RESUMO
Blue rubber bleb nevus syndrome (BRBNS) is a rare condition that presents with venous malformation blebs throughout the body, most commonly on the skin and gastrointestinal tract. There have only been a limited number of reports of benign BRBNS lesions involving the spine in children, which were detected after chronic symptomatology. We herein present a unique case of a ruptured BRBNS venous malformation into the epidural space of the lumbar spine in a child presenting with acute neurologic deficit and discuss the relevant surgical considerations for operating in the setting of BRBNS.
Assuntos
Hematoma Epidural Espinal , Nevo Azul , Neoplasias Cutâneas , Humanos , Criança , Nevo Azul/complicações , Nevo Azul/cirurgia , Nevo Azul/patologia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/patologia , Pele/patologiaRESUMO
PURPOSE: To describe two patients with blue nevi of the palpebral conjunctiva and to review the existing literature on common and cellular blue nevi of the conjunctiva. METHODS: Report of two cases and literature review. RESULTS: We present two cases of blue nevi in the palpebral conjunctiva: an atypical cellular blue nevus of the left upper eyelid and a common blue nevus around the lacrimal punctum of the left lower eyelid. Both patients underwent full thickness eyelid excision with wide margins. There was no tumor recurrence at 11 and 4 months postoperatively. CONCLUSIONS: Blue nevi are a group of melanocytic tumors that rarely involve the ocular adnexa. They may arise in the palpebral conjunctiva and should be considered in the differential diagnosis of pigmented lesions in this location as they can mimic melanoma.
Assuntos
Neoplasias da Túnica Conjuntiva , Nevo Azul , Neoplasias Cutâneas , Túnica Conjuntiva/patologia , Neoplasias da Túnica Conjuntiva/diagnóstico , Diagnóstico Diferencial , Humanos , Recidiva Local de Neoplasia/patologia , Nevo Azul/diagnóstico , Nevo Azul/patologia , Nevo Azul/cirurgia , Neoplasias Cutâneas/patologia , SíndromeRESUMO
A 37-yr-old woman presented to the gynecology clinic with abnormal uterine bleeding in the setting of known, large uterine fibroids. Preoperative endometrial biopsy identified atypical melanocytic cells concerning for uterine melanoma. Care was transferred to the gynecologic oncology service for hysterectomy. Intraoperative findings included macular, blue-black pigmentation of the peritoneum of the bladder and cervix, which was resected and sent for frozen section, confirming melanocytic neoplasia. The hysterectomy revealed multiple tan leiomyomas up to 12 cm, and a distinct 3 cm black, incompletely circumscribed mass in the endomyometrium composed of bland spindled cells with delicate melanin granules. The tumor cells were positive for Sox-10, BAP1, and Mart-1 (Melan-A) and negative for PRAME, PD-L1, and BRAFV600E by immunostains. Microscopic elements of similar melanocytes and melanophages were found in the cervix and bladder peritoneum. Molecular analysis of the uterine tumor identified a GNA11 mutation but no TERT or BAP1 mutation. The uterine melanocytic tumor has characteristic findings of a cellular blue nevus arising in association with dendritic melanocytosis of Mullerian and pelvic tissues, a rarely seen benign phenomenon that should be distinguished from malignant melanoma of the upper genital tract.
Assuntos
Subunidades alfa de Proteínas de Ligação ao GTP/genética , Leiomioma/diagnóstico por imagem , Melanoma/diagnóstico por imagem , Nevo Azul/diagnóstico por imagem , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Uterinas/diagnóstico por imagem , Adulto , Colo do Útero/patologia , Diagnóstico Diferencial , Endométrio/diagnóstico por imagem , Endométrio/patologia , Feminino , Humanos , Histerectomia , Leiomioma/patologia , Leiomioma/cirurgia , Melanócitos/patologia , Melanoma/patologia , Mutação , Nevo Azul/patologia , Nevo Azul/cirurgia , Pelve/diagnóstico por imagem , Pelve/patologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/cirurgia , Resultado do Tratamento , Bexiga Urinária/patologia , Neoplasias Uterinas/patologiaRESUMO
The cellular blue nevus tumor is a type of dendritic melanocytic nevus that is typically benign and exceedingly rare. The incidence of all blue nevi is about 1%, usually affecting the adult population and appearing on the extremities, sacrococcygeal or gluteal regions. There have only been a handful of case reports cited in the literature where cellular blue nevi present in the head and neck region, usually affecting the scalp and young adult population (7, 8). As such, it is exceedingly rare to encounter a cellular blue nevus tumor in the neck or infiltrating into neck lymph nodes. Here we report a rare case of a cellular blue nevus tumor presenting as a right neck mass in a pediatric 16-year-old patient, shown to invade into the submandibular lymph node and surrounding soft tissue. It is important to be aware of the cellular blue nevus tumor as a differential diagnosis in pediatric neck masses. Histological evaluation is necessary to determine tumor aggression and malignant potential which can guide further treatment in pediatric patients.
