Re-exploration for retroperitoneal lymph node metastases from testis tumors.

Prognosis has been poor for patients with retroperitoneal metastases from non-seminomatous testis tumors that are initially unresectable and persist after chemotherapy and irradiation or those that recur after initial lymphadenectomy. Eleven such patients have had re-explorations at our center and are described herein. In 4 patients only histologically benign teratoma and/or fibrosis was found. Therefore, the presence of a retroperitoneal mass in these circumstances does not necessarily mean that malignant tissue was present. The procedure provided an accurate diagnosis in all patients and permitted the elimination or reduction of subsequent chemotherapy or radiation in 4 patients who had no tumor. In addition, 5 of 7 patients with symptoms caused by bulky masses were improved and 3 patients with unresectable disease had radiopaque markers placed to facilitate subsequent radiotherapy. The operation was often difficult because of retroperitoneal adhesions from previous treatment but there was no significant morbidity and no mortality. Nine patients have survived from 6 months to 4 years and 7 have no evidence of disease. Two patients died of recurrent tumor 2 and 9 months after re-exploration. A good prognosis was indicated if the mass found at re-exploration was completely resectable and contained only histologically benign teratoma and/or fibrous tissue. We believe that re-exploration should be considered for selected patients with testis tumors who have 1) a retroperitoneal mass that appears after initial lymphadenectomy and persists after interval chemotherapy or radiation therapy and 2) retroperitoneal metastases that are initially unresectable and persist after subsequent chemotherapy or radiation therapy.     

Experience with "second-look" operations in pediatric solid tumors.

"Second-look" operations were performed in 32 infants and children with initially unresectable or recurrent solid tumors treated with combination chemotherapy and/or irradiation. Tumors were resectable in 26 of 32 cases (81%). These procedures often yielded information affecting staging and treatment. Disease-free survival was achieved in 18 of 32 patients (56%). Mortality was related to progressive disease in seven cases and opportunistic infections due to immunosuppression in three. Four additional patients are alive with evidence of persistent tumor. Second-look procedures were beneficial in patients with Wilms' tumor previously operated upon by a flank approach and in children with bilateral tumors. These procedures were particularly useful in children with stage III localized neuroblastoma and cases of metastatic neuroblastoma that respond to chemotherapy. Second-look operations were also useful in selected cases of rhabdomyosarcoma, teratoma, and Ewing's sarcoma. These observations suggest that combination chemotherapy has increased the use of second-look operations in a variety of less favorable (e.g. initially unresectable or recurrent) pediatric solid tumors.     

Improved survival in infants and children with primary malignant liver tumors

Forty-six infants and children with primary malignant liver tumors were studied. Thirty-three had hepatoblastomas, 9 hepatocellular carcinomas, and 4 had sarcomas. Thirty-eight patients underwent liver biopsy or excision of the tumor. Nineteen patients were treated between 1952 and 1971 (mean survival 5.5 months) and the remaining 19 were treated between 1972 and 1981 (mean length of survival 30 months, 2 year survival rate 53 percent). In 21 patients with an unresectable tumor, biopsy alone was performed (mean survival 7 months). Ten patients underwent primary resection of their liver tumor (mean length of survival 23 months, 2 year survival rate 37.5 percent). Seven other patients with an unresectable tumor were treated with chemotherapy alone or in combination with radiotherapy for a mean period of 6 months before second-look celiotomy was carried out. Six of these patients (all treated after 1975) had significant reduction in the size of their tumors. Mean length of survival in this group was 4 years 2 months, and the 2 year survival rate was 100 percent; at present, 5 of these patients are living without the disease 2.5 to 7 years after diagnosis. Improved techniques in liver resection and chemotherapy before second-look celiotomy is undertaken for an unresectable lesion have improved prognosis in children with a malignant liver tumor.     

