Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of "1", rather than a benign tumor of "0".
Osteoblastoma is a benign bone-forming neoplasm that occurs commonly in the posterior elements of the spine and the sacrum. However, so far there has been no report of intradural osteoblastoma described in the literature. We present a unique case of intraspinal dural-based osteoblastoma with aneurysmal bone cyst-like change without evidence of vertebral involvement. An 11-year-old Chinese girl presented with a 3-month history of gradually progressive back pain and a weakness of both lower limbs. Thoracic MRI revealed a well-demarcated subdural mass at the T5 level with heterogeneous enhancement. Histologically, the tumor was found to be attached to the dura and composed of numerous osteoid spicules and trabecular bone with diffusely scattered osteoclast-type, multinucleated giant cells. Ectactic blood vessels and blood-filled cystic spaces were also observed. A diagnosis of primary intraspinal dural-based osteoblastoma with aneurysmal bone cyst-like change was made. To our best knowledge, this is possibly the first case of primary osteoblastoma arising from meninges. Meningeal osteocartilaginous tumors are rare, with obscure histogenesis. The differential diagnosis of osteoblastoma in unusual locations is difficult and the confirmation of diagnosis should be cautiously made. Awareness of dural-based osteoblastoma and its histological features is important to avoid a diagnostic pitfall caused by histological similarities to other intra-craniospinal lesions with osteoid differentiation or bone formation.
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