SciELO - Scientific Electronic Library Online

 
vol.10 número4Comportamiento clínico y radiológico de 290 implantes con superficie tratada mediante un primer grabado de ácido fluorhídrico y un segundo de ácido fluorhídrico con ácido nítrico: valoración a los dos años de carga precozAdenocarcinoma Polimorfo de Bajo Grado de fosa nasal índice de autoresíndice de materiabúsqueda de artículos
Home Pagelista alfabética de revistas  

Medicina Oral, Patología Oral y Cirugía Bucal (Ed. impresa)

versión impresa ISSN 1698-4447

Med. oral patol. oral cir. bucal (Ed.impr.) vol.10 no.4  ago./oct. 2005

 

OK432 (picibanil) efficacy in an adult with cystic cervicallymphangioma. A case report
Eficacia del OK432 (picibanil) en un linfangioma cervical quístico del adulto.
Caso clínico y revisión de la bibliografía

 

Juan Alonso (1), Luis Barbier (2), Julio Alvarez (1), Laura Romo (1), Jesús C Martín (3), Iciar Arteagoitia (4), Joseba Santamaría (5)

(1) Cirujano Maxilofacial, Facultativo de Área. Servicio de Cirugía Maxilofacial. Hospital de Cruces. Osakidetza. Servicio Vasco de Salud
(2) Cirujano Maxilofacial, Facultativo de Área. Servicio de Cirugía Maxilofacial. Hospital de Cruces. Osakidetza/Servicio Vasco de Salud. 
Profesor Titular del Departamento de Estomatología. Universidad del País Vasco/Euskal Herriko Unibertsitatea
(3) Cirujano Maxilofacial, Facultativo de Área. Servicio de Cirugía Maxilofacial. Hospital de Cruces. Osakidetza/Servicio Vasco de Salud. 
Profesor Asociado del Departamento de Estomatología. Universidad del País Vasco/Euskal Herriko Unibertsitatea
(4) Médico Estomatólogo. Máster en Diseño y Estadística en Ciencias de la Salud. Profesora Asociada del Departamento de Estomatología. 
Universidad del País Vasco/Euskal Herriko Unibertsitatea
(5) Cirujano Maxilofacial, Jefe de Servicio. Servicio de Cirugía Maxilofacial. Hospital de Cruces. Catedrático del Departamento de Estomatología.
Universidad del País Vasco/Euskal Herriko Unibertsitatea.Bilbao.

Address:
Dr. Joseba Santamaría.
Hospital de Cruces. Servicio de Cirugía Maxilofacial.
c/. Plaza de Cruces. 48603 Baracaldo. Bizkaia. Basque Country.
Teléfono hospital: +48%946006469.
E-mail: joseba.santamaria@hcru.osakidetza.net

Received: 28-05-2004 Accepted: 10-10-2004

Alonso A, Barbier L, Alvarez J, Romo L, Martín JC, Arteagoitia I, Santamaría J.OK432 (picibanil) efficacy in an adult with cystic cervical lymphangioma. A case report. Med Oral Patol Oral Cir Bucal 2005;10:362-6.
© Medicina Oral S. L. C.I.F. B 96689336 - ISSN 1698-4447

ABSTRACT

Cervical cystic lymphangioma (CCL) is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels. The most accepted treatment continues to be surgical excision. However, when this infiltrates vital neurovascular neck structures, complete excision is difficult and if only partial, the recurrence rate is very high. The most frequently used alternative treatment is to inject sclerosants into the lesion. The use of these techniques has reported good results in children; however, there are few references thereof with regard to adults. We are reporting on a cervical cystic lymphangioma in a male aged 22, treated with an intra-lesion injection of 20 cc with 0.01 mg/cc dilution of OK-432 (picibanil) in physiological serum. Sole complications were fever and local reaction where the solution was injected. One month after treatment the lymphangioma had totally remitted and sixteen months later continues in remittance.

Palabras clave: OK432 (picibanil), Cervical cystic linphangioma, adult, efficacy.

RESUMEN

El linfangioma cervical quístico (LCQ) es una tumoración rara y benigna provocada por anormalidades congénitas o adquiridas de los vasos linfáticos. La escisión quirúrgica sigue siendo el tratamiento más aceptado. Sin embargo su completa escisión puede ser imposible si afecta a importantes estructuras neuro-vasculares del cuello. Si la extirpación es parcial su recurrencia es muy alta. El procedimiento alternativo más utilizado habitualmente, es la inyección de esclerosantes dentro de lesión. Con los esclerosantes se han comunicado buenos resultados en niños y sin embargo son muy pocas las referencias en adultos. Nosotros reportamos el tratamiento quirúrgico de un LCQ, en un varón de 22 años que fue tratado, mediante inyección intralesional de 20 cc de una de una dilución 0.01 mg/cc de OK-432 (picibanil) en suero fisiológico. Fiebre, eritema con dolor en el lugar de la inyección fueron las únicas complicaciones. Un mes mas tarde el LCQ remitió y 18 meses después continúa sin recidiva.

