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Pilomatrical Carcinoma: A Case Report by Dr. Tony Nakhla of OC Skin Institute



OC Skin Institute's Dr. Tony Nakhla, an expert in the medical dermatology field, reviews facts concerning Pilomatrical Carcinoma and refers to further details obtained from a case study. Dr. Nakhla ...

OC Skin Institute's Dr. Tony Nakhla, an expert in the medical dermatology field, reviews facts concerning Pilomatrical Carcinoma and refers to further details obtained from a case study. Dr. Nakhla now runs his own practice in Orange County California, where he provides treatments for all dermatological needs, including acne, skin cancer detection, mole & wart removal, and skin allergy testing.



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Pilomatrical Carcinoma: A Case Report by Dr. Tony Nakhla of OC Skin Institute Document Transcript

  • 1. Pilomatrica Ca noma: I rci Case Report Review and of the Literature Tony Nakhla, DO; Michael Kassardjian, DO carcinoma a raremalignant Pilomatrical is tumorthat originates from hairmatrixcells. Pilomatrical carcinoma may arisede novo as a solitarylesion, through transformation or from its benign pilomatrixoma. counterpart, Differentiationbetweenpilomatrixoma and pilomatrical carcinoma requiresclose histologic and often is difficult. examination pilomatrical Although uncommon, carcinoma the potential metastasize; has to therefore,promptdiagnosis appropriate and manage- ment is essential. ilomatrical carcinoma is the malignant In som e areas, the lesional cells are relatively bland and counterpart of pilomatrixoma, a benign noninfiltrative appearirg. cutaneous tumor originating from the hair However, this case also shorvs areas with larger more ma[rix. It is a rare, aggressive tumor with a squamoid appearing cells urth aLyprcalfeatures, includ- high probability of recurrence after simple irg Iargenuclei with prominent nucleoli as well as areasof excision, and the potential to metastasrze. infiltrative appearing cells, features highly concerning for We report a case of a 56-year-old white man malignancy (Figure 3). In the infiltrative appearrngarea, diagnosed with pilomatrical carcinoma. The patient there is dense stromal sclerosis associated u-ith highly presented with a 2-month history of ar1 enlarging atyprcal squamoid and spindle cells, with ser-eralmitotic asymptomatic growth on the cheek. Physical exami- figures found within these cells (Figure +) In many nation revealed a 2-cm, well-demarcated, nontender, areas of the biopsy, there is granulomatolls inflamma- moveable, hard subcutaneous nodule on the right tion, hemorrhage , and granulation tissue consistent mandible (Figure l). No skin changes or lymphad- with a reaction to ruptured material from the tumor enopathy was noted. The clinical diagnosis strongly (Figure 5) While the latter findings often are seen in favored a calcified epidermoid cyst or other benign ruptured pilomatrixoma, the infiltratn-e areas with adnexal tumor. An excisional biopsy was performed at atyprcal spindle cells would not be erpected in a the request of the patient. benign pilomatrixoma, and the findings are most con- Sections were evaluated histologically and revealed sistent with a diagnosis of malignant pliomarrixoma a multifragmented biopsy of dermal and subcutaneous (pilom afitcal carcinoma) . tissue containing basaloid proliferation with collections Multiple laboratory tests using immunohrstochemi- of ghost cells, typical of pilomatrixoma (Figure 2). cal stains, including p63, cytokeratrn i/6, synap- tophysin, p53, and Ki-67 also -ere rer-iewed. The Dr. I'laLthla is from OC Shin Institute, Santa Ana, California. tumor cells were strongly and diffusely- positive for Dr. Kassardlian an intern, PacificHospital,LongBeach, is California. p63, highlighting the nuclei of the infiltrative and The authors report no conflict tf interest in relation to spindle cells, which is positirre in mos[ primary cuta- thisarticle. neous malignancies including adnexal carcinomas. In Michael Kassardjian,DO, PO Box 2152, Correspondence: addition, results of cytokeratin 5/6 staining aiso were Pen,CA 90275 (miheygh@gmail.com). Verdes Palos moderately positive within lesional cells, including the 3I4 . JULY Dermatology@ Cosmetic . 