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| ORIGINAL ARTICLE |
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| Year : 2021 | Volume
: 9
| Issue : 3 | Page : 123-127 |
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Eyelid trichoblastoma – A case series
Gunja Chowdhury1, Meghana Tanwar1, Usha Kim1, Shanthi R Krishnan2
1 Department of Orbit, Oculoplasty and Ocular Oncology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
2 Department of Pathology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
| Date of Submission | 06-Oct-2020 |
| Date of Decision | 19-Apr-2021 |
| Date of Acceptance | 03-May-2021 |
| Date of Web Publication | 27-Sep-2021 |
Correspondence Address:
Meghana Tanwar
Aravind Eye Hospital, No. 1, Anna Nagar Main Road, Madurai - 625 020, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jcor.jcor_196_20
Purpose: This study aims to explore the clinicopathological features of a rare eyelid tumor, trichoblastoma (TBL). Methods: This is a retrospective study of patients undergoing excision biopsies of eyelid masses at a tertiary eye care center in South India, between January 2017 and December 2018. Patient demographics, clinical and differentiating histopathological features, and recurrence of TBL were studied. Results: Amongst all eyelid masses, 15 were histopathologically diagnosed with TBL. The average age at presentation was 64 years as female preponderance and average duration of mass being 786 days. Change in color was noted in three patients, change in size in 14 cases and history of bleeding from the mass in three. Lower lid was the most common site involved (eight patients). The average dimension of the lesions was 158.67 mm2. One patient had lid margin involvement with loss of lashes noted in three cases and conjunctival involvement in three patients. Morphologically, eight lesions were nodular while five were noduloulcerative. Pigmented TBL was noted in eight patients, TBL with atypia in three patients. TBL, malignant TBL, rippled variant and TBL with trichofolliculoma were noted in one patient each. Clinical and histopathological diagnoses correlated in four patients. No recurrence was noted with the maximum follow-up period being 23 months. Conclusion and Relevance: A differential diagnosis of TBL should be kept in mind while managing eyelid tumors because benign lesions can mimic commonly diagnosed malignancies and vice versa.
Keywords: Lid tumors, South India, trichoblastoma
How to cite this article:
Chowdhury G, Tanwar M, Kim U, Krishnan SR. Eyelid trichoblastoma – A case series. J Clin Ophthalmol Res 2021;9:123-7 |
Trichoblastoma (TBL) is a rare, benign neoplasm mainly constituted of follicular germinative cells.[1] The histogenesis of follicular neoplasms is complex and a complete classification of tumors with follicular differentiation is yet to be established.[1] Ever since the original description by Headington in 1976, several of these neoplasms have been reported. Based on the degree of maturation and the relative proportion of epithelial and mesenchymal components, Headington classified the tumors of the hair germ into four different categories (TBL, trichoblastic fibroma, trichogenic TBL, and trichogenic myxoma) according to the appearance of the stromal component.[1] In 1993, Ackerman et al. proposed the unifying term of TBL as a generic name for cutaneous neoplasms with circumscribed margins and a predominance of follicular germinative cells.[2] The most frequent sites of occurrence of TBLs are the head and neck, where they appear as superficial plaques, nodules, or papular lesions.[3],[4],[5],[6] To the best of our knowledge, so far in literature, only four cases of this tumor have been reported on the eyelids.[3],[4],[5],[6] The clinical and histological diagnosis can often be mistaken for basal cell carcinoma (BCC), a malignant epidermal skin tumor.[3],[4],[5],[6] In view of the rarity of the tumor and its close similarities with BCC, we wanted to share the clinicopathological profile of these cases for better understanding of the clinical presentation and histological features of these tumors.
| Methods | |  |
This was a retrospective observational study. The study adhered to the tenets of the declaration of Helsinki. An ethics committee clearance was obtained before undertaking a review of medical records. We reviewed the data from the medical records of our pathology department for patients who had undergone eyelid mass excision biopsies and histologically been diagnosed as TBL or its variants over a period of two years from January 2017 to December 2018. The diagnosis of TBL and its variants were based on specific histopathological characteristics. In all patients, eyelid mass excision biopsies were performed under local anesthesia. The decision to do a wide margin excision biopsy was made depending upon the presence of high risk features, such as loss of lashes, distortion of the lid margin architecture, and bleeding from the lesion. Following the clearance report from the pathologist, if necessary, the patients underwent lid reconstruction with good cosmetic outcome.