Preoperative chemotherapy for hepatoblastoma in children: report of six cases

Surgical excision has been the primary treatment for hepatoblastoma; however, at presentation, only one-third of such tumors are surgically resectable. Without operation, the disease is universally fatal. Six children with initially unresectable hepatoblastoma (two with pulmonary metastases) were treated with chemotherapy, which included Adriamycin. Four of the six children showed a significant reduction in tumor size, in three delayed resection of the primary lesion was possible, and the fourth patient died of Adriamycin cardiotoxicity. Two patients did not respond and developed pulmonary metastases after 2 and 16 mo of chemotherapy, respectively. Adriamycin alone, or in combination with other agents, has proven effective in primary and metastatic childhood hepatoblastoma. This preoperative chemotherapy regimen permits resection of previously unresectable hepatoblastoma at "second look" operation and reduces the morbidity and mortality of an otherwise extensive operation.     

Pancreatic tumors in childhood: analysis of 13 cases

Pancreatic tumors are rare in children. Over a 20-year period we have treated 13 children with pancreatic neoplasms. There were eight boys and five girls (age range, 4 months to 12 years). Seven tumors were benign, including five insulinomas, and two cystadenomas. Six lesions were malignant (rhabdomyosarcoma, 2; pancreatic carcinoma, 4). Children with insulinoma presented with hypoglycemia and irrational behavior. Three had abnormal insulin:glucose ratios ( greater than 1.0). The tumor was detected by computed tomography scan in three cases, at the time of surgery in one, and with intraoperative ultrasound in one. Surgical treatment included tumor enucleation in four cases and 80% pancreatectomy in one. Mucinous cystadenomas were observed in two patients, ages 4 months and 10 months. Tha latter infant underwent cyst excision alone, resulting in malignant recurrence at 18 months of age and death. The 4-month-old child had a distal pancreatectomy and is alive at 6 years. Two of the four children with pancreatic cancer had unresectable tumors at diagnosis, and were treated by biopsy (ductal adenocarcinoma), irradiation, and chemotherapy. Length of survival was 6 months and 9 months. Two others (ages 4 and 12 years) underwent 85% distal pancreatic resection for pancreatoblastoma and a pancreatoduodenectomy for papillary carcinoma, respectively. The latter is alive and tumor-free at 20 years of follow-up. The former underwent hepatic lobectomy for a 3.0 x 3.0 cm solitary liver metastases and is alive at 6 years with no evidence of disease. One child with rhabdomyosarcoma died of progressive disease, the other is alive with residual disease despite resection and chemotherapy. Most insulinomas can be treated by enucleation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Genitourinary rhabdomyosarcoma

Rhabdomyosarcoma is the most common soft tissue malignant neoplasm involving the pelvis of children. Debate still exists over whether the best treatment is pelvic exenteration, radiation and chemotherapy or chemotherapy as the cornerstone to treatment, with diminished needs for extensive surgery and prolonged radiotherapy. Contrariwise, there is little debate over the combined treatment modality for paratesticular rhabdomyosarcoma. We have evaluated and treated 19 children with rhabdomyosarcoma, including 17 with pelvic rhabdomyosarcoma and 2 with paratesticular rhabdomyosarcoma. This retrospective study was done to evaluate treatment regimens for patients with genitourinary rhabdomyosarcoma. For patients with pelvic rhabdomyosarcoma the study was divided into 2 series. In an early series 6 of 7 children had a pelvic exenteration with or without chemotherapy. Of these 7 children 3 are well 15 to 27 years following diagnosis. In a later series of 10 children, when chemotherapy was used more commonly, none underwent pelvic exenteration. Of these 10 patients 7 had chemotherapy or chemotherapy and biopsy only. Only 1 child, who presented with stage IV disease, died in this series. Another child with stage III disease had progressive disease despite chemotherapy and subsequent cystoprostatectomy. Thus, of 9 remaining patients 8 are well from 1 to 8 years. It appears that pelvic rhabdomyosarcoma can be treated effectively with chemotherapy, and limited surgery and radiation. Fortunately, pelvic exenteration can now be limited to a select few.     

Preoperative irradiation and surgery for initially unresectable adenocarcinoma of the rectum.

