sign in sign up
<<
Home , Lymphangioma

case reports 2018; 4(1)

https://doi.org/10.15446/cr.v4n1.67067 CERVICAL LYMPHANGIOMA IN ADULTS: CASE REPORT AND CURRENT TREATMENT

Keywords: Adult; Lymphangioma, Cystic; ; Picibanil. Palabras clave: Adulto; Linfangioma quístico; Escleroterapia; Picibanil.

Enrique Cadena-Piñeros Head and Neck Unit - Instituto Nacional de Cancerología - Bogotá D.C. - Colombia. Department of - Universidad Nacional de Colombia - Faculty of Medicine - Bogotá D. C. - Colombia. Otolaryngology Unit - Clínica de Marly - Bogotá D.C. - Colombia. Head and Neck Surgery Unit - Clínica de Marly - Bogotá D.C. - Colombia

Andrés Rojas Gutiérrez Head and Neck Unit - Instituto Nacional de Cancerología - Bogotá D.C. - Colombia.

Corresponding author: Enrique Cadena. Head and Neck Unit - Instituto Nacional de Cancerología - Bogotá D.C. - Colombia. E-mail [email protected].

Recibido: 15/08/2017 Aceptado: 18/01/2018 Case reports Vol. 4 No. 1: 61-8

62 RESUMEN static nodes, branchial cysts, thyroglossal cysts and lymphangiomas, among others. Clinically Introducción: Los tumores quísticos del cue- speaking, lymphangiomas are slow-growing soft llo son inusuales en los adultos. Sin embargo, masses located in different spaces of the neck. se pueden encontrar metástasis a ganglios, quistes branquiales, quistes tiroglosos, linfan- Case report: This paper reports the case of giomas, entre otros. Clínicamente, estos últimos a 36-year-old woman presenting with lymph- son masas blandas de crecimiento lento que se , who consulted due to a right lateral localizan en diferentes espacios del cuello. mass in the neck of 20 days of evolution with- out associated systemic symptoms. Although Reporte de caso: Se reporta un caso de lin- relapse is frequent, the patient was success- fangioma en una mujer de 36 años, quien con- fully treated with surgery, without evidence of sultó por masa lateral derecha del cuello con recurrence at 12 months of follow-up. evolución de 20 días sin síntomas sistémicos asociados. A pesar de que la recaída es fre- Discussion: When cystic tumors of the neck cuente, la paciente fue tratada con cirugía exi- occur in children, surgical urgencies may arise tosa sin evidencia de recidiva durante 12 me- due to obstruction of the airway. However, ses de seguimiento. lymphangioma in adults only produce contour deformity and rarely require urgent interven- Discusión: Cuando se presentan en niños, tion, which allows for conservative manage- los tumores quísticos del cuello se pueden ment such as observation, repeated drainage convertir en urgencias quirúrgicas debido a or sclerotherapy that can be done using OK- obstrucción de la vía aérea; no obstante, en 432 (Picibanil). Nevertheless, surgery remains los adultos solo producen deformidad de a good treatment option, but some complica- contorno y rara vez requieren una intervención tions may occur. apremiante, lo que permite conductas conser- vadoras como la observación, el drenaje repe- Conclusion: Different treatment options were tido o la escleroterapia. Esta última puede ha- reviewed, which led to conclude that surgical cerse con el OK-432 (Picibanil); sin embargo, resection of lymphangiomas continues to be a la cirugía es una buena opción de tratamiento good treatment for this complex neck lesion. sin estar exenta de complicaciones.

Conclusión: Se realizó revisión de las dife- INTRODUCTION rentes opciones de tratamiento y se concluyó que la resección quirúrgica de los linfagiomas Cystic lesions of the neck are rare and difficult continúa siendo la opción más adecuada para to interpret for clinicians, since they can be be- el manejo de esta compleja lesión del cuello. nign or malignant pathologies. The most com- mon location is the posterior triangle, where ABSTRACT inflammatory, metastatic adenopathies or lymphoproliferative diseases can occur. In ad- Introduction: Cystic tumors of the neck are dition, congenital malformations such as bran- rare in adults. Some of them include meta- chial cysts, and lymphangiomas cervical lymphangioma in adults

