Presentación atípica de tumor glómico en vía aérea: reporte de caso y revisión de literatura / Atypical presentation of glomus tumor in the airway: Case report and literature review

Introducción. Los tumores glómicos provienen de los cuerpos glómicos, que son estructuras con función de termorregulación y se encuentran distribuidas por todo el cuerpo humano, principalmente a nivel distal de las extremidades, donde es común encontrar lesiones características, aunque hay reportes de casos que se presentaron como neoformación en localizaciones más inusuales. Su etiología aun es desconocida. No se sospechan en muchos pacientes y el diagnostico se realiza de manera incidental, por estudios imagenológicos o anatomopatológicos. Caso clínico. Paciente femenina de 66 años, con presencia de tumor glómico en vía aérea, diagnosticado por histopatología e inmunohistoquímica, que fue sometido a resección quirúrgica, con buena evolución posterior. Discusión. Esta presentación atípica de tumor glómico en vía aérea se manifiesta principalmente con síntomas y signos relacionados con obstrucción de la vía aérea. El manejo oportuno es primordial y el diagnóstico definitivo es por histopatología e inmunohistoquímica, donde se observan las características de las células glómicas, estructuras vasculares, músculo liso y la positividad en la inmunotinción de marcadores como actina del músculo liso, CD34, y actina específica del músculo, entre otras. Conclusión. Los tumores glómicos son neoformaciones benignas raras, con presentación más común en zonas distales. Su aparición depende de factores intrínsecos y extrínsecos de los pacientes. Su tasa de recidiva es muy baja en comparación de otros tumores

Introduction. Glomus tumors originate from glomus bodies, which are structures with thermoregulatory function and are distributed throughout the human body, mainly at the distal level of the extremities where it is common to find these characteristic lesions. Although, there are case reports of neoformation presentations with more unusual locations. Their etiology is still unknown. Many times when there is evidence of lesions in atypical areas they are not suspected in many patients and the diagnosis is made incidentally by imaging and/or anatomopathological studies. Clinical case. A 66-year-old female patient with the presence of a glomus tumor in the airway diagnosed by histopathology and immunohistochemistry, underwent surgical resection and presented good evolution after surgery. Discussion. This atypical presentation of glomus tumor in the airway presents mainly with symptoms and signs related to airway obstruction. Timely management is paramount in these patients, and the definitive diagnosis is by histopathology and immunohistochemistry where the presence of the characteristics of glomus cells, vascular structures, smooth muscle and immunostaining positivity towards some markers such as smooth muscle actin, CD34, muscle specific actin, among others, are seen. Conclusion. This type of tumors are rare benign neoformations, with common presentations in distal areas. Their appearance depends on intrinsic and extrinsic factors of the patients and their recurrence rate is very low compared to other tumors

Glomus tumor of the crissum: A case report and review of literature

Glomus tumor is a rare mesenchymal neoplasm arising from the modified smooth muscle cells of the glomus body. Primary crissum glomus tumor is extremely rare without any published in the literature. In this article, we report the first case of primary crissum glomus tumor in an 80-year-old man with recurrent anal pain for 8 years, increased pain for 1 year. Rectal MRI for inflammatory lesions (sinus tract). Microscopic examination showed the tumor cells were arranged in sheets and nests, surrounding blood vessels and nerve bundles. At high magnification, the neoplastic cells show regular round shape with light eosinophilic and translucent cytoplasm. The cell boundary is clear, the nucleus is round and located in the center. The stroma of the tumor shows hyaline degeneration. Immunohistochemically, the tumor cells were positive for smooth muscle actin, h-caldesmon, Calponin, synaptophysin, Collagen IV and CD34, but completely negative for HMB45, S100, EMA, desmin, CgA and CD56. The histologic features and immunohistochemical profile supported a diagnosis of primary crissum glomus tumor. The patient was asymptomatic and disease free after the procedure.

