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Alkhulaifat D, Ramirez-Suarez KI, Otero HJ, Rapp JB, Srinivasan AS, Smith CL, Biko DM. Complex lymphatic anomalies. Pediatr Radiol 2025:10.1007/s00247-025-06167-9. [PMID: 39853393 DOI: 10.1007/s00247-025-06167-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/21/2024] [Revised: 12/20/2024] [Accepted: 01/02/2025] [Indexed: 01/26/2025]
Abstract
Complex lymphatic anomalies are unique diseases marked by abnormal lymphatic vessel development and growth. Imaging is crucial in the evaluation and management of complex lymphatic anomalies, with dynamic contrast-enhanced MR lymphangiography emerging as a valuable modality for visualizing abnormal lymphatic structures and informing treatment decisions. This article gives an overview of complex lymphatic anomalies and their management strategies, focusing specifically on generalized lymphatic anomaly, Gorham-Stout disease, Kaposiform lymphangiomatosis, and central conducting lymphatic anomalies. Simple lymphatic diseases are beyond the scope of this review.
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Affiliation(s)
- Dana Alkhulaifat
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
| | | | - Hansel J Otero
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
- Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA
| | - Jordan B Rapp
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
- Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA
| | - Abhay S Srinivasan
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
- Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA
| | - Christopher L Smith
- Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA
- Center for Lymphatic Imaging and Interventions, Children's Hospital of Philadelphia, Philadelphia, PA, USA
- Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
| | - David M Biko
- Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA
- Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA, USA
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Tuan NA, Hien NX, Trinh ND, Van Khanh L, Ly DV, Thinh NH, Anh NP, Cuong NN. Generalized lymphangiomatosis in patients treated for chylothorax following thoracoscopic sympathectomy: Case report. Radiol Case Rep 2024; 19:4725-4729. [PMID: 39228954 PMCID: PMC11366932 DOI: 10.1016/j.radcr.2024.07.118] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2024] [Revised: 07/18/2024] [Accepted: 07/21/2024] [Indexed: 09/05/2024] Open
Abstract
Generalized lymphangiomatosis (GLA) is a very rare condition in adults, characterized by diffused proliferation of lymphatic vessels that requires differential diagnosis from other vascular disorders such as cavernous or capillary hemangioma. This is because of overlapping characteristics on histopathological examination. Therefore, imaging features such as CT and MRI are useful to evaluate morphological characteristics, location, and the extent of the spread as well as differential diagnosis with other pathologies. We report a case of a 22-year-old female patient with left hemothorax after thoracoscopic sympathectomy for the treatment of hand sweating. The patient underwent drainage and cleaning of the left pleura. Chest computed tomography and lumbar spine magnetic resonance imaging showed multiple fat infiltration foci of the lumbar spine and pelvis. A wing bone biopsy of the pelvis was initially performed for the diagnosis of chronic osteomyelitis. Afterwards, the patient continued to have pleural drainage and developed hemothorax and chylothorax, amounting to 3000 mL. The chest tube was blocked with a mixture of biological glue and lipiodol (2 mL of glue, ratio of glue to lipiodol: 1:4) and a 3 i-ED coil complex. After the intervention, the pleural fluid decreased; the left pleural fluid was still 15 mm thick, and the amount of fluid drained after 1 week was 100 mL. Aspiration of the chest wall lesion showed fluid rich in fat droplets. Combined with the results of lumbar spine magnetic resonance imaging and the old biopsy, this was consistent with generalized lymphangiomatosis.
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Affiliation(s)
- Nguyen Anh Tuan
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Nguyen Xuan Hien
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Nguyen Duy Trinh
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Le Van Khanh
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Dao Văn Ly
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Nguyen Hoang Thinh
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Nguyen Phuong Anh
- Department of imaging and interventional radiology, Tam Anh hospital, Hanoi, Vietnam
| | - Nguyen Ngoc Cuong
- Department of imaging and interventional radiology, Ha Noi medical university hospital, Hanoi, Vietnam
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Tabrizi Z, Dadkhah A, soleimani S, Moaddab M. Generalized cystic lymphangiomatosis incidentally diagnosed in an asymptomatic adult: Imaging findings of a very rare case. Radiol Case Rep 2024; 19:130-135. [PMID: 37941984 PMCID: PMC10628799 DOI: 10.1016/j.radcr.2023.10.017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/25/2023] [Revised: 10/01/2023] [Accepted: 10/05/2023] [Indexed: 11/10/2023] Open
Abstract
Lymphangiomas are benign lesions of vascular origin with lymphatic differentiation, most commonly found in the head and neck. Generalized lymphangiomatosis is a very rare condition in adults, which is characterized by a diffuse proliferation of lymphatic vessels. The lymphangioma is composed of lymphatic endothelium-lined cystic spaces. This condition can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma. However, many cases of lymphangioma can be confused with other vascular disorders, because of overlapping histologic findings. radiologic examinations, such as CT scan and MR imaging, are useful for assessing the morphologic feature and also the extent of disease, it is important to know the radiologic findings of generalized lymphangiomatosis. In this paper, we report a case of generalized lymphangiomatosis in a 42-year-old male who presented with left flank pain and hematuria. The first differential diagnosis was renal colic; hence he underwent an abdominopelvic computed tomography scan (CT scan). In the performed CT scan multiple cystic lesions were seen in the liver and spleen. Also, lytic lesions were seen in bones. CT-guided biopsy was performed and the result was compatible with generalized lymphangiomatosis, confirmed by cytology. Generalized lymphangiomatosis is a rarely reported disease in children and young adults. Delayed diagnosis in older patients or misdiagnosis is common due to its rarity and nonspecific clinical presentation. Different imaging modalities can incidentally diagnose the disease in asymptomatic patients. So radiologists should be aware of the disease manifestations in imaging modalities to diagnose the disease sooner and help the clinician start the therapy if needed.
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Affiliation(s)
- Zhale Tabrizi
- Department of Radiology, Iran University of Medical Science, Tehran, Iran
| | - Adeleh Dadkhah
- Department of Radiology, Hasheminejad Kidney Center (HKC), Iran University of Medical Science, Tehran, Iran
| | - Shakiba soleimani
- Department of Radiology, Iran University of Medical Science, Tehran, Iran
| | - Maryam Moaddab
- Department of Radiology, Iran University of Medical Science, Tehran, Iran
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Sainz Sánchez J, Aranaz Murillo A, Andrés Villares E, García Maroto J. Generalized lymphatic anomaly in adult patients: An eminently radiological diagnosis. RADIOLOGIA 2023; 65:481-485. [PMID: 37758338 DOI: 10.1016/j.rxeng.2022.08.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2022] [Accepted: 08/09/2022] [Indexed: 10/03/2023]
Abstract
Generalized lymphatic anomaly (GLA) is an uncommon congenital disease secondary to the proliferation of lymphatic vessels in any organ except the central nervous system. GLA has a wide spectrum of clinical and radiological presentations, among which osteolytic lesions are the most widespread, being the ribs the most commonly affected bone. GLA is diagnosed mainly in children and young adults; nevertheless, on rare occasions it can remain asymptomatic and be detected incidentally in older patients. We present an unusual case of GLA in an asymptomatic 54-year-old man who had atypically distributed, purely cystic bone lesions on CT; measuring the Hounsfield (HU) of these lesions enabled us to suspect GLA. This suspicion was confirmed with MRI, PET/CT, CT-guided fine-needle aspiration biopsy, and fluoroscopy-guided percutaneous vertebral biopsy. After surgical resection of one of the lesions, histologic study provided the definitive diagnosis.
