الرابط:
https://ejo.springeropen.com/articles/10.1186/s43163-023-00432-0
Unilateral auricular multiple
trichoepitheliomas: a case report and review of literature
· Raid M. Al-Ani &
The
Egyptian Journal of Otolaryngology volume 39,
Article number: 63 (2023) Cite this article
·
49 Accesses
Abstract
Background
Trichoepithelioma is a
rare benign tumor. It could be an inherited or acquired condition.
Trichoepithelioma has mostly involved the face with bilateral multiple lesions.
Involvement of the auricle by this tumor is extremely rare encountered in
clinical practice.
Case presentation
A 36-year-old female
presented to the Dermatology Clinic with painless right auricular papules
4 years ago. Examination revealed multiple non-tender, firm, rounded,
dome-shaped, flesh-colored, pink, and shiny papules. There was mild bleeding
from the lesions after trivial trauma. There was no family history of similar
problem. No abnormality was found on other examinations. An excisional biopsy
of one lesion was performed under local anesthesia. The histopathological
evaluation confirmed the diagnosis of trichoepithelioma. The lesions under
local anesthesia were excised with primary closure of the wound. No recurrence
was seen during 2 years of follow-up.
Conclusion
Only five cases of trichoepitheliomas that
affected the auricle were reported in the PubMed database. Four of them are
affecting both auricles as well as other parts of the body, particularly the
face. The fifth case was with a unilateral single giant auricular lesion. Our
case was with non-familial multiple unilateral auricular trichoepitheliomas.
The tumors responded well on surgical excision and primary closure.
Background
Trichoepithelioma (TE)
is a rare benign (harmless) tumor that originates from the walls of the hair
follicle after puberty. Trichoepithelioma is mostly seen in the face, nose,
scalp, and/ or upper lip. It is characterized by the benign proliferation of
epithelial-mesenchymal origin cells [1]. TEs are categorized into three types:
solitary, multiple (2 types), and desmoplastic [2, 3]. Multiple familial TE type 1 is an
inherited disorder that generally progresses through adolescence and over time
they grow larger and increase in number. The genetic disorder can be triggered
by alterations in the CYLD gene or by changes in other genes which are quite
unknown. Meanwhile, the multiple familial TE type 2 is an acquired mutation
which is essential for the tumors to develop [4, 5]. The tumors affect women more frequently
and severely than men, and they can be mutilating and cause psychological
problems [3]. The tumors signify benign hamartomas of
the pilosebaceous apparatus. TEs have tumor islands of basaloid cells, horn
cysts, and immature hair papillae [5].
The rare incidence and absence of enough signs
made TE management a controversial domain in dermatologic surgery. Few cases
involving the auricle have been reported [6,7,8,9]. In all these reported cases, the
distribution of the tumors was on both auricles as well as the involvement of
the whole face, apart from the study by Genc et al. which reported a left giant
single auricular TE. Hence, our case differs from the abovementioned reported
cases in that the tumors were multiple and involved the right auricle only.
Case
presentation
A 36-year-old healthy
woman presented to the Dermatological Private Clinic with right auricular
papules of 4 years duration. Physical examination showed multiple
asymptomatic firms, rounded, dome-shaped, flesh-colored, pink, and shiny
papules that increase in number. It involved only the right side outer ear
canal (Fig. 1). The condition is sometimes associated with
slight bleeding after mild trauma. There was no family history of the same
condition. The rest of the ear, nose, throat, and systemic examination were
normal. We put the following as a differential diagnosis of these lesions:
benign trichoblastomas, trichofolliculomas, trichoadenomas and
trichoepitheliomas, and basal cell carcinoma. An excisional biopsy of a single
lesion was performed and sent for histopathological examination. The report
shows keratinized stratified squamous epithelium strands of the epidermis
consisting of a superficial nest of small basaloid cuboidal cells overlying the
dermis. Many keratinous cysts have a marginal border of basaloid cuboidal
cells, and keratinous cysts are enclosed by thick fibrous stroma (Fig. 2). Therefore, we reached the diagnosis of
non-inherited multiple TEs of the auricle. Surgical excision of the lesions
with primary closure was performed by an otolaryngologist under local
anesthesia. No recurrence was observed at a 2-year follow-up. Informed consent
was obtained from the patient for case publication with the related images.
