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 Table of Contents  
Year : 2021  |  Volume : 9  |  Issue : 2  |  Page : 119-122

Malignant proliferating trichilemmal tumor in neck: A rare clinical presentation and review of 50 cases from literature

Department of ENT, Hassan Institute of Medical Sciences, Hassan, Karnataka, India

Date of Submission14-Aug-2021
Date of Decision28-Aug-2021
Date of Acceptance29-Aug-2021
Date of Web Publication17-Dec-2021

Correspondence Address:
Dr. A T Fida Harish
Department of ENT, Hassan Institute of Medical Sciences, Hassan, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jhnps.jhnps_44_21

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Background: Proliferating trichilemmal tumor is a rare cutaneous neoplasm derived from the outer root sheath of the hair follicle which usually presents as a solitary lesion, in fourth to eighth decades of life, and has a predilection for women. The scalp is the most common site of occurrence of this tumor. Malignant proliferating trichilemmal tumor (MPTT) invades surrounding tissues or metastasizes with features of cellular pleomorphism and nuclear atypia. Local recurrences have been occurred in about 3.7%–6.6%. Complete surgical excision with a margin of normal tissue is the standard treatment. Aim: To present a rare case of MPTT over the upper part of the neck which presented to our ENT department and to review about 50 cases of this entity in terms of age, sex, location, treatment, and outcome. Methods: The detailed description of the case of MPTT presented over the upper part of the neck to our ENT department will be given. Literature was reviewed for similar cases of this entity and was studied in terms of age, sex, location of tumor, treatment, and outcome. Results: Out of the 50 cases reviewed, majority of the patients were females, between the age group 40 and 80 years. The scalp was the common site of tumor. All the cases were treated surgically. Conclusion: MPTT is a rare neoplasm which carries a diagnostic dilemma and is often confused with squamous cell carcinoma. Wide excision of MPTT with an adequate clearance margin is the treatment of choice.

Keywords: Malignant proliferating trichilemmal tumor, trichilemmal carcinoma, trichilemmal keratinization

How to cite this article:
Prasad K U, Harish A T. Malignant proliferating trichilemmal tumor in neck: A rare clinical presentation and review of 50 cases from literature. J Head Neck Physicians Surg 2021;9:119-22

How to cite this URL:
Prasad K U, Harish A T. Malignant proliferating trichilemmal tumor in neck: A rare clinical presentation and review of 50 cases from literature. J Head Neck Physicians Surg [serial online] 2021 [cited 2023 Jun 4];9:119-22. Available from: https://www.jhnps.org/text.asp?2021/9/2/119/332721

  Introduction Top

Proliferating trichilemmal tumor is an uncommon tumor of external root sheath derivation usually arising in a preexistent pilar cyst.[1] This tumor presents as a rapidly growing large nodule commonly on the head-and-neck area followed by the trunk. This uncommon lesion occurs mainly in the elderly with a marked predilection for females.[2] Their characteristic histological findings are sudden compact amorphous keratinization of the epithelial cells that cover the cyst wall without a granular layer; this phenomenon is called trichilemmal keratinization.[3] A tumor that invades neighboring tissues accompanied with anaplasia and necrosis is described as a malignant proliferating trichilemmal tumor (MPTT).[4] Only less number of cases of MPTT s have been documented in the literature. We hereby report a case of an 80-year-old female patient who presented with a swelling over the neck. We reviewed the literature of 50 cases of MPTT.

  Methods Top

Literature was reviewed for 50 cases of MPTT and was studied in terms of age, sex, location of tumor, treatment and outcome.

