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Journal of Case Reports
Glomus Tumor of Face

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Shahnawaz Muslim, Tushar Singh, Rajesh  Kumar, M. Arif
From the Departments of Surgery and Pathology, Katihar Medical College, Katihar-854105, Bihar, India.
Corresponding Author:
Dr. Tushar Singh
Email: tusharsingh020785@gmail.com
Received: 05-AUG-2014 Accepted: 13-OCT-2014 Published Online: 15-NOV-2014
DOI: http://dx.doi.org/10.17659/01.2014.0111
Abstract
Glomus tumor is a neoplastic proliferation of glomus body, which is a thermoregulatory neuromyoarterial unit seen predominantly in the subungual regions. These tumors are found mainly in the digits. Extra digital location is uncommon and involvement of face is rare. However, possibility of glomus tumor in a subcutaneous nodule of face should be considered particularly if it shows typical triad of intense paroxysmal pain, exquisite tenderness and sensitivity to cold. Though, the typical findings may be absent. Diagnosis depends upon high index of suspicion. Histopathological examination of the lesion confirms the diagnosis. Here, we present a case of 29 years old male who presented with a small painless subcutaneous nodule on the face.
Keywords : Glomus Tumor, Neoplasms, Face, Pain, Humans.
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Introduction

Glomus tumor arises from glomus body, a neuromyoarterial unit located in stratum reticularis of dermis [1]. It  accounts  for  1-2%  of  soft  tissue  tumor and is seen  predominantly  in  subungual  region  of  digits [2]. Extra digital location  for  the  tumor  has  been  described, but  face  is  considered  to  be rare  site. In  a  study, out  of  56  cases  of  extra digital  glomus  tumor  seen  at Mayo  clinic  over  a  period  of  20  years  (1985-2005), only  one  case  was reported  in  cheek [3]. Clinical  presentation  includes  triad  of  intense  paroxysmal  pain, exquisite  tenderness  and  sensitivity  to  cold [4].  These  features  may  or  may not  be  present  in  cases  of  glomus  tumor  of  face  and  patient  may  present with  asymptomatic  subcutaneous  nodule  or  chronic  facial  pain. Biopsy  is needed  for  diagnosis  and  is  therapeutic  as  well  in  many  cases.

Case Report

29  years  old  male  presented  with  a  painless  swelling  over  left  cheek  since  4  months. Examination  revealed  a non-tender, well defined, smooth, soft and bluish  swelling  of  about  2x2  cm  on  left  side  of face [Fig.1].  There was no clinically palpable node in the neck. Examination of oral cavity was within normal limits. Fine needle aspiration cytology (FNAC) of the lesion was obtained. It  was  reported  to  have  moderately cellular, scattered  and  dissociated  cluster  of cells  with  mild  anisonucleosis, bland  nuclear  chromatin  and  scanty cytoplasm  along  with  plasmacytoid  cells  showing  bluish  cytoplasm  and  cytoplasmic vacuoles. Lymphocytes and few macrophages were seen in the background. Features were considered to be suggestive of benign adnexal tumor.


Patient was scheduled for surgery. The  swelling  was  excised completely through  a skin-crease  incision  and  sent  for  histopathological  examination. On HPE  it  was  found  to  have  branching  vessels  lined  by  endothelial cells  and  surrounded  by  sheets  of  small  round  cells  with  round  bland nuclei  and  eosinophilic  cytoplasm [Fig.2]. There were also nests and aggregates of these cells in stroma. Occasional chronic inflammatory cells were present in stroma. Based on these findings diagnosis of glomus tumor was made.


Discussion

Glomus  tumor  is  a  neoplasm  of  glomus  body, first  described  by  Wood  in 1812. Masson in 1924 provided the first clinical description for this condition [4]. The  tumor  develops  from  glomus  body, which  is  a  specialized arterio-venous  fistula  situated  in  stratum  reticularis  of  dermis. The  arterial end  of  the  body  is  known  as  Sucquet-Hoyer canal  and is lined  by modified  smooth  muscle  cell  called  glomus  cell, derived  from  Zimmerman pericyte. Its  contraction  leads  to  heat  preservation  and  dilatation  to  heat loss  and  thus  plays  an  important  role  in  thermoregulation [1,5,6].

