RESUMEN
BACKGROUND: Perivascular Epithelioid cell tumors (PEComa) are rare mesenchymal tumors. They generally occur in the gynecologic or digestive tract. The diagnosis of Central Nervous System PEComa is exceptional and challenging. CASE DESCRIPTION: We report the case of a 46-year-old woman, with no particular medical history, who presented a secondary amenorrhea and a slight hyperprolactinemia. She was diagnosed on MRI with a pituitary tumor showing spontaneous hypersignal in T1-weighted images. After failure of medical treatment with cabergoline, surgical resection was required due to progressive tumor growth. Macroscopic aspect and initial immunohistochemical features were in favor of a primitive hypophyseal melanocytoma. However, molecular and transcriptional study through targeted exome- and RNA-sequencing led to the exceptional diagnosis of pituitary Perivascular Epithelioid Cell Tumor (PEComa). Three-years of postoperative radio-clinical follow-up showed an asymptomatic non-evolutive small remnant. CONCLUSION: PEComa is an exceptional diagnosis among pituitary tumors. It should be evoked as a potential differential diagnosis in case of primitive melanocytic lesion of the pituitary gland. Specific molecular analysis is mandatory to confirm the diagnosis and exclude differential diagnosis.
Asunto(s)
Neoplasias de Células Epitelioides Perivasculares , Neoplasias Hipofisarias , Humanos , Femenino , Persona de Mediana Edad , Neoplasias Hipofisarias/diagnóstico , Neoplasias Hipofisarias/cirugía , Neoplasias Hipofisarias/patología , Células Epitelioides/patología , Diagnóstico Diferencial , Hipófisis/patología , Neoplasias de Células Epitelioides Perivasculares/diagnóstico , Neoplasias de Células Epitelioides Perivasculares/cirugía , Neoplasias de Células Epitelioides Perivasculares/patologíaRESUMEN
Meningiomas are the most frequent among intracranial tumors, and represent more than 30% of primitive central nervous system neoplasms. Arising from the meninges, they are generally benign lesions and can be treated by either radio-clinical follow-up or surgical resection with excellent outcome. However, more than 20% of meningiomas harbor atypical or malignant features and represent challenges for both prognostic evaluation and therapeutic strategy. The discovery of the genetic and epigenetic landscapes of meningiomas enabled the identification of new prognostic markers and potential therapeutic targets for refractory meningiomas. This review summarizes current epidemiology, histological and molecular characteristics, diagnosis and treatments for meningiomas, and highlights the close relationship between the development of meningiomas and hormonal intake, as illustrated by recent recommendations of the "Agence Nationale de Securité du Medicament", the French national drug safety agency.
Asunto(s)
Neoplasias Encefálicas , Neoplasias Meníngeas , Meningioma , Epigenómica , Humanos , Neoplasias Meníngeas/diagnóstico , Neoplasias Meníngeas/epidemiología , Neoplasias Meníngeas/genética , Meningioma/diagnóstico , Meningioma/epidemiología , Meningioma/genética , PronósticoRESUMEN
Cerebral venous sinus thrombosis (CVST) following brain surgery is a feared complication, commonly described after direct injury to the sinus. However, distant CVST occurring away from the operative area are unexpected. Yet, there is a strong physio-pathological rational supporting the role of intracranial hypotension as a risk factor of CVST. Here, we report the case of a frontal arachnoid cyst depletion followed by an extensive contralateral CVST. Given the major prognostic consequences observed in this clinical illustration, we further investigated the hypothesis of intracranial hypotension as an etiology of CVST by carrying out a systematic review of the literature.
Asunto(s)
Hipotensión Intracraneal , Trombosis de los Senos Intracraneales , Humanos , Hipotensión Intracraneal/diagnóstico , Hipotensión Intracraneal/etiología , Trombosis de los Senos Intracraneales/diagnóstico por imagen , Trombosis de los Senos Intracraneales/etiologíaRESUMEN
A multicystic intraventricular tumour of the right ventricular atrium was incidentally diagnosed on follow-up imaging of a 61-year-old man with a history of prostatic adenocarcinoma. Surgical resection of the lesion was performed after a one-year radio-clinical follow-up due to progressive expansion of the lesion size and a rising prostate specific antigen blood-level. Morphological features with papillary pattern on pathological examination were compatible with malignant adenocarcinoma or choroid plexus carcinoma. The immunoprofile was conclusive for an exceptional choroid plexus metastasis (CPM) of a prostatic adenocarcinoma. To our knowledge, this is the first report of a proven prostatic origin of a CPM.
