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What is:

A syndrome of Lymphatic Dysplasia

Lymphangiomatosis (limf-an-jee-oh-mah-TOE-sis). In medical terminology lymphangiomatosis means: lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis). There is no standard definition describing this disease that has been agreed to by the medical/scientific community. (1)

Tumors (lymphangiomas) grow in the lymph system itself.  These are benign in nature but they spread throughout the body invading other body tissuesThey can spread to any area, but are more commonly found in the lungs (pulmonary), bones (skeletal), kidney (renal), the thoracic cavity, connective tissue and spleen.

While the tumors are not malignant, they can quickly interfere with organ function causing extreme pain, difficulty in breathing, destroy other tissue, thereby causing other serious potential complications.

IIf needed or necessary a lymphoscintigraphy exam can be used to determine lymph flow and/or blockage.

Signs and Symptoms:


Treatment is usually multi-focal and depends on where the location of the tumor is and the complications they may present.

There are three basic methods of treating lymphangiomatosis.

First is the use of a medication that stops tumor growth.  The medication is Interferon Alfa-2b. This drug  (Intron® A) is a man-made protein. Natural interferons are produced to help the immune system fight viral infections and certain cancer growths. Interferon alfa-2b has similar actions to natural interferons and is used to treat AIDS-related Kaposi's sarcoma, certain types of hepatitis, and leukemia or other cancers. Generic interferon alfa-2b injections are not yet available.

Chemotherapy may be used and radiation targeting specific tumor as well.

Other targeted treatment modalities are:

Cardiothoracic Pleurodesis, ligation of thoracic duct, pleurperitoneal shunt, radiation therapy, pleurectomy, surgical resection, thalidomide, Interferon Alpha2b, TPN nutrition, thoracocentesis, medium chain triglyceride and high protein diet, chemotherapy, sclerotherapy, transplantion
Gastrointestinal Interferon Alpha2b, sclerotherapy, resection, percutaneous drainage
Kidneys Percutaneous drainage
Liver Resection, transplantation
Skeleton Interferon Alpha2b, bisphosphonates (i.e. pamidronate), surgical resection, radiation therapy, sclerotherapy, percutaneous bone cement, bone grafts, prosthesis, surgical stabilization
Spleen Interferon Alpha2b, splenectomy (1)

The thoaracic duct is the central location in the body where the lymph system literally dumps excess fluid back into the cardiovascular system, to befinally removed throughthe kidneys.  Should this become blocked, for example by a tumor, the patient may experience lung fluid somewhat like a malignant effusion.  This means the fluid is extreme and rapidly reoccurs.  If a thoracentesis is not sufficient, a procedure called a pleurodesis.  In pleurodesis, an irritant (such as Bleomycin, Tetracyclines, or talc powder) is instilled inside the space between the pleura (the two layers of tissue lining the lungs) in order to create inflammation which tacks the two pleura together. This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid.

The lymphedema treatment program would include: Manual lymphatic drainage; compression wraps or compression bandages (using short stretch bandages), compression garments, compression sleeves.


A very special page dedication

In memory of and with respect to:  Marcus Petersen

September 10, 1995 - September 7, 2004

He fought a courageous battle for years with lymphangiomatosis and Gorham's Disease.  Please visit the family website at:

Lymphangiomatosis and Gorham's Vanishing Bone Disease

You will be remembered by many, Marcus.



Definition: (1)

Multiple intraosseous lymphangiomas occurring as a diffuse form as part of a spectrum of either cystic haemangiomatosis or massive osteolysis (Gorhams disease). For a general description, see lymphangiomatosis.