Fig. 1
Multiple papules that incompletely fill the
outer ear canal
Fig. 2
Tumor consisting chiefly of a nest of basaloid
cells with horn cyst (H&E; A with × 100, B with × 400)
Discussion
TEs are rare benign
tumors initiated from the pilosebaceous follicle. Typically, it affects females
in early childhood or early adolescence. In spite of that, several cases of
non-familial multiple TEs reported the involvement of adult women patients [10]. The exact prevalence of TEs is not yet
known. TEs are characterized by a slowly progressive increment in the size and
number of the lesion for long periods, and the usual presentation of the tumors
is for a cosmetic reason [11]. This case report presented a 36-year-old
woman lacking family history, complaining of right auricular TEs for
4 years duration. Table 1 shows the reported cases of TEs of the
auricle.
Table 1 Reported cases
of trichoepithelioma of the auricle in PubMed from 1990 to 2022
The patient in the
current study seeks medical advice because of their increased number of lesions
and frequent bleeding with slight trauma, unlike previous cases that were
presented to the medical clinics usually for cosmetic goals and to recover
their outlook. Others showed asymptomatic dense deformed tumors that cause
psycho-social problems [13, 14].
Basal cell carcinoma
(BCC) shares similar features to the TE except that it tends to affect older
age group individuals with a single ulcerative pigmented nodule as well as the
histology illustrates the presence of the mitotic figure, apoptotic cells,
retraction between epithelium and stroma, and ulceration. The present work
shows a young age patient with non-ulcerated multiple pink papules. While the
histological study was characterized by basaloid cells which develop from
undifferentiated germinative cells of the follicular-sebaceous unit and
keratinous cysts enclosed by thick fibrous stroma, these findings give a base
for the diagnosis of TEs. Besides, there are immunohistochemical methods that
may be worth distinguishing the two [15, 16]. However, this modality is not present in
our city as well as it is costly.
At the other end, the
TE shares histological features with trichoadenoma. However, this benign tumor
originates on the lines of the infundibular portion of the pilosebaceous unit
due to the existence of keratinized epidermoid cells in the cyst wall with
keratohyalin granules. Clinically, trichoadenoma presents as a single grayish
tumor, measures up to 15 mm in diameter, and affects the face or buttocks
[17].
TEs show a major
cosmetic worry and the treatments are challenging particularly in patients with
multiple auricular lesions similar to the presented case. Numerous modalities
have been useful in treating TEs such as surgery, electro destruction, ablative
resurfacing, obliteration by CO2 laser or Erbium-YAG laser, and topical
medications like imiquimod and rapamycin [18].
Surgical excision of
the TEs has been proven to be effective and prevent the deep damage of underlying
tissue. But TEs involving auricles had been reported in 3 cases that undergone
surgical removal that caused high rates of scarring and poor cosmetic outcome.
The laser irradiation that offers less pain and fast recovery was used for the
fourth patient but may give a bad outcome and scaring of the cartilage of the
auricle. However, management of multiple TEs is an extremely hard attempt and
all treatment methods carry a risk of side effects such as reddens swelling,
hyperpigmentation, recurrence, and sometimes scarring [11].
Although TEs are benign, they can quite rarely
experience malignant transformation to basal cell carcinoma or trichoblastic
carcinoma. The histopathological findings and immunohistochemistry if needed
have been considered for the differentiation [19].
Conclusion
TEs of the auricle are seldom to be seen in
daily clinical practice. Few cases of TEs involved the auricle that were
reported in the literature. All of them are bilateral and associated with other
lesions in the body except one case which was single, giant, and on one
auricle. The presenting case was with non-hereditary multiple TEs on one
auricle and without other lesions neither in the face nor other parts of the
body. Surgical excision of the lesion with primary closure was successful in
the presenting case.
Availability
of data and materials
All patientʼs data was presented in the
article.