Clinical presentation

An 80-year-old female patient, not a known case of diabetes mellitus or hypertension, presented to the ENT department of Hassan Institute of Medical Sciences in the year 2016 with complaints of swelling in the upper part of the neck for 30 years. The swelling was initially having a size of small gooseberry and then gradually progressed in size. She gives the history of the sudden increase in size in the last 3½ months, where the swelling progressed to a size of a tennis ball. There was associated pain in the swelling for 1 month which was dull aching type. On local examination, a nodular swelling of size approximately 12 cm × 7 cm was present in the left submandibular region with the overlying skin inflamed and appeared red [Figure 1]. The swelling extended from 3 cm below the mentum of the mandible unto the lower border of cricoid cartilage in the midline and laterally, extending from the midline to anterior border of the sternocleidomastoid muscle on the left side. On palpation, the swelling had a firm consistency and was slightly tender. The movement of swelling on deglutition was not appreciated but the swelling was mobile from underlying structures like hyoid bone. The skin over the swelling was not pinchable. On the left side of the neck, 2–3 mobile and nontender lymph nodes were palpable in levels II and III, each measuring 1–1.5 cm. Based on the history and examination, a provisional diagnosis of skin tumor was made.
Figure 1: Eighty-year-old female patient with swelling over neck

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CT scan report is not available. Routine investigations were within normal limits. Fine needle aspiration cytology done from the swelling showed inflammatory lymphadenitis. Wedge biopsy done from the swelling showed adnexal tumor. The patient underwent wide excision of the tumor with reconstruction and left supraomohyoid neck dissection using utility incision [Figure 2] and [Figure 3]. The excised tumor and lymph nodes were sent for histopathological evaluation. No. 14 drain was kept, which was removed on postoperative day 4. Postoperative period was uneventful.
Figure 2: Extent of tumor with marking for utility incision

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Figure 3: Surgical field post tumor excision

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The histopathology report came as MPTT [Figure 4]. As the margins of the specimen showed free of tumor, the patient did not receive radiotherapy. The patient was discharged after 1 week, came for follow-up after 1 month but failed to follow-up thereafter.
Figure 4: Cyst wall epithelial lining with malignant proliferating cells in the dermis

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  Results Top

Out of the 50 cases reviewed, majority of the patients were females, falling between the age group 40 and 80 years. Among them, 40 cases occurred in the head-and-neck area. In the head-and-neck area, the scalp was the common site of tumor (33 cases – 82%). Other sites of presentation in head and neck area included nape of neck, glabella, postauricular region, angle of jaw, etc. Other than the head-and-neck area, the sites involved were breast skin, gluteal region, back of hand and wrist, etc. Our case had presented in front of the neck, which was not documented in any of the literature. Most of the tumors were slow-growing with a long history of duration. 1 case presented with short duration of 4 months. Out of 40 cases in head and neck, 4 cases were diagnosed as squamous cell carcinoma preoperatively, which was diagnosed postoperatively as MPTT on histopathological examination. Metastasis was observed in few cases, out of which, lymph node metastasis was the most common 2 cases of intracranial extension and 1 case of lung metastasis have been reported. Out of 40 cases in the head-and-neck area, most of the cases underwent surgical excision, 2 cases received radiotherapy and 1 nonoperative case was treated with local ethanol injection. Out of 40 cases, 7 cases recurred after surgical line of treatment. Of these 7 cases, 1 case recurred with metastasis which was treated with surgery and radiotherapy. For a recurrent case, adjuvant chemotherapy had been given. Death was reported in 1 case, 2 months after palliation.

  Discussion Top

Trichilemmal cysts are keratinous cysts lined by stratified squamous epithelium with their wall resembling external hair root sheath.[1] A possibility of inheritance of pilar cysts in an autosomal-dominant mode, linked to chromosome 3, has been reported by Leppard et al. in one of their case reports.[13]

