RESUMEN
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.
Asunto(s)
Tumor Glómico/congénito , Tumor Glómico/patología , Neoplasias Cutáneas/congénito , Neoplasias Cutáneas/patología , Adolescente , Adulto , Femenino , Humanos , Enfermedades Raras/congénito , Enfermedades Raras/patología , Telangiectasia/congénito , Telangiectasia/patologíaAsunto(s)
Enfermedades en Gemelos/radioterapia , Tumor Glómico/radioterapia , Enfermedades del Prematuro/radioterapia , Láseres de Colorantes/uso terapéutico , Láseres de Estado Sólido/uso terapéutico , Terapia por Luz de Baja Intensidad/métodos , Neoplasias Cutáneas/radioterapia , Estética , Estudios de Seguimiento , Tumor Glómico/congénito , Humanos , Recién Nacido , Recien Nacido Prematuro , Masculino , Hombro , Neoplasias Cutáneas/congénitoRESUMEN
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.
Asunto(s)
Humanos , Femenino , Adulto , Neoplasias Cutáneas/congénito , Tumor Glómico/congénito , Tumor Glómico/patología , Neoplasias Cutáneas/patología , Telangiectasia/congénito , Telangiectasia/patología , Enfermedades Raras/congénito , Enfermedades Raras/patologíaRESUMEN
Glomovenous malformations are disseminated variants of cutaneous glomus tumors. These malformations are subdivided into regional or localized, disseminated, and congenital plaque-like forms. The congenital plaque-like form is the rarest variant. Most treatment modalities have been disappointing in the treatment of large glomangiomas, leading to high recurrence rates. We report a case of a 34-year-old man with a congenital plaque-like glomangioma on his left arm and forearm treated successfully with sequential pulsed-dye neodymium yttrium aluminum garnet laser.
Asunto(s)
Tumor Glómico , Terapia por Láser/métodos , Neoplasias Cutáneas , Adulto , Tumor Glómico/congénito , Tumor Glómico/patología , Tumor Glómico/cirugía , Humanos , Masculino , Neoplasias Cutáneas/congénito , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugíaRESUMEN
There are several reports of the collision of vascular and pigmentary anomalies (e.g., phakomatosis pigmentovascularis) and the association between congenital melanocytic nevi and infantile hemangiomas. We report a case of Spitz nevus arising in skin overlying a congenital plaque-like glomuvenous malformation (GVM). This is the first report of a Spitz nevus arising in direct contiguity to a GVM.
Asunto(s)
Tumor Glómico/complicaciones , Tumor Glómico/patología , Nevo de Células Epitelioides y Fusiformes/complicaciones , Nevo de Células Epitelioides y Fusiformes/patología , Paraganglioma Extraadrenal/complicaciones , Paraganglioma Extraadrenal/patología , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patología , Biopsia , Preescolar , Tumor Glómico/congénito , Humanos , Masculino , Paraganglioma Extraadrenal/congénitoRESUMEN
Congenital plaque-type glomuvenous malformation (GVM) is caused by loss of function mutations in glomulin gene. We report a newborn with this rare vascular disorder associated with chylous ascites. The common mesenchymal origin of GVM and lymphatic vessels as well as the glomulin expression in vascular smooth muscle cells in utero could help explain this unusual prenatal complication of glomuvenous malformations.
Asunto(s)
Proteínas Adaptadoras Transductoras de Señales/genética , Ascitis Quilosa/complicaciones , Ascitis Quilosa/genética , Ascitis Quilosa/congénito , Femenino , Tumor Glómico/complicaciones , Tumor Glómico/congénito , Tumor Glómico/genética , Humanos , Recién Nacido , Vasos Linfáticos/patología , Vasos Linfáticos/fisiología , Músculo Liso Vascular/patología , Músculo Liso Vascular/fisiología , Paraganglioma Extraadrenal/complicaciones , Paraganglioma Extraadrenal/congénito , Paraganglioma Extraadrenal/genéticaRESUMEN
Multiple congenital glomuvenous malformations (GVMs) are rare. Almost all reported cases describe the multiple congenital plaquelike GVM variant. We report a case of multiple congenital plaquelike GVMs suggestive of type 2 segmental involvement following Blaschko lines.
Asunto(s)
Tumor Glómico/patología , Neoplasias Cutáneas/patología , Femenino , Estudios de Seguimiento , Tumor Glómico/congénito , Tumor Glómico/diagnóstico , Humanos , Inmunohistoquímica , Recién Nacido , Neoplasias Cutáneas/congénito , Neoplasias Cutáneas/diagnósticoAsunto(s)
Tumor Glómico/congénito , Cuero Cabelludo/patología , Neoplasias Cutáneas/congénito , Preescolar , Femenino , Tumor Glómico/patología , Tumor Glómico/cirugía , Humanos , Inmunohistoquímica , Cuero Cabelludo/cirugía , Neoplasias Cutáneas/metabolismo , Neoplasias Cutáneas/patología , Neoplasias Cutáneas/cirugíaRESUMEN
Glomus tumours are solitary benign lesions most frequently located subungually on fingers and toes. In the rare case of a glomangiomatosis, the typical glomus cells are found on the altered vessel wall of the angiomatosis. Due to the rarity of this disease, no therapeutic golden standard has been mentioned in the literature, and a conservative treatment is usually adopted. The case of a 20-year-old craftsman with congenital, painful glomangiomatosis on his left dominant hand, progressively limiting the functionality of this limb, is reported. Two years after unsuccessful partial tumour resection, a surgical treatment based on radical tumour resection in terms of a finger amputation was performed. The 1-year follow-up showed no signs of pain for the patient.
