Marlon A. Guerrero, Chief Resident, Michael E. DeBakey Department of Surgery; Mufaddal M. Gomberawalla, Medical Student IV; Mary L. Brandt, Associate Professor, Michael E. DeBakey Department of Surgery; F.Charles Brunicardi, Chairman and Program Director, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX
Marlon A. Guerrero, MD
Michael E. DeBakey
Department of Surgery
Mufaddal M. Gomberawalla, BS
Medical Student IV
Mary L. Brandt, MD
Michael E. DeBakey
Department of Surgery
F. Charles Brunicardi, MD
Chairman and Program
Michael E. DeBakey
Department of Surgery
Baylor College of
Introduction: Lymphangiomas of the head and neck are sometimes referred to as cystic hygromas and result from abnormal growth of the lymphatic vessels. Lymphangiomas are most commonly found in children and rarely observed in adults. Most lymphangiomas are congenital, but they have also been reported to arise from infections, neoplasms, trauma, and iatrogenic injuries.
Results and discussion: The authors report the case of an adult woman with a slowly enlarging neck mass that was resected and identified by pathology as a lymphangioma. She had a lymphangioma excised from the same location more than a decade earlier. The literature is reviewed and the classification, symptoms, diagnosis, and treatment of lymphangiomas are discussed.
Conclusion: Observation is recommended for small, nonexpanding lesions. Surgical resection is recommended for lesions that persist, enlarge, or produce obstructive symptoms. Careful surgical planning is critical to avoid injury to adjacent neurovascular structures, and a multidisciplinary approach is warranted.
Lymphangiomas are benign lymphatic malformations that occur most commonly in children.1 Some authors refer to these lesions as cystic hygromas, a term first used by Wernher in 1843.2 The term cystic hygroma refers to the cystic nature of these lesions and their mass effect; hygroma means "moist tumor."2 Lymphangioma, however, is the more appropriate nomenclature because it denotes the tissue of origin.3 Lymphangiomas are thought to arise from an embryologic defect that consists of an abnormal connection from the jugular sacs to the peripheral lymphatic system.4
The etiology of these lesions has been debated, but it centers predominantly on two theories. The centrifugal theory proposes an abnormal sequestration of primitive lymphatic buds instead of the endothelial channels that proliferate centrifugally from the venous system to become the lymphatic system.5 The centripetal theory discusses aberrant congenital lymphatic development.6 The end result for both theories is the inability of the peripheral lymphatics to drain into the jugular sacs. In adults, lymphangiomas may occur from delayed proliferation of cell rests, either spontaneously or in response to infection or trauma.7
A 42-year-old woman presented to the hospital with a slow-growing left neck mass in the same location from which one had been surgically removed over a decade earlier. The original mass was identified when she was 30 years old and monitored for 2 years. After it failed to resolve, she underwent resection of the mass, which was diagnosed as a lymphangioma on histopathology examination. The patient remained asymptomatic for 10 years before noticing a recurrence of the mass.
Upon presenting to our hospital, the patient noted only slight discomfort in her neck, which occurred with movement. She reported experiencing no shortness of breath, dysphagia, dysphonia, or pain. On physical examination, the mass was soft and nonmobile. It extended from below the angle of the mandible to the supraclavicular region by tracking along the anterior border of the sternocleidomastoid muscle. Magnetic resonance imaging (MRI) of the neck revealed a large, left-sided supraclavicular cystic lesion in the base of the neck (Figure 1). There was no enhancing portion, and the lesion measured approximately 7 x 9 x 4 cm.?The trachea was shifted to the right, with no compromise of the patient's airway (Figure 2).
A multidisciplinary approach for surgical resection was undertaken. Because these lesions are typically found in children, this included general and pediatric surgeons. The mass was reached through a 7-cm transverse skin incision along the anterior border of the sternocleidomastoid muscle. Although the lesion was in close proximity to nerves, vessels, and muscles, there was no invasion into these structures, and the mass was circumferentially dissected and removed in its entirety. Final pathology revealed a lymphangioma with a few small, adjacent benign lymph nodes.?The patient tolerated the procedure well and was discharged from the hospital on postoperative day 1 in good condition.
Lymphangiomas are relatively rare congenital lesions that result from abnormal growth of the lymphatic vessels. It is rare for lymphangiomas to make their initial presentation during adulthood, and fewer than 100 such cases have been reported. Approximately 60% of lymphangiomas are present at birth, and up to 90% are detectable by 2 years of age.8 Although more than 90% of lymphatic malformations are clearly congenital, later presentations may occur as a result of trauma, infection, neoplasms, or iatrogenic injuries.1 The pathophysiology of adult lymphangiomas is not clearly understood, but these lesions may occur secondary to induction of dormant rests of embryonic lymphatic tissue that are stimulated to differentiate and grow.