Assuntos
Linfonodos/patologia , Mandíbula , Nevo Azul/patologia , Neoplasias Cutâneas/patologia , Adolescente , Biomarcadores Tumorais/análise , Diagnóstico Diferencial , Feminino , Humanos , Linfonodos/cirurgia , Antígeno MART-1/análise , Nevo Azul/diagnóstico , Nevo Azul/cirurgia , Fatores de Transcrição SOXE/análise , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Antígeno gp100 de Melanoma/análiseAssuntos
Coagulação Intravascular Disseminada , Neoplasias Gastrointestinais , Hemangioma , Nevo Azul , Neoplasias Cutâneas , Humanos , Coagulação Intravascular Disseminada/complicações , Nylons , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/cirurgia , Hemangioma/complicações , Hemangioma/diagnóstico por imagem , Hemangioma/cirurgia , Nevo Azul/complicações , Nevo Azul/cirurgia , Neoplasias Gastrointestinais/complicações , Neoplasias Gastrointestinais/cirurgiaAssuntos
Neoplasias Gastrointestinais , Hemangioma , Nevo Azul , Neoplasias Cutâneas , Humanos , Nevo Azul/diagnóstico por imagem , Nevo Azul/cirurgia , Neoplasias Gastrointestinais/cirurgia , Hemangioma/diagnóstico por imagem , Hemangioma/cirurgia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/cirurgia , ColoRESUMO
The paper describes a rare clinical case of intramedullary melanocytoma, provides a detailed description of a pathomorphological study, and addresses the issues of differential diagnosis and surgical treatment.
Assuntos
Nevo Azul/cirurgia , Neoplasias da Medula Espinal/cirurgia , Adulto , Feminino , Humanos , Imageamento por Ressonância Magnética , Nevo Azul/diagnóstico por imagem , Nevo Azul/fisiopatologia , Neoplasias da Medula Espinal/diagnóstico por imagem , Neoplasias da Medula Espinal/fisiopatologiaRESUMO
Nevus of Ota, also known as oculodermal melanocytosis, is a congenital pigmentary condition that can affect structures in the distribution of the ophthalmic and maxillary divisions of the trigeminal cranial nerve. Malignant transformation, although rare, may occur within nevus of Ota and result in uveal, cutaneous, orbital or even dural melanoma. We present a new association of Nevus of Ota complicated with a giant orbital Blue Nevus in a young white male and the management of this tumor.
Assuntos
Neoplasias Oculares/diagnóstico , Neoplasias Oculares/cirurgia , Nevo de Ota/diagnóstico , Nevo de Ota/cirurgia , Nevo Azul/diagnóstico , Nevo Azul/cirurgia , Diagnóstico Diferencial , Diagnóstico por Imagem , Neoplasias Oculares/patologia , Humanos , Masculino , Nevo de Ota/patologia , Nevo Azul/patologia , Adulto JovemAssuntos
Anemia Ferropriva/etiologia , Neoplasias Gastrointestinais/complicações , Doenças do Íleo/etiologia , Obstrução Intestinal/etiologia , Intussuscepção/etiologia , Neoplasias Musculares/complicações , Músculo Esquelético/patologia , Nevo Azul/complicações , Neoplasias Cutâneas/complicações , Dor Abdominal/etiologia , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/terapia , Artralgia/etiologia , Biópsia , Nádegas , Criança , Doença Crônica , Enteroscopia de Duplo Balão , Hemorragia Gastrointestinal/etiologia , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/cirurgia , Humanos , Doenças do Íleo/diagnóstico , Doenças do Íleo/cirurgia , Obstrução Intestinal/diagnóstico , Obstrução Intestinal/cirurgia , Intussuscepção/diagnóstico , Intussuscepção/cirurgia , Imageamento por Ressonância Magnética , Masculino , Neoplasias Musculares/diagnóstico , Invasividade Neoplásica , Nevo Azul/diagnóstico , Nevo Azul/cirurgia , Recidiva , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgiaAssuntos
Endoscopia por Cápsula/métodos , Enteroscopia de Duplo Balão/métodos , Hemorragia Gastrointestinal , Neoplasias Gastrointestinais , Trato Gastrointestinal , Nevo Azul , Escleroterapia/métodos , Neoplasias Cutâneas , Coagulação com Plasma de Argônio/métodos , Criança , Hemorragia Gastrointestinal/etiologia , Hemorragia Gastrointestinal/prevenção & controle , Neoplasias Gastrointestinais/complicações , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/fisiopatologia , Neoplasias Gastrointestinais/cirurgia , Trato Gastrointestinal/irrigação sanguínea , Trato Gastrointestinal/diagnóstico por imagem , Trato Gastrointestinal/cirurgia , Humanos , Masculino , Nevo Azul/complicações , Nevo Azul/diagnóstico , Nevo Azul/fisiopatologia , Nevo Azul/cirurgia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/fisiopatologia , Neoplasias Cutâneas/cirurgia , Resultado do Tratamento , Veias/anormalidadesAssuntos
