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Glomus Cancer
Introduction
Glomangiomas, or glomuvenous malformations (GVM), are rare cutaneous venous malformations that show glomus cells (undifferentiated smooth muscle cells, which are thermoregulatory units), along with the venous system in histology.[1] Glomus cells are specialized smooth muscle cells that regulate the temperature in the body.[2] Masson first described glomangiomas, and Papoff further extensively studied them.[3] There are 3 types of glomus tumors, classified based on their dominant component:
- Solid: mainly glomus cells
- Glomangioma: mainly blood vessels.
- Glomangiomyoma: mainly smooth muscle cells [4]
- Glomangiomas are further divided into regional, disseminated, and congenital plaque-like.[5]
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.[6][7] Rarely seen, the disseminated type distributes throughout the body.
Etiology
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Etiology
Glomus tumors, particularly glomangiomas, can arise through inherited or sporadic pathways. Understanding the underlying genetic mechanisms is essential for accurate diagnosis, counseling, and management.
Inherited or Familial Glomangiomas (38%–68%) The familial form of glomangiomas typically follows an autosomal dominant inheritance pattern with incomplete penetrance and variable expression. The responsible gene, glomulin, is located on chromosome 1p21-22, within a 4 cm to 6 cm region mapped using YAC and PAC libraries. To date, 14 distinct loss-of-function mutations in the glomulin gene have been identified in affected individuals, which lead to elevated levels of cyclin E and c-Myc, contributing to cellular dysregulation.[8][7][9][10] Approximately 60% of familial cases involve at least 1 additional affected family member. Clinical onset may occur at birth or emerge during adolescence. Another identified mutation, 157_161del, has been documented as contributing to GVMs.[11] A notable subtype, segmental type 2 glomangiomas, begins with a solitary primary lesion, followed by the development of multiple distal lesions. This pattern is considered a manifestation of mosaicism within the inherited form.
Sporadic or De Novo Mutations Sporadic glomangiomas, which are not associated with a family history, can also present at birth and arise from spontaneous genetic mutations.[12]
Epidemiology
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 so far. It is more predominant in the male gender. About one-third of the patients present before the age of 20.[8][13][14] 10% of cases are of the disseminated type.[10] The most common reason for referral among vascular anomalies is venous malformations.[15]
Histopathology
These lesions are distinguishable by glomus cells surrounded by enlarged, dilated vein-like tubes. Glomus cells are poorly differentiated smooth muscle cells. They stain positive for vimentin, calponin, and SMC alpha-actin. They are negative for S-100, von Willebrand factor, and desmin.[1][7]
History and Physical
Glomus cancers, particularly glomangiomas, typically present at birth as bluish-purple skin lesions arranged in a cobblestone-like pattern. These lesions are often papular or nodular, hyperkeratotic, and range from 2 mm to 10 mm in diameter, though their size and number can vary significantly. Tenderness on palpation is common, with pain frequently triggered by pressure or exposure to cold stimuli.[1][12] Lesions predominantly occur in areas rich in glomus bodies, especially the distal extremities such as the palms, wrists, forearms, feet, and subungual regions. Approximately 75% of cases involve the hands.[8]
Visceral involvement is extremely rare but has been reported in sites including the nasal cavity, mediastinum, gastrointestinal tract, respiratory and urogenital tracts, and hepatobiliary system. Additionally, some patients with GVMs have presented with congenital cardiac anomalies such as ventricular septal defects and transposition of the great vessels.[6][16][17] Though glomus bodies are absent in normal nerve tissue, there is a case report describing nerve involvement by glomangioma.[18] Tracheal lesions, though rare, may cause dyspnea, hemoptysis, and retrosternal chest pain.[19]
Compared to solitary glomus tumors, glomangiomas tend to be larger, less well-circumscribed, and demonstrate slower blood flow. These lesions generally grow gradually over time. While the classic clinical triad—hypersensitivity, intermittent pain, and pinpoint pain—is considered characteristic of glomus tumors, it is rarely observed in glomangiomas.[20] A rare variant, the plaque-like type, presents as indurated, nodular, or discolored lesions that are typically non-tender but may bleed easily with minor trauma. These lesions are usually larger than other glomangioma types and represent the rarest clinical presentation.[14]
Evaluation
The confirmation of the diagnosis is through histopathology.[8] 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.[20] Electron microscopy shows glomus cells with dense bodies and smooth muscle myofibrils [1]. An X-ray may show osseous defects. Magnetic resonance imaging and color Doppler ultrasonography help define shape, size, and accurate location (see Image).[21][13] Dynamic time-resolved contrast-enhanced MR angiography can define vascularity.[22]
Treatment / Management
