Despite the poor prognosis of the disease, 5 years after the treatment the patient has not presented new episodes of pleuropericardial effusions, nor has he required hospitalization for other symptoms, currently presenting only exertional dyspnea. [archbronconeumol.org]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

In this paper, We would like to present an unusual case of Diffuse pulmonary lymphangiomatosis involving a 28-year-old young female, who presented with progressive chest pain and polypnea of one year duration, and CT showed much left hydrothorax. [atm.amegroups.com]

This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [atsjournals.org]

Polyposis-like presentation of multiple lymphangiomas of the sigmoid colon. Tumori 2004;90:625-7. [indianjcancer.com]

• Pseudotumor

  Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus. 38 Mills MK...Stilwill SE 29468291 2018 23 Lymphangiomatosis in a 14-year-old female presenting with chylothorax and multiple cystic lesions. 38 Uribe R...Quiceno W 30002781 2018 24 Pseudotumor [malacards.org]

• Internal Bleeding

  Signs and symptoms Lymphangiomatosis (LYMF) can present with pericardial, pleural effusion, ascites (fluid in the abdominal cavity), and internal bleeding. [bionity.com]

• Prolapse

  Local genital examination revealed grade-4 uterine prolapse with decubitus cervical ulcer, grade-2 rectocele, and grade-3 cystocele. [urologyannals.com]

• Skin Lesion

  Ninety per cent of patients have skin lesions, including hypomelanotic patches called 'ashleaf' spots that can best be visualized under a Woods lamp. Symptoms vary widely depending upon the location and size of the growths. [disorders.eyes.arizona.edu]

Clinical Testing and Workup Pulmonary function tests may be used to help diagnose diffuse pulmonary lymphangiomatosis. This group of tests can evaluate how well the lungs are functioning. [rarediseases.org]

[…] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed “diffuse pulmonary lymphangiomatosis” (DPL). 3 Recognition of the disease requires a high index of suspicion and an extensive workup [lgdalliance.org]

MRI showed obvious tumor shrinkage at 6 ( f ), 12 ( g ), and 24 ( h ) months after the start of sirolimus treatment Pathological findings All patients with KLA underwent incisional biopsy during the diagnostic workup (Fig. 1 d). [ojrd.biomedcentral.com]

• Human Papillomavirus

  Papillomavirus Infection. 38 Radzikowska E...Wiatr E 28398067 2017 42 Detection of mosaic 15q11.1-q11.2 deletion encompassing NBEAP1 and POTEB in a fetus with diffuse lymphangiomatosis. 38 Chen CP...Wang W 28420514 2017 43 A novel case of lymphangiomatosis [malacards.org]

It may be erroneously diagnosed as asthma responding to bronchodilator treatment, which was the reason for the late diagnosis in our case, previously diagnosed with COPD with bronchodilator treatment. [archbronconeumol.org]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

Treatment with sirolimus and prednisolone was then initiated. [ojrd.biomedcentral.com]

Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis. [resourcerepository.org]

It has a favorable prognosis when it is localized, and a poor prognosis when vital organs, including vertebral medulla, are involved. 3 When the disease is diffuse and extensive in a particular organ, as in the lungs in our case, the prognosis is also [scielo.br]

Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis. [archbronconeumol.org]

[…] the prognosis is generally poor. [3] There is no evidence of visceral involvement in the present cases. [cancerjournal.net]

[…] often in thoracic cavity, also bone, extremities, soft tissue, viscera Clinical features Rare, idiopathic, occurs mostly in children Cases with visceral involvement are often fatal ( Am J Surg Pathol 1993;17:329 ), but cases involving limbs have good prognosis [pathologyoutlines.com]

The systemic form which was noted in this case above differs from the localised forms of the disease – which are isolated to a single anatomic location, classically bone, and have a good prognosis. [ghrnet.org]

Etiology Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. [orpha.net]

The disease can be considered a failure of normal lymphatic development, but the etiology remains poorly understood. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy. [genome.jp]

Pulmonary lymphangioleiomyomatosis (LAM) is a benign, uncommon disorder of unknown etiology affecting women of child-bearing age ( 7 ). [atm.amegroups.com]

The proliferative lymphatic aspect suggests a neoplastic etiology, and the structural disorganization indicates a hamartomatous origin. [scielo.br]

Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis. [archbronconeumol.org]

Summary Epidemiology The prevalence is unknown. [orpha.net]

Epidemiology Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection 8. Clinical features Patients present with "asthma", dyspnea or haemoptysis 8 ,12. [e-immunohistochemistry.info]

There is limited information available on the pathophysiology, treatment responses, and long-term outcomes of these disorders. [atsjournals.org]

It highlights the need for further research into the pathophysiology and management of lymphangiomatosis as current treatment options are limited. http://www.liebertonline.com/doi/abs/10.1089/lrb.2011.0021 .................... [lymphedemapeople.com]

Treatment Treatment Options: No effective preventative treatment exists but individual lesions can be surgically removed when indicated. [disorders.eyes.arizona.edu]

In the case of recurring chylothorax, drainage and pleurodesis are indicated, with the option of thoracic duct ligation 3 to prevent complications such as malnutrition, pulmonary fibrosis and deteriorated respiratory function. [archbronconeumol.org]

Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]

The biologic effect of imatinib, when prescribed at higher concentrations, has been shown to inhibit angiogenesis by blocking the activity of c-kit tyrosine kinase cell surface receptor and PDGFR kinase, 22 and through this mechanism, is perhaps preventing [jnccn.org]

This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid. [lymphedemapeople.com]