Surgical management of primary and metastatic sarcoma of the mobile spine

OBJECT: Sarcomas of the spine are a challenging problem due to their frequent and extensive involvement of multiple spinal segments and high recurrence rates. Gross-total resection to negative margins, with preservation of neurological function and palliation of pain, is the surgical goal and may be achieved using either intralesional resection or en bloc excision. The authors report outcomes of surgery for primary and metastatic sarcomas of the mobile spine in a large patient series. METHODS: A retrospective review of patients undergoing resection for sarcomas of the mobile spine between 1993 and 2005 was undertaken. Sarcomas were classified by histology study results and as either primary or metastatic. Details of the surgical approach, levels of involvement, and operative complications were recorded. Outcome measures included neurological function, palliation of pain, local recurrence, and overall survival. RESULTS: Eighty patients underwent 110 resections of either primary or metastatic sarcomas of the mobile spine. Twenty-nine lesions were primary sarcomas (36%) and 51 were metastatic sarcomas (64%). Intralesional resections were performed in 98 surgeries (89%) and en bloc resections were performed in 12 (11%). Median survival from surgery for all patients was 20.6 months. Median survival for patients with a primary sarcoma of the spine was 40.2 months and was 17.3 months for patients with a metastatic sarcoma. Predictors of improved survival included a chondrosarcoma histological type and a better preoperative functional status, whereas osteosarcoma and a high-grade tumor were negative influences on survival. Multivariate analysis showed that only a high-grade tumor was an independent predictor of shorter overall survival. American Spinal Injury Association scale grades were maintained or improved in 97% of patients postoperatively, and there was a significant decrease in pain scores postoperatively. No significant differences in survival or local recurrence rates between intralesional or en bloc resections for either primary or metastatic spine sarcomas were found. CONCLUSIONS: Surgery for primary or metastatic sarcoma of the spine is associated with an improvement in neurological function and palliation of pain. The results of this study show a significant difference in patient survival for primary versus metastatic spine sarcomas. The results do not show a statistically significant benefit in survival or local recurrence rates for en bloc versus intralesional resections for either metastatic or primary sarcomas of the spine, but this may be due to the small number of patients undergoing en bloc resections.     

Postradiation sarcoma involving the spine

Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer.     

Effect of resection and outcome in patients with retroperitoneal sarcoma

BACKGROUND: A consecutive series of 47 patients with retroperitoneal sarcoma (RPS) were resected and prospectively followed. METHOD: Between July 1994 and March 2005, 47 patients (24 men, 23 women; mean age, 56 years; range, 17-82 years) were evaluated. RESULTS: A total of 23 patients had primary RPS and 24 patients had recurrent RPS. A total of 30 out of 47 patients (64%) underwent removal of contiguous intra-abdominal organs. The peroperative mortality was nil and significant preoperative complications occurred in eight cases only (17%). High tumour grade and incomplete resection were significant variables for a worse survival in all 47 patients, both in the univariate and multivariate analyses (P = 0.008 and P = 0.016, respectively). Among 28 radically resected patients, only histological grade affected overall survival (90% 5-year survival for low-grade tumour vs 26% 5-year survival for high-grade tumour; P = 0.006) with a similar effect noted for disease-free survival. CONCLUSIONS: Histological grade was the only factor that affected overall and disease-free survival for RPS tumours. An aggressive surgical approach in both primary and recurrent RPS is associated with long-term survival.     