In this disorder, the lymphangiomas may involve two or more widely separated bones, several bones in the same region of the body or many bones in a diffuse pattern; in some cases lymphangiomas also occur in the soft tissues or viscera. Most often the long tubular bones and flat or irregular bones are affected. Lymphangiography is valuable in making a specific diagnosis through uptake of contrast agent in intraosseous tumours


Skeletal Lymphangiomatosis

This rare congenital disorder can affect the bones or the visceral organs. Skeletal involvement leads to pathological fracture, joint deformity and may cause pain. The bones are filled with a network of interconnected lymph channels containing fluid.

The pattern of skeletal involvement varies, with multiple contiguous bones heavily involved and others completely normal. Other patients present with multiple cystic lesions in non-contiguous bones combined with lymph fluid collections in the chest and abdominal cavities (chylothorax and chyloperitoneum). Generalized lymphedema of the extremity may occur.

There have been isolated cases of association with other mesenchymal dysplasias, such as Mafucci syndrome and osteochondromas. Gorham's disease (idiopathic massive osteolysis, dissapearing bone disease) may be an aggressive form of lymphangiomatosis. A familial cluster of cases with an autosomal dominant pattern of inheritance has been reported.

Individuals without visceral involvement have a better prognosis. The skeletal lesions may involute or inprove after skeletal maturity. The author is not aware of any report of sarcomatous degeneration in these lesions.

Treatment is centered on minimizing skeletal complications and the associated deformity. Bisphosphonate medications may limit the bone loss and the risk of pathologic fracture associated with expansion of the lesions. The optimum dosage and pharmacokinetics of the oral bisphosphonate medicines available for these conditions have not yet been fully established in children, but there use is under active investigation.

Surgical intervention is necessary for certain pathological fractures and to correct symptomatic deformity. Fractures treated with non-surgical techniques can be expected to heal. Other minimally invasive interventions that have been used to treat related lesions, such as percutaneous osteoplasty as used for hemangioma of bone, may hold some promise. One report of the use of radiation to control these lesions in an inoperable location has been published.

Since the condition is very rare, each case must be evaluated and treated as a unique entity.

In the case illustrated here, severe involvement of the pelvis and the resulting pathological collapse of the articular surface of the femoral head lead to disabling pain. This was treated by Girdlestone-type resection arthroplasty of the hip.

At surgery, the lymph fluid seeps and runs from the cut bone surface, with the fluid welling up and drawing back into the bone in response to slight changes of the central venous pressure.


Mirra, Bone Tumors Lea and Febinbger, pp1426

11/2002 HD


Progressive Lymphangiomatosis and Gorham's Disease: Case Report and Clinical Implications. 


Gordon KD, Mortimer PS.


1 Department of Dermatology, St George's Hospital , London, United Kingdom .

Address correspondence to:
Dr. Kristiana Gordon
Department of Dermatology
St George's Hospital
Blackshaw Road
London SW17 0QT
United Kingdom


Abstract Lymphangiomatosis is a rare proliferative disorder of the lymphatic system. The etiology is unknown, rendering it difficult to manage. This case report of lymphangiomatosis with features of Gorham's disease reveals the progressive and unexpected nature of the condition. It highlights the need for further research into the pathophysiology and management oflymphangiomatosis as current treatment options are limited.


Animal models for the mechanistic study of systemic lymphangiomatosis


Rockson SG.


Stanford University School of Medicine , Stanford, California.


Abstract The systematic study of focused animal models has produced an explosion of information regarding the mechanisms governing lymphatic development and the diseases associated with lymphatic dysfunction. Nevertheless, the pathogenesis of systemic lymphangiomatosis has, thus far, eluded mechanistic comprehension. In this review, recent molecular advances in lymphatic vascular development are considered within the context of the animal models that have produced evolving insights. The emerging role of the zebrafish within lymphatic investigation is discussed. Specific models of the human disease pathology are considered in detail. While much has been learned about the molecular framework that surrounds normal lymphatic vascular development, the defect responsible for systemic lymphangiomatosis remains elusive. Development of more robust, recapitulative models will also be invaluable to investigate new and emerging therapeutics for the often devastating disease of systemic lymphangiomatosis.


