Management of intravascular thrombus in cases of bilateral Wilms tumor or horseshoe kidney

PurposeTo describe the oncologic and surgical management of bilateral Wilms tumor or Wilms tumor arising in a horseshoe kidney with intravenous tumor thrombus to help pediatric surgeons negotiate this rare and difficult anatomic circumstance.MethodsA single-institution, retrospective medical record review identified 4 cases of bilateral WT and one case of WT arising in a horseshoe kidney with intravenous tumor thrombus between 2009 and 2021. The presentation, imaging, chemotherapy regimen, intraoperative approach, and surgical and oncologic outcomes were reviewed for each of these patients.ResultsAll patients received a total of 12 weeks of neoadjuvant chemotherapy. In two patients, a staged approach to the bilateral tumors was undertaken with the first side being operated on after six weeks of therapy and the other side undergoing surgery after an additional six weeks of therapy. Of five patients, four underwent nephron-sparing surgery of all tumors and one underwent unilateral radical nephroureterectomy with contralateral nephron-sparing surgery. Tumor thrombectomy was performed in four of five cases; one patient demonstrated a complete response of the intravenous tumor thrombus to neoadjuvant chemotherapy and did not require thrombectomy. Three patients received adjuvant flank radiotherapy. Three patients developed medically managed stage II or III chronic kidney disease and no patient required renal replacement therapy or kidney transplant to date.ConclusionNephron-sparing surgery is feasible and safe to perform in selected cases of bilateral Wilms tumor with intravascular thrombus by utilizing three-drug neoadjuvant chemotherapy, staged approaches to each kidney when appropriate, and detailed preoperative and/or intraoperative mapping of renal venous anatomy. Successful nephron-sparing surgery with tumor thrombectomy is dependent on a branched renal venous system or the presence of accessory renal veins.Level of evidenceLevel 4.     

Preoperative Wilms tumor rupture in children

Purpose

According to the guidelines of International Society of Pediatric Oncology (SIOP) and National Wilms Tumor Study (NWTS), Wilms tumor with preoperative rupture should be classified as at least stage III. Few clinical reports can be found about preoperative Wilms tumor rupture. The purpose of this study was to investigate our experience on the diagnosis, treatment and prognosis of preoperative Wilms tumor rupture.

Methods

Patients with Wilms tumor who underwent treatment according to the NWTS or SIOP protocol from January 2008 to September 2017 in Beijing Children’s Hospital were reviewed retrospectively. The clinical signs of preoperative tumor rupture were acute abdominal pain, and/or fall of hemoglobin. The radiologic signs of preoperative tumor rupture are as follows: (1) retroperitoneal and/or intraperitoneal effusion; (2) acute hemorrhage located in the sub-capsular and/or perirenal space; (3) tumor fracture communicating with peritoneal effusion; (4) bloody ascites. Patients with clinical and radiologic signs of preoperative tumor rupture were selected. Patients having radiologic signs without clinical symptoms were also selected. The clinical data, treatments and outcomes were analyzed. Meanwhile, patients without preoperative Wilms tumor rupture during the same period were collected and analyzed.