Forty-two patients with initially unresectable rectal adenocarcinoma were treated with preoperative irradiation and surgery between March 1970 and March 1986 at the University of Florida (Gainesville, FL). No patient received adjuvant chemotherapy. All patients had follow-up for at least 5 years; 23 (55%) had follow-up for 10 years or more. Five patients (12%) underwent an exploratory laparotomy before irradiation; the remaining 37 patients were clinically thought to have unresectable disease. Eighteen patients had tumor fixation to one structure, and 24 patients (57%) had fixation to two or more structures. Lesions that exhibited impaired mobility but not complete fixation were considered to be clinically resectable and are not included in this series. The dose of preoperative irradiation ranged from 3,500 to 6,000 cGy at 180 cGy per fraction; 30 of 42 patients received 4,500 to 5,000 cGy and only 4 patients received less than 4,000 cGy. Forty-one patients were operated on 1 to 11 (mean 4.6) weeks after the completion of radiotherapy; 1 patient died of aspiration pneumonia after completing irradiation but before the planned resection. Resection of the rectal cancer was accomplished in 37 patients. Four patients were found to have extensive unresectable disease at laparotomy. Twenty-one patients had a complete resection: 11 were stage B2, and 10 were stage C2. Twenty patients had incomplete resection due to positive margins (8 patients), distant metastases with or without positive margins (8 patients), or no resection due to extensive disease (4 patients). Two patients died after the operation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Recent experience in the treatment of rhabdomyosarcoma.

Twenty-two patients with rhabdomyosarcoma have presented in the years 1970--1977. Of 8 patients who had inadequate postoperative chemotherapy and radiotherapy, or none at all, only 2 patients with small completely resectable tumors are alive and well at 6 and 4 yr after surgery. Fourteen patients had intensive combination chemotherapy in addition to appropriate surgery and radiotherapy. Five have died and the remaining nine are well and free of disease at follow up ranging from 9 mo to over 5 yr. A continued place in the treatment regime is advocated for early surgery aimed at total excision of the primary tumor where this is feasible.     

Vaginal neoplasms in infancy: the combined role of chemotherapy and conservative surgical resection

This retrospective review of nine infants treated for vaginal tumors between 1964 and 1986 identifies the spectrum of lesions and examines the trend toward more conservative surgical therapy. Our patients' ages ranged from 1 to 30 months. Symptoms of vaginal bleeding or protruding tissue prompted examination under general anesthesia in all cases. Two children had benign protruding polypoid masses treated by simple excision. Seven had malignant tumors, two with endodermal sinus lesions and five with embryonal rhabdomyosarcoma. Early in this series, surgical therapy for embryonal rhabdomyosarcoma consisted of either anterior or complete pelvic exenteration, with adjuvant chemotherapy given in two of the three patients. One patient was operated on prior to the development of effective chemotherapy, and died of recurrent tumor 14 months after surgery. A second patient died from cardiac failure secondary to adriamycin toxicity 6 years after initial therapy. A third patient underwent total pelvic exenteration followed by successful reanastomosis of the colon to the anal verge. This patient, a female, is the oldest survivor and is free of disease 14 years after therapy. The most recent therapeutic approach used in two patients with embryonal rhabdomyosarcoma consisted of local tumor excision followed by postoperative chemotherapy. Both patients are alive and disease-free 9 and 11 years after therapy. Two patients with endodermal sinus tumor were treated with chemotherapy after simple excisional biopsy. They were then followed with serial vaginal biopsies at 3-month intervals. One required a partial vaginectomy for local recurrence 21 months after initial presentation. Both patients are alive and disease-free 18 months and 4 years after surgery.     

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: impact on survival.