The demographic data, clinical features, histopathological diagnoses, and incidence of recurrence were recorded and analyzed.
| Results | | |
Over a period of two years, a total of 15 patients were identified with eyelid TBL by the review of records, out of which eight patients had pigmented TBL, three TBL with atypia and one each of TBL [Figure 1], rippled variant TBL [Figure 2], TBL with trichofolliculoma and malignant TBL, respectively. | Figure 1: (a) Clinical picture of trichoblastoma; (b) Histopathological picture of trichoblastoma-H and E, ×10 showing bland looking basaloid cells resembling follicular germinative cells arranged in nests and cords with surrounding fibrous stroma; (c) Histopathological picture of trichoblastoma-H and E, ×40; (d) Clinical picture of malignant trichoblastoma; (e) Histopathological picture of malignant trichoblastoma – H and E, ×10 showing similar morphological features of benign counterpart with deep stromal infiltration and surface ulceration and necrosis; (f) Histopathological picture of malignant trichoblastoma – H and E, ×20
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 | Figure 2: (a) Clinical picture of rippled variant of trichoblastoma; (b) Histopathological picture of rippled variant of trichoblastoma – H and E, ×40 showing basaloid cells arranged as linear and parallel rows of nuclei resembling verocay of schwannoma; (c) Clinical picture of pigmented trichoblastoma; (d) Histopathological picture of pigmented trichoblastoma – H and E, ×10; (e) Histopathological picture of pigmented trichoblastoma – H and E × 40
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The demographic and clinical profile of the study subjects are given in [Table 1].
The average age of presentation was 64 years, the range being 40–80 years with a male:female ratio of 1:2.
Seven patients had right eyelid mass and eight had left eyelid involvement. Lower lid was the most common location (eight patients) and medial canthus (five) was more commonly involved than lateral canthus (three). There were four patients in which the tumor involved more than one structure.
The most common presenting complaint was a mass on the eyelid with the average duration being 786 days (range 90–2190). History of change in size was found in 14 patients, color change in four patients and history of bleeding from the mass was given by three of the patients.
Out of the 15 lesions, eight were nodular, five noduloulcerative, one diffuse and one ulcerative. The mean surface area of the lid masses was 158.67 mm2 (range 25–875 mm2). Lid margin involvement was seen in five patients but distortion of lid margin was seen in one patient only. Conjunctival involvement was found in two patients and fixity to the tarsus in two patients. The largest lesion measuring about 875 mm2 was a pigmented nodular lesion involving the upper lid, lower lid and lateral canthus with involvement of the lid margin and loss of lashes. Clinically, it was suspected to be a squamous cell carcinoma, but the histopathological diagnosis was pigmented TBL. Incidentally, all the lesions with high risk clinical features for malignancy were confirmed to be benign pigmented TBL on histopathology. The only ulcerative lesion, 120 mm2 in size, involving the lower lid was reported as TBL with atypia. The patient diagnosed to have trichoblastic carcinoma presented with a slow-growing diffuse pigmented lesion of 50 mm2 involving the right lower lid and medial canthus over a period of two years. Among the three patients with a history of previously removed eyelid mass at some other centers, two were reported as TBL with atypia and the third was pigmented rippled variant of TBL.
Four out of the fifteen cases were clinically suspected to be TBL, out of which two were pigmented TBLs, one rippled variant, and one was TBL with atypical features.
No recurrence was seen in any of the patients during the maximum follow-up period of 23 months.
A few cases stood out in their presentation and eventual diagnostic surprises which we thought significant enough to elaborate upon.
Case 1
A 61-year-old male presented with a history of a mass lesion involving his right eyelids, which had been growing steadily for the past two years. On examination, he had a pigmented nodular lesion with an irregular surface, raised margins, measuring 875 mm2 involving the lateral half of right lower lid, lateral canthus, and lateral one third of his upper lid with loss of lashes and distortion of the lid margin. Suspected as a squamous cell carcinoma, he underwent a wide margin excision which was histopathologically diagnosed with pigmented TBL [Figure 2]c, [Figure 2]d, [Figure 2]e. On one-year follow-up visit, there was no recurrence noted.
Case 2
A 73-year-old female patient presented to us with a history of an irregularity on her left lower lid for six months. She gave a history of progressive increase in size with an episode of bleeding from the lesion. On examination, she was found to have an ulcerative lesion with undermined edges and an irregular floor involving the left lower lid. A provisional diagnosis of rodent ulcer was made, and she underwent a wide margin excision biopsy. Histopathological diagnosis was TBL with atypical features. On her 15th-month follow-up visit, she was asymptomatic with no evidence of recurrence.
Case 3
A 80-year-old female came to our hospital with complaints of defective vision in both eyes. She had not noticed the dark-pigmented lesion on her left lower lid. On examination, she had a pigmented noduloulcerative lesion, measuring 80 mm2, arising from below the lateral canthus and involving the lateral end of the lower lid. A probable diagnosis of TBL was made which was confirmed as pigmented TBL by the pathologist [Figure 2]a and [Figure 2]b.