Asunto(s)
Tumor Glómico/cirugía , Mano/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Neoplasias Vasculares/cirugía , Amputación Quirúrgica , Angiografía , Diagnóstico Diferencial , Dedos/irrigación sanguínea , Dedos/patología , Dedos/cirugía , Tumor Glómico/congénito , Tumor Glómico/diagnóstico , Tumor Glómico/patología , Mano/irrigación sanguínea , Mano/patología , Humanos , Angiografía por Resonancia Magnética , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/cirugía , Reoperación , Neoplasias de los Tejidos Blandos/congénito , Neoplasias de los Tejidos Blandos/diagnóstico , Neoplasias de los Tejidos Blandos/patología , Neoplasias Vasculares/congénito , Neoplasias Vasculares/diagnóstico , Neoplasias Vasculares/patología , Adulto JovenAsunto(s)
Tumor Glómico/complicaciones , Hipertricosis/complicaciones , Neoplasias Cutáneas/complicaciones , Dermis/patología , Tumor Glómico/congénito , Tumor Glómico/patología , Folículo Piloso/patología , Humanos , Hipertricosis/patología , Lactante , Masculino , Neoplasias Cutáneas/congénito , Neoplasias Cutáneas/patologíaRESUMEN
BACKGROUND: Glomuvenous malformations (GVMs) are now considered a separate entity from venous malformations. The rarest type of GVM is the generalized congenital plaque-type GVM. OBSERVATIONS: We present 10 new cases of congenital plaque-type GVM and describe their clinical progression and treatment. Mutations in the glomulin gene were found in those patients who participated in the genetic study. CONCLUSIONS: Congenital plaque-type GVMs are unique in their congenital nature, extensive distribution, difficult to diagnose and treat, and progressive involvement after birth. Most cases are familial, yet affected relatives usually have only minor lesions. The lesions of congenital plaque-type GVM are severe, visible at birth, and usually mistaken for extensive venous malformations. Vascular malformations are divided by hemodynamic type into slow-flow and fast-flow lesions. Slow-flow lesions are subcategorized as capillary, lymphatic, and venous.(1) Capillary malformations are flat, sharply demarcated, red-pink vascular stains of the skin commonly referred to as port-wine stains. These persist throughout life and are characterized histologically by dilated capillaries within the dermis. They slowly increase in size with age. Lymphatic malformations are spongelike collections of abnormal channels and spaces that contain clear lymphatic fluid, causing an excess of fluid to accumulate and dilate the lymphatic channels. This results in swelling of the affected area and, if extensive, can cause enlargement of soft tissues and bones.
Asunto(s)
Tumor Glómico/diagnóstico , Neoplasias Cutáneas/diagnóstico , Proteínas Adaptadoras Transductoras de Señales/genética , Adolescente , Adulto , Niño , Diagnóstico Diferencial , Femenino , Predisposición Genética a la Enfermedad , Tumor Glómico/congénito , Tumor Glómico/genética , Tumor Glómico/patología , Humanos , Lactante , Recién Nacido , Masculino , Mutación , Neoplasias Cutáneas/congénito , Neoplasias Cutáneas/genética , Neoplasias Cutáneas/patologíaRESUMEN
A 13 year old girl presented with recurrent painful "varicosities" on her right calf. These lesions were subsequently clinically diagnosed as "cavernous haemangiomas" after normal duplex scanning and were excised. Histological examination revealed multiple glomangiomas (glomus tumours). A literature review revealed only two reported cases of nodular multiple glomangioma, so that this is the third case to be reported in the literature.
Asunto(s)
Tumor Glómico/congénito , Neoplasias Primarias Múltiples/congénito , Neoplasias de los Tejidos Blandos/congénito , Adolescente , Diagnóstico Diferencial , Femenino , Tumor Glómico/patología , Hemangioma Cavernoso/diagnóstico , Humanos , Pierna , Neoplasias Primarias Múltiples/patología , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
Congenital multiple glomus tumors are extremely rare, and less than 20 cases have been well documented. We report an uncommon case of congenital multiple glomangiomas with a segmental manifestation in a 9-year-old girl. Since birth, the child had presented asymptomatic angiomatous macules arranged in a segmental pattern on the neck and trunk. During a follow-up period of 9 years, disseminated smaller papulonodular lesions developed on both arms and the left leg with a segmental distribution. Histopathology of congenital and acquired lesions confirmed the diagnosis of glomangiomas. The family history was negative for glomus tumors. This type of presentation supports the recently described type 2 segmental manifestation of multiple glomus tumors, with a segmental involvement of congenital early developing lesions.
Asunto(s)
Tumor Glómico/patología , Neoplasias Cutáneas/patología , Biopsia , Niño , Femenino , Tumor Glómico/congénito , Humanos , Neoplasias Cutáneas/congénitoRESUMEN
Congenital glomus tumor is a rare variant of glomus tumor, and glomangiomyoma is the least frequent histological type of glomus tumor. We present a case of congenital multiple plaque-like glomangiomyoma in an 11-year-old child with multiple diffuse plaques on his right lateral trunk. Histopathologic study showed a picture of typical glomus cell undergoing transition to smooth muscle cell. After literature review, this might be the first case report of congenital multiple plaque-like glomus tumor in trunk with histological appearance of a glomangiomyoma.
Asunto(s)
Tumor Glómico/congénito , Tumor Glómico/patología , Niño , Humanos , MasculinoRESUMEN
An 8-year-old girl was seen in our outpatient clinic with a history of an angiomatous plaque on the chest since birth. She had complained of pain at the site of the lesion for 1 month. Histopathologic examination and immunohistochemical analysis led to the diagnosis of glomangioma, a rare congenital plaquelike glomus tumor. We review the literature and discuss the clinical presentation and evolution of this rare entity.