Bannayan-Zonana syndrome associated with lipomas, hemangiomas, and lymphangiomas

doi:10.1016/S0022-3468(05)80100-9

Journal of Pediatric Surgery

Volume 27, Issue 6, June 1992, Pages 722-723

https://doi.org/10.1016/S0022-3468(05)80100-9 Get rights and content

Bannayan-Zonana syndrome is a rare disorder characterized by macrocephaly and multiple soft tissue and visceral hamartomas. This report presents a sporadic patient with macrocephaly, lipomas, hemangiomas, and lymphangiomas who died of cardiac and respiratory failure due to progressive cervicomediastinal arteriovenous fistulous hemangiomas at the age of 9 years.

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Cited by (15)

Case of Cowden Syndrome with 15 Spinal Arteriovenous Fistulas
2020, World Neurosurgery
Citation Excerpt :
Thus somatic and germline PTEN loss of function has been associated with excessive or aberrant angiogenesis in patients. A limited number of cases of CS or CS-related disorders with vascular anomalies involving the spine and/or spinal cord have been reported (Table 2).1,39-45 In addition to SVM-induced hemodynamic disturbances, angiogenic growth factor overexpression has been proposed as another complementary mechanism for the development of multiple AVFs.46
Spinal vascular malformations are rarely multiple: fewer than 50 observations have been documented so far, with a maximum of 4 coexisting lesions per patient, always restricted to a single vertebral region (e.g., cervical or thoracic).
We describe the case of a 61-year-old woman with Cowden syndrome with 15 spinal arteriovenous fistulas (AVFs) at the cervical, thoracic, and lumbar levels and an adrenal AVF. She was initially referred for reevaluation of an upper cervical spinal epidural spinal arteriovenous fistula diagnosed 6 years earlier. Her history included breast carcinoma, a malignant salivary gland tumor, and removal of multiple ovarian, thyroid, and gastric hamartomas. Computed tomography and magnetic resonance imaging confirmed the presence of a prominent cervical vascular lesion. Spinal digital subtraction angiography revealed the presence of 15 additional vascular anomalies.
This multiplicity of AVFs appears to result from a combination of various factors including local regional hemodynamic changes, growth factor-mediated alterations involving notably vascular endothelial growth factor pathways, and the prothrombotic state associated with abnormalities in blood vessel structure.
Bannayan-Zonana syndrome fatalities
2009, Archives de Pediatrie
Le syndrome de Bannayan-Zonana est une affection rare caractérisée par l’association d’une macrocéphalie et de multiples hamartomes et lipomes des tissus mous et des viscères. La connaissance de ce syndrome est importante en raison du risque de survenue d’hémorragies parfois mortelles et de néoplasies viscérales à l’âge adulte. Nous rapportons un nouveau cas, original par son caractère sporadique et par son évolution rapidement fatale.
Bannayan-Zonana syndrome is a rare entity characterized by macrocephaly and multiple soft tissue and visceral hamartomas and lipomas. Knowledge of this syndrome is important given the risk of sometimes fatal bleeding and visceral neoplasia in adulthood. We report on a new case, original in its sporadic occurrence and rapidly fatal progression.
Cutaneous lipoma in children: 5 cases with Bannayan-Riley-Ruvalcaba syndrome
2006, Journal of Pediatric Surgery
Cutaneous lipoma is rare in children, but it can be part of a syndrome such as the Bannayan-Riley-Ruvalcaba syndrome (BRRS). The BRRS is a dominant autosomal disorder characterized by cutaneous lipomas, macrocephaly, intestinal polyps, and developmental delay associated with PTEN gene mutations. This syndrome is thought to represent a pediatric form of the Cowden syndrome, characterized among other features by an increased risk of cancer. We report 5 cases of BRRS, all diagnosed in children with lipoma and macrocephaly. Children presenting with lipomas need a complete physical examination to look for other signs of BRRS, because they may need further follow-up for tumor screening in adulthood.
Bannayan-Zonana syndrome: A rare autosomal dominant syndrome with multiple lipomas and hemangiomas: A case report and review of literature
1998, Surgical Neurology
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.
Cutaneous lipomas and macrocephaly as early signs of PTEN hamartoma tumor syndrome
2020, Pediatric Dermatology
Mobilization and homing of bone marrow stem cells after stroke
2016, Bone Marrow Stem Cell Therapy for Stroke

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^*: Presented at the 24th Annual Meeting of the Pacific Association of Pediatric Surgeons, Hong Kong, May 20–24, 1991.

¹: From the Departments of Pediatric Surgery and Dermatology, Tohoku University School of Medicine, Sendai, Japan.

View full text

Bannayan-Zonana syndrome associated with lipomas, hemangiomas, and lymphangiomas*

J Pediatr

Lipomatosis, angiomatosis, and macrocephalia

Arch Pathol

The Bannayan syndrome: An autosomal dominant disorder consisting of macrocephaly, lipomas and hemangiomas, and a risk for intracranial tumors

Pediatrics

Mental retardation in the Bannayan syndrome

Pediatrics

Macrocephaly with hamartomas: Bannayan-Zonana syndrome

Am J Med Genet