Results

565 Patients with Wilms tumor were registered in our hospital. Of these patients, 45 patients were diagnosed with preoperative ruptured Wilms tumor. All preoperative rupture were confirmed at surgery. Spontaneous tumor rupture occurred in 41 patients, the other 4 patients had traumatic history. Of the 45 patients, 41 were classified as stage III, 3 patients with pulmonary metastases were classified as stage IV, and one patient with bilateral tumors were classified as stage V. Of these patients with preoperative tumor rupture at stage III, 30 patients had clinical and radiologic signs of tumor rupture, the other 11 patients had radiologic signs without clinical symptoms. Among the 41 patients at stage III, 13 patients had immediate surgery without preoperative chemotherapy (immediate group), and 28 patients had delayed surgery after preoperative chemotherapy (delayed group). In immediate group, 12 patients had localized rupture, 1 patient underwent emergency surgery because of continuous bleeding. In delayed group, 4 had inferior vena cava tumor embolus (1 thrombus extended to inferior vena cava behind the liver, three thrombi got to the right atrium), 4 crossed the midline with large tumors, 20 had extensive rupture without localization. In immediate group, tumor recurrence and metastasis developed in 2 patients, and no death occurred. In the delayed group, tumor recurrence and metastasis developed in 8 patients, and 7 patients died. During the same period, 41 patients were classified as stage III without preoperative rupture. In the non-ruptured group, tumor recurrence and metastasis developed in 3 patients, and 4 patients died. The median survival time in the ruptured group (both immediate group and delayed group) and non-ruptured group were (85.1 ± 7.5) and (110.3 ± 5.6) months, and the 3-year cumulative survival rates were 75.1% and 89.6%, respectively. The overall survival rate between the ruptured and non-ruptured groups showed no statistic difference (P = 0.256). However, there was significant difference in recurrence or metastasis rate between the ruptured and non-ruptured groups (24.4% vs 7.3%; P = 0.031).

Conclusion

Contrast-enhanced computed tomography (CT) and ultrasonography (US) are of major value in the diagnosis of preoperative tumor rupture, and immediate surgery or delayed surgery are available therapeutic methods. The treatment plan was based on patients’ general conditions, tumor size, position and impairment degree of tumor rupture, extent of invasion and experience of a multidisciplinary team (including surgeon and anesthesiologists). In our experience, for ruptured preoperative tumor diagnosed with stage III, the criteria for immediate surgery are as follows: tumor not acrossing the midline, tumor without inferior vena cava thrombus, localized rupture, being capable of complete resection. Selection criteria for delayed surgery after preoperative chemotherapy are as follows: large tumors, long inferior vena cava tumor thrombus, tumors infiltrating to surrounding organs, unlocalized rupture, tumors can not being resected completely. Additionally, patients with preoperative Wilms tumor rupture had an increased risk of postoperative recurrence or metastasis.

     

Preoperative cholangitis is an independent risk factor for mortality in patients after pancreatoduodenectomy for pancreatic cancer

ObjectivesPreoperative biliary stenting is required for patients with obstructive jaundice from pancreatic adenocarcinoma who are receiving neoadjuvant chemotherapy. While in most patients this approach results in durable biliary drainage, some patients develop cholangitis during neoadjuvant treatment. Further, several studies have shown that preoperative cholangitis in patients with hepatobiliary malignancies can result in substantially unfavorable outcomes. The aim of this study was to evaluate the impact of preoperative cholangitis in patients who underwent pancreaticoduodenectomy after completing neoadjuvant chemotherapy.MethodsParticipants: all adult patients (n = 449) diagnosed with pancreatic adenocarcinoma from January 1st, 2013 to March 31st, 2018 who pursued treatment at the Massachusetts General Hospital were screened. Of these 449 patients, 97 met final inclusion criteria of receiving neoadjuvant chemotherapy with intent to pursue curative surgery. Data were collected via retrospective chart review including baseline characteristics, survival, episodes of preoperative cholangitis, and surgical complications.ResultsIn patients completing successful pancreaticoduodenectomy surgery, preoperative cholangitis is associated with increased mortality (HR 2.67, 95% CI:1.16–6.13). This finding is independent of postoperative outcomes or tumor recurrence rate. The presence of cholangitis did not impact completion of neoadjuvant chemotherapy (92% vs 85%, p = 0.5) or ability to proceed to surgery (76% vs 75%, p = 1.0). Preoperative cholangitis was not associated with postoperative morbidity (42.1% vs 45.1%, p = 1.0).ConclusionsOne episode of cholangitis during neoadjuvant chemotherapy is associated with increased mortality following successful pancreaticoduodenectomy, independent of immediate postoperative outcomes or tumor recurrence. Preoperative cholangitis does not affect ability to pursue neoadjuvant chemotherapy or complete successful surgery. Patients who develop cholangitis during the neoadjuvant chemotherapy treatment phase may reflect a distinct phenotype of patients with PDAC with a complex and more challenging clinical course.     

