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Pilomatricoma in the neck of an adult male

Keywords
Pilomatrixoma; Child; Head; Head and Neck neoplasms; Neoplasms

Pilomatricoma (also spelt Pilomatrixoma and known as Calcifying epithelioma of Malherbe), was first described by Malherbe and Chenantais11 Malherbe A, Chenantais J. Notes on calcifying epitheliomas of sebaceous glands. Prog Med. 1880;8:826. in 1880 as a calcified tumor, originating from sebaceous glands. Later, in 1961 Forbis and Helwig22 Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83(4):606-18. http://dx.doi.org/10.1001/archderm.1961.01580100070009. PMid:13700704.
http://dx.doi.org/10.1001/archderm.1961....
carried out histochemical and electron microscopic studies of pilomatricoma and deduced the cell of origin to be from the outer sheath cell of the hair follicle root. Pilomatricoma is often found in children and young adults, nonetheless, it can occur in any age group, with a higher incidence in females (female: male ratio 3:2).33 Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma): a statistical study. Arch Dermatol. 1973;108(4):532-4. http://dx.doi.org/10.1001/archderm.1973.01620250020004. PMid:4745286.
http://dx.doi.org/10.1001/archderm.1973....
The majority of affected individuals are Caucasians.33 Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma): a statistical study. Arch Dermatol. 1973;108(4):532-4. http://dx.doi.org/10.1001/archderm.1973.01620250020004. PMid:4745286.
http://dx.doi.org/10.1001/archderm.1973....
The tumor primarily affects children and adolescents with 40% of cases occurring before age 10 and 60% before age 20.44 Knight PJ, Reiner CB. Superficial lumps in children: what, when, and why? Pediatrics. 1983;72(2):147-53. http://dx.doi.org/10.1542/peds.72.2.147. PMid:6866598.
http://dx.doi.org/10.1542/peds.72.2.147...
The greatest incidence of this tumor is found between 8 and 13 years of age.

Clinically, the tumor usually appears as a firm solitary, painless, slow-growing nodule ranging from 0.5cm to 3cm in most cases.55 Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-8. http://dx.doi.org/10.1016/S0165-5876(00)00449-3. PMid:11165649.
http://dx.doi.org/10.1016/S0165-5876(00)...

6 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
-77 Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807. PMid:10485145.
http://dx.doi.org/10.1177/01455613990780...
The tumor is freely mobile within the relating surrounding tissue structures and overlying skin. They are commonly located in the head and neck region, followed by the upper extremities, the trunk, and the lower extremities.22 Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83(4):606-18. http://dx.doi.org/10.1001/archderm.1961.01580100070009. PMid:13700704.
http://dx.doi.org/10.1001/archderm.1961....
,33 Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma): a statistical study. Arch Dermatol. 1973;108(4):532-4. http://dx.doi.org/10.1001/archderm.1973.01620250020004. PMid:4745286.
http://dx.doi.org/10.1001/archderm.1973....
The usual locations in the head and neck region, are the neck and the frontal, temporal, periorbital, and periauricular areas of the head.66 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
,77 Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807. PMid:10485145.
http://dx.doi.org/10.1177/01455613990780...

Differential diagnoses of head and neck pilomatricoma include dermoid, epidermoid, and branchial cleft cysts, periauricular sinuses, adenopathy, salivary gland tumors, giant cell tumor, chondroma cutis, necrotizing granulomatous lymphadenitis and foreign body reactions.55 Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-8. http://dx.doi.org/10.1016/S0165-5876(00)00449-3. PMid:11165649.
http://dx.doi.org/10.1016/S0165-5876(00)...

6 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
-77 Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807. PMid:10485145.
http://dx.doi.org/10.1177/01455613990780...
Diagnosis of pilomatricoma can be made solely on the basis of histopathological features, typified by varying amounts of 2 components mixed together in a disorganized fashion in the dermis, that is anucleate pink “ghost” cells (sheets of dead keratinocytes in which the ghost/shadow outline of each individual cell can still be seen, and aggregates or sheets of small round blue cells representing a germinative/matrical epithelial component (similar to the round blue cells seen in the hair bulb/roots of normal hair follicles).88 Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA. PMid:30638401.
http://dx.doi.org/10.5858/arpa.2018-0189...
Each tumor nodule is often surrounded by brisk granulomatous inflammation, fibrosis, and foreign body giant cell reaction to keratin of the anucleate ghost cells.88 Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA. PMid:30638401.
http://dx.doi.org/10.5858/arpa.2018-0189...
Calcifications are often present and metaplastic bone formation can occur.88 Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA. PMid:30638401.
http://dx.doi.org/10.5858/arpa.2018-0189...
Mitoses may be quite frequent in the round blue cell matrical component, but nuclear pleomorphism should not be seen.88 Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA. PMid:30638401.
http://dx.doi.org/10.5858/arpa.2018-0189...
Brisk mitotic activity alone should not be regarded as evidence of malignancy.88 Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA. PMid:30638401.
http://dx.doi.org/10.5858/arpa.2018-0189...

