Glomangiomas: Are They Rare Tumours Or Rarely Diagnosed?
A Mathew
Keywords
glomangioma, glomus tumour
Citation
A Mathew. Glomangiomas: Are They Rare Tumours Or Rarely Diagnosed?. The Internet Journal of Hand Surgery. 2012 Volume 3 Number 2.
Abstract
Glomangiomas are rare tumours occurring mainly in the subungual region of the finger. The duration from time of onset of symptoms to surgery often extends to years. The condition is excruciatingly painful in this location and the patient would have consulted numerous physicians and surgeons prior to surgery. Evaluation of 7 patients with glomus tumours revealed an average duration of symptoms was 4.28years (2 to 8 yrs). An average of 3.2 physicians and 1.6 surgeons had seen these patients. 2 patients were evaluated for cardiac disease due to radiation of pain from the finger tip to the chest. 2 patients were evaluated for cervical spine disease. The lack of obvious clinical signs and the absence of definitive findings in basic imaging studies make diagnosis difficult. A greater awareness among physicians, neurologists and cardiologists; and inclusion of the same in undergraduate-medicine teaching curriculum will go a long way in reducing the average time to diagnosis in this acutely painful condition.
Introduction
Glomangiomas also called glomus tumours, are hamartomas occurring in the stratum reticularis of the dermis. In the hand they account for one to five percent of all tumours[1]. The commonest location is the subungual area of the finger. This location and the rich network of sensory nerves within the tumour makes it excruciatingly painful. The classic triad of symptoms include paroxysmal pain, cold hypersensitivity, and pinpoint tenderness[2]. A bluish-red nodule or discolouration of the nail bed may be visualized under the nail plate. Some patients may present with nail plate abnormalities like ridging, deformity, splitting and some cases partial or total absence of the same. Often however no obvious abnormality is seen on inspection over the distal end of the digit especially if the tumour is located proximal to the nail fold, under the germinal matrix of the nail-bed or in the pulp of the digit. Careful palpation with a blunt tipped probe (pin-head or paper clip) will elicit pinpoint tenderness[3] in most cases (The Love’s test). Cold hypersensitivity can be elicited in almost all cases of the patients when the finger is dipped in cold water. High resolution MRI will show a lesion with high signal intensity in T2-weighted spin-echo sequences with further enhancement with gadolinium [3]. Though a sensitive diagnostic modality, it is not specific. Radiographs may show scalloping of the involved phalanx under the nail bed[4].
One of the characteristics of this tumour is the long duration of symptoms[3] at presentation. This may extend for years. Most patients would have visited numerous physicians and surgeons only to be treated symptomatically. Some of the patients present with an atypical radicular pain, radiating to the shoulder or chest from the digit following accidental contact of the involved area. Few of the patients are even evaluated for cardiac or neurologic disease prior to presentation to a hand surgeon.
Materials And Methods
All patients who had presented with histology proven, glomangiomas over a four year period at the hospital were included in a cohort study. At admission all patients were questioned in detail regarding duration and type of symptoms, the number and type of practitioners visited in the past and any special tests undergone for the same. The patients were also asked about other medical illnesses, and regarding any long term medications prescribed for the same condition. All patients were requested to follow up after 6 months and one year after suture removal.
In the clinical evaluation of all patients the classic triad of symptoms including, paroxysmal pain, pin-point tenderness and cold hypersensitivity were elicited at admission. Details of any atypical symptoms were also noted. Post operatively patients were specifically evaluated for any complications like recurrence, nail plate deformities, infection.
All procedures were done after standard written consent and the patients were warned about a ten to twenty percent of chance of recurrence prior to surgery. All patients received one dose of prophylactic antibiotic prior to surgery. The surgeries were done under digital block and tourniquet the next day after presentation Surgery was done through a trans-ungual approach in all cases of subungual tumours and direct incision over the pulp for volar tumours (figures1,2).
In sub-ungual tumours the nail plate was partially or totally removed and the dorsal nail fold reflected to reveal the bluish-red discolouration of the tumour under the nail bed(Figure:3).
Figure 3
The nail bed was then carefully incised and reflected to reveal the tumour. The tumour was carefully dissected to the pedicle containing the nerve and afferent vessel. The lesion was removed by cauterizing the pedicle and separating it from the bed in all cases (figure: 4).
Figure 4
All patients requiring the trans –ungual approach had their nail beds repaired with 5-0 vicryl. The nail-plate was re-inserted into the nail fold after washing and scrapping of any remnant soft-tissue on its deep surface or a nail spacer was used to prevent adhesions between the dorsal and ventral nail folds. The spacer was removed after one week and any nail fold or skin sutures if present were removed on the tenth post operative day. All tissue biopsy samples were sent for histo-pathology examination. The biopsy reports were available within three days of the surgery.
Results
7 patients with glomangiomas in the finger tip presented to the hand unit of the hospital over the last four years. 5 patients were females, average age 43.4 yrs (37 to 50yrs); and two males (35, 59 yrs). The average duration of symptoms were 4.28yrs (2 to 8 years). All patients had sought medical advice prior to presentation having met a median of 5 practitioners not including the numerous visits to each one prior to definitive surgery. Details of visits to specialists and practitioners are summarised in
Figure 5
Symptom profiles of all patients are summarized in
Figure 6
Two tumours were located in the pulp while the rest were sub-ungual in location. One patient presented with a nail plate ridging (figure: 4), while another had a split nail (figure: 5). All other subungual tumours had no nail-plate abnormality at presentation. All tumours were confirmed glomangiomas after histo-pathological examination. One of them was of the mucoid variety. All others were of the solid type[1]. No angiomatous type of glomangiomas was seen in this series.
Figure 7
All patients had complete relief of symptoms post operatively. One patient developed minor superficial infection under the sutured nail fold which was treated with suture removal and a short course of antibiotics. One patients had persistent ridging of the nail 4 months after surgery. No patient required any long term pain or pain modifying medication after the surgery.
Discussion
The dramatic relief offered by surgery in this condition is heart warming. The unfortunate duration of symptoms till definitive diagnosis, is the most anguishing aspect for most patients. In this series only five out of forty practitioners or specialists were able to diagnose this condition and refer the patients for surgical management. Each patient had made an average of 10.5 visits to medical centres before definitive surgical treatment. Although the pain is severe diagnosis is not readily made[5,6].In the authors’ practice over four years at a hand centre the last two cases presented over the last one year after a presentation of the problem was made at local doctor’s association. Both the cases were correctly diagnosed by the next physician or specialist who saw those patients and referred them to the hand centre for further treatment. Most patients had the classic triad of episodic pain, cold hypersensitivity and pin point tenderness. These seven cases accounted for eleven percent of all hand tumours done at this hand centre, although other studies at large volume hand centres put the same as 1 to 5%[1,6]. The inclusion of this tumour in the under-graduate teaching curriculum and awareness of this problem among physicians, cardiologists and neurologists alike will go a long way in shortening the time to diagnosis and offering an early cure for this excruciatingly painful and rarely diagnosed condition.