17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 283

Chapter

Lymphatic Tumors 17 L. van den Hauwe, F.Ramon

Contents angiomyoma and its more widespread form, which is called lymphangiomyomatosis, are characterized by a 17.1 Introduction ...... 283 proliferation of smooth muscle in the lymphatic struc- 17.2 Lymphangioma ...... 283 tures of the mediastinum, retroperitoneum and the 17.2.1 Classification and Clinical Behavior ...... 283 lung. These benign lesions affect only women. The term 17.2.2 Imaging Findings ...... 288 lymphangiopericytoma has been abandoned [14]. 17.2.2.1 Ultrasonography ...... 288 The collective term of ‘angiosarcoma’ has been sug- 17.2.2.2 Computed Tomography ...... 289 gested instead of lymphangiosarcoma, as these malig- 17.2.2.3 Magnetic Resonance Imaging ...... 289 nant tumors not only are composed of groups of endo- 17.2.2.4 Lymphography ...... 291 thelium-lined empty spaces suggesting lymphatics, but 17.3 Lymphangiomyoma and Lymphangiomyomatosis . . . 291 also contain areas resembling hemangiosarcoma [11, References ...... 291 12]. They are seen exclusively in patients with long- standing lymph stasis such as is seen after radical mastectomy (postmastectomy lymphangiosarcoma) or in chronic lymphedema of the lower extremities [12]. 17.1 Introduction They are discussed in Chap. 16.

At the Workshop on Vascular Anomalies in Rome in June 1996, the Mulliken and Glowacki classification was 17.2 Lymphangioma accepted for various cutaneous vascular lesions. This classification is based upon clinical, histological and 17.2.1 Classification and Clinical Behavior cytological features [9, 10, 30]. According to Enzinger, lymphatic tumors can be classified into benign and ma- Histologically, three types of lymphangioma have tradi- lignant lesions [14]. As compared with tumors arising tionally been described based on the size of the lym- from blood vessels, soft tissue tumors of lymphatic ori- phatic channels. Capillary lymphangioma or simple gin are rare; benign lesions include the solitary lymph- lymphangioma (‘lymphangioma simplex’) is composed angioma, lymphangiomatosis, lymphangiomyoma and of small, capillary-sized endothelium-lined lymphatics, lymphangiomyomatosis. There is growing evidence whereas cavernous lymphangioma is made up of larger to suggest that the majority of these lesions represent lymphatic channels with adventitial coats. Cystic lym- hamartomas or developmental lymphangiectasias phangiomas or hygromas are multilocular masses, con- rather than true neoplasms [11, 40]. stituted of large macroscopic lymphatic spaces that pos- Although three types of lymphangioma have been sess investitures of collagen and smooth muscle [12]. described – capillary, cavernous and cystic – they all Some authors have added a fourth entity to this classifi- belong to a single group of lesions [40, 50]. They can cation system, which is called vasculolymphatic malfor- affect any part of the body, their presentation depend- mation [19, 50]. Although these classification systems ing on the surroundings, but a strong predilection for are widely used,there are arguments for considering the the neck and axilla has been noted [23]. group of lymphangiomas as a single clinical entity. The When bones, soft tissues and visceral organs are distinction between cavernous and cystic lymphan- involved in a diffuse or multifocal manner, the term gioma is not always clear-cut and is often arbitrary. ‘lymphangiomatosis’ is used. Like angiomatosis, this ex- Moreover, cystic and cavernous components of lymph- tremely rare disease is most often seen in children [14, angioma often coexist in the same lesion, suggesting 46]. Clinically and histopathologically,there is consider- that cystic lymphangioma can arise out of a long-stand- able overlap between these two entities [18]. Lymph- ing cavernous lymphangioma in which the cavernous 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 284

