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Squamous Cell Carcinoma of the Scalp Masquerading As Trichilemmal Tumor of the Scalp

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Squamous Cell Carcinoma of the Scalp Masquerading As Trichilemmal Tumor of the Scalp IJHNS A Nanjundappa et al 10.5005/jp-journals-10001-1201 CASE REPORT Squamous Cell Carcinoma of the Scalp Masquerading as Trichilemmal Tumor of the Scalp 1A Nanjundappa, 2Rajshekar Halkud, 3Bindu Venugopal, 4Purshottam Chavan, 5KT Sidappa, 6Siddharth Biswas 7M Samskruthi, 8Sudhir M Naik ABSTRACT INTRODUCTION Background: Adnexal components tumors of the skin are Malignant proliferating trichilemmal tumor (MPTT) is a very rare and are seen as benign lesions of the scalp in aged women. Trichilemmal tumor is usually benign and rarely under­ rare neoplasm arising from the external root sheath of the goes malignant transformation in a stepwise manner starting hair follicle seen in women over the 6th decade.1-3 They with an adenomatous stage of the trichilemmal cyst to an vary from well circumscribed to irregularly defined sub- epitheliomatous stage of the proliferating trichilemmal tumor (PTT) evolving into the carcinomatous stage of the malignant cutaneous nodules with squamoid cytologic features and proliferating trichilemmal tumor (MPTT). trichilemmal-type of keratinization with cystic to firm Case report: A 53­year­old woman reported with huge swelling in consistency.2,4 This initially described with confusing in the posterior region of the scalp. The swelling was mobile histologic features of squamous cell carcinoma (SCC).5 and soft to firm in consistency not fixed to the skull bone. The surface was smooth and getting under the swelling was not The tumor is also called epidermoid cyst, pilar tumor of possible, needle biopsy reported as trichilemmal tumor. Contrast the scalp, proliferating trichilemmal cyst, proliferating enhanced computed tomography (CECT) neck did not show any epidermoid cyst, giant hair matrix tumor, hydatidiform nodes in the neck, especially the posterior compartment. The tumor was widely excised with 1 cm margin and the periosteum keratinous cyst, trichochlamydo carcinoma, and invasive was kept intact with split skin graft. An area of periosteal adher­ hair matrix tumor.6 ence to the tumor seen was excised. The histopathology report Incidences are seen in older age females predominantly on serial sectioning reported grade 1 moderately differentiated 7, 8 squamous cell carcinoma (SCC). Postoperative external beam affecting the scalp and rarely eyelids, neck and face. radiotherapy of 66 Gy was given. The patient is being followed While MPTT usually arises as a subepidermal tumor, up and no recurrence is seen. the other similar tumor trichilemmal carcinoma (TLC) Conclusion: Malignant transformation in a proliferating trichilemmal tumor a rare entity and should be differentiated occurs as exophytic, popular or nodular lesion measu ring from scc and the better prognostic trichilemmal carcinoma. less than 2 cm.3 This malignant lesion is seen in the sun- A protocol­based adjuvant therapy is available for squamous cell exposed scalp, solar keratosis, scars of burns, following carcinoma but not for trichilemmal carcinoma (TLC) and MPTT. A major meta­analysis may help to establish a clinical outcome­ high-dose radiation exposure and in post-transplant based classification and management protocol for these tumors. states.5 The incidence of benign is more compared to the Keywords: Trichilemmal tumor, Squamous cell carcinoma, malignant appendageal tumors, only few reports in litera- Wide excision, External beam radiation. ture with an aggressive clinical course and a propensity How to cite this article: Nanjundappa A, Halkud R, Venugopal B, for nodal and distant metastases.9 Malignant prolife- Chavan P, Sidappa KT, Biswas S, Samskruthi M, Naik SM. Squamous Cell Carcinoma of the Scalp Masquerading as Trichi­ rating trichilemmal tumor (MPTT) is a very uncommon lemmal Tumor of the Scalp. Int J Head Neck Surg 2014;5(3):144­147. tumor may be seen as longstanding nodular scalp lesions Source of support: Nil and histological evidence of significant abnormal mitosis, Conflict of interest: None marked cellular pleomorphism, infiltrating margins and aneuploidy reflect malignant transformation.10 We report a case of SCC masquerading as MPTT of 1,6Professor, 2,4Associate Professor, 3Senior Resident the scalp. Initially, it appeared as a benign trichilemmal 5Assistant Professor, 7,8Fellow tumor, later on serial sectioning of the suspicious margins 1,2,4,5,7,8 Department of Head and Neck Oncosurgery, Kidwai moderately differentiated scc was confirmed. Memorial Institute of Oncology, Bengaluru, Karnataka, India 3 Department of Radiation Oncology, Kidwai Memorial Institute CASE REPORT of Oncology, Bengaluru, Karnataka, India 6Department of Pathology, Kidwai Memorial Institute of A 53-year-old woman reported with