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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 28  |  Issue : 3  |  Page : 239-241

Trichilemmal carcinoma of pinna: A rare presentation


1 Department of Otorhinolaryngology and Head & Neck Surgery, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India
2 Department of Pathology, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal, India

Date of Submission02-Jun-2021
Date of Acceptance19-Oct-2021
Date of Web Publication21-Nov-2022

Correspondence Address:
Dr. Meera Niranjan Khadilkar
Department of Otorhinolaryngology and Head and Neck Surgery, Kasturba Medical College, Mangalore, Manipal Academy of Higher Education, Manipal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.indianjotol_81_21

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  Abstract 


Trichilemmal carcinoma is a rare malignancy of hair follicles affecting sun-exposed and hair-bearing skin; occurrence over pinna is unusual. This case report describes one such uncommon presentation in a 71-year-old man who came with a pinna swelling clinically mimicking basal cell carcinoma. Diagnosis is an arduous task due to its close resemblance with more common cutaneous tumors. Misdiagnosis or delay in intervention may lead to recurrence and local metastasis.

Keywords: Adnexal and skin appendage neoplasms, ear auricle, ear neoplasms, external ear


How to cite this article:
Dosemane D, Khadilkar MN, Ventakataramana CG, Bajpai S, Samanth R. Trichilemmal carcinoma of pinna: A rare presentation. Indian J Otol 2022;28:239-41

How to cite this URL:
Dosemane D, Khadilkar MN, Ventakataramana CG, Bajpai S, Samanth R. Trichilemmal carcinoma of pinna: A rare presentation. Indian J Otol [serial online] 2022 [cited 2022 Dec 6];28:239-41. Available from: https://www.indianjotol.org/text.asp?2022/28/3/239/361647




  Introduction Top


Trichilemmal carcinoma (TC) is an exceptionally rare cutaneous adnexal tumor.[1] It is a slow-growing locally invasive malignant tumor affecting older individuals, with a predilection for sun-exposed hair-bearing skin.[2] Due to close resemblance clinically and histologically with malignant skin neoplasms such as squamous cell and basal cell carcinoma,[3] these cases may often go misdiagnosed. This case report describes an unusual presentation of such a lesion over the pinna and the treatment strategy.


  Case Report Top


A 71-year-old man presented to the otolaryngology department with growth over the right pinna for 5 years [Figure 1]a; it was insidious in onset and rapidly progressed over the past 6 months. There was no fever, pain over the swelling, bleeding, or discharge from the swelling. The patient had no history of trauma, burns, prior surgery, or radiation exposure. He was a known hypertensive under medication for 2 years. Local examination revealed a solitary swelling of size 2 cm × 3 cm over the right upper one-third of the lateral surface of pinna; it was firm, globular with a broad base, non-tender, fixed, did not bleed on touch, with well-defined margins. The skin over the swelling was hyperpigmented with an irregular fissured surface. The swelling extended superiorly to the helical margin involving superior crus of antihelix and scaphoid fossa, inferiorly up to 0.5 cm above the cymba concha, adhering to the underlying skin. Lymph node and systemic examination were normal. Routine blood investigations were within normal limits. The patient underwent wedge excision of the lesion with underlying cartilage and a 5-mm margin under local anesthesia [Figure 1]b. Primary closure was achieved, though with a slightly deformed pinna [Figure 1]c. Histopathologic examination showed a well-defined lesion containing lobules of tumor cells continuous with the overlying epidermis [Figure 2]. It consisted of outer basaloid cells and inner clear cells with focal palisading in the periphery. Few cells showed nuclear pleomorphism with increased mitosis. TC was the final diagnosis. The patient was followed up on a 3 monthly basis for 2 years and had no signs of recurrence.
Figure 1: Photograph of (a) solitary swelling (white arrow) over the upper one-third of the lateral surface of the right pinna with a hyper-pigmented fissured surface (b) planned wedge excision with adequate margins (c) right pinna 3 months postoperatively

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Figure 2: Histopathological photograph showing (a) proliferating interconnected lobules of epithelial cells (*) in the dermis with underlying cartilage (H and E, ×4) (b) lobular proliferation of pale eosinophilic cells with squamous morules (#) (H and E, ×10)

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  Discussion Top


Malignant hair follicle tumors account for 1% of adnexal carcinomas.[4] TC is a malignancy of hair follicles derived from or differentiating toward the external root hair sheath. It may arise from its benign counterpart, “trichilemmoma;” risk factors include prolonged low-dose irradiation, actinic exposure.[3] There are reports suggesting its association with trauma and burn scars, genetic disorders, but the pathogenesis remains unclear.[5] TC shows no gender preponderance; it predominantly affects the elderly with a high predilection for sun-exposed surfaces of the skin.[6] TC usually follows a nonaggressive indolent course mostly limited to the scalp, forehead and neck, trunk, or upper limbs.[1] Hamman and Shiang identified 103 cases with TC in a literature review; only 9% of cases were seen involving the ear;[5] this may be attributed to the presence of scanty hair follicles and the tightly adherent overlying skin in the pinna.

