Glomangiomas, or glomuvenous malformations (GVM), are rare cutaneous venous malformations that show glomus cells (undifferentiated smooth muscle cells, which are thermoregulatory units), along with venous system in histology. Glomus cells specialized smooth muscle cells that regulate the temperature in the body. Masson first described glomangiomas and Papoff further extensively studied. There are three types of glomus tumors, classified based on its dominant component:
1. Solid: mainly glomus cells.
2. Glomangioma: mainly blood vessels.
Glomangiomas usually present in multiples, often at birth or during childhood, and they do not involve the subungual region. A majority of glomangiomas are benign, although malignant cases have also been reported. Rarely seen, the disseminated type distributes throughout the body.
1. Inherited or familial (38% to 68% of glomangiomas):
Generally, it is autosome dominant with incomplete penetrance and variable expression, located in a 4--6--cm region of chromosome 1p21-22. Glomulin is the mutated gene which is located on the YAC and PAC maps. This gene includes 14 mutations in patients with this medical condition, which results in a loss of function. This loss of function increases cyclin E and c-Myc levels. In 60% of cases, one other family member is also affected. It can present at birth or later during adolescence. 157_161del mutation is another documented mutation that may have a role in GVM malformations.
Segmental type 2 is a variant of inherited glomangiomas. Initially they present with one primary lesion, followed by multiple distal lesions.
2. Sporadic or de novo mutation:
It presents during birth.
Glomangiomas are responsible for 1.6% to 2.0% of soft skin tumors and 20% of all glomus tumors. Plaque-like glomangiomas are very rare, with only 4 cases reported thus far. It is more predominant in the male gender. About one-third of the patients present before age 20. 10% of cases are of the disseminated type.
The most common reason for referral among vascular anomalies is venous malformations.
It typically presents as purple skin lesions with a cobblestone pattern at birth. Lesions are usually bluish-purple, papular or nodular, hyperkeratotic, and 2 to 10 mm. The size and number of them are variable. These lesions are tender on palpation. Pressure and cold trigger the pain. Areas rich with glomus bodies include the involved sites such as distal extremities, especially palms, wrists, forearms, feet, and subungual region. 75% of cases present in hands. Visceral organ involvement is very rare, although it has been reported in the nasal cavity, mediastinum, gastrointestinal tract, respiratory tract, urogenital tract, and hepatobiliary system. Ventricular septal defects and transposition of the great vessels were reported in patients with GVM. There is a case report of involvement of nerves with glomangioma, although normal human nerve is without glomus bodies. Tracheal involvement is associated with dyspnea, hemoptysis, and retrosternal chest pain.
Glomangiomas are larger and less well-circumscribed than solitary tumors. They have slow blood flow and usually grow over time.
The classic triad consists of the following : (a) hypersensitivity, (b) Intermittent pain, and (c) pinpoint pain. Most of the time, glomangiomas do not present the classic triad.
The plaque-like type presents as indurated, nodular, or discolored lesions, which are non-tender and bleed with minor trauma. They are larger than the other types of glomangiomas. This is the rarest type of glomangiomas.
The confirmation of the diagnosis is through histopathology. If a tumor has atypical histology, immunohistochemistry assists in diagnosis. The role of smoothelin should be considered as it is an indicator of the smooth muscle cell.
Electron microscopy shows glomus cells with dense bodies and smooth muscle myofibrils .
X-ray may show osseous defects.
The goal of treatment is to decrease the symptoms. It is difficult to treat the lesion when it reaches a specific size completely. For asymptomatic lesions, monitoring and observation are recommended. Surgery, electron-beam radiation, sclerotherapy with hypertonic saline or sodium tetradecyl sulfate, argon, flash lamp tunable dye laser (for multiple lesions), and CO2 lasers are different treatment modalities. Excision therapy is the preferred treatment with painful lesions. Sclerotherapy was shown to be more effective in venous malformation than glomuvenous malformations. In cases of nasal involvement, endoscopic excision or surgery is recommended. In cases of large glomangiomas that are difficult to excise, 1064-nm Nd:YAG laser is effective. Also, positive results with Nd:YAG laser has been reported in symptomatic familial cases.