Glomus tumor located in trachea: two cases reports / 中国医师杂志

Objective:To investigate the clinicopathological features and computed tomography (CT) findings of tracheal glomus tumor (GT) in order to improve the understanding and diagnosis of tracheal GT.Methods:The clinical and CT imaging data of 2 patients with tracheal GT diagnosed in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively. The image characteristics based on previous reports were analyzed.Results:The clinical manifestations of trachea GT were dyspnea, chest tightness, hemoptysis, etc., which were easy to be misdiagnosed. The CT manifestations were spherical or nodular protrusions in the trachea cavity, with uneven edges, which can be lobulated. Cystic changes can be seen in the focus. After enhancement, it showed progressive filling and obvious enhancement, without deep infiltration and distant metastasis.Conclusions:Chest CT can accurately localize tracheal GT, provide its morphological size, blood supply, growth characteristics and other characteristics, accurately display the overall morphology of the lesion, and provide some help for the development of the surgical plan, and its definitive diagnosis still relies on pathological examination.

Juxtaglomerular cell tumor: report of a case with unusual presentation

ABSTRACT Juxtaglomerular cell tumor is a benign, renin-secreting neoplasm. The tumor arises from the juxtaglomerular apparatus cells of the kidney. Because the tumor is hormonally active, patients usually suffer from hypokalemia, hyperaldosteronism, and hypertension. Herein, we describe a case of a 19-year-old Asian female with a somewhat unusual presentation. A 19-year-old Asian female presented with upper extremity weakness, numbness, and tingling. On physical examination, the only notable finding was hypertension. Extensive workup revealed elevated aldosterone level and plasma renin activity. CT scan of the abdomen revealed a 2.2 cm mass in the lower pole of the left kidney. The mass was resected by partial nephrectomy. On microscopic evaluation, the tumor had glomoid appearance with sheets of uniform, round to polygonal cells with clear to eosinophilic cytoplasm. Immunohistochemical stains showed the tumor cells to be positive for CD117, CD34 and CD10 and negative for ER, PR, CK7, PAX-8, pan-cytokeratin, EMA, S100, Melan-A, HMB45, SMA and CAIX. Diagnosis of Juxtaglomerular cell tumor was rendered. This case highlights the importance of a regular physical exam and a high index of suspicion in patients presenting with unusual complaints.

Glomus Carotideo Izquierdo

Resumen Los paragangliomas (cuerpo carotideo) son tumores benignos de alta vascularización ubicados en la región de los quimiorreceptores del cuerpo carotideo. Respecto a su localización, el glomus carotídeo, es del 0,01% en relación a los otros tumores de tipo paranganglioma, se observa con mayor incidencia en habitantes de lugares de altitud, más frecuente en sexo femenino. Clínicamente se presenta como una tumoración en región cervical de progresivo crecimiento, indoloro y con frémito. Dentro de los exámenes de gabinete auxiliares para diagnóstico, estan: la Ecografia Doppler, la Tomografía Axial Computarizada, la Resonancia Magnética y la Arteriografía. La técnica quirúrgica más frecuente para abordar este tumor es la disección subadventicial. Presentamos el caso clínico de una mujer de 57 años con tumor cervical, que fue intervenida quirúrgicamente, previo exámenes de laboratorio y gabinete, para la resección total del tumor y posterior estudio anatomo-patológico que reportó: paraganglioma de cuerpo carotideo.

Abstract Paragangliomas (carotid body) are benign tumors with high vascularization located in the region of the carotid body chemoreceptors. Regarding its location, the carotid glomus is 0.01% in relation to other paranganglioma type tumors, it is observed with higher incidence in high altitude populations, more frequent in females. Clinically it presents as a tumor in the cervical region of progressive growth, painless and with fremitus. Among the auxiliary tests for diagnosis are: Doppler Ultrasound, Computerized Axial Tomography, Magnetic Resonance Imaging and Arteriography. The most frequent surgical technique to address this tumor is subadventitial dissection. We present the clinical case of a 57-year-old woman with a cervical mass, who underwent surgery, after preliminary laboratory tests, for total resection with subsequent anatomo-pathological study that reported: paraganglioma of the carotid body.