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Affiliation(s)
- J Sainz Sánchez
- Servicio de Radiodiagnóstico, Hospital Universitario Miguel Servet, Zaragoza, Spain.
| | - A Aranaz Murillo
- Servicio de Radiodiagnóstico, Hospital Universitario Miguel Servet, Zaragoza, Spain
| | - E Andrés Villares
- Servicio de Urgencias y Medicina Familiar y Comunitaria, Hospital Hernest Lluch, Zaragoza, Spain
| | - J García Maroto
- Servicio de Radiodiagnóstico, Hospital Universitario Miguel Servet, Zaragoza, Spain
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Giant cervicofacial cystic lymphangioma. JOURNAL OF PEDIATRIC SURGERY CASE REPORTS 2022. [DOI: 10.1016/j.epsc.2022.102226] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
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Duffy P, Castro-Aragon I, Tivnan P, Volberg FM, Kipervasser E, Harkanyi Z, Paltiel HJ. Spleen and Peritoneal Cavity. PEDIATRIC ULTRASOUND 2021:481-561. [DOI: 10.1007/978-3-030-56802-3_13] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Hazarika D, Aggarwal A. A Case of Abdominothoracic Cystic Lymphangiomatosis Presenting as Left-Sided Inguinal Swelling in a Young Adult: Radiological Manifestation. JOURNAL OF GASTROINTESTINAL AND ABDOMINAL RADIOLOGY 2020. [DOI: 10.1055/s-0040-1718248] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
Abstract
AbstractLymphatic system imaging has been a challenging field in Radiology. We here describe a case of a 26-year-old male who presented with complaints of left-sided inguinoscrotal swelling and was subsequently diagnosed with abdomino-thoracic cystic lymphangiomatosis. Detailed imaging evaluation across the modalities of ultrasonography, computed tomography scan and magnetic resonance imaging evaluation revealed extensive involvement and diagnostic imaging appearance of the unique lymphatic system abnormality. Imaging findings were confirmed on histopathology.
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Affiliation(s)
- Dibyamohan Hazarika
- Department of Diagnostic & Interventional Radiology, Ayursundra Superspecialty Hospital, Guwahati, Assam, India
| | - Abhinav Aggarwal
- Department of Radiology, Mata Chanan Devi Hospital, New Delhi, Delhi, India
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Le HDT, Vo DS, Le DD, Dang CT, Nguyen Thanh T. Generalized lymphangiomatosis-A rare manifestation of lymphatic malformation. Radiol Case Rep 2020; 16:66-71. [PMID: 33193931 PMCID: PMC7642761 DOI: 10.1016/j.radcr.2020.10.044] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2020] [Revised: 10/22/2020] [Accepted: 10/23/2020] [Indexed: 11/21/2022] Open
Abstract
Lymphangiomatosis is a rare benign proliferation and dilation of the lymphatic channels that can involve multiple organs with a variety of clinical presentations. We report a case of generalized lymphangiomatosis in a 16-year-old male who presented with a subcutaneous swelling and pain over his lower back area. The patient was diagnosed of generalized lymphangiomatosis with involvement of soft tissues, pulmonary, thoracic and lumbosacral vertebrae, and spinal canal which subsequently confirmed by cytology. In this case report, we aim to discuss radiological features of the relevant differentials, and the patient's symptoms. We also briefly discuss the treatment options as well as prognosis.
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Affiliation(s)
- Hoang Di Thu Le
- Department of Radiology, Hue University of Medicine and Pharmacy, Hue University, Hue, Vietnam
| | - Da Suong Vo
- Department of Radiology, Hue University of Medicine and Pharmacy, Hue University, Hue, Vietnam
| | - Dinh Dam Le
- Department of Surgery, Hue University of Medicine and Pharmacy, Hue University, Hue, Vietnam
| | - Cong Thuan Dang
- Department of Histology, Embryology, Pathology & Forensic Medicine, Hue University of Medicine and Pharmacy, Hue University, Hue, Vietnam
| | - Thao Nguyen Thanh
- Department of Radiology, Hue University of Medicine and Pharmacy, Hue University, Hue, Vietnam
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Raufaste Tistet M, Ernst O, Lanchou M, Vermersch M, Lebert P. Imaging features, complications and differential diagnoses of abdominal cystic lymphangiomas. Abdom Radiol (NY) 2020; 45:3589-3607. [PMID: 32296900 DOI: 10.1007/s00261-020-02525-3] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
PURPOSE The aim of the study is to describe the imaging features, complications and differential diagnoses of abdominal cystic lymphangiomas (ACLs). RESULTS ACLs are benign lymphatic malformations that mainly arise in the subperitoneal space and the retroperitoneum. The typical presentation of an ACL is a multilocular lesion with homogenous serous content, presenting a thin wall and septa, usually free from adjacent organ compression. Atypical findings, including fat or hemorrhagic content, septal calcifications and unilocular presentation, are not uncommon. Rarely, ACLs can be revealed by acute complications, such as infection, hemorrhage, intussusception, complications with a twisting mechanism (including torsion around its own pedicle) or spontaneous rupture, which can be diagnosed by imaging. Ultrasonography and CT are the most useful modalities in emergency situations. MRI performs best in the noninvasive characterization of cystic lesions. ACLs should be differentiated from normal anatomic structures (e.g., cisterna chyli) or pitfalls (e.g., ascites, extrapancreatic necrosis, lymphocele) that can simulate ACLs. Among other primary peritoneal cystic lesions, benign cystic mesothelioma can be difficult to differentiate from ACL. Some neoplastic peritoneal lesions may have cystic components or content that looks like fluid on imaging (such as mucinous or myxoid content) and be misdiagnosed as ACL. Nodular or thick enhancement of the wall or septa should then be considered worrisome features and should not suggest ACL. ACLs mostly require a simple follow-up. If treatment is necessary, percutaneous sclerotherapy is a safe and effective alternative to surgery. CONCLUSION Imaging, especially MRI, allows the noninvasive diagnosis of ACL and helps to exclude potential malignant differential diagnoses.
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Affiliation(s)
- M Raufaste Tistet
- Department of Digestive Diagnostic and Interventional Radiology, University Hospital Claude Huriez - Regional University Hospital Center, rue Michel Polonoski, 59037, Lille Cedex, France.
| | - O Ernst
- Department of Digestive Diagnostic and Interventional Radiology, University Hospital Claude Huriez - Regional University Hospital Center, rue Michel Polonoski, 59037, Lille Cedex, France
| | - M Lanchou
- Department of Digestive Diagnostic and Interventional Radiology, University Hospital Claude Huriez - Regional University Hospital Center, rue Michel Polonoski, 59037, Lille Cedex, France
| | - M Vermersch
- Department of Digestive Diagnostic and Interventional Radiology, University Hospital Claude Huriez - Regional University Hospital Center, rue Michel Polonoski, 59037, Lille Cedex, France
| | - P Lebert
- Department of Digestive Diagnostic and Interventional Radiology, University Hospital Claude Huriez - Regional University Hospital Center, rue Michel Polonoski, 59037, Lille Cedex, France
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Hou G, Jiang Y, Jing H, Xu W, Xu KF, Chen L, Li F, Cheng W. Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders: Gorham-Stout disease, lymphangioma, and lymphangioleiomyomatosis. Medicine (Baltimore) 2020; 99:e22414. [PMID: 32991473 PMCID: PMC7523828 DOI: 10.1097/md.0000000000022414] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
The purpose of this study was to investigate the role of Tc-antimony sulfide colloid (ASC) lymphoscintigraphy and single photon emission computed tomography/computed tomography (SPECT/CT) in the evaluation of rare lymphatic disorders, including Gorham--Stout disease (GSD), lymphangioma, and lymphangioleiomyomatosis (LAM).Nine patients suspected to have rare lymphatic disorders were included in this retrospective study. All patients underwent Tc-ASC lymphoscintigraphy and SPECT/CT to evaluate the lesions. The lymphoscintigraphy results were compared with the clinical and immunopathological findings.Tc-ASC lymphoscintigraphy and SPECT/CT could provide lymphatic draining and anatomical information for rare lymphatic disorders. Among the 9 patients, 3 were diagnosed with GSD (1 female, 2 males; aged 15-34 years, range 27.0 ± 10.4 years), 3 with lymphangioma (1 female, 2 males; aged 17-42 years, range 32.0 ± 13.2 years), and 3 patients were diagnosed with LAM (3 females; aged 33-50 years, range 43.7 ± 9.3 years]. GSD is characterized by multiple bone destruction, including spine, ribs, ilium, pubis, ischium, and femur. The tracer uptake of involved bones and soft tissue around bone is increased, accompanied by chylothorax, chylopericardium, and chylous leakage in abdominal and pelvic cavity. Lymphangiomas present as multiple cystic lesions with increased tracer uptake in the peripancreatic, retroperitoneal, and iliac areas, and in the abdominopelvic cavity. LAM presents as multiple thin-walled cysts in the bilateral lungs and multiple retroperitoneal enlarged lymph nodes with increased tracer uptake.Tc-ASC lymphoscintigraphy and SPECT/CT could comprehensively and specifically detect some rare lymphatic disorders, namely, GSD, lymphangioma, and LAM. This technique is useful for the evaluation of GSD, lymphangioma, and LAM.
