Literature DB >> 19598310

Excision of a large abdominal wall lipoma improved bowel passage in a Proteus syndrome patient.

Yoshifumi Nakayama1, Shinichi Kusuda, Naoki Nagata, Koji Yamaguchi.   

Abstract

Proteus syndrome is an extremely rare congenital disorder that produces multifocal overgrowth of tissue. This report presents a surgical case of a large lipoma in the abdominal wall of a patient with Proteus syndrome. She was diagnosed with Proteus syndrome based on certain diagnostic criteria. The neoplasm increased in size gradually, producing hemihypertrophy of her left lower extremity and trunk, and spread to her retroperitoneum and her left abdominal wall. She experienced gradually progressive constipation, nausea, vomiting, and abdominal pain. Computed tomography (CT) of the abdomen demonstrated a large mass in the subcutaneous adipose tissue of the left lower abdominal wall which measured 12 cm x 8 cm x 6 cm in diameter and encased the left colon. This mass in the abdominal wall was excised. The weight of the excised mass was 1550 g. The histopathological diagnosis of this mass was lipoma. After surgery, the encasement of the left colon was improved, and the patient was able to move her bowels twice per day. The excision of the large lipoma in the abdominal wall contributed to the improved bowel passage in this patient with Proteus syndrome.

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Year:  2009        PMID: 19598310      PMCID: PMC2710790          DOI: 10.3748/wjg.15.3312

Source DB:  PubMed          Journal:  World J Gastroenterol        ISSN: 1007-9327            Impact factor:   5.742


  13 in total

1.  Lipomatosis of the colon complicating Proteus syndrome.

Authors:  G Mackay; L Spitz; K McHugh
Journal:  Arch Dis Child       Date:  2002-04       Impact factor: 3.791

2.  A newly recognized hamartomatous syndrome.

Authors:  M M Cohen; P W Hayden
Journal:  Birth Defects Orig Artic Ser       Date:  1979

Review 3.  The challenges of Proteus syndrome: diagnosis and management.

Authors:  Leslie Biesecker
Journal:  Eur J Hum Genet       Date:  2006-08-02       Impact factor: 4.246

4.  The tumor suppressor, PTEN/MMAC1, dephosphorylates the lipid second messenger, phosphatidylinositol 3,4,5-trisphosphate.

Authors:  T Maehama; J E Dixon
Journal:  J Biol Chem       Date:  1998-05-29       Impact factor: 5.157

5.  Association of germline mutation in the PTEN tumour suppressor gene and Proteus and Proteus-like syndromes.

Authors:  X Zhou; H Hampel; H Thiele; R J Gorlin; R C Hennekam; M Parisi; R M Winter; C Eng
Journal:  Lancet       Date:  2001-07-21       Impact factor: 79.321

6.  Macrodactyly in Proteus syndrome.

Authors:  H Miura; Y Uchida; K Ihara; Y Sugioka
Journal:  J Hand Surg Br       Date:  1993-06

7.  Proteus syndrome.

Authors:  H Yasuda; O Yamamoto; H Hirokawa; M Asahi; M Kashimura; A Sakai
Journal:  Dermatology       Date:  2001       Impact factor: 5.366

8.  Proteus syndrome.

Authors:  K Sayama; N Hato; O Matsuda; S Shiraishi; Y Miki
Journal:  Dermatology       Date:  1994       Impact factor: 5.366

9.  Overgrowth syndromes: is dysfunctional PI3-kinase signalling a unifying mechanism?

Authors:  Karen T Barker; Richard S Houlston
Journal:  Eur J Hum Genet       Date:  2003-09       Impact factor: 4.246

10.  The proteus syndrome. Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections.

Authors:  H R Wiedemann; G R Burgio; P Aldenhoff; J Kunze; H J Kaufmann; E Schirg
Journal:  Eur J Pediatr       Date:  1983-03       Impact factor: 3.183

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