Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contact Us Login 
An Official Publication of the Indian Association of Oral and Maxillofacial Pathologists

  Table of Contents    
Year : 2022  |  Volume : 26  |  Issue : 2  |  Page : 259-262

Intraoral malignant glomus tumor

1 Department of Oral and Maxillofacial Surgery, Madha Dental College and Hospital, Chennai, Tamil Nadu, India
2 Department of Pediatric Dentistry, Madha Dental College and Hospital, Chennai, Tamil Nadu, India
3 Department of Oral and Maxillofacial Pathology, Adhiparasakthi Dental College and Hospital, Chennai, Tamil Nadu, India
4 Department of Pediatric and Preventive Dentistry, Sathyabama Dental College and Hospital, Chennai, Tamil Nadu, India

Date of Submission20-Dec-2021
Date of Acceptance21-Jan-2022
Date of Web Publication28-Jun-2022

Correspondence Address:
K Sai Sarath Kumar
Department of Pediatric and Preventive Dentistry, Sathyabama Dental College and Hospital, 26/51, Sundar Moorthy Vinayagar Koil Street, Triplicane, Chennai - 600 005, Tamil Nadu
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jomfp.jomfp_444_21

Rights and Permissions



Glomus tumors are uncommon, benign solitary tumors derived from the glomus apparatus. We report here a case of a malignant glomus tumor in an 8-year-old child presenting as a multilocular ill-defined radiolucency of the mandible. The lesion microscopically showed sheets of round basophilic cells with high nuclear-cytoplasmic ratio, indistinct cell boundaries, nuclear hyperchromatism and nuclear pleomorphism. Immunohistochemically, the tumor was positive for vimentin and smooth muscle actin.

Keywords: Child, glomus tumor, malignant, mandible, oral, pedodontia, vimentin

How to cite this article:
Chandran S, Elangovan A, Vijayakumar S, Sarath Kumar K S. Intraoral malignant glomus tumor. J Oral Maxillofac Pathol 2022;26:259-62

How to cite this URL:
Chandran S, Elangovan A, Vijayakumar S, Sarath Kumar K S. Intraoral malignant glomus tumor. J Oral Maxillofac Pathol [serial online] 2022 [cited 2022 Aug 14];26:259-62. Available from: https://www.jomfp.in/text.asp?2022/26/2/259/348759

   Introduction Top

The glomus apparatus was identified in 1862 by Sucquet and described in detail by Hoyer in 1877. It is an arteriovenous anastomosis located in the stratum reticularis of the dermis predominantly on the palm and digits of the hand and the ventral surface of the feet.[1] It is involved in thermal regulation. The glomus tumor derived from the glomus apparatus accounts for <2% of soft tissue tumors.[2] It is a rare tumor that usually is seen in distal extremities. Other less common sites of involvement include the nasal cavity, middle ear, stomach, bone, lung and rarely oral cavity (0.6%).[3],[4]

We present here a rare case of an intraoral malignant glomus tumor in the mandible and review the literature concerning the glomus tumor of the oral cavity.

   Case Report Top

An 8-year-old girl presented to the Department of Pedodontics, Ragas Dental College and Hospital, with an intraoral growth in the alveolar region of the right body of the mandible of 1-year duration with a history of a gradual increase to the present size of 3 × 3 cm [Figure 1]. The sessile growth was soft in consistency and nontender on palpation. Mucosa over the swelling was smooth and not ulcerated. The first molar and deciduous canine and first molar on the affected side were mobile. The patient gave a history of incisional biopsy done a month earlier, reported as “insufficient tissue for diagnosis.” Orthopantomogram revealed an ill-defined multilocular radiolucency in the right body of the mandible measuring 3.5 × 1.5 cm in size and extending from the apical end of 83 to the mesial aspect of the 47 permanent tooth bud. Forty-six and 84 exhibited root resorption. The permanent tooth buds 44 and 45 were within the radiolucency [Figure 2]. The provisional diagnosis was ameloblastic fibrosarcoma.
Figure 1: Intraoral growth in the alveolar region body of the mandible

Click here to view
Figure 2: Orthopantomogram revealed ai ill-defined multilocular radiolucency

Click here to view

An excisional biopsy was done. The gross specimen submitted included hard and soft tissue and measured 2 × 4.5 × 2 cm in size [Figure 3].
Figure 3: Gross specimen

Click here to view

Histopathological examination showed uniform, monomorphic round blue cells arranged in sheets and cords. The cells were basophilic with a high nuclear-cytoplasmic ratio, indistinct cell boundaries [Figure 4]a, foci of nuclear hyperchromatism, nuclear pleomorphism and mitotic figures (9/10HPF) [Figure 4]b. The connective tissue stroma was fibrovascular with numerous dilated capillaries and focal edematous areas. The tumor cells were positive for vimentin and smooth muscle actin (SMA) [Figure 4]c and [Figure 4]d and negative for desmin, p63, CD34 and CD45.
Figure 4: (a) Sheets of round cells with basophilic cytoplasam, high nuclear-cytoplasmic ratio and indistinct cell boundaries (H and E, ×40). (b) The presence of mitotic figures (H and E, ×40). (c) The tumor cells show diffuse positivity for vimentin (immunohistochemistry, ×10). (d) The tumor cells show focal positivity for smooth muscle actin (immunohistochemistry, ×10)

Click here to view

The histopathological features were not consistent with the clinical diagnosis of ameloblastic fibrosarcoma because ameloblastic fibrosarcoma shows scattered odontogenic epithelial cell rests, intertwining fibrils in the connective tissue.