Palabras clave: OK432 (picibanil), Linfangioma cervical quístico, adulto, eficacia.

 

INTRODUCTION

Cervical cystic lymphangioma (CCL) is a rare and benign tumour involving congenital and cystic abnormalities derived from lymphatic vessels (1,2). Usually found in children (3-9) it is rare in adults (10-13).

90% of cystic lymphangiomas are located in the cervical-facial region, whose clinical presentation is a progressive and painless growth, although occasionally growth may be sudden (13).

Surgical excision continues to be the most widely accepted treatment (3,14) However, when this infiltrates vital neurovascular neck structures, complete excision is difficult, and if only partial the recurrence rate is very high, between 15%-53% (15). In addition surgical complications (infections, fistules, anti-aesthetic scars, etc) are frequent, between 19%-33% of the cases (5). Among treatments alternative to surgery (4,16-19) the most widely experimented on in children is to inject the lesion itself with sclerosant OK-432 (Picibanil) (4-7,9,15,20-23). OK432 is obtained from a lyophilized mixture of Group A pyogenes streptococcus, which after incubation with penicillin G loses its antigenicity and behaves like a sclerosant due to its immunomodulator activity. Although its efficacy in children has been reported, little is known in relation to adults. The response is more efficient in macrocystic lymphangiomas than in the microcystic (4,5,15,22).

We present a macrocystic CCL clinical case in an adult of unknown aetiology, treated efficiently with an 20 ml 0.01 mg/ml dilution of OK-432 in physiological serum according to the Oguita procedure (6). One month after treatment the lymphangioma had totally remitted. Fourteen months later the patient continues free of tumour.

CASE REPORT

Man aged 22 who had had an asymptomatic mass with progressive growth for the last six months in the right supraclavicular region. (Fig 1). He had no previous injuries, surgical interventions or respiratory infection in the last year. Furthermore, he was not allergic to penicillin or any of its derivatives.

On palpation a supraclavicular mass of 8.5 x 7 cms. was appreciated in the posterior cervical triangle, fluctuant, neither pulsating nor painful.

The cervical echography reported depressible multicystic tumoration of 9.1 x 5.6 cms., partially covered by the sternocleidomastoid muscle and located in front of the internal jugular vein.

The computed tomography (CT) reported a polylobulated cystic mass of 9.5 x 6.8 x 5 cms., whose most cranial position was located behind the right sternocleidomastoid, whereas the caudal portion stretched below the clavicle, adjacent to the deep jugular vein (Fig 2). Fine needle aspiration cytology (FNAC) reported the presence of yellowish serous liquid with mature lymphocytes without malignant cells corresponding to a lymphangioma.

With the cystic lymphangioma diagnosis, the different therapeutical options and possible side effects were explained to the patient. The patient authorised treatment with OK-432, on the informed consent form approved by the Hospital Ethics Committee. The patient was hospitalised for 1 day.

Treatment was carried out under local anaesthesia. After disinfecting the skin with povidone iodine, 1.8 cc. of articaine with epinephrine infiltrated locally. First of all, we punctured the tumours and sucked out 110 cc. of the liquid content, after which we injected the lesion with 20 cc of 0.01 mg/cc of OK-432 diluted in physiological serum.

During immediate post-operation the patient presented a general clinical syndrome with 39.5º fever, moderate pain in the area injected, leucocytosis and left deviation, resolved on administering magnesium metamizol.

The anatomopathological study confirmed the macrocystic type lymphangioma diagnosis.

One month later the tumour had clinically disappeared and the control cervical CT at three months confirmed there was no evidence of lesion (Fig 3). The situation remains unchanged today 16 months after treatment.

DISCUSSION

We report the complete regression of CCL with OK-432 in an adult male. Although a larger number of cases had been reported in children3-9,15 basically due to its congenital character, CCL cases treated with complete regression with OK-432 in adults are few OK-432 (20,22).