Dermoscopia/métodos , Nevo Azul/diagnóstico , Nevo Azul/cirurgia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia , Adulto , Biópsia por Agulha , Criança , Seguimentos , Pé , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Cirurgia de Mohs/métodos , Nevo Azul/patologia , Medição de Risco , Estudos de Amostragem , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular disorder characterized by multifocal venous malformations (VMs). Little is known about the perinatal management of pregnant women with BRBNS owing to the limited number of reported cases. We present the case of a 36-year-old primigravida with BRBNS who underwent an uneventful cesarean section under spinal anesthesia for breech presentation. A thorough systemic examination revealed VMs in various organs, including the skin, conjunctiva, larynx, gastrointestinal tract, lungs, and vulva. Prior to spinal anesthesia, careful examination using imaging modalities was conducted to assess the spinal and epidural involvement of the VMs to avoid complications, including accidental puncture of the VMs, associated bleeding, and epidural hematoma. In pregnant women with BBNS, it is imperative to scrutinize the localization and distribution of VMs throughout the body to anticipate potential complications and select the appropriate delivery mode and anesthetic management.
Assuntos
Cesárea , Nevo Azul , Neoplasias Cutâneas , Humanos , Feminino , Gravidez , Nevo Azul/cirurgia , Adulto , Neoplasias Cutâneas/cirurgia , Neoplasias Gastrointestinais/cirurgia , Raquianestesia/métodosRESUMO
INTRODUCTION: Cellular blue nevus is an uncommon neoplasm in the spine. PATIENT CONCERNS: Here, we present a case of a 24 years old male with a 2 months history of numbness in the right upper limb and shoulder. DIAGNOSIS: Cervical spine and subcutaneous tissue invasive cellular blue nevus. INTERVENTIONS: The patient underwent C4 laminectomy and partial C3 and C5 laminectomy for total resection of the lesion. Histopathology revealed a nodular tumor with unclear boundaries, which was composed of heavily pigmented dendritic cells and more pigmented spindle cells. OUTCOMES: There was no recurrence during 3 years follow-up. CONCLUSION: Invasive cellular blue nevus of the spine can be wrongly diagnosed as spinal meningeal melanocytoma and meningeal melanoma due to its special cell behavior and rarity. Therefore, it is important to understand its pathological and clinical characteristics to avoid over-treatment.
Assuntos
Melanoma , Neoplasias Meníngeas , Nevo Azul , Nevo Pigmentado , Neoplasias Cutâneas , Humanos , Masculino , Adulto Jovem , Vértebras Cervicais/patologia , Melanoma/patologia , Neoplasias Meníngeas/patologia , Nevo Azul/diagnóstico , Nevo Azul/cirurgia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/patologiaRESUMO
Large or giant cellular blue nevi are usually congenital and represent a challenge for the physician. Close anatomic structures may be altered by the size of the moles. In this article, we report the case of an uncommon large, agminated, cellular blue nevus of the 'plaque type' in a 42-year-old female. Due to the risks of malignant melanoma development on a large or giant blue nevus, we highlight the importance of proper histopathological diagnosis. Furthermore, because of the possibility that the nevus may invade the bone and cerebral tissues, we discuss the indication of a radiological diagnosis. The accurate correlation to clinical and histopathological findings and appropriate multidisciplinary management can save the lives of patients.