Tumor glômico em região hipotenar: relato de caso / Glomic tumor in hypotenar region: case report / Tumor glómico en región hipotenar: reporte de caso

Introdução: O tumor glômico é um hamartoma benigno, geralmente único, que se desenvolve a partir de uma estrutura neuromioarterial, o glomo. É uma doença incomum na prática dermatológica, muito frequente nas polpas digitais. Com relação à epidemiologia, corresponde a cerca de 1 a 5% dos tumores de mão e 65% dos casos estão localizados embaixo das unhas. Mostra-se mais prevalente em mulheres e mais frequente na terceira e quinta décadas de vida. Foi evidenciada uma escassez de estudos sobre tumor glômico em região palmar, falta de informações nacionais e necessidade de destacar a importância clínica desse tema. Objetivo: Relatar o caso de uma paciente com tumor glômico e enfatizar as características clínicas e o tratamento recomendado. Método: O caso é de uma paciente sexo feminino, negra, 61 anos apresentando dor intensa e localizada na lesão em região hipotenar da mão esquerda há dois anos. Caracteriza a dor como perene, nega irradiação, melhora ao usar compressa fria e piora ao usar compressa quente. Fez uso de analgésicos e anti-inflamatórios, não obtendo sucesso na melhora clínica. A dor se tornou de caráter intermitente e houve uma intolerância gástrica à medicação utilizada. Resultados: A melhora do quadro se deu após recorrer ao dermatologista, o qual realizou uma pequena cirurgia de retirada da lesão, apontando o diagnóstico de tumor. Conclusão: Diferentemente desse relato, em que a lesão é palmar, a maioria dos casos desse tumor benigno possuem lesões subungueais e nos dedos.(AU)

Introduction: Glomus tumor is a benign hamartoma, usually single, that develops from a neuromyoarterial structure, the glomus. It is an uncommon disease in dermatological practice, very common in digital pulps. Regarding epidemiology, it corresponds to about 1 to 5% of hand tumors and 65% of cases are located under the nails. It is more prevalent in women and more frequent in the third and fifth decades of life. A lack of studies on glomus tumors in the palmar region, lack of national information and the need to highlight the clinical importance of this topic was evidenced. Objective: Aimed to report the case of a patient with a glomus tumor and emphasize the clinical features and recommended treatment. Method: The case is a female patient, black, 61 years old with severe pain located in the lesion in the hypothenar region of the left hand for two years. Characterizes pain as perennial, denies irradiation, improves when using a cold compress and worsens when using a hot compress. He used analgesics and antiinflammatory drugs, with no success in clinical improvement. The pain became intermittent and there was gastric intolerance to the medication used. The condition improved after resorting to a dermatologist, who performed a minor surgery to remove the lesion, indicating the diagnosis of glomus tumor. Conclusion: Unlike this report, in which the lesion is palmar, most cases of this benign tumor have subungual lesions and lesions on the fingers.(AU)

Introducción: El tumor glómico es un hamartoma benigno, generalmente único, que se desarrolla a partir de una estructura neuromioarterial, el glomus. Es una enfermedad poco común en la práctica dermatológica, muy común en las pulpas digitales. En cuanto a la epidemiología, corresponde al 1 al 5% de los tumores de mano y con 65% ubicado en la región subungueal. Es más prevalente en mujeres y más frecuente en la tercera y quinta décadas de vida. Se evidenció la falta de estudios sobre los tumores glómicos en la región palmar, la falta de información nacional y la necesidad de resaltar la importancia clínica de este tema. Objetivo: Reportar el caso de un paciente con tumor glómico y enfatizar las características clínicas y el tratamiento recomendado. Metodología: El caso es una paciente mujer, negra, 61 años con dolor severo localizado en la lesión en la región hipotenar de la mano izquierda desde hace dos años. Caracteriza el dolor como perenne, niega la irradiación, mejora cuando se usa una compresa fría y empeora cuando se usa una compresa caliente. Utilizó analgésicos y antiinflamatorios, sin éxito en la mejoría clínica. El dolor se volvió intermitente y hubo intolerancia gástrica a la medicación utilizada. La condición mejoró tras acudir a un dermatólogo, quien realizó una cirugía menor para extirpar la lesión, indicando el diagnóstico de tumor glómico. Conclusión: A diferencia de este informe, en el que la lesión es palmar, la mayoría de los casos de este tumor benigno tienen lesiones subungueales y lesiones en los dedos.(AU)

Other Rare Sinonasal Malignant Tumours Involving the Anterior Skull Base.

