Childhood pancreatic tumors: a single institution experience

Purpose

The rarity and histopathologic diversity of primary pancreatic neoplasms in children have made it difficult to predict prognosis and to develop optimal management protocols.

Methods

A 90-year (1918-2007), single institution, retrospective review of all patients with neoplastic pancreatic masses was performed.

Results

Eighteen patients were identified with 7 distinct histopathologic subtypes. The most common were gastroenteropancreatic neuroendocrine, solid pseudopapillary, and acinar tumors. There were 6 benign and 12 malignant tumors. Six patients had disease outside the pancreas at their initial operation. There were 7 deaths (41%), 2 related to the initial operation, 3 from disease progression, 1 from a small bowel obstruction, and 1 from necrotizing pancreatitis. Five deaths were in patients with extrapancreatic disease found at initial operation. The median duration of follow-up for the 10 survivors was 41 months.

Conclusion

In adults, pancreatic ductal adenocarcinoma is by far the most common histopathologic subtype, with other subtypes more common in children. Stage is an important prognostic factor. Long-term disease-free survival in childhood pancreatic malignancies is achievable with complete surgical resection, prognosis, and adjuvant treatment, depending on the histopathologic type.     

Survival after gastric adenocarcinoma resection: Eighteen-year experience at a single institution

Gastric adenocarcinoma is the second leading cause of cancer death worldwide. In Western series, survival rates vary widely and are generally lower than those reported from Eastern series. We performed a retrospective analysis of cases operated on at the Johns Hopkins Hospital over the past 18 years and collected data on demographics, tumor characteristics, pathologic stage, treatment methods, complications, survival time, and other relevant factors. Survival according to stage of disease, Lauren tumor type, tumorlocation,time period, andadministration of adjuvant therapy wasanalyzed, andresultswerecompared with those of other Western series. During this period, 436 patients with gastric adenocarcinoma underwent resection. We have shown a statistically significant association between survival and margin status, stage of disease, and Lauren tumor type. Overall 5-year survival was 26%, and 5-year survival after R0 resection was 33%. No significant difference was detected between survival and tumor location, time period of treatment, or administration of adjuvant therapy. Analysis of various Western series reveals major differences between the cohorts under study, such as stage of disease, extent of resection, tumor type, and tumor location. Many of the reported differences among Western series may be due to cohort differences, such as stage of disease, extent of resection, tumor type, and tumor location.     

Primary lung tumors in children and adolescents: a 90-year experience

Purpose

Primary lung tumors in children are rare. A wide range of histopathologic tumor types occurs. The incidence of these lesions and their outcomes are still largely unknown. This study aims to determine the incidence of different primary lung tumors in children and to contribute data leading to the development of evidence-based treatment models.

Methods

A single institution retrospective review was performed with institutional review board approval. Patients were included if they had primary, nonhematologic lung tumors. Simple squamous papillomas subjected to endoscopic biopsy and not resected, and vascular lesions associated with multisystem lesions, such as hereditary hemorrhagic telangiectasia, were excluded. Medical records and pathologic material for patients from 1918 to 2008 were reviewed.

Results

Forty patients were identified (23 boys, 17 girls) with a mean age of 9.6 years (range, 3 months to 19 years). Fourteen distinct histopathologic tumor types were identified. The most common tumor types were carcinoid (8), inflammatory myofibroblastic tumor (7), and pleuropulmonary blastoma (6). Rare pediatric lung tumors including small cell carcinoma, adenocarcinoma, and pulmonary capillary hemangiomatosis were also seen. The mortality rate was 17.5% (7) in our series. Chemotherapy was used in 23% (9) and radiation in 20% (8) of the patients. Of the 33 survivors, 28 had follow-up with a median duration of 29.5 months (mean, 63.2 months; range, 1-471 months).

Conclusions

Primary lung tumors in children are rare and histopathologically diverse. The tumor spectrum involves many types not seen in adults, and unlike adults, patients rarely have a history of exposure to external predisposing factors. Although complete resection remains the standard for treatment of most tumors, addition of adjuvant therapy is dependent on both tumor stage and histopathologic type.     

