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1.
Int. arch. otorhinolaryngol. (Impr.) ; 27(3): 407-411, Jul.-Sept. 2023. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1514248

ABSTRACT

Abstract Introduction The surgical management of jugulotympanic paragangliomas has remained challenging. They are the second most common type of tumor of the temporal bone after acoustic neuroma. It has been noticed by the authors that the jugulotympanic paragangliomas may have extensions to the epitympanum and aditus in addition to the mesotympanum and hypotympanum. The modified technique could be an alternative to the conventional facial recess technique for complete removal of the tumors. Objective To highlight the modified surgical technique for the surgical treatment of jugulotympanic paragangliomas. Methods This is a retrospective review of 34 cases of jugulotympanic paragangliomas treated in a tertiary center with respect to clinical presentation, diagnosis, and surgical treatment. Tinnitus and hearing loss were predominant symptoms. A modified technique of postauricular transcanal posterior tympanectomy with extended hypo-tympanic access was performed in 29 patients. Only two cases were operated with a classical transcanal approach. A canal wall down the mastoidectomy was required in three patients. Results The patients operated on with the modified technique had complete excision evident by absence of any lesion in computed tomography and the disappearance of tinnitus. However, two patients had recurrence of symptoms and presence of tumor in the follow-up period. These two patients underwent revision surgery. None of the patients required postoperative radiotherapy or gamma knife therapy. Conclusions Jugulotympanic paragangliomas can be effectively managed with the modified technique to ensure complete removal of the lesions. This technique has not been reported earlier in the literature.

2.
Rev. colomb. cir ; 38(2): 357-362, 20230303. fig
Article in Spanish | LILACS | ID: biblio-1425216

ABSTRACT

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


Subject(s)
Humans , Tracheal Neoplasms , Immunohistochemistry , Glomus Tumor , Trachea , Biopsy , Airway Management
4.
Article | IMSEAR | ID: sea-221309

ABSTRACT

Introduction: Glomus tumours are rare, benign tumours of vascular origin, arising from the glomus body. Glomus tumours are neoplasms of mesenchyme derived from glomus bodies. Glomus tumours present as a purple or pink vascular lesion that can be confused with a vascular neoplasm such as hemangioma. In this case we are reporting a rare presentation of glomus tumour over lower lid. A 10 year old female child presented with lesion over left lower Case Report: lid since 3 months which was painless and progressive. On examination a 0.3x0.3 cm subcutaneous pink non encapsulated mass is seen over medial canthus of left eye. Mass was excised and subjected to histopathological examination which confirmed the diagnosis of glomus cell tumour(glomangioma). Postoperative period was uneventful with no recurrence for last 6months. Conclusion: All excised eyelid lesions should undergo detailed Histopathological evaluation and glomus tumour should be considered as one of the differential diagnosis in patients presenting with eyelid mass

5.
Indian J Pathol Microbiol ; 2022 Mar; 65(1): 164-166
Article | IMSEAR | ID: sea-223193

ABSTRACT

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.

6.
Journal of Chinese Physician ; (12): 1464-1467, 2022.
Article in Chinese | WPRIM | ID: wpr-956323

ABSTRACT

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.

7.
Autops. Case Rep ; 12: e2021406, 2022. graf
Article in English | LILACS-Express | LILACS | ID: biblio-1403562

ABSTRACT

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.

8.
Gac. méd. boliv ; 45(1)2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1385011

ABSTRACT

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.

9.
An. bras. dermatol ; 96(6): 765-767, Nov.-Dec. 2021. graf
Article in English | LILACS | ID: biblio-1355638

ABSTRACT

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.


Subject(s)
Humans , Brain Neoplasms , Neoplasms/diagnostic imaging , Pain , Extremities
10.
Rev. colomb. gastroenterol ; 36(4): 463-472, oct.-dic. 2021. tab, graf
Article in English, Spanish | LILACS | ID: biblio-1360970

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Pancreas , Carcinoma , Glomus Tumor , Cystadenoma , Leiomyoma , Neoplasms , Patients , General Surgery , Medical Records , Diagnosis, Differential , Gastrectomy , Literature
11.
Arq. bras. neurocir ; 40(2): 200-205, 15/06/2021.
Article in English | LILACS | ID: biblio-1362264