284 L. van den Hauwe, F.Ramon

Fig.17.1Ia,b. Cystic lymphangioma of the neck in a 6-day-old premature baby boy. a Sagittal SE T1-weighted MR image. b Sagittal TSE T2-weighted MR image. A heterogeneous multilocular cystic mass is detected in the region of the neck, extending into the floor of the mouth and oral cavity (a). The cystic aspect of the lesion is best demonstrated on the T2-weighted (b). Owing to a hemorrhage, most of the cystic components demonstrate high SI on the T1-weighted image with presence of fluid- fluid levels (a, b). Low SI of the dependent parts of the cystic components is indicative of the presence of hemosiderin

ab

spaces have progressively widened to form cystic spaces of the isolated lymphatic channels. This may result in [1]. It has been suggested by Bill and Sumner that the the formation of lymphangiomas, especially the more classically described histological differences are the re- voluminous central cystic hygromas [48]. A second the- flection of their anatomical location [3]. This means ory may help to explain the characteristics of the more that the morphology of the lesion is dictated by the his- peripherally located lesions, such as capillary and cav- tological composition of the surrounding tissues. This ernous lymphangiomas, and makes it easier to under- theory may explain why cystic lymphangiomas most stand them. When abnormal sequestration of lymphat- frequently arise in the neck and axilla (Figs. 17.1, 17.2), ic tissue occurs early in embryologic life, failure to join where loose connective tissue allows for the expansion the normally developed central lymphatic channels will of the endothelium lined lymphatic channels. Con- result in lymphatic malformations [41,50].Another the- versely, cavernous lymphangiomas are encountered in ory explains the branching and permeative growth pat- the mouth, lips, cheek, tongue or other areas where tern into the surrounding anatomical structures seen in dense connective tissue and muscle prevent expansion some cavernous lymphangiomas [25]. Aberrant buds of (Fig. 17.3) [6]. In the tougher dermal and epidermal lymphatic material lose their connections with the lym- elements, expansion is further limited, resulting in the phatic primordia and may give rise to lymph-filled formation of a capillary lymphangioma (e. g., lymph- cysts. These cysts keep their ability to branch and grow angioma circumscriptum) (Fig. 17.4) [50]. and do so in an uncontrolled, disorderly manner. There has been a lot of discussion about the exact eti- Compared with hemangiomas, lymphatic malforma- ology of these lesions, including whether they are true tions are a rare finding. There is no clear sexual neoplasms or represent developmental malformations. predilection. About 50–65 % of these lesions are found The ‘neoplastic’ aspect of these malformations has been at birth, and as many as 90 % may be noticed within the made responsible for the local aggressive potential of first 2 years of life. Cystic lymphangioma or cystic hy- some lesions [18]. Although lymphangiomas can arise groma of the head, neck and axilla is the most frequent- on a obstructive basis after trauma,surgery,radiation or ly encountered and best-known entity (Fig. 17.1). A infection, it is now widely believed by most authors that cervical lobulated, fluctuating mass in the supraclavicu- lymphangiomas are congenital lymphatic malforma- lar fossa, the posterior triangle or the axillary region, tions that are the result of noncommunication between not attached to the skin but fixed to the deep tissues of sequestered lymphoid tissue and the peripheral lym- the neck is seen in these patients [8, 40]. The mass is phatic system [4, 12, 15, 16, 31, 40, 44, 50]. usually made up of a conglomerate of cysts and sheets Three major theories have been proposed to explain of tissue, extending in various directions and separating this concept [48, 50]. Failure of the primordial lymphat- nerves, vessels and fascial planes. A clear or straw-col- ic sacs to drain into the veins will result in enlargement ored serous fluid can be aspirated out of the cystic com- 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 285

Chapter 17 Lymphatic Tumors 285

b

ac

Fig.17.2Ia–c. Fluctuating characteristics of lymphangioma arising filtrating mass is noted, separating vessels, nerves and fascial in the anterior cervical triangle. a Coronal TSE T2-weighted MR planes. Note the shift of the trachea (b) compared with the control image. b Axial TSE T2-weighted MR image. c Axial TSE T2-weigh- examination 6 weeks later (c). Spontaneous involution occurred. ted MR image at the same level, 6 weeks later. A heterogeneous in- There was no history of local infection or trauma

