骶尾部脊索瘤肢体多发性转移治疗一例报道(英文)

Chordoma is rare,locally aggressive malignant neoplasm originated from notochord remnants.Distant extrapulmonary metastases are rare and mostly occur in patient with local recurrence.In this case report,2 years after the radical resection of sacral chordoma,two times successive metastases to the upper limb were diagnosed by immunohistochemistry,without local recurrence.The metastatic tumors were radical excised,and the patient gained radiotherapy postoperatively.More than 2 years after the wide resection of the metastatic focuses,no sign of recurrence and metastasis was detected.     

原发性骶骨脊索瘤的手术治疗

目的 介绍原发性骶骨脊索瘤手术治疗的初步经验。方法 采用前后路联合入路切除或刮除术治疗原发性骶骨脊索瘤8例，局部病灶清除减压治疗复发性骶骨脊索瘤1例。结果 7例术后2.5～4年获得随访，均无瘤正常生存，1例失访。1例死亡。结论 早期前后路联合入路手术是治疗骶骨脊索瘤的较好方法。对于脊索瘤术后复发或转移，施行姑息性病灶清除减压术，有利于减轻患者痛苦。     

Recurrent Metastatic Chordoma to the Liver: A Case Report and Review of the Literature

Chordoma is a rare malignant neoplasm derived from notochordal tissue that primarily affects the axial skeleton. Almost 40% of patients have non-cranial chordoma metastases. The most common metastatic sites are the lungs, bones, lymph nodes, and subcutaneous tissue. We present a 52-year female with a history of sacral chordoma presenting with abdominal fullness, early satiety, and a palpable abdominal mass. Abdominal magnetic resonance imaging (MRI) revealed an isolated, highly vascularized, and multilobed liver mass in the left lateral segment. The mass was surgically removed using a clean surgical margin. A histological examination and immunohistochemical staining were consistent with a metastatic chordoma. Two years later, follow-up imaging studies showed a 6.5 × 4.0 × 2.0 cm right liver lesion with multiple lungs, chest wall, pleural, and diaphragmatic lesions. Microscopic- and immunohistochemical staining revealed a recurrent metastatic chordoma. Herein, we present a unique case of metastatic recurrent chordoma in the liver with the involvement of other sites. To the best of our knowledge, no other case of recurrent liver metastasis has been reported.     

Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma

BACKGROUND: Chordomas are rare tumors of the skull base and the spine. Treatment is difficult since conventional radiotherapy or chemotherapy have only limited effects. PATIENT AND METHODS: A patient with a sacral chordoma and pulmonary metastases received initial surgery and radiotherapy for a local recurrence. After 11 months another local recurrence as well as a progression of the pulmonary metastases was documented. As tumor biopsies revealed the expression of the EGF receptor an individual treatment approach with a combination of cetuximab and gefitinib was performed. RESULTS: Under the treatment with cetuximab/gefitinib the local recurrence and the pulmonary metastases showed a partial response over the follow-up period of 9 months. To date no treatment failure was observed. CONCLUSIONS: The inhibition of the EGF pathway seems to be an effective measure in the treatment of a chordoma. Further follow-up will have to prove the long-term efficiency.     

Radiofrequency ablation in palliative supportive care: early clinical experience

We report our early experience with radiofrequency ablation (RFA) in palliative supportive care. The medical files of eight patients were retrospectively reviewed. Four patients had a renal tumor, and nephrectomy was contraindicated in each patient since they had a poor general status. The fifth patient had a local recurrence in the site of a previous nephrectomy with a pancreatic tail extension, and surgical resection was contraindicated because of abdominal carcinomatosis. Two other patients had bone metastasis, one with a painful metastasis of mammary carcinoma in the head of the humerus resistant to radiotherapy, and the other with metastasis of the tibia of cutaneous melanoma. The last patient had a local recurrence of a sacral chordoma. Management, outcomes and complications were evaluated with 13.1+/-0.3 months follow-up. All five patients with renal carcinomas did not have local recurrence. The two patients treated for bone metastases had no pain 8 weeks after RFA and remained stable over time. One complication occurred 2 months after using the procedure to treat the chordoma, and this patient was hospitalized for a fistula between the sigmoid and hypogastric artery false aneurysm and subsequently died. In conclusion, RFA can be a safe and useful adjuvant treatment in supportive care or unresponsive cancer pain patients. However, the destruction of tumoral tissues in contact with sensitive structures using RFA should be done with caution due to potentially severe complications.     