Proliferating trichilemmal tumor is an uncommon benign adnexal tumor of skin seen in elderly females.[14] Shetty et al. reported a case of proliferating trichilemmal tumor with malignant features in a 32-year-old male patient.[15] Proliferating trichilemmal tumors develop in the course of time from the foci of proliferating epithelial cells in the trichilemmal cysts because of factors such as trauma or chronic inflammation.[3] Alternatively, Rahbari et al. have reported 2 cases where these tumors emerged de novo from organoid nevi.[16] The tumor presents as a solitary mass commonly on the scalp and neck. Other sites such as the upper extremities, back, and gluteal region have been reported.[14] A case of vulval proliferating trichilemmal tumor has also been reported by Ilana et al.[17] Among the 50 cases reviewed, 40 cases occurred in the head-and-neck area. In the head-and-neck area, the scalp was the common site of tumor (33 cases – 82%). Our case had presented in front of the neck, which was not documented in any of the literatures [Table 1] and [Table 2].
Table 1: 50 cases of malignant proliferating trichilemmal tumors reviewed[5],[6],[7],[8],[9]

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Table 2: Cases of malignant proliferating trichilemmal tumor in the head-and-neck region[7],[9],[10],[11],[12]

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The presence of trichilemmal keratinization and lack of granular layer are the histologic hallmarks of proliferating trichilemmal tumor.[2] If there is frank invasion into adjacent structures in association with tumor necrosis, cytological atypia and increased mitotic activity, the diagnosis of MPTT is made. Due to its rarity, the incidence of MPTT is unknown.[18] Although metastasis from proliferating trichilemmal tumors is rare, it has been reported for a sporadic malignant variant showing an aggressive clinical course and a tendency for lymph node and distal metastasis.[3] The rate of local recurrence and regional lymph node metastasis has been estimated to be between 3.7%–6.6% and 1.2%–2.6%, respectively.[2]

Ye et al. have recently proposed definition of benign and malignant variants of PTT.[19] Immunohistochemical studies have also been conducted for the diagnosis and differential diagnosis of PTTs and morphological descriptions. When compared with PTT, increased staining with Ki-67 and P53 was observed in MPTT.[20] For differentiation between SCC and MPTT, CD34 is an important immune determinant.[4] CD34 is known to be expressed in MPTT as it indicates trichilemmal differentiation and its expression is not seen in SCC.[21]

Simple excision is curative for benign PTT with an excellent prognosis, whereas no guidelines are available for the clinical management of malignant PTT due to the rarity of these tumors.[1] Wide local excision has often been the standard treatment with no established role of margin size or additional therapeutic interventions such as radiation therapy and chemotherapy.[14]

  Conclusion Top

MPTT in the neck is a rare neoplasm which carries a diagnostic dilemma and is often confused with squamous cell carcinoma. Histopathological features aids in diagnosis. Features favoring MPTT over SCC include the presence of trichilemmal type keratinization and the lack of a premalignant epidermal lesion. TLC is distinguished by a lobular proliferation of clear cells and continuity with the epidermis and clear evidence of trichilemmal differentiation.[22] Wide surgical excision gives a good outcome without recurrence.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


This material has never been published and is not currently under evaluation in any other peer reviewed publication.

Ethical approval

The permission was taken from Institutional Ethics Committee prior to starting the project. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

