Lymphatic Tumors 17

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angiomyoma and its more widespread form, which is called lymphangiomyomatosis, are characterized by a proliferation of smooth muscle in the lymphatic structures of the mediastinum, retroperitoneum and the lung. These benign lesions affect only women. The term lymphangiopericytoma has been abandoned [14].

The collective term of ‘angiosarcoma’ has been suggested instead of lymphangiosarcoma, as these malignant tumors not only are composed of groups of endothelium-lined empty spaces suggesting lymphatics, but also contain areas resembling hemangiosarcoma [11, 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].

They are discussed in Chap. 16.

17.2 Lymphangioma

17.2.1 Classification and Clinical Behavior

Histologically, three types of lymphangioma have tradi- tionally been described based on the size of the lymphatic channels. Capillary lymphangioma or simple lymphangioma (‘lymphangioma simplex’) is composed of small, capillary-sized endothelium-lined lymphatics, whereas cavernous lymphangioma is made up of larger lymphatic channels with adventitial coats. Cystic lymphangiomas or hygromas are multilocular masses, con- stituted of large macroscopic lymphatic spaces that pos- sess investitures of collagen and smooth muscle [12].

Some authors have added a fourth entity to this classiﬁ- cation system, which is called vasculolymphatic malformation [19, 50]. Although these classiﬁcation systems are widely used, there are arguments for considering the group of lymphangiomas as a single clinical entity. The distinction between cavernous and cystic lymphangioma is not always clear-cut and is often arbitrary.

Moreover, cystic and cavernous components of lymphangioma often coexist in the same lesion, suggesting that cystic lymphangioma can arise out of a long-standing cavernous lymphangioma in which the cavernous 17.1 Introduction

At the Workshop on Vascular Anomalies in Rome in June 1996, the Mulliken and Glowacki classification was accepted for various cutaneous vascular lesions. This classification is based upon clinical, histological and cytological features [9, 10, 30]. According to Enzinger, lymphatic tumors can be classified into benign and malignant lesions [14]. As compared with tumors arising from blood vessels, soft tissue tumors of lymphatic origin are rare; benign lesions include the solitary lymphangioma, lymphangiomatosis, lymphangiomyoma and lymphangiomyomatosis. There is growing evidence to suggest that the majority of these lesions represent hamartomas or developmental lymphangiectasias rather than true neoplasms [11, 40].

Although three types of lymphangioma have been described – capillary, cavernous and cystic – they all belong to a single group of lesions [40, 50]. They can affect any part of the body, their presentation depending on the surroundings, but a strong predilection for the neck and axilla has been noted [23].

When bones, soft tissues and visceral organs are involved in a diffuse or multifocal manner, the term

‘lymphangiomatosis’ is used. Like angiomatosis, this extremely rare disease is most often seen in children [14, 46]. Clinically and histopathologically, there is considerable overlap between these two entities [18]. Lymph-

Lymphatic Tumors

L. van den Hauwe, F. Ramon

17

17.1 Introduction . . . 283

17.2 Lymphangioma . . . 283

17.2.1 Classiﬁcation and Clinical Behavior . . . . 283

17.2.2 Imaging Findings . . . 288

17.2.2.1 Ultrasonography . . . 288

17.2.2.2 Computed Tomography . . . 289

17.2.2.3 Magnetic Resonance Imaging . . . 289

17.2.2.4 Lymphography . . . 291

17.3 Lymphangiomyoma and Lymphangiomyomatosis . . . 291

References . . . 291 Contents

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spaces have progressively widened to form cystic spaces [1]. It has been suggested by Bill and Sumner that the classically described histological differences are the re- ﬂection of their anatomical location [3]. This means that the morphology of the lesion is dictated by the histological composition of the surrounding tissues. This theory may explain why cystic lymphangiomas most frequently arise in the neck and axilla (Figs. 17.1, 17.2), where loose connective tissue allows for the expansion of the endothelium lined lymphatic channels. Con- versely, cavernous lymphangiomas are encountered in the mouth, lips, cheek, tongue or other areas where dense connective tissue and muscle prevent expansion (Fig. 17.3) [6]. In the tougher dermal and epidermal elements, expansion is further limited, resulting in the formation of a capillary lymphangioma (e. g., lymphangioma circumscriptum) (Fig. 17.4) [50].

