|Year : 2022 | Volume
| Issue : 1 | Page : 39-43
Glomus tumor: A case series study of 30 cases and review of the literature
CS Nikhil, Joe Davis, K Muraleedharan, Suresh S Pillai
Department of Orthopedics, Baby Memorial Hospital, Kozhikode, Kerala, India
|Date of Submission||23-Dec-2021|
|Date of Acceptance||27-Dec-2021|
|Date of Web Publication||26-Jun-2022|
C S Nikhil
Department of Orthopedics, Baby Memorial Hospital, Kozhikode - 673 004, Kerala
Source of Support: None, Conflict of Interest: None
Glomus tumors are rare usually benign neoplasms arising from the glomus body, accounting for 1%–2% of soft tissue tumors of the hand. They are commonly found in the hand and present as bluish or red-pink discoloration of the nail bed with the classic triad of symptoms and clinical signs. Although classical signs and symptoms have been described, there is a delay in diagnosis of the tumor mainly due to the lack of awareness and low prevalence of the tumor. Magnetic resonance imaging is the investigation of choice in the diagnosis of the glomus tumor and the treatment is complete excision which prevents recurrence.
Keywords: Early diagnosis, glomus tumor, hand, recurrence
|How to cite this article:|
Nikhil C S, Davis J, Muraleedharan K, Pillai SS. Glomus tumor: A case series study of 30 cases and review of the literature. J Orthop Assoc South Indian States 2022;19:39-43
|How to cite this URL:|
Nikhil C S, Davis J, Muraleedharan K, Pillai SS. Glomus tumor: A case series study of 30 cases and review of the literature. J Orthop Assoc South Indian States [serial online] 2022 [cited 2022 Aug 16];19:39-43. Available from: https://www.joasis.org/text.asp?2022/19/1/39/348316
| Introduction|| |
Glomus tumors are benign vascular neoplasms accounting for 1%–2% of soft tissue tumors. They arise from contractile neuromyoarterial structure found in the reticular dermis layer, known as the glomus body. The glomus body consists of the afferent arteriole, anastomotic vessel, primary collecting vein, intraglomerular reticulum, and capsular portion. Hyperplasia of any of these parts can result in tumor formation. The glomus body is involved in thermoregulation by regulating blood flow in the cutaneous vasculature, and is highly concentrated in the fingertips, particularly the nail bed. Hence, the most common site of its occurrence is the distal phalanx of the fingers, especially in the subungual region., Unusual location or extradigital sites such as the foot, forearm, lung, trachea, and mediastinum head are more frequently seen in the male population.,
The patients often present with the classic triad of symptoms-severe pain, localized tenderness, and cold sensitivity. The mean duration of diagnosis is often delayed, ranging from 2 to 9 years, due to small mass at presentation, low prevalence, and lack of awareness among primary care physicians. Magnetic resonance imaging (MRI) is the investigation of choice in detecting the tumor and also in delineating the size and location. Recommended treatment is complete surgical excision of the tumor to reduce the chance of recurrence.
| Methods|| |
Details of 30 patients who were diagnosed as having glomus tumor at our institution from January 1, 2010, to December 31, 2020, were retrieved from the electronic database. A retrospective chart review was performed to identify whose tumors were histologically confirmed glomus tumors. Clinical information collected includes age at presentation, gender, presenting symptoms, location of tumor, imaging features, and treatment, whether it metastasized or recurred. These cases have not been previously published in the literature.
| Results|| |
Thirty patients with histologically confirmed glomus tumors were analyzed. The patient's average age was 39.4 (range 21–62) years at the time of the first presentation. Nineteen patients were females (63.3%) and 11 were males (36.6%). The overall mean tumor size was 1.3 cm (0.8–2.5 cm [Figure 1]), with extradigital tumors (mean 1.68 cm; range 1–2.5 cm) being larger than digital tumors (mean 1.2 cm; range 0.8–2.3 cm). None of the patients had multiple tumors.
