pilomatrixoma

Pilomatricoma
	Micrograph of a pilomatricoma showing the characteristic "ghost" cells (anucleate squamous cells), benign viable squamous cells and multi-nucleated giant cells. H&E stain.
Classification and external resources
Specialty	oncology
ICD-10	D23
DiseasesDB	32128
eMedicine	article/1058965
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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/04/28 03:57:40」(JST)

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Pilomatricoma, also known as a calcifying epithelioma of Malherbe,^[1] Malherbe calcifying epithelioma, and Pilomatrixoma, is a benign skin tumor derived from the hair matrix.^[2]^[3] These neoplasms are relatively uncommon and typically occur on the scalp, face, and upper extremities. Clinically, pilomatricomas present as a subcutaneous nodule or cyst with unremarkable overlying epidermis that can range in size from 0.5-3.0 cm, but the largest reported cases was 24 cm.^[4]

Histologic features

Pilomatricomas consist of anucleate squamous cells (called "ghost cells"), benign viable squamous cells and foreign body giant cells. These neoplasms have a characteristic transition of cells. The lining of the cyst consists of basoloid cells with indistinct cell borders and basophilic nuclei that mature into the eosinophilic anucleated squamous cells. The presence of calcifications with foreign body giant cells is common with in the tumors.^[5]

Pathogenesis

Pilomatricoma is associated with high levels of beta-catenin caused by either a mutation in the APC gene or a stabilizing mutation in the beta-catenin gene, CTNNB1. High levels of beta-catenin increases cell proliferation, inhibit cell death, and ultimately leads to neoplastic growth.^[6]

Associations

Pilomatricomas have been observed in a variety of genetic disorders including Turner syndrome, myotonic dystrophy, Rubinstein-Taybi syndrome. Trisomy 9, and Gardner syndrome.^[7] It has been reported that the prevalence of pilomatricomas in Turner syndrome is 2.6%.^[6]

Hybrid cysts that are composed of epidermal inclusion cysts and pilomatricoma-like changes have been repeatedly observed in Gardner syndrome.^[8]^[9]^[10]^[11] This association has prognostic important since cutaneous findings in children with Gardner Syndrome generally precede colonic polyposis.^{[citation needed]}

References

^ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0. ^{[page needed]}
^ James, William Daniel; Berger, Timothy G.; Elston, Dirk M., eds. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. p. 670. ISBN 978-0-8089-2351-0.
^ Levy, Jaime; Ilsar, Michael; Deckel, Yael; Maly, Alexander; Anteby, Irene; Pe'er, Jacob (2008). "Eyelid Pilomatrixoma: A Description of 16 cases and a Review of the Literature". Survey of Ophthalmology. 53 (5): 526–35. doi:10.1016/j.survophthal.2008.06.007. PMID 18929763.
^ Gongidi, P.; Meshekow, J.; Holdbrook, T.; Germaine, P. (2015). "Giant Pilomatrixoma Presenting in the Posterior Thorax, a Rare Location and the Largest Described". Case Reports in Radiology. 2015: 590742. doi:10.1155/2015/590742. PMC 4339831. PMID 25763287.
^ Elder, David E.; Johnson, Bernett L.; Elenitsas, Rosalie, eds. (2005). Lever's Histopathology of the Skin. Lippincott Williams & Wilkins. ISBN 978-0-7817-3742-5. ^{[page needed]}
^ ^a ^b Glanz, Steven M.; Kessler, Harvey P.; Eskin, Thomas A.; Liu, Chen; Hassanein, Ashraf M. (2003). "b-Catenin Is Expressed Aberrantly in Tumors Expressing Shadow Cells Pilomatricoma, Craniopharyngioma, and Calcifying Odontogenic Cyst". American Journal of Clinical Pathology. 120 (5): 732–6. doi:10.1309/EALEG7LD6W7167PX. PMID 14608900.
^ Cooper, Philip H.; Fechner, Robert E. (1983). "Pilomatricoma-like changes in the epidermal cysts of Gardner's syndrome". Journal of the American Academy of Dermatology. 8 (5): 639–44. doi:10.1016/S0190-9622(83)70071-X. PMID 6863619.
^ Narisawa, Yutaka; Kohda, Hiromu (1989). "An Unusual Hybrid Cyst in Gardner's Syndrome with Partial Differentiation toward the Inner Root Sheath". The Journal of Dermatology. 16 (6): 492–5. doi:10.1111/j.1346-8138.1989.tb01591.x. PMID 2628457.
^ Rütten, A; Wenzel, P; Goos, M (1990). "Gardner-Syndrom mit pilomatrixomartigen Haarfollikelzysten" [Gardner syndrome with pilomatrixoma-like hair follicle cysts]. Der Hautarzt; Zeitschrift FüR Dermatologie, Venerologie, Und Verwandte Gebiete (in German). 41 (6): 326–8. PMID 2380070. INIST:19291018.
^ Narisawa, Yutaka; Kohda, Hiromu (1995). "Cutaneous cysts of Gardner's syndrome are similar to follicular stem cells". Journal of Cutaneous Pathology. 22 (2): 115–21. doi:10.1111/j.1600-0560.1995.tb01392.x. PMID 7560342.
^ Urabe, Kazunori; Xia, Jianxin; Masuda, Teiichi; Moroi, Yoichi; Furue, Masutaka; Matsumoto, Takayuki (2004). "Pilomatricoma-Like Changes in the Epidermoid Cysts of Gardner Syndrome with an APC Gene Mutation". The Journal of Dermatology. 31 (3): 255–7. doi:10.1111/j.1346-8138.2004.tb00669.x. PMID 15187352.