2010 vol. 23 No. 7 www.cosderm.com
  • 2. Fi gure 1. A 56-year-ol dw hi te man w i th a 2-cm, well-demarcated, nontender, move- abl e, hard subcutaneous nodul e presenton the ri ght mandi bl ew i th no ski n changes. infiltrative-appearing spindle cells, which confirmed of the tumors may vary from soft and friable to firm. that these were epithelial, and not mesenchymal, They may have red, yellow, white, and tan skin ceiis R.esults of synaptophysin staining were nega- changes. Lesions cannot reliably be distinguished [l-e and not consistent with a neuroendocrine tumor based solely on clinical appearance, and frequently such as ierkel cell carcinoma. Staining for p53 was are mistaken for epidermal cysts. The diagnosis of r.,'eakir b'-ri difiusely positive throughout the tumor cell pilomatrical malign ancy is made exclusively by careful nuclei. rnc^'-rd.rnE the infiltrative areas, a finding that histologic evaluation. also far-ored :rahgnancy. In addition, Ki-67 positivity Pilomatricalcarcinomahas a potential to metastasrze was high u ithrn thre basaloid cells and also positive in about 10o/o cases.5 of Cases metastasis the lung, of to within man c[ rhe spindle cells, highlighting up to bones, and lymphatics, ds well as invasion into the L}o/o of the entrre lesion. Thus, the overall histologic cranialvault, have been reported.3 and immunohistochemical findings supported the diagnosis of pilomalncal carcinoma. EPIDEMIOLOGY The epidemiology of pilomatrical carcinoma differs COMMENT from pilomatrixomas. Pilomatrixomas more often are Pilomatrixoma first -as de scribed in 1BB0 by Malherbe seen in women (female to male ratio of 3: I ) and and Chenantaisr as a calci.it'tng epithelioma that was tend to occur in patients younger than 20 years. The thought to originate from the sebaceous gland. In mean age of patients diagnosed with pilomatrixoma is L949, Lever and Griesemerr suggested that the actual B .7 years, rangirg from B months to 19 years.5 origin of the tumor was the harr matrix.3 Thus, the Pilomatrixomas occur most commonly on the head, approprlaLe term pilomatrtxonta was adopted, synony- followed by the upper extremities, neck, trunk, and mous with calcifying epithehoma of Malherbe, which lower extremities.3 Involvement of the face has been also is commonly used. reported in the frontal, temporal, cheek, periorbital, Clinically, the tumor is described as a solitary, slow and preauricular regions.6 Pilomatrical carcinomas are growing, asymptomatic, dermai or subcutaneous mass more predominant in men and more often middle- that mosl commonly is found in the posterior neck, aged or elderly adults. The mean age of patients with upper back, and preauricular area. Duration of tumors pilomat.rical carcinoma is 48 years, ranging from 2 to prior to surgery has been reported to range from BB years, and in this population are more common 4 months to 10 years.3 Pilomatrical carcinomas have in the posterior neck, upper back, and preauricu- been reported to range in size from 0.5 cm to 20 cffi, lar area.3'4Approximately 60o/o of tumors have been with a mean of 3.95 cm, which is slightly larger than its located on the head, among which half are in the benign counterpart, pilomatrixoma.a The consistency preauricular region.T www.cosderm.com vol. 23 No. 7 . JULv2010. Cosmetic Dermatology@ 315
