An exceptional case of malignant glomus tumor and a review of the literature.

Malignant glomus tumors (MGTs) are exceptional but pose diagnostic and therapeutic challenges. Wide resection is the recommended treatment method, however, no data are available concerning adjuvant therapies. We present an exceptional case of extradigital deep-seated MGT of the forearm, with an exceptional bone infiltration. Despite being treated with wide resection, the patient had an optimal functional outcome, no functional loss, no motor or sensitive deficits and has returned to his full daily activity.

Treatment of primary tracheal glomus tumors: Two case reports and a literature review.

RATIONALE: Glomus tumors (GTs) are rare soft tissue neoplasms. Several treatment options have been reported for tracheal GTs including thoracotomy, bronchoscopic electrocautery, Nd: YAG laser, and cryotherapy. However, few studies have evaluated the ideal treatment for tracheal GTs. PATIENT CONCERNS: A 30-year old man who presented with cough, and expectoration for 1 month, and who had been diagnosed as having a tracheal neoplasm by cervical, and thoracic computed tomography (CT). The patient was a47 years old man. He was admitted to our hospital presenting with intermittent hemoptysis for 3 years. Thoracic CT revealed a round tumor on the right posterior tracheal wall. DIAGNOSES: Both of them were diagnosed as benign GTs. Histopathology of the tumor showed clusters of round epithelioid cells with eosinophilic cytoplasm, and uniform round to ovoid nuclei surrounding dilated capillaries. Immunohistochemical staining was positive for smooth muscle actin (SMA). INTERVENTIONS: The tracheal tumor of first patient was located at the level of C7-T1. Tumor resection was performed under fiberoptic bronchoscopy. The tracheal tumor in second patient was located in the lower trachea. Surgical tracheal resection and anastomosis were performed. OUTCOMES: Both of them achieved good results and no recurrence was seen at the final follow-up LESSONS:: We recommend choosing the most appropriate approach to manage tracheal GTs based on patients' general condition, and tumor characteristics to obtain an excellent prognosis. Our 2 cases of tracheal GT were managed by different approaches, and both achieved good results.

[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.

Effect of bilateral carotid body resection on the counterregulatory response to hypoglycaemia in humans.

NEW FINDINGS: What is the central question of this study? Hyperoxia blunts hypoglycaemia counterregulation in healthy adults. We hypothesized that this effect is mediated by the carotid bodies and that: (i) hyperoxia would have no effect on hypoglycaemia counterregulation in carotid body-resected patients; and (ii) carotid body-resected patients would exhibit an impaired counterregulatory response to hypoglycaemia. What is the main finding and its importance? Our data indicate that the effect of hyperoxia on hypoglycaemic counterregulation is mediated by the carotid bodies. However, a relatively normal counterregulatory response to hypoglycaemia in carotid body-resected patients highlights: (i) the potential for long-term adaptations after carotid body resection; and (ii) the importance of redundant mechanisms in mediating hypoglycaemia counterregulation. Hyperoxia reduces hypoglycaemia counterregulation in healthy adults. We hypothesized that this effect is mediated by the carotid bodies and that: (i) hyperoxia would have no effect on hypoglycaemia counterregulation in patients with bilateral carotid body resection; and (ii) carotid body-resected patients would exhibit an impaired counterregulatory response to hypoglycaemia. Five patients (three male and two female) with bilateral carotid body resection for glomus tumours underwent two 180 min hyperinsulinaemic, hypoglycaemic (∼ 3.3 mmol l(-1)) clamps separated by a minimum of 1 week and randomized to either normoxia (21% fractional inspired O2 ) or hyperoxia (100% fractional inspired O2). Ten healthy adults (seven male and three female) served as control subjects. Hypoglycaemia counterregulation in carotid body-resected patients was not significantly altered by hyperoxia (area under the curve expressed as a percentage of the normoxic response: glucose infusion rate, 111 ± 10%; cortisol, 94 ± 6%; glucagon, 107 ± 7%; growth hormone, 92 ± 10%; adrenaline, 89 ± 26%; noradrenaline, 79 ± 15%; main effect of condition, P > 0.05). This is in contrast to previously published results from healthy adults. However, the counterregulatory responses to hypoglycaemia during normoxia were not impaired in carotid body-resected patients when compared with control subjects (main effect of group, P > 0.05). Our data provide further corroborative evidence that the effect of hyperoxia on hypoglycaemic counterregulation is mediated by the carotid bodies. However, relatively normal counterregulatory responses to hypoglycaemia in carotid body-resected patients highlight the importance of redundant mechanisms in mediating hypoglycaemia counterregulation.

Head-and-neck paragangliomas are associated with sleep-related complaints, especially in the presence of carotid body tumors.