   Discussion Top

The glomus tumor is a distinct neoplasm of perivascular cells that resemble modified smooth muscle cells seen in the glomus body. The glomus body is most frequently encountered in the subungual region, lateral areas of the digits and the palm where it is involved in thermal regulation. Glomus tumors are usually solitary, painful and well-circumscribed and treated by simple excision. Rarely, they can be multiple.[1] [Table 1] lists the cases of oral glomus tumors reported in the literature from 1949 to 2015.
Table 1: Cases of glomus tumor affecting the oral cavity

Click here to view

The term glomangioma for the benign tumor was coined by Bailey in 1935. Masson described the occurrence of three histologic patterns –i) angiomatous-most common, ii) solid comprising cellular areas of smooth muscle cells and epithelioid cells and iii) degenerative with hyalinization, edema and mucoid changes in a myxoid stroma. However, these patterns may be mixed in varying proportions in any glomus tumor.[4],[25]

The World Health Organization classifies glomus tumors as glomangioma (prominent vascular component), glomangiomyoma (prominent smooth muscle component) and solid glomus tumor (prominent cellular component).[3],[28]

Variants of glomus tumors include (1) Glomangiomatosis, a benign, diffuse-growing glomus tumor; (2) Symplastic glomus tumor, characterized by marked nuclear atypia (representing a degenerative phenomenon) and no other features of malignancy and (3) Malignant glomus tumor or glomangiosarcoma, which accounts for approximately 1% of all glomus tumors.[1]

Our present case showed sheets of round cells with basophilic cytoplasm, high nuclear-cytoplasmic ratio, nuclear hyperchromatism, nuclear pleomorphism and mitotic figures (9/10HPF), all suggestive of a malignant glomus tumor

Histological differential diagnosis included hemangiopericytoma, myopericytoma, leiomyosarcoma and gastrointestinal stromal tumor (GIST) [Table 2].
Table 2: Differential diagnosis

Click here to view

Hemangiopericytoma is a benign tumor, clinically usually large and situated deep in the connective tissue. Histopathologically, the neoplastic cells of hemangiopericytoma have ovoid to spindle morphology and immunohistochemically show positivity for CD34 and negativity for SMA. Immunohistochemically, our present case was negative for CD34 and positive for SMA.

Myopericytoma is a tumor of neoplastic pericytes with smooth muscle differentiation around vascular channels. The neoplastic cells of myopericytoma are spindle, while in the present case, the neoplastic cells were round to oval. Myopericytoma is positive for SMA and CD34 as was our present case.

Leiomyosarcoma is a malignant tumor of smooth muscles. The neoplastic cells of a leiomyosarcoma have a spindle to round morphology, whereas neoplastic cells of the glomus tumor are round to oval in morphology. Leiomyosarcoma is positive for desmin which was negative in the present case.

GIST is a mesenchymal neoplasm that arises in the gastrointestinal tract. In pediatric patients, GIST occurs as a component of Carney's triad (gastric GIST, extraadrenal paraganglioma and pulmonary chondroma). GIST has spindle cells and shows immunohistochemistry (IHC) positivity for c-KIT and CD34. The present case was in a pediatric patient, however, the cells were round and were negative for CD34.

The clinical, histopathological features (round cells, cellular and nuclear pleomorphism and mitotic figures) and IHC features (vimentin and SMA positivity) led to the diagnosis of malignant glomus tumor.

   Conclusion Top

  • Malignant glomus tumor is one of the rare sarcomas in the oral cavity
  • It is a high-grade sarcoma and should be treated immediately to avoid metastasis
  • It is necessary to consider malignant glomus tumor as one of the differential diagnoses in round cell sarcoma histopathologically
  • To our knowledge, this is the first case of malignant glomus tumor reported in the oral cavity.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

