The Clinical Research Of Pineal Region Tumors

Objective:Pineal region tumors have a great low incidence, but complicate in pathological types. On the base of the 2007 WHO classification of tumors of the central nervous system, pineal region can be divided into 4 types as follow:pineal parenchymal tumors, germ cell tumors, gliomas and other source tumors. Actually, different pathological has a great different treated strategy and leads to different follow-up. We summarize the treatment way about pineal region tumors nowadays, including operation, radiotherapy, chemotherapy and combination with the formers. Thus there is so much argument for it treatment strategy. So Through out investigating the clinical situation, neuroimaging, serum titer of tumors markers, treatment way, pathological distribution and follow-up, we want to find out clinical characteristic of pineal region tumors (PRTs) and treatment strategy.Methods:Part I Clinical pathological and treatment analysis of pineal region tumors.1. This study analyzed the clinical manifestations and pathology of 154 cases of pineal region tumors patients retrospectively, which were treated in Nanfang hospital during January 1999 to January 2009. It included 131 cases of operation group and 23 cases of non-operation group.2. Clinical data There were 118 males and 36 females, with a median age of 23.2 years. The rate of patients that had raised intracranial pressure, parinaud syndrome, incoordination, dysaudia, sexual precosity and dyspituitarism were 91.6%,40.9%,14.3%,5.8%,4.5%and 5.8%, respectively.3. Neuroimaging 94 patients had computerized tomography results. All patients had magnetic resonance imaging results.4. Serum titers ofα-fetoprotein,β-human chorionic gonadotropi and carcinoembryonic antigen 94 patients had the detection of serum titers of a-fetoprotein,β-human chorionic gonadotropi and carcinoembryonic antigen, including 71cases of operation group and 23 cases of non-operation group.5. Treatment Before tumor treatment,30 patinets were have treated because of serious hydrocephalus. In 131 cases of operation group,18 patients were treated radiotherapy before operation. The occipital transtentorial was the most common in our series.PartⅡAnalysis of suvival and prognosis factors of pineal region tumors1. All 154 cases of pineal region tumors in partⅠhave been included in the list of follow-up, with divided into two groups,131 cases of operation group and 23 cases of non-operation group.2. Clinical data and treatment The same as the partⅠ.3. Medical questionnaire design The prognosis of pineal region tumors may be related to gender, age, pathologic subtype, or WHO grade, extent of surgery, postoperative radiotherapy and chemotherapy in literature search. Therefore, we designed these follow-up survey form of prognostic factors of pineal region tumor.4. Follow-up The main methods of follow-up referral back to hospital, telephone and letter. Follow-up time from surgery date to death date. Non-operation group's start at the tumor treatment date. Censored time in January 2010. We adopt SPSS 13.0 statistic software to analysize the factors. Statistical methods:Fisher' exact test, Kaplan-Meier analysis and Cox regression.Results:1. Pathology In the cases which were treated by operation and had a complete pathological data, the histological diagnosis was germ cell tumors in 60 patients, pineal parenchymal tumors in 17 patients, gliomas in 27 patients and other resources tumors in 27 patients. Pure germ cell tumor, meningioma, teratoma and mixed germ cell tumors are more common than other pathological type of pineal region tumors. In the pineal region tumors, there are complex element with mixed germ cell tumors.2. Sex and age Sex ratio of the cases with germ cell tumors, pineal parenchymal tumors, gliomas and other resources tumors were 19.75:1 (Male:Female),2.4:1, 1.1:1 and 0.8:1, respectively. The median age was 15.7,24.7,27.4 and 41.2 years, respectively.3. The serum titers of a-fetoprotein(AFP),β-human chorionic gonadotropin((3-HCG) and carcinoembryonic antigen(CEA) were elevated in 33 patients. Except for 1 case of metastatic tumors, the rest of these 33 cases are germ cell tumors.4. Surgical results In 131 cases of surgical excision of the cases, there were 124 cases of complete resection,7 cases of subtotal resection. Perioperative death was 2 cases, mortality rate 1.5%. After surgery, there were 19 cases of intracranial hypertension,6 cases of intracranial hematoma.104 patients were good recovery.5. Neuroimaging Results In CT, germinoma showed high or medium high density. Most can be found in tumor calcification, or calcification of the pineal region. Teratoma boundary more clearly, heterogeneous density, more common cystic change, all cells