Glomus tumors - البروفيسور فريح ابوحسان – استشاري جراحة...

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Digital and Extradigital Glomus Tumors At Jordan University Hospital A Retrospective Review (1989 - 1999) Shaher El-Hadidi, Freih Abu Hassan, Shukri Aghabi, Jamal Al-Masad,Faiez Daoud, .Mahmoud Abu-Khalaf, Husam Al-Muhtaseb, Musleh Tarawneh. Jordan Univeisrtl.Hosprtal &l\4edicil Schoot. ABSTRACT Ten patients diagnosed to have glomus tumors were located in various parts of the body, treated at Jordan University Hospital between 1989- lgg9. Five out of six digital tumours were diagnosed clinically and confirmed by histological examination after surgical excision. Extradigital tumours were diagnosed only after surgical excision and histological examination. Most of our cases though presented late and in all cases were subjected to complete surgical excision did produced a pennenant relief of symptoms Clinical diagnosis is suflicient in digital lesions but plain X-rays can be added to exclude bony erosions. KEYWORDS Glomus, Digital, Pain, Triad, Tumour. INTRODUCTION Glomus tumours and uncommon benign hamartoma, were first described clinically by wood in l8l 2,t and, histologically described by Masson in 1924 and Popoff in 1934.2 . \ Masson described the origin of the tumour as hyperplasia of the neuromyoarterial elements of the glomus body.2 Glomus body is an arteriovenous shunt found in dermal- subdermal junction which acts as a regulator of skin circulation,''' and is frequently encountered in subungual region, abscent in people below one year of age and atrophied in elderly.a Glomus tumour is present in up to 75% of the distal phalan*,:': .but is expected to d found in any place of the body3'5'll and muitiple lesions have been ors.tiued .5 It forms l-l}yo of all hand tumours,6,7,8 and are commonly found in adult females up to 88%.8 Although it is a few mm in size 4 ,',','and usually less than I cm in noh4 rarely more than 2cm,e it proiuces severe 4 symptoms, pain, tenderness and cold intolerance. Diagnosis usually done by the classic typical triad of symptoms in most digital iesiorrr. t'o't'e r ^ Clinically: Tenderness almost present in all cases, hypersensitivity to touch and cold in 630A, nail deformity in 47% and blue-red discoloration spot in 430 .8 We rarely needed to proceed for further investigations once the full picture encountered. Few authors suggested to use X-rays to exclude bony erosioD,6'8 ultrasound 2'e or MRI for localization and diagnosis.6'7 ' In extradigital areas may present as a painful nodule with long history of pain and tenderness in relation to the soft tissue.4'lo'l I Provocative tests have been suggested to increase the pain, Love's test in 1944 by pressing the lesion with pin head 4'8 using the or ethyl ChloriOe cold test.a Grossly, the glomus tumour forms a capsulated nodule of less than 5mm but a larger lesion have been reported. e Histologically it is formed by benign glomus cells which are round or oval and are specialized perivascular muscle cells wilh dense, granular cytoplasm near a rich neural bed. 2'4 Local infiltrative tumours and sarcomatous lesions are rare but have been reported. l'3' l I 't2't3 Complete surgical excision was found to be the most effective method to eradicate infections in patients symptoms.4'8'lo Correspondence should be addressed to: S.T.F.El-Hadidi, FRCS. (Ed.), Jordan University, P.O. Box 13347-Amman, Jordan. 54 JORDAN MEDICAL JOURNAL, VOL.35, NO.(l), MAy 2001

Transcript of Glomus tumors - البروفيسور فريح ابوحسان – استشاري جراحة...

Page 1: Glomus tumors - البروفيسور فريح ابوحسان – استشاري جراحة العظام في الاردن

Digital and Extradigital Glomus Tumors At Jordan University HospitalA Retrospective Review (1989 - 1999)

Shaher El-Hadidi, Freih Abu Hassan, Shukri Aghabi, Jamal Al-Masad,Faiez Daoud, .Mahmoud Abu-Khalaf,Husam Al-Muhtaseb, Musleh Tarawneh.

Jordan Univeisrtl.Hosprtal &l\4edicil Schoot.

ABSTRACT

Ten patients diagnosed to have glomus tumors werelocated in various parts of the body, treated at JordanUniversity Hospital between 1989- lgg9.

Five out of six digital tumours were diagnosed clinicallyand confirmed by histological examination after surgicalexcision.

Extradigital tumours were diagnosed only after surgicalexcision and histological examination.

Most of our cases though presented late and in all caseswere subjected to complete surgical excision didproduced a pennenant relief of symptoms

Clinical diagnosis is suflicient in digital lesions but plainX-rays can be added to exclude bony erosions.

KEYWORDS

Glomus, Digital, Pain, Triad, Tumour.

INTRODUCTION

Glomus tumours and uncommon benign hamartoma,were first described clinically by wood in l8l 2,t and,histologically described by Masson in 1924 and Popoffin 1934.2 .