  References Top

Griffiths C, Barker J, Bleiker T, Chalmers R, Creamer D. Rook's Textbook of Dermatology. 9th ed. Vol. 4. New Jersey, US: Blackwell Publishing Ltd; 2016. p. 138.5.  Back to cited text no. 1
Sutherland D, Roth K, Yu E. Malignant proliferating trichilemmal tumor treated with radical radiotherapy: A case report and literature review. Cureus 2017;9:e999.  Back to cited text no. 2
Satyaprakash AK, Sheehan DJ, Sangüeza OP. Proliferating trichilemmal tumors: A review of the literature. Dermatol Surg 2007;33:1102-8.  Back to cited text no. 3
Chaichamnan K, Satayasoontorn K, Puttanupaab S, Attainsee A. Malignant proliferating trichilemmal tumors with CD34 expression. J Med Assoc Thai 2010;93 Suppl 6:S28-34.  Back to cited text no. 4
Ahn HJ. A case of malignant proliferating trichilemmal tumor on the lower extremity. J Am Acad Dermatol. 2007;76:AB6.  Back to cited text no. 5
Capurso-García MA, Bautista-Piña V, Pomerantz A, Galnares-Olalde JA, Blachman-Braun R, Rodríguez-Rodríguez S, et al. Atypical Proliferating Trichilemmal Cyst with Malignant Breast Skin Transformation: A Case Report and Review of the Literature. Case Rep Oncol Med 2016;2016: 7481569.  Back to cited text no. 6
Weiffenbach A, Katz K, Rupley K, Carter A, Gottlieb A, Shulman K. Rapidly Enlarging Malignant Proliferating Trichilemmal Tumor in a Young Female. J Drugs Dermatol. 2018;17:1325-7.  Back to cited text no. 7
Martinez AQ, Rios CN, Avila GB. Malignant proliferating trichilemmal tumor of the forearm: a case report of an unusual location of a rare cutaneous adnexal tumor. J Surg Case Rep 2018; 2018; rjy290.  Back to cited text no. 8
Lobo L, Amonkar AD, Dontamsetty VV. Malignant Proliferating Trichilemmal Tumour of the Scalp with Intra-Cranial Extension and Lung Metastasis-a Case Report. Indian J Surg 2016;78;493-5.  Back to cited text no. 9
Wisco O, Grande D, Reis K, Leary MO. A case of a malignant proliferating trichilemmal tumor with perineural invasion. J Am Acad Dermatol 2012;66:AB148.  Back to cited text no. 10
Dubhashi SP, Jadhav SK, Parasnis A, Patil CS. Recurrent malignant proliferating trichilemmal tumor with lymph node metastasis in a young woman. J Postgrad Med 2014;60;400-2.  Back to cited text no. 11
Fernnadez JA, Lopez CG, Santiago SA. Malignant proliferating trichilemmal tumor with spindle cell component. J Am Acad Dermatol 2012;66:AB80.  Back to cited text no. 12
Leppard BJ, Sanderson KV, Wells RS. Hereditary trichilemmal cysts. Hereditary pilar cysts. Clin Exp Dermatol 1977;2:23-32.  Back to cited text no. 13
Bury Y, Bloxham C. Proliferating trichilemmal tumour. Elsevier 2009;15:273-8.  Back to cited text no. 14
Shetty PK, Jagirdar S, Balaiah K, Shetty D. Malignant proliferating trichilemmal tumor in young male. Indian J Surg Oncol 2014;5:43-5.  Back to cited text no. 15
Rahbari H, Mehregan AH. Development of proliferating trichilemmal cyst in organoid nevus. Presentation of two cases. J Am Acad Dermatol 1986;14:123-6.  Back to cited text no. 16
Avinoach I, Zirkin HJ, Glezerman M. Proliferating trichilemmal tumor of the vulva. Case report and review of the literature. Int J Gynaecol Pathol 1989;8:163-8.  Back to cited text no. 17
Goyal S, Jain BB, Jana S, Bhattacharya SK. Malignant proliferating trichilemmal tumor. Indian J Dermatol 2012;57:50-2.  Back to cited text no. 18
[PUBMED]  [Full text]  
Ye J, Nappi O, Swanson PE, Patterson JW, Wick MR. Proliferating pilar tumors: A clinicopathological study of 76 cases with a proposal for definition of benign and malignant variants. Am J Clin Pathol 2004;122:566-74.  Back to cited text no. 19
Gulati HK, Deshmukh SD, Anand M, Morale V, Pande DP, Jadhav SE. Low-grade malignant proliferating pilar tumor simulating a squamous-cell carcinoma in an elderly female: A case report and immunohistochemical study. Int J Trichology 2011;3:98-101.  Back to cited text no. 20
Poblet E, Jimenez-Acosta F, Rocamora A. QBEND/10 (anti-CD34 antibody) in external root sheath cells and follicular tumors. J Cutan Pathol 1994;21:224-8.  Back to cited text no. 21
Lu ZR, Imhagwe G. Malignant proliferating trichilemmal tumour: A case report and literature review. J RCPA 2016;8:S75.  Back to cited text no. 22


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2]


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