Long-term control of multiple lung metastases from osteosarcoma obtained by conventional radiotherapy: a case report.

An osteosarcoma patient whose lung metastases were successfully treated with radiotherapy is reported. The patient developed multiple lung metastases repeatedly after successful treatment of the primary lesion in his femur. Resection of the lung metastases was performed 5 times during 4 years (12 metastatic nodules in total). One of the specimens was evaluated using the micronucleus test, which suggested moderate radiosensitivity of the tumor. He further developed 2 metastases 19 months after the last operation, and these tumors were treated with conventional radiation with 60 and 64 Gy, respectively. The tumors regressed slowly after radiation and disappeared later. Although he developed another lung metastasis 2 years later, the lesion was also treated with radiation with 66 Gy and all of the 3 irradiated tumors are now under control at 43 months after the first radiotherapy and 15 months after the second one. Our experience would suggest that some (at least) metastatic osteosarcomas have reasonable radiosensitivity, and radiation therapy including radiosurgery may be a good alternative to surgery in patients for whom operation is not indicated.     

Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients

BACKGROUND: The prognosis of patients with chordoma of the sacrum and mobile spine has been reported to be dismal and attributable in the majority of cases to intralesional surgery. The purpose of this study was to evaluate the clinical outcome of these patients using modern surgical principles aimed at complete resection and to identify prognostic factors. METHODS: The clinical and morphologic features, type of surgery, and follow-up of 39 consecutive patients with chordoma were reviewed and analyzed statistically. RESULTS: Thirty sacral and 9 mobile spine chordomas (size range, 3-20 cm; mean, 8 cm) occurring in 22 women and 17 men (median age, 55 years) were analyzed. The preoperative morphologic diagnosis was based on fine-needle aspiration (FNA) biopsy, core needle biopsy, or incisional biopsy. The final surgical margins were wide in 23 patients and marginal or intralesional in 16. The mean follow-up was 8.1 years (range, 0.1-23 years). Seventeen patients (44%) developed local recurrences and 11 patients (28%) developed metastases. The estimated 5-, 10-, 15-, and 20-year survival rates were 84%, 64%, 52%, and 52%, respectively. Local recurrence was associated significantly with an increased risk of metastasis and tumor-related death (P < 0.001). CONCLUSIONS: New surgical techniques have improved local control and survival of patients with sacral or spinal chordoma significantly and have decreased progressive neurologic deterioration. Larger tumor size, performance of an invasive morphologic diagnostic procedure outside of the tumor center, inadequate surgical margins, microscopic tumor necrosis, Ki-67 > 5%, and local recurrence were found to be adverse prognostic factors. FNA is the preferred method for establishing the preoperative morphologic diagnosis of chordoma.     

Primary malignant fibrous histiocytoma of the lung. A clinicopathologic and ultrastructural study of five cases

Primary sarcomas of the lung are rare malignant neoplasms which have usually been classified as fibrosarcomas or as leiomyosarcomas. Only five cases of primary malignant fibrous histiocytoma (MFH) of the lung have previously been reported in the literature. The authors reviewed 10 cases of primary pulmonary sarcoma of the lung seen at their institution during the last 12 years, and five of these cases met the morphologic criteria for the diagnosis of MFH. The morphology in these cases is identical to MFH of other body locations, and shows a combination of fibroblast-like and histiocyte-like cells at both the light and electron microscopic level. By electron microscopic examination there was no evidence for anaplastic carcinoma, rhabdomyosarcoma, or leiomyosarcoma. Of the three patients with adequate follow-up, one patient is alive without evidence of disease at 10 years, one died at 5 years without evidence of recurrent sarcoma, and one died at 7 months with liver metastasis. Two other patients are alive without recurrence at 8 months and 1 year, respectively, following surgery. Because MFH is primarily a tumor of the soft tissues of the extremities, patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin. The reported prognosis in patients with primary MFH of the lung is poor, but early surgical resection does offer the possibility of a cure.     