ponents of the mass. When cystic hygromas arise in the times these unicystic or multicystic masses extend from anterior cervical triangle – the submandibular area, the neck into the mediastinum,but primary mediastinal floor of the mouth, base of tongue or epiglottis – airway cystic hygroma can occur. In adults, the radiological di- obstruction and feeding problems can arise [8]. Also, agnosis of these lesions is much more difficult. They are second branchial cleft cysts arise anterolaterally in the not infrequently an incidental finding on imaging and neck, displacing the sternocleidomastoid muscle pos- are generally asymptomatic [36, 39]. Recurrent tumors terolaterally and the carotid artery posteromedially. following incomplete resection during childhood may Based on their unilocular aspect and their clinical pre- present in this manner [36].Sporadically,lesions arise at sentation, they can be differentiated quite easily from other sites in the body that are served by the lymphatic lymphangioma in most cases [26]. Sudden enlargement system, such as the chest wall (Fig. 17.5), the abdominal is indicative for superinfection of the lesion, which is wall (Fig. 17.6), the retroperitoneum (Fig. 17.7) and the frequently encountered after trauma or respiratory mesentery [2]. Parenchymal organs, including the lung, tract infection (Fig. 17.2) [8]. Cystic hygromas are often gastrointestinal tract, spleen, and liver, can also be in- isolated malformations, and in this case the remainder volved [13].The vast majority of lymphangiomas affect- of the lymphatic system is normal. In cases of general- ing the musculoskeletal system are soft tissue lesions, ized disease with an abnormally developed peripheral whereas osseous locations are a rare finding [22, 28]. In lymphatic system, an association with hydrops fetalis Maffucci’s syndrome both lymphangiomas and heman- and Turner’s syndrome is found [5, 50]. In recent years, giomas are found. fetal cystic hygroma has also been described in associa- Lymphangiomas are seldom life-threatening; serious tion with other congenital malformation syndromes, medical problems can, however, arise depending on the such as Noonan’s syndrome, fetal alcohol syndrome, size, location and type of the lesion, and these require a distichiasis–lymphedema syndrome, familial ptery- surgical approach, which is the treatment of choice. In gium colli, and several chromosomal aneuploidies [5, this context, resection of well-circumscribed cystic 48]. In these cases where a hygroma is detected during lymphangiomas has a higher success rate than resection fetal life, careful sonographic examination of the entire of cavernous lymphangiomas, arising in and infiltrating fetus, determination of the fetal karyotype and an eval- the muscle fibers of the tongue or cheek. Inadequate uation of the family history are indicated [5]. Some- tumor removal almost invariably results in a higher rate 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 286

286 L. van den Hauwe, F.Ramon

a b

c

d

Fig.17.3Ia–e. Intramuscular cavernous lymphangioma in the right vastus lateralis muscle in a 12-year-old boy complaining of pain. a Axial SE T1-weighted MR image. b Axial SE T2-weighted MR image. c Axial SE T1-weighted MR image after Gd-contrast injection. d Coronal SE T1-weighted MR image after Gd-contrast injection. e Coronal fat suppressed MR image. On a T1-weighted image (a) SI is equal to that of muscle. On T2-WI (b) the mass has a higher SI than the surrounding muscles.After gadolinium-injec- tion (c, d) a more heterogeneous uptake of contrast medium is e seen. Note the serpiginous characteristics of the lesion, indicative for a lymphangioma

Fig.17.4I. Capillary lymphangioma of the right lower limb in a 6-year-old girl. Sagittal SE T1-weighted MR images. Disappear- ance of the normal high SI of the fat on both T1- and T2-weighted images by a cluster composed of small, capillary-sized endotheli- um-lined lymphatics. The tougher dermal and epidermal ele- ments prevent expansion of the lymphatics resulting in the forma- tion of a capillary lymphangioma 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 287

Chapter 17 Lymphatic Tumors 287

a

a

b

Fig.17.7Ia,b. Cystic retroperitoneal lymphangioma of the upper b right abdomen in a 43-year-old woman, presenting with com- plaints of constipation and intermittent upper right flank pain. a CT scan after iodine contrast injection. b Electrophoretic analy- Fig.17.5Ia,b. Swelling at the right axilla in a three-year-old boy that appeared after aspiration-puncture by the general physician: sis of the sample.A large retroperitoneal cystic tumor with negati- a coronal fat suppressed TSE T2-weighted image (STIR); b axial ve attenuation value can be seen in Morrison’s space, extending in a STIR-image. Huge heterogeneous mass in the right axilla extend- the parietocolic wall and the retropancreatic space ( ). More than ing cranially to the supraclavicular fossa and caudally in the tho- 400 ml of yellow pale fatty fluid was obtained by percutaneous as- b racic wall. The lesion has a polylobular appearance. Low SI areas piration of the lesion.Lipids electrophoretic analysis ( ) revealed a correspond probably with hemorrhagic foci high content of pre-beta lipoproteins and chylomicrons,indicating the presence of lymph fluid. (Courtesy of V.Poncelet, Centre Hos- pitalier de Sainte-Ode, Belgium)