Reoperative pulmonary resection in patients with metastatic soft tissue sarcoma

Resection of pulmonary metastases from soft tissue sarcoma has been shown to be associated with a 3-year survival of 25% to 30%. The role of multiple resections for recurrent pulmonary metastases, however, has not been clearly defined. Since 1976, 43 patients have had two or more thoracic explorations for the purpose of resecting pulmonary metastases from adult soft tissue sarcoma at our institution. In 89 reexplorations, through either median sternotomy or lateral thoracotomy, the operative mortality was 0%, and 31 of the 43 patients (72%) could be rendered free of disease at the second thoracotomy. Median survival from the second thoracotomy for the patients with resectable disease was 25 months, whereas median survival of patients who had unresectable disease was 10 months. A disease-free interval between the first and second thoracotomies of greater than 18 months was associated with prolonged survival from the second thoracotomy. Owing to lack of other therapies with proven salvage efficacy and in the absence of randomized trials, repeated thoracotomies to render patients free of disease from pulmonary soft-tissue sarcoma metastases appear justified considering the potential survival benefit and low attendant risk.     

Treatment and outcome of metastatic paraganglioma of the spine

Background

Spinal metastatic paraganglioma (MPG) is rare and only reported in individual case reports. The low incidence makes it difficult to define appropriate therapy and prognosis. Our study illustrated the largest series to discuss the possible treatment and outcomes of patients with spinal MPG.

Methods

A retrospective study of 15 patients with spinal MPG who were surgically treated between 2005 and 2014 was performed. Three surgical modalities were applied, and radiotherapy and chemotherapy were utilized as adjuvant therapy.

Results

The mean patients age was 40.9 (range 23–58) years. The period between primary surgery and spinal metastasis averaged 8.2 (0.5–15) years. Lesions were mainly located in cervical spine (2), thoracic spine (8), lumbar spine (3), and sacrum (2). The mean follow-up period was 35.0 months. Lesion progression was detected in nine patients, whereas five patients (33.3%) passed away. For solitary spine, multiple bone and both bone and nonosseous metastasis cases, the mean progression-free survival was 41 (range 9–56), 22.5 (range 12–38) and 8.3 (range 3–18) months, respectively.

Conclusions

The cases presented in the current study highlight the crucial role of surgery. Total en bloc for solitary spinal MPG could result in a satisfying prognosis and piecemeal total resection with postoperative radiotherapy could be an alternative therapy. Radiotherapy and chemotherapy were advocated, especially for the multiple metastasis.

     

Combined posterior pelvis and lumbar spine resection for sarcoma

The oncologic outcome in the treatment of pelvic sarcomas is not comparable with that in the extremity. Particular problems arise when the tumor involves the posterior pelvis and the sacrum or even the lower lumbar spine. Because of the difficulty in achieving local control and the major functional deficits after extensive surgery, some authors suggest conservative, nonsurgical treatment for these patients. The purpose of the current study was to analyze the oncologic outcome of patients who were treated for a pelvic sarcoma necessitating resection of the ilium, part of the sacrum and part of the lower spine, which is defined as extended hemipelvectomy. Between 1979 and 1999, 11 males and seven females with a mean age of 34.5 years (range, 14-67 years) had an extended hemipelvectomy for a sarcoma of the posterior pelvis. The tumors included seven osteogenic sarcomas, six chondrosarcomas, and five fibrosarcomas: 13 were classified as high-grade lesions. The mean size of the tumor was 11 x 9 x 6 cm. In 11 patients, a hemivertebrectomy of L5 and in seven patients of L4 combined with L5 was done to achieve an adequate margin. In four patients, the resection was followed by reconstruction with a vascularized fibula or autograft. At a mean followup of 56 months (range, 3-220 months), 12 of 18 patients were alive and without disease. Six patients died; four of these patients had metastasis to the lung at presentation. The two remaining patients died of complications of adjuvant treatment. The authors conclude from this small series of patients that an aggressive surgical resection for localized, nonmetastatic, high-grade sarcomas of the posterior pelvis may be justified to provide local control and improve survival.     

