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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 6  |  Issue : 1  |  Page : 39-41

Solitary extradigital glomangioma with thrombus presenting as a painful nodule


1 Department of Dermatology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
2 Department of Dermatology, Dermapure Skin and Laser Clinic, Pathankot, Punjab, India

Date of Web Publication13-Jun-2019

Correspondence Address:
Dr. Uday S Khopkar
Department of Dermatology, Seth GS Medical College and KEM Hospital, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_54_18

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  Abstract 


Glomangioma, currently termed as glomuvenous malformation, is a rare type of venous malformation (VM) with a rim of glomus cells around the dilated vessels. This glomus cell lesion is not usually as painful as solid glomus tumor. Rarely, thrombus may be seen in the vessels like other VMs. A male with adult-onset extradigital glomangioma presented with a painful nodule with thrombus seen on histopathology. The thrombus was responsible for the tenderness of the lesion, and its presence confirms it to be a malformation than a tumor.

Keywords: Extradigital, glomangioma, solitary, thrombus


How to cite this article:
Kumari I, Mohanty S, Khopkar US, Charak P. Solitary extradigital glomangioma with thrombus presenting as a painful nodule. Indian J Dermatopathol Diagn Dermatol 2019;6:39-41

How to cite this URL:
Kumari I, Mohanty S, Khopkar US, Charak P. Solitary extradigital glomangioma with thrombus presenting as a painful nodule. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2019 Nov 19];6:39-41. Available from: http://www.ijdpdd.com/text.asp?2019/6/1/39/260192




  Introduction Top


Glomuvenous malformation (GVM), traditionally termed as “glomangioma,” resembles a venous malformation (VM) on histopathology where lesional vessels are rimmed by glomus cells. It accounts for 10%–20% of all glomus cell hamartomas or tumors. It often presents as multiple soft, red to blue nodules or plaques during infancy or childhood.[1] In comparison to glomus tumor, GVMs are less painful. Thrombi or phleboliths may be present in the dilated vessels in GVM, but are more common in VMs without glomus cells. We report a case of acquired, solitary, and painful glomangioma located at the arm in an adult male with histopathology showing thrombus in dilated vessels.


  Case Report Top


A 43-year-old male presented with a single, bluish elevated lesion over the right arm for 6 months. The lesion gradually increased in size and was painful for few days. He also complained of unusual tingling and burning sensation at the lesion. No seasonal variation in pain and temperature sensitivity was present. On examination, bluish, tender nodule of size 0.8 cm and soft to firm in consistency was present at the right arm [Figure 1]. There was no history of trauma or bleeding or similar lesion in the family. The differential diagnosis involved glomus tumor, blue nevus, spiradenoma, and leiomyoma. Routine hematological investigations were within normal range. Histopathological examination showed multiple dilated vascular spaces in the dermis with large thrombus. The vessels were surrounded by multiple layers of cuboidal glomus cells with ovoid nuclei and eosinophilic cytoplasm [Figure 2] and [Figure 3]. The findings were suggestive of glomangioma. The lesion was surgically excised.
Figure 1: Tender bluish nodule present over the arm

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Figure 2: (a and b) Histopathology showing dilated vascular spaces with large thrombus and rim of cuboidal glomus cells surrounding the vessels (H and E, ×10, ×20)

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Figure 3: (a and b) Higher magnification depicting the pericytes and the glomus cells (H and E, ×20, ×40)

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  Discussion Top


Masson described the neuromyoarterial glomus in 1924, which was later renamed as the neurovascular glomus. Glomus cells are modified smooth muscle cells located in the walls of the Sucquet–Hoyer canal, a specialized arteriovenous anastomosis which plays an important role in thermoregulation. Tumors comprising glomus cells account for 1%–2% of all soft-tissue tumors. On histopathology, glomus cells appear cuboidal with central round nucleus and scant eosinophilic cytoplasm and form a perivascular “collar” around the vessels. On immunohistochemistry, the glomus cells stain positively for smooth muscle-α-actin and vimentin.