Case 4
A 70-year-old female patient presented to us with complaints of a progressively growing mass lesion on her right lower lid. On examination, she was found to have a diffuse lesion measuring 50 mm2 with irregular margins, patchy areas of pigmentation and central ulceration, involving the medial canthus and medial one fourth of the right lower lid. However, there was no lid margin distortion or loss of lashes or bleeding nor any palpable lymph nodes. Wide margin excision with frozen section and biopsy was done. Histopathological diagnosis made was Trichoblastic carcinoma [Figure 1]d, [Figure 1]e, [Figure 1]f. She was advised close follow-up, and on the sixth-month follow-up visit, there were no clinical features suggestive of recurrence.
| Discussion | | |
TBLs are well-marginated benign neoplasms of follicular germinative cells of the skin.[2] These tumors arise from the hair follicle and grow slowly. It was acknowledged that BCC (trichoblastic carcinoma) is the malignant counterpart of TBL.[3] Both TBL and BCC are made of germinative cells that have the potential to differentiate towards hair follicle, sebaceous gland, and apocrine gland.[4] TBLs have previously been typically described as sporadic, symmetric, solitary and as being common in middle-aged adults with no gender predilection.[4] Although TBLs are commonly situated on the head and neck, they seldom occur in the region of the eyelids.[5],[6],[7],[8] The reported cases of TBL on the eyelid include three women[5],[6],[8] and one man[7] with ages ranging from 45 to 79 years. Our patients showed a definite female preponderance, with the average age being 64 years (range was 40–80 years). The sizes of the documented masses vary from 5 to 12 mm and they all appeared to be well-circumscribed.[5],[6],[7],[8] However, in our case series of 15 patients, the average area of the lesions was 158.67 mm2 (range 25–875 mm2). The patient with the tumor of size 875 mm2 had an extensive pigmented TBL involving the lower lid, lateral canthus and lateral one third of the upper lid. Previously reported cases by Mencía-Gutiérrez et al.[5] and Eshraghi et al.[8] was on the right upper eyelid, whereas the location of the lesions reported by Johnson et al.[6] and Wladis et al.,[7] involved the medial canthus and the lower eyelids. In our study, lower lid was the most common site with medial canthus being more commonly involved than lateral canthus. Recurrence of TBL was observed by Johnson et al.[6] and Wladis et al.[7] In our series, the follow-up period ranged from 4 to 23 months, but we did not find any recurrence. Complete excision with adequate safe margins is generally required to minimize the likelihood of recurrence or malignant transformation of TBL.
Histologically, TBLs consist of well-circumscribed dermal or dermal-subcutaneous nests of basaloid epithelial cells, follicular papillae, and germinative cells. These basaloid cell islands show peripheral palisading without an epidermal connection and are separated by fibrous stroma arranged in cords, sheets, or lobules.[9] TBL is divided into five major histological patterns which include large nodular (including pigmented), small nodular, cribriform, racemiform, and retiform. Less common forms like adamantoid, columnar, rippled, subcutaneous and superficial forms have also been described.[9] The diagnosis of its variants depends upon the association with pigmentation and arrangement of cells. As per literature pigmented variants of TBL are very rare. Only few cases have been reported till date.[9],[10],[11] However, in our series the most common variant we saw was pigmented TBL. Malignant alteration and aggressive features in TBLs can lead to BCC or trichoblastic carcinoma,[12] but this is rare. Trichoblastic carcinoma lesions are deeply infiltrative, demonstrate surface ulceration and necrosis, show cytological atypia or mitotic activity and may result in systemic metastasis.
The principal histopathological differential diagnosis of TBL is BCC which can be much more irregular, invasive and destructive than TBL.[13] In BCC, there are clefts between the epithelium and stroma, while TBL has periepithelial stromal clefts with surrounding dermal collagen. Mucinous stroma or amyloid deposits are typically present in BCC whereas TBL has more pronounced stroma as compared to BCC, which is fibrous in nature. Mitoses and apoptotic bodies are characteristics of BCC, but rare in TBL. Rather, papillary mesenchymal bodies are characteristically present in TBL in contrast to BCC. Moreover, TBL does not show retraction artifacts unlike BCC.[13] Immunohistochemical analysis may be a better differentiating tool when diagnosis is challenging.[13] Expression patterns of Bcl-2, CD34, and CD10 can be useful in this context. Bcl-2 expression in TBL is limited to the basaloid keratinocytes in the outermost layer, whereas BCC shows diffusive staining.[13] In TBL, CD34 and CD10 are expressed only in the peritumoral stromal cells, but BCC shows intraepithelial staining.[13],[14] CK20-positive Merkel cells may be abundantly found in TBL but are lacking in BCC.[15] Expression of PHLDA1,[16] a marker of follicular stem cells, has been reported in TBL but not in BCC. In our study population immunohistochemical analysis was done using CD10 and CK20 markers but no specific statistically significant correlation was found with TBL.