Surgical treatment strategies and outcome in patients with breast cancer metastatic to the spine: a review of 87 patients

Aggressive surgical management of spinal metastatic disease can provide improvement of neurological function and significant pain relief. However, there is limited literature analyzing such management as is pertains to individual histopathology of the primary tumor, which may be linked to overall prognosis for the patient. In this study, clinical outcomes were reviewed for patients undergoing spinal surgery for metastatic breast cancer. Respective review was done to identify all patients with breast cancer over an eight-year period at a major cancer center and then to select those with symptomatic spinal metastatic disease who underwent spinal surgery. Pre- and postoperative pain levels (visual analog scale [VAS]), analgesic medication usage, and modifed Frankel grade scores were compared on all patients who underwent surgery. Univariate and multivariate analyses were used to assess risks for complications. A total of 16,977 patients were diagnosed with breast cancer, and 479 patients (2.8%) were diagnosed with spinal metastases from breast cancer. Of these patients, 87 patients (18%) underwent 125 spinal surgeries. Of the 76 patients (87%) who were ambulatory preoperatively, the majority (98%) were still ambulatory. Of the 11 patients (13%) who were nonambulatory preoperatively, four patients were alive at 3 months postoperatively, three of which (75%) regained ambulation. The preoperative median VAS of six was significantly reduced to a median score of two at the time of discharge and at 3, 6, and 12 months postoperatively (P < 0.001 for all time points). A total of 39% of patients experienced complications; 87% were early (within 30 days of surgery), and 13% were late. Early major surgical complications were significantly greater when five or more levels were instrumented. In patients with spinal metastases specifically from breast cancer, aggressive surgical management provides significant pain relief and preservation or improvement of neurological function with an acceptably low rate of complications.     

Hepatic resection for primary and metastatic tumors

Thirty-four hepatic resections were performed on 33 patients. These included 4 trisegmentectomies, 14 lobectomies, 7 segmentectomies, and 9 wedge resections. Twenty patients had metastatic colorectal cancer, 4 had a primary liver tumor, 2 had giant cavernous hemangioma, 1 had metastatic leiomyosarcoma, 5 had various benign lesions including focal nodular hyperplasia, and 1 patient had resection for trauma. Operative morbidity included four subphrenic abscesses, one bile leak, one bile duct injury, one case of cholestasis, and one case of phlebitis. There were no operative deaths. The median survival of the patients with metastatic colorectal cancer was 40 months, and the 5-year actuarial survival rate was 35 percent. Survival rates were not significantly different between patients with a solitary metastasis and those with multiple lesions and was not influenced by size of the metastases. However, survival was significantly better in patients whose primary colorectal lesion was Dukes' B as compared with those whose lesion was Dukes' C. The results indicate that liver resection can be performed safely with acceptable morbidity and improved long-term survival.     

Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients

Pulmonary metastases of endometrial stromal sarcoma (ESS) are uncommon and can pose diagnostic problems. We reviewed lung specimens from 16 patients with metastatic ESS. Patients were 31-77 years of age at the time of lung biopsy. Uterine ESSs were diagnosed an average of 9.8 years before lung biopsy in 11 patients. Uterine ESSs were originally called smooth muscle tumors in three additional patients. Thirteen patients were evaluated for new pulmonary nodules, seven of whom were asymptomatic. Nodules were multiple in 14 and solitary in four, ranging from 1.0 to 8.0 cm in greatest dimension. One patient died of metastatic disease; 14 were alive and seven of these were without disease (mean follow-up 4.1 years). Diagnostic considerations in 12 consultation cases included ESS, sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis, endometriosis, hemangiopericytoma, and lymphoma. Tumors were well circumscribed and usually solid, composed of plump spindle cells arranged in short fascicles. Two tumors were predominantly cystic. Sex cord-like stromal differentiation was identified in three. Neoplastic cells stained for vimentin (93%), estrogen and progesterone receptor (100%), smooth muscle actin (57%), desmin (50%), and keratin (46%). Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women.     