Esthesioneuroblastoma: 25-year experience at a single institution

OBJECTIVES: To evaluate outcomes for patients with esthesioneuroblastoma treated at a single institution during a 25-year period. DESIGN: Eighteen patients with pathologic diagnosis of esthesioneuroblastoma between 1980 and 2004 were retrospectively identified. RESULTS: Two patients had Kadish A, seven had Kadish B, and nine had Kadish C disease. The mean follow-up was 71 months. Treatment regimens consisted of surgery alone (four patients), surgery followed by postoperative radiation (six patients), surgery followed by postoperative chemoradiotherapy (three patients), preoperative radiotherapy (two patients), preoperative chemoradiotherapy (one patient), chemoradiotherapy (one patient), and surgery plus chemotherapy (one patient). Surgical approaches (n = 17) consisted of 13 traditional craniofacial resections, one endoscopic-assisted cranionasal resection, and three minimally invasive endoscopic resections. The 10-year disease-specific survival was 80%. The overall recurrence-free survival at five and 10 years was 62% and 46%, respectively. Positive surgical margins and TNM staging predicted survival. CONCLUSION: Both endoscopic and open surgical approaches have been successful in treating a small number of esthesioneuroblastoma patients with high survival and low rate of surgical complications.     

Teratomas in infancy and childhood. A 54-year experience at the Children''s Hospital Medical Center

The clinical and pathologic features of 254 teratomas from 245 patients are reviewed. All patients were 21 years of age or younger and were treated at the Children's Hospital Medical Center from 1928 to 1982. Tumors arose in the following anatomic sites: sacrococcygeal (102), ovary (94), head and neck (14), retroperitoneum (12), mediastinum (11), testes (eight), central nervous system (nine), liver (two), abdominal wall, and back (one each). One hundred twenty-four tumors (49%) were detected in the newborn period. Teratomas characteristically contained elements derived from all three embryonic germ layers. Tumors with any recognizable component of embryonal carcinoma or other malignant germ cell elements at the time of initial surgery were excluded. Immature teratomas were significantly larger than mature tumors in nearly all sites where statistical analysis was possible. The single most important factor affecting prognosis was whether the tumor could be resected successfully at initial surgery. No patient who did not undergo surgery, or in whom only partial resection was possible, survived the disease--regardless of other treatments used. Based upon the experience reported here the authors conclude: 1) complete surgical resection is the treatment of choice for all childhood teratomas; and 2) this is one of the few childhood tumors where decisions regarding adjuvant therapy must be individualized, particularly with regard to site of origin and age of the patient.     

Anaplastic thyroid carcinoma: a 50-year experience at a single institution.

BACKGROUND: Anaplastic thyroid carcinoma (ATC) is among the most aggressive of human malignancies. However, there have been few large studies of histologically well-defined ATC. We report the results of a 50-year experience of this lethal malignancy. METHODS: We reviewed all cases of ATC managed in this institution between 1949 and 1999. One pathologist (J.R.G.) reviewed all pathologic material. Clinical details were obtained from medical records, and current status of all patients was determined. RESULTS: There were 134 cases, with a female-to-male ratio of 1.5:1 and a mean age of 67 years. Benign thyroid disease was present in 27 cases (20%) and well-differentiated thyroid carcinoma in 31 (23%). Sixty-two patients (46%) had distant metastases at diagnosis, and 98% of the tumors were locally invasive. Primary treatment was surgical for 96 patients (72%). Complete resection was achieved in 29 cases (30%), with "minimal residual disease" in 25. Neither extent of operation nor completeness of resection affected survival (P > .4). Postoperative radiotherapy gave slightly longer median survival (5 vs 3 months), which was not significant (P < .08). Multimodal therapy, including operation, chemotherapy, and radiotherapy, did not improve survival. CONCLUSIONS: The outlook for patients with ATC remains grim. Novel treatments for ATC are desperately needed.     

一家医院胃癌外科治疗疗效20年之变迁

目的探讨20年来胃癌临床病理特征及外科治疗效果的变迁。方法回顾性分析中山大学肿瘤防治中心1990年1月至2009年12月期间行胃癌手术切除的2518例患者的临床病理资料,将其按入院时间段分为前阶段组（1990-1999年）和近阶段组（2000-2009年）,比较两组患者的临床病理特征及生存差异。结果全组患者5年生存率为48．1％,其中行根治性切除患者5年生存率为53．7％。前阶段组与近阶段组患者肿瘤大小、病理类型、脉管癌栓、T分期、N分期、TNM分期和淋巴结清扫数目的差异有统计学意义（均P〈0．05）。对于根治性切除患者,近阶段组平均清扫淋巴结数目为（20．1±8.3）枚／例,明显多于近阶段组的（9．5±6．0）枚／例（P〈0．01）。前阶段组和近阶段组患者5年生存率分别为40．1％和51．5％,其中根治性切除患者5年生存率分别为45．7％和57．1％．差异均有统计学意义（均P〈0．05）。多因素预后分析证实,时间段是胃癌患者的独立预后因素（HR=0．763,95％CI：0．669～0．872）。结论与20世纪90年代相比,近10年来胃癌外科治疗效果得到了确切提高。     