The follow-up period for three cases, one of which had atypia was less than six months. A periodical follow-up of at least a year would be more beneficial in order to rule out a recurrence or differentiate atypia from a malignancy. There is sufficient literature on immunohistochemical markers available for diagnosis of BCC, one of the major differential diagnoses, which would have been beneficial in substantiating our diagnosis of TBL by exclusion.
What does this article add to existing literature
Eyelid TBL has been reported to be a very rare entity. But our study has found it to be otherwise. Hence, TBL should also be kept in mind as a differential diagnosis of eyelid neoplasm along with other more common lesions. TBL is often misdiagnosed as BCC. Therefore, clinicians should be familiar with the clinical signs and the histopathological differences between these two types of tumors.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Headington JT. Differentiating neoplasms of hair germ. J Clin Pathol 1970;23:464-71.  |
| 2. | Ackerman AB, Reddy VB, Soyer HP. Neoplasms with Follicular Differentiation. New York: Ardor Scribendi; 2001. |
| 3. | LeBoit PE, Burg G, Weedon D, Sarasin A. World Health Organization Classification of Tumors. Pathology and Genetics of Skin Tumors. Vol. 152. Lyon: IARC Press; 2006. p. 155. |
| 4. | Ackerman AB, de Viragh PA, Chongchitnant N. Neoplasms with Follicular Differentiation. Philadelphia: Lea & Febiger; 1993. p. 359. |
| 5. | Mencía-Gutiérrez E, Gutiérrez-Díaz E, Ricoy JR, Rodríguez-Peralto JL. Eyelid TBL: An unusual localization. Int J Dermatol 2003;42:201-2. |
| 6. | Johnson TV, Wojno TH, Grossniklaus HE. TBL of the eyelid. Ophthal Plast Reconstr Surg 2011;27:e148-9. |
| 7. | Wladis EJ, Linos K, Carlson JA. Trichoblastic fibroma of the eyelid. Ophthalmic Plast Reconstr Surg 2012;28:e62-4. |
| 8. | Eshraghi B, Riazi-Esfahani H, Katoozpour R, Anvari P, Mortazavi M, Asadi Amoli F. TBL of the upper eyelid. J Ophthalmic Vis Res 2017;12:219-21. [ PUBMED] [Full text] |
| 9. | Gozel S, Donmez M, Akdur NC, Yikilkan H. Development of six tumours in a sebaceus nevus of Jadassohn: Report of a case. Korean J Pathol 2013;47:569-74. |
| 10. | Cho HK, Song JS, Kang WH, Ro BI. Pigmented TBL arising from the nevus sebaceous: A rare case in Korea. Ann Dermatol 2009;21:406-8. |
| 11. | Kamat G, Yelikar B, Shettar S, Karigoudar MH. Pigmented TBL with sebaceous hyperplasia. Indian J Dermatol Venereol Leprol 2009;75:506-8. [ PUBMED] [Full text] |
| 12. | Schulz T, Proske S, Hartschuh W, Kurzen H, Paul E, Wünsch PH. High-grade trichoblastic carcinoma arising in TBL: A rare adnexal neoplasm often showing metastatic spread. Am J Dermatopathol 2005;27:e9-16. |
| 13. | Córdoba A, Guerrero D, Larrinaga B, Iglesias ME, Arrechea MA, Yanguas JI. Bcl-2 and CD10 expression in the differential diagnosis of TBL, basal cell carcinoma, and basal cell carcinoma with follicular differentiation. Int J Dermatol 2009;48:713-7. |
| 14. | Schirren CG, Rütten A, Kaudewitz P, Diaz C, McClain S, Burgdorf WH. TBL and basal cell carcinoma are neoplasms with follicular differentiation sharing the same profile of cytokeratin intermediate filaments. Am J Dermatopathol 1997;19:341-50. |
| 15. | Kurzen H, Esposito L, Langbein L, Hartschuh W. Cytokeratins as markers of follicular differentiation: An immunohistochemical study of TBL and basal cell carcinoma. Am J Dermatopathol 2001;23:501-9. |
| 16. | Yeh I, McCalmont TH, LeBoit PE. Differential expression of PHLDA1 (TDAG51) in basal cell carcinoma and trichoepithelioma. Br J Dermatol 2012;167:1106-10. |
[Figure 1], [Figure 2]
[Table 1]
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