Results of hepatic resection for sarcoma metastatic to liver

OBJECTIVE: To evaluate the outcome of patients with liver metastases from sarcoma who underwent hepatic resection at a single institution and were followed up prospectively. SUMMARY BACKGROUND DATA: The value of hepatic resection for metastatic sarcoma is unknown. METHODS: There were 331 patients with liver metastases from sarcoma who were admitted to Memorial Hospital from 1982 to 2000, and 56 of them underwent resection of all gross hepatic disease. Patient, tumor, and treatment variables were analyzed to assess outcome. RESULTS: Of the 56 patients who underwent complete resection, 34 (61%) had gastrointestinal stromal tumors or gastrointestinal leiomyosarcomas. Half of the patients required an hepatic lobectomy or extended lobectomy. There were no perioperative deaths in the completely resected group, although 3 of the 75 patients who underwent exploration (4%) died. The postoperative 1-, 3-, and 5-year actuarial survival rates were 88%, 50%, and 30%, respectively, with a median of 39 months. In contrast, the 5-year survival rate of patients who did not undergo complete resection was 4%. On multivariate analysis, a time interval from the primary tumor to the development of liver metastasis greater than 2 years was a significant predictor of survival after hepatectomy. CONCLUSIONS: Complete resection of liver metastases from sarcoma in selected patients is associated with prolonged survival. Hepatectomy should be considered when complete gross resection is possible, especially when the time to the development of liver metastasis exceeds 2 years.     

Impact of primary resection on the outcome of patients with perforated diverticulitis

BACKGROUND: Primary resection has replaced the conventional drainage procedure in the management of patients with generalized peritonitis complicating diverticular disease of the colon. This study investigates the impact of primary resection on operative mortality, identifies predictors of mortality, and compares the results with those of our earlier experience. HYPOTHESIS: Primary resection of the perforated diseased segment of the colon is associated with lower mortality rates than the drainage procedure in patients with Hinchey stages 3 and 4 diverticulitis. DESIGN: Retrospective analysis. SETTING: Tertiary care referral center. PATIENTS: We included 138 consecutive patients who underwent emergent operation for generalized peritonitis complicating diverticular disease of the colon (Hinchey stages 3 and 4) during a period of 16 years (January 1983 to May 1999). MAIN OUTCOME MEASURES: The 30-day mortality rate was analyzed and predictors of mortality identified. RESULTS: Patients were classified as having spreading purulent peritonitis (n = 44, 31.9%), diffuse peritonitis (n = 64, 46.4%), or fecal peritonitis (n = 30, 21.7%). One hundred thirty-one patients (94.9%) underwent primary resection, 6 patients (4.3%) underwent resection and primary anastomosis, and 1 patient required total colectomy and end ileostomy. Thirteen of the 138 patients in the present group died (1983-1998), representing a perioperative mortality rate of 9%. There was no significant difference in mortality when compared with our earlier study (1972-1982), which had a mortality rate of 12%, considering that more than 25% of the patients in that group were managed by colostomy and drainage alone. Factors identified univariately as predictors of mortality were age of more than 70 years (P = .047), 2 or more comorbid conditions (P<.01), obstipation at initial examination (P = .02), use of steroids (P = .01), and perioperative sepsis (P<.001). CONCLUSIONS: Primary resection has become the standard practice for patients with generalized peritonitis complicating diverticulitis. Mortality rates have not significantly declined despite more aggressive surgical management of the septic source. Because advanced age, comorbid conditions, and perioperative sepsis predict mortality, it is suggested that further reduction in mortality will require improvement in medical management of perioperative sepsis and comorbid conditions.     

Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases

Angiosarcoma occurs very rarely in the intestinal tract as either a primary or metastatic malignancy and can present great diagnostic difficulty, especially when it displays epithelioid cytomorphology. Since only isolated case reports have been published, the purpose of this study is to more fully delineate the histopathological and clinical features from a series of 8 angiosarcomas involving the gastrointestinal tract. There were 5 male and 3 female patients whose ages ranged from 25-85 years (median 57). Presenting symptoms included intestinal bleeding, anemia and pain. Five cases involved the small bowel and 3 involved the colon/rectum. Four cases were primary to the intestinal tract, 2 patients initially presented with secondary involvement of the large bowel from occult retroperitoneal primaries, 1 patient presented with disseminated disease including small bowel involvement, and 1 case was metastatic from a breast primary. Seven cases were composed predominantly of sheets of malignant appearing epithelioid cells with subtle areas forming cleft-like spaces suggestive of vascular differentiation. Immunohistochemical studies revealed the lesional cells to be immunoreactive for CD31 (8/8), CD34 (8/8), Factor VIII (8/8), cytokeratins AE1/AE3 (7/8), cytokeratin 7 (2/8), Cam5.2/cytokeratin 8 (5/8), and cytokeratin 19 (5/8). Cytokeratin 20 was negative in all eight cases, which contrasts sharply with the characteristic positivity for cytokeratin 20 in virtually all intestinal carcinomas. One case was weakly and focally positive for EMA and all cases were negative for S-100 protein. Cytokeratin staining was variable and ranged from focal to extensive. Follow-up was available in eight cases and ranged from 1-33 months (median 12.5). Five patients died of disease, between 1 and 33 months (median 6) after diagnosis. One recently diagnosed patient is alive with disease 18 months after diagnosis, and one patient is free of disease 27 months after original diagnosis. Angiosarcomas of the gastrointestinal tract commonly display epithelioid cytomorphology, may be diffusely and strongly positive for cytokeratins and only show subtle signs of vascular differentiation, creating potential diagnostic confusion with primary or metastatic carcinoma. Given the clinically aggressive behavior of angiosarcoma, proper classification and treatment is important. Immunohistochemistry with vascular markers, CK20, and S-100 protein may be helpful in differentiating angiosarcoma from carcinoma and melanoma.     

Functional outcome following surgical treatment of metastatic tumors involving the femur

Thirty-eight patients treated surgically for metastatic tumors of the femur were reviewed to evaluate the effects of surgical treatment on quality of life. Average age at surgery was 59 years. The most common origin site of metastatic tumors was the lung. The intertrochanteric area was involved in 14 patients, followed by the subtrochanteric area in 11, femoral neck in 7, and shaft in 6 patients. Surgery was based on the principles that tumor excision should be maximal, and to permit early postoperative ambulation, internal fixation should be rigid with cement augmentation. Types of internal fixation were applied according to lesion site to allow early ambulation and included intramedullary nailing (11), compression hip screw (9), and Rowe plates (7). Prosthetic replacement was performed in 9 patients. Average performance scores improved from 3.8 preoperatively to 2 postoperatively, based on the Functional Classification of the New York Heart Association. In 31 patients (82%), the degree of pain relief was more than a lot according to the criteria of Kaiko. Thirty-five patients survived >5 months postoperatively. In patients with a single metastatic lesion, survival was 21 months, and in patients with multiple lesions, survival was 10 months. Surgical stabilization of a pathologic lesion involving the femur resulted in improvement in the quality of life, including pain relief and early ambulation. In selected patients with a single metastatic lesion and a low-grade primary tumor, prolonged postoperative survival may be expected.     

Histologic evaluation and treatment outcome after sequential radiofrequency ablation and hepatic resection for primary and metastatic tumors

Operative manipulation during hepatic resection (HR) causes tumor cell shedding which is a factor in disease recurrence. Radiofrequency ablation (RFA) causes coagulative necrosis and was used to destroy the tumor before HR. We evaluated tumor necrosis and recurrence of hepatic malignancies treated by sequential RFA/HR. A retrospective review of patients treated with sequential RFA/HR from April 1999 to January 2002 was performed. A Radionics 500-kW RF generator was used to ablate lesions via H2O-cooled electrodes under ultrasound guidance. Segmental HR was performed after RFA. Resected specimens were reviewed with hematoxylin and eosin staining and for apoptosis. Patient follow-up ranged from 10 to 33 months with evaluation of salient clinical, radiologic, and laboratory parameters. Seven patients (four male and three female) ages 62.1 +/- 10.3 years had sequential RFA/HR. Four patients had hepatocellular carcinoma (HCC) and three had colorectal metastases (CRm). The tumors were unifocal right-lobe lesions measuring 4.1 +/- 0.9 cm with a resection margin of 0.4 to 2.5 cm. Extensive necrosis was noted but intact nests of tumor cells occurred in all specimens with minimal apoptosis. Three of seven patients (two HCC and one CRm) developed pulmonary metastases at 3 to 20 months with one HCC patient developing concurrent liver metastases. Two deaths occurred in the HCC group. Sequential RFA/HR may minimize local recurrence; however, the high incidence of pulmonary metastases raises concern of transvenous migration. The histologic findings demonstrate foci of intact tumor cells after RFA. Controlled study of additional patients with long-term follow-up is necessary to better understand these findings.     

