wegener's disease has rarely been reported. Although cardiac involvement in Wegener's granulomatosis sometimes is suspected, it is usually thought to have no major impact on the course of the disease.By its dramatic clinical and morphologic presentation this case illustrates that the heart, in addition to the lungs and kidneys, may determine the outcome of the idiopathic granulomatous vasculitis of Wegener.     

Acute Cavitary Histoplasmosis in Rhesus Monkeys: Influence of Immunological Status

Male rhesus monkeys were randomized into groups according to body weight, immunized with different Histoplasma capsulatum antigens, and two weeks later were infected intratracheally with 10(8)H. capsulatum yeast cells. Complement fixation antibody titers, skin tests, and chest X rays were performed at weekly intervals from immunization until autopsy, at which time the spleens were cultured and the lungs and other organs were dissected. Pulmonary cavities were found in 33% of the animals, and extrapulmonary dissemination was present in 85% of the animals. Delayed hypersensitivity and circulating antibody activity was detected in all animals at some time during the experimental period; however, animals which developed pulmonary cavities had a longer period before circulating antibodies were detected than animals which did not develop pulmonary cavities. Delayed hypersensitivity developed at approximately the same time in both cavitary and noncavitary animals. Early appearance of delayed hypersensitivity was associated with reduced amounts of extrapulmonary dissemination, in that animals with a later onset of skin test reactivity had more H. capsulatum cultured from the spleen. There was no correlation between the onset or titers of circulating antibodies and the spleen culture results.     

慢性阻塞性肺疾病骨骼肌萎缩的治疗现状

慢性阻塞性肺疾病(chronic obstructive pulmonary disease,COPD)不仅是一种局限于呼吸道和肺部的疾病,还是一种可以累及肺外各器官的全身疾病,其肺外效应包括心血管疾病、焦虑和抑郁及骨骼肌萎缩等疾病,其中骨骼肌萎缩严重影响患者的生活质量及预后,造成巨大的社会和经济负担.然而,肌肉组织固有的适应性为骨骼肌萎缩提供了治疗机会.适当的干预措施可逆转或延迟骨骼肌萎缩的发展进程.     

A QUANTITATIVE STUDY ON THE DISTRIBUTION OF ASBESTOS BODIES IN EXTRAPULMONARY ORGANS

We made a quantitative study of the distribution of asbestos bodies (ABs) in 13 extrapulmonary organs. We selected 26 male subjects and subdivided them into three groups: Group I had 10² to less than 10³ ABs per gram of wet tissue in their lungs; group II had 10 to 99 ABs; and group III had no ABs. In group I, one or more ABs were identified in at least 6 (53.8%) of the examined extrapulmonary organs. In group II, 23.7% of the examined organs had one or more ABs. In group III, no ABs were found. These results seem to indicate that with modest exposure in the lung, the other organs are often exposed to asbestos with some degree. Moreover, the number of AB in the esophagus was significantly larger than that in 9 of the other 12 organs in group I. The pancreas and the spleen were also supposed to be more preferential sites of exposure. However, the relationship between exposure and its effects in extrapulmonary organs remains to be elucidated.     

A quantitative study on the distribution of asbestos bodies in extrapulmonary organs

We made a quantitative study of the distribution of asbestos bodies (ABs) in 13 extrapulmonary organs. We selected 26 male subjects and subdivided them into three groups: Group I had 10(2) to less than 10(3) ABs per gram of wet tissue in their lungs; group II had 10 to 99 ABs; and group III had no ABs. In group I, one or more ABs were identified in at least 6 (53.8%) of the examined extrapulmonary organs. In group II, 23.7% of the examined organs had one or more ABs. In group III, no ABs were found. These results seem to indicate that with modest exposure in the lung, the other organs are often exposed to asbestos with some degree. Moreover, the number of AB in the esophagus was significantly larger than that in 9 of the other 12 organs in group I. The pancreas and the spleen were also supposed to be more preferential sites of exposure. However, the relationship between exposure and its effects in extrapulmonary organs remains to be elucidated.     

Disseminated mucormycosis in AIDS

Mucormycosis is a rare often fatal opportunistic condition. It mainly occurs in immunocompromised subjects but rarely in AIDS patients. An eighteen-year-old HIV+ man from Zaire died rapidly from disseminated mucormycosis with pulmonary, cardiac, renal, hepatic, splenic and gastric involvement. Fungi were observed in all these organs with a particular vascular trophism. Rhizopus orizae was identified on cultures. Rapid microscopic diagnosis, in the localized stage, could improve the poor prognosis of this infection.     

Diffuse Sclerosing Variant of Papillary Carcinoma of the Thyroid Report of Three Cases

Three cases of an unusual diffuse sclerosing variant of papillary carcinoma were found among 370 consecutive malignant tumors of the thyroid gland retained in the surgical pathology files of Kanazawa University Hospital over the last 10 years. The tumors were characterized by diffuse involvement of one or both lobes of the thyroid, dense fibrosis, papillary carcinoma with marked squamous metaplasia and abundant psammoma bodies, heavy lymphocytic infiltration and extensive lymphatic permeation. In two cases, the neoplasms showed more aggressive regional lymph node metastases and local invasion; the patient in the first case underwent surgery three times after initial radical surgery because of regional lymph node recurrences, and the second case showed direct invasion of the thyroid tumor to the larynx and distant metastasis to the lungs. Although the number of examined cases was small, the findings suggest that the diffuse sclerosing variant type has a less favorable prognosis and should be differentiated from papillary carcinomas with excellent prognosis.