Hepatoblastoma in childhood,long term survival achieved: 2 case reports and literature review

IntroductionHepatoblastoma is the most common primary liver tumor for children under 5 years of age. It usually presents as an abdominal mass, symptomatic only when large enough to cause mass effect on nearby organs. Symptoms such as early satiety, anorexia, abdominal pain or weight loss are the most common. Diagnosis depends on imaging studies, AFP levels and percutaneous biopsy. Treatment modality is usually surgical with neoadjuvant chemotherapy.CasesIn this article, we present 2 cases of hepatoblastoma treated 15 years ago by neoadjuvant chemotherapy and surgery, and are presenting for long term follow-up with complete disease remission.DiscussionComplete resection and remission can be achieved as demonstrated below by our 2 cases of hepatoblastoma, especially when performing a true anatomical hepatectomy, along with a neoadjuvant chemotherapy regimen. Although one of the cases did not respond to chemotherapy very well a complete resection was achieved and therefore a disease free survival of 15 years.ConclusionHepatoblastoma are rare tumors of the pediatric age group. Management depends highly on combined surgical and pediatric oncological knowledge. A complete disease remission can be achieved when both modalities are treatment are optimal. Therefore, hepatoblastoma cases should be referred to specialized centers for management.     

The use of preoperative chemotherapy in Wilms' tumor with contained retroperitoneal rupture

Purpose

The National Wilms Tumor Study currently describes 3 indications for the use of preoperative chemotherapy: extensive caval involvement, bilateral tumors, and patients who only have a single kidney. However, the management of patients who present with a contained retroperitoneal rupture is not specifically addressed. This is relevant because of the strong possibility of peritoneal contamination when performing a primary resection and the resultant requirement for total abdominal radiation. The use of neoadjuvant chemotherapy in this subgroup of patients may be warranted.

Methods

We retrospectively reviewed our experience with Wilms' tumor and identified 3 cases with contained rupture at presentation. Details of their initial evaluation and therapy, resection and pathologic findings, and follow-up constitute this report. Institutional review board waiver was obtained for the purposes of this review.

Results

Two male patients, aged 2.9 years, and 1 female patient, aged 9.3 years, were identified. All patients received preoperative chemotherapy with vincristine and dactinomycin (n = 1) plus doxorubicin (n = 2) for 4 to 6 weeks before surgical resection. One patient underwent pretreatment computed tomography-guided biopsy of the kidney mass for diagnostic purposes. Presurgical computed tomographic scans showed resolution of perinephric blood and fluid with tumor shrinkage. Histopathologic analyses showed all tumors were resected with negative margins, and there was no intraoperative tumor spillage. All patients received 1050 to 1080 cGy of flank radiation postoperatively. All patients are currently alive at follow-up without evidence of local recurrence or distant disease.

Conclusions

Neoadjuvant chemotherapy allowed for complete resection and avoidance of total abdominal radiation in 3 patients with ruptured Wilms' tumor and hematoma within the retroperitoneum. These data support the use of initial chemotherapy in children with retroperitoneal rupture and hematoma of Wilms' tumor at diagnosis.     

The occurrence of Wilms tumor in horseshoe kidneys: a report from the National Wilms Tumor Study Group (NWTSG)