Repeated resection of liver in primary and metastatic cancer

164 operations for malignant tumors of the liver were performed in Cancer Research Center. 14 patients underwent repeated resections of the liver, 5 of them due to primary cancer of the liver and 9--with metastatic liver disease. Recurrence was revealed in 6 from 14 patients (42.8%), within a year after resection, in 4 (28.6%)--during two year after the first resection of the liver. In the rest 4 patients (28.6%) recurrence was revealed after two and more years. The indications for repeated resections were: recurrent cancer in the liver which should be localized, with absence of extrahepatic metastasis, good functional condition of the liver and absence of concomitant diseases. Repeated resections of the liver are technically more complicated due to the following causes: functional condition of the liver is rather deteriorated, especially after previous extended resections; anatomical interrelations are disturbed, there are intensive adhesions. There were no lethal outcomes after repeated resections of the liver. 3 years survival of operated patients was higher (60%) than in patients who underwent resection of the liver once, both in a primary cancer of the liver (46.8%) and in metastatic cancer (40%).     

Surgical resection of primary soft-tissue sarcoma of the extremities]

We evaluated 5 patients who had soft tissue sarcoma of the extremity treated by limb sparing surgery. All tumors were adjacent to bone, with no features of infiltration. According to the Surgical Staging System, all tumors were IIB. The mean follow-up was 13 months. To achieve wide resections of the tumors in such cases we recommend resections of the tumor with part of the bone, which is in direct contact with the tumor. Wide resection margins were achieved in all patients and none had local recurrence during the observation period.     

Ewing sarcoma of the rib: results of an intergroup study with analysis of outcome by timing of resection

OBJECTIVE: We sought to establish the outcome and optimal therapeutic sequence for patients with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the chest wall. METHODS: Patients 30 years of age or younger with nonmetastatic Ewing sarcoma/primitive neuroectodermal tumor of the bone were randomly assigned to receive vincristine, doxorubicin, cyclophosphamide, and dactinomycin or those drugs alternating with ifosfamide and etoposide. Local control was obtained with an operation, radiotherapy, or both. RESULTS: Fifty-three (13.4%) of 393 patients had primary tumors of the chest wall (all rib). Event-free survival at 5 years was 57% for the chest wall compared with 61% for other sites (P >.2). Ifosfamide and etoposide improved outcome in the overall group (5-year event-free survival, 68% vs 54%; P =.002), and a similar trend occurred in chest wall lesions (5-year event-free survival, 64% vs 51%). Patients with chest wall lesions had more attempts at initial surgical resection (30%) than those with other primary tumor sites (8%, P <.01). The attempt at initial resection for chest wall lesions did not correlate with size. Initial resections at other sites were restricted to smaller tumors. Initial resection resulted in negative pathologic margins in 6 of 16 patients, whereas the delayed resection resulted in negative margins in 17 of 24 patients (P =.05). Although there was no difference in survival by timing of the operation in rib lesions, a higher percentage of patients with initial surgical resection received radiation than those with resection after initial chemotherapy (P =. 13). CONCLUSIONS: Although rib primary tumors are significantly larger than tumors found in other sites, their outcome is similar. We favor delayed resection whenever possible to minimize the number of patients requiring radiation therapy.