These tumors are typically solitary, but multiple tumors have been reported. Multiple pilomatricomas are seen in patients with Gardner syndrome (the extraintestinal variant of familial adenomatous polyposis)99 Cooper PH, Fechner RE. Pilomatricoma-like changes in the epidermal cysts of Gardner’s syndrome. J Am Acad Dermatol. 1983;8(5):639-44. http://dx.doi.org/10.1016/S0190-9622(83)70071-X. PMid:6863619.
http://dx.doi.org/10.1016/S0190-9622(83)...
and as a cutaneous sign of myotonic dystrophy.1010 Chiaramonti A, Gilgor RS. Pilomatricomas associated with myotonic dystrophy. Arch Dermatol. 1978;114(9):1363-5. http://dx.doi.org/10.1001/archderm.1978.01640210058018. PMid:686751.
http://dx.doi.org/10.1001/archderm.1978....
Malignant transformation of pilomatricoma is rare,66 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
and the standard treatment is by complete surgical excision.22 Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83(4):606-18. http://dx.doi.org/10.1001/archderm.1961.01580100070009. PMid:13700704.
http://dx.doi.org/10.1001/archderm.1961....
,55 Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-8. http://dx.doi.org/10.1016/S0165-5876(00)00449-3. PMid:11165649.
http://dx.doi.org/10.1016/S0165-5876(00)...

6 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
-77 Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807. PMid:10485145.
http://dx.doi.org/10.1177/01455613990780...
,1111 Agarwal RP, Handler SD, Matthews MR, Carpentieri D. Pilomatrixoma of the head and neck in children. Otolaryngol Head Neck Surg. 2001;125(5):510-5. http://dx.doi.org/10.1067/mhn.2001.117371. PMid:11700451.
http://dx.doi.org/10.1067/mhn.2001.11737...
Recurrence of this tumor is rare after excision, with an incidence of 0-3%.22 Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83(4):606-18. http://dx.doi.org/10.1001/archderm.1961.01580100070009. PMid:13700704.
http://dx.doi.org/10.1001/archderm.1961....
,55 Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-8. http://dx.doi.org/10.1016/S0165-5876(00)00449-3. PMid:11165649.
http://dx.doi.org/10.1016/S0165-5876(00)...

6 Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481. PMid:11735819.
http://dx.doi.org/10.1001/archotol.127.1...
-77 Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807. PMid:10485145.
http://dx.doi.org/10.1177/01455613990780...
,1111 Agarwal RP, Handler SD, Matthews MR, Carpentieri D. Pilomatrixoma of the head and neck in children. Otolaryngol Head Neck Surg. 2001;125(5):510-5. http://dx.doi.org/10.1067/mhn.2001.117371. PMid:11700451.
http://dx.doi.org/10.1067/mhn.2001.11737...

The images sum up a 22-year-old man who presented with a right lateral subcutaneous neck nodule, which was not affixed to overlying skin and underlying related tissue structures. The mass was excised and submitted to histopathological examination.

Gross examination revealed a 200g nodule with a normal overlying ellipse of skin measuring 7x5x3cm and 9x6cm, respectively. Sectioning the mass disclosed a well-circumscribed firm grey-white chalky lesion measuring 6.5x3cm (Figure 1A). Histopathological examination of the lesion showed biphasic dermal lesion composed of islands of anucleate “ghost” cells (Figure 1B and 1C) and islands of small round blue cells (Figure 1B and 1C) with abrupt trichilemmal type keratinization (Figure 1C). In areas, the islands of anucleate ghost cells are surrounded by foreign body type giant cells in response to keratin of the ghost cells (Figure 1B, and 1D). Elsewhere calcification is present (Figure 1D). Based on these classic features, a diagnosis of Pilomatricoma was made.

Figure 1
A – macroscopic view of the cut surface of the nodule, disclosing a well-circumscribed firm chalky appearance; B and C – photomicrographs showing biphasic anucleate pink ghost cells (white star), lined by foreign body type giant cells (black arrowhead) admixed with islands of matrical/germinative epithelial component (small round blue cells) (red arrowhead) with abrupt trichilemmal type keratinization(white arrows) (H&E 40X); D – Photomicrograph showing islands of anucleate ghost cells (white star) with associated foreign body type giant cells (black arrowhead). Focus of calcification is present (black arrow) (H&E 40X).