Clinical Presentation and Tumor Location
Eighty-six percent of the tumors (26 cases) were in the fingers, of which 22 were in the subungual region and 4 in the volar aspect of distal phalanx [Figure 2]. Of the 14% of extradigital tumors (four cases) two cases were in the foot, one in the chest wall, and one on the medial aspect of the knee.
Ninety-three percent (28 patients) of patients had pain and tenderness as the main symptoms, and 26 of these belonged to digital groups (100%) while one patient presented with cold hypersensitivity as the main symptom. Ten of these 26 patients (digital group) had nail deformities, including deformation or discoloration. The one asymptomatic patient had tumor on the medial aspect of the knee (extradigital).
The diagnosis is mainly clinical, with the classic triad of symptoms and three main tests, Love's pin test, Hildreth test, and cold sensitivity together can offer high sensitivity and specificity. However, we do imaging as confirmatory before we proceed with surgical excision.
For digital tumors, radiographic imaging should be obtained comparing the phalanx of opposite extremity also. Ultrasound and MRI were also done for all patients.
Pathological examination of all 30 patients revealed glomus tumor proper. No patients had malignant glomus tumor.
| Management and Outcomes|| |
All tumors in this series were removed by direct excision. Transungual excision was done in 18 patients (81% of digital tumors), nail preserving lateral subperiosteal approach in five patients, transungual in two patients, and Keyser–Littler approach in one patient. Of the total, only three patients presented with nail deformity after surgery (all three were done via transungual approach) [Figure 3].
Two cases had recurrence (both digital tumors), one was due to incomplete excision and it was reexplored. The other patient was lost to follow-up.
| Discussion|| |
Glomus tumor, also known as Barré–Mason syndrome or tumor of Popoff is a rare benign vascular neoplasm first described by Wood in 1812 and later explained in detail including the histological characteristics by Barré and Mason in 1924. Glomus tumor arise from the arterial portion of the glomus body or the Sucquet–Hoyer canal of the apparatus. They are classified as solitary or multiple, the majority being solitary tumors, more frequently seen in the fingertips. The tumors are encapsulated and contain numerous small vascular lamina. The World Health Organization has classified glomus tumor into three types:
- Solid type: most common variant with scarce vasculature and minimal muscle component
- Angiomatous type: ganglioma and predominantly vascular
- Glomangiomyoma: predominantly vascular and smooth muscle components.
The etiology of glomus tumors is relatively unknown and could be related to age, sex, trauma, or inheritance. Some researchers have proposed that a weakness in the structure of the glomus apparatus body could lead to reactive hypertrophy after trauma. Recent studies have linked a familial variant of glomus tumor to chromosome 1p21-22 and involved truncating mutations in the glomulin gene, which encode a 68-kDa protein.
The glomus tumor usually presents as a small, slightly raised bluish or red painful nodule [Figure 4] and when subungual in location can elevate, deform, and discolor the nail. The clinical features include the classical triad of localized tenderness, severe pain, and cold sensitivity.,
|Figure 4: Tumor presenting as reddish-pink discoloration beneath the nail bed|
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Pain occurs due to increase in intracapsular pressure due to the contraction of myofilaments of glomus cells, and this is carried by the unmyelinated nerve fibers.
Three other clinical tests have been described in the literature which helps to diagnose glomus tumor. They include (i) Love's test: pressure is applied to the suspected area with a pinhead, which would produce excruciating pain for the patient [Figure 5]. This test had 100% sensitivity in our study. (ii) Hildreth's test: a tourniquet is applied along the arm and inflated to induce transient ischemia. Eliciting Love's pin test would be painless at this point of time indicating a positive test. The test is also considered positive when releasing the tourniquet cuff causes a sudden onset of pain and tenderness in the area of tumor (91%–100% specificity). This can be attributed to temporary restricted blood supply as it is a vascular tumor. (iii) Cold sensitivity test: ice cube or cold water applied to the affected area will cause severe pain to the patient. The mechanism for this may depend on the vasodilation of the Sucquet–Hoyer arteriovenous channels, which dilates in response to cold to prevent excessive heat loss (100% sensitivity and specificity).