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Affiliation(s)
- Guozhu Hou
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
| | - Yuanyuan Jiang
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
| | - Hongli Jing
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
| | - Wenshuai Xu
- Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Kai-Feng Xu
- Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Libo Chen
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
| | - Fang Li
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
| | - Wuying Cheng
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
- Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Beijing, China
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Itkin M, Rabinowitz DA, Nadolski G, Stafler P, Mascarenhas L, Adams D. Abnormal Pulmonary Lymphatic Flow in Patients With Lymphatic Anomalies and Respiratory Compromise. Chest 2020; 158:681-691. [PMID: 32220591 DOI: 10.1016/j.chest.2020.02.058] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2019] [Revised: 02/09/2020] [Accepted: 02/15/2020] [Indexed: 10/24/2022] Open
Abstract
BACKGROUND Pulmonary involvement in lymphatic anomalies (LA) is associated with significant morbidity and mortality. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is capable of imaging the lymphatic system in a variety of pulmonary lymphatic disorders. RESEARCH QUESTION The objective of this study is to describe the central lymphatic anatomy in patients with LA and pulmonary involvement on DCMRL. STUDY DESIGN AND METHODS This prospective observational study enrolled 16 patients with LA (mean age, 17 years; range, 6-63 years; ratio of female to male patients, 9:7) with pulmonary involvement. All patients underwent DCMRL. The lymphatic system was assessed for the presence of mediastinal masses, interstitial lung disease, size and tortuosity of the thoracic duct (TD), and presence of abnormal pulmonary lymphatic flow. RESULTS T2-weighted imaging showed the following: mediastinal soft tissue masses in 10 patients, diffuse pulmonary interstitial thickening in 13 patients, and bone involvement in 15 patients. DCMRL revealed abnormal pulmonary lymphatic flow in 14 of 16 patients. Abnormal pulmonary lymphatic flow originated from the TD in three of 14 patients, the retroperitoneum in six of 14 patients, and both the TD and retroperitoneum in four of 14 patients. In nine of 16 patients, the TD was dilated and tortuous. In two patients the TD was not identified, and in five patients it was normal. INTERPRETATION Abnormal pulmonary lymphatic flow/perfusion from the TD or retroperitoneum into the lung parenchyma occurred in the majority of patients in this study. These findings can explain the interstitial lung disease and chylothorax resulting in deterioration of respiratory function in these patients. Future studies will determine whether mechanical cessation of this abnormal flow can improve pulmonary function and prolong survival in patients with LA. CLINICAL TRIAL REGISTRATION Clinicaltrials.gov; No.: NCT02744027; URL: www.clinicaltrials.gov.
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Affiliation(s)
- Maxim Itkin
- Center for Lymphatic Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA; Department of Radiology, Nemours/AI DuPont Children's Hospital, Wilmington, DE.
| | | | - Gregory Nadolski
- Center for Lymphatic Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA
| | - Patrick Stafler
- Pulmonary Institute, Schneider Children's Medical Center, Petach-Tikva, Israel
| | - Leo Mascarenhas
- Division of Oncology and Hematology, Children's Hospital of Los Angeles, Los Angeles, CA
| | - Denise Adams
- Cancer and Blood Disorders Center, Boston Children's Hospital, Boston, MA
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Sephien A, Mousa MS, Bui MM, Kedar R, Thomas K. Leiomyosarcoma of the Inferior Vena Cava with Hepatic and Pulmonary Metastases: Case Report. J Radiol Case Rep 2019; 13:30-40. [PMID: 31558957 DOI: 10.3941/jrcr.v13i5.3641] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022] Open
Abstract
Sarcomas are connective tissue tumors accounting for only 1% of all adult malignancies. Leiomyosarcoma (LMS) is a sarcoma arising from smooth muscle cells, and accounts for 10-20% of all sarcomas. A subtype of LMS are those originating from the smooth muscle of blood vessels. Leiomyosarcoma of the inferior vena cava is a sarcomatous tumor, with less than 350 cases described in the literature. It carries a poor prognosis, with 5- and 10-year survival rates of 31.4% and 7.4%, respectively. We present a case of a 46-year-old female with no significant past medical history presented to the emergency department with mild abdominal pain and distention, early satiety, and weight loss for three weeks, found to have unresectable metastatic leiomyosarcoma of the inferior vena cava.
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Affiliation(s)
- Andrew Sephien
- Department of Diagnostic Imaging and Interventional Radiology, H. Lee Moffitt Cancer and Research Institute, Tampa, FL, USA
- Department of Radiology, University of South Florida Morsani College of Medicine, Tampa, FL, USA
| | - Mina S Mousa
- Department of Diagnostic Imaging and Interventional Radiology, H. Lee Moffitt Cancer and Research Institute, Tampa, FL, USA
- Department of Radiology, University of South Florida Morsani College of Medicine, Tampa, FL, USA
| | - Marilyn M Bui
- Department of Pathology, H. Lee Moffitt Cancer and Research Institute, Tampa, FL, USA
| | - Rajendra Kedar
- Department of Radiology, University of South Florida Morsani College of Medicine, Tampa, FL, USA
| | - Kerry Thomas
- Department of Diagnostic Imaging and Interventional Radiology, H. Lee Moffitt Cancer and Research Institute, Tampa, FL, USA
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Xv FY, Sun A, Gan Y, Hu HJ. Gastric duplication cyst mimicking large cystic lymphangioma in an adult: A rare case report and review of the literature. World J Clin Cases 2019; 7:2087-2093. [PMID: 31423442 PMCID: PMC6695537 DOI: 10.12998/wjcc.v7.i15.2087] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/19/2019] [Revised: 06/25/2019] [Accepted: 07/03/2019] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Gastric duplication cysts (GDCs) are a relatively uncommon congenital developmental abnormality, mainly occurring in infants but very rarely in adults. Because of the variability in clinical presentation, it is often quite challenging to diagnose GDCs in adults. We are presenting a case report of an adult diagnosed operatively as having a GDC with a literature review to summarize clinical and imaging features and the treatment selections of GDCs in adults so that doctors could have a comprehensive understanding of this disease and make a precise diagnosis and a suitable therapeutic decision for patients.
CASE SUMMARY A 51-year-old man presented with recurrent epigastric pain and fullness for two years. No significant findings were noted during physical examination and routine blood tests were unremarkable. An abdominal ultrasound revealed a large cyst in the upper left abdominal quadrant. A following contrast-enhanced abdominal computed tomography (CT) scan demonstrated a hypodense cystic lesion between the spleen and stomach. The lesion had scattered calcification in the cyst wall without any significant enhancement. The lesion was initially thought to be a cystic lymphangioma. The patient underwent a surgical resection and intraoperatively it was noted that the lesion was closely adherent to the greater curvature of the stomach. Subsequently, a resection of the gastric mass along with a partial gastrectomy was performed. The patient recovered quickly with a complete symptomatic relief and did not show any further complications during the 8-month follow-up.
CONCLUSION GDCs are quite rare in adults, with a multitude of symptoms, which is quite challenging for precise diagnosis before histological examination. Some imaging techniques involving CT, magnetic resonance imaging, and endoscopic ultrasound could provide valuable morphological features for differential diagnosis.
