Selected case from the Arkadi M. Rywlin international pathology slide series: lymphangiomatosis of the spleen associated with ipsilateral abdominopelvic and lower extremity venolymphatic malformations: a case report and review of the literature.

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.

Solitary fibrous tumor of the central nervous system: a 15-year literature survey of 220 cases (August 1996-July 2011).

We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.

Preoperative assessment of stenosis of the epiaortic vessels: can colour-Doppler ultrasound really supplant angiography?

PURPOSE: To evaluate the diagnostic role of digital angiography compared with colour-Doppler US in the study of epiaortic vessel stenoses and the degree of morbidity associated with angiography. MATERIALS AND METHODS: Out of 2,000 angiographic examinations of the epiaortic vessels performed for the concurrent presence of clinical findings of cerebrovascular insufficiency and colour-Doppler US findings of carotid-vertebral stenosis, a randomized sample of 200 patients was evaluated. The retrospective review of medical records provided the surgical data of the stenosis which were subsequently compared with the angiography and colour-Doppler US findings, and with the clinical and laboratory data to identify the possible complications of angiography. RESULTS: Diagnostic agreement was 75%. Colour-Doppler US underestimated stenosis in 16.5% and overestimated stenosis in 8% of cases. In 18% of cases there was a diagnostic gain of angiography (vascular lesions associated with main lesion), which also provided clinically important incidental findings (intracranial aneurysms, meningiomas) in 6.5% of patients. No relevant complications resulting from the angiographic procedure were recorded. CONCLUSIONS: Angiography allowed a more accurate evaluation of the degree of stenosis compared with colour-Doppler ultrasound. Moreover, it allowed diagnosis of important associated conditions that may affect therapeutic planning. In our series, we recorded no important complications related to angiography. Therefore, angiography remains the standard of reference in the preoperative evaluation of patients with clinically diagnosed cerebral ischaemia. Though fairly reliable as a first-line investigation, Colour-Doppler US is not sufficiently exhaustive as a pre-operative evaluation tool.

Endovascular treatment of intracranial arterio-venous malformations with Onyx embolization: preliminary experience.

PURPOSE: Intracranial arterio-venous malformations (AVM) often represent a complex clinical problem as regards indications to treatment, the choice of treatment and the technical difficulties related to treatment. In the last twenty years, endovascular treatment of intracranial arterio-venous malformations was primarily based on an acrylic glue (Hystoacril) whose endovascular use is not free from risks. A new product for endovascular embolization, named ONYX, has been recently been made commercially available. It is a bio-compatible liquid polymer that precipitates and solidifies in contact with blood, thus forming a soft and spongy embolus. The aim of this study was to evaluate our preliminary results in the endovascular embolization of intracranial AVM with Onyx. MATERIALS AND METHODS: Ten patients were treated (7 men, 3 women; mean age: 29 years, range: 12-48 years) for a total of 37 embolizations, 22 with Onyx and 15 with acrylic resin. RESULTS: Nidus occlusion was complete in 2 cases, >90% in 2 cases, >50 and <90% in 3 cases and <50% in the remaining 3 cases. The following complications were recorded: two transient and one mild permanent neurological deficits, two clinically silent cases of moderate subarachnoid haemorrhage, four catheters glued to the injection site, three cases of treatment discontinuation due to continuous and massive reflux of Onyx into the afferent artery peduncle. CONCLUSIONS: This early experience showed that while Onyx has good embolization potential it also presents some disadvantages, which need to be overcome before this product can be considered easy and safe to use on a large scale.