of local recurrences and such complications as nerve palsies [8, 40]. Recently, percutaneous sclerotherapy of unresectable lymphangiomas has been described as safe and effective for palliative treatment in these pa- tients [29]. Superinfection, which can arise from trau- matic events or therapeutic aspiration, can also occur and be an indication for radical surgical extirpation [12]. For all these reasons, the role of medical imaging is to reveal the exact location and full extent of the lesion, as this may determine the therapeutic approach to be adopted in the particular patient [7]. Fig.17.6. Cavernous lymphangioma of the abdominal wall in a Lymphangiomatosis is characterized by abnormal 47-year-old man. Frank swelling with discoloration of the overly- lymph tissue at multiple sites, involving every tissue ing skin was noted.Axial enhanced CT scan.The normal density of the fatty tissue of the right abdominal wall has been replaced by a type, except for nervous tissue [47]. Like angiomatosis, heterogeneous mass. Multiple cystic structures and internal con- this extremely rare disease is most often seen in chil- trast-enhancing septations can be discerned 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 288

288 L. van den Hauwe, F.Ramon

dren [14, 46] (Fig. 17.8). Clinically and histopathologi- Overlying skin changes with hyperplasia, vesicle forma- cally, there is considerable overlap between these two tion and pigmentation changes are noted. On palpation, entities [18]. In patients with visceral involvement dif- the accumulated fluid can be moved within the swollen ferential diagnoses include: lymphoma, leukemic infil- area. Differential diagnosis against angiomatosis of the tration, metastatic disease, and plain cysts [27]. ‘Spinal soft tissues is based on clinical grounds when a contigu- involvement consists of replacement of bone with ous large segment of the body is affected. lymph tissue, resulting in direct neural compression or bony instability [47]. Gomez et al. recently described a variant form of lymphangiomatosis with limited in- 17.2.2 Imaging Findings volvement, predominantly involving the soft tissues of the limbs and bones [18].Clinical behavior,pathological 17.2.2.1 Ultrasonography features, and outcome of the disease are different from those seen in other patients with lymphangiomatosis. Since cystic hygromas are usually located superficially, On clinical examination, a fluctuant and sponge-like they can easily be examined with high-resolution ultra- swelling of the limb – either focal or diffuse – is noted. sonography (US) [38].As expected from their cystic na- ture, they will typically appear as a multiloculated sonolucent mass in the posterior triangle of the neck (Fig. 17.9) [48]. A more heterogeneous appearance is seen when internal septations of variable thickness and/or solid echogenic components of varying size arising from the cyst wall or septa are demonstrated (Fig. 17.15) [38]. An increasing number of lesions are detected on prenatal ultrasonography. Three-dimen- sional US and MRI may help in assessing the extent of the lesion [35].When the diagnosis of lymphangioma is made in utero amniocentesis and follow-up examina- tion are mandatory, as these lesions often show tenden- cy to grow with an increase in size of the afflicted area, and diffuse skin edema may develop [48]. Also, any as- sociated anomalies should be ruled out, as these lymph- a angiomas can be seen in a variety of congenital syn-

b

Fig.17.8Ia,b. Lymphangiomatosis in a ten-year-old boy with a Fig.17.9. Cystic hygroma of the neck in a newborn patient. Longi- prenatal ultrasound diagnosis of swelling at the right upper arm tudinal sonogram shows an inhomogeneous multilocular mass and hemithorax: a axial SE T1-weighted image; b coronal fat sup- lesion. Multiple anechogenic cystic components are separated by pressed TSE T2-weighted image (STIR). Large ill-defined mass solid components of mixed echogenicity indicating the internal lesion at the extensor compartment of the forearm, extending septations of the lesion proximally and over the axilla to the right thoracic wall.The exten- sive involvement of different compartments (muscles, subcutis and cutis) by moniliform and serpiginous structures is superbly demonstrated on STIR images 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 289