The differential diagnosis of other painful tumors such as neuroma, leiomyoma, hemangioma, and conditions such as gouty arthritis should be kept in mind while evaluating solitary glomus tumors. The neuroma gives comparable pain and there would be a trigger zone but will be without hypersensitivity to cold and without vascular signs on MRI. Melanoma would be painless and be visible under the skin. Multiple glomus tumors, which can be painless, should be differentiated carefully from cavernous hemangioma and blue rubber nevus syndrome.
Plain radiographs may be normal in the majority of cases. However, long-standing cases may show scalloping of the distal phalanx [Figure 6]. Cortical thinning or erosive changes in the adjacent bone of the tumor have also been reported. If the location of the suspected tumor is in the distal phalanx, radiograph should be obtained comparing the phalanx of contralateral side also, that is, both phalanges side by side in a single film [Figure 7]. This could help in earlier detection of the tumor.
|Figure 7: X-ray showing phalanges of both hands taken simultaneously to see the subtle differences|
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Ultrasonography helps in confirming the site, size, and shape of tumor, but may be influenced by the radiologist's experience. Color duplex sonography helps in differentiating the tumor from other hypoechoic subungual tumors.
MR imaging is the most sensitive imaging modality available and it helps with location, sizing, and diagnosis of glomus tumor. The tumor typically appears as high signal central dot surrounded by a zone of lower signal intensity. T1-weighted images show low signal intensity and T2-weighted images show high signal intensity [Figure 8]a and [Figure 8]b. After gadolinium injection, T1-weighted images show enhancement.
|Figure 8: (a) Magnetic resonance sagittal image showing hyperintense lesion in T2-weighted images. (b) Axial section of magnetic resonance imaging|
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Histopathology confirms the diagnosis of the tumor. Macroscopically, they appear as a well-encapsulated gray–pink mass. Microscopically, they are composed of glomus cells, smooth muscle cells, and blood vessels. The glomus cells are small, uniform cells with round monomorphic nuclei and eosinophilic cytoplasm. The WHO classification into three types is based on the histopathological characteristics. It is an exceedingly rare phenomenon for the tumor to turn malignant. Large tumors (more than 2 cm in size) and deeply located tumors are considered to be malignant, but studies have shown that most of these cases behave in a clinically benign fashion. Histopathologically malignant glomus tumors have been described as those with marked nuclear atypia and elevated mitotic rates <5 mitoses per 50 high-power fields, or (ii) display atypical mitotic Figures.
Even though the tumor is said to be benign, the treatment of choice is wide local excision. Complete surgical excision is curative for the lesion. Various approaches have been described in the literature including direct transungual excision [Figure 9] which is the more routinely used one, Keyser–Littler approach which is through a high midlateral incision below the lateral nail fold, subungual approach, modified periungual approach which is through an L-shaped incision over the periungual area and the more recently described nail preserving modified lateral subperiosteal approach which uses a lateral incision which is curved distally along the pulp of the finger. The subungual region is accessed directly without any nail bed dissection.
Even after surgical excision, a recurrence rate of 4%–50% has been reported. Incomplete excision or the presence of a second tumor not previously diagnosed and excised is said to be cause for “early recurrence”(presenting within 1 year). Development of new lesion at or near the excision site attributes to “late recurrence” (presenting after 1 year). Measures recommended to reduce recurrence include (i) microscopic monitoring or intraoperative ultrasound which ensures adequate excision, (ii) double tourniquet exsanguination (one at midarm level and other at the base of the digit) helps in better intraoperative visualization. If the symptoms of glomus tumor persist for more than 3 months, repeat imaging and reexploration should be done.
| Conclusion|| |
Glomus tumors are rare, painful benign neoplasms with classical clinical presentations. However, they are often associated with delayed diagnosis due to variation in presenting symptoms and sometimes obscure symptoms combined with a lack of awareness among primary care clinicians. A history of misdiagnosis and/or surgical procedures combined with the classical triad of symptoms should prompt an MRI. Complete surgical excision is mandatory to get complete relief of symptoms and to prevent recurrence.
Department of Pathology, Baby Memorial Hospital.
Department of Radiodiagnosis, Baby Memorial Hospital.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]