External compression by elastic compressive garments worsens the pain.
The chance of malignancy is very low. Risk factors for malignancy are the following: size greater than 2 cm, deep lesions, muscle, and bone invasion, and high mitotic activity. Cases of metastasis were reported but are exceedingly rare.
These lesions can be life-threatening due to the risk of growth, bleeding, or vital organ obstruction.
One case reported Spitz nevi growing upon a congenital glomuvenous malformation. There is a theory that hyperemia in glomangioma can nourish the hair follicles. Mutated glomulin may also have a role in this case.
It is recommended that patients return to their physician in case of further growth, bleeding, or recurrence.
Sometimes it is challenging to diagnose GVM as there are different similar medical conditions, as discussed above.
Glomangiomas may exhibit signs and symptoms like papule or nodule.
It is important to consult with specialists, including dermatologists or surgery for the management of this medical condition. Specialty care nurses coordinate care and provide patient education. Radiologists may be involved during the assessment. Care can be enhanced by the interprofessional team. [Level 5]
|||Brouillard P,Boon LM,Mulliken JB,Enjolras O,Ghassibé M,Warman ML,Tan OT,Olsen BR,Vikkula M, Mutations in a novel factor, glomulin, are responsible for glomuvenous malformations ( [PubMed PMID: 11845407]|
|||Abbas A,Braswell M,Bernieh A,Brodell RT, Glomuvenous malformations in a young man. Dermatology online journal. 2018 Oct 15; [PubMed PMID: 30677819]|
|||Arens C,Dreyer T,Eistert B,Glanz H, Glomangioma of the nasal cavity. Case report and literature review. ORL; journal for oto-rhino-laryngology and its related specialties. 1997 May-Jun; [PubMed PMID: 9186975]|
|||Chatterjee JS,Youssef AH,Brown RM,Nishikawa H, Congenital nodular multiple glomangioma: a case report. Journal of clinical pathology. 2005 Jan; [PubMed PMID: 15623496]|
|||Munoz C,Bobadilla F,Fuenzalida H,Goldner R,Sina B, Congenital glomangioma of the breast: type 2 segmental manifestation. International journal of dermatology. 2011 Mar; [PubMed PMID: 21342169]|
|||Tewattanarat N,Srinakarin J,Wongwiwatchai J,Areemit S,Komvilaisak P,Ungarreevittaya P,Intarawichian P, Imaging of a glomus tumor of the liver in a child. Radiology case reports. 2020 Apr; [PubMed PMID: 31988680]|
|||Leger M,Patel U,Mandal R,Walters R,Cook K,Haimovic A,Franks AG Jr, Glomangioma. Dermatology online journal. 2010 Nov 15; [PubMed PMID: 21163162]|
|||Souza NGA,Nai GA,Wedy GF,Abreu MAMM, Congenital plaque-like glomangioma: report of two cases. Anais brasileiros de dermatologia. 2017; [PubMed PMID: 29267443]|
|||Cabral CR,Oliveira Filho Jd,Matsumoto JL,Cignachi S,Tebet AC,Nasser Kda R, Type 2 segmental glomangioma--Case report. Anais brasileiros de dermatologia. 2015 May-Jun; [PubMed PMID: 26312686]|
|||Jha A,Khunger N,Malarvizhi K,Ramesh V,Singh A, Familial Disseminated Cutaneous Glomuvenous Malformation: Treatment with Polidocanol Sclerotherapy. Journal of cutaneous and aesthetic surgery. 2016 Oct-Dec; [PubMed PMID: 28163461]|
|||Suárez-Magdalena O,Monteagudo B,Figueroa O,Gómez-Pérez MI, Glomulin gene c.157_161del mutation in a family with multiple glomuvenous malformations. International journal of dermatology. 2019 Feb; [PubMed PMID: 30460983]|