There are three types of lesions comprising glomus cell proliferation. The three different tumor variants have minor clinical differences and are mainly differentiated by their histological features. The more common glomus tumor or solid form has mainly glomus cells with a poor vasculature and scant smooth muscle components, while glomangiomas have a prominent distorted vascular component along with few glomus cells, and glomangiomyomas are composed of prominent vascular and smooth muscle components.[2]

The term glomangioma was coined by Bailey in 1935 for lesions with wider vascular lumen. The term was also used for multiple and early onset glomus tumors. However, currently, they are categorized as GVMs due to their resemblance to VMs and early onset of presentation. Glomangiomas present mostly as multiple nodules and are located on the upper extremities and occasionally on the lower extremities, head, and back.[3] Rarely, solitary and plaque forms are also reported.[4] They may be present at birth or appear during infancy and childhood. The lesions grow with increasing age. The late age of onset is less commonly seen as in our case. The familial variant of glomangioma is known to be associated with chromosome 1p21-22, encoding for the glomulin gene.[5] Two-thirds of cases are familial, although sporadic cases are also known.[6]

In comparison to the glomus tumor, glomangiomas may be less commonly tender on palpation, and attacks of pain may occur in association with menstruation and pregnancy. Although uncommon by site, clinically our case presented as a glomus tumor as the lesion was solitary and painful, but histologically it turned out to be a VM with the rim of glomus cells and large thrombus. Other histological variants of glomangioma include the presence of mucin.[7] The presence of thrombus in vessels is more commonly seen in painful VMs. In GVMs, it has rarely been mentioned. The diagnostic differences between glomus tumor, GVMs, and VMs are highlighted in [Table 1].
Table 1: Most common diagnostic features of glomus tumors, glomuvenous malformations, and venous malformations

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It must be differentiated from other cutaneous painful tumors such as eccrine spiradenoma, leiomyoma, neural tumors, granular cell tumor, angiolipoma, and when multiple blue rubber bleb nevus.

This case is being reported for the rarity of occurrence of acquired, solitary, and symptomatic glomangioma and the presence of large thrombus in numerous dilated vessels indicating glomangioma to be more of VM than a glomus cell tumor. Treatment is not always indicated, particularly in asymptomatic cases of glomangioma. However, symptoms in our case warranted for surgical excision considering solitary and small lesion. Laser treatment, electromagnetic radiation, and sclerotherapy are other modalities available.[8]

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.
Jha A, Ramesh V, Singh A. Disseminated cutaneous glomuvenous malformation. Indian J Dermatol Venereol Leprol 2014;80:556-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med 2008;132:1448-52.  Back to cited text no. 2
    
3.
Rao AG, Indira D, Kamal J. Extra digital glomangioma. Indian J Dermatol 2010;55:397-8.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Requena L, Galvan C, Sánchez Yus E, Sangueza O, Kutzner H, Furio V, et al. Solitary plaque-like telangiectatic glomangioma. Br J Dermatol 1998;139:902-5.  Back to cited text no. 4
    
5.
Brouillard P, Boon LM, Mulliken JB, Enjolras O, Ghassibé M, Warman ML, et al. Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations (“glomangiomas”). Am J Hum Genet 2002;70:866-74.  Back to cited text no. 5
    
6.
Mounayer C, Wassef M, Enjolras O, Boukobza M, Mulliken JB. Facial “glomangiomas”: Large facial venous malformations with glomus cells. J Am Acad Dermatol 2001;45:239-45.  Back to cited text no. 6
    
7.
Godse KV. Glomus tumor with mucinous change. Indian J Dermatol Venereol Leprol 2005;71:367-8.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Myers RS, Lo AK, Pawel BR. The glomangioma in the differential diagnosis of vascular malformations. Ann Plast Surg 2006;57:443-6.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]



 

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