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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 3  |  Page : 414-416
Extradigital glomus tumor as a cause of chronic perianal pain


1 Department of Pathology, Command Hospital (WC), Chandimandir, India
2 Department of Surgery, Command Hospital (WC), Chandimandir, India

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Date of Web Publication12-Aug-2009
 

   Abstract 

Glomus tumor is a rare perivascular benign tumor arising from the Sucquet-Hoyer canal of the normal glomus body, most commonly in the digital areas. We report a serving soldier with such a tumor in an atypical site, the perianal region, presenting with episodic shooting pain. Total surgical excision was performed. Histopathology revealed a well-circumscribed tumor composed of clusters of monotonous polygonal cells surrounding capillary-sized blood vessels. Tumor cells also showed immunopositivity for smooth muscle antigen and vimentin. Following excision, the patient was completely relieved of pain and there was no recurrence on follow-up for 6 months.

Keywords: Extradigital, glomus tumor, painful perianal nodule

How to cite this article:
Rathi K R, Jena J, Dash B M, Mitra D, Patnaik P K, Basu A R. Extradigital glomus tumor as a cause of chronic perianal pain. Indian J Pathol Microbiol 2009;52:414-6

How to cite this URL:
Rathi K R, Jena J, Dash B M, Mitra D, Patnaik P K, Basu A R. Extradigital glomus tumor as a cause of chronic perianal pain. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Oct 21];52:414-6. Available from: http://www.ijpmonline.org/text.asp?2009/52/3/414/55012



   Introduction Top


Glomus tumors are benign vascular tumors. They are derived from the modified smooth muscle cells of the glomus body. A normal glomus is a specialized form of arteriovenous anastomosis that is responsible for blood and temperature regulation. The most common site for these tumors is the distal extremities, especially in the subungual digital areas, although they have been described in extradigital locations such as bone, tongue, stomach, rectum, mesentery, lung, mediastinum, sacrum, coccyx and head and neck areas. [1],[2] The symptom triad is paroxysmal pain, contact tenderness and temperature hypersensitivity, especially to cold. Treatment outcomes are excellent but diagnosis of glomus tumors can sometimes test the diagnostic acumen of the physicians. This applies especially to those tumors with unusual locations and manifestations. Correct diagnosis may not be made, which results in the long duration of symptoms suffered by the patients. Here, we report a case of typical glomus tumor in a very unusual location, i.e. the perianal region.


   Case Report Top


A 22-year-old serving soldier presented with history of painful nodule in the perianal region for 2 years. It was initially thought clinically as a case of healed perianal fistula-in-ano; however, the diagnosis could not be substantiated and he was managed conservatively. The patient's symptom of pain increased in intensity; however, there was no increase in the size of the nodule. The nodule was intensely painful to touch, pressure and accidental trauma. There was no effect of any routine pain killers and pain relieved spontaneously after 2-3 hours. He had been avoiding physical military training for fear of accidental trauma as the slightest injury even by mild friction of buttocks could cause excruciating pain. There was no history of any bowel or bladder disturbances or history of exposure. Physical examination revealed a perianal swelling 1.5 cm in diameter on the right side, which was extremely tender to touch. X-ray did not reveal any abnormality. A magnetic resonance imaging scan of the perianal region revealed a 1.5 cm well-circumscribed growth lying free in the perianal fat with no communication with the rectum, urethra or skin. Peroperative examination revealed a shiny encapsulated nodule, which was excised in toto, and was sent for histopathological examination. There was a 1.5 cm 1.0 cm 1.0 cm single grayish-blue nodule with a smooth encapsulated surface. Hematoxylin and eosin-stained sections demonstrated a relatively well-circumscribed lesion characterized by solid aggregates of round cells around small capillary-sized vessels in a myxoid stroma. The cells were round, regular shaped with a sharply punched-out rounded nucleus[Figure 1]. Immunoreactivity for smooth muscle antigen (SMA) and vimentin was observed in the cytoplasm of the tumor cells. These cells were negative for cytokeratin (CK), epithelial membrane antigen (EMA) and S-100 protein [Figure 2]. Based on clinical presentation and features of histopathology and immunohistochemistry, a diagnosis of glomus tumor was made. Post operatively, the patient was completely relieved of pain.