Extradigital glomus tumor: dermoscopic description and histopathological correlation

Abstract Glomus tumors are rare benign neoplasms arising from the neuromyoarterial structure called glomus body. They present as angiomatous papules, soft and painful, especially to cold and pressure. In general, they are solitary and affect the extremities, located mainly the subungual bed. Extradigital lesions are rare and can constitute a diagnostic challenge. This is the report of a patient with an extradigital lesion on the left arm, and its dermoscopic aspects, including angiomatous lagoons circumscribed by a pale halo, a structure not previously described in the two reports of extradigital glomus tumor with dermoscopic features, found in the literature.

Tumores gástricos peculiares: tumor glómico, schwannoma, leiomioma y cistoadenoma en el páncreas ectópico. Serie de casos y revisión de la literatura / Unusual gastric tumors: Glomus tumor, schwannoma, leiomyoma, and cystadenoma in ectopic pancreas. A case report and literature review

Resumen Introducción: la patología gástrica es excepcionalmente benigna, se registra a nivel mundial un porcentaje de aparición de las mismas del 0,005 %-4 %. Además de lo anterior, suele plantearse como primer diagnóstico diferencial un carcinoma gástrico, por lo que la mayoría de los pacientes termina en un procedimiento quirúrgico mayor. El objetivo de este estudio fue describir una serie de casos registrados en una institución de alta complejidad, así como la revisión de la literatura al respecto del tema. Pacientes y métodos: se realizó la revisión de las bases de datos del servicio de cirugía general de la Clínica Universitaria Colombia y se encontraron 4 tumores gástricos de presentación inusual, de los cuales se hizo revisión de la historia clínica y las imágenes disponibles (diagnósticas y de las piezas de cirugía). Resultados: en la revisión de la base de datos, se encontraron 4 casos, 2 de sexo femenino y 2 de sexo masculino, con una media de edad de 51 años (26-75 años). El 100 % de los pacientes tenía una lesión en la región antral y solo uno de ellos tenía una segunda lesión a nivel prepilórico. La totalidad de los pacientes fue llevada a gastrectomía subtotal con reconstrucción en Y de Roux por abordaje laparoscópico y la media de estancia hospitalaria fue de 3,5 días (2-6 días). Conclusión: para determinar la mejor opción de tratamiento en general en pacientes con lesiones gástricas siempre hay que considerar tanto las características endoscópicas y endosonográficas como las histológicas.

Abstract Introduction: Gastric pathology is exceptionally benign with a percentage of appearance between 0.005% - 4% worldwide. Moreover, gastric carcinoma is often suggested as the first differential diagnosis and for that reason, the outcome for a vast majority of admitted patients is a major surgical procedure. This study aims to describe a series of cases recorded in a high complexity medical institution and to carry out a literature review related to that subject. Patients and Methods: Databases from the general surgery service of the Clínica Universitaria Colombia were reviewed, and four unusual gastric tumors were found. A review of the related medical records and available images (diagnostic images and surgical elements) was performed. Results: After the database review, four cases were found: two female cases and two male cases, with a mean age of 51 years (26-75 years). All the patients had lesions in the antrum area and only one of them has an additional lesion in the prepyloric region. All patients were treated with a laparoscopic subtotal gastrectomy with Roux-en-Y reconstruction and the mean length of hospital stay was 3.5 days (2-6 days). Conclusion: It is required to consider the endoscopic, endosonographic, and histological examinations and their features to determine the best treatment for patients with gastric lesions.

A case report of glomus tumor of the kidney / 中华泌尿外科杂志

Glomus tumor of kidney is very rare in clinic. One case with glomus tumor of the kidney was reported. CT demonstrated a well-defined mass located at the lower pole of the left kidney. The mass densely enhanced in arterial phase and constantly enhanced in venous and portal phase. Laparoscopic enucleation of left renal tumor was successfully performed. The pathological diagnosis was glomus tumor of the kidney. Recurrence and metastasis were not seen after 35 months follow-up.