There has been a lot of discussion about the exact eti- ology of these lesions, including whether they are true neoplasms or represent developmental malformations.

The ‘neoplastic’ aspect of these malformations has been made responsible for the local aggressive potential of some lesions [18]. Although lymphangiomas can arise on a obstructive basis after trauma, surgery, radiation or infection, it is now widely believed by most authors that lymphangiomas are congenital lymphatic malformations that are the result of noncommunication between sequestered lymphoid tissue and the peripheral lymphatic system [4, 12, 15, 16, 31, 40, 44, 50].

Three major theories have been proposed to explain this concept [48, 50]. Failure of the primordial lymphatic sacs to drain into the veins will result in enlargement

of the isolated lymphatic channels. This may result in the formation of lymphangiomas, especially the more voluminous central cystic hygromas [48]. A second theory may help to explain the characteristics of the more peripherally located lesions, such as capillary and cavernous lymphangiomas, and makes it easier to under- stand them. When abnormal sequestration of lymphatic tissue occurs early in embryologic life, failure to join the normally developed central lymphatic channels will result in lymphatic malformations [41, 50].Another theory explains the branching and permeative growth pat- tern into the surrounding anatomical structures seen in some cavernous lymphangiomas [25]. Aberrant buds of lymphatic material lose their connections with the lymphatic primordia and may give rise to lymph-ﬁlled cysts. These cysts keep their ability to branch and grow and do so in an uncontrolled, disorderly manner.

Compared with hemangiomas, lymphatic malformations are a rare finding. There is no clear sexual predilection. About 50–65 % of these lesions are found at birth, and as many as 90 % may be noticed within the first 2 years of life. Cystic lymphangioma or cystic hygroma of the head, neck and axilla is the most frequently encountered and best-known entity (Fig. 17.1). A cervical lobulated, fluctuating mass in the supraclavicular fossa, the posterior triangle or the axillary region, not attached to the skin but fixed to the deep tissues of the neck is seen in these patients [8, 40]. The mass is usually made up of a conglomerate of cysts and sheets of tissue, extending in various directions and separating nerves, vessels and fascial planes. A clear or straw-col- ored serous fluid can be aspirated out of the cystic com-

284

Fig. 17.1Ia, b. Cystic lymphangioma of the neck in a 6-day-old premature baby boy.

aSagittal SE T1-weighted MR image.

bSagittal 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

a b

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times these unicystic or multicystic masses extend from the neck into the mediastinum, but primary mediastinal cystic hygroma can occur. In adults, the radiological diagnosis of these lesions is much more difficult. They are not infrequently an incidental finding on imaging and are generally asymptomatic [36, 39]. Recurrent tumors following incomplete resection during childhood may present in this manner [36]. Sporadically, lesions arise at other sites in the body that are served by the lymphatic system, such as the chest wall (Fig. 17.5), the abdominal wall (Fig. 17.6), the retroperitoneum (Fig. 17.7) and the mesentery [2]. Parenchymal organs, including the lung, gastrointestinal tract, spleen, and liver, can also be involved [13]. The vast majority of lymphangiomas affecting the musculoskeletal system are soft tissue lesions, whereas osseous locations are a rare finding [22, 28]. In Maffucci’s syndrome both lymphangiomas and hemangiomas are found.