   Discussion Top


Glomus tumors are rare, small, painful and benign tumors that arise from the Sucquet-Hoyer canal of the glomus body, located in the subcutaneous tissue, which is responsible for regulation of temperature and blood pressure. Accordingly, glomus tumors are commonly located in the subungual region of the finger because normal glomus bodies are highly concentrated around the digital areas. Extradigital glomus tumors are not a rare subgroup of glomus tumors and these have been reported from the respiratory tract, head and neck, gastrointestinal tract, sacrum, coccyx and bone. [2],[3]

Glomus tumors may be single or multiple. Usually, single tumors are idiopathic and multiple ones are inherited as an autosomal dominant trait. Solitary glomus tumors, particularly subungual lesions, are more common in females than in males. Multiple lesions are slightly more common in males. Solitary glomus tumors are more frequent in adults than in others. Multiple glomus tumors develop 10-15 years earlier than single lesions. [4] Based on clinical and histological features, glomus tumors have been divided into three main categories, such as glomus tumors proper, glomangioma and glomangiomyoma. The classical triad of aching pain, exquisite tenderness and temperature sensitivity often provide the diagnosis, but an atypical anatomical site may make this difficult. In our case, there was painful perianal swelling and clinically no firm diagnosis could be made. Fine needle aspiration cytology was advised, but the patient did not even allow the lesion to be touched. There was also no clue from radiological investigations to confirm the diagnosis.

Grossly, solitary glomus tumors are blue or purple nodules that are usually less than 1 cm in size. Multiple glomus tumors could be localized, disseminated or congenital plaque-like forms. [5]

Microscopically, the glomus tumors are relatively well-circumscribed capsulated lesions and show numerous small, vascular lumina surrounded by clusters of monotonous polygonal glomus cells set in a hyalinized or myxoid stroma. The glomus cells are characterized by a faintly eosinophilic cytoplasm and large, punched-out rounded pale nuclei with delicate chromatin and inconspicuous nucleoli. Two rare variants of glomus cells have been described - epithelioid and oncocytic. The epithelioid lesions are composed of large polygonal to spindle-shaped cells with abundant eosinophilic cytoplasm and large, irregularly shaped nuclei. [6] The oncocytic variant shows abundant granular, eosinophilic cytoplasm. [7] Rapidly growing atypical and malignant glomus tumors have also been described in the literature, which are more than 1 cm in size and are located in the deep soft tissue. [8]

Immunohistochemically, the glomus tumors are positive for a-SMA and vimentin and negative for CK, EMA, S-100, CEA and chromogranin. Focally, CD34 may be positive in 25% of the cases. [9] Although the index tumor showed characteristic clinical and histological features of a glomus tumor, few differential diagnoses such as adnexal tumor, carcinoid tumors and nevus were entertained due to the very unusual location of the tumour. The tumor cells revealed a strong immunopositivity for SMA and vimentin in the cytoplasm and negativity for CK and S-100 protein.

The treatment of choice for solitary glomus tumors is surgical excision. For multiple glomus tumors, excision may be more difficult because of their poor circumscription and the large number of lesions. The overall prognosis is good when they are treated with wide excision. In the present case, the tumor was completely excised and there was no recurrence of pain on follow-up for 6 months.

This case report emphasized the importance of considering glomus tumor in the differential diagnosis of any painful swelling even in an atypical site. An early and accurate diagnosis would prevent unnecessary delay in treatment. Of course, final diagnosis can be provided by the histopathological findings.

 
   References Top

1.Kale SS, Rao VK, Bentz ML. Glomus tumor of the index finger. J Craniofac Surg 2006;17:801-4.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Enzinger FM, Weiss SW. Perivascular tumors. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. 4th ed. St. Louis: Mosby, 2001:985- 1035.  Back to cited text no. 2    
3.Heys SD, Brittened J, Atkinson P, Eremin O . Glomus tumour: An analysis of 43 patients and review of the literature. Br J Surg 1992;79:345-7.  Back to cited text no. 3    
4.Moor EV, Goldberg I, Westreich M. Multiple glomus tumor: A case report and review of the literature. Ann Plast Surg 1999;43:436-8.  Back to cited text no. 4  [PUBMED]  
5.Murphy RX, Rachman RA. Extradigital glomus tumor as a cause of knee pain. Plast Reconstr Surg 1993; 92:1371-474.  Back to cited text no. 5    
6.Pulitzer DR, Martin PC, Reed RJ. Epithelioid glomus tumor. Hum Pathol 1995;26:1022-7.  Back to cited text no. 6  [PUBMED]  
7.Slater DN, Cotton DW, Azzopardi JG. Oncocytic glomus tumor: A new variant. Histopathology 1987;11:523-31.  Back to cited text no. 7  [PUBMED]  
8.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. 8  [PUBMED]  [FULLTEXT]
9.Miettinen M, Paal E, Lasota J, Sobin LH. Gastrointestinal glomus tumors. A clinicopathologic, immunohistochemical and molecular genetic study of 32 cases. Am J Surg Pathol 2002;26:301-11.  Back to cited text no. 9    

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Correspondence Address:
K R Rathi
153 GH, C/O 56 APO.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.55012

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