De Chiara A, Apice G, Mori S, Silvestro G, Losito SN, Botti G, et al. Malignant glomus tumour: A case report and review of the literature. Sarcoma 2003;7:87-91.  Back to cited text no. 1
Gopal MG, Manjunath NC, Kumar S, Ramesh M, Nandini AS, 2014. Glomangioma: a rare case report. Journal of Evolution of Medical and Dental Sciences, 3(1), pp.127-133.  Back to cited text no. 2
Boros AL, Davis JP, Sedghizadeh PP, Yamashita DD. Glomus tumor: Report of a rare case affecting the oral cavity and review of the literature. J Oral Maxillofac Surg 2010;68:2329-34.  Back to cited text no. 3
Biswas KD, Sen A, Guha G, Misra S, Sinha R. 2014. Glomus tumor in floor of mouth: a case report. Journal of Evolution of Medical and Dental Sciences, 3(22), pp.6036-6043.  Back to cited text no. 4
Von Langer R. Glomustumor am harten Gaumen. Wein Med Wochenschr 1949;99:67.  Back to cited text no. 5
King ES. Glomus tumor. Aust N Z J Surg 1954;23:280.  Back to cited text no. 6
Kirschner H, Strassburg M. A glomus tumor localized on the alveolar process of the lower jaw. Dtsch Zahnarzl Z 1962;17:912.  Back to cited text no. 7
Grande R, D'Angelo E. A glomic tumor of the hard palate. Arch Ital Laringol 1962;70:89.  Back to cited text no. 8
Frankel VG. Occurrence of a leiomyofibromangioma (Glomangiomas). Dtsch Zahnartzl Z 1965;20:168.  Back to cited text no. 9
Harris R, Griffin CJ. Glomus tumour of the periodontal tissues. Aust Dent J 1965;10:33-7.  Back to cited text no. 10
Sidhu SS, Subherwal GL. Glomus tumor of the palate. Indian Dent 1967;39:167.  Back to cited text no. 11
Charles NC. Multiple glomus tumors of the face and eyelid. Arch Ophthalmol 1976;94:1283-5.  Back to cited text no. 12
Sato M, Shirasuna K, Sakuda M, Yanagawa T, Yoshida H, Imai J, et al. Fine structure of a glomus tumor of the tongue and expression of C type virus in its tumor cells. Int J Oral Surg 1979;8:199.  Back to cited text no. 13
Tajima Y, Weathers DR, Neville BW, Benoit PW, Pedley DM. Glomus tumor (glomangioma) of the tongue: A light and electron microscopic study. Oral Surg Oral Med Oral Pathol 1981;52:288.  Back to cited text no. 14
Saku T, Okabe H, Matsutani K, Sasaki M. Glomus tumor of the cheek: An immunohistochemical demonstration of actin and myosin. Oral Surg Oral Med Oral Pathol 1985;60:65-71.  Back to cited text no. 15
Ficarra G, Merrell PW, Johnston WH, Hansen LS. Intraoral solitary glomus tumor (glomangioma): Case report and literature review. Oral Surg Oral Med Oral Pathol 1986;62:306-11.  Back to cited text no. 16
Moody GH, Myskow M, Musgrove C. Glomus tumor of the lip. A case report and immunohistochemical study. Oral Surg Oral Med Oral Pathol 1986;62:312-8.  Back to cited text no. 17
Stajcić Z, Bojić P. Intraoral glomus tumour. A case report. J Craniomaxillofac Surg 1987;15:376-8.  Back to cited text no. 18
Geraghty JM, Thomas RW, Robertson JM, Blundell JW. Glomus tumour of the palate: Case report and review of the literature. Br J Oral Maxillofac Surg 1992;30:398-400.  Back to cited text no. 19
Kusama K, Chu L, Kidokoro Y, Kouzu M, Uehara T, Honda M, et al. Glomus tumor of the upper lip. J Nihon Univ Sch Dent 1995;37:97-101.  Back to cited text no. 20
Sakashita H, Miyata M, Nagao K. Glomus tumor in the upper lip. A case report. Int J Oral Maxillofac Surg 1997;26:301-2.  Back to cited text no. 21
Yu HJ, Kwon SJ, Bahn JY, Park JM, Park YW. Localized multiple glomus tumors of the face and oral mucosa. J Dermatol 2000;27:211-3.  Back to cited text no. 22
Kessaris P, Klimis T, Zanakis S. Glomus tumour of the hard palate: Case report and review. Br J Oral Maxillofac Surg 2001;39:478-9.  Back to cited text no. 23
Rallis G, Komis C, Mahera H. Glomus tumor: A rare location in the upper lip. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004;98:327-36.  Back to cited text no. 24
Lanza A, Moscariello A, Villani R, Colella G. Glomus tumor of the lower lip. A case report. Minerva Stomatol 2005;54:687-90.  Back to cited text no. 25
Dérand P 3rd, Warfvinge G, Thor A. Glomangioma: A case presentation. J Oral Maxillofac Surg 2010;68:204-7.  Back to cited text no. 26
Mohan M, Pandya K, Pradeep S. Glomangioma on the upper lip is rare. Indian Dent Res Rev 2015;10:46-7.  Back to cited text no. 27
World Health Organization Classification of Tumors. In: Fletcher DC, Unni KK, Mertens F, editors. Glomus tumors. Pathology and Genetics of Tumors of Soft Tissue and Bone. Lyon: IARC; 2002. p. 137.  Back to cited text no. 28


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

  [Table 1], [Table 2]


Print this article  Email this article


    Similar in PUBMED
  Related articles
    Article in PDF (2,242 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Report
    Article Figures
    Article Tables

 Article Access Statistics
    PDF Downloaded42    
    Comments [Add]    

Recommend this journal

Journal of Oral and Maxillofacial Pathology | Published by Wolters Kluwer - Medknow
Online since 15th Aug, 2007