Clinico-epidemiological profile and management outcome of subungual digital glomus tumor-Indian experience.

BACKGROUND: Glomus tumors are rare tumors arising from the mesenchymal smooth muscle cells of the glomus body. They are extremely painful tumors but because of their subungual location, remain mostly underdiagnosed. AIM: To characterize the demographic, clinical, onychoscopic, radiological features and management outcome of subungual glomus tumor. Material & methods- 15 patients with a total of 16 subungual glomus tumors were evaluated and their demographic data, history, clinical features, investigations, treatment, and follow-up were analyzed. RESULTS: Glomus tumors had a female preponderance (11/15) with thumb being the commonest site. All patients presented with intractable pain. Nail discoloration was observed in 11/16 (68.8%) lesions and nail plate deformity in 6/16 (37.5%) lesions. Common features on onychosocpy were pink glow and linear vascular structures. Doppler sonography and/or magnetic resonance imaging confirmed the diagnosis of glomus tumor in all the lesions. Surgical excision was done under local anesthesia using a trans-ungual approach and no recurrence was seen. CONCLUSIONS: High index of suspicion, meticulous clinical assessment along with radiological investigations can help in the early diagnosis. Complete surgical excision is the treatment of choice to prevent recurrence.

Tumor glómico gástrico: un reto en el diagnóstico preoperatorio de las lesiones subepiteliales del estómago / Gastric glomus tumour: A challenge in the preoperative diagnosis of stomach subepithelial lesions

No disponible

[Etiology and pathogenesis of glomus tumours (chemodectomas) of the base of the skull].

The objective of the present work was to describe the poorly known history of the development of the nomenclature of glomus tumours (chemodectomas) of the base of the skull, elucidate etiology and pathogenesis of these neoplasms. The authors present a chronological table illustrating the progress of etiological and pathogenetic studies as well as the surgical treatment of the tumours. The results of analysis and discussion of the controversial issues of the nomenclature are reported.

The association between glomus tumors and neurofibromatosis.

PURPOSE: To determine whether an epidemiologic association exists between glomus tumors and neurofibromatosis. METHODS: Using a pathology database, we established a study cohort consisting of all patients who had undergone excision of a glomus tumor of the hand between 1995 and 2010. We created a control cohort by randomly selecting 200 patients who had undergone excision of a ganglion cyst over the same period. We reviewed medical records for each cohort to identify patients with a diagnosis of neurofibromatosis. We calculated the odds ratio was calculated and performed Fisher's exact test to determine the significance of the association. RESULTS: We identified 21 patients with glomus tumors of the hand. Six of these patients carried the diagnosis of neurofibromatosis (29%). In contrast, no patients in the control group carried the diagnosis of neurofibromatosis. The odds ratio for a diagnosis of neurofibromatosis in association with a glomus tumor compared with controls was 168:1. CONCLUSIONS: This study provides evidence of a strong epidemiologic association between glomus tumors and neurofibromatosis. Glomus tumor should be included in the differential diagnosis in neurofibromatosis patients who present with a painful lesion of the hand or finger. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic III.

Estudo do perfil epidemiológico e avaliação pós-cirúrgica de oito casos de tumor glômico / Study of epidemiological profile and post-surgical evaluation of 8 glomus tumor cases

Introdução: O tumor glômico é lesão benigna rara que ocorre mais comumente em falanges distais e representa de 1% a 4,5% das neoplasias da mão. O tipo subungueal é mais frequente em mulheres nas terceira e quinta décadas de vida. Objetivo: Este trabalho objetivou avaliar o perfil epidemiológico de pacientes com tumor glômico, sua satisfação em relação ao tratamento, e a recidiva por retirada incompleta. Método: Estudaram-se os prontuários de oito pacientes com diagnóstico de tumor glômico tratados em serviço de dermatologia de 1992 a 2011 avaliando-se sexo, idade, profissão, região acometida, satisfação do paciente em relação ao tratamento e se houve recidiva. Resultados: A média de idade dos casos foi de 63 anos, superior à faixa etária descrita pela literatura. A profissão mais relacionada foi "prendas domésticas". Todos os pacientes ficaram satisfeitos com o tratamento do ponto de vista estético. A melhora da dor foi plena em três dos seis pacientes que retornaram para avaliação. Naqueles em que a melhora não foi total, houve recidiva. A taxa de recidiva por retirada incompleta foi de 50%. Conclusão: Conclui-se a persistência da dor como indicadora de recidiva e percebe-se a efetividade da cirurgia na melhora da dor.