Radiotherapy of chordoma--a report of 10 patients

A consecutive series of 10 patients with chordoma was treated in our hospital from 1956 to 1982. Six were male and four were female. The age ranged 19 approximately 52 years. Eight lesions occurred in the base of sphenoid and two in the sacrum. All were treated by postoperative radiotherapy with a medium dose of 4,500 approximately 6,000 rad. Recurrent patients were given a hyperfractionated radiotherapy (100 rad, 4/D, at intervals of 3 hours, total dose 2000 rad). The 5 and 10 year survival rates were 75% and 50%. No complications were found after radiation. In this paper, literature on chordoma and radiotherapy for chordoma are reviewed. An optimal schedule is proposed: operation be the choice of treatment, which be as thorough as possible; two months after the operation, radiotherapy be given at a suitable dose of 5000 approximately 6000 rad; the recurrent lesions be treated by hyperfractionated radiotherapy.     

Expression of insulin-like growth factor II mRNA-binding protein 3 (IMP3) in sacral chordoma

Sacral chordoma is a rare and aggressive tumor, with a high rate of local recurrence even when the tumor is radically resected. The fundamental knowledge of its biological behavior remains unknown. Insulin-like growth factor II mRNA-binding protein 3 (IMP3) is one of the RNA binding proteins and is expressed during embryogenesis and in various malignant tumors. This study evaluated expression of IMP3 in sacral chordoma for association with patient’s clinicopathological factors. A total of 32 patients with sacral chordoma (17 male and 15 female) and 10 samples of distant normal tissues were collected for analysis of IMP3 expression using immunohistochemistry. Association between IMP3 expression and clinicopathological factors (such as patient’s age, gender, tumor location, tumor size, surrounding muscle invasion, Ki-67 expression, and tumor recurrence) were statistically analyzed. IMP3 was expressed in 20 (62.5 %) patients, whereas there was no expression in the 10 distant normal tissues. IMP3 expression was associated with tumor invasion into the surrounding muscle (P = 0.028), high levels of Ki-67 expression (P = 0.009), and tumor recurrence (P = 0.012). The log-rank test revealed that patients with positive IMP3 expression had a shorter continuous disease-free survival time than those with negative IMP3 expression (P = 0.016). IMP3 expression was independent of age, gender, tumor location and tumor size. These results indicate that IMP3 was overexpressed in sacral chordoma and this expression was associated with tumor invasion and recurrence; thus, IMP3 may play an important role in tumor progression and could serve as a prognostic biomarker for sacral chordoma and IMP3 could be used as a potential therapeutic target for the treatment of sacral chordoma.     

Pulmonary metastasectomy for osteosarcomas and soft tissue sarcomas

To evaluate the efficacy of aggressive pulmonary metastasectomy for treating osteosarcomas and soft tissue sarcomas, we reviewed 105 cases treated in our hospital between 1990 and 2002. There were 57 males and 48 females, 44 osteosarcomas (OS), 21 synovial sarcomas (Syno), 16 malignant fibrous histiocytomas (MFH), 4 leiomyosarcomas (Leio), 4 alveolar soft part sarcomas (ASPS) and 16 others (including chondrosarcoma and liposarcoma). A total of 904 metastases were resected (8.6(0-49)/patients) and 244 thoracotomies (2.3/patients) were performed. Mean number of initial metastasectomies was 3.7 (0-26), disease-free interval (DFI) was 13. 8 months (0-96), and mean diameter of maximum resected metastases for one patient was 20.4 (5-90) mm. Five-and 10-year survival rates of all cases were 44.9% and 32.0% respectively. Those of OS were 45.8% and 38.5%, and those of soft tissue sarcomas were 44.2% and 25.5%, respectively. The highest 5-and 10-year survival rates among soft tissue sarcomas was attained in ASPS and Leio (75%), and others (51.6% and 38.7%), followed by Syno (42.9% and 12.4%). Long-term survival was not attained in MFH (30.9%: 5 years). Analysis of histological types of the tumors and numbers of resected pulmonary metastases showed that the largest number of metastases were resected in ASPS (16/case) and Syno (13.8/case), followed by OS (9.0/case). MFH had the largest (27.1 mm) mean diameter of maximum resected metastases, followed by Leio (27.0 mm). Analysis of prognostic factors indicated that curativity was the most important prognostic factor: curative cases 42.2% (10-year survival) vs noncurative cases 4.2% (6-year survival). Number of resected metastases and mean diameter of maximum tumor size also affected the patient survival.     

Myxoid malignant fibrous histiocytoma with multiple primary sites

Malignant fibrous histiocytoma (MFH) is one of the most common types of soft tissue sarcomas in adults. The most common location of MFH are the extremities and the trunk, with the most common site for distant metastases being the lung. We describe a case with multiple synchronous sites of myxoid MFH but no lung metastases and presence of abnormalities of 19p13.     

