Bannayan Syndrome—generalized Lipomatosis Associated With Megalencephaly and Macrodactyly

Okumura, Kenzo; Sasaki, Yoshiro; Ohyama, Makiko; Nishi, Toshiharu

doi:10.1111/j.1440-1827.1986.tb01479.x

Cited by 16 publications

(12 citation statements)

References 4 publications

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“…A 1986 report by Okumura et al describes a fiveyear-old girl with a phenotype virtually identical to the one reported by Bannayan 15 years earlier [Okumura et al, 1986]. The lipomatosis was extremely aggressive, involving the pleural and abdominal cavities, the former covered with thick sheets of tumor.…”

Section: Bannayan Riley Zonana Ruvalcaba and Related Observationsmentioning

confidence: 86%

Inherited macrocephaly-hamartoma syndromes

DiLiberti

1998

Am. J. Med. Genet.

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Recent discoveries in the molecular biology of the phosphatase and tensin homolog (PTEN) locus in the q22-23 region of chromosome 10 prove and/or suggest that several syndromes previously considered to be clinically and genetically distinct entities should actually be unified into a single entity. This conclusion is most secure for the Cowden and "Bannayan-Zonana" phenotypes, but almost certainly should also include the "Riley-Ruvalcaba" and Lhermitte-Duclos phenotypes as well benign familial macrocephaly and external hydrocephalus. The clinical and molecular data supporting this unification are presented along with a proposal for new nomenclature-the PTEN MATCHS (macrocephaly, autosomal dominant, thyroid disease, cancer, hamartomata, skin abnormalities) syndrome-based on the observed clinical abnormalities.

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Section: Bannayan Riley Zonana Ruvalcaba and Related Observationsmentioning

confidence: 86%

Inherited macrocephaly-hamartoma syndromes

DiLiberti

1998

Am. J. Med. Genet.

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“…Examples of this uncommon lesion are reported in the literature as only a handful of individual case reports. 1–21 Associations with rare childhood syndromes such as Shwachman-Diamond, 12,19 Bannayan, 20 or Johanson-Blizzard 13 have been made. Lipomatous pseudohypertrophy often forms a benign mass mimicking cancer, resulting in unnecessary resection and morbidity.…”

mentioning

confidence: 99%

Lipomatous Pseudohypertrophy of the Pancreas

Altinel¹,

Basturk²,

Sarmiento³

et al. 2010

Pancreas

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Objectives Owing to the challenges in obtaining pancreatic biopsies, pancreatic resection for presumed malignancy is often performed without histological confirmation. As a result, benign lesions are sometimes surgically removed. One such condition, which is poorly defined in the literature, is referred to as lipomatous pseudohypertrophy (LPH) of the pancreas. Methods Five cases of LPH were analyzed. Results Four patients underwent surgical resection, 3 of which were diagnosed preoperatively by radiology as having ductal adenocarcinoma. The fourth case was correctly interpreted by magnetic resonance imaging as LPH, but the patient underwent resection because of the intractable pain due to pancreatitis. The fifth patient has been placed on watchful waiting. Two tumors were in the pancreatic head, one in the tail, one in the uncinate process, and one demonstrated diffuse involvement. Microscopically, they were characterized as having normal lipocytes without lipoblasts or inflammation. Within the adipose tissue, scattered microscopic foci of pancreatic parenchyma could be seen. Conclusion Lipomatous pseudohypertrophy of the pancreas is a distinct entity characterized by localized/diffuse replacement of pancreatic parenchyma with mature adipose tissue. It forms a pseudotumor that may be difficult to distinguish clinically from pancreatic adenocarcinoma. This entity should be considered when evaluating patients with a new diagnosis of a hypodense pancreatic neoplasm on imaging.

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“…The soft tissue and visceral tumours can be present at birth or appear in the first years of life. Their evolution is variable and foreseeable with difficulty: they can grow to become lifethreatening (four deaths reported), remain stable in size, or decrease spontaneously (2)(3)(4)(8)(9)(10). As far as the treatment is concerned, self limiting tumours without symptoms can be treated conservatively otherwise they should be surgically excised.…”

Section: Discussionmentioning

confidence: 96%

“…Subsequently Zonana et al found the association in a father and in his two sons, and suggested that the condition was inherited as an autosomal dominant trait (13). Since then, few cases of this rare syndrome, included five sporadic ones, have been reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). We present a 7 year old child with the syndrome and mental retardation in whom multiple lipomas were treated with suction-assisted lipectomy.…”

mentioning

confidence: 97%

MACROCEPHALY WITH MULTIPLE SOFT TISSUE AND VISCERAL HAMARTOMAS ("BANNAYAN-ZONANA" SYNDROME): Case report

Campiglio

Giuseppe²,

Grappolini³

1998

Scandinavian Journal of Plastic and Reconstructive Surgery and

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Since its first description few cases of the rare association of macrocephaly with multiple soft tissue and visceral hamartomas (Bannayan-Zonana syndrome) have been reported. In this paper we report a case of a 7 year old child in whom lipomas of the abdomen and right flank have been treated with liposuction. We could find no previous evidence that this surgical technique has been used in the treatment of this syndrome.

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Bannayan Syndrome—generalized Lipomatosis Associated With Megalencephaly and Macrodactyly

Cited by 16 publications

References 4 publications

Inherited macrocephaly-hamartoma syndromes

Inherited macrocephaly-hamartoma syndromes

Lipomatous Pseudohypertrophy of the Pancreas

MACROCEPHALY WITH MULTIPLE SOFT TISSUE AND VISCERAL HAMARTOMAS ("BANNAYAN-ZONANA" SYNDROME): Case report

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