Tumor glómico en mucosa bucal: reporte de caso / Tumor in oral mucosa: case report

El tumor glómico es una neoplasia vascular originada de las células del músculo liso del componente neuromioarterial, responsable del control del flujo sanguíneo microvascular. Representa el 1,6% de todos los tumores de tejidos blandos, localizándose principalmente en la zona subungüeal y clínicamente muy doloroso. Su diagnóstico puede ser tardío debido a su pequeño tamaño, manifestaciones clínicas inespecíficas y localizaciones anatómicas inusuales. El tratamiento principalmente es quirúrgico, el cual es curativo, permitiendo además la confirmación histopatológica. Se presenta el reporte de caso de una paciente de 13 años de edad con un tumor glómico de localización inusual en mucosa bucal.

The glomus tumor is a vascular neoplasm originating from smooth muscle cells of neuromyoarterial component, responsible for the control of microvascular blood flow. It represents 1.6% of all soft tissue tumors, being located mainly in the subungual area and clinically very painful. Diagnosis may be delayed because of their small size, nonspecific clinical manifestations and unusual anatomical locations. Treatment is primarily surgical, which is healing, also allowing the histopathologic confirmation. The case report of a 13-year-old patient with an unusual-located glomus tumor in the oral mucosa is presented.

Tumor glômico múltiplo em paciente com neurofibromatose tipo 1: relato de caso e revisão de literatura / Multiple glomus tumor in a patient with neurofibromatosis type 1: a case report and literature review

Neurofibromatose tipo I (NF1) é uma doença autossômica dominante, com incidência de 1/2.500-3.000 nascimentos e prevalência de aproximadamente 1/4.000-5.000 indivíduos; é causada por mutações genéticas no gene NF1, que afetam tecidos neurais e cutâneos. Tumor glômico é uma neoplasia benigna originada do glomo, uma estrutura neuromioatrial da pele presente nas pontas dos dedos e envolvida na termorregulação. São considerados historicamente tumores isolados esporádicos, porém existem estudos que comprovam sua relação com a neurofibromatose tipo I. Pacientes com neurofibromatose tipo 1 devem ser investigados. O relato de caso fornece suporte adicional à noção de que NF1 tem um risco associado a múltiplos tumores glômicos.

Neurofibromatosis type I (NF1) is an autosomal dominant disease, with an incidence of 1/2,500-3,000 births and a prevalence of approximately 1/4,000-5,000 individuals. Genetic mutations in the NF1 gene cause it, affecting neural and skin tissues. Glomus tumor is a benign neoplasm originating from the glomus, a neuromyoatrial structure of the skin present at the fingertips and involved in thermoregulation. The literature historically considered these tumors isolated and sporadic, but some studies have proved a relationship with neurofibromatosis type I. Thus, patients with neurofibromatosis type 1 should be investigated. The case report provides additional support for the notion that NF1 has a risk associated with multiple glomus tumors.

Gastrointestinal glomus tumors: a clinicopathological analysis of fifteen cases / 中华病理学杂志