Lymphangiomas are seldom life-threatening; serious medical problems can, however, arise depending on the size, location and type of the lesion, and these require a surgical approach, which is the treatment of choice. In this context, resection of well-circumscribed cystic lymphangiomas has a higher success rate than resection of cavernous lymphangiomas, arising in and inﬁltrating the muscle ﬁbers of the tongue or cheek. Inadequate tumor removal almost invariably results in a higher rate ponents of the mass. When cystic hygromas arise in the

anterior cervical triangle – the submandibular area, ﬂoor of the mouth, base of tongue or epiglottis – airway obstruction and feeding problems can arise [8]. Also, second branchial cleft cysts arise anterolaterally in the neck, displacing the sternocleidomastoid muscle pos- terolaterally and the carotid artery posteromedially.

Based on their unilocular aspect and their clinical presentation, they can be differentiated quite easily from lymphangioma in most cases [26]. Sudden enlargement is indicative for superinfection of the lesion, which is frequently encountered after trauma or respiratory tract infection (Fig. 17.2) [8]. Cystic hygromas are often isolated malformations, and in this case the remainder of the lymphatic system is normal. In cases of general- ized disease with an abnormally developed peripheral lymphatic system, an association with hydrops fetalis and Turner’s syndrome is found [5, 50]. In recent years, fetal cystic hygroma has also been described in association with other congenital malformation syndromes, such as Noonan’s syndrome, fetal alcohol syndrome, distichiasis–lymphedema syndrome, familial pterygium colli, and several chromosomal aneuploidies [5, 48]. In these cases where a hygroma is detected during fetal life, careful sonographic examination of the entire fetus, determination of the fetal karyotype and an evaluation of the family history are indicated [5]. Some-

Fig. 17.2Ia–c. Fluctuating characteristics of lymphangioma arising in the anterior cervical triangle. a Coronal TSE T2-weighted MR image. b Axial TSE T2-weighted MR image. c Axial TSE T2-weigh- ted MR image at the same level, 6 weeks later. A heterogeneous in-

ﬁltrating mass is noted, separating vessels, nerves and fascial planes. Note the shift of the trachea (b) compared with the control examination 6 weeks later (c). Spontaneous involution occurred.

There was no history of local infection or trauma

a c

b

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Fig. 17.3Ia–e. Intramuscular cavernous lymphangioma in the right vastus lateralis muscle in a 12-year-old boy complaining of pain.

aAxial 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 seen. Note the serpiginous characteristics of the lesion, indicative for a lymphangioma

a

c

e

b

d

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 endothelium-lined lymphatics. The tougher dermal and epidermal elements prevent expansion of the lymphatics resulting in the formation of a capillary lymphangioma

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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 patients [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].

Lymphangiomatosis is characterized by abnormal lymph tissue at multiple sites, involving every tissue type, except for nervous tissue [47]. Like angiomatosis, this extremely rare disease is most often seen in chil-

Fig. 17.5Ia, b. Swelling at the right axilla in a three-year-old boy that appeared after aspiration-puncture by the general physician:

acoronal fat suppressed TSE T2-weighted image (STIR); b axial STIR-image. Huge heterogeneous mass in the right axilla extending cranially to the supraclavicular fossa and caudally in the thoracic wall. The lesion has a polylobular appearance. Low SI areas correspond probably with hemorrhagic foci

a

b

Fig. 17.7Ia, b. Cystic retroperitoneal lymphangioma of the upper right abdomen in a 43-year-old woman, presenting with com- plaints of constipation and intermittent upper right ﬂank pain.

aCT scan after iodine contrast injection. b Electrophoretic analysis of the sample. A large retroperitoneal cystic tumor with negati- ve attenuation value can be seen in Morrison’s space, extending in the parietocolic wall and the retropancreatic space (a). More than 400 ml of yellow pale fatty ﬂuid was obtained by percutaneous as- piration of the lesion. Lipids electrophoretic analysis (b) revealed a 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)