\

Masson described the origin of the tumour as

hyperplasia of the neuromyoarterial elements of theglomus body.2

Glomus body is an arteriovenous shunt found in dermal-subdermal junction which acts as a regulator of skincirculation,''' and is frequently encountered insubungual region, abscent in people below one year ofage and atrophied in elderly.a

Glomus tumour is present in up to 75% of the distalphalan*,:': .but is expected to d found in any place ofthe body3'5'll and muitiple lesions have been ors.tiued .5

It forms l-l}yo of all hand tumours,6,7,8 and arecommonly found in adult females up to 88%.8

Although it is a few mm in size 4 ,',','and usually lessthan I cm in noh4 rarely more than 2cm,e it proiucessevere 4 symptoms, pain, tenderness and coldintolerance.

Diagnosis usually done by the classic typical triad ofsymptoms in most digital iesiorrr. t'o't'e r ^

Clinically: Tenderness almost present in all cases,hypersensitivity to touch and cold in 630A, naildeformity in 47% and blue-red discoloration spot in430 .8

We rarely needed to proceed for further investigationsonce the full picture encountered.

Few authors suggested to use X-rays to exclude bonyerosioD,6'8 ultrasound 2'e or MRI for localization anddiagnosis.6'7 '

In extradigital areas may present as a painful nodulewith long history of pain and tenderness in relation tothe soft tissue.4'lo'l I

Provocative tests have been suggested to increase thepain, Love's test in 1944 by pressing the lesion withpin head 4'8 using the or ethyl ChloriOe cold test.a

Grossly, the glomus tumour forms a capsulated noduleof less than 5mm but a larger lesion have beenreported. e

Histologically it is formed by benign glomus cellswhich are round or oval and are specializedperivascular muscle cells wilh dense, granularcytoplasm near a rich neural bed. 2'4

Local infiltrative tumours and sarcomatous lesions arerare but have been reported. l'3' l I 't2't3

Complete surgical excision was found to be the mosteffective method to eradicate infections in patientssymptoms.4'8'lo

Correspondence should be addressed to:S.T.F.El-Hadidi, FRCS. (Ed.),Jordan University, P.O. Box 13347-Amman, Jordan.

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DIGITAL AND EXT,\.ADIGITAL GLOMUS TUMORS AT JUH. SH. EL-HADIDI ET AL.

We did a. retrospective review of l0 cases of suchtumours over a l0 year period encountered in theGeneral Surgery and the Orthopaedic Surgerydepartments at the Jordan University Hospital.

PATIENTS AND METHODS

A total of 10 proven glomus tumours involving thedigital and .- extradigital soft tissues confirmedhistologically were retrospectively reviewed with amean follow-up of 5 years (6 months- 13 year). Therewere 6 males and four females with a mean age of 42.1years (range 20-60 years). Five tumours were located todistal phalanx and one located to the volar pad ofproximal phalanx. Table l. While the remaining extradigital tumours were located to the (forearffi, thigh,hypothenars and natal cleft) as shown in tabl e 2.

Clinically, all the digital tumours had pain as initialsymptoms or more than 2 years, four of them had typicaltriad of glomus tumour in the form of severepain/hypersensitivity to the touch and pressure withmarked tenderness and cold intolerance, especially inwinter. One patient has severe pain only and another had

a painful nodule. Five had a red-blue discoloration ofthe nail at the site of the tumour. Nail deformity inonly 3 cases.

While extradigital tuinours were presented as a painfulnodule for more than the duration of one year and only2 had the triad presentation of a glomus tumour.(Table 2).

Inspite of the typical clinical triad in 6 of our cases, allthese cases were missed initially by physicians. The 4digital cases with triad were clinically diagnosed byorthopaedic and hand surgeons, the other 2 werediagnosed after excision and histopathology.

The remaining 4 were typical presentation, onesuspected clinically and the other 3 diagnosed afterexcision and histopathology.

Pre operatively three cases assessed radiologically,two by plain x-ray and both showed bony erosion dueto pressure. ( Figure 3 ),One patient assessed by an MRI scan and showedbright lesions with the characteristics.

Of vascular origin in T2 image. Case No.: l, table I ,figure ( I ).

Table 1. Demographic characteristics and symptoms of the digital lesions in the study group.

JORDAN MEDICAL JOURNAL, VOL. 35, NO.(l), MAY 2001

Case Age Sex Symptomsduration

Site .Presenta

tionRed - blue

discolorationNail

deformitySize Location

I

53years

Male 3 years Thumb Triad Positive Positive 0.5 x 0.5 cm Subungual

2-54

yearsMale 3 years Big toe Pain Positive Negative 0.4 x 0.4 cm Subungual

.,J.