Sinonasal tumours are rare, and among these there exist a small number of histologic subtypes that are infrequently encountered and rarely mentioned in the literature. These have been presented as either case reports or small case series, and their very low incidence makes prospective studies practically impossible. This review analyses the available literature, including our own experience and endeavours to outline management strategies, which involve a high index of suspicion and counselling of patients. In most instances, these tumours require aggressive multimodal treatment to improve survival outcomes. The overall prognosis remains dismal.

Glomus Tumor of Sella Turcica With Synaptophysin Expression Mimicking Pituitary Adenoma.

Glomus tumor can rarely arise in the central nervous system as a sella turcica mass. In this article, we report a case of sellar glomus tumor in a female patient who presented at the age of 8 years with visual impairment. The tumor recurred at 4 years and 26 years after initial excision and gamma knife therapy. Histologic examination showed a monotonous population of oval cells accompanied by delicate blood vessels, features mimicking pituitary adenoma. The tumor showed histologic progression at the second recurrence. Synaptophysin staining was positive, but chromogranin and CD56 were negative. The tumor cells were negative for epithelial markers but expressed actin and SMA. Awareness of the rare occurrence of glomus tumor at this region, careful analysis of morphology, and appropriate immunohistochemical workup are essential to solve this diagnostic challenge. The clinicopathologic features of all previously reported cases are reviewed.

[Diagnosis and treatment of glomus tumors of the skull base and neck]. / Diagnostik und Therapie von Glomustumoren der Schädelbasis und des Halses.

Glomus tumors (paragangliomas) are rare, usually benign, neuroendocrine tumors that arise from nonchromaffin cells and can be multifocal or familial. They grow slowly, often infiltratively and rarely form metastases. A distinction is made between carotid, jugular, tympanicum and vagal tumors. Depending on the location, clinical symptoms include neck swelling, hearing loss with pulse-synchronous ear noise or cranial nerve failures (IX-XI). Diagnosis is often delayed because of nonspecific symptoms. Diagnostically, tumor expansion and perfusion can be well visualized by color Doppler sonography. In the T2-weighted MRI, the tumor is hyperintense, after contrast agent administration a strong enhancement occurs due to the strong vascularization. In DSA (digital subtraction angiography), even small glomus tumors can be detected. Therapeutically, operative tumor resection after embolization with, e.g., polyvinyl alcohol particles or liquid embolization can be performed. If the patient is in poor general condition or if the tumor is inoperable, different treatment techniques (intensity-modulated radiotherapy, gamma or cyber-knife) may be considered as a conservative therapeutic alternative.

Mechanical Embolectomy of Distally Migrated Onyx After Surgical Resection of Glomus Vagale Tumor: Technical Report and Review of Literature.

BACKGROUND: Endovascular embolization is an important tool in the management of multiple pathologies as a preoperative adjunct in the care of arteriovenous malformations or vascular tumors. CASE DESCRIPTION: We report a case of delayed distal Onyx migration after surgical resection of a glomus vagale tumor, which had been preoperatively embolized. In this report, the patient underwent successful embolectomy of the migrated Onyx fragment using manual aspiration. CONCLUSIONS: This case represents the first in the literature to describe this potential delayed complication, as well as its management strategy.

Synchronous primary glomus tumor in a patient with adenocarcinoma of the ipsilateral lung.

Glomus tumors are rare mesenchymal neoplasms arising from the glomus bodies in the deep dermis of the extremities or derive from the modified smooth muscle cells of the normal glomus body. Primary pulmonary glomus tumors are particularly rare and infrequently reported. We report a case of a primary glomus tumor occurring in the lung with adenocarcinoma in the ipsilateral lung as synchronous lung cancers in a 69-year-old man. He underwent lobectomy for adenocarcinoma and wedge resection for the glomus tumor with mediastinal lymph node dissection and was doing well without recurrence or metastasis at the last follow-up.

Glomus tumor: a comprehensive review of the clinical and histopathologic features with report of two intraoral cases.