Lymphatic malformations can be categorized as superficial cutaneous lymphangioma, cavernous lymphangioma, cystic hygroma, or diffuse systemic lymphangioma.1,3,9 They are also classified according to size: capillary lymphangiomas contain microscopic cavities, and cavernous lymphangiomas contain cystic cavities that are usually larger than 1 cm.6
Most lymphangiomas are asymptomatic. They have no gender predilection and present as a painless mass that progressively enlarges. Typically, the mass is soft, nontender, and ill-defined. Symptoms may develop when the lymphatic malformation enlarges to where it compresses surrounding tissue. Obstructive symptoms of dysphagia, dyspnea, or emergent airway compromise may develop. Cervical lymphangiomas have not been shown to lateralize to any particular side of the neck.10
Ultrasonography and computed tomography (CT) scanning have been used extensively to evaluate the anatomy of lymphangiomas. On ultrasound examinations, these lymphatic malformations appear as thin-walled, multiseptate, multicystic, hypoechoic masses.11 Lymphangiomas appear as multiloculated cystic lesions on CT scanning. On T2-weighted MRI, lymphangiomas appear isointense to cerebrospinal fluid, whereas their intensity varies on T1-weighted images due to variable protein content.12
Fine-needle aspiration (FNA) is routinely used to evaluate most neck masses, although it is almost never used in the pediatric population. In adults, where the risk of neoplasm is greater, there is no consensus in the literature concerning the use of FNA to diagnose these lesions. FNA findings include small and round lymphocytes with intermingling histiocytes without mitoses or atypia. In adults with compromised airways, FNA may be therapeutic, as well as diagnostic.13 Immunohistochemistry may reveal polyclonal B lymphocytes mixed with T cells.14 Microscopically, these lesions appear as endothelial-lined lymphatic spaces with intervening fibrous tissue and lymphoid aggregates.15
Observation is frequently the first step for small, nonexpanding lesions. Those that persist, continue to grow, or present with obstructive symptoms should be resected. To avoid injuring adjacent neurovascular structures, surgical planning is crucial before resection. Some surgeons prefer to use MRI to facilitate resection because it allows improved anatomical demarcation.1,16 Ultrasonography has limited ability to delineate the anatomical planes of resection or the extent of structural involvement.
Complications of resection include infection, bleeding, hematoma, and postoperative seromas. Injury to facial, hypoglossal, glossopharyngeal, recurrent laryngeal, and lingual nerves has been reported.1,4,17
Complete excision of a cystic hygroma has been shown to have an 81% cure rate. When only part of the lymphatic malformation is excised, there is an 88% recurrence rate.10 Several studies have shown an increase in rates of recurrence, morbidity, and complications for lymphangiomas located in the suprahyoid versus infrahyoid region,1,17,18 whereas one study found no difference in recurrence rates when comparing these locations.5
Charabi and associates conducted a 35-year follow-up on 44 adult patients who were treated surgically for macrocystic lymphatic malformations of the head and neck.18 Fifty percent of these patients had residual or recurrent disease, and 40% had complications involving speech, food intake, breathing, or swallowing. A direct correlation was observed between the extent of the lesion and the number of operations performed, rate of recurrence, and residual disease. Charabi and associates state that nonencapsulated lesions recur more frequently because of their tendency to infiltrate major neurovascular structures.18
Sclerosing agents and radiation therapy have not been shown to play a role in the primary treatment of cystic hygromas. They may be indicated in lymphatic malformations that are macrocystic and not amenable to surgical resection.10,15,19,20 Sclerotherapy with OK-432, a lyophilized, low-virulence SU strain of group A Streptococcus pyogenes, has been suggested as a possible therapy for macrocystic lesions. To date, however, the efficacy of OK-432 has not been proven in prospective, controlled trials.6,20-22
Surgical excision remains the treatment of choice for lymphangiomas. Despite possible complications, operative management can allow for complete resection, which affords lower rates of recurrence when compared with less invasive treatments. In cases that prove to be unresectable at the time of surgery, intraoperative treatment using sclerosing agents may help decrease the rate of recurrence.20-22
The authors have no relationship with any commercial entity that might represent a conflict of interest with the content of this article and attest that the data meet the requirements for informed consent and for the Institutional Review Boards.