  • 3. PnouATRIcnr CaRcINoMA Figure 2. A fragrnented biopsy specimen revealed b as aloidpro l i f e r a t i o n n d g h o st ce lls ( H&E, r ig in a l a o magnif ic at i o nx 1 0 0 ) . Fi gure 3. I n f i l t r a t i v e s q u a m o id a n d sp in d le ce lls w it h at y pi c a l f e a t u r e s , i n c lu d in g la r g e n u cle i w it h promin e n t n u c l e o l i ( H & E,o r ig in a l m a g n ifica - ti o n x 400). HISTOPATHOLOGY carcinoma of the skin, and mixed tumors of the skin.7 The histologic differential diagnosis of pilomatrical Pilomatrical carcinomas have the characteristic features carcinoma includes pilomatrixoma, squamous cell of epithelial islands of pleomorphic basaloid cells with carcinoffi?, trichoepithelioma, ly-phoepitheliomalike vesicular nuclei and prominent nucleoli. Shadow or . JULY Dermatology@ 316 Cosmetic . 2010 vot-.23 No. 7 www.cosderm.com
  • 4. PnouATRrcAL CARCTNoMA l Figure4. Stromalsclerosis, highly atypicalsqua- moid and spindlecells, and severalmitoticfigures (H&E, original X400). magnification Figure5. Areas hemorrhage granulation of and tis- suesurrounded infiltrative by atypical spindlecells (H&E, original X400). magnification ghost cells, along with zones of necrosiswith surround- of retained nuclei from basaloid cells to the anucleate, ing stromaldesmoplasia also are observed.The basaloid eosinophilicshadow cells often is seen.8 Tumor necrosis cellshave deeplybasophilicoval or round nuclei and are usually is present, as well as frequent atypical mitotic found at the periphery of the islands.A transition zorae figures.Basaloidcells may infiltrate the entire dermis and www.cosderm.com VOL.23 NO. 7 . JULY2010 . Cosmetic Dermatology@ 317
  • 5. PrlouATRrcAL CnncrNoMA extend into the subcutaneous fat, deep fascia,and skel- calcifications, while the inhomogeneous signal intensi- muscle. In pilomatrical carcinoma,the shadow cells etal. ties related to varying degrees of tumor proliferation. tend to form a nested pattern, instead of the flat sheet- High signal intensity was atrributable ro cysric spaces like pattern usually observedin benign pilomatrixomas.3 forming in areas of tumor necrosis Histologic criteria for pilomatrical carcinoma include Pilomatrical carcinoma is a rare malignant form vessel invasion,mitotic index, apoptoticcount, aswell as of pilomatrixoma, which arises from hair matrix molecular markersof cell death and adhesion.e cells. Careful histologic evaluation is necessary to distinguish benign pilomarrixoma from pilomarri- IMMUNOHISTOCHEMISTRY cal carcinoma. Pilomatrical carcinoma rnay arise Immunohistochemical studies have not d.finirively de novo or from a preexisting benign pilomatrixoma, distinguished the markers that differentiate piloma- which may be clinically indistinguishable. In cases trixomas from pilomatrical carcinomas . Lazar et alI0 where previously excised or curetted pilomatrixomas studied a series of 15 pilomatrical carcinomas and recur, a reexcision with careful histologic evaluation 13 benign pilomatrixomas to assess expression of is indi cated.T B,-catenin using immunohistochemical staining and Pilomatrical carcinoma occurs more often in middle- DNA sequencing of exon 3 from the Bl-catenin gene, aged to older individuals, more commonly in men, CTNNBI, the defect that leads to the expression of and has a predilection for the posterior neck, upper pilomatrixomas. B-Catenin is a downstream effector in back, and preauricular area. Pilomatrical carcinomas the Wnt signaling pathway that signals for proliferarion frequently recur; however, treatment with wide local and differentiation. Mutations in the CTNNBI gene excision or