Pulmonary lymphangiomatosis.


Satria MN, Pacheco-Rodriguez G, Moss J.


Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health , Bethesda, Maryland.


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.


Lymphangiomatosis: clinical overview.



Vascular Birthmark Institute of New York/ St. Luke's Roosevelt Hospital , New York, New York.


Abstract "Lymphangiomatosis" is a general term for excessive growth of aberrant lymphatic vessels. The impact oflymphangiomatosis can be devastating due to osteolysis and/or multi-organ involvement. The disorders are heterogeneous, and treatment is dependent upon disease location and symptoms. Most reports are single cases or small case series, predominantly in the orthopedic and radiologic literature. Basic research focused on lymphatic disorders may translate into new therapies for these disorders.


The successful management of diffuse lymphangiomatosis using sirolimus: a case report. 

Sept 2011

Reinglas J, Ramphal R, Bromwich M.


Faculty of Medicine, University of Ottawa, Ottawa, Canada.


Lymphangiomatosis is a rare and fatal congenital lymphatic malformation. Because the natural course of the disease affects multiple body systems, the management can be challenging. This article presents a novel approach to the treatment of diffuse lymphangiomatosis using sirolimus. The reported case involves a 4-month-old male with a known lymphatic malformation who presented to the emergency department with respiratory difficulties. Sirolimus was successful at significantly reducing our patient's mass at a relatively low target level of 5 to 10 μg/L. The use of sirolimus for the treatment of lymphangiomatosis should be studied further in the setting of a formal trial.


Diffuse lymphangiomatosis of bone

Australasian Radiology
Volume 43 Issue 4 Page 535  - November 1999
Case Report
Diffuse lymphangiomatosis of bone
Daniel D Maki, Mark E Nesbit & Harry J Griffiths1Department of Radiology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania,, 2Department of Pediatric Oncology, University of Minnesota Medical School, Minneapolis, Minnesota and, 3Department of Radiology, University of Missouri Health Sciences Center, Columbia, Missouri, USA

Two cases of lymphangiomatosis of bone, a very rare systemic condition characterized by both skeletal and parenchymal lesions, are presented. The skeletal changes have an appearance similar to haemangiomas in the spine, and soap-bubbly lesions in the flat bones. One case carried the diagnosis of eosinophilic granuloma for 18 years. The findings on MRI, which have not been previously well-established, are discussed.

Key Words: Renal Lymphangiomatosis,  eosinophilic granuloma
 parenchymal lesions


Renal lymphangiomatosis: Sonographic findings

J Clin Ultrasound. 2008 May

Bagheri MH, Zare Z, Sefidbakht S, Nabavizadeh SA, Meshksar A, Roozbeh J, Salehipour M.

Department of Radiology, Shiraz University of Medical Sciences, Zand Street, Shiraz, Iran.

Renal lymphangiomatosis is an exceedingly rare disorder characterized by developmental malformation of the lymphatic system surrounding the kidneys. We report a case of bilateral renal lymphangiomatosis in a 21-year-old man who underwent abdominal sonographic examination that revealed numerous cystic areas of varyious sizes around both kidneys with extension along the renal hilum. Subsequent abdominal CT examination demonstrated bilateral, multilocular, fluid-filled cystic masses with thin walls in the perirenal and peripelvic region. MRI of the patient revealed bilaterally enlarged kidneys with multiple hyperintense lesions in both perirenal spaces and the peripelvic area on T2-weighted images. These cystic spaces appeared hypointense on T1-weighted images with no enhancement in postcontrast images. The diagnosis of renal lymphangiomatosis was made based

Wiley InterScience

Bilateral renal lymphangiomatosis: conservative management

Sept 2007

Tornero Ruiz JI, Ojados Castejón F, Nicolás Torralba J, Escudero Bregante F, Pérez Albacete M.