Introduction: Glomus tumors are rare benign lesions that are most often found on the tips of the fingers; they account for 1.0-4.5% of neoplasias on the hand. Subungual glomus tumors occur more frequently in women in their 30s and 50s. Objective: This study evaluated glomus tumor patients' epidemiological profiles, their satisfaction with the treatment and the rate of recurrence due to incomplete removal. Method: The records of patients that were treated for glomus tumors at a dermatology clinic between 1992 and 2011 were evaluated regarding gender, age, profession, affectedarea of the body, satisfaction with the treatment and possible recurrence. Results: The patients (n = 8, average 63 years) were older than those usually described in the literature. The most frequently described profession was that of housewife. All patients were satisfied with the aesthetic results of the treatment. There was a total improvement of the pain in threepatients, while patients with a partial improvement in pain experienced a recurrence. The recurrence rate due to incomplete removal was 50%. Conclusion: The surgery's effectiveness can be measured by the improvement of the pain; persistence of pain can indicate recurrence.

Bronchial glomus tumor mimicking a COPD exacerbation.

We report the case of a glomus tumor originating in the left main bronchus diagnosed in a 79 year old Caucasian man. A glomus tumor is an extremely rare neoplasm in the bronchi with nonspecific clinical features. Bronchoscopy allows the diagnosis through biopsy and subsequent histopathological examination of the tissue and in selected cases may represent a valid alternative to surgery permitting a radical tumor excision.

Recurrent digital glomus tumor: analysis of 75 cases.

BACKGROUND: Glomus tumors are rare, benign, cutaneous neoplasms that must be excised completely to prevent recurrence. OBJECTIVE: To investigate factors associated with recurrence of glomus tumors after surgery. METHODS AND MATERIALS: Fifty-eight women and 17 men with digital glomus tumors underwent surgery between 1990 and 2008 at our hospital. These cases were retrospectively analyzed. RESULTS Mean age at diagnosis was 41.8, with an average diagnostic delay of 3.9 years. The tumor was located on a finger in 70 cases (right, 29; left, 41) and a toe in five (right, 3; left, 2). The tumor recurred in 13 (17%) patients. Recurrence was more likely if the tumor was skin-colored (odds ratio (OR)=31.67, 95% confidence interval (CI)=2.68-373.74, p=.006) or located within the nail matrix (OR=5.79, 95% CI=1.03-32.49, p=.046). No recurrence occurred in patients who had had preoperative magnetic resonance imaging or ultrasound studies. CONCLUSION: Skin-colored tumors or those in the nail matrix are at higher risk of recurrence. The authors have indicated no significant interest with commercial supporters.

Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

This article discusses the epidemiology, etiology, presentation, pathology, evaluation and staging, and treatment of unusual skin tumors, such as Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

Current assessment and management of glomus tumors.

PURPOSE OF REVIEW: To provide an overview on the recent advances in the diagnosis and treatment of glomus tumors (paragangliomas). RECENT FINDINGS: The review focuses on the genetics of paragangliomas and discusses the role of newer therapeutic modalities in the management of jugulotympanic paragangliomas. SUMMARY: Recent advances in the field of genetics allowed the identification of three main loci involved in hereditary paragangliomas. Genetic screening of patients with family history identifies affected individuals. The management of glomus tumors continues to be a challenging task. Surgery remains the mainstay of treatment for young healthy patients with functional cranial nerve deficits. Gamma knife radiosurgery is offered as an adjunctive to limited surgery and as the primary treatment modality in poor surgical candidates or patients with bilateral disease.

Glomus tumors in patients of advanced age: a conservative approach.

OBJECTIVES: Identify and discuss controversies in the management of paragangliomas in elderly patients. Assess and evaluate a conservative treatment strategy involving limited surgical resection and vigilant monitoring of the outcome measures of tumor control, peritreatment morbidity, symptom resolution, and hearing preservation. STUDY DESIGN: Retrospective case review. METHODS: All of the patients in this study were over age 60 with temporal bone glomus tumors. Primary outcome assessment included length of hospitalization, perioperative morbidity, symptom resolution, hearing preservation, and long-term tumor control. RESULTS: Twelve female patients with mean age of 74.5 years (range 61-85 years) with follow-up from 24 months to 33 years (mean/median: 5/7.8 years) were identified. Nine (75%) of the patients presented with pulsatile tinnitus. Seven patients (58%) underwent surgical excision of the middle ear component of the paraganglioma. Tumors extending to the jugular foramen were purposely not resected. Five patients (45%) had relative or absolute contraindications to surgical resection and were treated with observation or primary radiation therapy. Post-treatment audiometric evaluation confirmed stable or improved hearing. Pulsatile tinnitus resolved in all patients. No patient experienced cranial nerve deficits, extended hospitalization, or blood transfusions. All patients were followed closely with radiological imaging. The majority of patients demonstrated no disease or stable disease, while two patients demonstrated tumor growth 6 years after diagnosis. CONCLUSION: A prolonged natural history and the morbidity associated with surgical intervention have led to controversies in the treatment of glomus tumors in an elderly population. Our experience supports recent limited reports advocating conservative surgical excision and vigilant long-term monitoring in this population.