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Affiliation(s)
- Fang-Yi Xv
- Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
| | - Alex Sun
- Diagnostic Radiology - Musculoskeletal Imaging, University of California, San Diego, CA 92093, United States
| | - Yi Gan
- Department of Pathology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
| | - Hong-Jie Hu
- Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
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Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus. Skeletal Radiol 2018; 47:1293-1297. [PMID: 29468291 DOI: 10.1007/s00256-018-2903-y] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2017] [Revised: 01/29/2018] [Accepted: 01/31/2018] [Indexed: 02/02/2023]
Abstract
Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported. We will review the clinical and imaging manifestations of lymphangiomatosis and provide a differential diagnosis for masses of the lumbosacral plexus. Although lower extremity pain and weakness encountered in the emergency department or outpatient setting is most frequently caused by lumbar spine pathology, occasionally, abnormalities of the lumbosacral plexus may prove to be the cause. While peripheral nerve sheath tumors lead the differential diagnosis of tumor or tumor-like entities involving the lumbosacral plexus, lymphangiomatosis is a rare differential consideration.
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Lymphangiomatosis in a 14-year-old female presenting with chylothorax and multiple cystic lesions. Radiol Case Rep 2018; 13:782-787. [PMID: 30002781 PMCID: PMC6041379 DOI: 10.1016/j.radcr.2018.05.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2018] [Revised: 05/01/2018] [Accepted: 05/05/2018] [Indexed: 01/02/2023] Open
Abstract
Lymphangiomatosis is a rare congenital disease; diagnosis is made in the first 2 decades and affects almost all body parts. Imaging findings play an important role in the diagnosis. We present a case of a patient with lymphangiomatosis whose diagnosis was made solely with imaging findings; we also include a small review of the topic.
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Rübenthaler J, Paprottka K, D'Anastasi M, Reiser M, Clevert DA. Diagnosis of perinephric retroperitoneal lymphangioma supported by contrast-enhanced ultrasound (CEUS). Clin Hemorheol Microcirc 2017; 65:43-47. [PMID: 27716656 DOI: 10.3233/ch-169000] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Lymphangiomas are a rare condition, which are characterized by multiple cystic lesions of a single or multiple organs that are thought to originate from intrauterine atypically distended and connected lymphatic tissue. We describe a case of a 56 years old woman with the final diagnosis of a perinephric lymphangioma. With the use of contrast-enhanced ultrasound (CEUS) it was possible to add valuable diagnostic information regarding the extent of the lymphangioma to surrounding tissue without the necessity to use additional ionizing radiation or nephrotoxic contrast agents.
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Valakada J, Madhusudhan KS, Ranjan G, Garg PK, Sharma R, Gupta AK. Abdominal Lymphangiomatosis With Intestinal Lymphangiectasia Diagnosed by Magnetic Resonance Lymphangiography: A Case Report. Curr Probl Diagn Radiol 2017; 47:200-202. [PMID: 28554788 DOI: 10.1067/j.cpradiol.2017.04.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/06/2017] [Accepted: 04/06/2017] [Indexed: 12/20/2022]
Affiliation(s)
- Jineesh Valakada
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Kumble S Madhusudhan
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.
| | - Gyan Ranjan
- Department of Gastro-enterology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Pramod Kumar Garg
- Department of Gastro-enterology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Raju Sharma
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Arun Kumar Gupta
- Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
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Luisi F, Torre O, Harari S. Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis). Eur Respir Rev 2017; 25:170-7. [PMID: 27246594 PMCID: PMC9487238 DOI: 10.1183/16000617.0018-2016] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2016] [Accepted: 04/21/2016] [Indexed: 01/05/2023] Open
Abstract
Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches. Possible diagnostic and therapeutic approaches to generalised lymphatic anomaly (lymphangiomatosis)http://ow.ly/4n4pgU
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Affiliation(s)
- Francesca Luisi
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
| | - Olga Torre
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
| | - Sergio Harari
- Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Multimedica IRCCS, Milan, Italy
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Intraabdominal Lymphatic Malformations: Pearls and Pitfalls of Diagnosis and Differential Diagnoses in Pediatric Patients. AJR Am J Roentgenol 2017; 208:637-649. [DOI: 10.2214/ajr.16.17008] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
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20
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Putta T, Irodi A, Thangakunam B, Oliver A, Gunasingam R. Young patient with generalized lymphangiomatosis: Differentiating the differential. Indian J Radiol Imaging 2016; 26:411-415. [PMID: 27857472 PMCID: PMC5036344 DOI: 10.4103/0971-3026.190416] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/07/2022] Open
Abstract
We present the case of a 19-year-old man who was extensively evaluated in multiple centres for long-standing cough, dyspnea, and hemoptysis without a definitive diagnosis. His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. Computed tomography of the thorax showed a confluent, fluid-density mediastinal lesion enveloping the mediastinal viscera without any mass effect. There were bilateral pleural effusions, prominent peribronchovascular interstitial thickening, interlobular septal thickening and lobular areas of ground glass density with relative sparing of apices. There were a few dilated retroperitoneal lymphatics and well-defined lytic lesions in the bones. In this case report, we aim to systematically discuss the relevant differentials and arrive at a diagnosis. We also briefly discuss the treatment options and prognosis along with our patient's course in the hospital and final outcome.
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Affiliation(s)
- Tharani Putta
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | - Aparna Irodi
- Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
| | | | - Ashwin Oliver
- Department of Pulmonary Medicine, Christian Medical College, Vellore, Tamil Nadu, India
| | - Rajesh Gunasingam
- Department of Radiotherapy, Christian Medical College, Vellore, Tamil Nadu, India
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Saadi A, Ayed H, Karray O, Kerkeni W, Bouzouita A, Cherif M, Slama RB, Derouiche A, Chebil M. [Retroperitoneal cystic lymphangioma: about 5 cases and review of the literature]. Pan Afr Med J 2016; 25:73. [PMID: 28292036 PMCID: PMC5324145 DOI: 10.11604/pamj.2016.25.73.10002] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/08/2016] [Accepted: 09/16/2016] [Indexed: 11/27/2022] Open
Abstract
Le lymphangiome kystique est une tumeur bénigne malformative rare des vaisseaux lymphatiques à localisations diverses. La localisation rétropéritonéale est moins fréquente comparée à celle mésentérique. Sa présentation clinique est polymorphe. Le diagnostic est évoqué par l'imagerie mais il nécessite une confirmation histologique. Le traitement de choix est chirurgical. Notre objectif est d'étudier les manifestations cliniques, les complications, les aspects diagnostiques et thérapeutiques de cette tumeur. Nous rapportons une série de 5 cas de lymphangiomes kystiques rétropéritonéaux (4 femmes et un homme) opérés dans notre service entre les années 2004 et 2014. Leurs dossiers ont été examinés rétrospectivement. Le suivi était basé sur l'examen clinique et l'échographie abdominale. L´âge moyen était de 45 ans. Le suivi moyen était de 32,6 mois. La symptomatologie révélatrice la plus fréquente était les douleurs et/ou une masse abdominale. Le scanner abdominal était l'examen le plus utile au diagnostic. Une exérèse complète était réalisée d'emblée chez quatre patients et elle était différée après cinq ans de surveillance par une échographie annuelle chez un. Dans un cas, on a eu recours à une néphrectomie. Aucune récidive ni complication n´ont été notées chez les 5 patients. le lymphangiome kystique à localisation rétropéritonéale est une affection rare. Sa prise en charge thérapeutique repose sur une exérèse complète, de cas de lésions symptomatiques ou de complications, pour limiter le risque de récidive. Cette dernière peut être différée chez les patients asymptomatiques.