Servicio de Urología, Hospital Universitario Virgen de la Arrixaca, Murcia, Españ

iOBJECTIVE: Lymphangiomatosis is a benign disease of difficult diagnosis. The bilateral form is very rare, being in some cases an incidental finding. 

METHODS: We report the case of a female patient with the incidental radiological diagnosis of bilateral lymphangiomatosis, who did not present symptoms in relation to the disease. 

RESULTS: After a meticulous study with ultrasound, abdominal CT scan and the MRI the diagnosis was asymptomatic bilateral lymphangiomatosis and expectant management was decided with good outcome. 

CONCLUSIONS: Most cases reported have been treated by nephrectomy for diagnosis, but in our case it was possible to do precise diagnosis adding the experience of the radiologist and the urologist and she had a good outcome with conservative management.



Treatment of thoracic lymphangiomatosis

A Y Rostom

Department of Radiotherapy, The Royal Marsden Hospital, Downs Road, Sutton, Surrey SM2 5PT, UK;83/2/138


Cervical and thoracic components of multiorgan lymphangiomatosis managed surgically

Ali Shahriari, MDa, John A. Odell, FRCS (Ed)b

a Section of Cardiothoracic Surgery, Mayo Medical Center, Jacksonville, Jacksonville, Florida, USA
b Department of General Surgery, Mayo Medical Center, Jacksonville, Jacksonville, Florida, USA

Accepted for publication May 1, 2000.


Splenic lymphangiomatosis in children.

Wadsworth DT, Newman B, Abramson SJ, Carpenter BL, Lorenzo RL.

Mallinckrodt Institute of Radiology, St Louis, Mo, USA.

PURPOSE: To study the clinical and imaging features of splenic lymphangiomatosis. MATERIALS AND METHODS: The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients. RESULTS: Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. CONCLUSION: Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.

PMID: 8988208 [PubMed - indexed for MEDLINE]


Diffuse pulmonary lymphangiomatosis


There is a diffuse proliferation of lymphatics and smooth muscle. By definition, in diffuse pulmonary lymphangiomatosis both lungs are involved and there is no extrathoracic abnormality8., although many cases are systemic


Pulmonary lymphangiectasis1,2,3, generalized lymphangiectasis4, (intra)thoracic lymphangiomatosis5,6, and diffuse pulmonary angiomatosis7. The confusing nomenclature of pulmonary lymphatic disorders has been reviewed8.


Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection8.

Clinical features

Patients present with "asthma", dyspnea or haemoptysis8,12. Pleural effusions are common8,12.


There are increased interstitial markings. CT shows smooth thickening of the interlobular septa, major fissures, central airways and pleura, perihilar and mediastinal fat11. There are often patchy pulmonary ground glass changes11.

Macroscopic appearances

The lymphatic proliferation renders the bronchovascular bundles, pleura and septa prominent.


Endothelium-lined lymphatic spaces permeate pleura, interlobular septa and bronchovascular sheaths12. There may be bland spindle cells between the vascular channels. There is often eosinophilic acellular material. The lung parenchyma may contain iron-laden macrophages12 but the lung architecture is preserved8.



cells lining lymphatics

spindle cells





Ulex europaeus































Differential diagnosis


Percutaneous sclerotherapy with doxycycline has been employed with good results9.


The condition is commonly fatal and tends to be more aggressive in younger children12.

*Link no longer available


External Links:


Unusual clinical manifestation of lymphangiomatosis

Jul-Aug 2011


Diffuse pulmonary lymphangiomatosis with mediastinal affectation. 

Sept 2011


Mediastinal involvement in lymphangiomatosis: a previously unreported MRI sign. 

Aug 2011


Renal lymphangiomatosis, interrupted IVC with persistent primitive hepatic venous plexus and multiple anomalous venous channels: parts of an overlap syndrome? 

July 2011



Propranolol for intractable diffuse lymphangiomatosis.  