may be observed in this area. (1) Branchial cleft a review of the subject is presented with a 63 cysts and hemangiomas are easily diagnosed description of the different treatment options. in children under 2 years of age due to their clinical characteristics. These lesions are ob- CASE PRESENTATION served as a mass that usually does not involve inflammatory changes and presents a slow and A 36 year-old female patient, Caucasian, from painless growth or lymphangiomas, which are the city, consulted due to a right lateral mass rare after the third decade of life. (2-5) in the neck of 20 days of evolution without as- The current trend to treat lymphangiomas sociated systemic symptoms, dysphonia, dys- is different; conservative interventions such phagia or pain. No personal or family history of as sclerotherapy, now performed with new importance in relation to the current disease substances, have relegated surgery to the and no history of neck trauma or infection of background due to the high rate of relapse the head and neck area were described. On or persistence. (4,5) Considering that lymph- physical examination, a right lateral soft mass are rare in adults (4) and that the of 6cm was found, which was tender on pal- patient treated was a 36-year-old woman who pation and with displacement and lateralization underwent a complete surgical resection, of the sternocleidomastoid muscle (Figure 1).

Figure 1. Deformity of the right contour of the neck. A) Anterior aspect of the neck; B) Lateral aspect of the neck. Source: Own elaboration based on the data obtained in the study.

A contrast study of the neck using computed angioma (). Due to the lack of tomography (CT) showed a cystic lesion with diagnostic clarity, a surgery was performed thin walls of 55x21x43mm, which compressed 15 days later. During the procedure, a medial the internal jugular , occupied the right cystic mass was found in the sternocleido- III and IV levels and was clearly delimited by mastoid muscle adhered to the internal jugular vascular and muscular structures (Figure 2). vein and to the phrenic, hypoglossal, spinal Fine needle aspiration (FNA) helped to and vagus nerves. Finally, the lesion was diagnose cyst content with lymphoid tissue completely resected along with the entire lining, which could be lymphocele or lymph- capsule (Figure 3). Case reports Vol. 4 No. 1: 61-8

64

Figure 2. Contrast computed tomography of the neck. A) Thin-walled cyst medial to the sternocleidomastoid muscle; B) Cystic lesion occupying the right III and IV levels. Source: Own elaboration based on the data obtained in the study.

Figure 3. Intraoperative appearance. A) Transverse incision of the neck. A deep lesion of the sternocleidomastoid muscle was observed along with junctions to the internal jugular vein and accessory nerve. B) Lymphangioma was dissected from the internal jugular vein. Source: Own elaboration based on the data obtained in the study.

Figure 4. Surgical specimen: transillumination revealed a thin-walled cyst. Source: Own elaboration based on the data obtained in the study. cervical lymphangioma in adults

Transillumination of the surgical specimen Lymphangiomas correspond to 25% of all 65 revealed a thin-walled, cystic-like mass with vascular tumors in children and adolescents; citrine material content, which correlated with 70% of cases are found in the head and the the diagnosis of preoperative FNA (Figure 4). neck. In 2006, Thompson (11) proposed The final pathology reported a cystic classifying them into cystic (cystic hygroma), mass of 6.9x3.2x3cm, with smooth external capillary or cavernous, but there are different surface and a clear liquid content. Additionally, histological classifications: the smooth inner surface was accompanied Colbert et al. (12) classified them into by an adipose tissue of 2.5x1.8x1cm with macrocysts, formerly known as cystic hy- another tissue of 1.5x0.6x0.5cm. The final gromas, and in microcysts, which may occur diagnosis of the pathology was lymphangioma simultaneously in a lesion. and five reactive lymph nodes, negative for Bhayya et al. (13) classified them in tumor infiltration. The patient did not present lymphangioma simplex, composed of small any complication during the postoperative thin-walled lymphatics; cavernous lymphan- period, nor motor deficit related to the dis- gioma, comprised of dilated lymphatic vessels sected structures, nor evidence of relapse or with surrounding adventitia, cystic lymph- persistence of the lesion after 12 months of angioma, consisting of a large lesion, with follow-up. No complaints were made by the macroscopic lymphatic spaces surrounded by patient, who reported excellent functional and fibrovascular tissue and , and cosmetic results. benign lymphangioendothelioma, lymphatic channels that seem to be dissected through DISCUSSION dense collagen bundles. In 1995, de Serres et al. (14) proposed a Lymphangiomas, also known as cystic hygro- classification according to anatomical location: mas, are congenital malformations that are Stage I, unilateral infrahyoid lesion; Stage II, usually diagnosed at birth and can cause air- unilateral suprahyoid lesion; Stage III, unilat- way obstruction, thus constituting a medical eral suprahyoid and infrahyoid lesion; Stage emergency. (6) These lesions are considered IV, bilateral suprahyoid lesion, and Stage V, vascular anomalies of the bilateral suprahyoid and infrahyoid lesion. and are classified as based on Based on the last classification system, pathology findings. (7) Different etiological the case presented here corresponds to stage and pathogenic factors have been described I (right lateral extension from a horizontal as causal, including traumatic, infectious and plane to the hyoid bone and to the superior chronic inflammatory factors, as well as al- clavicular ridge). This made dissection and terations in embryological development. The complete resection easier, without evidence latter includes sequestration of lymphatic tis- of residual tissue on the surgical bed. sue, defects in the fusion of the venous sys- The diagnostic studies used to determine tem, and lymphatic obstruction that causes its cystic tumors of the neck include high resolution expansion and obstruction. (8) In adults, late ultrasound, which can prove the presence of proliferation of cellular nests of the lymphatic a cystic lesion with multiple septa and without system may appear due to stimulation caused internal vascular flow that allows differentiating by trauma or infection. (9,10) them from mixed vascular lesions. However, Case reports Vol. 4 No. 1: 61-8