[Glomus tumors of the middle ear in children].

Glomus tumor (GT) is a rare tumor of the middle ear in children. In spite of cytologically benign nature of this tumor, its course can be characterized as malignant because of GT infiltrative growth, destruction of the adjacent bone tissue, possible intracranial involvement. Because of scare symptoms, early GT diagnosis is problematic. At admission to hospital principal complaints of the children are poor hearing, discharge from the ear. These often underlie misdiagnosis -- acute otitis media. GT of the middle ear are detected with application of computed tomography which is able to show changes in the soft tissue and bone destruction, and angiography. Combination of the above two methods allows assessment of location and advance of the pathological process. GT treatment planning depends on the examination data and can include radiotherapy, endovascular occlusion of the vessels carrying blood to the tumor, surgical treatment and combination of the methods. Modern approaches to GT diagnosis and treatment are described. A case of GT of the temporal bone with involvement of the middle cranial fossa in a 4-year-old child is reported.

Extradigital glomus tumors: a 20-year experience.

OBJECTIVE: To review a large series of extradigital glomus tumors in order to gain a better understanding of their presentation and provide guidelines to aid in their diagnosis and treatment. PATIENTS AND METHODS: We performed a retrospective review of all extradigital glomus tumors seen at our institution during a 20-year period (1985-2005) to document the incidence of the classic triad of symptoms, the duration of symptoms, the contribution of imaging to making a definitive diagnosis, and the effectiveness of treatment. RESULTS: Fifty-six different patients with extradigital glomus tumors presented as follows: glomus tumors in the hand (3), wrist (4), forearm (11), elbow (4), arm (4), shoulder (2), buttock (1), thigh (5), knee (10), leg (3), ankle (2), foot (2), back (1), nose (1), cheek (1), ear lobe (1), and trachea (1). Forty-eight patients presented with pain and localized tenderness, but only 1 patient presented with cold Intolerance. The average duration of symptoms was greater than 7 years, with most patients being evaluated previously and having their conditions misdiagnosed. Magnetic resonance imaging proved to be the most useful modality for localization of these lesions. Surgical resection was the definitive treatment and generally provided immediate and sustained pain relief. CONCLUSIONS: Extradigital glomus tumors are not a rare subgroup of glomus tumors. Treatment outcomes are excellent, but misdiagnosis and delayed diagnosis are common. Improved guidelines regarding symptoms and diagnosis of these neoplasms may reduce the morbidity, ensuing chronic pain, and psychiatric consequences of delayed diagnosis or misdiagnosis.

Glomuvenous malformation (glomangioma) and venous malformation: distinct clinicopathologic and genetic entities.

OBJECTIVES: To develop clinical criteria that permit clinical distinction between inherited glomuvenous malformation (GVM), known as glomangioma, and inherited cutaneomucosal venous malformation and to test these criteria on sporadic lesions. DESIGN: Clinical data were compiled for 1685 patients with inherited or sporadic cutaneous venous anomalies. Based on a cohort of patients with a mutation in the TIE2 or glomulin gene or a histologic diagnosis, we defined clinical criteria for inherited GVM and cutaneomucosal venous malformation. We then applied these criteria to sporadic cases in a blinded manner and genetically or histologically confirmed this clinical diagnosis whenever possible. RESULTS: Glomuvenous malformations accounted for 5.1% of venous anomalies and were frequently inherited (63.8%), whereas venous malformations were rarely familial (1.2%). Glomuvenous malformations were nodular and scattered, or plaque-like and segmental, with color varying from pink to purplish dark blue, whereas most venous malformations (VMs) were soft, blue, and often localized vascular lesions. Glomuvenous malformations were mainly located on the extremities and involved skin and subcutis, whereas VMs commonly affected muscles and joints (P<.001). Glomuvenous malformations had a distinct raised, often hyperkeratotic cobblestone-like appearance and could not be completely emptied by compression, unlike VMs. Glomuvenous malformations were painful by compression, whereas VMs were painful on awakening, after activity, or with hormonal changes. Elastic compressive garments aggravated pain in GVMs, in contrast to VMs. CONCLUSIONS: This large series of patients with superficial venous anomalies established clinical features that distinguish VMs and GVMs. This differential diagnosis is essential, as the outcome and the treatment for GVMs differ.