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Affiliation(s)
- Ahmed Saadi
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Haroun Ayed
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Omar Karray
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Walid Kerkeni
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Abderrazak Bouzouita
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Mohamed Cherif
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Riadh Ben Slama
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Amine Derouiche
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
| | - Mohamed Chebil
- Service d'Urologie, Hôpital Charles-Nicolle, Faculté de Médecine de Tunis, Université de Tunis El Manar, boulevard 9-Avril, 1006 Tunis, Tunisie
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Rana SS, Chhabra P, Sharma V, Pervez N, Sharma R, Srinivasan R, Bhasin DK. Disseminated lymphangiomatosis presenting as chylous ascites and diagnosed with endoscopic ultrasound. Endosc Ultrasound 2016; 5:210-211. [PMID: 27386481 PMCID: PMC4918307 DOI: 10.4103/2303-9027.183974] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/08/2014] [Accepted: 08/23/2015] [Indexed: 11/09/2022] Open
Affiliation(s)
- Surinder Singh Rana
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Puneet Chhabra
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Vishal Sharma
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Nadeem Pervez
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Ravi Sharma
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Radhika Srinivasan
- Department of Cytology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
| | - Deepak Kumar Bhasin
- Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Leroy A, Garabedian C, Fourquet T, Clouqueur E, Coulon C. [Iconographic imaging (ultrasound/MRI) in prenatal evaluation of cervical cystic lymphatic malformations]. ACTA ACUST UNITED AC 2016; 44:269-73. [PMID: 27118341 DOI: 10.1016/j.gyobfe.2016.03.011] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/16/2015] [Accepted: 03/18/2016] [Indexed: 12/01/2022]
Abstract
OBJECTIVES Assess imaging (ultrasound±MRI) in the diagnosis of cervical cystic lymphangioma. METHODS Retrospective, descriptive study of the patients who underwent reference ultrasound screening for fetal anterior cervival masses in multidisciplinary prenatal diagnosis center of the Lille Regional University Hospital from 1997 to 2014. RESULTS Seventeen lymphangiomas were identified. Seventy-three percent of lymphangiomas (n=12) were diagnosed in the baseline ultrasound and 85% (n=11) in MRI. An extra-cervical extension was identified in 10 (62%) with ultrasound and 11 (85%) with MRI. Main sites of mass extension objectived by ultrasound and MRI were respectively the face (4,23%/6,46%), the base of tongue (3, 18%/2, 15%) and buccal floor (3, 18%/3, 15%). Pharyngeal disease (5,38%) and mandible (4,31%) were detected by MRI and not found on ultrasound. Associated malformations detected with ultrasound were 2 (12%) urogenital malformation, 1 (6%) heart defect and 1 (6%) facial anomalies. Lymphangiomas remained stable in volume in 12 (71%) cases and disappeared before birth in 2 (12%) cases. CONCLUSION MRI and ultrasound seem inseparable to assess and optimally manage fetal cervical masses. Detection of associated lesions seems to help in the final lymphangioma diagnosis. Most of the time, lymphangiomas seem to be stable lesion during pregnancy and a good prognosis in cases of uncomplicated cervical lymphatic mass and without pejorative extension.
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Affiliation(s)
- A Leroy
- Service de gynécologie-obstétrique, maternité Jeanne-de-Flandres, centre hospitalier régional universitaire de Lille, 59037 Lille cedex, France.
| | - C Garabedian
- Service de gynécologie-obstétrique, maternité Jeanne-de-Flandres, centre hospitalier régional universitaire de Lille, 59037 Lille cedex, France; Faculté de médecine Henri-Warembourg, université Lille nord de France, avenue Eugène-Avinée, 59045 Lille, France
| | - T Fourquet
- Service d'imagerie de la femme, maternité Jeanne-de-Flandres, centre hospitalier universitaire de Lille, 59037 Lille cedex, France
| | - E Clouqueur
- Service de gynécologie-obstétrique, maternité Jeanne-de-Flandres, centre hospitalier régional universitaire de Lille, 59037 Lille cedex, France
| | - C Coulon
- Service de gynécologie-obstétrique, maternité Jeanne-de-Flandres, centre hospitalier régional universitaire de Lille, 59037 Lille cedex, France
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24
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Herruela-Suffee C, Warin M, Castier-Amouyel M, Dallery F, Bonnaire B, Constans JM. Whole-body MRI in generalized cystic lymphangiomatosis in the pediatric population: diagnosis, differential diagnoses, and follow-up. Skeletal Radiol 2016; 45:177-85. [PMID: 26545884 DOI: 10.1007/s00256-015-2280-8] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/17/2015] [Revised: 10/18/2015] [Accepted: 10/26/2015] [Indexed: 02/02/2023]
Abstract
OBJECTIVE Generalized cystic lymphangiomatosis is a particularly rare disease with variable involvement of skeletal and extraskeletal sites. The key role of imaging in the diagnosis of this disease is no longer in doubt. The aim of our study was to demonstrate the contribution of whole-body magnetic resonance imaging (WB-MRI) at the diagnostic stage and during the follow-up in the pediatric population. SUBJECTS AND METHODS Three children were included from 2008. The inclusion criteria were radiological images (conventional radiographs, computed tomography, and MRI) compatible with histological confirmation. Each child included received WB-MRI at the diagnosis stage and during follow-up. RESULTS WB-MRI detected multiple hypointense T1 and hyperintense T2-STIR cystic bony lesions. One unnoticed mass in the retroperitoneum on computed tomography was easily detected by MRI. Mediastinal involvement was observed in one asymptomatic case. Histology was contributory in all cases. Preventive intramedullary nailings was done in one case. Follow-up WB-MRI detected new asymptomatic lesions in all cases. One child presented a third episode of lymphangitis of the right thigh during follow-up. CONCLUSIONS Due to its high sensitivity to diagnose, ability to eliminate differential diagnoses and make exhaustive lesions assessment, and its non-radiating character for long-term follow-up, WB-MRI is highly recommended for generalized cystic lymphangiomatosis in the pediatric population.
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Affiliation(s)
| | - Mathilde Warin
- Service de Radiologie, CHU Amiens-Picardie, 80054, Amiens Cedex 1, France
| | | | - Florine Dallery
- Service de Radiologie, CHU Amiens-Picardie, 80054, Amiens Cedex 1, France
| | - Bruno Bonnaire
- Service de Radiologie, CHU Amiens-Picardie, 80054, Amiens Cedex 1, France
| | - Jean-Marc Constans
- Service de Radiologie, CHU Amiens-Picardie, 80054, Amiens Cedex 1, France
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Díaz Marugán VM, Lopez-Gutierrez JC. Primary Intestinal Lymphangiectasia and its Association With Generalized Lymphatic Anomaly. JOURNAL OF PEDIATRICS REVIEW 2016. [DOI: 10.17795/jpr-4790] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
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Lyazidi Y, Bakkali T, Zoulati M, Chtata H, Taberkant M. Drop-Attacks Revealing a Cervical Cystic Lymphangioma. Ann Vasc Surg 2015; 31:208.e5-7. [PMID: 26620381 DOI: 10.1016/j.avsg.2015.09.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2015] [Revised: 09/06/2015] [Accepted: 09/08/2015] [Indexed: 10/22/2022]
Abstract
We report the case of a 28-year-old man who presented in emergency for recurrent drop-attacks. Ultrasound imaging and angio computed tomography revealed a left cervical tumor, and the patient underwent surgery. The diagnosis of cystic lymphangioma was done on the anatomic characteristics of the surgical specimen and the results of the histological study. This is the first reported case of late diagnosis of a cervical cystic lymphangioma revealed by recurrent drops attack.