66 CT and magnetic resonance imaging (MRI) group A Streptococcus pyogenes incubated determine more precisely the relationship of with penicillin, which is initially used as immu- the mass with the adjacent structures. The notherapy for cancer in cases of pleural and T1 and T2 sequences of the MRI facilitate peritoneal carcinomatosis. (19) the visualization of cleavage planes with the These authors used this preparation in muscles and can define whether it is a vascular 83 patients with benign cystic lesions of the malformation. (12) neck; 12 of them had lymphangiomas, achiev- Fageeh et al. (15) proposed observation, ing complete reduction in 67% and almost percutaneous drainage, carbon dioxide laser complete in 33% of the cases. Only some

(CO2) and Nd-YAG laser, diathermy and patients developed minor complications such surgical resection as management options. as fever between 37.5°C and 38°C for a few Recently, Miceli & Stewart (16) recommended days after the injection, which was controlled sclerotherapy with doxycycline or radiotherapy with antipyretics and without antibiotics. (19) as treatment options. Observation may be Unilateral lesions composed of micro- indicated for patients younger than 3 years cysts, delimited and extended to one or two who do not have obstruction of the airway and compartments of the neck, are candidates for have lesions <4cm, since they could present surgical resection, as they may be completely spontaneous regression. (15,17) resected. However, the lesions located near Surgical resection has traditionally been the floor of the mouth or the parapharyngeal advocated as the best treatment option, but space are more likely to be partially resected. in cases where the lesions extend into deep (6) In this patient, with no family history or neck spaces, as the floor of the mouth or genetic alterations, the lymphangioma was parapharyngeal space, complete removal secondary to interruption of lymphatic drain- may be difficult to achieve without damaging age, probably due to previous neck trauma the nervous and vascular structures. This that went unnoticed. Similar to Gow et al. situation has led to seek alternatives such (6), for this case, surgical resection of the as drainage and sclerotherapy with different mass was decided because the CT showed substances such as tetracycline, bleomycin a single cyst circumscribed to the III and IV and triamcinolone. is right levels of the neck (Figure 2). another therapeutic option to treat this entity. Although, it extended to level III, IV and Vb, (12) Complications and associated morbidity it was possible to remove the mass without can include infections, tissue necrosis, cranial breaking the cysts (Figure 3). Macroscopic nerve lesions, vascular thrombosis and even examination showed that the lesion had a endocrine disorders. (6,15) clear content that allowed transillumination, Ogita et al. (18) described the use of a confirming a lymphangioma (Figure 4). streptococcal preparation OK-432 (Picibanil) as a safe alternative to perform sclerosis in CONCLUSIONS cystic lesions of the neck, considering that this substance produces fibrosis secondary Radiological images such as neck CT, MRI to inflammatory and cicatricial changes with and neck ultrasound with FNA are of great the consequent contraction of the lymphan- help for diagnosing and planning the man- gioma. (18) OK-432 is a lyophilisate made of agement of lymphangiomas of the neck. cervical lymphangioma in adults