Chapter 17 Lymphatic Tumors 289

Fig.17.10Ia,b. Cystic lymphangioma in a 17-year-old male patient who presented with a fluctuating mass lesion in the right ante- rior right neck. a Axial enhanced CT-scan. b Sagittal reformatted image. Multilocular nonenhancing, cystic mass in the submandi- bular space, deep to the sternocleidomastoid muscle and slightly pushing the carotid art- ery medially can be seen on the axial image (a). Extension of the lesion into the media- stinum can be excluded on the sagittal reformatted images (b)

ab

dromes (e. g., Turner’s syndrome, Noonan’s syndrome, ed images, where the mass has a higher signal intensity fetal alcohol syndrome, familial pterygium colli, Down’s (SI) than the surrounding muscles (Fig. 17.3 a) [40]. syndrome, . . .) [5, 48, 50]. Based on the findings of pre- However, SI are nonspecific and are similar to those of natal US and clinical examination, Schuster et al. intro- hemangiomas [26, 34]. For the evaluation of lymph- duced a classification for lymphangioma colli,to predict angiomas located in the subcutaneous fat, STIR-se- the expected morbidity and prognosis with surgical quences are strongly recommended (Fig. 17.3 e). With treatment [37]. the use of SE T1-weighted imaging with fat-suppression Other cystic masses arising in the neck are branchial lymphangiomas may be differentiated from hem- cleft cysts or thyroglossal duct cysts. However, these le- angiomas, possibly obviating the use of gadolinium. sions have a typical location and tend to be unilocular. Hemangiomas will have a high SI, whereas lymph- Differential diagnosis should be made against atypical angiomas may have a low SI [17]. Larger series however manifestation forms of abscesses, resolving hematomas are necessary to validate this finding. On T1-weighted and necrotic lymph nodes [38]. images, SI is equal to or slightly lower than that of mus- cle (Fig. 17.3 b), unless complicated by hemorrhage (Fig. 17.1), infection or prior surgery [26]. As already 17.2.2.2 Computed Tomography mentioned before, hemangiomas may show the pres- ence of phleboliths which can be visualized on MRI us- Findings on CT are highly variable; a uniformly cystic ing gradient echo techniques. Flow voids arising from mass or a more heterogeneous aspect of the lesion, feeding arteries or draining veins can be seen in high- including both cystic and solid components can be seen flow hemangiomas and allow rejection of the diagnosis (Fig. 17.10). After intravenous administration of con- of lymphangioma. Gadolinium injection may offer trast agent,no definite enhancement of the lesion can be some help in differentiation between hemangiomas and discerned [36]. CT might hold a minor advantage over lymphangiomas, as hemangiomas show a homoge- MRI in differentiating lymphangiomas from heman- neous enhancement whereas lymphangiomas have a giomas, since the latter group may show the presence of more heterogeneous uptake of contrast medium calcified phleboliths in the lesion [26]. (Fig. 17.3 c, d) [33]. Although dynamic contrast-en- hanced MR-imaging may help to differentiate slow flow lesions (lymphangiomas) from high flow lesions (he- 17.2.2.3 Magnetic Resonance Imaging mangiomas, fistula), time constraints may prohibit the routine use of this sequence in the work-out of both As in other abnormalities seen throughout of the body, these benign lesions [43]. More studies are needed to MRI has the benefits of multiplanar acquisitions and demonstrate the role of magnetic resonance angiogra- the lack of ionizing radiation. The strength of MRI in phy (MRA) and magnetic resonance lymphography in the evaluation of such soft tissue lesions as lymph- the work-up of lymphatic tumors [46,24].Magnetic res- angiomas and hemangiomas lies in its capability to onance lymphography with superparamagnetic injec- demonstrate the full extent of the lesion (Fig. 17.11) [7, tion of iron oxide is currently still in an experimental 26, 49]. This can be most easily achieved on T2-weight- phase, but may prove its validity in the future [1, 20, 42]. 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 290

