Glomus tumor is a rare benign vascular tumor that derives from structures called glomus bodies. These are neuromyoarterial devices responsible for the regulation of local blood flow and thermoregulation. It was first described by Wood in 1812, being named after Masson in 1924.
They are located in the dermis of any part of the integument but are more concentrated in the hands and feet. Up to 80% are located in the upper extremities, being especially frequent in the subungual region.
Definition of glomus tumors
Glomus tumors are solid tumors that mainly refer to two types of structures. They come from:
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Masson’s neurovascular glomus, located under the skin in the dermis, plays a role in the body’s thermal regulation. This is then a neuro-Myo-arterial proliferation.
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Paraganglia, structures belonging to the neuroendocrine system, resulting from embryonic neural crests, and located from the base of the skull to the cervical level and along the vascular axes of the neck (jugular vein, carotid bifurcation).
Composition of glomus tumors
The composition varies according to the type:
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Glomus tumors of the extremities contain glomus cells forming islets centered by vessels, smooth muscle cells, and blood vessels.
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Paraganglionic glomus tumors have a vascular component, but they have so-called neuro-endocrine properties and can thus secrete vasoactive substances, which have an action on the diameter of the blood vessels.
Causes of glomus tumor
The glomus tumors of the extremities are often isolated cases in a family (sporadic), they rather appear in adults with an average age of 40 years with a female predominance. There may be hereditary family forms (neurofibromatosis type 1). In this case, the tumors are rather multiple.
Good to know: Glomus tumors are rare tumors.
Glomus tumors of the paraganglioma type can be isolated but they are often familial (mutation of the SDH gene, multiple endocriennial neoplasms, Von Hippel Lindau disease, type 1 neurofibromatosis), and can be associated with other tumors such as pheochromocytomas or other tumors derived from neural crests.
Evolution of glomus tumors
The evolution of glomus tumors of the extremities is slow, characterized by an extension with progressive destruction of the adjacent tissues. These tumors are generally benign but present a risk of local recurrence after their removal.
Glomus tumors related to paragangliomas can invade adjacent structures and, due to their endocrine properties, generate serious complications such as pressure surges.
Note: in both cases, they are generally benign but there are rare malignant forms that generate metastases.
Locations of glomus tumors
The glomus tumors of the extremities develop under the skin:
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digitally, on the pulp of the finger or under the nail bed. It is the most frequent form;
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sometimes in the limbs (palm, wrist, forearm, foot);
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more rarely: soft tissues, nasal cavity, trachea, stomach.
Type Glomus tumors paraganglioma develop:
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at the level of the neck and the base of the skull: at the top of the internal jugular vein under the base of the skull, along the cervical nerve;
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at the level of the cervical ganglion of the vagus nerve, at the tympanic level in the middle ear (along the jacobsonian nerve);
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at the level of the carotid bifurcation (we speak of chemodectoma).
Symptoms of glomus tumor
Symptoms depend on the location of the glomus tumor:
- For the subungual form: very intense or even excruciating pain at the slightest contact or during exposure to cold, localized on the tumor area. The tumor is often hidden under the nail bed or in the pulp of the finger. Sometimes it is visible as a small purplish spot, very rarely it is palpable.
Diagnosis is often difficult as the tumor is often invisible on physical examination.
- Pulsatile tinnitus with hearing loss for tympanic forms.
- Neurological signs for jugular forms (damage to the cranial nerves and the posterior fossa) and cervical forms (paralysis of the vagus nerve and the great hypoglossal nerve), headaches.
- Vagal discomfort due to vagus nerve stimulation.
- Neck pain.
- Palpable mass in the neck for carotid forms.
Diagnosis of glomus tumors
The diagnosis is mentioned:
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on standard radiography (cortical notch in aggressive subungual forms that reach the bone);
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CT scan or MRI;
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angiography in the event of localization at the level of the neck or the base of the skull, in order to explore the carotid and vertebrobasilar axes;
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Octreoscan scintigraphy (injection of a radioactive marker through a vein) makes it possible to visualize locations in the body, with tumors specifically binding a marker called somatostatin.
Summary: It is confirmed by histology, an examination carried out on a biopsy or on a surgical part. Immunohistochemistry makes it possible to specify the diagnosis by looking for the presence of markers.
Urinary and blood assays of catecholamines can be useful in order to detect possible complications (pressure surges) in the event of paraganglioma.
Good to know: a genetic investigation may be necessary.