Background/Purpose: An increased incidence of Wilms tumor has been noted in patients with a horseshoe kidney. These represent a difficult diagnostic and therapeutic challenge. The charts of all National Wilms Tumor Study Group (NWTSG) patients with Wilms tumor occurring in a horseshoe kidney were reviewed. Methods: From 1969 to 1998, 8,617 patients were enrolled in the NWTSG. Forty-one patients were found to have a Wilms tumor arising in a horseshoe kidney for an incidence of 0.48%. Their records were reviewed retrospectively. Results: Horseshoe kidney was not recognized preoperatively in 13 patients, 10 of whom were evaluated with computed tomography (CT). Four of the 10 also had renal ultrasonography and one an intravenous pyelogram (IVP). Two of the 13 were evaluated with an IVP only, and the last had no preoperative imaging studies performed. Stage at presentation was stage I, 10 pts; stage II, 10; stage III, 12; stage IV, 6; stage V, 3. Primary surgical resection was performed in 26 patients, including 23 nephrectomies and 3 partial nephrectomies. Fifteen children were treated with preoperative chemotherapy after initial biopsy of the tumor. The mean total remaining renal parenchyma after all operations (excluding treatment of relapses) was approximately 75%. Surgical complications occurred in 14.6% of patients, including 2 urine leaks, 2 ureteral obstructions, and 1 ureteral injury. Two patients had transient renal failure. Conclusions: The diagnosis of horseshoe kidney often was missed on preoperative imaging. Accurate preoperative diagnosis is important in planning the operative approach and may help to decrease complications related to transection of the urinary collecting system. Although 37% of patients with Wilms tumor arising in a horseshoe kidney were judged inoperable at initial exploration, all were amenable to resection after chemotherapy. J Pediatr Surg 37:1134-1137.     

Fluorescence-guided lymph node sampling is feasible during up-front or delayed nephrectomy for Wilms tumor

BackgroundLymph node sampling is critical to surgical staging in Wilms tumor; failure to sample lymph nodes is associated with under-staging and an increased incidence of local relapse. However, no standard lymphatic mapping method is currently being utilized for Wilms tumor to aid identification of regional draining lymph nodes. Herein, we describe the use of fluorescence-guided lymphatic mapping for Wilms tumor.Materials and methodsTwo tertiary level referral centers independently began indocyanine green (ICG) fluorescence-guided nodal mapping. In one center, this was achieved with ipsilateral intra-parenchymal (IP) injection of ICG during minimally invasive tumor nephrectomy (MIN) following neoadjuvant chemotherapy and in the other, with Peri?Hilar (PH) injection during upfront, open tumor nephrectomy (ON). Successful lymph node mapping was defined as the presence of fluorescence signal in draining lymph nodes.ResultsEight patients (median age of 2.5 years) underwent fluorescence-guided lymphatic mapping (four IP and four PH injection). Lymphatic mapping was successful in seven patients (88%) including each of the four patients with IP injection.ConclusionsFluorescence-guided lymphatic mapping of Wilms tumor drainage is feasible by both IP injection and PH injection techniques. However, whether lymphatic mapping improves the precision of lymph node sampling is unknown and should be studied in prospective trials.     

Intravascular Extension of Wilms Tumor

OBJECTIVE: To define the incidence and manifestations of and optimal therapy for children with intravascular extension of Wilms tumor. METHODS: Children on a collaborative study of Wilms tumor who had intravascular extension into the inferior vena cava (IVC) or atrium were identified. Surgical checklists and surgical and pathology reports were reviewed. RESULTS: One hundred sixty-five of 2,731 patients had intravascular extension of Wilms tumor. The level of extension was IVC in 134 and atrium in 31. Sixty-nine had received preoperative therapy (55 with IVC extension and 14 with atrial extension) for a median of 8 weeks. Complications during preoperative chemotherapy were seen in five patients (tumor embolism and tumor progression in one each, and three with adult respiratory distress syndrome, one of which was fatal). The intravascular extension of the tumor regressed in 39 of 49 children with comparable pre- and posttherapy radiographic studies, including 7 of 12 in whom the tumor regressed from an atrial location, thus obviating the need for cardiopulmonary bypass. Surgical complications occurred in 36.7% of the children in the atrial group and 17.2% in the IVC group. The frequency of surgical complications was 26% in the primary resection group versus 13.2% in children with preoperative therapy. When all the complications of therapy were considered, including those that occurred during the interval of preoperative chemotherapy (one of the five also had a surgical complication), the incidence of complications among those receiving preoperative therapy was not statistically different from the incidence among those who underwent primary resection. The difference in 3-year relapse-free survival (76.9% for 165 patients with intravascular extension, 80.3% for 1,622 patients with no extension) was not statistically significant whether or not it was adjusted for stage and histology. CONCLUSIONS: Preoperative treatment of these children may facilitate resection by decreasing the extent of the tumor thrombus, but the overall frequency of complications is similar in both groups.     