There are three histological lymphangioma subtypes: capillary (comprising fine capillary walls), cavernous (comprising dilated and adventitia lymphatic spaces) and cystic or cystic hygroma (comprising cysts delimited by an endothelial layer of variable size) (2). Although the three types frequently co-exist in a single lesion, the histological type relates to the tissue where it settles. The cavernous lymphangioma is more frequent where muscle and fibrous tissue predominate as in the tongue, floor of the mouth and cheek. Cystic lymphangioma is present in territories with predominance of fascial planes and lax tissue such as the neck, where it can expand and create large cystic spaces (1,2) Depending on cystic space size, they are classified: macrocystic, microcystic and mixed (2,4,15,22).

Very frequently the CCL appears in the posterior cervical triangle as in our case (3,14,21).

Diagnosis in adults is rare.10-13 In adults, the sudden appearance is frequently related to traumatic history and acute infectious processes in the upper respiratory tract (10-13). In our case there was no history of this nature, therefore we consider the aetiology unknown.

The patient’s clinical symptomatology was the most common in adult cystic lymphangioma, i.e. with progressively growing mass, fluctuant, multiocular, soft, easily compressible and painless. Dysphagia and respiratory tract obstruction is more common in children particularly if pharyngeal territory is invaded (3).

We performed imaging diagnosis via the most commonly used techniques to determine depth and extension. We included thoracic study in the CT to discard any possible extension to the mediastinum.

FNAC is important for diagnosis and in our case the result coincided with the final anatomopathological report of macrocystic lymphangioma.

CCL treatment is not fully standardised. Many surgeons have considered the surgical excision as the first therapeutical option.4 When the excision is complete, reappearance is infrequent.1,4 However, when the lymphangioma infiltrates important vascular and nerve structures, complete excision of the same is difficult and in some cases reappearance is as high as 50% (4,14,15). Moreover, the complication and sequela percentage after surgery is very high. Several surgical interventions are usually necessary in very extensive lymphangiomas to obtain satisfactory results (14).

Among alternative treatments total and partial remissions have been reported with the use of intra-lesion sclerosant substances like fibrin sealant (16), triamcinolone (17), bleomicina (18), and hydrocolloid dental impression material (19). The reduced sample size of these sclerosants does not provide a great deal of evidence.

Ogita (6) developed the lymphangioma treatment with the OK-432 intra-lesion injection, which is the most experimented sclerosant in children for treating CCL (1-9).

OK-432 is produced by lyophilisation of the non-virulent strain Su of the pyogenes streptococcus from group A, treated with penicillin G, provoking production inhibition of streptolisin S. The action causes induction and production of macrophages, lymphocytes, NK cells, interleukins (1-2-6) and tumoral necrosis factor, which would increase permeability in the lymphangioma endothelium, accelerating lymph drainage, and emptying of the cystic spaces. Another action mechanism suggested is apoptosis of the lymphatic vessel endothelial cells (17,21).

The OK-432 effect is sclerosant and immunomodulator, and the amplitude of the sclerosant response is believed to be related to cystic lymphatic spaces (20). In the macrocystic or mixed regression is complete or very marked in 86%-100% of the cases (15,22). The microcystic type continues to be object of debate, and although the result is not so efficient, its use is recommended as first line treatment prior to surgery (4,7). In our case it was a macrocystic cervical lymphangioma.

Febrile syndrome and leucocytosis with left deviation occurred immediate post-operation, which was controlled in the days following with magnesium metamizol. This is the most common complication together with a local inflammatory reaction in the injection area (4,7). Possible hypersensitivity reactions must be controlled and hospital vigilance during day one is convenient.

In our case a single session of one 20 ml injection, immediately having aspirated 110 cc of the lymphangioma contents via puncture of the lesion, was sufficient to complete regression. One month after treatment the lymphangioma had totally remitted and sixteen months later the lesion continued in total remission. Although the recommended volume for the first injection must not exceed 20 cc, the maximum amount of OK-432 in the event of several injections being necessary has yet to be determined.

The best results in cervical cystic lymphangioma treatment with OK-432 have been reported in children, however, our experience in this clinical case, also indicates first line treatment in an adult is sclerosis using OK-432.