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Affiliation(s)
- Youssef Lyazidi
- Department of Vascular Surgery, Service de Chirurgie Vasculaire, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco.
| | - Tarek Bakkali
- Department of Vascular Surgery, Service de Chirurgie Vasculaire, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco
| | - Mohamed Zoulati
- Department of Vascular Surgery, Service de Chirurgie Vasculaire, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco
| | - Hassan Chtata
- Department of Vascular Surgery, Service de Chirurgie Vasculaire, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco
| | - Mustapha Taberkant
- Department of Vascular Surgery, Service de Chirurgie Vasculaire, Hôpital Militaire d'Instruction Mohammed V, Rabat, Morocco
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Simple lymphangioma to generalized lymphatic anomaly: role of imaging in disclosure of a rare and morbid disease. Case Rep Radiol 2015; 2015:603859. [PMID: 25954563 PMCID: PMC4410542 DOI: 10.1155/2015/603859] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2015] [Accepted: 03/30/2015] [Indexed: 11/20/2022] Open
Abstract
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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Gutiérrez Schiaffino G, Leiva Gea I, Martín Tejedor B, Jiménez Hinojosa J, Madrid Rodríguez A, Urda Cardona A. ¿Es la actitud expectante en el síndrome de Gorham una opción terapéutica? An Pediatr (Barc) 2014; 81:e64-5. [DOI: 10.1016/j.anpedi.2014.02.022] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2013] [Revised: 02/13/2014] [Accepted: 02/17/2014] [Indexed: 11/27/2022] Open
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Importance of MRI in the diagnosis of vertebral involvement in generalized cystic lymphangiomatosis. Skeletal Radiol 2014; 43:1633-8. [PMID: 24950879 DOI: 10.1007/s00256-014-1935-1] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2014] [Revised: 04/30/2014] [Accepted: 06/01/2014] [Indexed: 02/02/2023]
Abstract
A 9-year-old boy presented with the sudden onset of pleuritic chest pain and on CT was found to have a large pleural effusion, mediastinal fluid, splenic lesions and multiple apparently sclerotic vertebral bodies. Subsequent MRI showed that those vertebral bodies that appeared sclerotic were in fact normal, and the vertebral bodies initially interpreted as normal had an abnormal T1 and T2 hyperintense signal on MRI and were relatively lucent on CT. MRI also demonstrated abnormal heterogeneous T2 hyperintense paraspinal tissue and several multicystic soft tissue masses. Biopsy of two adjacent vertebral bodies, one relatively sclerotic and one lucent, demonstrated findings of bony remodeling without a specific diagnosis. Biopsy of an infiltrative mediastinal mass confirmed the diagnosis of generalized cystic lymphangiomatosis. MRI should be included in the assessment of vertebral involvement in this condition because CT and biopsy findings may be nonspecific.
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Nosher JL, Murillo PG, Liszewski M, Gendel V, Gribbin CE. Vascular anomalies: A pictorial review of nomenclature, diagnosis and treatment. World J Radiol 2014; 6:677-692. [PMID: 25276311 PMCID: PMC4176785 DOI: 10.4329/wjr.v6.i9.677] [Citation(s) in RCA: 50] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/25/2014] [Accepted: 07/29/2014] [Indexed: 02/06/2023] Open
Abstract
Vascular anomalies, including vascular malformations and tumors, are frequently straightforward to detect; however, accurate diagnosis and appropriate treatment are often challenging. Misdiagnosis of these lesions can lead clinicians in the wrong direction when treating these patients, which can have unfavorable results. This review presents an overview of the classification systems that have been developed for the diagnosis of vascular lesions with a focus on the imaging characteristics. Pictorial examples of each lesion on physical examination, as well as non-invasive and minimally invasive imaging are presented. An overview of the endovascular treatment of these lesions is also given. In some cases, vascular anomalies may be associated with an underlying syndrome and several of the most commonly encountered syndromes are discussed. Understanding of the classification systems, familiarity with the treatment options and knowledge of the associated syndromes are essential for all physicians working with this patient population. The approach to the described entities necessitates an organized multi-disciplinary team effort, with diagnostic imaging playing an increasingly important role in the proper diagnosis and a combined interventional radiologic and surgical treatment method showing promising results.
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31
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Telischak NA, Wu JS, Eisenberg RL. Cysts and cystic-appearing lesions of the knee: A pictorial essay. Indian J Radiol Imaging 2014; 24:182-91. [PMID: 25024531 PMCID: PMC4094974 DOI: 10.4103/0971-3026.134413] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022] Open
Abstract
Cysts and cystic-appearing lesions around the knee are common and can be divided into true cysts (synovial cysts, bursae, ganglia, and meniscal cysts) and lesions that mimic cysts (hematomas, seromas, abscesses, vascular lesions, and neoplasms). The specific anatomic location of the cystic lesion often permits the correct diagnosis. In difficult cases, identifying a cystic mass in an atypical location and/or visualizing internal solid contrast enhancement on magnetic resonance imaging (MRI) should raise concern for a neoplasm and the need for further evaluation and intervention.
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Affiliation(s)
- Nicholas A Telischak
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston MA 02215, USA
| | - Jim S Wu
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston MA 02215, USA
| | - Ronald L Eisenberg
- Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, 330 Brookline Avenue, Boston MA 02215, USA
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DePalma AAR, Shenoy K, Sharan AD. Cystic Angiomatosis: A Rare Cause of Thoracic Radiculopathy: A Case Report. JBJS Case Connect 2014; 4:e55. [PMID: 29252524 DOI: 10.2106/jbjs.cc.m.00256] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/07/2023]
Affiliation(s)
| | - Kartik Shenoy
- Albert Einstein College of Medicine, 1925 Eastchester Road, Apt. 17E, Bronx, NY 10461.
| | - Alok D Sharan
- Department of Orthopaedic Surgery, Albert Einstein College of Medicine, 1250 Waters Place, 11th Floor, Bronx, NY 10461. Email address:
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Park SH, Moon SK, Sung JY. Sonographic findings in a case of scrotal lymphangioma. JOURNAL OF CLINICAL ULTRASOUND : JCU 2014; 42:234-236. [PMID: 24691940 DOI: 10.1002/jcu.22095] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/08/2013] [Revised: 06/06/2013] [Accepted: 08/08/2013] [Indexed: 06/03/2023]
Abstract
The scrotum is an extremely rare site for lymphangioma. We report the case of a scrotal lymphangioma in a 20-year-old male patient who developed painless scrotal swelling. Typical sonography and MRI findings are shown. Surgical excision and histopathology confirmed the diagnosis.
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Affiliation(s)
- So Hyun Park
- Department of Radiology, Kyung Hee University Hospital, Seoul, South Korea; School of Medicine, Kyung Hee University, Seoul, South Korea
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34
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Brennan C, Kajal D, Khalili K, Ghai S. Solid malignant retroperitoneal masses-a pictorial review. Insights Imaging 2013; 5:53-65. [PMID: 24293303 PMCID: PMC3948907 DOI: 10.1007/s13244-013-0294-0] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/29/2013] [Revised: 08/31/2013] [Accepted: 10/11/2013] [Indexed: 12/25/2022] Open
Abstract
Primary retroperitoneal masses are a rare but important group of neoplasms. Cross-sectional imaging has revolutionised the investigation of patients with retroperitoneal neoplasms. Both computed tomography (CT) and magnetic resonance imaging (MRI) can contribute to tumour diagnosis, though histological confirmation is often required because of the considerable overlap of imaging features. Cross-sectional imaging is key to the pre-operative staging and planning of retroperitoneal masses, though ultrasound may also help in certain instances. Imaging also helps to select and guide the site to biopsy from these usually large and heterogeneous neoplasms. This article aims to review many of the primary retroperitoneal neoplasms that may be encountered by the radiologist.