BACKGROUND: Glomus tumors are benign neoplasms that most commonly present in the subungual region. Their occurrence in the oral cavity is exceedingly rare. Here, we present 2 cases from the oral cavity, detail their clinical and histopathologic features, and review the literature for solitary cases involving the oral regions. STUDY DESIGN: The English language literature was queried for cases of benign glomus tumors in/around the oral cavity. Additional citations were cross-referenced from the identified sources. RESULTS: Thirty-one cases of solitary glomus tumor in the oral and paraoral regions have been described, including the present cases. Patient age ranged from 10 to 85 years, with an average age of 47 years. In 12 of the 31 cases, the tumors occurred in the lips, 5 in the palate, 4 in the tongue, 4 in the buccal mucosa, 3 in the gingiva, and 1 each in the parotid, pterygoid fossa, and oropharynx. Only 18 of these cases had accompanying immunohistochemical stains, with 14 expressing positivity for muscle cell markers. CONCLUSIONS: Although glomus tumors have distinct histopathologic features, diagnostic confusion may exist with regard to extradigital locations. Detailed documentation and discussion of the clinical and histopathologic features of rare tumors like these are vital to understanding them.

BRAF V600E Mutations Occur in a Subset of Glomus Tumors, and Are Associated With Malignant Histologic Characteristics.

Glomus tumors are rare mesenchymal neoplasms with a phenotype akin to the modified smooth muscle cells of the glomus body. Most are benign, but rare examples show malignant histologic characteristics and aggressive behavior. We recently encountered a malignant glomus tumor with BRAF V600E mutation. We sought to study a large cohort for this mutation, with particular attention to associated malignant histologic characteristics. Tumors were classified based on WHO criteria as benign, uncertain malignant potential (glomus tumors of uncertain malignant potential-GT-UMP), or malignant. Tumors were screened for BRAF V600E by immunohistochemistry, and positive staining was evaluated further by Sanger sequencing. A total of 102 glomus tumors were included and classified as benign (57, 56%), GT-UMP (15, 15%) and malignant (30, 29%). Tumors occurred in patients aged 8 to 89.9 years (median: 50.2), without sex predilection (55% men). Most occurred in the superficial soft tissue (84%) and upper extremities (55%). Six of 95 tested cases had BRAF V600E mutation (6%), including 0 of 57 benign tumors, 3 of 14 GT-UMP (21%), and 3 of 24 malignant tumors (12%). Follow-up was obtained for 59 cases (median: 75.7 mo, range: 7.8 to 268.5). Three of 11 malignant tumors (27%) had progressive disease: 1 with metastasis to brain and heart, 1 with enlarging residual disease, and 1 with recurrence. Two of 4 GT-UMP (50%) had progressive disease: 1 with metastasis to lung, and 1 with local recurrence (50%). Three of 44 benign tumors (7%) had local recurrence. Two of 5 patients with BRAF V600E had progression, including 1 GT-UMP with local recurrence and 1 malignant tumor with enlarging residual disease. In summary, BRAF V600E mutation was detected in 6% of glomus tumors, all of which were malignant or GT-UMP. This mutation may be associated with a malignant phenotype, although study of additional cases is needed. In patients with progressive disease, BRAF could be a promising therapeutic target.

Malignant glomus tumors of the head and neck in children and adults: Evaluation and management.

OBJECTIVES/HYPOTHESIS: To describe our current multidisciplinary approach to pediatric malignant glomus tumors of the head and neck and review the current literature. STUDY DESIGN: Retrospective chart review at a tertiary referral children's hospital and a comprehensive literature review. METHODS: A comprehensive literature search of PubMed, Embase, Web of Science, Google Scholar, and EBSCO with respect to malignant glomus tumors of the head and neck was conducted. We obtained expert input from other pertinent specialties, including oncology, pathology, and radiology. To highlight the difficulty of evaluation and management of these patients, we also present a pediatric patient with a left neck malignant glomus tumor and lung metastases. RESULTS: Only two cases of pediatric malignant glomus tumor (including our own) have been reported in the English literature. Overall, 14 malignant glomus tumors have been reported in the head and neck (11 primary and three metastatic). Surgical resection is the mainstay of treatment, but local recurrence is common (five of 11, 45%). CONCLUSIONS: Malignant glomus tumor of the head and neck is an extremely rare tumor in children. Evaluation consists of imaging, and tissue biopsy is necessary for definitive diagnosis. Management options include surgical resection with or without an adjuvant chemotherapy protocol similar to those designed for sarcoma. Additional reports are necessary so that we may determine the utility, if any, of radiotherapy in the management of this tumor. Laryngoscope, 127:2873-2882, 2017.