Objective@#To investigate the clinicopathological features, diagnosis and differential diagnosis of gastrointestinal glomus tumors (GIGT).@*Methods@#Totally 15 cases of GIGT were collected at the First Affiliated Hospital, Zhengzhou University, from January 2011 to June 2018. The clinicopathological features, immunophenotype, BRAF V600E mutation and prognosis were retrospectively analyzed.@*Results@#The 15 patients′ age ranged from 37 to 59 years(median 49 years, mean 50 years). Eleven patients presented with intermittent abdominal pain and distention, three showed antral space-occupying lesions at physical examination, and one had abdominal pain accompanied by fecal blood. Fourteen tumors were located in the stomach, and one was in the ileum. Imaging showed the gastric glomus tumors were located in the submucosal layer with obvious enhancement in the arterial phase, and the ileum glomus tumor involved the whole layer of intestinal wall causing luminal obstruction. The maximum diameters of the tumors ranged from 1.5 to 3.0 cm (mean 2.3 cm). Grossly, the gastric glomus tumors were solid. Microscopically, the gastric glomus tumors were mostly located in the muscularispropria layer and were vascular. The tumor boundary was distinct but without capsule formation. The tumor cells were round or oval, and showed perivascular hemangiopericytoma-like or solid nest-like structures. The tumor cells were mildly pleomorphic, with rare mitosis and no necrosis. Two tumors had focal calcification, two showed mucosal invasion, two showed vascular invasion and five showed perineural invasion. The ileum glomus tumor was cellular, with prominent cellular atypia, and the mitotic count in hot spots was about 5-6/HPF. Immunohistochemistry showed that SMA and collage Ⅳ were strongly expressed in all the tumor cells; caldesmon and calponin were moderately expressed in some regions, and syn was weakly expressed in 12 cases. The Ki-67 proliferation index in the gastric glomus tumors ranged from 1% to 30% (mean 6%); and that in the ileum glomus tumor was about 70%. BRAF V600E mutations were not detected in any of 15 GIGTs. All patients did not receive radiotherapy or chemotherapy post operatively. Thirteen patients were followed up by telephone for 18-90 months (mean 42 months). Twelve patients with gastric glomus tumors survived without recurrence and metastasis, and the patient with ileum glomus tumor had liver metastasis 15 months after operation.@*Conclusions@#Glomus tumors is a rare mesenchymal tumor of the gastrointestinal tract. It should be differentiated from gastrointestinal stromal tumors, neuroendocrine tumor, leiomyoma, solitary fibrous tumor and paraganglioma. Most GIGTs are benign and have good prognosis. More experience is needed to understand the biologic behavior and prognostication of GIGTs.

A distal curved incision approach of lunula for treatment of subungual glomus tumor in nail root / 中国修复重建外科杂志

Obejective: To investigate the feasibility and effectiveness of the distal curved incision approach of lunula in treating subungual glomus tumor in nail root. Methods: Between March 2017 and October 2019, 16 patients (16 fingers) with subungual glomus tumor in nail root were treated. There were 2 males and 14 females with an average age of 35.3 years (range, 21-67 years). The disease duration ranged from 5 months to 17 years, with a median duration of 15 months. There were 6 cases of thumb, 3 cases of index finger, 2 cases of middle finger, 4 cases of ring finger, and 1 case of little finger. All of them showed typical "triad syndromes", and the cold sensitivity test and Love test were positive before operation. Each patient removed the nail, the tumor was exposed under the nail bed via the distal curved incision approach of lunula. After the tumor was removed completely, the nail bed was reducted in situ without suturing. The self-made nail template was pressurized and fixed on the surface of the nail bed with suture. The conditions of nail bed healing, pain, and growth of nail were observed after operation. Results: All wounds of the nail bed with laceration healed by first intention. Postoperative pathological results confirmed the diagnosis of subungual glomus tumor in all the patients. All cases were followed up 3-26 months with an average of 15 months. The pain symptom disappeared and no obvious pain occurred during dressing change in all cases; and the cold sensitivity test and Love test were negative. The nails recovered smoothly with satisfactory appearance, and no obvious complications such as longitudinal ridge deformities or recurrences was observed. Conclusion: The treatment of subungual glomus tumor in nail root via the distal curved incision approach is an effective method, which can easily operated, resect the tumor completely, protect the nail bed effectively, and also has a significant effect on preventing postoperative nail deformity.

Glomus tumor of the oral cavity: report of a rare case and literature review

Abstract Glomus tumor is a benign neoplasm composed of a perivascular proliferation of glomic cells that resembles the normal glomus body. Usually, it appears as a solitary, symptomatic small blue-red nodule, located in the deep dermis or subcutis of upper or lower extremities of young to middle-aged adults. Cases affecting the oral cavity are very rare, with only 23 well-documented cases reported in the English-language literature. Herein, we present a rare case of glomus tumor of the upper lip, and review the literature of cases involving the mouth.