34years

Male 4 years Ring finger Triad Positive Positive 1.5 x 0.5 cm Subungual

4-52

yearsFemale 2 years Thumb Triad Positive Negative 0.7 x0.7 cm Subungual

5-20

yearsFemale 4 years Little

fingerTriad Positive Positive 1.5 x 0.5 cm Subungual

6-60

years

(yr) inall

Male 4 years

(yr)inall

Indexfinger

Painfulnodule

Negative Negative 2xlcm Volar pad

ofproximalphalanx

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DIGITAL AND EXTRADIGI.I'AL GLOMUS TUMORS AT JUH. SH. EL.HADIDI ET AL.

Table 2. Demoeraphic characteristic and symptoms of the extradigital tumours.

Case Age Sex Symptomduration

Site Presentation Size

I 3l yedrs Male I year Dorsum offorearm

Painful nodule +Triad

0.8 x 0.8 cm

2- 54 years Female I year Ant. thigh Painful nodule +Triad

1.5 x 1.5 cm

3- 20 years Male 5 years Hypothenar area Pain I x0.6cm

4- 43 years

(yr) in all

Female' 2 years

(yr) in all

Natal cleft Painful nodule lxlcm

RESULTS

All patients with subungual lesions had surgical excisionthrough transungual approach with partial nail removalis followed by nail bed reconstruction, the remainingtumours needed local excision.

All tumours were found to be localized and capsulated'without evidence of infiltrative or sarcomatous changes.

All patients were completely relieved of the syryptomsafter surgical intervention with no evidence of localrecuffence.

DISCUSSION

Glomus tumour is a benign lesion of the subungual area

in 59-7 5% of cases as reported by, take | & Graham et

al.5 however, in orlr study it is present in 84% of our 6

digital tumours which involves the subungual region, the

extra digital tumours can affect any paft of the body,3's'lt on. of our cases did affect the natal cleft, (case No.4 table 2) which have never been described in thislocation before.

Middle aged women are usually affected in 8804,8 only40% of our cases were females, the mean age of ourcases matches the literature.

Inspite of the classic triad of presentation more cases

were not diagnosed at the early presentation evenpatients had u long history of symptoms up to l0 years,s

but in our cases the mean symptofft duration, rangedbetween I -5 years.

It is a tradition that most of these cases are presented

to general physicians before they ask the advice of the

orthopaedic or the general surgeon. We have encoun-

tered the same problem in our cases, due to lack ofawareness of this uncommon tumour in general practicewhich adds to the long duration suffering of the patients.

Clinical diagnosis can be made in most digital lesions

without the need of any further investigation.Provocative tests has been suggested to enhance the

clinical picture, as Love's pin head pressure test a'8 and

the uppiiration of Ethyl chloride to the lesion,a butsometimes a plain X-rays is needed to exclude bonyerosion in long standing lesion which was found in 2 ofour cases.

Few surgeons and radiologisls- used the ultrasound, orMzu to localize these lesions2'6'7'e but usually it will notadd too much to the diagnosis, one of our patients had

MRI and it did showed a bright vascular lesion whichdid not add anything to the management.

MRI is an expensive investigation and rarely change the

surgical decision and only indicated when the clinicalpiciure is uncl ear.6''

Ultrasound has been reported valuable in locali-zation2'e but we did not use it in our cases, whilearteriography and bone scan is of doubtful value. 8

In digital lesions, we suggest to only to plain X-rays toexclude bony erosion and no other investigations are

needed for tiny tumours.

In extradigital tumours, a high degree of suspicion is

needed if the triad symptoms are present but at the end,histology after excision will be the rnost diagnostic.

Meticulous surgical excision is needed for this painf'ul

tumour to eradicate the patient agony, and all ourpatients symptoms disappeared after excision.

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t

DIGITAL AND EXTRADIGITAL GLOMUS TUMORS AT JUH. SH. EL-HADIDI ET AL

Transungual approach with reconstruction of the nail

bed is considered to be the safest8 to avoid local

reculTence, aS no reculTence was seen in our cases.

In extradigital lesions, usually the local excision issufficient to cure the patient's symptoms.

Figure l. MRI scan T2 image showing the glomus

at the base of distal phalanx Ap view.

Figure 2.Same Case lateral view T2 image.

Figure 3. Plain A.p X-ray of both thumbs shows erosion of the

taft on the left one.

Figure 4. Lateral view iri plain X-ray.The same case in figure 3.

CONCLUSION

Glomus tumours are of uncommon pathologY,

usually missed at initial presentation in spite of the

classic picture specially in digital lesions, we need to

raise awareness of physicians to this pathology

which produces a lot of suffering to the patient.

Surgical excision offers a pennanent relief ofsymptoms.

Contrary to other series, most of our patients were

males.

REFERENCES

Lumely J.S.P, Stansfeld A.G. InfiltratingGlomus Tumour of lower limb. BMJ1972;1,484-5.

Fornage B. Glomus Tumours in the finger:

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1 98 8;167: 1 83-5.

Gould E.W., Manivel J.C., Saaredra J.A.,

Monforle H., Locally infiltrative glomus' tumour and glomangiosarcoma. Cancer;

65:3 10- I 8.

1.

2.

3.

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