Venous thromboembolism risk in patients receiving neoadjuvant chemotherapy for bladder cancer

IntroductionThere is limited evidence to inform thromboprophylaxis use for patients receiving neoadjuvant chemotherapy prior to surgery in bladder cancer. We sought to determine the incidence of venous thromboembolism (VTE) in patients receiving neoadjuvant chemotherapy and cystectomy. We also assessed if the Khorana score was associated with VTE risk.MethodsA retrospective cohort study was performed on consecutive patients who received a radical cystectomy for bladder cancer at The Ottawa Hospital between January 2016 and August 2020. Demographic information, chemotherapy data, operative characteristics, VTE and bleeding outcomes were collected from the start of treatment to 90 days postoperative. A Khorana score was calculated for each patient who received neoadjuvant chemotherapy. The primary outcome for this study was the incidence of VTE from the time the patient started treatment with neoadjuvant chemotherapy until 90 days post-cystectomy. Secondary outcomes included risk factors for VTE during neoadjuvant chemotherapy.ResultsAmong 181 radical cystectomy cases during the study period, 123 had muscle-invasive disease and 72 (39.8%) received neoadjuvant chemotherapy. Eighteen (25.0%) patients who received neoadjuvant chemotherapy and radical cystectomy developed a VTE from the start of chemotherapy to 90 days postoperative. Thirteen of the 18 VTEs (72%) occurred while the patient was receiving chemotherapy. In multivariable analysis, the only factor associated with a significantly increased risk of VTE was treatment with neoadjuvant chemotherapy (Relative risk (RR) 3.05, 95% confidence interval [CI] 1.16–8.02; P = 0.02). A higher Khorana score was not associated with an increased risk of VTE in patients who received neoadjuvant chemotherapy (RR = 0.33, 95% CI 0.08–1.28, P = 0.11). One (1.4%) patient had a major bleeding event during neoadjuvant chemotherapy.ConclusionsPatients receiving neoadjuvant chemotherapy and radical cystectomy are at very high-risk of VTE. Prospective studies that assess the benefits and harms of pharmacologic thromboprophylaxis in this population are needed.     

Intussusception following resection of Wilms tumor

Postoperative intussusception is a documented complication of pediatric surgical and pediatric urologic abdominal operations. In contrast to "primary" intussusception's triad of crampy abdominal pain, palpable abdominal mass, and "currant jelly" stools, postoperative intussusception is generally characterized by abdominal pain and vomiting. An abdominal mass is not usually palpable, and few children have bloody stools. Proper diagnosis and treatment may be delayed because of similar abdominal symptoms in children who may be receiving radiation and chemotherapy, or with prolonged ileus. Two children operated on for Wilms tumor demonstrate the need for awareness of this potential problem in the postoperative patient.     

Neoadjuvant Treatment for Resectable Cancer of the Esophagus and the Gastroesophageal Junction: A Meta-Analysis of Randomized Clinical Trials

Background: There is no general agreement on the effect of neoadjuvant treatment for esophageal cancer on patient survival.Methods: A meta-analysis was performed to determine the effect of preoperative treatment on survival of patients with resectable esophageal cancer and the effect of preoperative treatment on patient mortality. A standard variance-based method was used to derive summary estimates of the absolute difference in both 2-year survival and treatment-related mortality.Results: Eleven randomized trials involving 2311 patients were analyzed. Preoperative chemotherapy improved 2-year survival compared with surgery alone: the absolute difference was 4.4% (95% confidence interval [CI], .3%–8.5%). Marginal evidence of heterogeneity was eliminated by restricting attention to the four most recent studies, which increased the estimate to 6.3% (95% CI, 1.8%–10.7%). For combined chemoradiotherapy, the increase was 6.4% (nonsignificant; 95% CI, –1.2%–14.0%). Treatment-related mortality increased by 1.7% with neoadjuvant chemotherapy (95% CI, –.9%—4.3%) and by 3.4% with chemoradiotherapy (95% CI, –.1%–7.3%), compared with surgery alone.Conclusions: There seems to be a modest survival advantage for patients who receive neoadjuvant chemotherapy followed by surgery, as compared with surgery alone. There is an apparent increase in treatment-related mortality, mainly for patients who receive neoadjuvant chemoradiotherapy.     

