Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature

Pediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from pluripotent embryologic migratory neural crest cells. We report a 4-year-old boy who initially presented with a pelvic mass and inguinal lymphadenopathy at 6 months of age. Inguinal lymph node biopsy revealed a distinct biphasic tumor with microscopic and immunophenotypic characteristics diagnostic for both alveolar rhabdomyosarcoma and poorly differentiated neuroblastoma. The patient received national protocol chemotherapy against rhabdomyosarcoma with good response and presented with a cerebellar mass 21 months later. The metastatic tumor revealed sheets of primitive tumor cells and diagnostic areas of rhabdomyosarcoma and neuroblastoma were identified only by immunohistochemistry. Cytogenetic analysis of metastatic tumor demonstrated complex karyotype with multiple chromosomal deletions and duplications. The patient received national protocol chemotherapy against neuroblastoma and adjuvant radiotherapy after surgical resection of the cerebellar tumor with good response. He is currently off from any treatment for 18 months with no evidence of tumor recurrence or metastasis

Foot and ankle injuries during the Athens 2004 Olympic Games

<p>Abstract</p> <p>Background</p> <p>Major, rare and complex incidents can occur at any mass-gathering sporting event and team medical staff should be appropriately prepared for these. One such event, the Athens Olympic Games in 2004, presented a significant sporting and medical challenge. This study concerns an epidemiological analysis of foot and ankle injuries during the Games.</p> <p>Methods</p> <p>An observational, epidemiological survey was used to analyse injuries in all sport tournaments (men's and women's) over the period of the Games.</p> <p>Results</p> <p>A total of 624 injuries (525 soft tissue injuries and 99 bony injuries) were reported. The most frequent diagnoses were contusions, sprains, fractures, dislocations and lacerations. Significantly more injuries in male (58%) versus female athletes (42%) were recorded. The incidence, diagnosis and cause of injuries differed substantially between the team sports.</p> <p>Conclusion</p> <p>Our experience from the Athens Olympic Games will inform the development of public health surveillance systems for future Olympic Games, as well as other similar mass events.</p

Sustentaculoma: Report of a case of a distinctive neoplasm of the adrenal medulla

A case of a morphologically distinctive tumor of the adrenal medulla occurring in a 54-year-old woman is described. On microscopic examination, the tumor was well circumscribed and characterized by the presence of ill-defined, irregular nests of spindle cells with oval to elongated nuclei, tiny nucleoli, and abundant eosinophilic cytoplasm. The tumor was associated with a moderate infiltrate of lymphocytes and plasma cells with occasional lymphoid follicles. Necrosis, marked cellular atypia, and mitoses were absent. Immunohistochemical studies demonstrated the tumor cells to be strongly reactive for vimentin, S-100 protein, and CD56, and non-reactive for glial fibrillary acidic protein, chromogranin, synaptophysin, melanoma-associated antigens, and dendritic cell markers. Ultrastructural examination showed elongated cells with interdigitating cytoplasmic processes devoid of a basal lamina. No secretory granules were noted. The morphology, immunophenotype, and ultrastructure of this unique neoplasm suggest derivation from sustentacular cells of the adrenal medulla. We propose the designation sustentaculoma for this hitherto undescribed neoplasm of the adrenal gland. Copyright © 2006 by Lippincott Williams & Wilkins

Long-Term Survival with Primary Glioblastoma Multiforme: A Clinical Study in Bulgarian Patients

Glioblastoma multiforme (GBM) is the most common and aggressive primary brain tumor with an extremely poor prognosis in spite of multimodal treatment approaches. The estimated median survival in cases with GBM is about 12–16 months. Those patients who survive ≧3 years after the initial diagnosis are defined as long-term survivors. In this study, we retrospectively analyze 50 consecutive cases of Bulgarian patients with newly diagnosed GBM surgically treated at our institution for a period of 1 year. Four of them survived for more than 36 months after the initial intervention. The histological re-examination revealed features typical of primary GBM in 3 of these cases, which are described in detail in the present paper. A brief review of the relevant literature is also given

Navigational guidance in transsphenoidal pituitary adenoma surgery

Objective: To assess whether frameless stereotaxy can further increase safety and efficacy of transsphenoidal microsurgery.Methods: We conducted a retrospective analysis of 29 patients with pituitary adenomas (10 recurrent, 12 micro, 17 macro), who had undergone image guided endonasal transsphenoidal surgery during an 18-month period in the Department of Neurosurgery, Medical University - Sofia, Bulgaria. In the preoperative planning process, the adenoma volume and both carotid arteries were segmented in a MRI/CT-3 D dataset (T1-weighted, 3 D FLASH after Gadolinium). An optical infrared-based neuronavigation system (Vector Vision, BrainLAB®, Heimstetten, Germany) was used in all cases for frameless guidance. Using Z-touch infrared markerless or landmark registration (in 3 cases) a mean calculated accuracy of 1.47±0.4 mm was achieved; intraoperative accuracy was checked every 10 min. Intraoperative records were reviewed with attention to the utility of the navigational guidance.Results: The time requirements for set-up, registration and navigational control were minimal (<16 min). In none of the cases the system did interfere with surgical manipulations. True accuracy at surgery was obtained in 29/30 cases. A mean calculated accuracy of 1.47±0.4 mm was achieved during co-registration (1.45±0.69 mm for MRI-based NN and 1.53±0.64 mm for CT-based guidance), which is in concordance with the data from the literature, using similar navigational system , . The frameless technique was used to determine the midline, the depth and trajectory of the approach, as well as to reduce safely working area (in 29/29); in later stages, it provided fast and correct anatomical orientation in relation to the perisellar structures (24/29). Guidance was especially useful in asymmetrical/atypical microsella (in 9/9 pts), in conchal type of sella (3/3 cases), and helpful in locating accurately eccentric microlesions. Conclusions: We found frameless guidance during transsphenoidal surgery useful in certain occasions: misleading sphenoidal sinus anatomy, narrow/asymmetrical sella, eccentric adenomas with perisellar extension/distortion and re-operations for recurrent/residual tumors with obscured bony landmarks. In macroadenomas, however, the accuracy and reliability of the technique are compromised after debulking due to movements of the adenoma capsule