huge swelling in the Oncology, Bengaluru, Karnataka, India posterior region of the scalp. It was a soft-tissue swelling Corresponding Author: Sudhir M Naik, Fellow, Department 12 × 12 × 12 cm in dimensions. The swelling was mobile of Head and Neck Oncosurgery, Kidwai Memorial Institute of and soft to firm in consistency not fixed to the skull Oncology, Bengaluru, Karnataka, India, Phone: 09916807109 bone (Fig. 1). The surface was smooth and getting under e­mail: [email protected] the swelling was not possible. Routine investigations, fine 144 IJHNS Squamous Cell Carcinoma of the Scalp Masquerading as Trichilemmal Tumor of the Scalp needle aspi ration cytology (FNAC), contrast enhanced extension with a 1 cm margin. Customized cerrobend computed tomography (CECT) brain with enhanced bone electron cut-outs were made, and the patient was treated window, magnetic resonance imaging (MRI) was done. in prone position to avoid scatter dose to critical structures Trichilemmal tumor of skin appendages was reported. and achieve uniform dose distribution. The patient was CECT neck did not show any nodes in the neck, espe- examined at the beginning of treatment and weekly there- cially the posterior compartment. The tumor was widely after till the end of treatment. She experienced grade II excised with 1 cm margin and the periosteum was kept skin reaction at the end of therapy. The patient is being intact. Split skin graft was harvested from the medial side followed up and no recurrence is seen. of the thigh and the defect covered with it under general anesthesia (Fig. 2). An area of periosteal adherence to the DISCUSSION tumor was excised. The histopathology report on serial Tumors arising from the adnexal components of the skin sectioning reported grade 1 moderately differentiated pilosebaceous unit, eccrine or apocrine glands are very SCC. Patient was planned with postoperative radio- rare and display more than one line of differentiation.11 therapy of 66 Gy in 33 fractions with electrons of 6 MeV to Most of the tumors are benign, but malignant varieties a depth of 2 cm prescribed to 90% isodose. The treatment also exist which are very rare, locally aggressive, having area was defined as the volume of tumor with microscopic potential for nodal and distant metastasis, with a poor prognosis.11 So, hunting for features of malignancy in these mostly benign tumors seems challenging as the incidences are very low.11 Adnexal components tumors of the skin are very rare and are seen as benign lesions of the scalp in aged women.12,13 The benign includes trichofolliculoma, des- moplastic trichoepithelioma, trichoblastoma, trichoblastic fibroma, trichoadenoma, proliferating trichilemmal tumor, trichilemmoma, desmoplastic trichilemmoma, pilomatricoma, sebaceous adenoma and sebaceous epithelioma.12,13 The malignant includes TLC, tricho- blastic carcinoma, MPTT, pilomatrix carcinoma, seba- ceous carcinoma, basal cell carcinoma with sebaceous differentiation.11 They are not seen in the alopecic individuals, as lanugo hair follicles of the bald scalp and follicles of other areas devoid of non-terminal hair are unlikely to Fig. 1: Huge swelling in the posterior region of the scalp produce these tumors.9 They are seen in areas of excess hair growth arising through increased epithelial prolife- ration within pilar or sebaceous cysts.9 The tumor may be of a long standing cyst like lesion on the scalp which has to be differentiated from well-differentiated carcinoma.14 Trichilemmal tumor is usually benign and rarely may undergo malignant transformation in a stepwise manner starting with an adenomatous stage of the trichilemmal cyst to an epitheliomatous stage of the PTT evolving into the carcinomatous stage of the MPTT.5 Malignant and benign tumors can be differentiated on the basis of morphological criteria, i.e. rate of growth, invasion and metastasis and functional criteria, i.e. anaplasia and cellular atypia.15 Clear cells appearing from the outer root sheath of hair exhibiting marked pleomorphism and atypical mitosis, with nodular trabe- cular formation of keratinocytes with a sharp border Fig. 2: Postoperative 1 week after the excision and bet ween parenchyma and stroma confirms the trichilemmal skin grafting tumor.13 The rare malignant change has a circumscribed International Journal of Head and Neck Surgery, September-December 2014;5(3):144-147 145 A Nanjundappa et al nodular or a diffuse spindle cell type which has to diffe- or chemotherapy to improve disease-free survival.6 rentiated from metastases of spindle cell tumors with Ye et al reported 50% recurrence in MPTT within 2 to regions of squamous differentiation.1 MPTT has trichi- 14 months where none had been given adjuvant therapy.4 lemmal keratinization, lobular pattern and the lack of Trichilemmal carcinoma (TLC) is treated with wide local a precursor epidermal lesion such an actinic keratosis
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