Clinical presentation varies and may mimic squamous or basal cell carcinoma, nodular melanoma, or keratoacanthoma.[4] Lesions may be ulcerated, crater-like, beaded, or scaly,[5] as seen in the present case. Due to its slow-growing nature, TC is often neglected until a period of abrupt growth, necessitating medical evaluation.[1] However, it typically behaves as a benign tumor amenable to surgical resection.

Histopathology plays a vital role in differentiating TC from its mimics. Headington in 1976 defined TC as a “histologically invasive, cytologically atypical clear cell neoplasm of adnexal keratinocytes, in continuity with the epidermis and/or follicular epithelium.” The glycogen-abundant tumor cells show peripheral palisading with a periodic acid–Schiff reactive, diastase-sensitive basement membrane, and trichilemmal keratinization.[7] Many histopathologists consider TC a clear cell variant of squamous cell carcinoma.[8] Immunohistochemistry (IHC) shows CD34 negativity.[2] However, IHC was not required in the present case.

A surgical margin of at least 1 cm is recommended.[1] Over the past few years, Mohs micrographic surgery has proven to lessen recurrence rates, conserving the surrounding normal tissue. Trapezoid skin flap from mastoid region was performed in a similar case with excellent postoperative outcome.[6]

Since it is considered a low-grade malignancy, with limited reports of metastases and local spread to cervical lymph nodes, it is advisable to treat TC as nonmelanoma skin cancers.[1],[4],[8] In the present case, even though only a 5-mm margin was feasible due to the unusual location of the tumor, complete surgical clearance was achieved; no recurrence or metastasis was noted. Although recurrence rates are meager, regular follow-up of the patient in the postoperative period is necessary for early detection of distant metastasis and recurrence.[1] In cases with distant metastasis, adjuvant radiation[7] or systemic chemotherapy (four cycles of cyclophosphamide and cisplatin) may be considered to diminish the progress of the disease.[1] Topical imiquimod cream, an immunomodulator, has also been successfully used to treat locally confined lesions with promising outcomes.[7]


  Conclusion Top


TC is a rare malignant neoplasm of the hair follicles, highly unusual in the pinna, as seen in this case report. This entity must always be kept in mind as an elementary differential, mimicking malignant tumors of the skin and its appendages. Total surgical excision with adequate clearance of margins is the standard treatment; recurrence rates are low.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Zhuang SM, Zhang GH, Chen WK, Chen SW, Wang LP, Li H, et al. Survival study and clinicopathological evaluation of trichilemmal carcinoma. Mol Clin Oncol 2013;1:499-502.  Back to cited text no. 1
    
2.
Sofianos C, Chauke NY, Grubnik A. Metastatic trichilemmal carcinoma in a patient with breast cancer. BMJ Case Rep 2016;2016:bcr2016217661.  Back to cited text no. 2
    
3.
Ha JH, Lee C, Lee KS, Pak CS, Sun CH, Koh Y, et al. The molecular pathogenesis of trichilemmal carcinoma. BMC Cancer 2020;20:516.  Back to cited text no. 3
    
4.
Maya-Rico AM, Jaramillo-Pulgarín C, Londoño-García Á, Peña-Zúñiga B. Locally aggressive trichilemmal carcinoma. An Bras Dermatol 2018;93:579-81.  Back to cited text no. 4
    
5.
Hamman MS, Brian Jiang SI. Management of trichilemmal carcinoma: An update and comprehensive review of the literature. Dermatol Surg 2014;40:711-7.  Back to cited text no. 5
    
6.
Bhargava S, Feliciani C, Longo R, Dominici MM. Two-stage ear reconstruction with a retroauricular skin flap after excision of trichilemmal carcinoma. J Cutan Aesthet Surg 2020;13:149-51.  Back to cited text no. 6
  [Full text]  
7.
Kannan K, Mahesh KC, Manickam N, Ramdas A. Trichilemmal carcinoma of scalp masquerading as squamous cell carcinoma: A report of a rare case with histogenesis and literature review. Int J Trichol 2020;12:82-5.  Back to cited text no. 7
  [Full text]  
8.
Craig PJ. An overview of uncommon cutaneous malignancies, including skin appendageal (adnexal) tumours and sarcomas. Clin Oncol 2019;31:769-78.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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