|||Boon LM,Mulliken JB,Enjolras O,Vikkula M, Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities. Archives of dermatology. 2004 Aug; [PubMed PMID: 15313813]|
|||Gonçalves R,Lopes A,Júlio C,Durão C,de Mello RA, Knee glomangioma: a rare location for a glomus tumor. Rare tumors. 2014 Oct 27; [PubMed PMID: 25568752]|
|||Tony G,Hauxwell S,Nair N,Harrison DA,Richards PJ, Large plaque-like glomangioma in a patient with multiple glomus tumours: review of imaging and histology. Clinical and experimental dermatology. 2013 Oct; [PubMed PMID: 24073652]|
|||Boon LM,Brouillard P,Irrthum A,Karttunen L,Warman ML,Rudolph R,Mulliken JB,Olsen BR,Vikkula M, A gene for inherited cutaneous venous anomalies ( [PubMed PMID: 10364524]|
|||Cullen RD,Hanna EY, Intranasal glomangioma. American journal of otolaryngology. 2000 Nov-Dec; [PubMed PMID: 11115526]|
|||Goujon E,Cordoro KM,Barat M,Rousseau T,Brouillard P,Vikkula M,Frieden IJ,Vabres P, Congenital plaque-type glomuvenous malformations associated with fetal pleural effusion and ascites. Pediatric dermatology. 2011 Sep-Oct; [PubMed PMID: 21133993]|
|||Scheithauer BW,Rodriguez FJ,Spinner RJ,Dyck PJ,Salem A,Edelman FL,Amrami KK,Fu YS, Glomus tumor and glomangioma of the nerve. Report of two cases. Journal of neurosurgery. 2008 Feb; [PubMed PMID: 18240933]|
|||Parker KL,Zervos MD,Donington JS,Shukla PS,Bizekis CS, Tracheal glomangioma in a patient with asthma and chest pain. Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 2010 Jan 10; [PubMed PMID: 19858390]|
|||Larsen DK,Madsen PV, [Glomus tumour of the distal phalanx]. Ugeskrift for laeger. 2018 Jul 23; [PubMed PMID: 30037386]|
|||Aneiros-Fernandez J,Retamero JA,Husein-ElAhmed H,Carriel V,Ruiz Villaverde R,O'Valle F,Aneiros-Cachaza J, Smoothelin and WT-1 expression in glomus tumors and glomuvenous malformations. Histology and histopathology. 2017 Feb; [PubMed PMID: 27184662]|
|||Flors L,Norton PT,Hagspiel KD, Glomuvenous malformation: magnetic resonance imaging findings. Pediatric radiology. 2015 Feb; [PubMed PMID: 24996811]|
|||Chirila M,Rogojan L, Glomangioma of the nasal septum: a case report and review. Ear, nose, [PubMed PMID: 23599117]|
|||Sharma JK,Miller R, Treatment of multiple glomangioma with tuneable dye laser. Journal of cutaneous medicine and surgery. 1999 Jan; [PubMed PMID: 10082598]|
|||Rivers JK,Rivers CA,Li MK,Martinka M, Laser Therapy for an Acquired Glomuvenous Malformation (Glomus Tumour): A Nonsurgical Approach. Journal of cutaneous medicine and surgery. 2016 Jan; [PubMed PMID: 26177926]|
|||Phillips CB,Guerrero C,Theos A, Nd:YAG laser offers promising treatment option for familial glomuvenous malformation. Dermatology online journal. 2015 Apr 16; [PubMed PMID: 25933083]|
|||Jha A,Ramesh V,Singh A, Disseminated cutaneous glomuvenous malformation. Indian journal of dermatology, venereology and leprology. 2014 Nov-Dec; [PubMed PMID: 25382523]|
|||Babeau F,Knafo S,Rigau V,Lonjon N, Paravertebral glomangioma mimicking a schwannoma. Neuro-Chirurgie. 2013 Aug-Oct; [PubMed PMID: 24367799]|
|||Jiga LP,Rata A,Ignatiadis I,Geishauser M,Ionac M, Atypical venous glomangioma causing chronic compression of the radial sensory nerve in the forearm. A case report and review of the literature. Microsurgery. 2012 Mar; [PubMed PMID: 22407591]|
|||Arica DA,Arica IE,Yayli S,Cobanoglu U,Akay BN,Anadolu R,Bahadir S, Spitz nevus arising upon a congenital glomuvenous malformation. Pediatric dermatology. 2013 May-Jun; [PubMed PMID: 22304367]|