Resumo Tumor glômico é uma neoplasia benigna composta de uma proliferação perivascular de células glômicas que lembram o corpo glômico normal. Usualmente, ele se apresenta como um nódulo pequeno, solitário, sintomático e azul-avermelhado, localizado na derme profunda ou subcutânea de extremidades superiores ou inferiores de adultos jovens e de meia-idade. Casos afetando a cavidade oral são muito raros, com apenas 23 casos bem documentados relatados na literatura de língua Inglesa. A seguir, nós apresentamos um caso raro de tumor glômico do lábio superior, e revisão da literatura dos casos envolvendo a boca.

Glomus tumor in the abdominal wall / Tumor glômico de parede abdominal

ABSTRACT The glomus tumor is a rare benign soft tissue tumor. We report a case of a 45-year-old male patient who presented with a painful mass in the abdominal wall. The patient underwent total tumor resection and the anatomical pathology diagnosis was of glomus tumor. The glomus tumor presents differential diagnosis of carcinoid tumor, hemangiopericytoma and vascular leiomyoma.

RESUMEN El tumor glómico es un tumor raro y benigno de tejidos blandos. Se presenta el caso de un paciente de 45 anos, con el hallazgo de un nódulo doloroso en la pared abdominal. Se realizó una resección completa de la lesión y el diagnóstico por anatomia patológica fue de tumor glómico. El diagnóstico diferencial del tumor glómico se plantea con tumor carcinoide, hemangiopericitoma y leiomioma vascular.

RESUMO O tumor glômico é um tumor raro e benigno de tecidos moles. Relatamos o caso de um paciente do sexo masculino, 45 anos, que apresentava nódulo doloroso na parede abdominal. Foi realizada a ressecção total do tumor; o diagnóstico anatomopatológico foi de tumor glômico. Este apresenta diagnóstico diferencial, com tumor carcinoide, hemangiopericitoma e leiomioma vascular.

An Extradigital Glomus Tumor on the Back, Mimicking a Keloid Scar / 대한피부과학회지

No abstract available.

Clinicopathologic features of glomus tumor of the kidney / 中华病理学杂志

Objective@#To investigate the clinicopathologic and differential diagnostic features of glomus tumor of the kidney.@*Methods@#Four cases of glomus tumor of the kidney were collected from the archives of Peking University Third Hospital, the Second Hospital of Tianjin Medical University, Ningbo Yinzhou Second Hospital and Zhejiang Provincial People′s Hospital between January 2012 to June 2017; the clinical and radiologic features, histomorphology, immunohistochemistry, ultrastucture and prognosis were analyzed and the relevant literature was reviewed.@*Results@#Patients consisted of 2 men and 2 women with ages ranging from 37 years to 66 years (mean 55 years). Three patients had history of hypertensive disease (grade Ⅱ, 3 to 10 years). The tumors measured in maximum diameter from 3.0 cm to 4.0 cm (mean 3.6 cm) and showed gray-white to yellow and tan on cut surface. Macroscopical examinations showed all tumors were circumscribed but non-encapsulated. Histologically, 1 tumor presented as glomus tumor with extensive myxoid change, 1 as cellular and solid pattern glomus tumor, 1 as glomangioma with focal myopericytoma-like pattern and 1 as symplastic glomus tumor with areas resembling myopericytoma. The tumor cells in two cases showed scant cytoplasm and uniform, bland-appearing nuclei without mitoses. In one case, the tumor cells were epithelioid with abundant eosinophilic cytoplasm and relatively well-defined cell borders. There was an increased mitosis of 4/50 HPF; however, no evidence of atypical mitosis or nuclear atypia was noted. In the symplastic glomus tumor the tumor cells showed frequently nuclear pleomorphism without mitoses. By immunohistochemistry, all tumors showed strong and diffuse reactivities to at least 3 of the 4 muscle-associated markers (SMA, h-Caldesmon, MSA and Calponin), 3 tumors strongly and diffusely expressed collagen Ⅳ, 2 expressed CD34 and 1 focally expressed desmin; whereas markers including epithelial, neuroendocrine, nephrogenic, melanoma-associated, STAT6, S-100 protein, CD117 and β-catenin all were negative in all the 4 tumors. Ultrastuctural analysis was done in 2 cases and showed prominent cytoplasmic actin bundles and pericellular basement membrane, and lacking of rhomboid renin crystals in both tumors. The hypertension persisted after surgical resection for all the 3 patients with this medical history. Follow-up information (range: 6-64 months, mean: 44 months)showed that no evidence of local recurrence or distant metastasis was identified in all 4 patients.@*Conclusions@#Glomus tumor rarely occurs in the kidney and usually has a good prognosis. Careful attention to its morphology with the judicious use of immunohistochemistry and ultrastuctural analysis can be helpful for its diagnosis and differential diagnosis.