Long-term survival in children with primary hepatic malignancies can not be expected without complete tumor resection. In the last ten years we have treated 21 children with hepatocellular carcinoma (HCC) and 21 children with hepatoblastoma (HEP), with tumor extirpation our surgical goal. Operative treatment included partial hepatectomy ([PH] 20), either primary (10) or delayed (following chemotherapy) (10), total hepatectomy and orthotopic liver transplantation ([OLT] 13), or upper abdominal exenteration and multiple organ transplantation (2). Two patients had both PH and subsequent total hepatectomy and OLT. Overall survival was 48% (20/42), with 9 patients dying of progressive disease prior to removal of their tumor. HEP patient survival was 67% (14/21), including 2 of 6 who underwent primary PH, 7 of 8 who had delayed PH, and 5 of 6 who underwent OLT. Survival for the children with HCC was 29% (6/21), including 1 of 4 after primary PH, 1 of 2 following delayed PH, 3 of 7 following OLT, and 1 of 2 after exenteration and multiple organ transplantation. Preoperative chemotherapy facilitated removal of 10 initially unresectable tumors (8 HEP, 2 HCC) at a second-look procedure. Total hepatectomy and OLT markedly improved survival in patients with disease unresectable by standard methods. Partial hepatectomy, either primary or delayed, should be attempted in all children with hepatic malignancies. Total hepatectomy and OLT appears to be a viable adjunct in the treatment of childhood malignancies, and should be used for otherwise unresectable tumors as part of a carefully planned protocol.     

Malignant presacral teratoma in children.

Malignant presacral teratoma is a rare tumor seen predominantly in young female children. The introduction of planned multidisciplinary treatment has improved the outlook for patients with this once dismal disease. Six female children were seen at Memorial Hospital with the diagnosis of malignant presacral teratoma. Five children were age 17 mo to 3 yr and the sixth child was 13 yr old at diagnosis. Presenting symptoms included masses in the buttock or groin, constipation, difficulty voiding, and local pain. Pathological features were varied and complex but three had predominantly endodermal sinus features. One child had the malignant presacral teratoma develop 18 mo after successful resection of a benign sacrosoccygeal teratoma in the newborn period. Treatment varied in the six cases since all were referred after failure of treatment elsewhere. All children had surgery, irradiation, and multiple drug chemotherapy. Four of the six children are surviving disease-free, 3 more than 24 mo off treatment. Evolution of treatment up to the present protocol management is discussed.     

Preoperative systemic chemoimmunotherapy and sequential resection for unresectable hepatocellular carcinoma

OBJECTIVE: To examine the surgical and pathologic findings of 15 patients who had initially unresectable hepatocellular carcinoma (HCC) and received preoperative systemic chemoimmunotherapy and sequential resection. SUMMARY BACKGROUND DATA: More than 80% of patients with HCC present for treatment at an unresectable stage. Conventional treatment has produced a low tumor response rate in this group of patients. Recently, new systemic chemoimmunotherapy has been found to be effective and able to make previously unresectable HCC resectable. Sequential resection after response to chemoimmunotherapy could therefore induce complete clinical remission. METHODS: From July 1996 to February 1999, 150 patients with unresectable HCC were treated with systemic chemoimmunotherapy consisting of cisplatin, alpha-interferon, doxorubicin, and 5-fluorouracil for a maximum of six cycles. The residual tumors were reassessed for resectability after treatment aiming at complete remission in the patients after combined modality treatment. Twenty-seven patients had a more than 50% regression in tumor size (2 complete remissions, 25 partial remissions). Fifteen patients had resectable disease after treatment, and all underwent sequential resection with curative intent. Treatment outcome and the surgical and pathologic features of these 15 patients were studied. RESULTS: Fifteen of 150 patients responded to chemoimmunotherapy and underwent sequential resection. They were considered to have unresectable disease as a result of extensive local disease (with and without major vascular involvement) in 10 patients and the presence of extrahepatic or metastatic disease in 5 patients. All patients except two were hepatitis B carriers. Surgical resection of the residual lesion after chemoimmunotherapy was successful for all patients. Eight of the patients had complete pathologic remission. The rest had minimal residual disease (<5%) only. All 15 patients entered complete clinical remission after surgery. Thirteen patients were still alive as of this writing and two had died of recurrent disease. The 1-, 2-, and 3-year survival rates were 100%, 100%, and 53%, respectively. The mean follow-up period was 27 months (range 15-37). Neither the median disease-free nor overall survival had been reached. Ten patients remained in complete remission as of this writing. CONCLUSION: Combined modalities with systemic chemoimmunotherapy and surgical resection can achieve complete clinical remission and long-term control of disease in patients with unresectable HCC.     