Mohs micrographic surgery has been encoding B-catenin are present in both benign and shown to lower the raLe of recurrence.4,5 malignant neoplasms. A11 cases showed nuclear local- Distant metastases have been reported in up to l0o/o tzatton of B-catenin, mutations on exon 3, as well as of cases.5Due to the potential for metastasis, prompt expression of nuclear cyclin D 1 . Howev er, 2 pilomatri- diagnosis followed by wide local excision or Mohs caI carcinomas exhibited accumulation of p53, which micrographic surgerl- and close clinical and radiologic was absent in aIL 13 benign pilomatrixomas. I0 Past follow-up is recommended. studies also have reported high constant expression of CD44v6 and P-cadherin. 11 REFERENCES 1. Malherbe A, ChenantaisJ. ore sur lepirheliome calcifie des glandes sebaces.ProgJIed LSS0:325-837. TREATMENT 2. Lever Wf; Griesemer RD. Calficnng epithelioma of Malherbe; The most widely reported treatment for pilomatrical report of 15 cases,riith ccT-nrnenis its differentiation from cal- on carcinoma is wide local excision with histologically cified epidermal cyst anC on iis histogenesis. Arch Derm Syphilol. confirmed clear margins. Because pilomatrtcal carcinoma L949;59:506-5 18. 3. sau B Lupton GB Graham JH. Pilomatrix carcinoma. cancer. is identifiable by hematoxylin and eosin srain, Mohs L993:7 L:249L-2498. micrographic surgery also is an excellent treatment 4. Niwa T, Yoshida T. Doiuchi T, et al. Pilomatrix carcinoma of the option. Currently, there is no consensus on surgical man- axtlla CT and MRI features. J Radiol.20a5;78:257-260. Br 5. ScheinfeldN. Pilomatrical carcinoma:a case a patient with HIV in agement, and standard excisional margins have not been and hepatitisC. Dermatol Online 2008;L4:4. J. defined.s Adjuvant radiation therapy may be necessary 6. Yencha MW Head and neck pilomatricoma in the pediatric age postexcision. Chemotherapy has been used in cases of group: a retrospective study and literature review. Int J Pediatr extensive tumor invasion and in cases of metastasis. Otorhinolaryngol. I ;57'.123-L28. 200 7. Barbosa A, Guimaraes N, Sadigursky M. Pilomatrix carcinoma Appropriate Iaboratory testing includes liver func- (malignant pilomatricoma): a casereport and revlew of literature. tion tests, calcium levels, and chest x-ray examination. AnatsBrasileiro de D ermatolo s gia. 2000 ;75 :5BI - 5B5 . If aggressive local invasion is suspected, a computed B. Sassmannshausen Chaffins M. Pilomatrix carcinoma: a repori J, of a case arising from a previously excised pilomatrixoma and a tomography scan or magnetic resonance imaging review of the literature.J Am Acad Dermatol.2001;44(suppl 2). should be performed to define tumor extension.T Past 358-36r. studies have found that the radiologic findings of pilo- 9. omidi AA, Bagheri R, Tavassollan H. Pilomatrix carcinoma matrixoma typically demonstrate a well-circumscribed with subsequent pulmonary metastases: case report. Tanaffos. a 20065:57-60. lesion with homogeneous or sandlike calcifications on 10. Lazar AJ, Calonje E, Grayson W Pilomatrix carcinomas contain plain radiograph and computed tomography studies.12 mutations in CTlNBl, the gene encoding beta-catenin.J Cutan Niwa et aIa reported a case of pilomatrical carcinoma Pathol.2005;32:I 48- L57. I l. Bassarova Nesland JM, SedloevT, et al. Pilomatrix carcinoma A,, of the axilla, which demonstrated a diffuse inhomoge- with lymph node metastesJ CutanPathol.2004;3I:330-335. . neous mass with cystic changes on magnetic resonance 12. De BeuckeleerLH, De Schepper AM, Neetens I. Magnetic reso- rmaging. Areas of low signal intensity corresponded to nance imaging of pilomatricoma. Eur Radiol. 1996;6.72-60, I 318 Cosmetic . JULv Dermatology@ . 2010 vol. 23 No. Z www.cosderm.com