Clinical and pathological analysis of one case of multiple malignant glomus tumor / 上海交通大学学报（医学版）

Malignant glomus tumor (MGT) is a kind of malignant stromal tumor derived from perivascular glomus. Clinically, glomus tumors are mostly benign, malignant and multiple ones are rarely reported. This study reviewed one case of multiple MGT. The lesions were discovered successively in a 75-year-old woman's breast, ileum, brain and colon. According to the result of histomorphology and immunohistochemistry, the lesions in the four organs were diagnosed as MGT. Suffered from MGT in successive sites, the patient is still alive to date after all of tumors were resected.

Tumor de corpo carotídeo em jovem adolescente com pré-síncope / Carotid body tumor in teenager with pre-syncope

O tumor do corpo carotídeo (TCC) é uma neoplasia rara, mas entre os paragangliomas é o mais freqüente, com incidência de 1: 1.170.000. Desenvolve-se na adventícia do vaso, próximo à bifurcação carotídea, afetando principalmente indivíduos entre a quarta e a quinta décadas de vida. É um tumor de crescimento lento, geralmente benigno e unilateral, e 10% deles são familiares. Em cerca de 5% dos casos, a lesão é bilateral, atingindo 32% se autossômica dominante. Podem ser assintomáticos até se manifestarem como uma massa cervical pulsante, de crescimento lento, indolor, na região lateral do pescoço, próximo ao ângulo da mandíbula. A TCC pode causar rouquidão e dificuldade de deglutição por compressão extrínseca e também síndrome do corpo carotídeo hipersensível (SCCHS). Esta síndrome é conseqüência da hiperestimulação dos barorreceptores, causando hipotensão postural, acompanhada de diaforese, pré-síncope ou síncope. Neste relato descrevemos uma paciente de 17 anos com TCC familiar e SCCHS, diagnosticada e tratada com sucesso, evoluindo sem seqüelas e assintomática. (AU)

Carotid body tumor (CBT) is a rare neoplasm, but among paragangliomas it is the most frequent, with an incidence of 1: 1,170,000. It develops in the adventitia of the vessel, near the carotid bifurcation, affecting mainly individuals between the fourth and fifth decades of life. It is a slow-growing tumor, usually benign and unilateral, and 10% of them are familial. In about 5% of the cases, the lesion is bilateral, reaching 32% if it is autosomal dominant. They may be asymptomatic until they manifest as a slowly growing, painless, pulsatile cervical mass in the lateral region of the neck, near the angle of the mandible. CBT can cause hoarseness and difficulty of swallowing by extrinsic compression and also hypersensitive carotid body syndrome (SCCHS). This syndrome is a consequence of hyperstimulation of the baroreceptors, causing postural hypotension, accompanied by diaphoresis, pre-syncope or syncope. In this case report we describe a 17-year-old patient with familial CBT and SCCHS, diagnosed and treated successfully, evolving without sequelae and asymptomatic. (AU)

Congenital plaque-like glomangioma: report of two cases

Abstract: Glomus tumors are rare hamartomas that originate from the glomus body. These tumors can be divided into solitary and multiple, the latter also known as glomangioma. We report the case of two patients with a rare variety of glomangioma called congenital plaque-like glomangioma. It presents as numerous red to bluish compressible papules, that increase in size in proportion with the weight and height growth of the child. Diagnostic confirmation is with histopathology and the treatment is surgical.