ABSTRACT

Glomus jugular tumors, also known as paragangliomas (PGLs), are rare and related to several clinical syndromes described. These are located in the carotid body, the jugular glomus, the tympanic glomus and the vagal glomus. The symptoms are directly related to the site of involvement and infiltration. These lesions have slow growth, are generally benign and hypervascularized, have a peak incidence between the age of 30 to 50 years old; however, when associated with hereditary syndromes, they tend to occur a decade earlier. Several familial hereditary syndromes are associated with PGLs, including Von Hippel- Lindau disease (VHL) in< 10% of the cases. The diagnosis and staging of PGLs are based on imaging and functional exams (bone window computed tomography [CT] with a "ground moth" pattern and magnetic resonance imaging (MRI) with a "salt and pepper" pattern). The cerebral angiography is a prerequisite in patients with extremely vascularized lesions, whose preoperative embolization is necessary. The histopathological finding of cell clusters called "Zellballen" is a characteristic of PGLs. Regarding the jugular foramen, the combination of two or three surgical approaches may be necessary: (1) lateral group, approaches through themastoid; (2) posterior group, through the retrosigmoid access and its variants; and (3) anterior group, centered on the tympanic and petrous bone. In the present paper, we report a case of PGL of the jugular foramen operated on a young female patientwho underwent a surgery with a diagnosis ofVonHippel-Lindau Disease (VHL) at the Neurosurgery Service of the Hospital Heliópolis, São Paulo, state of São Paulo, Brazil in 2018, by the lateral and posterior combined route.


Subject(s)
Humans , Female , Adult , Paraganglioma/surgery , Paraganglioma/diagnostic imaging , Skull Base Neoplasms/surgery , Jugular Foramina/surgery , Cerebral Angiography/methods , Embolization, Therapeutic/methods , Jugular Foramina/abnormalities , Hearing Loss, Sensorineural/etiology , von Hippel-Lindau Disease/complications
12.
Rev. colomb. cir ; 36(2): 257-267, 20210000. fig
Article in Spanish | LILACS | ID: biblio-1223912

ABSTRACT

Introducción. El carcinoma del glomus carotideo es infrecuente y se presenta en el 6 % de los paragangliomas carotideos. Suele haber dificultad en su diagnóstico preoperatorio y no se diferencia fácilmente de su contraparte benigna, lo que puede conllevar a complicaciones quirúrgicas. Este trabajo describe la experiencia en su abordaje clínico y quirúrgico en una serie de casos, además de una revisión de la literatura profundizando en su diagnóstico, abordaje terapéutico, sobrevida y mortalidad. Métodos. De los pacientes operados por glomus carotideos durante un periodo de 20 años, se identificaron aquellos llevados a cirugía por carcinoma. Se realizó una búsqueda bibliográfica extensa en PubMed, haciendo énfasis en diagnóstico y tratamiento. Resultados. De un total de 139 pacientes sometidos a cirugía de resección de tumor del cuerpo carotideo, tres pacientes (2,2 %) presentaron carcinoma. Dos tenían metástasis cervicales y uno metástasis hepáticas. Uno presentaba glomus bilateral asociado a trastorno genético de origen familiar. Se realizó cirugía y adyuvancia en todos los casos. Discusión. El diagnóstico diferencial preoperatorio entre glomus benigno y maligno es difícil, por lo cual deben buscarse hallazgos clínicos o factores de riesgo que puedan sugerir malignidad. Las imágenes pocas veces muestran características claras de invasión vascular. Siempre que sea posible, en el tratamiento del cáncer del glomus carotideo, debe realizarse resección quirúrgica, acompañada de un vaciamiento funcional de cuello, dejando la radioterapia, la quimioterapia y la hormonoterapia como terapias adyuvantes


Introduction. Carcinoma of the carotid glomus is rare and occurs in 6% of carotid paragangliomas. There is often difficulty in its preoperative diagnosis, and it is not easily differentiated from its benign counterpart, which can lead to surgical complications. This study describes the experience in its clinical and surgical approach in a series of cases, as well as a review of the literature of its diagnosis, therapeutic approach, survival, and mortality.Methods. Of the patients operated for carotid glomus over a 20-year period, those undergoing surgery for carcinoma were identified. An extensive literature search was conducted in PubMed, with an emphasis on diagnosis and treatment.Results. Out of a total of 139 patients who underwent carotid body tumor resection surgery, three patients (2.2%) had carcinoma. Two had cervical metastases and one had liver metastases. One had bilateral glomus associated with a genetic disorder of familial origin. Surgery and adjuvant surgery were performed in all cases.Discussion. The preoperative differential diagnosis between benign and malignant glomus is difficult, for which clinical findings or risk factors that may suggest malignancy should be sought. Images rarely show clear features of vascular invasion. Whenever possible in the treatment of carotid glomus cancer, surgical resection should be performed, accompanied by a functional neck dissection, leaving radiotherapy, chemotherapy, and hormone therapy as adjuvant therapies


Subject(s)
Humans , Carotid Body , Paraganglioma , Neoplasms
13.
Chinese Journal of Urology ; (12): 944-945, 2021.
Article in Chinese | WPRIM | ID: wpr-911157

ABSTRACT

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.

