Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

Ahead of print publication  

A malignant glomus tumor in the nasal cavity responding to radiation therapy

 Department of Radiation Oncology, Biomedical Research Institute, Pusan National University Hospital and Pusan National University School of Medicine, Busan, Korea

Date of Submission17-Aug-2021
Date of Acceptance23-Sep-2021
Date of Web Publication03-May-2022

Correspondence Address:
Donghyun Kim,
Department of Radiation Oncology, Pusan National University Hospital and Pusan National University School of Medicine, Busan
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_1394_21

 > Abstract 

Malignant glomus tumors of the head and neck are extremely rare, and to our knowledge, a response to high-dose radiation has not been described previously. We report one case in an 80-year-old woman with right nasal cavity mass. Histological examination revealed sheets of atypical round glomus cells. The presence of increased mitotic activity (25 per 10 high-power fields), cellular atypism, and tumor necrosis suggested malignancy. The smooth muscle actin, vimentin, and h-caldesmon immunohistochemistry stains the tumor cells. Two cycles of doxorubicin and cyclophosphamide chemotherapy were done and the tumor size was slightly increased. Salvage radiation therapy (RT) was delivered to the primary mass over 4 weeks (50 Gy in 20 fractions) and leading to nearly complete regression of tumor. Additional investigations are warranted so that we may determine the usefulness of RT in the management of this rare tumor.

Keywords: Head and neck, malignant glomus tumor, radiation therapy

How to cite this URL:
Nam J, Park D, Kim W, Kim D. A malignant glomus tumor in the nasal cavity responding to radiation therapy. J Can Res Ther [Epub ahead of print] [cited 2022 Nov 29]. Available from: https://www.cancerjournal.net/preprintarticle.asp?id=344710

 > Introduction Top

Malignant glomus tumors (MGTs) are rare mesenchymal tumors that arise from the glomus body.[1] The glomus body is a form of arteriovenous anastomosis functioning as a shunt to modify blood flow, facilitating temperature regulation, and most frequently located in the subungual region of the extremities.[2] MGTs have been reported in the oral mucosa, larynx, paranasal sinuses, and eyelids.[3] Surgical resection is the mainstay of treatment but can be challenging in the locally advanced MGTs. To our knowledge, a response of MGTs to high-dose radiation therapy (RT) has not been reported.

 > Case Report Top

A previously healthy 80-year-old woman visited our hospital because of persistent epistaxis. Magnetic resonance imaging [Figure 1]a showed a well-enhancing mass on the right ethmoid, maxillary sinus, and nasal cavity. There were orbital space extensions with the destruction of lamina papyracea and invasion of the right pterygopalatine fossa. The metastatic lesion was not presented in the initial positron emission tomography scan. A punch biopsy was performed. Histologic evaluation showed sheets of atypical round cells with intervening blood vessels, hyperchromatic nuclei, and brisk mitotic activity (25/10 high-power fields [HPF]). Areas of necrosis and hemorrhage were also present. Immunohistochemical analysis revealed positive staining for smooth muscle actin, vimentin, and h-caldesmon [Figure 2]. Staining for desmin, S-100 protein, CD34, myogenin, leukocyte common antigen and cytokeratin were all negative. Based on these features, the case was consistent with MGT.
Figure 1: (a) The axial (left) and coronal (right) plane of magnetic resonance imaging scans before treatment. (b) The axial (left) and coronal (right) plane of magnetic resonance imaging scans after chemotherapy. (c) The axial (left) and coronal (right) plane of magnetic resonance imaging scans after salvage radiation therapy. All magnetic resonance imaging protocol is fat-suppressed T1-weighted

Click here to view
Figure 2: (a) Microscopic finding shows sheets of atypical round cells with intervening blood vessels, hyperchromatic nuclei, and mitoses (H and E, ×400). (b) Tumor cells are diffusely immune-reactive for smooth muscle actin (SMA, ×400)

Click here to view

Complete resection would be impossible without the sacrifice of the right orbit. Therefore, following multidisciplinary review, the patient was initially treated with two cycles of doxorubicin 60 mg/m2 and cyclophosphamide 600 mg/m2 chemotherapy but had no response [Figure 1]b. Salvage RT was delivered to the primary mass over 4 weeks [50 Gy in 20 fractions, [Figure 3]] and leading to nearly complete regression of tumor [Figure 1c]. Computed tomography scan of the chest and abdomen revealed multiple lung and liver metastases at 3-months after completion of RT. She expired 4 months thereafter.
Figure 3: Color wash radiation dose distribution in the axial (a), sagittal (b), and coronal (c) planes