骶骨脊索瘤外科治疗长期随访

目的 回顾骶骨脊索瘤外科治疗后长期随访的结果,分析评估影响外科治疗效果的临床因素.方法 1978年10月至2000年10月,我院共收治68例骶骨脊索瘤,男性60例,女性8例,年龄25～74岁,中位年龄55.5岁,部位为S1～5 7例,S2～5 15例,S3～5 33例,S4～5 5例,其他8例.外科手术共104例次,首次在我院外科治疗的原发病例48例,肿瘤外科切除边界为广泛切除4例（8.3%）;边缘切除21例（43.7%）;囊内切除23例（48.0%）;复发病例20例.结果 随访1-365个月,平均81.84个月.存活53例（77.9%）;死亡15例（22.1%）,其中,围手术期内死亡7例,占死亡总数的46.7%.总体5年生存率87.3%,10年生存率73.3%,中位生存时间（月）282.0±88.7.其中囊内切除者、边缘切除者和广泛切除者5年生存率,三者比较统计学无明显差异（P=0.18）.手术囊内切除者复发34例（81.0%）,边缘切除者复发8例（36.4%）,统计学差异明显（P=0.000）;总体1年无复发生存率77.4%;3年33.2%,5年24.3%.我院首次手术与非我院首次手术患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.边缘切除与囊内切除患者的无复发生存率比较,统计学差异明显（log-rank P=0.000）.Cox回归分析显示是否我院首次手术及不同手术边界均为预测局部复发的独立因素.结论 骶骨脊索瘤外科切除,局部复发率高,生存期较长,外科切除边界是影响局部复发的重要因素,首次手术对于预后有重要影响.     

CT引导下放射性粒子植入治疗恶性纤维组织细胞瘤肺转移瘤临床疗效研究

  目的  研究CT引导下经皮穿刺植入放射性125I粒子治疗恶性纤维组织细胞瘤术后肺转移瘤的临床疗效。  方法  选取自2006年1月至2011年8月在天津医科大学附属第二医院接受治疗的恶性纤维组织细胞瘤术后经病理明确诊断出现肺转移瘤的患者10例, 对转移病灶行CT引导下经皮穿刺肿瘤内放射性125I粒子植入治疗。术后6个月复查胸部CT, 与粒子植入前比较肿瘤变化, 按照国际标准判定疗效。随访自2006年6月至2012年6月。  结果  全部患者顺利完成粒子植入治疗, 靶区接受的平均照射剂量为(207.4±43.1)Gy, D100(99.7±10.6)Gy, D90(127.5±16.1)Gy。植入术后6个月随访, 有效率80.0%。截至2012年6月, 10例患者中2例生存, 8例死亡, 死亡患者术后平均生存时间14.6±3.5个月, 中位生存期13个月。术中6例患者出现气胸, 其中3例行胸腔闭式引流术, 3例行胸腔穿刺抽气; 6例患者出现肺内针道出血, 不伴咳血, 无进行性血胸, 止血处理后症状消失, 1个月后复查出血吸收。  结论  放射性粒子对于恶性纤维组织细胞瘤术后肺部转移病灶短期局部效果明显, 可以作为一种有效的局部治疗手段。      

Particle beam radiotherapy with a surgical spacer placement for unresectable sacral chordoma

Sacral chordomas constitute more than half of all chordomas and have a slower local growth than other bone malignant tumors. Although complete radical resection produces a longer local control and disease-free survival at the initial visit, chordomas are already often too large for complete resection to be possible. Particle radiotherapy consisting of proton and carbon-ion is a promising new modality which has an inherent anti-tumor effect against many types of malignancies. However, the application of particle radiotherapy for tumors adjacent to the gastrointestinal tract like sacral chordoma is restricted because the tolerance dose of the intestine is extremely low. A novel two-step treatment was developed with surgical spacer placement and subsequent proton radiotherapy to administer particle radiotherapy with curative intent. This report presents a case of a patient with a huge sacral chordoma treated by this method. This new strategy may potentially be an innovative and standard therapy for unresectable sacral chordoma in the near future.     