Asunto(s)
Adenocarcinoma , Carcinoma , Neoplasias del Plexo Coroideo , Neoplasias de la Próstata , Adenocarcinoma/patología , Plexo Coroideo , Neoplasias del Plexo Coroideo/diagnóstico , Neoplasias del Plexo Coroideo/secundario , Neoplasias del Plexo Coroideo/cirugía , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias de la Próstata/patologíaRESUMEN
The reconstruction of anterior skull base defects after carcinologic surgery is challenging. Large defects can require the use of autologous free tissue transfer. Currently, most reconstructions use soft-tissue flaps. We describe the use of an osteocutaneous radial forearm free flap to reconstruct a large defect secondary to a malignant paraganglioma extending into the anterior cranial fossa and both orbits. The surgical resection required endonasal and transcranial approaches. We reconstructed the defect with a free osteocutaneous radial forearm flap. We laid the bone flap across the defect, resting on the orbital roof on each side, and sutured the soft component to the edge of the dura. The pedicle was funnelled from the craniotomy to a prepared cervicotomy and the micro-anastomoses were performed onto the facial artery and two satellite veins. Potential indications and major drawbacks of this technique are briefly discussed. Osteocutaneous radial forearm free flaps can be a valuable reconstructive option for patients with a large defect of the anterior skull base, needing both rigid support and a watertight closure.
Asunto(s)
Colgajos Tisulares Libres , Procedimientos de Cirugía Plástica , Craneotomía , Antebrazo/cirugía , Humanos , Base del Cráneo/cirugíaRESUMEN
We report a case of malignant melanoma revealed by a metastasis to the pituitary gland. The tumor was misdiagnosed as a pituitary adenoma and aggressive transsphenoidal surgery was complicated by a cerebrospinal fluid fistula. Nine weeks later, the patient presented multiple leptomeningeal and brain metastases spreading from the sellar region. Regarding these observations, we conducted a systematic review of the literature in order to investigate clinicoradiological features that should lead clinicians to suspect pituitary metastasis and how it should impact the surgical management.
Asunto(s)
Adenoma/patología , Melanoma/patología , Neoplasias Hipofisarias/patología , Adenoma/diagnóstico , Neoplasias Encefálicas/secundario , Errores Diagnósticos , Resultado Fatal , Femenino , Humanos , Imagen por Resonancia Magnética , Melanoma/diagnóstico , Persona de Mediana Edad , Neoplasias Hipofisarias/diagnóstico , Tomografía Computarizada por Tomografía de Emisión de PositronesRESUMEN
Pituitary surgery is performed via a transsphenoidal approach in the vast majority of cases according to various methods that have changed over the years. A microscopic transseptal approach via a sublabial mucosal incision or a nasal mucosal incision has also been extensively used. An endoscopic transnasal approach was first described in the 1990's, followed by the concept of a microscopic transseptal approach and an endoscopic strictly endonasal approach. We use an entirely endoscopic transseptal transsphenoidal approach via an incision in the nasal mucosa for both access and tumour resection. This procedure has a number of advantages: strictly midline approach to the sella turcica, large operative field, no interference between instruments and a low rate of nasal complications.
Asunto(s)
Adenoma/cirugía , Tabique Nasal/cirugía , Neoplasias Hipofisarias/cirugía , Cirugía Endoscópica Transanal/métodos , Puntos Anatómicos de Referencia , Humanos , Posicionamiento del Paciente/métodos , Silla Turca/cirugía , Seno Esfenoidal/cirugía , Técnicas de Sutura , Cirugía Endoscópica Transanal/instrumentaciónRESUMEN
OBJECT: Spinal instrumentation has a high rate of surgical site infection (SSI), but results greatly vary depending on surveillance methodology, surgical procedures, or quality of follow-up. Our aim was to study true incidence of SSI in spinal surgery by significant data collection, and to compare it with the results obtained through the hospital information system. METHODS: This work is a single center prospective cohort study that included all patients consecutively operated on for spinal instrumentation by posterior approach over a six-month period regardless the etiology. For all patients, a "high definition" prospective method of surveillance was performed by the infection control (IC) department during at least 12 months after surgery. Results were then compared with findings from automatic surveillance though the hospital information system (HIS). RESULTS: One hundred and fifty-four patients were included. We found no hardly difference between "high definition" and automatic surveillance through the HIS, even if HIS tended to under-estimate the infection rate: rate of surgical site infection was 2.60% and gross SSI incidence rate via the hospital information system was 1.95%. Smoking and alcohol consumption were significantly related to a SSI. CONCLUSION: Our SSI rates to reflect the true incidence of infectious complications in posterior instrumented adult spinal surgery in our hospital and these results were consistent with the lower levels of published infection rate. In-house surveillance by surgeons only is insufficiently sensitive. Further studies with more patients and a longer inclusion time are needed to conclude if SSI case detection through the HIS could be a relevant and effective alternative method.