Treatment of glomus tumor
Treatment consists of surgical resection or embolization. Sometimes it is supplemented by radiation therapy to limit the risk of recurrence.
In case of digital localization:
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Surgical excision is sufficient with coagulation of the edges to prevent a recurrence.
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The pain subsides immediately after the procedure.
5 patients with epigastric glomus tumor
| No | Gender | Age | Symptom | Gastric location | Site | Size (cm) | Follow-up (months) | Operation methods |
|---|---|---|---|---|---|---|---|---|
| 1 | F | 59 | Epigastric discomfort | Antrum | Mucosa to muscularis | 3.5 | Segmental resection | |
| 2 | F | 51 | Epigastric discomfort | Antrum | Submucosa to muscularis | 1.5 | Segmental resection | |
| 3 | F | 44 | Epigastric discomfort | Antrum | Submucosa to muscularis | 2.5 | Segmental resection | |
| 4 | M | 62 | Epigastric pain | Antrum | Submucosa to muscularis | 1.5 | Segmental resection | |
| 5 | M | 40 | None | Antrum | Submucosa to muscularis | 2.3 | Segmental resection |
Epidemiology
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Glomus tumors are rare, constituting 1–5% of hand tumors 6. 25 to 75% of these are located in the subungual area, which is more common in women, with other locations more common in men.
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pathogenesis
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Anatomically, the glomus bodies from which these tumors originate consist of an afferent arteriole, anastomotic vessels (Suquet-Hoyer canals), a collecting vein, the intraglomerular reticulum (containing glomus cells, nerve fibers, and interstitial cells). and a capsule.
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Although the cause of glomus tumors is unknown, some authors have proposed that a weakness in this structure can lead to reactive hypertrophy secondary to trauma, or that they constitute hamartomas in which the components are hypertrophied.
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Glomus cells are specialized smooth muscle cells that derive from Zimmermann’s pericytes and are particularly dense around these dilated vascular spaces.
In addition, increased numbers of nerve fibers and mast cells can also be found. It is not uncommon for patients to associate the appearance of the lesion with a previous trauma in that location, but the causal relationship has not been proven.
Classification of glomus tumor
- Glomus tumors can be classified into two clinicopathological variants: solitary or multiple. The former is the most frequent, they usually appear in adulthood and are preferably located in acral areas, especially in the subungual bed, as we have commented.
The presence of multiple digital glomus tumors is rare and has been described several times in patients with type 1 neurofibromatosis.
- Multiple glomus tumors also called glomangiomas or glomovenous malformations due to their angiomatous appearance, constitute less than 10% of cases.
Unlike solitaries, they appear at a younger age and are usually inherited in an autosomal dominant pattern with variable expressiveness and incomplete penetrance. The gene has been located on chromosome 1p21-22 (glomalin gene).
Summary :These tumors have a slight predominance in males and appear as soft bluish nodules, dividing in turn, according to their distribution, into multiple disseminated glomangiomas, multiple localized glomangiomas, and congenital plaque glomangioma. Multiple glomus tumors are usually not painful, in contrast to solitary ones.
Here are 4 most frequently asked questions:
Frequently Asked Questions
1. What are glomus tumors?
Glomus tumors are benign (non-cancerous) tumors of vascular origin (arising from the cells that make up the blood or lymphatic vessels) and of unknown cause. Glomus tumors are rare (1-2%) and are more prevalent in women. They are usually located on the fingertips and under the nail, causing significant functional problems, due to the pain they generate.
2. What are the symptoms of glomus tumor?
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Paroxysmal pain and severe pain are the main symptoms
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Sensitivity to cold in the affected area
3. What do we find on physical examination?
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Bluish nodules, sometimes difficult to see in the subungual region
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Changes in the color or shape of the nail
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Pressure pain when touching objects
4. How are glomus tumors diagnosed?
Due to its infrequency, and its little knowledge outside the scope of hand surgery, the diagnosis of a glomus tumor often takes time. In order to carry out a diligent diagnosis, in addition to the symptoms described request X-rays (they can affect the bone) and MRI (to be able to see the soft tissue involvement).
However, the definitive diagnosis is made by means of the pathological study (study of the samples of the affected tissue) after its extirpation.
Conclusion:
Glomus tumors more frequently affect middle-aged patients, but they have been described in all ages. The clinical triad of intense paroxysmal pain, exquisite location of the pain point, and sensitivity to cold are characteristic.
The presence of these symptoms is quite suggestive of the diagnosis, but in many cases, it is delayed, so it is not uncommon for patients to consult for intense subungual pain after years of evolution.