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Percutaneous Sclerotherapy With OK-432 of a Cervicomediastinal Lymphangioma

2015; Elsevier BV; Volume: 100; Issue: 5 Linguagem: Inglês

10.1016/j.athoracsur.2014.10.020

1552-6259

Gloria Golinelli, Andrea Toso, Giovanni Borello, Paolo Aluffi, Francesco Pia,

Tumors and Oncological Cases

The present study reports a case of percutaneous sclerotherapy of a giant cystic cervicomediastinal lymphangioma using OK-432. To the best of our knowledge, percutaneous sclerotherapy of a mediastinal lymphangioma using OK 432 has not previously been reported in the English literature. The present study reports a case of percutaneous sclerotherapy of a giant cystic cervicomediastinal lymphangioma using OK-432. To the best of our knowledge, percutaneous sclerotherapy of a mediastinal lymphangioma using OK 432 has not previously been reported in the English literature. Mediastinal lymphangioma, a rare congenital development anomaly, accounts for 0.7% to 4.5% of all mediastinal tumors [1Oshikiri T. Morikawa T. Jinushi E. Kawakami Y. Katoh H. Five cases of the lymphangioma of the mediastinum in adult.Ann Thorac Cardiovasc Surg. 2001; 7: 103-105PubMed Google Scholar]. Lymphangiomas are commonly present in the head and neck areas and detected in 90% of cases by the end of the second year, whereas mediastinal localization accounts for less than 1% and is mostly asymptomatic and found accidentally in adulthood. The management of lymphangioma remains a challenge. Surgical excision has traditionally been the first line of treatment, but in cases of mediastinal localization, the close proximity of vital structures can lead to incomplete excision and recurrence. More recently the use of OK-432 (Picibanil; Chungai Pharmaceuticals Co, Tokyo, Japan) has gained popularity, especially in the management of macrocystic lesions [2Okazaki T. Iwatani S. Yanai T. et al.Treatment of lymphangioma in children: our experience of 128 cases.J Pediatr Surg. 2007; 42: 386-389Abstract Full Text Full Text PDF PubMed Scopus (173) Google Scholar]. The aim of this study is to report a case of cervicomediastinal lymphangioma treated with OK-432. A 43-year-old woman was admitted to the ENT Division of the University of Piemonte Orientale for a right-side supraclavicular mass present for approximately a year with accelerated growth during recent months. The mass was painless, mobile, and of soft elastic consistency. Ultrasonography of the neck showed a bulky cystic lesion 10 × 13 cm, extending from the neck to the thoracic cavity deep behind the clavicle. A Doppler ultrasound study showed no vascular flow inside the mass. Magnetic resonance imaging showed a bulky oval cystic lesion lying between the sternocleidomastoid muscle and the upper mediastinum that was hypodense in T1 sequences and homogeneously hyperdense in T2, suggesting a cervicomediastinal lymphangioma (Figs 1A-1B). A transcutaneous sclerotherapy with OK-432, was scheduled. Injection was performed with ultrasound guidance under local anesthesia. After aspiration of fluid from the lesion (more than 280 mL) with a 14-gauge needle, 20 mL of OK-432 at a concentration of 0.01 mg/mL was introduced. No collateral effects after the procedure were reported. The liquid aspirated was sent for cytologic examination to confirm the diagnosis of lymphangioma. After 46 months the patient is in good condition, and magnetic resonance imaging follow-up demonstrated no evidence of recurrence (Fig 2). The exact incidence of mediastinal lymphangioma is unknown, but its prevalence is estimated to be 0.01% to 4.5% of mediastinal tumors based on various retrospective surgical and autopsy reports [3Park J.G. Aubry M.C. Godfrey J.A. Midthun D.E. Mediastinal lymphangioma: Mayo Clinic experience of 25 cases.Mayo Clin Proc. 2006; 81: 1197-1203Abstract Full Text Full Text PDF PubMed Scopus (54) Google Scholar]. Lymphangioma neither becomes malignant nor has a familial tendency [4Alqahtani A. Nguyen