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Year : 2019  |  Volume : 8  |  Issue : 3  |  Page : 197-202

Pilomatrixoma arising from maxillary sinus – First of its kind

Department of ENT and HNS, SCB MCH, Cuttack, Odisha, India

Date of Web Publication01-Aug-2019

Correspondence Address:
Dr. T R Sruthi
Department of ENT and HNS, SCB MCH, Cuttack, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sajc.sajc_54_19

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How to cite this article:
Sruthi T R, Behera SK, Swain S, Patsani M. Pilomatrixoma arising from maxillary sinus – First of its kind. South Asian J Cancer 2019;8:197-202

How to cite this URL:
Sruthi T R, Behera SK, Swain S, Patsani M. Pilomatrixoma arising from maxillary sinus – First of its kind. South Asian J Cancer [serial online] 2019 [cited 2020 Sep 23];8:197-202. Available from:

Dear Editor,

Pilomatrixomas are uncommon tumors, which arise due to uncontrolled proliferation of hair matrix cells. They are said to represent 0.001%–0.003% of all dermatopathologic specimens.[1] Head and neck is the most common site. PubMed data search shows no data on pilomatrixoma of the nasal cavity or paranasal sinuses. Hence, this is the first reported case of pilomatrixoma involving the nasal cavity and maxillary sinus.

A 34-year-old male patient presented with progressive nasal obstruction and mass in the left nasal cavity for the past 6 months. It was associated with rhinorrhea and hyposmia. There was no history of pain, fever, epistaxis, trauma, or nasal surgery. He was on fluticasone furoate nasal spray. On examination, a pale-pinkish mass was seen filling the left nasal cavity, extending up to the anterior nares, with ulcerations on the exposed surface [Figure 1].
Figure 1: Clinical photograph showing left-sided nasal mass

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It was firm, nontender, not bleeding on touch and was arising from the lateral wall. Posterior rhinoscopy showed pinkish mass at the left choana, rest is normal. Nasal endoscopy showed the deviation of the septum to the right side, and mass arising from the left middle meatus. Computed tomography (CT) showed homogenous mass filling left nasal cavity and left maxillary sinus, extending up to the choana. Under general anesthesia, the mass of 7.5 cm was excised endoscopically. Attachment to the posterior maxillary wall was removed with microdebrider. The histopathologic examination showed outer respiratory epithelium, below which was stratified squamous epithelium. It had large areas showing shadow cells, multinucleated giant cells, areas of calcifications, small clusters of basaloid cells with dense chronic inflammatory (lymphoplasmacytic) infiltration suggestive of nasal pilomatrixoma [Figure 2]. The patient is on follow-up with no evidence of any recurrence.
Figure 2: Histopathology picture showing ghost cells and clusters of basaloid cells suggestive of nasal pilomatrixoma

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Pilomatrixoma or calcifying epithelioma of Malherbe is a benign tumor that arises from hair follicles, first described by Malherbe and Chenantais in 1880.[2] The term pilomatrixoma was coined by Forbis and Helwing in 1961. The most common site is the head and neck, followed by the limbs. Nose, however, is a very rare site. Since there are no hair follicles in the maxillary sinus, this case becomes an interesting one. Pilomatrixoma has a female preponderance. It is common in first two decades of life.

The exact etiology of pilomatrixoma is still unknown. Mutation of the β-catenin gene and viral etiology are suggested. It presents as a slowly growing, painless subcutaneous or intradermal mass, which is firm and gritty to palpation and mobile. It can stretch the skin over it, producing a “tent sign.” Their usual size ranges from 1 cm to 3 cm. However, here, it was quite large, 7.5 cm. They have an association with Gardner's syndrome, Myotonic dystrophy, Turners syndrome, and Xeroderma pigmentosum. In CT, sand-like or nodular calcifications may be seen. Malignant transformation occurs very rarely, termed as pilomatrix carcinoma. Jones et al. reported that only 16% of pilomatrixomas are accurately diagnosed clinically.[3] Treatment is complete excision, and recurrence is rare.

Pilomatrixoma is a rare tumor of the hair follicles. This case adds a new site to this peculiar lesion, the nasal cavity and sinus.

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.


We would like to thank all the faculties and residents of the ENT department for their valuable suggestions and help in this article.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma). A statistical study. Arch Dermatol 1973;108:532-4.  Back to cited text no. 1
Malherbe A, Chenantais J. Note on calcifying fibroma of sebaceous glands. Prog Med 1880;8:826-37.  Back to cited text no. 2
Jones CD, Ho W, Robertson BF, Gunn E, Morley S. Pilomatrixoma: A comprehensive review of the literature. Am J Dermatopathol 2018;40:631-41.  Back to cited text no. 3


  [Figure 1], [Figure 2]


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