The post-operative period was uneventful and at the last follow-up, no recurrence of the nodule was noted.

  • How to cite: Ulzen-Appiah K. Pilomatricoma in the neck of an adult male. Autops Case Rep [Internet]. 2022;12:e2021379. https://doi.org/10.4322/acr.2021.379
  • This study carried out at the ACT Pathology Consult, Cape Coast, Ghana.
    Ethics statement: Informed consent was sought and signed by next of kin authorizing the autopsy and data publication.
  • Financial support: None.

REFERENCES

  • 1
    Malherbe A, Chenantais J. Notes on calcifying epitheliomas of sebaceous glands. Prog Med. 1880;8:826.
  • 2
    Forbis R Jr, Helwig EB. Pilomatrixoma (calcifying epithelioma). Arch Dermatol. 1961;83(4):606-18. http://dx.doi.org/10.1001/archderm.1961.01580100070009 PMid:13700704.
    » http://dx.doi.org/10.1001/archderm.1961.01580100070009
  • 3
    Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma): a statistical study. Arch Dermatol. 1973;108(4):532-4. http://dx.doi.org/10.1001/archderm.1973.01620250020004 PMid:4745286.
    » http://dx.doi.org/10.1001/archderm.1973.01620250020004
  • 4
    Knight PJ, Reiner CB. Superficial lumps in children: what, when, and why? Pediatrics. 1983;72(2):147-53. http://dx.doi.org/10.1542/peds.72.2.147 PMid:6866598.
    » http://dx.doi.org/10.1542/peds.72.2.147
  • 5
    Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-8. http://dx.doi.org/10.1016/S0165-5876(00)00449-3 PMid:11165649.
    » http://dx.doi.org/10.1016/S0165-5876(00)00449-3
  • 6
    Danielson-Cohen A, Lin SJ, Hughes CA, An YH, Maddalozzo J. Head and neck pilomatrixoma in children. Arch Otolaryngol Head Neck Surg. 2001;127(12):1481-3. http://dx.doi.org/10.1001/archotol.127.12.1481 PMid:11735819.
    » http://dx.doi.org/10.1001/archotol.127.12.1481
  • 7
    Thomas RW, Perkins JA, Ruegemer JL, Munaretto JA. Surgical excision of pilomatrixoma of the head and neck: a retrospective review of 26 cases. Ear Nose Throat J. 1999;78(8):541-8. http://dx.doi.org/10.1177/014556139907800807 PMid:10485145.
    » http://dx.doi.org/10.1177/014556139907800807
  • 8
    Fulton EH, Kaley JR, Gardner JM. Skin adnexal tumors in plain language: a practical approach for the general surgical pathologist. Arch Pathol Lab Med. 2019;143(7):832-51. http://dx.doi.org/10.5858/arpa.2018-0189-RA PMid:30638401.
    » http://dx.doi.org/10.5858/arpa.2018-0189-RA
  • 9
    Cooper PH, Fechner RE. Pilomatricoma-like changes in the epidermal cysts of Gardner’s syndrome. J Am Acad Dermatol. 1983;8(5):639-44. http://dx.doi.org/10.1016/S0190-9622(83)70071-X PMid:6863619.
    » http://dx.doi.org/10.1016/S0190-9622(83)70071-X
  • 10
    Chiaramonti A, Gilgor RS. Pilomatricomas associated with myotonic dystrophy. Arch Dermatol. 1978;114(9):1363-5. http://dx.doi.org/10.1001/archderm.1978.01640210058018 PMid:686751.
    » http://dx.doi.org/10.1001/archderm.1978.01640210058018
  • 11
    Agarwal RP, Handler SD, Matthews MR, Carpentieri D. Pilomatrixoma of the head and neck in children. Otolaryngol Head Neck Surg. 2001;125(5):510-5. http://dx.doi.org/10.1067/mhn.2001.117371 PMid:11700451.
    » http://dx.doi.org/10.1067/mhn.2001.117371

Publication Dates

  • Publication in this collection
    02 May 2022
  • Date of issue
    2022
Hospital Universitário da Universidade de São Paulo Hospital Universitário da Universidade de São Paulo, Av. Prof. Lineu Prestes, 2565 - Cidade Universitária, 05508-000 - São Paulo - SP - Brasil, (16) 3307-2068, (16) 3307-2068 - São Paulo - SP - Brazil
E-mail: autopsy.hu@gmail.com