14.
Rev. otorrinolaringol. cir. cabeza cuello ; 80(2): 166-171, jun. 2020. graf
Article in Spanish | LILACS | ID: biblio-1115831

ABSTRACT

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.


Subject(s)
Humans , Female , Adolescent , Mouth Neoplasms/pathology , Glomus Tumor/pathology , Mouth Neoplasms/diagnosis , Glomus Tumor/diagnosis , Mouth Mucosa/pathology
15.
Acta méd. costarric ; 62(1): 43-46, ene.-mar. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1088535

ABSTRACT

Resumen Los tumores de glomus yugulo-timpánico o paragangliomas son originados de células de la cresta neural y representan un número bajo de los tumores de cabeza y cuello (0,5 %). Su crecimiento lento y síntomas progresivos provocan que se retarde el diagnóstico. Conocer la clínica que frecuentemente cursa con pérdida auditiva unilateral, tinnitus pulsátil y afectación de algún par craneal, puede ayudar a sospechar el diagnóstico de glomus. Se mencionan diferentes tipos de métodos diagnósticos, así como nuevas opciones de tratamiento. Dada la localización y su importante vascularización, hay casos en los que la cirugía abierta es muy riesgosa y se buscan procedimientos terapéuticos que disminuyan las complicaciones y morbilidades en estos pacientes, tanto como procedimientos endovasculares. En el artículo se discute el caso de una femenina de 31 años con clínica de inicio insidioso con hipoacusia izquierda progresiva, tinnitus pulsátil izquierdo y disfonía. En 2013, se realiza resonancia magnética nuclear donde se observa proceso infiltrativo tumoral sugestivo de paraganglioma. Se hace embolización endovascular con onyx de glomus yugulotimpánico en cuatro ocasiones, con seguimiento cada 6 meses por no ser candidata a otro tipo de tratamiento, debido al tamaño y localización de la masa y su condición no resecable.


Abstract Jugulo-tympanic glomus tumors or paragangliomas originate from neural crest cells and represent a low number of head and neck tumors (0.5%). Its slow growth and progressive symptoms cause the diagnosis to be delayed. The knowledge of the clinic that frequently involves unilateral hearing loss, pulsatile tinnitus and cranial nerve involvement can help to suspect the diagnosis. Different types of diagnostic methods are helpfun, as well as new treatment options have been proposed. Given the location and its important vascularization, there are cases in which open surgery is highly risky and alternative therapeutic procedures trying to reduce complications and morbidities in these patients need to be considered, as well as endovascular procedures. This article discusses the case of a 31-year-old female with an insidious onset of progressive left hearing loss, left pulsatile tinnitus and dysphonia. In 2013, nuclear magnetic resonance imaging was performed and a tumor infiltrative process suggesting paraganglioma was observed. Endovascular embolization was performed with onyx of jugulotympanic glomus on four occasions, with follow-up every 6 months, due to the size and location of the mass and its unresectable condition the tumor was considered non surgically resectable.


Subject(s)
Humans , Female , Paraganglioma , Glomus Tympanicum Tumor , Glomus Jugulare , Head and Neck Neoplasms
16.
Surg. cosmet. dermatol. (Impr.) ; 12(4 S2): 228-231, fev.-nov. 2020.
Article in Portuguese | LILACS-Express | LILACS | ID: biblio-1368000

ABSTRACT

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.

17.
Chinese Journal of Pathology ; (12): 22-27, 2020.
Article in Chinese | WPRIM | ID: wpr-798947

ABSTRACT

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.

18.
Chinese Journal of Reparative and Reconstructive Surgery ; (12): 1294-1297, 2020.
Article in Chinese | WPRIM | ID: wpr-856243

ABSTRACT

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.

19.
Article | IMSEAR | ID: sea-209130

ABSTRACT

Glomus tumors of the subungual region are a group of benign hamartomas due to proliferation of glomus cells. The lesionpresents as a brownish purple lesion in the nail bed. Classical triad suggestive of the disease consists of severe pain, localtenderness, and sensitivity to cold. Diagnostic tests include Love’s pin test, Hildreth’s test, and transillumination test. Completesurgical excision by either the transungual or periungual approach usually provides symptomatic relief with little chance ofrecurrence. Periungual approach is associated with less complications.

20.
Braz. dent. j ; 30(2): 185-190, Mar.-Apr. 2019. tab, graf
Article in English | LILACS | ID: biblio-1001429

ABSTRACT

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.


Subject(s)
Humans , Adult , Middle Aged , Glomus Tumor , Subcutaneous Tissue , Mouth
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