Click here to view

 > Discussion Top

Glomus tumors (GTs) are distinct from paraganglioma which arise from the glomus jugulare or glomus tympanicum.[4] GTs are composed of smooth muscle cells and paraganglioma originates from the neural crest.[3] Therefore these tumors have different disease course and different treatment strategy is needed. Histologically, GTs are composed of a sheet-like proliferation of uniform, round-to-polygonal cells with eosinophilic cytoplasm and central round nuclei. Intervening blood vessels are present as well as a variable amount of smooth muscle cells. All of the GTs expressed smooth muscle actin, collagen type IV and vimentin, 19% expressed CD34, 6% expressed desmin, and no case was immunoreactive for S-100 or cytokeratin.[5]

Less than 1% of GTs are malignant. MGT, also known as glomangiosarcoma, is a highly aggressive and metastatic tumor.[5] Based on the World Health Organization classification,[6] criteria of MGTs are as follows: (1) marked nuclear atypism and mitotic activity (>5 of 50 HPF) or (2) atypical mitotic figures.

The effect of RT on MGTs has not been formally evaluated. In reported cases, tumor continue to progress after the application of RT.[7],[8] One patient underwent excision followed by adjuvant RT (total dose was not provided) to the right cheek and infratemporal area, and the tumor recurred after 4 years.[9] The other case report presented a patient with a tracheal MGT that received RT (total dose of 7.5 Gy) for palliation of respiratory symptoms; however, the tumor progressed and the patient expired.[10] Due to lack of evidence, radical, adjuvant, or palliative aim RT is usually not recommended.

Metastasis is the main cause of mortality in MGTs. Initially, MGTs were considered low-metastatic potential, locally aggressive malignancies. However, large case series demonstrated that MGTs have aggressive disease course, with metastasis and death of disease were recorded up to 40%.[5] Therefore, MGT should be managed as high-grade sarcoma although standard treatment guidelines have not yet been established.[6] Early multiple distant metastasis occurred in this case as well despite regression of primary tumor after RT. Therefore, meticulous attention is highly recommended, even though the primary tumor has been completely regressed.

We describe our experience with a head and neck MGT showing nearly complete response after high-dose RT. Due to the rarity of this tumor, further studies are needed to determine the utility of RT in the treatment of this malignancy. We believe that this report may be beneficial in future decision-making for the management of patients with unresectable head and neck MGTs.

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


Conflicts of interest

There are no conflicts of interest.

 > References Top

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. 1
Kayal JD, Hampton RW, Sheehan DJ, Washington CV. Malignant glomus tumor: A case report and review of the literature. Dermatol Surg 2001;27:837-40.  Back to cited text no. 2
Aslam N, Qazi ZU, Ahmad AH, Khan RU. Malignant glomus tumour of larynx: First case report and literature review. J Laryngol Otol 2012;126:743-6.  Back to cited text no. 3
Mishra P, Padhi S, Behera G. Thyroid paraganglioma: A case-based systematic review of literature. J Can Res Ther 2020;16:11-21.  Back to cited text no. 4
[PUBMED]  [Full text]  
Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: Analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1-12.  Back to cited text no. 5
Folpe AL, Brems H, Legius E. Glomus tumours. In: Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. World Health Organization Classification of Tumours of Soft Tissue and Bone. Lyon: IARC; 2013. p. 116-7.  Back to cited text no. 6
Yu DK, Cho KH, Kim YJ, Heo DS. Tracheal glomangiosarcoma with multiple skin metastasis. J Dermatol 2004;31:776-8.  Back to cited text no. 7
Wolter NE, Adil E, Irace AL, Werger A, Perez-Atayde AR, Weldon C, et al. Malignant glomus tumors of the head and neck in children and adults: Evaluation and management. Laryngoscope 2017;127:2873-82.  Back to cited text no. 8
Gould EW, Manivel JC, Albores-Saavedra J, Monforte H. Locally infiltrative glomus tumors and glomangiosarcomas. A clinical, ultrastructural, and immunohistochemical study. Cancer 1990;65:310-8.  Back to cited text no. 9
Choi YJ, Yang KH, Gang SJ, Kim BK, Kim SM. Malignant glomus tumor originating in the superior mediastinum – An immunohistochemical and ultrastructural study. J Korean Med Sci 1991;6:157-63.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


     Search Pubmed for
    -  Nam J
    -  Park D
    -  Kim W
    -  Kim D
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

  >Abstract>Introduction>Case Report>Discussion>Article Figures
  In this article

 Article Access Statistics
    PDF Downloaded10    

Recommend this journal