Surgical treatment of small bowel cancer: A 20-year single institution experience

Small bowel malignancies are rare. The aims of this study were to evaluate the outcomes associated with surgical therapy for small bowel cancers and to define prognostic factors. The medical records of 96 consecutive patients with primary small bowel cancer (excluding lymphoma) treated at our institution over a 20 year period were reviewed. Survival was analyzed using the Kaplan-Meier method (mean follow-up period 57 months). Mean patient age was 56 years, and 58% of patients were male. Sixty percent of patients had an adenocarcinoma, 21% had a sarcoma, and 19% had a carcinoid tumor. The percentages of patients who underwent complete (curative) resection were 51%, 90%, and 50% for those with adenocarcinoma, sarcoma, and carcinoid tumor, respectively. For patients with adenocarcinoma who underwent curative resection, tumor (T) and node (N) stages were significant prognostic factors predicting overall survival. For patients with sarcomas who underwent curative resection, tumor grade was a significant prognostic factor predicting overall survival. The prognosis for patients with small intestinal carcinoid tumors is uniformly favorable. The prognosis for patients with sarcomas and adenocarcinomas is generally poor, although long-term survival is achieved by patients with favorable prognostic factors. Presented at the Society of Surgical Oncology Fifty-Sixth Annual Cancer Symposium, Los Angels, California, March 5–9, 2003.     

Mediastinitis after pediatric cardiac surgery: a 15-year experience at a single institution

BACKGROUND: The spectrum of sternal wound infections after cardiac surgery ranges from superficial infections to a deep sternal infection known as mediastinitis. Mediastinitis is a rare but clinically relevant source of postoperative morbidity and mortality in adult and pediatric patients after cardiac surgery. METHODS: We retrospectively identified all patients diagnosed with mediastinitis after cardiac surgery from January 1987 to December 2002 (17 patients/7,616 surgeries = 0.2%). Demographic data, cardiac diagnosis, cardiac surgery, hospital length of stay, associated medical diagnosis, and surgical treatment for mediastinitis were collected. RESULTS: Fifteen pediatric patients (age < 18 years) were diagnosed with mediastinitis (mean age at diagnosis 37.5 months, range 21 days to 17 years. The median postoperative day of diagnosis was 14 days (6 to 50 days). The most common organism was Staphylococcus species (n = 9). Six patients had an associated bacteremia. The median hospital length of stay for all patients was 42.5 days (range 16 to 163 days). The hospital mortality was 1 of 15 (6%). Each patient was treated with intravenous antibiotics; sternal debridement; and rectus abdominus flap reconstruction (n = 7), pectoralis muscle flap reconstruction (n = 3), omentum reconstruction (n = 1), or primary sternal closure (n = 4). Three patients have undergone redo-sternotomy with orthotopic heart transplantation, bidirectional cavopulmonary anastomosis, and replacement of a right ventricle to pulmonary artery homograft. CONCLUSIONS: Timely diagnosis, aggressive sternal debridement, and liberal use of rotational muscle flaps can potentially minimize the morbidity and mortality in pediatric postoperative cardiac patients. Subsequent redo-sternotomy has not been problematic.     

The Bianchi procedure: a 20-year single institution experience

Background/Purpose

The emergence of improved outcomes for small bowel (SB) transplantation has raised questions regarding the utility of autologous intestinal lengthening for patients with short bowel syndrome (SBS). Chronic immunosuppression, multiple hospitalizations, and posttransplant lymphoproliferative disease are significant adverse factors. The purpose of this study is to evaluate the 20-year single institution experience with the Bianchi procedure and analyze its role in the management of pediatric SBS.

Methods

Medical records for 19 consecutive patients who underwent the Bianchi procedure from 1984 to 2004 were reviewed. Patients were categorized into 3 groups: less than 5 years, 5 to 9.9 years, and 10 years or more after surgery. Various outcome variables were evaluated. Data are presented in tabular format as the number of patients (%) or average (range).

Results

Nineteen patients were included in the study. Of 16 patients weaned from total parenteral nutrition (TPN), 7 (44%) responded to Bianchi procedure alone and 9 patients (56%) required SB transplant at an average of 4.09 years (range, 0.7-13.64 years) post-Bianchi. Four patients (21%) died, 1 received SB transplant and died of unrelated causes, and 3 were still on TPN and had not received SB transplantation.

