43 Lymphology 11 (1978) 43- 48 Experience with Lymphangioma* Van Cauwelaert Ph.** and Gruwez J. A.*** Department of General Surgery, A. Z . Sr. Rafael, Leuven, Belgium Summary again , if this mechanism fails , induce cystic Lymphangioma is a rare and benign tumor, mostly forma lion. encountered in childhood. Our experience extends to 21 patients, all of which were operated upon. Rece nt experimental work on the early Early raoical excision in moderate form s o r cervical development of the lymphatic system by locations and delayed excision or combined manage­ Van der Putte is of strong support fo r the ment in complex cervical forms arc the more pre· centrifugal theory. A more or less regular fcrable .,;:.y of treatment. series of small and blind ending outgrowths of the major embryonic veins fom1 the early lymphatic system . The system is subdivided in four singular and twelve groups of primordia. History and Ontogenesis Theories Lymphangioma is a developmental defect of Except for some openings at the jugular the vascu lar system. To date, the pathogenesis subclavian junction all connections with veins is still s newhat controversial. In the develop­ disappear. ment of the lymphatic system, cell buds, The primordial ex tensions grow out centnfu­ representing the lymphati c primordium, are gally and invade the surrounding tissues. pinchec. off the venous channels and form a Finally, many regard lymphangiomas as capillary network growing into different harmartomas (Willis) i. e. an embryological tissues. tumor formed by an excessive growili of At some stage of the development these primi­ tissues in their normal location. tive lymnhatic sacs reunite witl1 the venous system . Frequency According to tllis centri fugal theory (Dowd, Lymphangioma is a rare tumor and accounts Goetsc", Sabin, Me. Clure, Silvester, Godart) for only 6% of benign tumors in childhood lymphangiomas develop when the primitive and for 5% of vascular growths. In a series of lymph ~·:c sacs fail to re-establish venous !52 benign neck tumors only 4lymphangiomas communication. We have then a sequestration were Hsted. of lymphatic channels which have a certain growin ~ potentiality. Pathology-Fonns The centripetal the01y (Huntington, Kamp­ Three main forms of lymphangiomas exist. meier) .1 ims that at a very early stage of I. Capillary lymphangioma, an uncommon foeta l development (9- 12 mm), mese nchymal and insignificant lesion, appearing as an slits apnear in the reticulum of venous ordinary wart or a group of small vesicles. plexu se~ . By coalescence of these spaces, Histologically it consists of a network of larger lymphatic cavities are formed which spaces formed by small and medium sized open se Jndarily into the venous system or vessels. • Re· ·at the Vlth Internatio nal Congress of Lymphology in Prague •• Resident in Surgery, A . Z. St. Rafael, Leuven •u Professor of Surgery, Katholieke Universiteit Leuven 0024- 7766/78 1400--0043 S 04.00 © 1978 Georg T hieme Publishers Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. 44 Van Cauwclacn Ph. and Gruwcz J. A. ,. Fig. l Resection specimen of cystic hygroma: lobulated endothelial-lined lymphatic spaces 2. The second form is the cavemous lymphan­ location. In lips, cheek and tongue, limited gioma. They occur usually in the skin and expansion is possible and the cavernous form subcutaneous tissue, the salivary gland, is induced. In the axilla, neck and chest wall tongue and lips, where they are responsible areas with loose fatty tissue permit relati vely for macroglossia and macrocheilia. They unlimited dilatation and cystic hygroma are diffuse, spongy, compressible masses, occurs. the margins of which are often difficult to Some authors classify the following other delineate. The microscopic appearance l tumors as lymphangioma: i. consists chieny of multiple dilated lymph channels lined with single or multiple 1) Lymphangio-haemangioma layers of endothelial cells. These tumo rs 2) Lymphangio-lipoma are noted for their invasive tendencies and 3) Lymphangiosarcoma they frequently penetrate underlying 4) Lymphangioma of bone muscle and contiguous structures. This disease has other names like "Vanishing 3. The third form is cystic hygroma. It is bone'·, "Phantom bone", multiple lymphan­ the most frequent of the three forms and giectasis of bone. The X-ray appear- it develops in the neck, the axilla (Fig. I) ance is diagnostic and scintigraphy is very and the _retroperitoneal region. helpful in locating the lesion. Osteolysis is slowly progressive and results in complete Less frequently it occurs in the mediastinum disappearance of bone after months or years. and the limbs, and rarely in bone, the wall of (The authors presented three cases in extenso the gastrointestinal tract, the mesenterium, at the IVth International Congress of the omentum, the spleen, the pancreas or the Lymphology). Fig. 2-3). liver and in the urogenital tract. Even in the heart a lymphangioma has been described and [tis worthwhile to mention two other relat~d recently we came across a case of lymphan­ conditions: gioma of the parotic gland. The tumor is I. Lymphangiomyomatosis of the lung and multicystic, and the thin wall ed cysts are the mediastinum. A chronic progressive "' lined by lymphatic endothelium. disease encountered in middle-aged females, Bill and Sumner support the theory that the often associated with chylothorax, hormono· I three forms of lymphangiomas are basically dependant and carrying a very poor . similar entities and that their morphologic prognosis of a mean survival rate of differences arc related to their anatomic 18 months. Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. Experience with Lymphangioma 45 ·I .- I .... a li I I I • m1 111 I 1 ,. I I Lymphangioma of bone: (Fig. 2) Radiographic and (Fig. 3) scintigraphic appearance 2. Lymphangiomatosis of liver and spleen was followed by infection with rapid with bone involvement has a similar poor enlargement of the tumor. prognosis. A number of symptoms depend on the loca­ We colk r: ted 2 1 cases of lymphangiomas, the tion of the tumors and rare clinical pictures distribu non of which among the different belonging to the chylous syndromes can occur. forms is mentioned on the fo llowing table. One of our patients, a young woman, presented with vulvar chylorrhea. In­ 21 PATIENTS vestigation brought about a huge Lympha; oma capillarc I cavernous lymphangioma of the pelvis. Lymphangioma cavernosum 4 (Fig. 4) Lymphangioma cysticum 8 Lymphangioma is often a disease afflicting Mixed fo. 1 (cavern.+ cyst.) 4 Lipo-lymphangioma I children as can be concluded from our series Lymphangioma of bone 3 where 65 % of the patients appeared in the pediatric age group. Symptm¥Js and Diagnosis 2 1 PATIENTS Usually the diagnosis is obvious: an abnormal present at birth 7 33% mass progressively increasing in size, trans­ 0- 2 y 11 55 % lucent <u1d sometimes rapidly enlarging as a 2- 14 y 2 LO% consequence of bleeding or infection. 14- 30 y 3 14 % 1 30- 65 y 5 24 % Iu l little girl with a large lymphan­ gioma of the axilla, puncture for biopsy Rate o f occurence related to age Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. 46 Van Cauwelaert Ph. and Gruwez J. A. Fig. 4 Lymphangiography in a case of vulvar chylorrhea due to cavernous lymphangioma of the retroperi­ toneum and the pelvis. The different locations in 453 patients which I . Spontaneous remission did not show in ow is a review of the literature, compared with patients. our own 21 patients are listed in the following 2. Sclerosing injections were used by us for a table. very mild recurrence after resection in the patient with vulvar lymphorrhea. Location Literature Own pt. 3. Radiation can be dangerous and in our Neck 39 % series we have a possible example of 30 % Head 20% neoplastic potentialization. In a man of Axilla 13% 25 % 30 years who initially had an angiofibroml Trunk 8% 10% of the right thigh which was treated by Limb 11 % 15% resection and radioactive gold seeds implan· Anatomical distribution tation recurrences occurred and the man finally ended up with a presacral Associated Developmental Pathology liposarcoma and metastases. Six of our patients showed other associated 4. Incision and drainage may be used as a palhology as: dysmaturity, microcephaly, low temporary measure to overcome life­ implantation of the ears, mental retardation, threatening situations. In one case of our large hiatu s hernia, pubertas tarda, eosinophilic series with extensive lymphangioma of the granuloma-lesions, angiofibroma, haeman­ neck, cheek and mouthfloor, this way of gioma, liposarcoma, fibro-lipo-haemangioma treatment had to be applied after partial and a gastric haemangioma. resection of the tumor. 5. Surgical excision seems to be the most Treatment reliable form of therapy and should be The goals of the treatment are (I) eradication used at the earliest opportunity. Many of the lesion and complete healing; (2) absence patients have to be operated upon several of morbidity and mortality; (3) good esthetic times as is listed in the next table. result. (Fig. 5-6). Permission granted for single print for individual use. Reproduction not permitted without permission of Journal LYMPHOLOGY. Experience w ith Lymphangioma 47 Fig. 5-6 Huge cervical cystic lymphangioma and morphological result, several years after excision. Conclusion 1 operation 16 pts. 2 operatio ns 3 pts. Allthough a benign condition, lymphangioma 3 r!lerations 1 pt. can be puzzling in regard to the histology and 4 operations 2 pts. the radical treatment. Especially large, in­ ftltrating forms in the head and neck region of N:: "lber of operatio ns per pat ient infants, represent a challenge for radical cure without morbidity. We arc convinced that an eclectic attitude comprising early radical F ~r large lymphangiomas a multistage proce­ excision in moderate forms or non cervical dure can be preferable.