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pathology in which fat accumulates in lipomas in the body

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/06/21 21:02:50」(JST)

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Mediastinal lipomatosis

Lipomatosis is believed to be an autosomal dominant condition in which multiple lipomas are present on the body. Many discrete, encapsulated lipomas form on the trunk and extremities, with relatively few on the head and shoulders.^[1] In 1993, a genetic defect within lipomas was localized to chromosome 12q15, where the HMGIC gene encodes the high-mobility-group protein isoform I-C.^[2] This is one of the most commonly found mutations in solitary lipomatous tumors but lipomas often have multiple mutations. Reciprocal translocations involving chromosomes 12q13 and 12q14 have also been observed within.^[3]^[4]^[5]

Although this condition is benign, it can sometimes be very painful depending on location of the lipomas. Some patients who are concerned with cosmetics seek removal of individual lipomas. Removal can include simple excision, endoscopic removal, or liposuction.^[6]

Other entities which are accompanied by multiple lipomas include Proteus syndrome, Cowden syndrome and related disorders due to PTEN gene mutations, benign symmetric lipomatosis (Madelung disease),Dercum's Disease, familial lipodystrophy, hibernomas, epidural steroid injections with epidural lipomatosis,^[7] and familial angiolipomatosis.

References

^ Familial multiple lipomatosis
^ Schoenmakers, EF; Wanschura, S; Mols, R; Bullerdiek, J; Van Den Berghe, H; Van De Ven, WJ (1995). "Recurrent rearrangements in the high mobility group protein gene, HMGI-C, in benign mesenchymal tumours". Nature Genetics 10 (4): 436–44. doi:10.1038/ng0895-436. PMID 7670494.
^ Weiss, SW (1996). "Lipomatous tumors". Monographs in pathology 38: 207–39. PMID 8744279.
^ Gologorsky, Y; Gologorsky, D; Yarygina, AS; Surti, U; Zirwas, MJ (2007). "Familial multiple lipomatosis: Report of a new family". Cutis; cutaneous medicine for the practitioner 79 (3): 227–32. PMID 17674589.
^ http://www.genome.jp/dbget-bin/www_bget?omim+151900 Lipomatosis, Familial Multiple Lipomatosis, FML
^ Familial multiple lipomatosis
^ https://www.biomedcentral.com/1471-2253/14/70 Multiple Epidural Steroid Injections and Epidural Lipomatosis

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UpToDate Contents

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1. 皮膚の良性病変の概要 overview of benign lesions of the skin
2. カウデン症候群などのPTEN過誤腫症候群 pten hamartoma tumor syndrome including cowden syndrome
3. 浸潤性甲状腺疾患 infiltrative thyroid disease
4. シュワッハマン・ダイアモンド症候群 shwachman diamond syndrome
5. ミトコンドリアミオパシー：臨床的特徴および診断 mitochondrial myopathies clinical features and diagnosis

English Journal

The first radiographic image of a peripheral nerve disorder? Lipomatous macrodactyly (unrecognized lipomatosis of nerve).

Mahan MA1, Prasad N, Spinner RJ.
Acta neurochirurgica.Acta Neurochir (Wien).2015 Jun;157(6):1059-62. doi: 10.1007/s00701-015-2416-6. Epub 2015 Apr 12.
Lipomatosis of nerves (LN) involves benign fibro-fatty infiltration and is often associated with territorial overgrowth of soft tissue and bone; this distinctive disease pattern can be visualized on plain radiographs. We recently discovered a case (presented by Sir Robert Jones in 1898 to the Patho
PMID 25862172

Syndromic lipomatosis of the head and neck: a review of the literature.

Tadisina KK1, Mlynek KS, Hwang LK, Riazi H, Papay FA, Zins JE.
Aesthetic plastic surgery.Aesthetic Plast Surg.2015 Jun;39(3):440-8. doi: 10.1007/s00266-015-0478-8. Epub 2015 Apr 10.
INTRODUCTION: Patients with syndromic causes of lipomatosis of the head and neck, although rare, often present late in the course of the disease in a myriad of ways, including concomitant airway obstruction, severe functional limitations, and/or significant cosmetic defects. The goal of this report
PMID 25860506

An unusual complication of morbid obesity: epidural lipomatosis.

Noh E1.
The American journal of emergency medicine.Am J Emerg Med.2015 May;33(5):742.e3-4. doi: 10.1016/j.ajem.2014.11.043. Epub 2014 Dec 2.
PMID 25618765

Japanese Journal

高度肥満により腰椎硬膜外脂肪腫症を発症した1例

上松幸三郎,渕上徹郎,津守伸浩,田中信次,森本典夫
整形外科と災害外科 61(3), 557-560, 2012-09-25
NAID 10031013409

Spinal Subdural Hematoma Mimicking Epidural Lipomatosis

KAMO Minobu,WATANABE Yoshiyuki,NUMAGUCHI Yuji,SAIDA Yukihisa
Magnetic resonance in medical sciences : MRMS 11(3), 197-199, 2012-09-01
… Magnetic resonance imaging showed lesions of homogeneous high intensity in the spinal canal on both T1- and T2-weighted images, findings closely resembling those for epidural lipomatosis. …
NAID 10031046913