67 Clinical suspicion of lymphangioma helps 4. Rolekar NG, Shah DK. Recurrent cystic lym- to establish the treatment for a patient with soft phangioma of the neck in an adult: A case re- mass and radiological images suggestive of port of rare entity. Int J Med Sci Public Health. neck cysts. However, the definitive diagnosis 2014;3(2):243-5. http://doi.org/cj36. is only obtained with the final pathology. 5. Costache A, Dumitru M, Tweedie D, Sara- In adult patients, conservative observa- foleanu C, Anghel I. Adult cervical lymphan- tion treatment or sclerotherapy with different gioma - ultrasonography, surgical removal, and preparations such as picibanil are management pathology results. Case report. Med Ultrason. options, especially when dealing with a com- 2015;17(3):411-3. http://doi.org/cj37. plex lesion extended to the parapharyngeal 6. Gow L, Gulati R, Khan A, Mihaimeed F. space, submandibular space, pterygoid fossa Adult-onset cystic hygroma: a case report and re- or floor of the mouth. view of management. Grand Rounds. 2011;11:5- Surgery is recommended for patients with 11. http://doi.org/c7k5tw. lymphangioma simplex stage I, with single or 7. Rey-Marcos M, Martín-Sánchez V, Cor- multiple cysts that extent to one or two lev- dero-Civantos C, Blanco-Pérez P, Batue- els of the neck, and radiological planes well cas-Caletrío A, Tapia-Risueño M. Linfangioma defined by images. quístico en el adulto. Rev. Soc. Otorrinolaringol. Castilla Leon Cantab. La Rioja. 2012;3(36):291-6. CONFLICT OF INTEREST 8. Wiegand S, Eivazi B, Barth PJ, von Rau- tenfeld DB, Folz BJ, Mandic R, et al. Pa- None stated by the authors. thogenesis of lymphangiomas. Virchows Arch. 2008;453(1):1-8. http://doi.org/c6pjts. FUNDING 9. Suk S, Sheridan M, Saenger JS. Adult lym- phangioma: a case report. Ear Nose Throat J. None stated by the authors. 1997;76(10):881-3. 10. Aneeshkumar MK, Kale S, Kabbani M, REFERENCES David VC. Cystic lymphangioma in adults: can trauma be the trigger? Eur Arch Otorhinolaryn- 1. Brea-Álvarez B, Roldan-Fidalgo A. Quis- gol. 2005;262(4):335-7. http://doi.org/dcms3t. tes en el triángulo cervical posterior en adultos. 11. Thompson L. Lymphangioma. Ear Nose Throat Acta Otorrinolaringol Esp. 2015;66(2):106-10. J. 2006;85(1):18-9. http://doi.org/f2rvqc. 12. Colbert SD, Seager L, Haider F, Evans 2. Derin S, Sahan M, Dere Y, Çullu N, Sa- BT, Anand R, Brennan PA. Lymphatic mal- han L. Cervical Cystic Hygroma in an Adult. formations of the head and neck-current con- Case Reports in Pathol. 2014;2014:209427. cepts in management. Br J O Maxillof Surg. http://doi.org/gb99bt. 2013;51(2):98-102. http://doi.org/cj39. 3. Gaddikeri S, Vattoth S, Gaddikeri RS, Stuart 13. Bhayya H, Pavani D, Avinash-Tejasvi ML, R, Harrison K, Young D, et al. Congenital Geetha P. Oral lymphangioma: A rare case cystic neck masses: embryology and imaging report. Contemp Clin Dent. 2015;6(4):584-7. appearances, with clinicopathological correla- http://doi.org/cj4b. tion. Curr Probl Diagn Radiol. 2014;43(2):55- 14. de Serres LM, Sie KC, Richardson MA. Lym- 67. http://doi.org/cj35. phatic malformations of the head and neck. A pro- Case reports Vol. 4 No. 1: 61-8

68 posal for staging. Arch Otolaryngol Head Neck tum Using Fractional Carbon Dioxide Laser Surg. 1995;121(5):577-82. http://doi.org/dp89pq. Ablation. Pediatr Dermatol. 2013;30(5):584-6. 15. Fageeh N, Manoukian J, Tewfik T, Schloss http://doi.org/f49g5p. M, Williams H, Gaskin D. Management of 18. Ogita S, Tsuto T, Tokiwa K, Takahashi T. In- Head and Neck Lymphatic Malformations in tracystic injection of OK-432: a new sclerosing Children. J Otolaryngol. 1997;26(4):253-8. therapy for cystic hygroma in children. Br. J. 16. Miceli A, Stewart KM. Lymphangioma. In: Surg. 1987;74(8):690-1. http://doi.org/dxjpfb. StatPearls. Treasure Island: StatPearls Publi- 19. Ohta N, Fusake S, Watanabe T, Ito T, Ao- shing; 2017 [cited 2018 Feb 1]. Available from: yagi M. Effects and mechanism of OK-432 https://goo.gl/7iBg5s. therapy in various neck cystic lesions. Acta 17. Shumaker PR, Dela Rosa KM, Krakowski Otolaryngol. 2010;130(11):1287-92. http:// AC. Treatment of Lymphangioma Circumscrip- doi.org/d74qrb.