April 2011


Generalised lymphangiomatosis in an 8-year-old girl who presented with cardiomegaly. 

Aug 2011


Thoracic lymphangiomatosis with massive chylothorax after a tumor biopsy and with disseminated intravenous coagulation--lymphoscintigraphy, an alternative minimally invasive imaging technique: report of a case.  

July 2011


Lymphangiomatosis (1)

Skeletal Lymphanagiomatosis with Chylous Pleural Effusion

CT and MR imaging of generalized cystic lymphangiomatosis in pediatric patients
P Wunderbaldinger, K Paya, and B Partik
AJR 2000; 174: 827. [Abstract/Full Text]  
Diffuse lymphangiomatosis of bone
DD Maki, ME Nesbit, and HJ Griffiths
Australas Radiol 1999; 43: 535. Ingentaconnect
Bilateral renal lymphangiomatosis: US and CT findings
JR Varela, A Bargiela, and I Requejo
Eur Radiol 1998; 8: 230. Indian Journal of Urology
Case report: CT appearance of capillary and cavernous lymphangiomatosis of the spleen in an adult
TR Bader, G Ranner, and M Klimpfinger
Clin Radiol 1998; 53: 379. Elsevier/ ScienceDirect
Sclerotic variant of lymphangiomatosis of bone: imaging findings at diagnosis and long-term follow-up
R Forstner, C Datz, and O Dietze
Skeletal Radiol 1998; 27: 445. Springerlink
Splenic lymphangiomatosis in children
DT Wadsworth, B Newman, SJ Abramson, BLM Carpenter, and RL Lorenzo
Radiology 1997; 202: 173. [Abstract] 
Extensive intraosseous gas associated with lymphangiomatosis of bone: report of three cases
RR Brown, MN Pathria, PM Ruggieri, JA Jacobson, JG Craig, and D Resnick
Radiology 1997; 205: 260. [Abstract]

Splenic lymphangiomatosis: a rare cause of splenomegaly.

May 1987 PubMed

Diffuse pulmonary lymphangiomatosis: imaging findings

2005 Internentional Radiology

Molitch, H. I., E. C. Unger, C. L. Witte, and E. vanSonnenberg. 1995. Percutaneous sclerotherapy of lymphangiomas. Radiology 194: 343-347

Ramani P,Shah A Lymphangiomatosis. Histologic and immunohistochemical analysis of four cases. Am J Surg Pathol 1993; 17:329-35

Swensen SJ, Hartman TE, Mayo JR, et al. Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr 1995; 19:348-52

Kirchner, J., V. Jacobi, M. Schneider, and R. Wagner. 1997. Primary congenital pulmonary lymphangiectasiaa case report. Wien. Klin. Wochenschr. 12:109:922-924.

White, J. E., D. Veale, D. Fishwick, L. Mitchell, and P. A. Corris. 1996. Generalised lymphangiectasia: pulmonary presentation in an adult. Thorax 51: 767-768

Swank, D. W., N. G. Hepper, K. E. Folkert, and T. V. Colby. 1989. Intrathoracic lymphangiomatosis mimicking lymphangioleiomyomatosis in a young woman. Mayo Clin. Proc. 64: 1264-1268

Margraf, L. R.. 1996. Thoracic lymphangiomatosis. Pediatr. Pathol. Lab. Med. 16: 155-160

Canny GJ, Cutz E, MacLusky IB, et al. Diffuse pulmonary angiomatosis. Thorax 1991; 46:851-3

Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000; 161:1037-46

Felman, A. H., R. M. Rhatigan, and K. K. Pierson. 1972. Pulmonary lymphangiectasia: observation in 17 patients and proposed classification. Am. J. Roentgenol. Radium. Ther. Nucl. Med. 116: 548-558

Lloyd, E. S., and H. C. Press Jr.. 1979. Congenital pulmonary lymphangiectasis. South. Med. J. 72: 1205-1206


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