Does “Conversion Chemotherapy” Really Improve Survival in Metastatic Colorectal Cancer Patients with Liver-Limited Disease?

Background

The clinical benefits of conversion chemotherapy followed by liver resection for initially unresectable colorectal liver metastases are still controversial. The criteria for unresectability vary from one team to another. To clarify this issue, we retrospectively assessed the survival and characteristics of metastatic colorectal cancer (mCRC) patients with liver-limited disease (LLD) who underwent conversion therapy.

Method

Our criteria for resectability depended on the size of the remnant liver volume (>30 %) and expected function after removal of all metastases. Between December 2007 and September 2011, a total of 115 patients were diagnosed as having mCRC with LLD and received chemotherapy. Among them, 47 had tumors that were initially diagnosed as resectable. They underwent hepatic resection after chemotherapy (resected group). Of the 67 tumors were initially diagnosed as unresectable, 12 became resectable after chemotherapy (conversion group), leaving 55 tumors that remained unresectable after chemotherapy (unresected group).

Results

The median follow-up was 25.2 months. Hepatic resection was more invasive in the conversion group than in the resected group. Median disease-free survival was significantly higher in the resected group than in the conversion group (p = 0.013). Overall survival (OS) was also higher in the resected group, but the difference was not significant (p = 0.36). However, OS was significantly higher in the conversion group than in the unresected group (p = 0.034). Multivariate analysis of the resected and conversion groups showed that OS was significantly negatively influenced by abnormal carcinoembryonic antigen levels at surgery (p = 0.037) and a hospital stay >30 days (p = 0.009).

Conclusions

Our results showed that conversion chemotherapy could contribute to longer OS in mCRC patients with LLD.     

Primary Malignant Mediastinal Tumors

Thirty-eight patients with primary malignant mediastinal tumors of all cell types are the basis for this review. Eleven of these patients had germ cell tumors. Five germ cell tumors were seminomas, two were malignant teratomas, and two were endodermal sinus tumors. Mean survival for all patients with germ cell tumors was 3.3 years. Eight children had surgical excision of mediastinal neuroblastomas, and all but 1 are alive for a mean survival of 6.7 years. Seven patients had lymphoproliferative disorders; 6 of these patients had nodular sclerosing Hodgkin's disease, and 1 had lymphoblastic (thymic) lymphoma. Mean survival was 5.1 years. There were five carcinomas of various cell types and one angiopericytoma. None of the patients with these lesions survived more than 2 years. Four patients had thymoma with an average survival of 3.7 years. Two patients had carcinoid tumors of thymic origin; neither survived more than 1 year.In 1972, we reported 5-year disease-free survival of 26% in a series of patients with primary mediastinal tumors. Our experience since 1970 shows current survival of 47.3% and 5-year disease-free survival of 34.2%. We use combined methods of therapy, including aggressive surgical resection, combination chemotherapy, and often mediastinal irradiation for most types of mediastinal tumors. Primary mediastinal malignancies should be treated aggressively using a multidisciplinary approach, since many of these tumors are curable.     