UpToDate Contents

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1. 皮膚付属器癌 cutaneous adnexal tumors
2. 皮膚石灰沈着症：病院および患者の評価 calcinosis cutis etiology and patient evaluation
3. 皮膚の良性病変の概要 overview of benign lesions of the skin
4. 新生児や乳児の皮膚結節 skin nodules in newborns and infants
5. ターナー症候群の臨床症状と診断 clinical manifestations and diagnosis of turner syndrome

English Journal

Fine-needle aspiration cytology in primary cutaneous tumors.

Bharani V1, Kumar R1, Gupta N2, Srinivasan R2, Rajwanshi A2, Nahar U3, Dey P2.
Diagnostic cytopathology.Diagn Cytopathol.2017 Aug;45(8):681-688. doi: 10.1002/dc.23741. Epub 2017 May 4.
PMID 28474476

Calcifying epithelioma of malherbe (Pilomatrixoma): Clinical and sonographic features.

Lin SF1, Xu SH1, Xie ZL1.
Journal of clinical ultrasound : JCU.J Clin Ultrasound.2017 Jul 5. doi: 10.1002/jcu.22517. [Epub ahead of print]
PMID 28677820

Pilomatrixoma located on the cruris: an uncommon localization.

Burak Atalay I1, Korkmaz I1, Gungor S1.
Journal of surgical case reports.J Surg Case Rep.2017 Jul 4;2017(7):rjx114. doi: 10.1093/jscr/rjx114. eCollection 2017 Jul.
PMID 28690776

Japanese Journal

Immunohistochemical expression of podoplanin in so-called hard α-keratin-expressing tumors, including calcifying cystic odontogenic tumor, craniopharyngioma, and pilomatrixoma

Kikuchi Kentaro,Ito Shigeru,Inoue Harumi,González-Alva Patricia,Miyazaki Yuji,Sakashita Hideaki,Yoshino Atsuo,Katayama Yoichi,Terui Tadashi,Ide Fumio,Kusama Kaoru
Journal of Oral Science 54(2), 165-175, 2012
… In this study, we examined the expression of podoplanin in calcifying cystic odontogenic tumor (CCOT) in comparison with that in other so-called hard α-keratin-expressing tumors such as craniopharyngioma (CP) and pilomatrixoma (PM). …
NAID 130002070030

皮膚混合腫瘍の1例

藤田美穂,岸田功典,下方征,峯村徳哉,長谷哲男,澁谷誠,新谷真理子
Skin cancer : official organ of the Japanese Society for Skin Cancer = 皮膚悪性腫瘍研究会機関誌 24(1), 53-56, 2009-05-20
症例68歳男。30年以上前に前頸部に結節気付くものの放置していた。その後徐々に増大してきたため2008年1月24日当科紹介受診。結節は表面常色，30×18×7mmで硬，下床との可動性良好であった。石灰化上皮腫などを疑い全摘除術を施行した。病理組織学的には病変部は真皮内に被膜を有する境界明瞭な腫瘍性病変であった。腫瘍を構成する細胞は，索状，胞巣状ないし一部管状に配列する上皮細胞と粘液腫様間質に散在す …
NAID 10025619063

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