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Affiliation(s)
- Cressida Brennan
- Division of Abdominal Imaging, Joint Department of Medical Imaging, University Health Network - Mount Sinai Hospital - Women's College Hospital, University of Toronto, Toronto, ON, Canada
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35
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Lala S, Mulliken JB, Alomari AI, Fishman SJ, Kozakewich HP, Chaudry G. Gorham-Stout disease and generalized lymphatic anomaly--clinical, radiologic, and histologic differentiation. Skeletal Radiol 2013; 42:917-24. [PMID: 23371338 DOI: 10.1007/s00256-012-1565-4] [Citation(s) in RCA: 131] [Impact Index Per Article: 10.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/01/2012] [Revised: 12/09/2012] [Accepted: 12/11/2012] [Indexed: 02/02/2023]
Abstract
PURPOSE Gorham-Stout disease (GSD) is a rare vascular disorder of lymphatic origin characterized by progressive osteolysis. Generalized lymphatic anomaly (GLA) is a multisystem disorder that also commonly affects bone. We hypothesized that Gorham-Stout disease is different from other osseous lymphatic anomalies. We proposed to discriminate these entities by analyzing findings on skeletal imaging. METHODS Clinical data, imaging studies, and histopathologic findings were retrospectively reviewed in patients presenting to our Vascular Anomalies Center with lymphatic anomalies of bone. FINDINGS Within a cohort of 51 patients with lymphatic disorder and radiological evidence of bony involvement, two distinct categories emerged. Nineteen patients met the imaging criteria for GSD: progressive osteolysis with resorption and cortical loss. Thirty-two were categorized as GLA: Discrete radiolucencies and increasing numbers of bone affected over time, but without evidence of progressive osteolysis. The ribs were the most common site in both groups, followed by the cranium, clavicle, and cervical spine in GSD, and thoracic spine, humerus, and femur in GLA. Fewer bones were involved in GSD, with relative sparing of the appendicular skeleton. Associated infiltrative soft tissue abnormality was seen in 18 in GSD, but only six with GLA. Macrocystic lymphatic malformations were identified in 14 with GLA, but none with GSD. CONCLUSIONS There are significant radiological differences between GSD and GLA, although there are some overlapping features. The major distinguishing characteristic is the progressive osteolysis seen in GSD. Findings suggestive of GLA are more extensive involvement, particularly of the appendicular skeleton, presence of discretemacrocystic lymphatic malformations and visceral organ lesions.
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Affiliation(s)
- Shailee Lala
- Vascular Anomalies Center and Division of Vascular and Interventional Radiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA
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36
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Baskın D, Narcı A, Okur N, Uğraş M, Nadirgil Köken G, Ali Tuncer A, Tokyol Ç, Çetinkurşun S. Cystic lymphangiomatosis with severe intra-abdominal bleeding in a newborn: case report. JOURNAL OF CLINICAL ULTRASOUND : JCU 2013; 41:261-264. [PMID: 22729896 DOI: 10.1002/jcu.21956] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 08/26/2011] [Accepted: 05/07/2012] [Indexed: 06/01/2023]
Abstract
We report the case of a newborn girl with intestinal cystic lymphangiomatosis who presented with abdominal distension and intra-abdominal bleeding following a prenatal ultrasound diagnosis of intestinal anomaly. Postnatal abdominal ultrasound revealed disseminated submucosal and intramural cystic dilatations of various sizes in the bowel and intestinal lymphangiomatosis was diagnosed. The presence of severe bleeding diathesis and widespread disease led to conservative treatment. The patient died on postnatal day 7 and postmortem examination confirmed cystic lymphangiomatosis. Detection of intestinal hyperechogenicity and/or dilatation in prenatal ultrasonography and the persistence of these findings during pregnancy are suggestive for pathologies such as meconium ileus, meconium peritonitis, and intestinal atresia. Although rare, intestinal lymphangiomatosis should be kept in mind in patients whose prenatal sonographic findings persist until birth.
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Affiliation(s)
- Didem Baskın
- Department of Pediatric Surgery, Afyon Kocatepe University Medical Faculty, Afyonkarahisar, Turkey
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37
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Kwag E, Shim SS, Kim Y, Chang JH, Kim KC. CT features of generalized lymphangiomatosis in adult patients. Clin Imaging 2013; 37:723-7. [PMID: 23391872 DOI: 10.1016/j.clinimag.2012.12.003] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/18/2012] [Revised: 09/05/2012] [Accepted: 12/17/2012] [Indexed: 12/28/2022]
Abstract
OBJECTIVE The objective was to present computed tomographic (CT) findings of generalized lymphangiomatosis in young adults that was at first misdiagnosed with malignancy. MATERIALS AND METHODS We reviewed the clinical and radiological findings of three young adults who had histologically confirmed generalized lymphangiomatosis. RESULTS Bony lesions were cystic forms and appeared as variably sized, well-circumscribed, irregular, microlobulated-contour, multiseptated masses. Lymphangiomas involving soft tissue were similar to bony lesions on CT. CONCLUSION CT features of generalized lymphangiomatosis may be useful to prevent invasive procedures in young subjects. Generalized lymphangiomatosis involving bones in young adults shows less extensive and aggressive osteolytic pattern than known features in child.
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Affiliation(s)
- Eujean Kwag
- Department of Radiology, Mokdong Hospital, Ewha Womans University School of Medicine, Seoul 158-710, Korea
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38
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Abstract
Lymphangiomatosis is a rare disease characterized by diffuse infiltration of lymphangiomas in the lung, bone, and other tissues. Due to its rarity, the spectrum of lymphangiomatosis is beginning to be elucidated based on case reports. The limited pathological, radiological, and clinical studies have shed light on this disease. Treatments have been tested in unblinded manner with promising results; however, further understanding of the pathogenesis of disease, as well as its natural history, is needed to facilitate drug development.
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Affiliation(s)
- Melissa N Satria
- Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-1590, USA
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39
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Rajiah P, Sinha R, Cuevas C, Dubinsky TJ, Bush WH, Kolokythas O. Imaging of uncommon retroperitoneal masses. Radiographics 2012; 31:949-76. [PMID: 21768233 DOI: 10.1148/rg.314095132] [Citation(s) in RCA: 171] [Impact Index Per Article: 13.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Retroperitoneal masses not arising from major solid organs are uncommon. Although there is no simple method of classifying retroperitoneal masses, a reasonable approach is to consider the masses as predominantly solid or cystic and to subdivide these into neoplastic and nonneoplastic masses. Because the treatment options vary, it is useful to be able to differentiate these masses by using imaging criteria. Although the differential diagnosis of retroperitoneal masses can be narrowed down to a certain extent on the basis of imaging characteristics, patterns of involvement, and demographics, there is still a considerable overlap of imaging findings for these masses, and histologic examination is often required for definitive diagnosis. Computed tomography (CT) and magnetic resonance (MR) imaging play an important role in characterization and in the assessment of the extent of the disease and involvement of adjacent and distant structures. Familiarity with the CT and MR imaging features of various retroperitoneal masses will facilitate accurate diagnosis and staging for aggressive lesions.
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Affiliation(s)
- Prabhakar Rajiah
- Imaging Institute, Cleveland Clinic Foundation, Cleveland, OH 44195, USA.
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40
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Generalised lymphangiomatosis in an 8-year-old girl who presented with cardiomegaly. Cardiol Young 2011; 21:465-7. [PMID: 21362206 DOI: 10.1017/s1047951111000205] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Generalised lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. The computed tomography, ultrasonography and magnetic resonance images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung, and lower neck. We report here a case that was referred to us for investigation of marked cardiomegaly.
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41
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Shah V, Shah S, Barnacle A, Sebire NJ, Brock P, Harper JI, McHugh K. Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. Pediatr Radiol 2011; 41:985-92. [PMID: 21660638 DOI: 10.1007/s00247-011-2124-1] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/16/2010] [Revised: 01/23/2011] [Accepted: 01/29/2011] [Indexed: 11/28/2022]
Abstract
BACKGROUND Multifocal lymphangiomatosis is a rare systemic disorder affecting children. Due to its rarity and wide spectrum of clinical, histological and imaging features, establishing the diagnosis of multifocal lymphangiomatosis can be challenging. OBJECTIVES The purpose of this study was to describe a new imaging sign in this disorder: paraspinal soft tissue and signal abnormality at MRI. MATERIALS AND METHODS We retrospectively reviewed the imaging, clinical and histopathological findings in a cohort of eight children with thoracic involvement from this condition. RESULTS Evidence of paraspinal chest disease was identified at MRI and CT in all eight of these children. The changes comprise heterogeneous intermediate-to-high signal parallel to the thoracic vertebrae on T2-weighted sequences at MRI, with abnormal paraspinal soft tissue at CT and plain radiography. CONCLUSION Multifocal lymphangiomatosis is a rare disorder with a broad range of clinicopathological and imaging features. MRI allows complete evaluation of disease extent without the use of ionising radiation and has allowed us to describe a previously unreported imaging sign in this disorder, namely, heterogeneous hyperintense signal in abnormal paraspinal tissue on T2-weighted images.