The value of laparoscopic staging for patients with colorectal metastases

BACKGROUND: Resection offers the only chance of cure for hepatic colorectal metastases. However, preoperative staging does not always reliably detect unresectable disease. The aim of this study was to investigate the role that laparoscopy with ultrasound may have in detecting unresectable disease, thus sparing patients from unnecessary laparotomy with the associated morbidity and cost. METHODS: A retrospective review of all patients considered for liver resection of colorectal metastases during a 3-year period was performed, analyzing factors likely to predict resectable disease, rates of resectability, and success of laparoscopic staging at detecting unresectable disease. RESULTS: Of 73 patients with resectable disease on computed tomography, 24 were deemed to need laparoscopy, and 49 proceeded directly to laparotomy. Those first undergoing laparoscopy had shorter disease-free intervals between diagnosis of colorectal cancer and detection of hepatic recurrence and greater numbers of hepatic metastases. Twelve of the 24 patients who underwent laparoscopy had unresectable disease, and 8 of these were detected at laparoscopy. Forty-six of the 49 patients proceeding to laparotomy directly had resectable disease. CONCLUSIONS: Laparoscopic staging of hepatic colorectal metastatic disease detects most unresectable disease, preventing unnecessary laparotomy. The likelihood of disease being unresectable is in part predicted by the disease-free interval and the number of hepatic metastases.     

Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma

Orthotopic liver transplantation (OLT) alone for unresectable cholangiocarcinoma is often associated with early disease relapse and limited survival. Because of these discouraging results, most programs have abandoned OLT for cholangiocarcinoma. However, a small percentage of patients have achieved prolonged survival after OLT, suggesting that adjuvant approaches could perhaps improve the survival outcome. Based on these concepts, a protocol was developed at the Mayo Clinic using preoperative irradiation and chemotherapy for patients with cholangiocarcinoma. We report our initial results with this pilot experience. Patients with unresectable cholangiocarcinoma above the cystic duct without extrahepatic or extrahepatic metastases were eligible. Patients initially received external-beam irradiation plus bolus fluorouracil (5-FU), followed by brachytherapy with iridium and concomitant protracted venous infusion of 5-FU. 5-FU was then administered continuously through an ambulatory infusion pump until OLT After irradiation, patients underwent an exploratory laparotomy to exclude metastatic disease. To date, 19 patients have been enrolled onto the study and have been treated with irradiation. Eight patients did not go on to OLT because of the presence of metastasis at the time of exploratory laparotomy (n = 6), subsequent development of malignant ascites (n = 1), or death from intrahepatic biliary sepsis (n = 1). Eleven patients completed the protocol with successful OLT Except for 1 patient, all had early-stage disease (stages I and 11) in the explanted liver. All patients who underwent OLT are alive, 3 patients are at risk at 12 months or less, and the remaining 8 patients have a median follow-up of 44 months (range, 17 to 83 months; 7 of 9 patients > 36 months). Only 1 patient developed tumor relapse. OLT in combination with preoperative irradiation and chemother apy is associated with prolonged disease-free and overall survival in highly selected patients with early-stage cholangiocarcinoma.     

Second-look laparotomy for Wilms' tumor: indications and results in 19 patients

Nineteen of 74 children with Wilms' tumor underwent second-look laparotomy. Transperitoneal operation was done in five cases referred after flank operation elsewhere. Four had a change in stage from I to II or III, while one considered "inoperable" was resected (4/5 survived). Reoperation was done in two late referrals after operations with tumor spill. One had recurrent flank disease; the other had a flank mass with unrecognized diaphragmatic and intracaval extension (1/2 survived). Three children with giant tumors initially considered unresectable were successfully resected after cytoreduction with chemotherapy (3/3 survived). Two of three patients with bilateral Wilms' tumor survived reoperative procedures (partial or total nephrectomy). Five children with late intraabdominal recurrence (3 liver: 2 flank) eventually died despite reoperation and adjunctive therapy. All five had unfavorable histology. One child with en bloc hepatic resection had successful reoperation for suprahepatic vena caval obstruction due to regeneration of liver, but subsequently died. Ten of the 19 patients survived (52.6%) following reoperation and adjunctive therapy. Second-look laparotomy is quite useful in patients inadequately staged with flank operations, in cases of bilateral Wilms' tumor, and in children with initially unresectable tumors following cytoreduction. Patients with extensive tumor spill at a previous procedure may benefit from early reoperation. Late recurrence of tumor (especially with unfavorable histology) and/or liver metastases carried an ominous prognosis.     