Conclusion

The Bianchi procedure facilitated weaning from TPN and eliminated the need for supplemental nutrition in select patients. Although the role of surgery is primarily adjunctive in the treatment of SBS, it offers therapeutic benefit in decreasing parenteral nutrition requirements and promoting intestinal adaptation. In particular, the Bianchi procedure has significant potential to improve the prognosis of pediatric patients with SBS.     

Carvedilol in children with cardiomyopathy: 3-year experience at a single institution.

BACKGROUND: Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. METHODS: We reviewed the medical records of 24 children with dilated cardiomyopathy and left ventricular ejection fraction of 相似文献   

Benefits of laparoscopic adrenalectomy: a 10-year single institution experience

INTRODUCTION: We aimed to compare the outcomes of laparoscopic and open adrenalectomies and to assess the impact of the availability of advanced laparoscopy on adrenal surgery at our institution. MATERIALS AND METHODS: A retrospective analysis of data of all patients who underwent adrenalectomy at the University of Massachusetts Medical Center over a 10-year period. RESULTS: Sixty-four consecutive patients underwent adrenalectomy during the study periods. There were 19 open (OA) and 45 laparoscopic (LA) adrenalectomies performed. There was no significant difference between the average size of adrenal masses removed for the LA and the OA groups [4.3 vs. 5.5 cm, respectively (P=0.23)]. LA proved superior to OA, resulting in shorter operative times (171 vs. 229 min, P=0.02), less blood loss (96 vs. 371 mL, P<0.01), shorter time to regular diet (1.9 vs. 4.4 d, P<0.001), and shorter hospital stay (2.5 vs. 5.8 d, P=0.02). In addition, the average annual number of adrenalectomies increased significantly since the establishment of our advanced laparoscopic program (10.0 vs. 2.0, P=0.02). CONCLUSIONS: LA offers superior results when compared to OA in terms of operative time, blood loss, return of bowel function, duration of hospital stay, and functional recovery. The availability of advanced laparoscopy has resulted in a significant increase in the number of adrenalectomies performed at our institution without a shift in surgical indications.     

A 10-year experience with 290 pancreas transplants at a single institution.

Since our report at the 1984 American Surgical Association meeting of 100 pancreas transplants from 1966 through 1983, another 190 have been performed. The current series, begun in 1978, now numbers 276 cases, and includes 133 nonuremic recipients of pancreas transplants alone (PTA), 46 simultaneous pancreas/kidney transplants (SPK), and 97 pancreas tranplants after a kidney transplant (PAK). Duct management techniques used were free intraperitoneal drainage in 44 cases, duct occlusion in 44, enteric drainage in 89, and bladder drainage in 128. The 1-year patient and graft survival rates in the entire cohort of 276 were 91% and 42%. One-year patient survival rates were 88% in the first 100, 91% in the second 100, and 92% in the last 76 cases; corresponding 1-year graft survival rates were 28%, 47%, and 56% (p less than 0.05). A prospective comparison of bladder drainage (n = 82) versus enteric drainage (n = 46) in PAK/PTA cases since November 1, 1984 favored bladder drainage (1-year graft survival rates of 52% vs. 41%) because of urinary amylase monitoring. The best results were in recipients of primary SPK bladder-drained transplants (n = 39), with a 1-year pancreas graft survival rate of 75%, kidney graft survival rate of 80%, and patient survival rate of 95%. Logistic regression analysis, with 1-year graft function as the independent variable, showed significant (p less than 0.05) predictors of success (odds ratio) to be technique: bladder drainage (5.8) versus enteric drainage (2.5) versus duct injection (1.0); category: SPK (6.0) versus PAK from same donor (3.2) versus PAK from different donor (1.2) versus PTA (1.0); and donor HLA DR mismatch: 0 (5.0) versus 1 (2.5) versus 2 (1.0) antigens. On April 1, 1989, 90 patients had functioning grafts (60 euglycemic and insulin-free for more than 1 year, 10 for 5 to 10 years); these, along with 24 others whose grafts functioned for 1 to 6 years before failing, are part of an expanding cohort in whom the influence of inducing a euglycemic state on pre-existing secondary complications of diabetes is being studied. Only preliminary data is available. In regard to neuropathy, at more than 1 year after transplant in patients with functioning grafts, conduction velocities in some nerves were increased over baseline. In regard to retinopathy, deterioration in grade occurred in approximately 30% of the recipients by 3 years, whether the graft functioned continuously or failed early, but thereafter retinopathy in the patients with functioning grafts remained stable.(ABSTRACT TRUNCATED AT 400 WORDS)     

Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): A 10-year experience

Background: Prior studies have shown a high rate of local recurrence and a dismal overall prognosis in malignant peripheral nerve sheath tumors (MPNSTs). Methods: Thirty-three patients who had undergone primary treatment for localized extremity MPNSTs between 1982 and 1992 were reviewed. These cases were derived from a prospective database of 890 adult extremity soft-tissue sarcomas (STS). MPNSTs were compared with other extremity STS. Results: MPNSTs were more often high grade and deep compared with other extremity STS (94% vs. 72% [p=0.009] and 97% vs. 76% [p=0.01], respectively). Location (upper or lower extremity), size (>5 cm vs. ⩽5 cm), and status of margins after surgical resection (positive or negative for disease) did not differ between the two groups. When deep and high-grade MPNSTs were compared with deep and high-grade STS, a more aggressive local treatment was applied to MPNSTs with a higher number of amputations for MPNSTs (32%) compared with STS (9%; p<0.001). In order to obtain adequate margins, 16 of 21 MPNSTs arising from major nerves required either amputation (n=8) or nerve resection (n=8). Adjuvant radiotherapy was used in 48% of deep and high-grade MPNSTs, and 3-year local disease-free survival was 70%. Survival of deep and high-grade MPNSTs was comparable with other deep and high-grade STS (3-year survival 50% vs. 69%, respectively; p=0.1). Conclusion: MPNSTs show adverse clinicopathologic features compared with other STS. However, when treated aggressively, MPNSTs have an outcome similar to other deep and high-grade extremity STS. Presented at the 47th Annual Cancer Symposium of The Society of Surgical Oncology, Houston, Texas, March 17–20, 1994.     

腹腔镜下胃部分切除治疗胃间质瘤:附22例报告

目的：探讨腹腔镜下胃部分切除治疗胃间质瘤的可行性及临床应用价值。 方法：回顾分析施行腹腔镜下胃部分切除治疗胃间质瘤22例的临床资料。 结果：肿瘤位于小弯侧13例,大弯侧9例;直径1.5~3 cm。22例手术均顺利完成,术中快速冷冻切片提示胃梭形细胞瘤,术后病理切缘均阴性,手术时间40~110 min（平均55 min）,术中出血量50～150 mL（平均80 mL）,术后24～48 h肠道功能恢复,术后住院3～7 d,无1例发生并发症。术后随访1～17个月,胃镜复诊未发现复发及转移。 结论：腹腔镜下胃部分切除治疗肿瘤为1.5～3 cm的胃间质瘤是安全、可行、有效的。胃腔外切除有胃内容物不外溢,污染少等优点,可不放置腹腔引流管,值得临床推广。     

Reversal of Roux en Y gastric bypass: largest single institution experience

BackgroundThere is a paucity of literature on patients who have undergone reversal of Roux-en-Y gastric bypass (RYGB) to normal anatomy. We present the largest single institution experience with reversal of RYGB for serious chronic complications.ObjectiveTo describe our experience including indications, outcomes, and complications of RYGB reversal.SettingAcademic-affiliated private practice.MethodsRetrospective review of 48 patients who underwent laparoscopic reversal of RYGB between 2012 and 2016.ResultsNinety-six percent (n = 46) of patients were female, and the mean age was 48.6 (range, 23–72). Indications for reversal of RYGB included marginal ulcer (n = 25, 12 of whom were malnourished and 17 had coexisting substance abuse), malnutrition alone (n = 11), chronic pain and nausea (n = 7), and postprandial hyperinsulinemic hypoglycemia (n = 5). Overall 30-day complication rate was 29% (n = 14), including gastrogastric anastomotic leak (n = 5), sepsis (n = 5), and bleeding requiring transfusion (n = 3). Weight gain after surgery increased in all patients, especially those patients deemed severely malnourished. All patients reported resolution of symptoms leading to reversal of RYGB, although 58% of patients were lost to follow-up at 1 year after surgery.ConclusionsLaparoscopic reversal of Roux-en-Y gastric bypass is a complex revisional operation that can be safely performed in a select group of patients with serious complications. The main indications for reversal of RYGB included malnutrition with and without recalcitrant marginal ulcers. Weight gain and resolution of malnutrition occurred soon after reversal of gastric bypass. Because the complication rates are high, reversal should be considered only after all salvage attempts have failed. Reversal to normal anatomy carries high morbidity, including sepsis, leaks and bleeding, high reoperative rates, and readmission. Although reversal of RYGB has a role in the treatment of a select group of patients, it should be undertaken by surgeons with considerable experience in RYGB revision.