Spinal adrenal cortical adenoma associated with Beckwith-Wiedemann syndrome: case report and review of the literature.

PURPOSE: Ectopic adrenal cortical adenoma in the spinal region is extremely rare. The majority of cases of ectopic adrenocortical tissue are found along the path of embryonic migration within the urogenital tract. Beckwith-Wiedemann syndrome (BWS) is a pediatric overgrowth disorder involving a predisposition to tumor development, including adrenal lesions. To date, only eight spinal cases have been reported. This is the third reported case in pediatric population, the first one associated with genetic syndrome and the first benign to recur. We review the current literature on this topic. CASE DESCRIPTION: We present a 2-year-old boy affected by Beckwith-Wiedemann syndrome who developed a tumor at L4-L5 level. He underwent a gross total resection with MRI post-surgery demonstrating non-residual tumor. Histology disclosed an ectopic adrenal cortical adenoma with oncocytic features. Immunohistochemically was positive for inhibin-alpha, synaptophysin, and melan-A. It was negative for chromogranin A, GFAP, S-100, and other markers. One year later, he developed a recurrence at the same level being necessary a second surgery leaving a small sheet of residual tumor. CONCLUSION: Spinal adrenocortical adenomas are exceptional, and its behavior could be related to other conditions such as BWS. Gross total resection can be curative but a tight follow-up is needed. Immunohistochemical studies that include inhibin-alpha, synaptophysin, and melan-A can be useful in differential diagnosis as ultrastructural study. The decision on how to treat these patients is difficult given the low number of cases.

[Spinal sinovial cysts: Surgical treatment and clinical outcomes in a series of 18 cases]. / Quistes articulares espinales: evolución clínica y resultados según intervención quirúrgica. Revisión de 18 casos.

OBJECT: A series of 18 patients with symptomatic synovial cysts was analysed from May 2009 to November 2013. Different approaches were performed for their removal. MATERIAL AND METHODS: The study included 18 patients, 8 men and 10 women, aged between 50 and 77 years. An analysis was made of the variables including age, gender, symptoms, imaging studies, histopathology, surgery, follow-up, complications, and clinical outcome. RESULTS: An articular synovial cyst was diagnosed in 17 cases, and a ganglion in one cases. The most common symptom was back pain with radiculopathy (94%). Motor deficits occurred in 4 cases (22%), and 1 case (5%) presented with urinary incontinence. The most common level was L4- L5 (67%), with one atypical case observed in the D12 -L1 location. Hemi-laminectomy was performed in 14 cases, with 9 of them having an interspinous spacer (ISP) device inserted. A laminectomy with a fusion procedure was performed in 3 patients and 1 patient had a bilateral decompression using a unilateral approach. The patients were followed-up for between 6 months to 2 years. CONCLUSIONS: Synovial cysts are a cause of radiculopathy/neurogenic claudication. Spinal cysts are commonly found at the L4-L5 level. MRI is the tool of choice for diagnosis. The most common symptom was back pain with radiculopathy. Synovial cysts resistant to conservative therapy should be treated surgically. In our series, surgical resection of symptomatic juxtafacet cysts showed a good clinical outcome, but the optimal approach for patients with juxtafacet cysts remains unclear.

[Myxoid/round cell liposarcoma of the brachial plexus]. / Liposarcoma mixoide de células redondas de plexo braquial.

Myxoid/round cell liposarcoma is a soft tissue sarcoma that is extremely rare in the brachial plexus. We report a case of a myxoid/round cell liposarcoma originating in the brachial plexus that was surgically resected and evolved well, with no deficit or recurrence after 2 years of follow-up. To date, there has been no other case of this sarcoma in the literature.

Free Mucosal Graft for Reconstruction after Nonfunctional Pituitary Adenoma Surgery.

BACKGROUND: In the search for an effective closure without nasosinusal morbidity, we have studied the efficacy of free mucosal graft as a reconstructive technique of the sellar floor after the resection of nonfunctioning pituitary adenomas (NFPA). METHODS: In 100 endonasal endoscopic surgeries, we analyzed the personal history, radiological and intraoperative aspects that could have an impact on the risk of postoperative cerebrospinal fluid (CSF) leak. They were divided into three groups: no mucosa flap/graft, mucosal free graft, and nasoseptal pedicled flap. RESULTS: The characteristics of the patients and adenomas were the same in all three groups. Intraoperative CSF leak was observed in 1/13 cases of the group without graft/flap (7%), in 16/50 of the free mucosal graft (32%) and 12/37 (32%) of pedicle flap. The proportion of cases in which other means of reconstruction were used in addition (fat, collagen matrix, and sealant) was similar in the different groups. No CSF leaks were observed, except for a doubtful one in the free mucosal graft group, which resolved spontaneously within 24 h, without receiving any type of treatment. CONCLUSIONS: The middle turbinate free mucosal graft can be of great value in endonasal surgery: It achieves a hermetic closure in cases of low-flow CSF leaks, it can be useful as a rescue for cases where nasoseptal mucosa is not available to perform a pedicled nasoseptal flap, minimizes the nasosinusal complications of the pedicled flap by leaving a smaller surface area of the nasal cavity devoid of the mucosa, and achieves greater nasosinusal functionality because proper reepithelialization occurs in the area.

Optimization of the Therapeutic Approach to Patients with Sarcoma: Delphi Consensus.

Soft tissue sarcomas (STS) constitute a heterogeneous group of rare solid tumors associated with significant morbidity and mortality. The evaluation and treatment of STS require a multidisciplinary team with extensive experience in the management of these types of tumors. National and international clinical practice guidelines for STS do not always provide answers to a great many situations that specialists have to contend with in their everyday practice. This consensus provides a series of specific recommendations based on available scientific evidence and the experience of a group of experts to assist in decision-making by all the specialists involved in the management of STS.

Primary Epidural Lumbar Ewing Sarcoma: Case Report and Review of the Literature.

STUDY DESIGN: Case report. OBJECTIVE: We present a case of isolated primary epidural lumbar Ewing sarcoma and review the current literature on the standard management. We also propose laminoplasty as safe procedure in this patient population that can provide good stabilization in young people. SUMMARY OF BACKGROUND DATA: Primary epidural Ewing's sarcoma is a very rare entity. The best generally accepted treatment option in sarcomas is to achieve a gross total resection with safe margins followed by local radiotherapy and chemotherapy. A total resection with safe margins is a great challenge in neurosurgical patients. METHODS: We present a previously healthy 17-year-old girl who complained of right sciatica with an epidural lumbar mass at L3-L4. She underwent complete resection of the tumor and a laminoplasty, which, in our experience, is a good way to preserve stability. RESULTS: At surgery, an isolated and noninvasive lesion was identified. Histopathological confirmation of Ewing sarcoma was obtained by immunohistochemical study and EWSR1 gene rearrangement detection. Treatment with 6 months of chemotherapy resulted in no further identifiable lesions by PET and MRI imaging at 4 years postsurgery. The laminoplasty has remained stable. CONCLUSION: Primary epidural Ewing sarcoma is extremely rare. The detection of the EWSR1 gene rearrangement can help to diagnose these tumors. The decision on how to treat these patients is difficult and can hardly be based on data from the current literature because of the small number of patients. The laminoplasty procedure can be safely performed in the setting of sarcoma of the epidural space.