290 L. van den Hauwe, F.Ramon

a b

Fig.17.11Ia–c. Lymphangiomatosis with involvement of the head and neck region in a 2-month-old baby-girl. a Axial SE T1-weigh- ted MR image. b Axial TSE T2-weighted MR image. c Axial SE T1-weighted MR image after Gd-contrast injection. Diffuse invol- vement of the neck bilaterally with extension into the deep spaces c of the neck. Involvement of the parotid glands which are difficult to individualize 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 291

Chapter 17 Lymphatic Tumors 291

17.2.2.4 Lymphography References

In the work-up of mediastinal or retroperitoneal le- 1. Anzai Y, Blackwell K, McLachlan S, Fu YS, Abemayor E, Lufkin RB (1993) Initial clinical experience with an iron-based MR sions, the aim of lymphography was to demonstrate the imaging contrast agent for lymphnodes. Radiology 189 (P):108 presence of abnormally dilated and cystic lymphatic 2. Arts M, De Schepper A, Bracke P,Kockx M (1994) Unusual pre- channels [32, 45]. However, now that CT and MRI are sentation of a retroperitoneal lymphangiectatic cyst: a case re- port. Eur Radiol 4:262–264 widely available, we believe lymphography is no longer 3. Bill AA, Sumner DS (1965) A unified concept of lymphan- indicated for the diagnosis of lymphangioma. gioma and cystic hygroma. Surg Gynecol Obstet 120:79–86 4. Blumenthal EZ, Gottehrer NP, Dollberg M, Halevy J (1994) A giant tuberculous lymphangioma extending from the medi- astinum to the inguinal region. Chest 105:1279–1280 17.3 Lymphangiomyoma 5. Chervenak FA, Isaacson G, Blakemore KJ, Breg WR, Hobbins and Lymphangiomyomatosis JC, Berkowitz RL, Tortora M, Mayden K, Mahoney MJ (1984) Fetal cystic hygroma. N Engl J Med 309:822–825 6. Chisin R, Fabian R, Weber AL, Ragozzino M, Brady TJ, Good- Lymphangiomyomatosis is a rare, progressive disease, man M (1988) MR Imaging of a lymphangioma involving the only afflicting young women of childbearing age masseter muscle. J Comput Assist Tomogr 12:690–692 7. Dubois J, Garel L (1999) Imaging and therapeutic approach of (Fig. 17.10). Despite its aggressive nature, lymphangio- hemangiomas and vascular malformations in the pediatric age myomatosis is not a true neoplastic process, because of group. Pediatr Radiol 29:879–893 its highly organized structure lacking any cellular dis- 8. Emery PJ, Bailey CM, Evans JNG (1984) Cystic hygroma of the head and neck: a review of 37 cases. J Laryngol Otol 98:613– array, cell atypia or mitotic activity. Abnormal smooth 619 muscle proliferation and subsequent gradual oblitera- 9. Enjolras O (1997) Classification and management of the vari- tion of small airways, lymphatics and vascular struc- ous superficial vascular anomalies: hemangiomas and vascu- lar malformations. J Dermatol 24: 701–710 tures may result in dyspnea, recurrent pneumothorax, 10. Enjolras O, Mulliken JB (1997) Vascular tumors and vascular chylous effusion and hemophtisis. Plain chest radio- malformations. Adv Dermatol 13:375–423 graphy and CT of the thorax may demonstrate pleural 11. Enzinger FM, Weiss SW (1995) Malignant vascular tumors. In: Enzinger FM, Weiss SW (eds) Soft tissue tumors, 