Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 7  |  Issue : 3  |  Page : 309-311

Extradigital glomus tumor of left forearm: An unusual cause for persistent forearm pain


1 Department of Vascular and Endovascular Surgery, Yashoda Hospitals, Hyderabad, Telangana, India
2 Department of Radiodiagnosis, Yashoda Hospitals, Hyderabad, Telangana, India

Date of Submission16-Jan-2020
Date of Decision21-Jan-2020
Date of Acceptance07-Mar-2020
Date of Web Publication12-Sep-2020

Correspondence Address:
Devender Singh
Department of Vascular and Endovascular Surgery, Yashoda Hospitals, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_8_20

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  Abstract 


Glomus tumors are rare, benign, and vascular neoplasm of the glomus body, accounting <2% of all soft tissue tumors. The most common site is the subungual region of the fingers and toes. Extradigital sites, including the forearm, are uncommon and usually misdiagnosed because of their rarity and nonspecific presentation. Diagnosis is often delayed due to low level of suspicion. A strong clinical suspicion, magnetic resonance imaging, and a properly planned surgery remain the treatment. We report a 70-year-old male presented with a very small painful left forearm swelling for the last 20 years who had undergone surgical excision.

Keywords: Extradigital glomus, forearm, surgical excision, vascular tumor


How to cite this article:
Singh D, Polineni S, Anurshetru B, Shalini A. Extradigital glomus tumor of left forearm: An unusual cause for persistent forearm pain. Indian J Vasc Endovasc Surg 2020;7:309-11

How to cite this URL:
Singh D, Polineni S, Anurshetru B, Shalini A. Extradigital glomus tumor of left forearm: An unusual cause for persistent forearm pain. Indian J Vasc Endovasc Surg [serial online] 2020 [cited 2020 Dec 4];7:309-11. Available from: https://www.indjvascsurg.org/text.asp?2020/7/3/309/294914




  Introduction Top


Glomus tumors are benign neoplasms containing cells from the glomus apparatus, which is responsible for thermoregulatory control, accounting 1–2% of all soft tissue tumors.[1],[2] Histologically, glomus tumors and their variants are benign neoplasms requiring only a simple excision.[3] Painful subcutaneous nodule formation located on the subungual area of the digits is the usual presentation of glomus tumors.[2] Extradigital glomus tumors are rare lesions, and their diagnosis is often delayed due to low level of suspicion.[4] We present a case of extradigital glomus tumor of the left forearm suffering for the last 20 years, treated with correct diagnosis and surgical excision.


  Case Report Top


We report a 70-year-old male otherwise healthy presenting with complaints of painful, tender swelling on the radial aspect of the left distal forearm for the last 20 years. Pain is excruciating and forced him to visit multiple specialists, including neurologists, orthopedicians, surgeons, and physicians for the last 4 years. The swelling is 2 cm × 1 cm, bluish, and extremely tender, not even allowing to be touched [Figure 1]. He was treated with various combination of drugs, i.e., analgesics, anxiolytics, antidepressants, and alternative medicines, but of no improvement. On examination, he was pointing over a spot which was exquisitely tender. Hildreth's and Love's signs were positive. Plain films of the affected thumb did not reveal any abnormality. With a strong suspicion of extradigital glomus tumor, he underwent ultrasound and magnetic resonance imaging (MRI), which revealed a subcutaneous vascular lesion (18 mm × 9 mm) encasing cephalic vein of the left distal forearm [Figure 2]. The swelling was excised [Figure 3] with a part of the cephalic vein [Figure 4] under local anesthesia and sent for histopathological examination, which confirmed glomus tumor [Figure 5] and [Figure 6]. The patient is pain-free after excision.
Figure 1: Extradigital glomus tumor of left forearm

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Figure 2: Magnetic resonance imaging showing the lesion in the left forearm

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Figure 3: Peroperative picture showing glomus tumor of the left cephalic vein

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Figure 4: Specimen of extradigital glomus tumor of left forearm cephalic vein

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Figure 5: Routine H and E staining demonstrates a perivascular, proliferation of homogenous round cells with round-to-ovoid nuclei arranged in multicellular layers around blood vessels

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Figure 6: Routine H and E staining demonstrates a perivascular, proliferation of homogenous round cells with round-to-ovoid nuclei arranged in multicellular layers around blood vessels. Lesional cells are set in a background of myxoid matrix with stellate cells

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  Discussion Top


The extradigital glomus tumor is an uncommon condition rarely coming into consideration in routine practice, and therefore, it commonly creates a confusion in diagnosis.[5] Extradigital glomus tumor is a rare presentation commonly encountered in the lower extremity.[6] In this patient, it was over left forearm. These patients give a typical history of excruciating pain and to the extent of demanding an amputation.[7] Careful history and clinical examination are the important tools in the diagnosis.

