Haemangiomas, have rarely been encountered in the spinal intradural extramedullary space and the MRI findings of this entity have been described only in a few cases. We present the Magnetic Resonance Imaging (MRI) and surgical findings of a rare case of intradural extramedullary cavernous angioma located at the T1-T2 level in a 65-year-old man presented progressive paraparesis and upper thoracic back pain. On MRI, a well-circumscribed intradural solid mass, 1 cm in diameter, was detected and another enhancing nodular mass was found at the nerve roots of the cauda equina. The thoracic spinal lesion was removed and the histological diagnosis confirmed cavernous haemangioma. Although very uncommon, haemangioma should be included in the differential diagnosis when a spinal intradural extramedullary lesion is discovered and some neuroradiological findings could allow a presumptive diagnosis.
BackgroundSchwannoma and angiosarcoma are infrequent pathologies that have been rarely reported in the kidney. Angiosarcoma is an uncommon malignant tumor presenting a recognizable vascular differentiation. It can develop in any site but the most common locations include the skin, soft tissues, breast, bone, liver, and spleen while renal localization has been very rarely reported in the literature. Schwannoma is a benign peripheral nerve sheath tumor composed of cells with the immunophenotype and ultrastructural features of differentiated Schwann cells. It has a wide anatomical distribution but the most frequent locations include subcutaneous tissues of the extremities and the head and neck region and the retroperitoneal and mediastinal soft tissues. The occurrence of an angiosarcoma in a pre-existing schwannoma is an extremely rare event with <20 cases reported in worldwide literature. In the present study, a renal case of angiosarcoma arising in schwannoma is presented with a detailed review of the pertinent literature.Case PresentationA 56-year-old man was admitted with a few days history of lower back pain and hematuria. Abdominal ultrasound showed a mass inside the left renal medulla. Subsequent imaging investigations with computed tomography and magnetic resonance confirmed the presence of the lesion and showed a pulmonary metastasis.ConclusionsThe final histopathological examination led to the diagnosis of epithelioid angiosarcoma arising in a schwannoma. The patient came to death a few months later due to a massive hemothorax. To the best of our knowledge, the present is the first case of an angiosarcoma arising in a schwannoma of the kidney.
The pathogenesis of cervical artery dissection is poorly understood. Deficiency of the elastase inhibitor alpha-1-antitrypsin may represent a predisposing condition. Biochemical and genetic analyses in a series of 12 consecutive patients with spontaneous dissection of the neck vessels showed 3 cases associated to alpha-1-antitrypsin deficiency, in combination with transient precipitating factors. A disequilibrium between proteolytic enzymes and protease inhibitors may contribute to the pathogenesis of cervical artery dissection leading to structural abnormalities of the extracellular matrix and increasing the susceptibility of the vessel wall to additional short-lived trigger mechanisms.
Ehlers-Danlos syndrome is a complex hereditary connective tissue disorder that is characterized by abnormalities of the skin and joints and visceral and neurological manifestations. At present, at least 11 forms are recognized on the basis of their clinical characteristics, methods of transmission, and biochemical defect. The neurologic manifestations include cerebrovascular disease, peripheral neuropathy, plexopathy, periventricular subependymal heterotopias, and epilepsy. Previously, 2 females were reported to be affected with subependimal periventricular heterotopias and Ehlers-Danlos syndrome type 1. The authors report a new case of a 12-year-old girl with similar clinical and neuroradiological features.
Pituitary metastases are unusual complications of malignancies. In about only 2% of patients they origin from colorectal cancer (CRC), with breast and lung as the most common primary tumors. Nevertheless, some authors reported a recent increase of the incidence of metastases in infrequent sites, such as brain or bone, arising from gastrointestinal cancers, probably due to the expanded treatment options and the resulting improved survival. Here, we report the case of a 54-year old woman diagnosed with lung metastases from rectal cancer, who, after several cycles of radio- and chemotherapy, presented symptoms and signs of pituitary disfunction (i.e. diabetes insipidus, hypothyroidism and diplopy). The diagnosis of pituitary metastasis from rectal cancer was histologically confirmed after surgery.
RIASSUNTO -Le malformazioni caratterizzate da divisione del midollo spinale ("Split cord malformation") sono rare forme di disrafismo spinale occulto che si sviluppano precocemente nel corso della vita embrionale. I pazienti che ne sono affetti presentano elevati rischi di sviluppare deficit neurologici gravemente invalidanti nel corso della vita, per l'ancoraggio midollare sempre presente. Già alla nascita, la maggior parte di essi presenta anomalie cutanee (ipertricosi, nevi, angiomi, fossette o fistole e lipomi sottocutanei) localizzate generalmente in regione lombo-sacrale che costituiscono spesso l'unico segno della sottostante patologia. Descriviamo gli aspetti neuroradiologici e neurochirurgici di un raro caso di divisione midollare associata a tehered cord, doppio seno dermico ed anomalie vertebrali multiple. Neonata da parto spontaneo alla 38°settimana con peso alla nascita di g. 3100 e Apgar 8/10, che presenta stigmate disrafiche cutanee ed una modesta iperreflessia rotulea bilaterale con lieve ipotonia degli arti inferiori. Al 2°giorno di vita viene effettuato studio neuroradiologico encefalo-spinale con Risonanza Magnetica (RM), Tomografia Computerizzata (TC) ed ecografia che mette in evidenza la presenza di una doppia corda midollare con unico rivestimento durale (tipo II di Pang), un duplice seno dermico, a L3-L4 ed a livello coccigeo con ancoraggio midollare ed alcune anomalie vertebrali (vertebra a farfalla a T12 e schisi multiple). A 22 giorni dalla nascita la piccola paziente è stata sottoposta con successo ad intervento chirurgico per disancoraggio del midollo. I reperti operatori hanno confermato l'ipotesi diagnostica neuroradiologica. Nel corso dei mesi successivi sono stati eseguiti controlli clinici e strumentali seriati, compresa una valutazione urodinamica, che non hanno mostrato deficit neurologici o sfinterici. A distanza di un anno la piccola presenta un regolare sviluppo psicomotorio ed è neurologicamente indenne. I disrafismi spinali, comprendono spesso malformazioni complesse che sebbene rari esistono e necessitano di una diagnosi precoce e di un trattamento rapido, prima dell'insorgenza di deficit neurologici gravi ed irreversibili. Le anomalie cutanee rappresentano spesso l'unico segno della malformazione, in particolare nei neonati, nei quali i segni clinici sono assenti o difficilmente valutabili. La diagnostica neuroradiologica (RM in particolare) è del tutto affidabile nell'individuare le cause che determinano l'ancoraggio midollare al fine di favorire un trattamento neurochirurgico efficace, come confermato anche in questa nostra esperienza. SUMMARY-The malformations characterising split cord malformation are rare forms of occult spinal dysraphism which develop early in foetal life. Affected patients are at high risk of developing severely invalidating neurological deficits due to the tethered cord. At birth, most babies present skin abnormalities (hypertrichosis, naevi, angiomas, foveolas or fistulae and subcutaneous lipomas) usually in the lumbosacral
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