3rd edn. St effusion, multiple granular opacities and both mediasti- Louis, Mosby, pp 641–677 nal masses and adenopathies. Pulmonary involvement, 12. Enzinger FM, Weiss SW (1995) Tumors of lymph vessels. In: which is responsible for most of the morbidity and mor- Enzinger FM,Weiss SW (eds) Soft tissue tumors 3rd edn. Mos- by, St Louis, pp 679–700 tality, is an indicator of a poor prognosis [21]. However, 13. Enzinger FM, Weiss SW (1996) Lymphatic tumors. In: Meis- recent findings suggest a favorable prognosis in patients Kindblom JM, Enzinger FM (eds) Color atlas of soft tissue tu- with localized disease, treated with progestational mors. St Louis, Mosby, pp 174–178 14. Enzinger FM, Lattes R, Torloni R (1969) Histological typing of agents [13]. Since patients with tuberous sclerosis may soft tissue tumors. World Health Organization, Geneva have similar lesions affecting lungs and lymph nodes, 15. Fisher D, Hiller N (1994) Giant tuberculous cystic lymphan- speculation has been expressed as to whether lymph- gioma of posterior mediastinum, retroperitoneum and groin. Clin Radiol 49:215–216 angiomyomatosis is a forme fruste of this disease [14]. 16. Fisher I, Orkin M (1970) Acquired lymphangioma (lymphang- iectasis). Arch Dermatol 101:230 17. Gielen JL, De Schepper AM, Parizel PM, Wang XL, Vanhoen- acker F (2003) Additional value of magnetic resonance with Things to remember: spin echo T1-weighted imaging with fat suppression in char- 1. Tumors of lymphatic origin are rare, and are usu- acterization of soft tissue tumors. J Comput Assist Tomogr ally detected early in childhood. 27:434–441 18. Gomez CS, Calonje E, Ferrar DW, Browse NL, Fletcher CDM 2. Lymphangiomas have been reported in every type (1995) Lymphangiomatosis of the limbs: clinicopathologic of tissue except for neural tissue. analysis of a series with good prognosis. Am J Surg Pathol 3. An increasing number of lesions are detected on 19:125–133 19. Harms SE, Greenway G (1992) Musculoskeletal tumors. In: prenatal ultrasonography and fetal MRI. Stark DD, Bradley WG Jr (eds) Magnetic resonance imaging 4. MRI is the imaging method of choice to demon- 2 nd edn. Mosby, St. Louis, pp 2107–2222 strate the full extent of the lesion, especially on fat 20. Heautot JF,Weissleder R, Schaffer B, Brady TJ (1993) Intraarte- rial MR lymphography: technique to improve accumulation of suppressed images. contrast agents in lymph nodes. Radiology 189 (P):246 5. SI of lymphangioma are non-specific and are sim- 21. Johnson SF, Davey DD, Cibull ML, Schwartz RW, Strodel WE ilar to those of hemangioma. The absence of flow (1993) Lymphangioleiomyomatosis. Am Surg 59:395–399 22. Jumbelic M, Feuerstein IM, Dorfman HD (1984) Solitary in- voids and the lack of significant enhancement af- traosseous lymphangioma. J Bone Joint Surg [Am] 66:1479– ter Gd-contrast injection are in favor for the diag- 1481 nosis of lymphangioma. 23. Kransdorf MJ (1995) Benign soft-tissue tumors in a large re- ferral population: distribution of specific diagnoses by age, sex, and location. AJR Am J Roentgenol 164:395–402 17_DeSchepper_Lymphatic 15.09.2005 13:28 Uhr Seite 292