OBJECTIVES: The carotid body functions as a chemoreceptor. We hypothesized that head-and-neck paragangliomas (HNP) may disturb the function of these peripheral chemoreceptors and play a role in sleep-disordered breathing. DESIGN: This is a case-control study. SETTING: This study was conducted in a tertiary referral center. PARTICIPANTS AND MAIN OUTCOME MEASURES: We assessed fatigue, sleep, and exercise capacity in 74 HNP patients using three questionnaires (Epworth Sleepiness Scale, St. George Respiratory Questionnaire, and a standard clinical sleep assessment questionnaire). Outcomes were compared to those of age- and sex-matched controls. RESULTS AND CONCLUSIONS: Activity, disturbance of psychosocial function, and total score were worse compared to controls (15.4 ± 18.5 vs. 7.2 ± 9.9, P = 0.007; 5.3 ± 10.5 vs. 1.2 ± 2.6, P = 0.008; and 10.4 ± 12.9 vs. 5.0 ± 4.8, P = 0.006, respectively). Patients reported more daytime fatigue, concentration difficulties, and depression (51% vs. 24%, P = 0.006; 31% vs. 10%, P = 0.010; and 19% vs. 2%, P = 0.012). Waking up was reported to be less refreshing in HNP patients (53% vs. 73%, P = 0.038). Dysphonia was a predictor of symptoms, activity, disturbance of psychosocial function, and total scores. Remarkably, the presence of a carotid body tumor was an independent predictor of increased daytime sleepiness (ß = 0.287, P = 0.029). In conclusion, patients with HNP have remarkable sleep-related complaints. Especially the presence of carotid body tumors appears to be associated with increased daytime somnolence.

Extradigital glomus tumor as a cause of chronic perianal pain.

Glomus tumor is a rare perivascular benign tumor arising from the Sucquet-Hoyer canal of the normal glomus body, most commonly in the digital areas. We report a serving soldier with such a tumor in an atypical site, the perianal region, presenting with episodic shooting pain. Total surgical excision was performed. Histopathology revealed a well-circumscribed tumor composed of clusters of monotonous polygonal cells surrounding capillary-sized blood vessels. Tumor cells also showed immunopositivity for smooth muscle antigen and vimentin. Following excision, the patient was completely relieved of pain and there was no recurrence on follow-up for 6 months.

Gastric multicentric glomangioma: a case report of this rare cause of abdominal pain.

BACKGROUND: A glomus tumor is a rare neoplasm derived from glomus cells, specialized cells that surround small blood vessels which are important in regulating peripheral blood flow. Glomangiomas are a subset of glomus tumors. They are usually localized in the skin and subcutaneous tissue. They have also been reported in viscera, most notably in the gastrointestinal tract. CASE REPORT: A case of gastric multicentric glomangioma is described in an 18-year-old male who presented with chronic continuous abdominal pain over a 4-6 month period. Preoperative diagnosis, operative findings, histology, and immunohistochemistry of the tumor are discussed in detail. CONCLUSIONS: Case reports of this rare tumor are important because of the paucity of studies noted in the gastro-intestinal literature as a result of poor identification prior to the advent of modern immunohistochemistry. The significance of accurately diagnosing a gastrointestinal glomangioma is crucial for appropriate treatment.

A clinicopathologic study of glomus tumor of the stomach.

Glomus tumors are benign lesions and originate from modified smooth muscle cells of the glomus body. These tumors are commonly observed in the dermis or subcutis, but only rarely found in the stomach. Here we report a case of a 67-year-old patient in whom glomus tumor of the stomach was diagnosed after 10-day history of melena. We have reviewed the 13 cases selected from literature of gastric glomus tumor in Korean, including our case. The age of onset ranged from 30 to 68 years (median 54 y) with a strong female dominance (10 females and 3 males). The tumors typically presented with gastrointestinal bleeding or ulcerlike symptom, and 6 tumors had mucosal ulceration. The tumor sizes varied from 1.2 to 3.8 cm (median 2.5 cm), and all tumors presented in antrum of stomach. Microscopically, the tumors were well-circumscribed, and composed of uniform, small, round to polygonal cells. Mitotic activity was usually low. The characteristics of gastric glomus tumors in Korean patients are very similar to those of Western populations. Although gastric glomus tumors can be considered more likely benign, a small possibility of malignant behavior cannot be ruled out.

Malignant glomus tumour--a case report.

Glomus tumours are usually benign neoplasms of glomus bodies. Here we report a rare case of malignant glomus tumour of soft tissue. The tumour was 8 cm in size, infiltrating deep tissue, with nuclear atypia, high mitotic activity (6/HPF), atypical mitosis and showed recurrence, thus fulfilling all the criteria of malignancy.

[Expression and significance of substance P, neurofilament-H in glomus tumors with chronic pain].