REFERENCES

1. Kennedy TL, Whitaker M, Pellitteri P, Wood WE. Cystic Hygroma/Lymphangioma: a rational approach to management. Laryngoscope 2001;111:1929-37.        [ Links ]

2. Kennedy TL. Cystic Hygroma/Lymphangioma: a rare and still unclear entity. Laryngoscope 1989;99:1-10.        [ Links ]

3. Charabi B, Bretlau P, Bille M, Holmelund M. Cystic hygroma of the head and neck- a long term follow- up of 44 cases. Acta Otolaryngol 2000; 543:248-50.        [ Links ]

4. Sanlialp I, Karnak I, Tanyel FC, Senocak ME, Buyukpamukcu N. Sclerotherapy for lymphangioma in children. Int J Pediatr Otorrhinolaryngol 2003;67:795-800.        [ Links ]

5. Smith R, Burke D, Sato Y, Poust RI, Kimura K, Bauman NM. OK-432 therapy for lymphangiomas. Arch Otolaryngol Head Neck Surg 1996;122:1195-9.        [ Links ]

6. Ogita S, Tsuto T, Tokiwa K, Takahashi T. Intracystic injection of OK-432: a new sclerosing therapy for cystic hygroma in children. Br J Surg 1987;74:690-1.        [ Links ]

7. Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N. OK-432 therapy for lymphangiomas in children: why and how does it work?. J Pediatr Surg 1996;31:477-80        [ Links ]

8. Mulliken JB, Glowacki J. Haemangiomas and vascular malformations in infants and children: A classification based on endothelial characteristics. Plast Reconstr Surg 1982;69:412-23.        [ Links ]

9. Banieghbal B, Davies MRQ. Guidelines for the successful treatment of lymphangioma with OK-432. Eur J Pediatr Surg 2003;13:103-7.        [ Links ]

10. Gleason TJ, Yuh WT, Tali ET, Harris KG, Mueller DP. Traumatic cervical cyst lymphangioma in an adult. Ann Otol Rhinol Laryngol 1993;102:564-6.        [ Links ]

11. Wiggs WJ, Sismanis A. Cystic hygroma in the adult: two cases report. Otolaryngol Head Neck Surg 1994;110:239-41.        [ Links ]

12. Morley SE, Ramesar KC, Mc Leod DA. Cystic hygroma in an adult: a case report. J R Coll Surg Edimb 1999;44:57-8.        [ Links ]

13. Antoniades K, Kiziridou A, Psimopoulou M. Traumatic cervical cystic hygroma. Int J Oral Maxillofac Surg 2000;29:47-8.        [ Links ]

14. Riechelmann H, Muehlfay G, Keck T, Mattfeldt T, Rettinger G. Total, subtotal, and partial surgical removal of cervicofacial lymphangiomas. Arch Otolaryngol Head Neck Surg 1999;125:643-8.        [ Links ]

15. Luzzatto C, Midrio P, Tchaprassian Z, Guglielmi M. Sclerosing treatment of lymphangiomas with OK-432. Arch Dis Child 2000;82:316-8.        [ Links ]

16. Castanon M, Margarit J, Carrasco R, Vancells M, Albert A, Morales L. Long term follow-up of nineteen cystic lymphangiomas treated with fibrin sealant. J Pediatr Surg 1999;48%:1276-9.        [ Links ]

17. Farmand M, Kuttemberg J. A new therapeutic concept for the treatment of cystic hygroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1996;81:389-95.        [ Links ]

18. Zhong PQ, Zhi FX, Li R, Xue JL, Shu GY. Long-term results of intratumorous bleomycin-A5 injection for head and neck lymphangioma. Oral Surg Oral Med Oral Pathol 1995;82:139-44.        [ Links ]

19. Katsuno S, Ezawa S, Minemura T. Excision of cervical cystic lymphangioma using injection of hydrocolloid dental impression material. J Oral Maxillofac Surg 1999;28:295-6.        [ Links ]

20. Rautio R, Keski-Nisula L, Laranne J, Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil). Cardiovasc Intervent Radiol 2003;26:31-6.        [ Links ]

21. Sung MW, Lee DW, Kim DY, Hwang CH, Park SW, Kim KH. Sclerotherapy with picibanil (OK-432) for the congenital lymphatic malformation in the head and neck. Laryngoscope 2001;111:1430-3.        [ Links ]

22. Giguere CM, Bauman N, Sato Y, Burke DK, Greinwald JH, Pransky S et al. Treatment of lymphangiomas with OK-432 (Picibanil) sclerotherapy: a prospective multi-institutional trial. Arch Otolaryngol Head Neck Surg 2002;128:1137-44.        [ Links ]

23. Claesson G, Kuylenstierna R. OK-432 therapy for lymphatic malformation in 32 patients (28 children). Int J Pediatr Otolaryngol 2002;65:1-6.        [ Links ]

Creative Commons License Todo el contenido de esta revista, excepto dónde está identificado, está bajo una Licencia Creative Commons