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词条 Pilomatricoma
释义

  1. Presentation

     Associations 

  2. Histologic features

  3. Pathogenesis

  4. Diagnosis

  5. See also

  6. References

  7. External links

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| caption = Micrograph of a pilomatricoma showing the characteristic "ghost" cells (anucleate squamous cells), benign viable squamous cells and multi-nucleated giant cells. H&E stain.
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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 case was 24 cm.[4]

Presentation

Associations

Pilomatricomas have been observed in a variety of genetic disorders including Turner syndrome, myotonic dystrophy, Rubinstein-Taybi syndrome. Trisomy 9, and Gardner syndrome.[5] 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.[6][7][8][9] This association has prognostic import, since cutaneous findings in children with Gardner Syndrome generally precede colonic polyposis.{{citation needed|date=July 2016}}

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.[10]

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.[11]

Diagnosis

{{Empty section|date=May 2018}}

See also

  • Malignant pilomatricoma
  • List of cutaneous conditions
  • List of cutaneous neoplasms associated with systemic syndromes

References

1. ^{{cite book |last1=Rapini |first1=Ronald P. |last2=Bolognia |first2=Jean L. |last3=Jorizzo |first3=Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}{{page needed|date=July 2016}}
2. ^{{cite book |editor1-first=William Daniel |editor1-last=James |editor2-first=Timothy G. |editor2-last=Berger |editor3-first=Dirk M. |editor3-last=Elston |title=Andrews' Diseases of the Skin: Clinical Dermatology |publisher=Saunders Elsevier |location= |year=2006 |page=670 |isbn=978-0-8089-2351-0 }}
3. ^{{cite journal |last1=Levy |first1=Jaime |last2=Ilsar |first2=Michael |last3=Deckel |first3=Yael |last4=Maly |first4=Alexander |last5=Anteby |first5=Irene |last6=Pe'er |first6=Jacob |title=Eyelid Pilomatrixoma: A Description of 16 cases and a Review of the Literature |journal=Survey of Ophthalmology |volume=53 |issue=5 |pages=526–35 |year=2008 |pmid=18929763 |doi=10.1016/j.survophthal.2008.06.007 }}
4. ^{{cite journal |last1=Gongidi |first1=P. |last2=Meshekow |first2=J. |last3=Holdbrook |first3=T. |last4=Germaine |first4=P. |title=Giant Pilomatrixoma Presenting in the Posterior Thorax, a Rare Location and the Largest Described |journal=Case Reports in Radiology |volume=2015 |issue= |pages=590742 |year=2015 |pmid=25763287 |pmc=4339831 |doi=10.1155/2015/590742 }}
5. ^{{cite journal |last1=Cooper |first1=Philip H. |last2=Fechner |first2=Robert E. |title=Pilomatricoma-like changes in the epidermal cysts of Gardner's syndrome |journal=Journal of the American Academy of Dermatology |volume=8 |issue=5 |pages=639–44 |year=1983 |pmid=6863619 |doi=10.1016/S0190-9622(83)70071-X }}
6. ^{{cite journal |last1=Narisawa |first1=Yutaka |last2=Kohda |first2=Hiromu |title=An Unusual Hybrid Cyst in Gardner's Syndrome with Partial Differentiation toward the Inner Root Sheath |journal=The Journal of Dermatology |volume=16 |issue=6 |pages=492–5 |year=1989 |pmid=2628457 |doi=10.1111/j.1346-8138.1989.tb01591.x }}
7. ^{{cite journal |last1=Rütten |first1=A |last2=Wenzel |first2=P |last3=Goos |first3=M |title=Gardner-Syndrom mit pilomatrixomartigen Haarfollikelzysten |trans-title=Gardner syndrome with pilomatrixoma-like hair follicle cysts |language=German |journal=Der Hautarzt; Zeitschrift FüR Dermatologie, Venerologie, Und Verwandte Gebiete |volume=41 |issue=6 |pages=326–8 |year=1990 |id={{INIST|19291018}} |pmid=2380070 }}
8. ^{{cite journal |last1=Narisawa |first1=Yutaka |last2=Kohda |first2=Hiromu |title=Cutaneous cysts of Gardner's syndrome are similar to follicular stem cells |journal=Journal of Cutaneous Pathology |volume=22 |issue=2 |pages=115–21 |year=1995 |pmid=7560342 |doi=10.1111/j.1600-0560.1995.tb01392.x }}
9. ^{{cite journal |last1=Urabe |first1=Kazunori |last2=Xia |first2=Jianxin |last3=Masuda |first3=Teiichi |last4=Moroi |first4=Yoichi |last5=Furue |first5=Masutaka |last6=Matsumoto |first6=Takayuki |title=Pilomatricoma-Like Changes in the Epidermoid Cysts of Gardner Syndrome with an APC Gene Mutation |journal=The Journal of Dermatology |volume=31 |issue=3 |pages=255–7 |year=2004 |pmid=15187352 |doi=10.1111/j.1346-8138.2004.tb00669.x }}
10. ^{{cite book |editor1-first=David E. |editor1-last=Elder |editor2-first=Bernett L. |editor2-last=Johnson |editor3-first=Rosalie |editor3-last=Elenitsas |year=2005 |title=Lever's Histopathology of the Skin |publisher=Lippincott Williams & Wilkins |isbn=978-0-7817-3742-5 |page= }}{{page needed|date=July 2016}}
11. ^{{cite journal |last1=Glanz |first1=Steven M. |last2=Kessler |first2=Harvey P. |last3=Eskin |first3=Thomas A. |last4=Liu |first4=Chen |last5=Hassanein |first5=Ashraf M. |title=b-Catenin Is Expressed Aberrantly in Tumors Expressing Shadow Cells Pilomatricoma, Craniopharyngioma, and Calcifying Odontogenic Cyst |journal=American Journal of Clinical Pathology |volume=120 |issue=5 |pages=732–6 |year=2003 |pmid=14608900 |doi=10.1309/EALEG7LD6W7167PX }}

External links

{{Medical resources
| DiseasesDB = 32128
| ICD10 = {{ICD10|D|23||d|23}}
| ICD9 =
| ICDO =
| OMIM =
| MedlinePlus =
| eMedicineSubj = article
| eMedicineTopic = 1058965
| MeshID =
}}{{Tumors of skin appendages}}

1 : Cutaneous congenital anomalies

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