Subungual glomus tumor: clinical manifestations and outcome of surgical treatment.

Surgical excision is the treatment of choice for subungual glomus tumor. However, the anatomical location has inherent difficulties. We report the outcomes of surgical treatments for subungual glomus tumor. Sixteen patients, who were seen over an eight-year period (1995-2003) and confirmed as gloums tumor by histopathologic examination were reviewed. The most common subjective symptom was pain induced by contact in 81%. The tumor presented as a discolorated spot or subungual nodule and 38% of tumors were acccompanied with nail dystrophy. All tumors showed discolorated spots or subungual nodules. As shown in the Table 2, the dystrophic nail change was found in 38% of tumors. Differently oriented incisions were made according to the location of tumor, matrix, or bed. The original nail plate was restored in eight patients. Thirteen patients (81%) had cosmetically excellent nail plates, and three patients (19%) had partial distal splits of nail plates. There was no recurrence. Our series suggests that a transungual approach with nail avulsion and an incision selected according to the tumor location can produce an excellent outcome with minimal postoperative complications. Dressing with a trimmed nail plate may also be beneficial in managing the wound and preventing postoperative nail deformity.

[Glomus tumors of the finger: a report on 60 cases]. / Parmak yerlesimli glomus tümörü: 60 olgunun incelenmesi.

OBJECTIVES: We evaluated patients who underwent surgery for glomus tumors of the finger. METHODS: During a 23-year period, 60 patients (48 females, 12 males; mean age 34.6 years; range 5 to 60 years) underwent surgery for glomus tumors of the finger. The patients were evaluated with respect to symptoms on presentation, diagnosis, operation types, and postoperative complications. The mean follow-up was 16 months (range 6 to 72 months). RESULTS: The most common symptom was pain (67%), which intensified when exposed to cold in 42%. Other symptoms included tenderness in 37 patients (62%), swelling in the finger tip in six patients (10%), and nail deformities in 34 patients (57%). The tumors were most frequently encountered in the fourth and fifth decades of age. Localization of the tumor was in the right hand in 32 patients (53.3%), and in the left hand in 28 patients (46.7%). The time to surgery from the onset of symptoms ranged from six months to 30 years (mean 6 years). Fifty-one patients were examined by plain radiographs, 27 of which showed concave-surface erosions in the bone; nine patients underwent magnetic resonance imaging studies besides plain radiography. The tumors were subungual in 49 patients (82%) and in the pulp in 11 patients (18%), for which transungual and midlateral approaches were applied, respectively. Postoperatively, nine patients (15%) developed recurrences, all of whom achieved clinical recovery following a subsequent operation. CONCLUSION: Given the considerably delayed time to diagnosis, glomus tumors should be taken into consideration in the presence of severe finger tip pain of unknown origin.

Multiple familial facial glomus: case report and review of the literature.

Facial paraganglioma is an extremely rare tumor that originates from abnormal paraganglionic tissue situated in the intrapetrous facial canal. A review of the English-language literature shows that only 8 cases of facial nerve paraganglioma have been published. In each case the facial glomus presented itself sporadically, completely independent of any other form of paraganglioma. This study reports an intrapetrous facial glomus that occurred in a case of multiple paragangliomas with a hereditary pattern. To our knowledge, this is the first report of such a combination.

Glomangioma / Glomangioma

Los tumores glómicos se pueden clasificar en solitarios y múltiples o glomangiomas, con marcadas diferencias clínicas, histopatológicas, epidemiológicas e etiopatogénicas entre ambos tipos. Exponemos el caso de un varón de 24 años con una única lesión de 4,5 x 2 cm de diámetro que, sin embargo, por su histopatología e historia familiar quedaba mejor encuadrada dentro de los glomangiomas, en lugar de un tumor glómico solitario. (AU)

[Glomangioma or "glomus tumor" of the nasal cavity: apropos of a new case and review of the literature]. / Glomangiome ou "tumeur glomique" des cavités nasales: à propos d'un nouveau cas et revue de la littérature.

Glomangioma, or "glomic tumour" is a benign soft tissue tumour appearing most commonly at the distal extremities, in the nailbed and subcutaneous tissue. This is a vascular tumour, rare for the dermatologist, and exceptional in an ENT site. We describe here what is, as far as we can tell from the literature, the eighteenth case. The problem is the diagnosis of a vascular tumour, arising usually from the septal mucosa, which has to be distinguished histopathologically from a haemangiopericytoma. Radiological investigation will define the extent of the tumour and guide the treatment plan, which is purely surgical and consists of wide excision to avoid the possibility of a local recurrence.