showed calcification. Hematoma was common in choriocarcinoma. In MRI, germinoma show increased significantly, signal uniformity. Most tumors were solid, and some may be sign with typical butterfly sign. Teratoma and mixed germ cell tumor showed mixed signal, more common in cystic and fat signal.6. Follow-up results 23 cases were lost(18.2%) in 154 cases. Except 2 patients died in 1 month after opertation,131 patients were followed up for a length of 13 months to 119 months, with an average follow-up time of 51.6 months.7. Results of clinical symptoms and relapse During follow-up, the original nerve function improved.16 cases of tumor recurrence(the recurrence rate of 14.6%) in surgery group.6 cases of tumor recurrence(the recurrence rate of 27.3%) in non-surgery group. It showed no significant difference in recurrence rate of the two groups.8. Survival analysis Surgical excision of the cases (n= 109) in the 5-year survival rate was 81.0%. The 5-year survival rate in germ cell tumors, pineal parenchymal tumors, gliomas and other resources tumors were77.2%,83.6%,82.0%and 88.7%, respectively. These four group were no significant difference in survival curve. The 5-year survival rate in surgery group and non-surgery group were 94.4%and 88.9%, respectively, with on significant difference.9.Results of Cox regression In germ cell tumors, it showed direct operation better than first radiation cases, Radiotherapy and chemotherapy better than those without it, surgical resection of germinoma cases better than other germ cell tumors group.Conclusions:1,There are various types of pathology of pineal region tumors. In these datas, the proportion of germ cell tumors, pineal parenchymal tumors, gliomas and other resources tumors are 45.8%,13%,20.6%and 20.6%, respectively. Germ cell tumors patients are more common in male, with average age 15.7 years. The peak age range was 10-20 years old.2,Complex pathological types of pineal region tumors has brought a lot of difficulties of diagnosis. And different pathology significantly affected the prognosis. Undoubtedly, the most accurate pathological diagnostic result come from the whole tumor specimen which total resection of pineal region tumors gain. However, the clinical manifestations, serum tumor markers and neuroimaging lack of specificity to diagnose the pathological subtypes. Tumor markers in the serum and cerebrospinal fluid can divide germ cell tumors from non-germ cell tumors. But it can not further correctlydistinguish the subtypes.3,It is important to treat obstructive hydrocephalus which most pineal region tumors patients have. Preoperative hydrocephalus should not be treated too early. If symptoms is not heavy, postoperative hydrocephalus can be treated with dehydration. If not alleviated, we suggested treating hydrocephalus by endoscopic third ventriculostomy.4,Aggressive surgical treatment supplemented by radiotherapy and chemotherapy is the main treatment project of pineal region tumors. With neurosurgical microscope developing, surgery complications and mortality of pineal region tumors has been reduced to an acceptable level. The perioperative mortality rate in this data was 1.5%. Total resection, postoperation radiotherapy and chemotherapy are the protective factors. Preoperation radiotherapy is the risk factors. By complete surgical resection, benign tumors, such as mature teratoma, pineal gland tumors and meningiomas, were completely cured. For malignant tumors, we also try to complete remove the tumor, combining radiotherapy and chemotherapy after operation. Except to highly malignant germ cell tumor, the other patients who had total resection have had a better prognosis.5,There is no significant difference in survival curves between operation group and nonoperation group of germinomas. If you can highly suspect the patient's diagnosis was germinomas, direct radiotherapy and chemotherapy is a good choice. But if you can not determine the pathology, surgical resection is still the preferred treatment.6,Summarizes the initial diagnosis of pure germ cell tumors experience:1.10 to 25 years old male patient; 2. In MRI examination, germinomas are iso-signal areas on T1 and T2 and homogeneously enhanced significantly. The typical butterfly sign or the existence of multiple lesions always appear in MRI of pure germinomas; 3.β-hCG and AFP in blood and cerebrospinal fluid were negative; 4. There can be as far as possible to make biopsy if any doubt; 5. Recheck head MRI at postradiotherapy one month later. If tumor is not decreaded obviously or even increased, this patient must be operated. If tumors significantly reduced, recheck head MRI after three month. And if the tumor did not completely disappear after three months, operation can not be avoided.