According  to  WHO  classification, glomus  tumor  can  be  divided  into  three subtypes  depending  upon  the  predominance  of  different  histological component. A  tumor  having  predominant  vascular  component  is  known  as glomangioma, while  that  with  predominant muscular component as glomangiomyoma and predominant cellular  component  as  solid. Other variants  include (i) glomangiomyomatosis - A  benign  and  diffuse  form  (ii) symplastic  glomus  tumor - Tumor with marked nuclear atypia, considered  a degenerative  phenomena  in  absence  of  other  malignant  feature  and  (iii) glomangiosarcoma -  the  malignant  variant, accounts  for  1%  of  all  glomus tumor [7]. Glomus  body  is  predominantly  located  in  palms, digits  and  soles  and  so the  glomus  tumors  are  usually  seen  in  limbs  particularly  in  subungual regions  of  digits [5]. Extra digital location of glomus tumor has been described. It includes face, colon, stomach, lung, bone, nervous system and fallopian tube [3].  Extra-digital  glomus  tumors  are  more  common  in  men  and  male  outnumber the  female  by  a  ratio  is  4:1 [3]. Usually  these  tumors  do  not  exceed  the size  of  1x1  cm, but  large  glomangiomas  mimicking  venous  malformation have  been  reported. MRI  is  considered  to  be  the  most  sensitive  investigation  for  glomus  tumor in  extremity.

Excisional biopsy usually settles the diagnosis. The  characteristic appearance  of  glomus  cells  and  nesting  pattern  referred  to  as  zellbalen  is usually  sufficient  to  establish  the  diagnosis  on  routine  microscopy. Though in  cases, where  these  features  are  not  evident, immunohistochemistry  may help  to  reach  the  conclusion. According  to  Rallis et al. IHC  profile  that  suggests  the  diagnosis  of  glomus  tumor  is  immunopositivity  with  smooth  muscle  actin, muscle  spindle actin  and  vimentin  and  negative  immune  reactivity  with  S-100  and  epithelial  marker [8].

Treatment is adequate  surgical  excision; though,  recurrence  can  be  seen  in  12-33%  of  cases. Recurrence  occurring  within days  to  weeks  indicates  inadequate  surgical  excision  while  2-3  years  after may  be  because  of  multiple  tumor [9].

In our case, patient presented with an asymptomatic subcutaneous nodule on face, which on excision and biopsy was proved to be glomus tumor. Since complete excision was already performed at initial surgery, no further intervention was required. Post-operative recovery was excellent and patient didn’t reveal any sign of recurrence in follow up period of six months.

Conclusion

Glomus tumor of face is a rare condition and only presentation may be an asymptomatic subcutaneous nodule. Complete surgical is usually curative & overall prognosis is good.

References
  1. Fletcher C. Tumours of blood  vessels  and  lymphatics.  In: Diagnostic  Histopathology  of  Tumors. Churchill  Livingstone, Edinburgh, UK; 2000: pp.75-76.
  2. Enzinger SW, Weiss FM. Soft  Tissue  Tumors. Mosby, St.Louis, Mo, USA, 3rd edition; 1995.  
  3. Schiefer TK, Parker WL, Anakwenze OA, Amadio PC, Inwards CY, Spinner RJ. Extradigital glomus  tumors: a  20-year experience.  Mayo Clinic Proceedings. 2006;81:1337-1344.
  4. Tomak  Y,  Akcay  I, Debak  N, Eroglu  L. Subungual  glomus tumors  of  the  hand: diagnosis  and  treatment  of  14  cases . Scand  J  Plastic Reconstruction  Surgery  Hand  Surgery. 2003;37:121.
  5. TB Fitzpatrick, RA Johnson, K Wolf, MK Polano D. Suurmond. Color  Atlas  and  Synopsis  of  Clinical  Dermatology, McGraw-Hill, 3rd  edition;1997.
  6. Venkatachalam MA, Greally JG. Fine  structure  of  glomus tumor: similarity  of  glomus  cells  to  smooth  muscle  Cancer. 1969;23(5):1176-1184.
  7. Fletcher  DC, Unni  KK, Mertens  F (eds). Glomus tumors, in World  Health  Organization  Classification  of  Tumors. Pathology  and  Genetics  of  Tumors  of  Soft  Tissue  and  Bone. Lyon, IARC  Press; 2002: pp. 137.
  8. Rallis G, Komis C, Mahera  H. Glomus  tumor: A  rare  location  in the  upper  lip.  Oral  Surg  Oral  Med  Oral  Pathol  Oral  Radiol  Endod. 2004;98:327.
  9. Saglam Y, Basak K, Köse HI, Kiliçkap Y, Karadayi N. Glomus Tumor of Nasal Cavity. Journal of Case Reports. 2014;4(2):375-378.
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Muslim S, Singh T, Kumar R, Arif M.Glomus Tumor of Face.JCR 2014;4:443-445
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Muslim S, Singh T, Kumar R, Arif M.Glomus Tumor of Face.JCR [serial online] 2014[cited 2024 Mar 19];4:443-445. Available from: http://www.casereports.in/articles/4/2/Glomus-Tumor-of-Face.html
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