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Affiliation(s)
- Vikas Shah
- Department of Radiology, Great Ormond Street Hospital for Children, London, WC1N 3JH, UK.
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42
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El-Essawy MT, Hassan HH. Cystic hygroma of the neck with multiple bone lymphangiomas. THE EGYPTIAN JOURNAL OF RADIOLOGY AND NUCLEAR MEDICINE 2011. [DOI: 10.1016/j.ejrnm.2011.05.009] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022] Open
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43
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Rasalkar DD, Chu WCW. Generalized cystic lymphangiomatosis. Pediatr Radiol 2010; 40 Suppl 1:S47. [PMID: 20574655 DOI: 10.1007/s00247-010-1694-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/29/2009] [Revised: 03/29/2010] [Accepted: 04/03/2010] [Indexed: 02/06/2023]
Affiliation(s)
- Darshana D Rasalkar
- Department of Diagnostic Radiology, The Chinese University of Hong Kong, Prince of Wales Hospital, Ngan Shing St., Shatin, New Territories, Hong Kong
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Graves consecuencias del tratamiento mediante esclerosis con ácido acético en un caso de linfangioma quístico retroperitoneal. Actas Urol Esp 2010. [DOI: 10.1016/j.acuro.2010.01.029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
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Lymphangiomatose kystique osseuse diffuse et localisation splénique : forme mineure d’une atteinte systémique. ACTA ACUST UNITED AC 2010; 91:907-10. [DOI: 10.1016/s0221-0363(10)70134-7] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
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Affiliation(s)
- L Donato
- Pôle Médico-Chirurgical de Pédiatrie, Hôpitaux Universitaires de Strasbourg, France.
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Lohrmann C, Foeldi E, Langer M. Diffuse lymphangiomatosis with genital involvement--evaluation with magnetic resonance lymphangiography. Urol Oncol 2009; 29:515-22. [PMID: 19914101 DOI: 10.1016/j.urolonc.2009.09.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/09/2009] [Revised: 09/06/2009] [Accepted: 09/08/2009] [Indexed: 10/20/2022]
Abstract
OBJECTIVE To assess, for the first time, the morphology of the lymphatic system in patients with diffuse lymphangiomatosis and genital involvement by magnetic resonance lymphangiography (MRL). MATERIALS AND METHODS Ten patients with diffuse lymphangiomatosis and genital involvement were examined by MRL. Three locations were examined: first, the lower leg and foot region; second, the upper leg and the knee region; and third, the pelvic with retroperitoneal region. MR imaging was performed with a 1.5-T system equipped with high-performance gradients. For MRL, a T1-weighted 3D-spoiled gradient-echo and a T2-weighted 3D-TSE sequence were used. RESULTS The size of the genital lymphangiomas, which were revealed in all patients, varied between 6 and 85 mm. In 60% of the patients, lymphangiomas were additionally detected at the level of the lower legs, and in 70% patients at the level of the upper leg. Furthermore, lymphangiomas were seen in the inguinal and retroperitoneal regions in 80%, and in the pelvic region and anterior abdominal wall in 90% of the patients examined. The genital lymphangiomas feeding lymphatic vessels were detected in 80% of the patients in the anterior abdominal wall and in 90% of the patients in the inguinal and pelvic regions; 90% of the patients suffered consecutively from a lymphedema of the lower extremities. All patients suffered from recurrent infections in the genital region; 80% of the patients repeatedly experienced genital lymphorrhea due to lympho-cutaneous fistulas and lymphcysts. CONCLUSION MRL is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic circulation in patients with diffuse lymphangiomatosis and genital involvement. Because the site and extent of the lymphangiomas with their feeding lymphatic vessels are important prognostic factors, performing radiologic evaluation with a high resolution is crucial for the therapeutic planning of patients.
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Affiliation(s)
- Christian Lohrmann
- Department of Radiology, University Hospital of Freiburg, Freiburg, Germany.
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48
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Lohrmann C, Foeldi E, Langer M. Assessment of the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging. Eur J Radiol 2009; 80:576-81. [PMID: 19913379 DOI: 10.1016/j.ejrad.2009.10.021] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2009] [Accepted: 10/14/2009] [Indexed: 01/18/2023]
Abstract
OBJECTIVE To assess the lymphatic system in patients with diffuse lymphangiomatosis by magnetic resonance imaging. MATERIALS AND METHODS 15 patients with diffuse lymphangiomatosis were examined by magnetic resonance imaging. Three locations were examined: first, the lower leg and foot region; second, the upper leg and the knee region; and third, the pelvic with retroperitoneal and abdominal region. For magnetic resonance lymphangiography a T1-weighted 3D spoiled gradient-echo and a T2-weighted 3D-TSE sequence was used. RESULTS The size of the genital lymphangiomas, which were revealed in all patients, varied between 5 and 83 mm. In 47% of the patients lymphangiomas were detected at the level of the lower legs, and in 87% of the patients at the level of the upper leg and retroperitoneum. Furthermore, lymphangiomas were seen in the inguinal and pelvic region in 100% and intraabdominally in 40% of the patients. The lymphangiomas extended into the abdominal wall in 93% of the examined patients. A chylous pleural effusion was revealed in 20% and a chylous ascites in 13% of patients. 93% of patients suffered due to the diffuse lymphangiomatous pathologies from a lymphedema of the lower extremities, while a generalized lymphedema of the trunk was found in 87% of the patients. CONCLUSION Magnetic resonance imaging is a safe and accurate minimal-invasive imaging modality for the evaluation of the lymphatic system in patients with diffuse lymphangiomatosis. Since the localization and extension of the lymphangiomas are important prognostic factors, it is crucial to perform a safe radiologic evaluation with a high resolution for the patient's therapeutic planning.
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Affiliation(s)
- Christian Lohrmann
- Department of Radiology, University Hospital of Freiburg, Hugstetter Strasse 55, D-79106 Freiburg i. Br., Germany.
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49
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Whole-body MRI in the pediatric patient. Eur J Radiol 2009; 70:442-51. [DOI: 10.1016/j.ejrad.2009.02.012] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2009] [Accepted: 02/24/2009] [Indexed: 11/21/2022]
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50
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Darge K, Jaramillo D, Siegel MJ. Whole-body MRI in children: current status and future applications. Eur J Radiol 2008; 68:289-98. [PMID: 18799279 DOI: 10.1016/j.ejrad.2008.05.018] [Citation(s) in RCA: 82] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/08/2008] [Accepted: 05/20/2008] [Indexed: 10/21/2022]
Abstract
Whole-body MRI (WBMRI) is a novel technique that makes imaging of the whole patient in a manner similar to scintigraphy or positron emission tomography (PET) possible. Unlike the latter two methods, it is without exposure to radiation and thus gaining increasing importance and application in pediatrics. With the introduction of a moving tabletop, sequential movement of the patient through the magnet has become possible with automatic direct realignment of the images after acquisition. The common scan plane is coronal with additional planes being added depending on the indication. WBMRI is targeted for maximum coverage of the body within the shortest possible time using the minimum number of sequences. The evaluation of the bone marrow has been the primary indication thus inversion recovery sequences like STIR or TIRM are mostly used with the T1-weighted sequence being added variably. For correct evaluation of the bone marrow in the pediatric age group understanding normal pattern of marrow transformation is essential. The primary role of WBMRI has been in oncology for the detection of tumor spread and also for the follow-up and evaluation of complications. The initial comparative studies of WBMRI with scintigraphy and PET in children have shown the high diagnostic potential of WBMRI. Emerging potential applications of WBMRI include the evaluation for osteonecrosis, chronic multifocal recurrent osteomyelitis, myopathies, and generalized vascular malformations. Future use of WBMRI may incorporate non-accidental trauma, virtual autopsy, body fat mapping and diffusion-weighted imaging.
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Affiliation(s)
- Kassa Darge
- Department of Radiology, Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd., Philadelphia, PA 19104, USA.
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