Fig. 17.6. Cavernous lymphangioma of the abdominal wall in a 47-year-old man. Frank swelling with discoloration of the overlying skin was noted.Axial enhanced CT scan. The normal density of the fatty tissue of the right abdominal wall has been replaced by a heterogeneous mass. Multiple cystic structures and internal contrast-enhancing septations can be discerned

a

b

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dren [14, 46] (Fig. 17.8). Clinically and histopathologically, there is considerable overlap between these two entities [18]. In patients with visceral involvement differential diagnoses include: lymphoma, leukemic inﬁl- tration, metastatic disease, and plain cysts [27]. ‘Spinal involvement consists of replacement of bone with lymph tissue, resulting in direct neural compression or bony instability [47]. Gomez et al. recently described a variant form of lymphangiomatosis with limited involvement, predominantly involving the soft tissues of the limbs and bones [18]. Clinical behavior, pathological features, and outcome of the disease are different from those seen in other patients with lymphangiomatosis.

On clinical examination, a ﬂuctuant and sponge-like swelling of the limb – either focal or diffuse – is noted.

Overlying skin changes with hyperplasia, vesicle formation and pigmentation changes are noted. On palpation, the accumulated ﬂuid can be moved within the swollen area. Differential diagnosis against angiomatosis of the soft tissues is based on clinical grounds when a contigu- ous large segment of the body is affected.

17.2.2 Imaging Findings 17.2.2.1 Ultrasonography

Since cystic hygromas are usually located superﬁcially, they can easily be examined with high-resolution ultrasonography (US) [38]. As expected from their cystic nature, 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-dimensional 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 examination 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 lymphangiomas can be seen in a variety of congenital syn-

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Fig. 17.8Ia, b. Lymphangiomatosis in a ten-year-old boy with a prenatal ultrasound diagnosis of swelling at the right upper arm and hemithorax: a axial SE T1-weighted image; b coronal fat sup- pressed TSE T2-weighted image (STIR). Large ill-deﬁned mass lesion at the extensor compartment of the forearm, extending 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

a

b

Fig. 17.9. Cystic hygroma of the neck in a newborn patient. Longi- tudinal sonogram shows an inhomogeneous multilocular mass lesion. Multiple anechogenic cystic components are separated by solid components of mixed echogenicity indicating the internal septations of the lesion

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ed images, where the mass has a higher signal intensity (SI) than the surrounding muscles (Fig. 17.3 a) [40].

However, SI are nonspeciﬁc and are similar to those of hemangiomas [26, 34]. For the evaluation of lymphangiomas located in the subcutaneous fat, STIR-se- quences are strongly recommended (Fig. 17.3 e). With the use of SE T1-weighted imaging with fat-suppression lymphangiomas may be differentiated from hemangiomas, possibly obviating the use of gadolinium.

Hemangiomas will have a high SI, whereas lymphangiomas may have a low SI [17]. Larger series however are necessary to validate this finding. On T1-weighted images, SI is equal to or slightly lower than that of muscle (Fig. 17.3 b), unless complicated by hemorrhage (Fig. 17.1), infection or prior surgery [26]. As already mentioned before, hemangiomas may show the presence of phleboliths which can be visualized on MRI us- ing gradient echo techniques. Flow voids arising from feeding arteries or draining veins can be seen in high- flow hemangiomas and allow rejection of the diagnosis of lymphangioma. Gadolinium injection may offer some help in differentiation between hemangiomas and lymphangiomas, as hemangiomas show a homoge- neous enhancement whereas lymphangiomas have a more heterogeneous uptake of contrast medium (Fig. 17.3 c, d) [33]. Although dynamic contrast-enhanced MR-imaging may help to differentiate slow flow lesions (lymphangiomas) from high flow lesions (hemangiomas, fistula), time constraints may prohibit the routine use of this sequence in the work-out of both these benign lesions [43]. More studies are needed to demonstrate the role of magnetic resonance angiography (MRA) and magnetic resonance lymphography in the work-up of lymphatic tumors [46, 24]. Magnetic resonance lymphography with superparamagnetic injection of iron oxide is currently still in an experimental phase, but may prove its validity in the future [1, 20, 42].

dromes (e. g., Turner’s syndrome, Noonan’s syndrome, fetal alcohol syndrome, familial pterygium colli, Down’s syndrome, . . .) [5, 48, 50]. Based on the ﬁndings of prenatal US and clinical examination, Schuster et al. intro- duced a classiﬁcation for lymphangioma colli, to predict the expected morbidity and prognosis with surgical treatment [37].