L.T. Flageole H. Shaw K. Laberge J.M. 25 years’ experience with lymphangiomas in children.J Pediatr Surg. 1999; 34: 1164-1168Abstract Full Text PDF PubMed Scopus (312) Google Scholar]. Generally they grow slowly, and spontaneous regression occurs in only about 6% of cases. As their size increases, they may cause cosmetic impairment or interfere with breathing and swallowing. History and physical examination usually suggest the diagnosis of lymphangioma, computed tomography scan and ultrasonography are helpful, and magnetic resonance imaging is the most useful modality to assess diagnosis. Cytology on the aspirated fluid can confirm diagnosis. Surgical excision of localized lymphangioma has been the treatment of choice, but the nature and the insinuating growth of the lesion often make it difficult to achieve a complete resection, with a high risk of recurrence. It is widely accepted that OK-432 therapy is effective for lymphangioma, especially in the management of macrocystic lesions. OK-432 is a lyophilized biologic preparation containing the cells of Streptococcus pyogenes Su-strain treated with benzylpenicillin. It was originally developed as an immunotherapeutic agent for cancer. The first results of intralesional injection of OK-432 as treatment for lymphangioma were reported in 1987 [5Ogita 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: 690-691Crossref PubMed Scopus (260) Google Scholar]. When injected into the cystic spaces it produces sclerosis that does not spread outside the lesion. The main advantage of OK-432 compared with other sclerosing agents is the absence of perilesional fibrosis [2Okazaki T. Iwatani S. Yanai T. et al.Treatment of lymphangioma in children: our experience of 128 cases.J Pediatr Surg. 2007; 42: 386-389Abstract Full Text Full Text PDF PubMed Scopus (173) Google Scholar]. OK-432 causes inflammatory cells to invade the cyst and stimulates immunologic cells to secrete a variety of cytokines, including interleukin 6, tumor necrosis factors, and interferon-γ, increasing the permeability of the endothelium and resulting in contraction of the cyst. In addition, OK-432 enhances cyst contraction by increasing the activities of natural killer cells and the number of T lymphocytes. Recently the use of OK-432 has gained popularity, and the outcome of sclerotherapy might be more dependent on the type of lymphangioma rather than the location. Single cystic and macrocystic lesions, as often occur in mediastinal lymphangioma, showed the best response to therapy [2Okazaki T. Iwatani S. Yanai T. et al.Treatment of lymphangioma in children: our experience of 128 cases.J Pediatr Surg. 2007; 42: 386-389Abstract Full Text Full Text PDF PubMed Scopus (173) Google Scholar]. Recurrence of the disease after OK-432 therapy is unusual [6Rautio R. Keski-Nisula L. Laranne J. Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil).Cardiovasc Intervent Radiol. 2003; 26: 31-36Crossref PubMed Scopus (65) Google Scholar]. Failure of sclerosing therapy may be caused by insufficient contact between OK-432 and the inner surface of the cyst, as frequently can occur in multilocular cysts or cysts containing thick fluid. Side effects of sclerotherapy are usually poor. A potentially serious side effect can be compression of the surrounding structures owing to acute swelling of the lymphangioma after OK-432 injection. In the present case no side effects were observed. There is no published evidence regarding the optimal dose of OK-432 to be injected for giant mediastinal lymphangioma. In this case the required dose of OK-432 was adjusted to the volume and the site of the lesion. Rautio and colleagues [6Rautio R. Keski-Nisula L. Laranne J. Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil).Cardiovasc Intervent Radiol. 