Clinically, glomus tumors are characterized by a triad of sensitivity to cold, localized tenderness, and severe and intermittent pain,[8] which can be excruciating and is described as a burning or bursting. The exact cause of the pain is unknown, but nerve fibers containing the pain neurotransmitter substance P have been identified in the tumors.[9] Diagnosis of glomus tumors is primarily clinical. Several clinical tests are useful for diagnosing glomus tumors. Love reported that localization of the tenderness to an area and the size of a pinhead were suggestive of glomus tumor.[10] For a positive Love's pin test, the patient should experience severe pain when the skin overlying the tumor is pressed with a ballpoint pen, end of a paperclip, pinhead, or K-wire. The cold-sensitivity test is positive when immersing the hand in cold water for 60 s elicits severe pain in and around the lesion. In addition, there should be a history of cold weather aggravating the symptoms being evident in our patient. Hildreth's test is another reliable clinical sign for the diagnosis of glomus tumors.[11] This test is performed by elevating the patients' arm to exsanguinate it. A tourniquet is inflated to 250 mmHg, and if the test is positive, then there is a reduction in pain and tenderness. A test is also positive when releasing the cuff causes a sudden onset of pain and tenderness in the area of the tumor. Ultrasound imaging is considered as the initial and usually the most useful method for the evaluation of the glomus tumor.[12] MRI may further be useful for the detection of the tumor.[13]

The preferred treatment for isolated glomus tumor is a complete surgical excision.[14] The incidence of tumor recurrence after surgical excision ranges from 5% to 50%, depending primarily on the surgical technique.[15]


  Conclusion Top


Glomus tumors are benign vascular tumors but usually impact patients' quality of life, due to the severity of the pain and the persistence of symptoms for years before being diagnosed. Atypical locations and presentations of extradigital tumors can lead to misdiagnosis, particularly where the lesion is rarely reported. Therefore, being aware of this variation and special characteristics of extradigital tumors can lead to early diagnosis and treatment. Finally, physical examination of painful subcutaneous mass in the limbs should always be conducted with the raise suspicion of glomus tumor.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Enzinger SW, Weiss FM. Perivascular tumors. In: Soft Tissue Tumors. 5th ed. St. Louis: Mosby Elsevier; 2008. p. 751-65.  Back to cited text no. 1
    
2.
Veros K, Markou K, Filitatzi C, Kyrmizakis DE. Glomus tumor of the cheek: A case report. Case Rep Med 2012;2012:307294.  Back to cited text no. 2
    
3.
Nigam JS, Misra V, Singh A, Karuna V, Chauhan S. A glomus tumour arising from the flexor aspect of the forearm: A case report with review of the literature. J Clin Diagn Res 2012;6:1559-61.  Back to cited text no. 3
    
4.
Frumuseanu B, Balanescu R, Ulici A, Golumbeanu M, Barbu M, Orita V, et al. A new case of lower extremity glomus tumor up-to date review and case report. J Med Life 2012;5:211-4.  Back to cited text no. 4
    
5.
Anagnostou GD, Papademetriou DG, Toumazani MN. Subcutaneous glomus tumors. Surg Gynecol Obstet 1973;136:945-50.  Back to cited text no. 5
    
6.
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;25:1-2.  Back to cited text no. 6
    
7.
Singh D. Subungal glomus tumor: Rare but real. Indian J Vasc Endovascular Surg 2015;2:115-7.  Back to cited text no. 7
    
8.
Vandevender DK, Daley RA. Benign and malignant vascular tumors of the upper extremity. Hand Clin 1995;11:161-81.  Back to cited text no. 8
    
9.
Kishimoto S, Nagatani H, Miyashita A, Kobayashi K. Immunohistochemical demonstration of substance P-containing nerve fibres in glomus tumours. Br J Dermatol 1985;113:213-8.  Back to cited text no. 9
    
10.
Love JG. Glomus tumors: Diagnosis and treatment. Proc Staff Meet Mayo Clin 1944;19:113-6.  Back to cited text no. 10
    
11.
Giele H. Hildreth's test is a reliable clinical sign for the diagnosis of glomus tumours. J Hand Surg Br 2002;27:157-8.  Back to cited text no. 11
    
12.
Höglund M, Muren C, Brattström G. A statistical model for ultrasound diagnosis of soft-tissue tumours in the hand and forearm. Acta Radiol 1997;38:355-8.  Back to cited text no. 12
    
13.
Takei TR, Nalebuff EA. Extradigital glomus tumour. J Hand Surg Br 1995;20:409-12.  Back to cited text no. 13
    
14.
Mullis WF, Rosato FE, Rosato EF, Butler CJ, Mayer LJ. The glomus tumor. Surg Gynecol Obstet 1972;135:705-7.  Back to cited text no. 14
    
15.
van Geertruyden J, Lorea P, Goldschmidt D, de Fontaine S, Schuind F, Kinnen L, et al. Glomus tumours of the hand. A retrospective study of 51 cases. J Hand Surg Br 1996;21:257-60.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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