Linfangiomatosis pulmonar difusa con afectación mediastínica

Caballero, Yulennis Vega; Pérez, Darío Morais; Cano, Juan

doi:10.1016/j.arbres.2011.06.001

Cited by 7 publications

(3 citation statements)

References 7 publications

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“…Though our follow-up was short, our case had no recurrence after repeated thoracocentesis. Thoracoscopic ligation of thoracic duct is also successfully reported in adult patient [ 7 ]. However, surgery for thoracic duct is successful if the effusion is chylous and lymphangiography suggests obstruction or leakage in thoracic duct [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

Simple Lymphangioma to Generalized Lymphatic Anomaly: Role of Imaging in Disclosure of a Rare and Morbid Disease

Joshi

Phansalkar

2015

Case Reports in Radiology

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Generalized lymphatic anomaly is a rare multisystem congenital disorder in which multiple organs are involved. Imaging features often overlap with other complex lymphatic anomalies and diagnosis is difficult. Treatment options are limited, not remedial and prognosis is poor. We report a 12-year-old male who presented with axillary and chest wall lymphangioma but was subsequently diagnosed as having diffuse lymphangiomatosis affecting lungs, liver, spleen, and bones on computerized tomography scan. We suggest complete radiological evaluation of susceptible adolescent children with lymphangioma to avoid diagnostic delay in this morbid condition. We also discuss radiological features of other similar complex lymphatic anomalies and crucial role of imaging in diagnosis.

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Section: Discussionmentioning

confidence: 99%

Simple Lymphangioma to Generalized Lymphatic Anomaly: Role of Imaging in Disclosure of a Rare and Morbid Disease

Joshi

Phansalkar

2015

Case Reports in Radiology

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“…DPL mainly affects the lungs and pleura, and can also cause diffuse involvement of extrapulmonary tissue (such as lymph nodes and their surrounding soft tissue), which is lacking in pathological reports. [ 14 ] The manifestations of lymph nodes involvement are mainly lymphoid hyperplasia and fibrous thickening capsule in which there are different degrees of proliferation, expansion, and deformity of the lymphatic vessels. Soft tissue around lymph nodes is often involved, and its pathological findings include hyperplasia, dilatation, and deformity of lymph vessels with different degrees besides the hyperplasia of fibrous adipose tissue and smooth muscle cells of the tube wall.…”

Section: Discussionmentioning

confidence: 99%

Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue

et al. 2017

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Background:Diffuse pulmonary lymphangiomatosis (DPL) mainly affects the lung and pleura. There are very few pathological reports of lung damage accompanied by diffuse involvement of the extrapulmonary lymph nodes and surrounding soft tissue. The clinicopathological significance of coexistence of pulmonary and extrapulmonary lesions is unknown.Methods:Here, we report a 16-year-old male patient. The pathological specimens of the supraclavicular lymph node and soft tissue together with the lung biopsy were analyzed by pathological observation and immunohistochemical staining. Literatures were reviewed and clinical and imaging findings were discussed.Results:The patient presented with coughing and expectoration for 1 year and intermittent hemoptysis for 4 months. Ultrasound revealed swollen lymph nodes in bilateral neck, left armpit, and pubic symphysis. Chest CT scan showed diffuse grid and linear shadows, bilateral pleural thickening, and nodule formation. Multiple enlarged lymph nodes were mainly investigated in bilateral hilar, mediastinal, para-aortic, lesser curvature, and retroperitoneal. Supraclavicular lymph node biopsy confirmed the lymphatic hyperplasia and expansion in the capsule and surrounding soft tissue. The thoracoscopic examination found bloody chylothorax on the left chest. And lung biopsy showed the lymphatic vessel hyperplasia and expansion on the pleura and adjacent lung tissue. Immunohistochemical stains showed that the lymphatic endothelial cells were positive for D2–40 and CD31. Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue was diagnosed based on the aforementioned histological findings.Conclusion:Lymphangiomatosis of superficial lymph node mainly involves the capsule of lymph nodes and its surrounding soft tissue. The information obtained from the lymph node biopsy can prompt and assist the diagnosis of DPL.

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“…This was supported by episodes of fever, increased WBC count and slightly decreased density of opacity after the antibiotic infusion in this case. To the best of our knowledge, all reported patients have had involvement of the pulmonary lymphatic vessels bilaterally or mediastinal structures including lymph nodes, thoracic duct and occasionally the pericardium [ 4 ]. There are rare reports of diffuse lymphangiomatosis involving one organ such as the kidney [ 5 ] and breast [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

A case of diffuse pulmonary lymphangiomatosis with unilateral lung invasion: Figure 1:

et al. 2015

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Diffuse pulmonary lymphangiomatosis (DPL) is a rare interstitial lung disease characterized by intrathoracic lymphatic system abnormalities often with involvement of both lungs. Here, we report a 24-year-old male patient with DPL initially located in one lung, presenting only with transient fever. Resection of the right middle and lower lobes was performed for diagnosis and complete removal of the lesions. The pathologic features shown by diffuse smooth thickening of the interlobular septa, bronchovascular bundles, infiltration of patchy ground glass opacities and specific immunohistologic D2-40 and CD34 positive staining confirmed the diagnosis of DPL. The patient did not show signs of relapse during the 2-year follow-up period, which suggests that surgery is an effective and reasonable method for treating DPL with relatively localized lesions.

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Linfangiomatosis pulmonar difusa con afectación mediastínica

Cited by 7 publications

References 7 publications

Simple Lymphangioma to Generalized Lymphatic Anomaly: Role of Imaging in Disclosure of a Rare and Morbid Disease

Simple Lymphangioma to Generalized Lymphatic Anomaly: Role of Imaging in Disclosure of a Rare and Morbid Disease

Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue

A case of diffuse pulmonary lymphangiomatosis with unilateral lung invasion: Figure 1:

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