Preoperative chemotherapy and surgical resection in the management of posterior paraspinal tumors. Report of three cases

Three patients with posterior paravertebral tumors are presented to illustrate the value of preoperative chemotherapy prior to surgical resection. All three had massive tumors adjacent to the spine, extending over several vertebral segments, with myelographic evidence of epidural involvement in one patient. Tissue diagnosis by incisional biopsy confirmed the diagnosis of extraosseous osteogenic sarcoma in one, extraosseous Ewing's sarcoma in the second, and embryonal rhabdomyosarcoma in the third. All patients were treated with chemotherapy preoperatively. Marked regression in tumor size was noted, and surgical excision of the tumor along with involved segments of spine was easily accomplished. Histological examination following surgery revealed completely necrotic tumor in two patients. Preoperative chemotherapy for fully malignant tumors offers the following advantages: 1) extremely bulky and otherwise unresectable tumors can be reduced in size to enable complete surgical resection; 2) systemic chemotherapy is instituted earlier in the course of disease to prevent more effectively the appearance of metastases; and 3) the information gained regarding the sensitivity of the tumor to a particular chemotherapy regimen allows postoperative adjuvant chemotherapy to be used on a more rational basis.     

Chemotherapy in patients with teratoma with malignant transformation

OBJECTIVE: Germ-cell tumours (GCTs) with a non-GCT malignant component are a unique and rare phenomenon called teratoma with malignant transformation (TMT). The only published series of patients with TMT treated with chemotherapy comprised 10 patients. We report here our experience in treating 14 patients with TMT. PATIENTS AND METHODS: Sarcoma was identified in 10 of 14 patients, with rhabdomyosarcoma ranking first (n=4). Other histological types included adenocarcinoma (n=3) and bronchoalveolar carcinoma (n=1). Immunohistochemistry was performed to help in identifying the malignant non-GCT component. RESULTS: Primary treatment consisted of surgery alone in 4 patients. The remaining 10 patients received first-line cisplatin-based chemotherapy with resection of residual masses (n=5): 4 patients had a complete response and 5 had a partial response. Overall, 9 patients developed a relapse with a median time of 84 mo (range: 6-168). At relapse, 8 patients received a chemotherapy regimen directed to the non-GCT component. Four of these patients achieved a partial response. With a median follow-up of 59 mo (range: 3-180), 4 of 14 patients are alive, including 3 who are disease-free. CONCLUSION: To our knowledge, this is by far the largest reported European series of chemotherapy in TMT. Although TMT has a poor prognosis compared to GCT, its management may be improved by adapted chemotherapy associated with surgical resection of residual masses.     

Surgical Resection after Downsizing Chemotherapy for Initially Unresectable Locally Advanced Biliary Tract Cancer: A Retrospective Single-center Study

Background

Surgical resection is the only method for curative treatment of biliary tract cancer (BTC). Recently, an improved efficacy has been revealed in patients with initially unresectable locally advanced BTC to improve the prognosis by the advent of useful cancer chemotherapy. The aim of this study was to evaluate the effect of downsizing chemotherapy in patients with initially unresectable locally advanced BTC.

Methods

Initially unresectable locally advanced cases were defined as those in which therapeutic resection could not be achieved even by proactive surgical resection. Gemcitabine was administered intravenously once a week for 3 weeks followed by 1 week’s respite. Patients whose disease responded to chemotherapy were reevaluated to determine whether their tumor was resectable.

Results

Chemotherapy with gemcitabine was provided to 22 patients with initially unresectable locally advanced BTC. Tumor was significantly downsized in nine patients, and surgical resection was performed in 8 (36.4%) of 22 patients. Surgical resection resulted in R0 resection in four patients and R1 resection in four patients. Patients who underwent surgical resection had a significantly longer survival compared with those unable to undergo surgery.

Conclusions

Preoperative chemotherapy enables the downsizing of initially unresectable locally advanced BTC, with radical resection made possible in a certain proportion of patients. Downsizing chemotherapy should be proactively carried out as a multidisciplinary treatment strategy for patients with initially unresectable locally advanced BTC with the aim of expanding the surgical indication.