References
1. Gupta A, Ali MJ,
Mishra DK, Naik MN (2015) Solitary trichoepithelioma of the eyelid: a
clinico-pathological correlation. Int J Trichology 7(2):80
2. Jedrych J, Leffell D,
McNiff JM (2012) Desmoplastic trichoepithelioma with perineural involvement: a
series of seven cases. J Cutan Pathol 39(3):317–323
3. Wang Q, Ghimire D,
Wang J, Luo S, Li Z, Wang H et al (2015) Desmoplastic trichoepithelioma: a
clinicopathological study of three cases and a review of the literature. Oncol
Lett 10(4):2468–2476
4. Dissanayaka D,
Dassanayaka DKB, Jayasooriya PR. Clinical, histopathological, and management
challenges of multiple familial trichoepithelioma: a case report of a patient
presenting with multiple facial papules. Case Rep Dent. 2020;2020. https://doi.org/10.1155/2020/5648647
5. Salhi A, Bornholdt D,
Oeffner F, Malik S, Heid E, Happle R et al (2004) Multiple familial
trichoepithelioma caused by mutations in the cylindromatosis tumor suppressor
gene. Cancer Res 64(15):5113–5117
6. Bibi CO, Fliss DM,
Avinoach I, Brenner H, Tovi F (1990) Multiple trichoepithelioma occluding both
external auditory canals. Head Neck 12(3):257–260
7. D’Souza M, Garg BR,
Ratnakar C, Agrawal K (1994) Multiple trichoepitheliomas with rare features. J
Dermatol 21(8):582–585
8. Kaluskar SK (2005)
KTP/532 laser excision of trichoepithelioma of the ear. Indian J Otolaryngol
Head Neck Surg 57(1):63–65
9. Genc S, Ugur SS,
Arslan IB, Demir A, Tuhanioglu B, Kuskonmaz I et al (2012) A giant solitary
trichoepithelioma originating from the auricle. Dermatologic Surg
38(9):1527–1528
10.Pariser RJ (1986)
Multiple hereditary trichoepitheliomas and basal cell carcinomas. J Cutan
Pathol 13(2):111–117
11.Mandekou-Lefaki I,
Theodosiou G, Delli F-S, Oikonomou D, Papageorgiou M. Multiple nonfamilial
unilateral trichoepitheliomas: report of a case—mini review of the literature.
Case Rep Dermatol Med. 2019;2019. https://doi.org/10.1155/2019/6821854
12.Sehrawat M, Jairath V,
Jain VK (2016) Nonfamilial multiple trichoepithelioma: few and far between.
Indian J Dermatol 61(1):78
13.Shaffelburg M, Miller
R (1998) Treatment of multiple trichoepithelioma with electrosurgery.
Dermatologic Surg 24(10):1154–1156
14.Zhang X-J, Liang Y-H,
He P-P, Yang S, Wang H-Y, Chen J-J et al (2004) Identification of the
cylindromatosis tumor-suppressor gene responsible for multiple familial
trichoepithelioma. J Invest Dermatol 122(3):658–664
15.Poniecka AW, Alexis JB
(1999) An immunohistochemical study of basal cell carcinoma and
trichoepithelioma. Am J Dermatopathol 21(4):332–336
16.Arits AHMM, Parren
LJMT, Van Marion AMW, Sommer A, Frank J, Kelleners-Smeets NWJ (2008) Basal cell
carcinoma and trichoepithelioma: a possible matter of confusion. Int J Dermatol
47:13–17
17.Huet P, Jegou MH,
Bourlond F, Dupin C, Cribier B (2020) Anatomoclinical and dermatoscopic study
of trichoadenoma. Annales de Dermatologie et de Venereologie. pp 334–9
18.McGee JS, Suchter MF,
Milgraum SS (2016) Multiple Familial Trichoepithelioma Successfully Treated
With CO 2 Laser and Imiquimod. Skinmed 14(6):467–468
19.Karimzadeh I, Namazi
MR, Karimzadeh A (2018) Trichoepithelioma: a comprehensive review. Acta
Dermatovenerologica Croat 26(2):162