OBJECTIVE: To observe the expression and distribution of substance P (SP), neurofilament-H (NFH) in glomus tumors with chronic pain, and to discuss the process of chronic pain and the relationship with pain degree. METHODS: Twenty-seven patients diagnosed as glomus tumor with chronic pain were enrolled as case group, and divided into light pain symptomatic group (LPSG) (n = 12) and severe pain symptomatic group (SPSG) (n = 15) according to clinical manifestations. Control group (CG) were enrolled by 30 patients with amputated extremities or hands after trauma. Immunohistochemical methods were used to determine the expression of SP, NFH which were detected quantitatively by computer graph analysis system too. RESULTS: The positive expression and distribution of SP, NFH existed in all the three groups and SPSG expression level was the highest [Grayscale Value(SP) (143.3 +/- 7.5), Grayscale Value(NFH) (167.7 +/- 4.4)], LPSG followed [Grayscale Value(SP) (156.2 +/- 8.2), Grayscale Value(NFH) (194.8 +/- 4.0)], control group was the third [Grayscale Value(SP) (208.2 +/- 16.6), Grayscale Value(NFH) (225.1 +/- 8.3)]; The difference of expression level among three groups was significant [SPSG vs LPSG (P(SP) = 0.002, P(NFH) < 0.0001), SPSG vs CG (P(SP) < 0.0001, P(NFH) < 0.0001), LPSG vs CG (P(SP) < 0.0001, P(NFH) < 0.0001)]. The findings of Pearson product-moment correlation analysis between quantitative grayscale value of SP, NFH respectively and pain score in all the patients with glomus tumor showed linear negative correlation (r(SP) = -0.8974, P(SP) = 0.000001; r(NFH) = -0.6545, P(NFH) = 0.000212). CONCLUSION: SP is the mainly afferent pain transmitter in the process of chronic pain in glomus tumor, and NFH plays an important role in pain-transmitted activities.

Glomangioma at the cervical spine as an extradural dumbbell-shaped tumor--a case report.

A case of glomangioma located at an unusual site, the extradural space of the cervical spine, is presented. To our knowledge, this is the first case that has been identified at this location. Radiographic features of computed tomography (CT) and magnetic resonance (MR) image were collected as a dumbbell-shaped tumor at epidural space of the cervical spine. These features correspond with the histological findings.

Tumor glômico / Glomic tumors

umores glômicos são neoplasias dolorosas, originados de shunts arteriovenosos, podendo ocorrer em várias partes do corpo. O objetivo deste artigo é apresentar possíveis manifestações clínicas, achados histopatológicos e tratamento desta patologia

Supraclavicular glomus tumor, 20 year history of undiagnosed shoulder pain: a case report.

A long-standing case of severe dysesthesia due to a supraclavicular glomus tumor is presented. Chronic pain caused by a subcutaneous glomus (non-chemodectoma) tumor is rare and usually misdiagnosed. The supraclavicular location, presentation, and coincidence of trauma history are unique in this case. A 62-year-old male complained of 20 years of intractable right shoulder and supraclavicular region pain, which started 6 months after a fall. The pain was unrelieved by repeated and extensive physical therapy, chiropractic manipulation, local steroid injections, and two shoulder operations. The cause of the condition remained undiagnosed and obscure. Local surgical exploration revealed a subcutaneous grayish mass with pathologically proven glomus tumor. Immediate alleviation of the pain and tenderness followed complete resection of the mass. The patient remained free of pain at a 2-year follow-up. Subcutaneous glomus (non-chemodectoma) tumors can occur in unusual sites, and should be considered in chronic regional pain syndromes. Immediate cure is generally achieved by local resection. Pertinent literature is reviewed.

Tumor glômico: estudo retrospectivo clínico-histopatológico de 44 casos / Glomus tumor: a clinical histopathological retrospective study of 44 cases

FUNDAMENTO - O tumor glômico apresenta-se como lesäo de relativa raridade e de escassa informaçäo nacional. OBJETIVO - Caracterizar seu comportamento epidemiológico, clínico e histopatológico no Brasil. MÉTODO - Foram analisados 44 pacientes, tratados entre 1973 e 1995 no Rio de Janeiro e Niterói, mediante estudo retrospectivo multicêntrico. RESULTADOS - Em pacientes do sexo feminino, os tumores preferiram os quirodáctilos em 70,9 porcento e, nos do sexo masculino, em 38,4 porcento. A tríade "dor paroxística, sensibilidade local e hipersensibilidade à alteraçäo de temperatura"näo foi relatada. Recidivas ocorreram em 12 porcento dos casos, 83,3 porcento deles seis meses depois da cirurgia. A cápsula tumoral esteve presente em um terço das preparaçöes. Tumores múltiplos näo foram observados. CONCLUSÖES - A sintomatologia dolorosa pode ser discreta ou mesmo näo estar presente. A distribuiçäo topográfica do tumor foi diferente entre os sexos. O curto acompanhamento e as recidivas tardias sugerem a necessidade de seguimento pós-operatório mais longo