Elsevier

Surgical Neurology

Volume 50, Issue 2, August 1998, Pages 164-168
Surgical Neurology

Genetics
Bannayan-zonana syndrome: a rare autosomal dominant syndrome with multiple lipomas and hemangiomas: A case report and review of literature

https://doi.org/10.1016/S0090-3019(98)00039-1Get rights and content

Abstract

BACKGROUND Bannayan-Zonana syndrome is a rare hamartomatous disorder, characterized by macrocephaly, multiple lipomas, and hemangiomas. Inheritance is by autosomal dominant transmission with few reported sporadic cases. Male predominance is also reported.

METHODS We describe a patient who presented with multiple subcutaneous lipomas, mild macrocephaly, and an extradural spinal hemangioma. Other affected family members and 24 other previously reported cases are discussed.

RESULTS Spinal hemangiomas have not been described previously with this syndrome. The patient also had a “malignant bone tumor” removed from his humerus 20 years ago. Two of the patient’s siblings also had lymphoma, which is an unusual accompaniment not reported previously. Only the male members in the family showed multiple subcutaneous lipomas.

CONCLUSION Some patients with Bannayan-Zonana syndrome may have hamartomatous lesions producing cord compression or intracerebral hemorrhage, or they may rarely have other malignant tumors; therefore it is important that neurosurgeons are aware of the entity. The early diagnosis of BZS is also important for genetic counseling.

Section snippets

Case report

A 41-year-old male presented with a 2–3 week history of numbness in both lower extremities. The numbness began in his toes and gradually ascended to his thighs. He also complained of mild leg weakness and poor balance. The patient suffered from an upper respiratory tract infection around the time that the numbness began. His symptoms were exacerbated by prolonged walking or standing and alleviated by lying down. Valsalva maneuvers did not worsen the symptoms.

His past medical history is notable

Discussion

This patient and many of his family members display the characteristic hamartomatous lesions previously described in BZS, namely multiple lipomas and hemangiomas. Hamartomas with lymphangiomyomatous differentiation or mixtures of the above histologic components have also been seen [6]. Most of the lesions are slow growing and subcutaneous lipomas are easily resectable. However, locally aggressive behavior may occur, as reported in a case of lipohemangioma that required leg amputation because of

Acknowledgements

We thank Valarie Lindgren, Ph.D. (Director, Cytogenetics, Loyola University Medical Center) for doing the chromosomal analysis in her lab and for her helpful review of the manuscript.

References (12)

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