Tumors of neuroepithelial origin are extremely rare in teratoma and tend to be derived from glial or primitive neuroectodermal cells. We describe a case of 2- month-old baby girl with an oligodendroglioma arising in an immature teratoma of the sacrococcygeal region. Histologically, the tumor was identical in appearance to low grade oligodendroglioma within the adult brain. Because immature teratoma was grade II, the patient received adjuvant chemotherapy. The patient died of progression of the intra-abdominal tumor 6 months after surgical excision. The authors believe this to be the first presentation in the world literature.
Fatal Outcome ; Female ; Humans ; Infant ; Oligodendroglioma/*pathology ; Sacrococcygeal Region/*pathology ; Spinal Neoplasms/*pathology ; Teratoma/*pathology

BACKGROUNDPresacral tumors are highly infrequent tumors located in the space known as presacral or retrorectal space. Although there have been substantial improvements in the prognosis of patients with malignant presacral tumors, the development of newer surgical strategy is likely to further improve the oncologic outcomes of malignant presacral tumors. The aim of this article was to report our experience in 33 cases, and to review the surgical strategy, pathological features and the prevention of complications from our experience.

METHODSA retrospective analysis was conducted on 33 cases (20 male and 13 female) with presacral tumors surgically treated in our hospital between January 1998 and April 2009. The surgical approaches included trans-abdominal in 10 cases (30%), trans-sacral in 18 cases (55%) and combined abdominal-sacral in 5 cases (15%). All patients got followed up (14 - 123 months, mean of 45.1 months). At last, the general information, clinical symptoms, histodiagnosis, surgical types and postoperative complications of all cases in our series were assessed.

RESULTSAges of 33 patients ranged from 18 to 71 years, with an average of 48.5 years.

PATHOLOGICAL FINDINGS6 epidermoid cysts, 5 teratomas, 3 leiomyomas, 9 neurofibromas, 5 neurilemmomas, 1 enterogenous cyst, 1 liposarcoma, 1 leiomyosarcoma, 1 angiosarcoma, and 1 neurofibrosarcoma. All tumors were excised with no perioperative death. A colostomy was taken in one case with angiosarcoma involving the rectum because of the intraoperative injury of the rectum. Blood loss during surgery was 400 - 11 000 ml (mean of 2400 ml). Four (12%) cases had local recurrence during follow-up: 2 because of inadequate drainage after dermoidectomy, both of them were cured by surgical resection and drainage; recurrence occurred in a case of teratoma in 18 months after surgery, cured by a trans-sacral excision; local recurrence and lung metastasis occurred simultaneously in a case of angiosarcoma in 6 months postoperatively and the patient died one month later of respiratory failure.

CONCLUSIONSThe main treatment of most presacral tumors is surgical resection. Selection of surgical approach is very important for complete resection of the presacral tumors. The location, size and peculiarities of tumors, conditions of the skin and soft tissues and the patients' somatotype are all determinative factors. Multidisciplinary cooperation is also very necessary.

Adolescent ; Adult ; Aged ; Female ; Humans ; Male ; Middle Aged ; Retroperitoneal Neoplasms ; pathology ; surgery ; Retrospective Studies ; Sacrococcygeal Region ; pathology ; surgery ; Young Adult

Although sacrococcygeal mass is rare and usually found in infants or children, adolescent or adult patients with protruding mass in sacrococcygeal region occasionally come to us simply for a cosmetic problem. In this situation, even though there is no definite neurological deficit, it should be evaluated whether or not the underlying bony pathology or dural defect exists. Few cases about the sacrococcygeal mass have been reported in adults. We reviewed our cases including preoperative evaluation methods and postoperative diagnosis. From March, 1993 to February, 1997, we experienced 6 adult patients with sacrococcygeal mass and no neurological abnormality. Preoperative evaluation were made by plain X-ray, myelogram, computed tomography(CT), and magnetic resonance imaging (MRI), as needed. Postoperative diagnoses were 2 meningoceles, 2 lipomyelomeningoceles, 1 desmoid tumor, and 1 teratoma. From our experiences, CT or MRI is essential to evaluate the sacrococcygeal mass preoperatively. These methods can visualize the precise anatomic location and extent of the mass, its relation to the spinal cord, and associated bony abnormalities. MRI is superior to CT, especially in defining the nature of the mass and involvement of the spinal cord. Conclusively, even a simple mass in the sacrococcygeal region in adults needs MRI or CT evaluation, and MRI is the most valuable method of evaluating the mass preoperatively and provides important information to establish a treatment plan.
Adolescent ; Adult* ; Child ; Diagnosis ; Fibromatosis, Aggressive ; Humans ; Infant ; Magnetic Resonance Imaging ; Meningocele ; Pathology ; Sacrococcygeal Region* ; Spinal Cord ; Teratoma