A case of postoperative recurrent breast cancer with multiple lung metastases that completely responded to combination therapy of docetaxel (TXT) and medroxyprogesterone acetate (MPA)

A 54-year-old female had undergone surgery for breast cancer 5 years ago, after which she developed multiple lung metastases, in spite of treatment with various postoperative chemoendocrine therapies. The patient who had recurrent breast cancer with multiple lung metastases was treated with a combination of docetaxel (TXT) 80 mg/body three cycles, two courses of 40 mg/body four times and medroxyprogesterone acetate (MPA) 600 mg p.o. daily. Six months later, the lung metastases had completely disappeared on chest CT-scan. Complete remission has been maintained for one and half years. The use of combined chemoendocrine therapy with TXT and MPA is considered effective for recurrent breast cancer as second-line therapy.     

Expression of vascular endothelial growth factor and matrix metalloproteinase-9 in sacral chordoma

Sacral chordoma is a vessel-rich and infiltrative tumor, but the fundamental knowledge of its biological behavior remains unknown. This study was designed to investigate the expression levels and contributions of vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 (MMP-9) in the angiogenesis and recurrence of sacral chordoma and their correlations. An immunohistochemical method was used to investigate the expression of VEGF, MMP-9, and microvascular density (MVD) in 36 patients with sacral chordoma. Their differences in expressions were statistically analyzed and their correlations with angiogenesis and recurrence were evaluated. The mean MVD of sacral chordomas was significantly higher than that of the adjacent normal tissues (P = 0.033). Immunoreactivity for VEGF and MMP-9 was significantly higher in sacral chordoma tissues than in adjacent normal tissues (P = 0.008, P = 0.005). The mean MVD of VEGF and MMP-9 were statistically higher in positive group than in negative group (P = 0.015, P = 0.004), respectively . Moreover, a significant correlation was found between the VEGF and MMP-9 (P = 0.002). The log-rank test revealed that continuous disease-free survival time (CDFS) was significantly shorter in the MMP-9-positive group than in the MMP-9-negative group (P = 0.019), but the difference in the VEGF-positive group and the VEGF-negative group was not statistically significant (P = 0.938). Our data suggest that VEGF and MMP-9 might act with a synergistic effect and can positively regulate the angiogenesis in sacral chordoma. Positive expression of MMP-9 might indicate the local recurrence of sacral chordoma. The result suggests that some specific drugs which inhibit VEGF, MMP-9, or their receptors may have a good therapeutic effect for sacral chordoma.     

Sacral chordomas: A 10-year study

A retrospective analysis of 14 cases of sacral chordoma seen between 1984 and 1993 at the Kidwai Memorial Institute of Oncology is reported. The mean patient age was 54 years, with 13 males and one female. Seventy-one per cent of patients presented with symptoms related to a painful sacral mass. Eight patients underwent radical surgery and two had adjuvant radiotherapy. One patient had a partial tumour resection. Three patients received radical radiotherapy. Two patients were offered only pain relief medication. The median follow-up was 33 months. The actuarial overall survival (OS) at 5 years was 48%. In the 12 patients in whom curative therapy was attempted, the 5-year OS was 56% and progression-free survival was 36%. Four patients developed local recurrence and three developed distant metastasis.     

Two-year survivor in response to gemcitabine-based chemotherapy for advanced pancreatic cancer with multiple lung metastases

A 65-year-old woman was admitted to our hospital for appetite loss and jaundice. Abdominal CT scan and ultrasonography both revealed a tumor in the head of the pancreas. Chest CT scan showed multiple nodules in both lungs. The patient was diagnosed as having advanced pancreatic cancer with multiple lung metastases. The primary tumor showed a partial response to the administration of gemcitabine 1,000 mg/m2 on days 1, 8 and 15 of a 28-day cycle. Concurrent treatment with 5-fluorouracil (200 mg/day) was efficacious against the lung metastases. One year later the pancreatic and lung tumors had enlarged, and cisplatin (20 mg/body) was added to the protocol. For 20 months the patient was treated as an outpatient, maintaining good quality of life. The patient died of progressive disease 25 months after her first hospital admission.     

Immunocompetence and spontaneous regression of metastatic renal cell carcinoma

Spontaneous remission of pulmonary metastases from renal cell carcinoma was correlated with a positive response to dinitrochlorobenzene (DNCB). When first seen, the patient was DNCB negative and a chest radiograph showed nodular densities in the right lung and left midlung. Six months after sensitization, the patient had a positive response to DNCB and no evidence of lung metastases. Three mo later, the patient developed brain metastases although X-ray examination showed no pulmonary nodules. A diminished response to DNCB was noted over the next several months and chest X ray verified the return of pulmonary metastases.