Other cystic masses arising in the neck are branchial cleft cysts or thyroglossal duct cysts. However, these lesions have a typical location and tend to be unilocular.

Differential diagnosis should be made against atypical manifestation forms of abscesses, resolving hematomas and necrotic lymph nodes [38].

17.2.2.2 Computed Tomography

Findings on CT are highly variable; a uniformly cystic mass or a more heterogeneous aspect of the lesion, including both cystic and solid components can be seen (Fig. 17.10). After intravenous administration of contrast agent, no deﬁnite enhancement of the lesion can be discerned [36]. CT might hold a minor advantage over MRI in differentiating lymphangiomas from hemangiomas, since the latter group may show the presence of calciﬁed phleboliths in the lesion [26].

17.2.2.3 Magnetic Resonance Imaging

As in other abnormalities seen throughout of the body, MRI has the beneﬁts of multiplanar acquisitions and the lack of ionizing radiation. The strength of MRI in the evaluation of such soft tissue lesions as lymphangiomas and hemangiomas lies in its capability to demonstrate the full extent of the lesion (Fig. 17.11) [7, 26, 49]. This can be most easily achieved on T2-weight-

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.

bSagittal reformatted image. Multilocular nonenhancing, cystic mass in the submandibular space, deep to the sternocleidomastoid muscle and slightly pushing the carotid artery 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)

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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 involvement of the neck bilaterally with extension into the deep spaces of the neck. Involvement of the parotid glands which are difﬁcult to individualize

a

c

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17.2.2.4 Lymphography

In the work-up of mediastinal or retroperitoneal lesions, the aim of lymphography was to demonstrate the presence of abnormally dilated and cystic lymphatic channels [32, 45]. However, now that CT and MRI are widely available, we believe lymphography is no longer indicated for the diagnosis of lymphangioma.

17.3 Lymphangiomyoma and Lymphangiomyomatosis

Lymphangiomyomatosis is a rare, progressive disease, only afﬂicting young women of childbearing age (Fig. 17.10). Despite its aggressive nature, lymphangiomyomatosis is not a true neoplastic process, because of its highly organized structure lacking any cellular dis- array, cell atypia or mitotic activity. Abnormal smooth muscle proliferation and subsequent gradual oblitera- tion of small airways, lymphatics and vascular structures may result in dyspnea, recurrent pneumothorax, chylous effusion and hemophtisis. Plain chest radio- graphy and CT of the thorax may demonstrate pleural effusion, multiple granular opacities and both mediastinal masses and adenopathies. Pulmonary involvement, which is responsible for most of the morbidity and mor- tality, is an indicator of a poor prognosis [21]. However, recent ﬁndings suggest a favorable prognosis in patients with localized disease, treated with progestational agents [13]. Since patients with tuberous sclerosis may have similar lesions affecting lungs and lymph nodes, speculation has been expressed as to whether lymphangiomyomatosis is a forme fruste of this disease [14].

Things to remember:

1. Tumors of lymphatic origin are rare, and are usually detected early in childhood.

2. Lymphangiomas have been reported in every type of tissue except for neural tissue.

3. An increasing number of lesions are detected on prenatal ultrasonography and fetal MRI.

4. MRI is the imaging method of choice to demonstrate the full extent of the lesion, especially on fat suppressed images.

5. SI of lymphangioma are non-specific and are similar to those of hemangioma. The absence of flow voids and the lack of significant enhancement after Gd-contrast injection are in favor for the diagnosis of lymphangioma.

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