Endoscopic resection of invasive glomangiopericytoma following preoperative embolisation.

We present a case of a 60-year-old woman with a 2-year history of nasal obstruction and unilateral recurrent epistaxis. Anterior rhinoscopy identified a unilateral hypervascular lobular mass occupying the entire left nasal cavity. Imaging studies documented an extensive soft tissue density lesion in the left nasal cavity with complete infiltration of the anterior ethmoid, limited indentation of the medial wall of the orbit, bone demineralisation of the cribriform plate and involvement of the anterior cranial fossa. The diagnosis of locally invasive glomangiopericytoma was obtained preoperatively through biopsy of the lesion, which resulted in a self-limited epistaxis that was easily controlled by nasal packing, confirming the vascular nature of the lesion. We discuss the diagnostic work up, imaging and surgical approach of this tumour, and review the recent literature on endoscopic treatment of these lesions.

Laser Therapy for an Acquired Glomuvenous Malformation (Glomus Tumour): A Nonsurgical Approach.

BACKGROUND: Glomuvenous malformations (GVMs) (previously known as glomus tumours) are uncommon, benign, vascular neoplasms. Current treatments include surgical excision and sclerotherapy, often with high recurrence rates and poor cosmetic results. OBJECTIVE: We sought to use a nonsurgical approach for treatment of a GVM. METHODS: We present a patient with an acquired, biopsy-proven GVM of the heel unamenable to surgical excision, treated with a long pulsed 1064-nm neodymium-doped yttrium aluminum garnet (Nd:YAG) laser. RESULTS: Excellent cosmesis and long-term remission were achieved after several treatment sessions. CONCLUSION: Our experience provides further evidence to support the safety and effectiveness of the 1064-nm Nd:YAG laser in the management of large and surgically challenging GVMs.

Embolization in the treatment of an intraosseous glomus tumor in the upper thoracic spine complicating compression myelopathy: a case report and a literature review.

Glomus tumors are very infrequent in the spine. The lesions can grow intraosseously along the entire spinal axis. A single female presenting with back pain from the upper thoracic spine is reported on. Removal of this lesion may require reconstruction of the anterior column with posterior fixation resulting in significant blood loss. The current report describes an embolization procedure prior to removal in order to reduce the significant blood loss that occurs with removal of this lesion, and summarizes the clinical and pathological characteristics of this rare tumor. A single, recent case and removal of an intraosseous tumor arising from the upper thoracic vertebra of T2-T4 is described. A 45-year-old female presenting with symptoms secondary to a glomus tumor of the upper thoracic vertebra of T2-T4 underwent resection of the lesion followed by reconstruction of the anterior column following preoperative emobolization. She had neurological symptoms for 3 years, and an irregular crescent-shaped lesion was seen going through the foramen at T3 to the chest cavity in the MRI scans. The operation was performed with a posterior approach in a single stage. The use of preoperative embolization of the T2-T4 segmental arteries resulted in significantly less blood loss as compared to without an embolization procedure. It was confirmed by histopathological examination that the glomus tumor rose from the smooth muscle cells in the right paravertebral muscles of T2. The glomus tumor has not recurred in the MRI during the five-year follow-up. Intraosseous glomus tumors are rare lesions that may extend into the epidural space and through the neural foramina and chest compartments resulting in neurological compromise. Over time, they may grow very large. Radiotherapy can be useful for eradication of this rare lesion. However, it can reoccur requiring extensive surgery resulting in significant blood loss. Preoperative embolization results in a reduction of blood loss and can be a very useful technique when performing the resection of large lesions suspected to be glomus tumors.

Clinical evaluation and management of benign soft tissue tumors of the extremities.

Benign lesions comprise a majority of soft tissue tumors. It has been estimated that their incidence outnumbers that of malignant tumors by a factor of at least 100 [1]. While history and physical examination can start the diagnostic process, imaging including the use of magnetic resonance imaging can be more helpful. Biopsy of these tumors is sometimes necessary and can be performed in a number of ways, often in conjunction with definitive treatment. Specific diagnostic and treatment strategies for a number of the more commonly encountered benign soft tissue tumors including lipomas, pigmented villonodular synovitis and hemangiomas are reviewed. An algorithm for the management of benign soft tissue tumors is discussed.