The goal of treatment is to decrease the symptoms. 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.[8][7] Excision therapy is the preferred treatment for painful lesions. Sclerotherapy was shown to be more effective in venous malformation than GVMs. In cases of nasal involvement, endoscopic excision or surgery is recommended.[9][23][24] In cases of large glomangiomas that are difficult to excise, the 1064-nm Nd: YAG laser is effective.[25] Also, positive results with the Nd: YAG laser have been reported in symptomatic familial cases.[26][27] External compression by elastic compressive garments worsens the pain.[12](B2)
Differential Diagnosis
Differential diagnosis for glomus cancers includes:
- Venous malformations: Glomangiomas are limited to the skin and mucosa. In contrast, other types of venous malformations can extend to deeper layers like muscles.[12]
- Schwannoma [28]
- Blue rubber bleb nevus syndrome: Characterized by multiple compressible venous malformations involving the skin and visceral organs, particularly the gastrointestinal tract. Lesions are typically sporadic and may resemble glomangiomas in appearance. Gastrointestinal bleeding is a common complication and the primary cause of mortality.[7]
- Neuroma
- Hemangiopericytoma
- Angioleiomyoma
- Hamartoma
- Hemangioma [13]
- Subdermal mass
- Carcinoid tumors
- Hemangiopericytoma [19]
- Paraganglioma
- Maffucci syndrome: multiple subcutaneous vascular nodules on the toes and fingers
- Glomus tumor: seen in the adult population, painful, more commonly involves nail beds, and genetic/histology is cellular dominant with glomus cell infiltration.
- Spiradenoma
- Leiomyoma
- Venous malformation: compressible, painful
Prognosis
The prognosis for glomus cancers, particularly glomangiomas, is generally favorable when the lesion is completely excised, with low rates of recurrence and excellent symptom resolution.[3] However, the presence of metastasis, although extremely rare, is associated with a poor prognosis and may indicate malignant transformation or aggressive disease behavior.[10] Long-term outcomes depend on early diagnosis, complete surgical removal, and close follow-up to monitor for recurrence or complications.
Complications
Glomus tumors and GVMs may be associated with several clinical complications, though many are rare. Recurrence following surgical excision occurs in approximately 10% to 33% of cases, often due to incomplete removal or multifocality of the lesions.[9][13] While malignant transformation is exceedingly rare, certain features are associated with a higher risk. These include lesions measuring greater than 2 cm, deep anatomical location, invasion of muscle or bone, and elevated mitotic activity.[14] Although metastasis has been reported in isolated cases, it remains extremely uncommon.[10] Nerve compression is a possible complication, particularly when lesions grow in proximity to major nerve pathways, leading to pain, numbness, or functional impairment.[29] In rare scenarios, these lesions may become life-threatening due to progressive growth, bleeding, or obstruction of vital organs such as the airway or gastrointestinal tract.[12] An unusual case documented the development of Spitz nevi overlying a congenital glomuvenous malformation. One hypothesis suggests that hyperemia within the glomangioma may stimulate surrounding structures such as hair follicles. Additionally, mutations in the glomulin gene may play a contributing role in this process.[30]
Deterrence and Patient Education
Patients should be advised to monitor for any signs of lesion recurrence, increased size, bleeding, or new symptoms. Prompt follow-up with a healthcare provider is essential if such changes occur, as they may indicate progression or complications requiring further evaluation and possible intervention.
Pearls and Other Issues
Diagnosing GVMs can be challenging due to their clinical overlap with other vascular anomalies and soft tissue tumors, such as venous malformations, hemangiomas, blue rubber bleb nevus syndrome, and other cutaneous lesions. Misdiagnosis may delay appropriate treatment and lead to unnecessary interventions. A thorough patient history, including family history, and a detailed physical examination are critical. Imaging studies and, when appropriate, histopathological analysis help confirm the diagnosis.
Enhancing Healthcare Team Outcomes
Glomangiomas often resemble other dermatologic lesions, such as papules or nodules, making accurate diagnosis a multidisciplinary effort. Optimal management requires collaboration among dermatologists, surgeons, and radiologists, especially when imaging or excision is indicated. Specialty care nurses play a vital role in coordinating follow-up, monitoring post-procedural outcomes, and delivering patient education regarding symptom monitoring and recurrence.
Radiologists contribute to diagnosis by identifying lesion depth, vascular involvement, or atypical features through imaging studies such as magnetic resonance imaging. Genetic counselors may also be involved in cases with a suspected familial pattern. An interprofessional team approach—facilitating timely referrals, shared decision-making, and integrated care planning—significantly improves diagnostic accuracy, treatment outcomes, and patient satisfaction.
Media
(Click Image to Enlarge)
Subungual Mass, STIR Magnetic Resonance Imaging. STIR sagittal image of the left index finger demonstrates a markedly hyperintense subungual mass.
Contributed by H Barazi, MD
References
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