292 L. van den Hauwe, F.Ramon

24. Laor T, Hoffer FA, Burrows PE, Kozakewich HP (1998) MR 39. Shin MS, Berland LL, Ho KJ (1985) Mediastinal cystic hygro- lymphangiography in infants, children, and young adults. AJR mas: CT characteristics and pathogenetic consideration. Am J Roentgenol 171:1111–1117 J Comput Assist Tomogr 9:297–301 25. Lee K (1980) Surgery of cysts and tumours of the neck. In: 40. Siegel MJ, Glazer HS, St Amour TE, Rosenthal DD (1989) Lym- Paparella M, Shunrick D (eds) Otolaryngology. Saunders, phangiomas in children: MR imaging. Radiology 170:467–470 Philadelphia, p 2987 41. Smith DW (1982) Recognizable patterns of human malforma- 26. Mancuso AA, Dillon WP (1989) The neck. Radiol Clin North tion: genetic, embryologic, and clinical aspects, 3rd edn. Saun- Am 27:407–434 ders, Philadelphia, pp 472–473 27. Melzer G, Kullnig P, Fluckiger F, Beham A (1991) CT image of 42. Sophocleous S, Ehrenheim C, Hamann A, Hundeshagen H diffuse visceral lymphangiomatosis. Fortschr Rontgenstr 155: (1994) Cavernous lymphangioma of the pelvic area – MR to- 282–283 mographic and lymphoscintigraphic findings. Fortschr Ront- 28. Méndez JA, Hochmuth A, Boetefuer IC, Schumacher M (2002) genstr 160:574–576 Radiologic appearance of a rare primary vertebral lymphan- 43. Rijswijk, van, CS, Geirnaerdt MJ, Hogendoorn PC, Taminiau gioma. Am J Neuroadiol 23:1665–1668 AH, van Coevorden F, Zwinderman AH, Pope TL, Bloem JL 29. Molitch HI, Unger EC,Witte CL, van Sonnenberg E (1995) Per- (2004) Soft-tissue tumors: value of static and dynamic cutaneous sclerotherapy of lymphangiomas. Radiology 194: gadopentetate dimeglumine-enhanced MR imaging in predic- 343–347 tion of malignancy. Radiology 233:493–502 30. Mulliken JB, Glowacki J (1982) Hemangiomas and vascular 44. Vanel D, Shapeero LG, De Baere T, Gilles R, Tardivon A, Genin malformations in infants and children: a classification based J,Guinebretière JM (1994) MR Imaging in the follow-up of ma- on endothelial characteristics. Plast Reconstr Surg 69:412–422 lignant and aggressive soft-tissue tumors: results of 511 exam- 31. Nosan DK, Martin DS, Stith JA (1995) Lymphangioma present- inations. Radiology 190:263–268 ing as a delayed posttraumatic expanding neck mass. Am J 45. Viamonte M, Jr, Rüttimann AL (1980) Atlas of lymphography. Otolaryngol Head Neck Med Surg 16:186–189 Thieme, Stuttgart, pp 164–172 32. Peh WCG, Ngan H (1993) Lymphography – still useful in the 46. Vogl TJ, Hammerstingl R, Schnell B, Klein Ch, Hauser M, diagnosis of lymphangiomatosis. Br J Radiol 66:28–31 Pfluger T, Lissner J (1992) Magnetic resonance tomography 33. Peiss J, Fuzesi L, Bohndorf K, Neuerburg J, Urhahn R, Gunther and magnetic resonance angiography of lymphangiomatosis. RW (1993) MR morphology of haemangiomas and lymphan- Fortschr Rontgenstr 157:414–419 giomas of the peripheral soft parts – correlation with angio- 47. Watkins RG IV, Reynolds RAK, McComb JG, Tolo VT (2003) graphy and histology. Fortschr Rontgenstr 158:463–470 Lymphangiomatosis of the spine. Two cases requiring surgical 34. Ramon F (1992) Vascular soft tissue tumors: medical imaging. intervention. Spine 28:E45–E50 J Belge Radiol [BTR] 75:303–310 48. Weingast GR, Hopper KD, Gottesfeld SA, Manco-Johnson ML 35. Ruano R, Aubry JP, Simon I, Grebille AG, Sonigo P, Dumez Y, (1988) Congenital lymphangiectsia with fetal cystic hygroma: Dommergues M (2003) Prenatal diagnosis of a large axillary report of two cases with coexistent Down’s syndrome. J Clin cystic lymphangioma by three-dimensional ultrasonography Ultrasound 16:663–668 and magnetic resonance imaging. J Ultrasound Med 22:419– 49. Wunderbaldinger P, Paya K, Partik B, Turetschek K, Hormann 423 M, Horcher E, Bankier AA (2000) CT and MR imaging of gen- 36. Schuster T, Grantzow R, Nicolai T (2003) Lymphangioma colli eralized cystic lymphangiomatosis in pediatric patients. AJR – a new classification contributing to prognosis. Eur J Pediatr Am J Roentgenol 174:827–832 Surg 13:97–102 50. Zadvinskis DP,Benson MT, Kerr HH, Mancuso AA, Cacciarelli 37. Shaffer K, Rosado-de-Christenson ML, Patz EF Jr, Young S, AA,Madrazo BL,Mafee MF,Dalen K (1992) Congenital malfor- Farver CF (1994) Thoracic lymphangioma in adults: CT and mations of the cervicothoracic lymphatic system: embryology MR imaging features. AJR Am J Roentgenol 162:283–289 and pathogenesis. Radiographics 12:1175–1189 38. Sheth S, Nussbaum AR, Hutchins GM, Sanders RC (1987) Cys- tic hygromas in children: sonographic-pathologic correlation. Radiology 162:821–824