PURPOSE: A sacral chordoma is a rare, slow-growing, primary bone tumor, arising from embryonic notochordal remnants. Radical surgery is the only hope for cure. The aim of our present study is to analyse our experience with the challenging treatment of this rare tumor, to review current treatment modalities and to assess the outcome based on R status. METHODS: Eight patients were treated in our institution between 2001 and 2011. All patients were discussed by a multidisciplinary tumor board, and an en bloc surgical resection by posterior perineal access only or by combined anterior/posterior accesses was planned based on tumor extension. RESULTS: Seven patients underwent radical surgery, and one was treated by using local cryotherapy alone due to low performance status. Three misdiagnosed patients had primary surgery at another hospital with R1 margins. Reresection margins in our institution were R1 in two and R0 in one, and all three recurred. Four patients were primarily operated on at our institution and had en bloc surgery with R0 resection margins. One had local recurrence after 18 months. The overall morbidity rate was 86% (6/7 patients) and was mostly related to the perineal wound. Overall, 3 out of 7 resected patients were disease-free at a median follow-up of 2.9 years (range, 1.6-8.0 years). CONCLUSION: Our experience confirms the importance of early correct diagnosis and of an R0 resection for a sacral chordoma invading pelvic structures. It is a rare disease that requires a challenging multidisciplinary treatment, which should ideally be performed in a tertiary referral center.
Chordoma* ; Cryotherapy ; Diagnosis ; Diagnostic Errors* ; Follow-Up Studies ; Hope ; Humans ; Notochord ; Perineum ; Rare Diseases ; Recurrence ; Sacrum ; Tertiary Care Centers ; Wounds and Injuries

We present a rare case of a gastric mixed adenoneuroendocrine tumor and review the related English literature. A 77-year-old Caucasian woman was admitted to our department with nausea, anorexia, weight loss, and anemia. Esophagogastroduodenoscopy showed a large (>7 cm) ulcerative mass in the greater curvature of the stomach. Biopsy showed the presence of an adenocarcinoma with moderate differentiation. The patient underwent D2 subtotal gastrectomy. Histopathological analysis revealed a diagnosis of mixed gastric adenoneuroendocrine carcinoma. The post-operative course was uneventful, and at the 6-month follow-up, the patient was alive without evidence of recurrence. Our review of the English literature suggested that such cases are most often reported from eastern countries. Multimodal treatment should be the aim for these patients because of the neuroendocrine component of the tumor.
Adenocarcinoma ; Aged ; Anemia ; Anorexia ; Biopsy ; Combined Modality Therapy ; Diagnosis ; Endoscopy, Digestive System ; Female ; Follow-Up Studies ; Gastrectomy ; Humans ; Nausea ; Recurrence ; Stomach ; Stomach Neoplasms ; Ulcer ; Weight Loss