Successful Treatment of Macroglossia Due to Lymphatic Malformation With Sirolimus

Abstract

Objective:

To evaluate the effectiveness and safety of sirolimus therapy in a child with macroglossia due to lymphatic malformation.

Methods:

Sirolimus treatment was applied to the patient with an initial dosing of 0.8 mg/m² per dose, administered orally, twice daily at approximately 12-hour intervals.

Results:

After 9 months of sirolimus therapy, there was a nearly complete resolution of lymphatic malformation. The last evaluation was performed 6 months after withdrawal of treatment, and the lesion had almost completely resolved.

Conclusion:

This article presents a novel approach to the treatment of lymphatic malformation of the tongue using sirolimus, which appears to be safe and effective for the management of complex cases.

Keywords

lymphatic malformation macroglossia sirolimus

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References

Griessenauer

Bilal

Kankirawatana

. Lymphatic malformation of the tongue with coexisting intractable epilepsy treated with corpus callosotomy: a case report. Clin Neurol Neurosurg. 2014;117:68-70.

Standring

HG. Section 3. Head and neck posterior triangle. In: Gray’s Anatomy. British ed. London: Churchill Livingstone; 2011:1825-1861.

Nitnaware

Sakhare

Kapre

. Cystic hygroma with extensive tongue involvement. Indian J Otolaryngol Head Neck Surg. 2011;63:89-92.

Hammill

Wentzel

Gupta

. Sirolimus for the treatment of complicated vascular anomalies in children. Pediatr Blood Cancer. 2011;57:1018-1024.

Thottam

Al-Barazi

Madgy

Rozella

. Submucosal resection of a microcystic oropharyngeal lymphatic malformation using radiofrequency ablation. Int J Pediatr Otorhinolaryngol. 2013;77:1589-1592.

Azizkhan

Rutter

Cotton

. Lymphatic malformations of the tongue base. J Pediatr Surg. 2006;41:1279-1284.

Brennan

Miller

Chen

. Lymphangiomas of the oral cavity: a clinicopathologic, immunohistochemical, and electron-microscopic study. J Oral Maxillofac Surg. 1997;55:932-935.

Fageeh

Manoukian

Tewfik

. Management of head and neck lymphatic malformation in children. J Otolaryngol. 1997;26:253-258.

Gallagher

Mahoney

Gosche

. Cystic hygroma in the fetus and newborn. Semin Perinatol. 1999;23:341-356.

10.

Boardman

Cochrane

Roebuck

. Multimodality treatment of pediatric lymphatic malformations of the head and neck using surgery and sclerotherapy. Arch Otolaryngol Head Neck Surg. 2010;136:270-276.

11.

Goswami

Singh

Gokkulakrishnan

Singh

. Lymphangioma of the tongue. Natl J Maxillofac Surg. 2011;2:86-88.

12.

Neville

Damm

Allen

Bouqout

. Soft tissue tumors. In: Neville

, ed. Oral and Maxillofacial Pathology. 2nd ed. New Delhi: Saunders Publishers; 2002:475-477.

13.

Fliegelman

Friedland

Brandwein

Rothschild

. Lymphatic malformation: predictive factors for recurrence. Arch Otolaryngol Head Neck Surg. 2000;123:706-710.

14.

Marx

Stern

. Oral and Maxillofacial Pathology: A Rationale for Diagnosis and Treatment. Carol Stream, IL: Quintessence Publishing Co, Inc; 2003:435-437.

15.

Karmady

Fortson

Calcaterra

. Lymphangiomas of the head and neck in adults. Otolaryngol Head Neck Surg. 1982;90:283-288.

16.

Vignot

Faivre

Aguirre

. mTOR-targeted therapy of cancer with rapamycin derivatives. Ann Oncol. 2005;16:525-537.

17.

Guba

von

Steinbauer

. Rapamycin inhibits primary and metastatic tumor growth by antiangiogenesis: involvement of vascular endothelial growth factor. Nat Med. 2002;8:128-135.

18.

Perry

Banyard

McLaughlin

. AKT1 overexpression in endothelial cells leads to development of cutaneous vascular malformations in vivo. Arch Dermatol. 2007;143:504-506.

19.

Reinglas

Ramphal

Bromwich

. The successful management of diffuse lymphangiomatosis using sirolimus: a case report. Laryngoscope. 2011;121:1851-1854.

20.

Akyüz

Ataş

Varan

. Treatment of a tongue lymhangioma with sirolimus after failure of surgical resection and propranolol. Pediatr Blood Cancer. 2014;61:931-932.