Primary cardiac tumors are extremely rare and can originate within the heart or be the result of tumor spread from other sites. We report a female patient with a pulmonary vein tumor extending into the left atrium that had a suspicious primary malignant origin with a sacral metastatic carcinoma. The patient was admitted complaining of pain in her buttock area as a result of a sacral tumor. It was believed that the sacral tumor was a metastasis from the imaging study and clinical manifestation. The primary malignant origin was evaluated. The chest CT showed a left atrium thrombus-like lesion without a pulmonary abnormality. After a transesophageal echocardiogram, the patient was diagnosed with a pulmonary vein tumor extending to the left atrium. The patient was given palliative radiotherapy for the sacral pain. Initially, the clinical impression was a metastatic sacral tumor with a thromboembolism of the left atrium. However, this patient was finally diagnosed with a pulmonary vein tumor with a left atrium extension by a transesophageal echocardiogram.
Vascular Neoplasms/*diagnosis/pathology ; Thromboembolism/diagnosis ; Sacrococcygeal Region/pathology ; Pulmonary Veins/*pathology ; Palliative Care ; Humans ; Heart Neoplasms/*diagnosis/pathology ; Heart Atria/*pathology ; Female ; Diagnosis, Differential ; Aged

OBJECTIVETo evaluate the efficacy of transsacral local wide resection for mid-lower rectal tumors.

METHODSClinical data of 133 patients undergone transsacral local wide resection for mid-lower rectal tumors between September 1994 and September 2005 were analyzed retrospectively.

RESULTSNo patient died during operation. Fecal fistula occurred in 6(4.5%) patients. Negative resection margin was proved histologically in all the patients. Postoperative diagnosis was adenoma in 28 patients, hyperplastic polyp in 3 patients, carcinoid in 8 patients, gastrointestinal stromal tumor in 1 patient,adenoma with intra-mucosal carcinogenesis in 29 patients and adenocarcinoma invading into submucosa in 64 patients. Median follow-up was 76 months in 64 patients with T(1) adenocarcinoma, whose 5-year cumulative local recurrence and overall survival were 2.0% and 100% respectively. No local recurrence was observed in other patients.

CONCLUSIONTranssacral local wide resection is simple and safe for mid-lower rectal tumors, which is an appropriate procedure for mid-lower rectal benign tumor and can serve as a sphincter-saving operation for selected T(1) lower rectal carcinoma.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Male ; Middle Aged ; Neoplasm Staging ; Rectal Neoplasms ; pathology ; surgery ; Retrospective Studies ; Sacrococcygeal Region ; surgery ; Young Adult

Tailgut cysts (TGCs) are rare congenital cysts that occur in the retrorectal or presacral spaces. Although most tailgut cysts have been reported as benign, there have been at least 9 cases associated with malignant change. We report herein on an unusual case of a 40-year-old woman with a carcinoembryonic antigen (CEA) -producing adenocarcinoma arising within a TGC who underwent surgical resection and local radiation therapy. Despite the complete resection, metastatic adenocarcinoma developed five months after surgery. CEA-producing adenocarcinoma from a TGC is extremely rare and only two cases, including this case, have been reported in the English medical literature. Besides CEA, the serum levels of CA 19-9 became markedly elevated in this patient. Given that the serum CEA level decreased to the normal range after complete resection of tumor and that the tumor recurrence was associated with a rebound of the CEA serum level, our case shows that serial measurements of serum CEA can be used for treatment planning and for assessing the patient's treatment response for this rare disease.
Adenocarcinoma/blood/pathology/*therapy ; Adult ; CA-19-9 Antigen/blood ; Carcinoembryonic Antigen/*blood ; Cysts/blood/pathology/*therapy ; Female ; Hamartoma/blood/pathology/*therapy ; Humans ; Rectal Neoplasms/blood/pathology/*therapy ; Sacrococcygeal Region

Chordoma ; metabolism ; pathology ; surgery ; Disease Progression ; Fatal Outcome ; Humans ; Immunohistochemistry ; Keratins ; analysis ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Mucin-1 ; analysis ; Neoplasm Seeding ; S100 Proteins ; analysis ; Sacrococcygeal Region ; Spinal Cord Neoplasms ; metabolism ; secondary ; surgery

OBJECTIVETo evaluate the effectiveness and safety of fluoroscopy-guided percutaneous intratumor injection of pingyangmycin lipiodol emulsion (PLE) in the management of recurrent sacrococcygeal chordomas.

