Abstract
Background: Madelung’s disease is a rare lipodystrophy that presents with multiple fatty masses in the neck, trunk, and upper extremities. The fatty accumulation is considered a benign disease, but compression of the aerodigestive tract may occur in long-standing disease.
Methods: Eight Chinese patients with Madelung’s disease were reviewed. All were male, aged 48 to 67 years, with a history of disease ranging from 4 to 20 years. Two of the eight patients developed aerodigestive symptoms and were subsequently found to have head and neck cancers. These two patients are described.
Results: The possible mechanism that may account for an increase in malignant tumors of the airway in this group of patients is the synergistic effect of smoking and alcohol abuse as risk factors for both Madelung’s disease and malignant tumors of the airway. Currently it is recommended that these patients should have their fatty lesions removed surgically. The removal of fat facilitates examination of the neck for signs of cervical lymphadenopathy in malignant disease.
Conclusions: Patients with Madelung’s disease should be followed regularly. The development of aerodigestive symptoms should be fully investigated with endoscopy and imaging. The cause of symptoms should not be attributed to fatty compression until a carcinoma of the upper airway has been excluded.
Similar content being viewed by others
REFERENCES
Kohan D, Miller PJ, Rothstein SG, Kaufman D. Madelung’s disease: case reports and literature review. Otolaryngol Head Neck Surg 1993;108:156–159.
Enzi G, Fiore D, Mazzoleni F. Computed tomography of deep fat masses in multiple symmetrical lipomatosis. Radiol 1982;144:121–124.
Parmar SC, Blackburn C. Madelung’s disease: an uncommon disorder of unknown aetiology? Br J Oral Maxillofac Surg 1996;34:467–470.
John DG, Fung HK, van Hasselt CA, King WWK. Multiple symmetrical lipomatosis—a condition not previously reported in the Chinese. Eur Arch Otorhinolaryngol 1992;249:277–278.
Wu WH, Fang RH. Multiple symmetrical lipomatosis (Madelung’s disease): a case report. Chin Med J (Taipei) 1996;58:139–142.
Luscher NJ, Prein J, Spiessl B. Lipomatosis of the neck. Ann Plast Surg 1986;16:502–508.
Ahuja AT, King AD, Chan ESY, et al. Madelung disease: distribution of cervical fat and preoperative findings at sonography, MR, and CT. Am J Neuroradiol 1998;19:707–710.
Enzi G. Multiple symmetric lipomatosis: an updated clinical report. Medicine 1984;63:56–64.
Tizian C, Berger A, Vykoupil KF. Malignant degeneration in Madelung’s disease. Case report. Br J Plast Surg 1983;36:187–189.
Stavropoulos PG, Zouboulis CC, Trautmann C, Orfanos CE. Symmetric lipomatoses in female patients. Dermatology 1997;194:26–31.
Enzi G, Angelini C, Negrin P, Armani M, Pierobon S, Fedele D. Sensory, motor and autonomic neuropathy in patients with multiple symmetric lipomatosis. Medicine 1986;64:388–392.
Pollock M, Nicholson GI, Nukada H, Cameron S, Frankish P. Neuropathy in multiple symmetric lipomatosis. Brain 1988;111:1157–1171.
Borges A, Torrinha F, Lufkin RB, Abemayor E. Laryngeal involvement in multiple symmetric lipomatosis: the role of computed tomography in diagnosis. Am J Otolaryngol 1997;18:127–130.
Ruzicka T, Vieluf D, Landthaler M, Braun-Falco O. Benign symmetric lipomatosis. J Am Acad Dermatol 1987;17:663–674.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Chan, E.S.Y., Ahuja, A.T., King, A.D. et al. Head and Neck Cancers Associated With Madelung’s Disease. Ann Surg Oncol 6, 395–397 (1999). https://doi.org/10.1007/s10434-999-0395-7
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s10434-999-0395-7