2003; 26: 31-36Crossref PubMed Scopus (65) Google Scholar] injected the same volume of OK-432 that was aspirated from the cystic space in cervical lymphangiomas until a maximum of 10 mLwas injected. Okazaki and associates [2Okazaki T. Iwatani S. Yanai T. et al.Treatment of lymphangioma in children: our experience of 128 cases.J Pediatr Surg. 2007; 42: 386-389Abstract Full Text Full Text PDF PubMed Scopus (173) Google Scholar] injected a maximum of 20 mL of OK-432 solution after aspiration of fluid from each lesion (as proposed in the label for Picibanil). The only sedation needed for adults is local anesthesia without preventive medication, especially antibiotics because they would have suppressed the effect of the treatment. Nerve injuries and cosmetic problems are avoided. The injections of OK-432 were performed with ultrasound guidance only after cytologic analysis of fine-needle biopsy and aspiration achieved the diagnosis of lymphatic cyst. Desir and coworkers [7Desir A. Ghaye B. Duysinx B. Dondelinger R.F. Percutaneous sclerotherapy of a giant mediastinal lymphangioma.Eur Respir J. 2008; 32: 804-806Crossref PubMed Scopus (13) Google Scholar] previously performed a successful transcutaneous sclerotherapy with Ethibloc of a giant mediastinal lymphangioma. To the best of our knowledge, only one other case of sclerotherapy of mediastinal lymphangioma, using OK-432, has been reported in the Japanese literature [7Desir A. Ghaye B. Duysinx B. Dondelinger R.F. Percutaneous sclerotherapy of a giant mediastinal lymphangioma.Eur Respir J. 2008; 32: 804-806Crossref PubMed Scopus (13) Google Scholar]. Lymphangioma is a benign lesion, so the aim of the treatment could be the reduction of the mass until the patient becomes asymptomatic, as occurred in our case; otherwise, attempting to achieve a complete removal can damage the surrounding structures. The real problem of the sclerosing therapy is to allow a definitive diagnosis of the mass before treatment is initiated; in the case of lymphangioma, the diagnosis is radiologic (by magnetic resonance imaging) and cytologic. Some authors limit the use of sclerosing agents because of the possibility of spreading outside the thin-walled lesion and causing damage to the surrounding structures, making subsequent surgery difficult. It has been demonstrated that surgical excision is considered not to be more difficult after OK-432 therapy [2Okazaki T. Iwatani S. Yanai T. et al.Treatment of lymphangioma in children: our experience of 128 cases.J Pediatr Surg. 2007; 42: 386-389Abstract Full Text Full Text PDF PubMed Scopus (173) Google Scholar, 6Rautio R. Keski-Nisula L. Laranne J. Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil).Cardiovasc Intervent Radiol. 2003; 26: 31-36Crossref PubMed Scopus (65) Google Scholar]. However, it has been reported that incomplete surgical resection as first-line therapy impaired successive treatment with OK-432 [6Rautio R. Keski-Nisula L. Laranne J. Laasonen E. Treatment of lymphangiomas with OK-432 (Picibanil).Cardiovasc Intervent Radiol. 2003; 26: 31-36Crossref PubMed Scopus (65) Google Scholar]. Long-term effects of OK-432 remain unknown [4Alqahtani A. Nguyen L.T. Flageole H. Shaw K. Laberge J.M. 25 years’ experience with lymphangiomas in children.J Pediatr Surg. 1999; 34: 1164-1168Abstract Full Text PDF PubMed Scopus (312) Google Scholar]. Some authors report that initial and long-term response rates were equally good (83.5% and 76.3%, respectively) [8Yoo J.C. Ahn Y. Lim Y.S. et al.OK-432 sclerotherapy in head and neck lymphangiomas: long-term follow-up result.Otolaryngol Head Neck Surg. 2009; 140: 120-123Crossref PubMed Scopus (47) Google Scholar]. Because sclerotherapy induces cyst collapse, long-term follow-up is required to detect recurrences. Sclerotherapy for mediastinal lymphangioma is little known, although well documented in cervical lymphangioma. We report a case of cervicomediastinal lymphangioma treated by OK-432 achieving the shrinking of the mass until it becomes asymptomatic without complications. If a lymphangioma recurs, repeated sclerotherapy can be performed. The previous sclerosing treatment did not impair the surgical resection if at any time it should be planned.