METHODSSeven patients with recurrent sacrococcygeal chordomas presenting with severe local pain with visual analogue score (VAS)≥8 received treatment sessions of fluoroscopy-guided percutaneous intratumor injection of PLE. The patients were followed up every 3 months after the last session to assess their clinical responses and observe the changes in the tumor size measured by computed tomography. The changes in the VAS, tumor necrosis and pain relief as well as the adverse events were recorded.

RESULTSA total of 22 sessions of fluoroscopy-guided percutaneous intratumoral PLE injection was performed in these cases (3 or 4 sessions in each case). The total average pingyangmycin dose delivered was 48.0 mg and the average lipiodol dose was 40.0 ml in each case. Five patients showed low fever and vomiting 48 after the injection. During the follow-up (median time of 21.7 months, range 10-26 months), all the patients showed obviously reduced tumor size and VAS, and partial remission was achieved in 6 patients and stable disease (SD) in 1 patient. None of the patients had complications during the follow-up.

CONCLUSIONFluoroscopy-guided percutaneous intratumoral injection of PLE can be effective and safe and may serve as a alternative for treatment of recurrent sacrococcygeal chordomas.

Adult ; Aged ; Bleomycin ; administration & dosage ; analogs & derivatives ; therapeutic use ; Chordoma ; drug therapy ; Emulsions ; administration & dosage ; therapeutic use ; Ethiodized Oil ; administration & dosage ; therapeutic use ; Female ; Humans ; Injections, Intralesional ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; drug therapy ; Sacrococcygeal Region ; pathology

OBJECTIVETo study the clinicopathologic features and biologic behavior of pediatric immature teratoma.

METHODSThe clinical data, pathologic features, immunohistochemical findings (for cyclin D1, P27 and Ki-67) and follow-up information of 39 cases of pediatric immature teratoma were analyzed.

RESULTSAmongst the 39 cases studied, 12 arose in the sacrococcygeal region, 12 in testis, 5 in retroperitoneum, 4 in ovary, 4 in abdomen and 2 in mediastinum. Histologically, 16 cases were of grade 1, 8 cases of grade 2 and 15 cases of grade 3. Seven of the cases contained foci of yolk sac tumor. Immature neuroepithelial features used in histologic grading included the presence of primitive neural tubules, immature rosettes, undifferentiated neuroblastoma cells and primitive neuroectodermal structures. Immunohistochemical study showed that cyclin D1 was positive in 3 cases of grade 1 tumors, 4 cases of grade 2 tumors and 9 cases of grade 3 tumors. The positivity rates for p27 were 8, 3 and 6 cases respectively, while those for Ki-67 were 3, 4 and 13 cases respectively. Follow-up data were available in 30 cases. Three of them, including 2 cases with histologic grade 3 (with or without yolk sac tumor component), recurred after operation.

CONCLUSIONSThe expression of cyclin D1 and Ki-67 is a useful adjunct in histologic grading. On the other hand, p27 overexpression shows little correlation with tumor grade. The prognosis of immature teratoma in children is different from that in adults. Sacrococcygeal immature teratoma occurring in patients younger than 1 year old and with low histologic grade do not require postoperative chemotherapy if the tumor is completely excised. Similarly, for testicular immature teratoma occurring in patients below 1 year of age, regardless of tumor grading, need no adjunctive therapy. On the other hand, ovarian immature teratoma with high histologic grade requires postoperative chemotherapy, regardless of age of the patients. The presence of microscopic foci of yolk sac tumor is a useful predictor of recurrence in pediatric immature teratoma.

Adolescent ; Cyclin D1 ; metabolism ; Endodermal Sinus Tumor ; drug therapy ; metabolism ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Ki-67 Antigen ; metabolism ; Male ; Mediastinal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Proliferating Cell Nuclear Antigen ; metabolism ; Retroperitoneal Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Sacrococcygeal Region ; Survival Rate ; Teratoma ; drug therapy ; metabolism ; pathology ; surgery ; Testicular Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; alpha-Fetoproteins ; metabolism