Effective Local Control and Long-Term Survival in Patients with T4 Locally Advanced Breast Cancer Treated with Breast Conservation Therapy

Background: The presence of skin involvement has been accepted as a relative contraindication to breast preservation because it is believed to be associated with an increased local failure rate. This study was conducted to assess the outcome of a carefully selected group of patients who presented with breast cancer involving the skin and who had breast conservation therapy (BCT) following neoadjuvant chemotherapy.Methods: Between 1987 and 1999, 33 patients with stage IIIB or IIIC breast cancer completed treatment consisting of four cycles of neoadjuvant chemotherapy, lumpectomy, radiation therapy, and consolidative chemotherapy. Clinicopathologic factors were analyzed and patients were followed for locoregional and distant recurrence.Results: Initial median tumor size was 7 cm. All patients had skin involvement, defined as erythema, skin edema, direct skin invasion, ulceration, or peau dorange. Following chemotherapy, median pathologic tumor size was 2 cm. Complete resolution of skin changes occurred in 29 patients (88%). At median follow-up time of 91 months in surviving patients, 26 patients (79%) were alive without evidence of disease. The 5-year, disease-free survival rate was 70%, and the 5-year overall survival rate was 78%. The actuarial ipsilateral breast cancer recurrence rate was 6% at 5 years.Conclusions: Patients who present with T4 breast cancer who experience tumor shrinkage and resolution of skin changes with neoadjuvant chemotherapy represent a select group of patients who can have BCT. These patients have favorable rates of long-term local control and survival. Mastectomy is not mandatory for all patients with breast cancer who present with skin involvement.Presented at the 57^th Annual Society for Surgical Oncology Cancer Symposium in New York City, New York, March 18–21, 2004     

RENAL ANGIOMYOLIPOMA IN CHILDREN: DIAGNOSTIC DIFFICULTY IN 3 PATIENTS

Purpose

Because angiomyolipoma is less common in children than in adults, its diagnosis can be difficult. We present 3 cases of pediatric angiomyolipoma in which diagnostic problems resulted due to the presenting characteristics.

Materials and Methods

We report on 3 children with unilateral renal angiomyolipoma. Computerized tomography (CT) and ultrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diameter, respectively. A correct diagnosis was not made preoperatively in any case by CT, ultrasound or fine needle biopsy. Wilms tumor was suspected in the first patient who received preoperative chemotherapy. Imaging was inconclusive in the other 2 cases.

Results

All patients underwent surgical exploration and subsequent nephrectomy due to the large size of the tumor. At followup 33, 23 and 13 months postoperatively all children were well without signs of recurrence.

Conclusions

It has been reported that the demonstration of fat on renal ultrasound and CT can diagnose angiomyolipoma in 95% of the cases. Most radiologists rely solely on CT demonstration of lipid density in the renal mass to diagnose angiomyolipoma but the identification at imaging of lipid tissue may be difficult in small tumors. In our cases the fat content of the tumors was less than 10% despite the large size. This low fat content results in misdiagnosis, since fatty tissue is also present in other renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors. We recommend conservative surgery when tumor size permits in pediatric patients with angiomyolipoma to avoid chemotherapy.     

Effect of increases in tumor volume after neoadjuvant chemotherapy on the outcome of stage II osteosarcoma regardless of histological response

Background  We assessed volume changes after neoadjuvant chemotherapy and evaluated relations between tumor size changes and clinical characteristics. In addition, we sought to determine whether tumor size change influences patient outcome. Methods  The records of 127 patients with stage II osteosarcoma who showed more than a 15% volume change after chemotherapy were retrospectively reviewed. Patients were divided into two groups depending on whether tumors increased or decreased in size. Fisher’s exact test was performed to analyze correlations between tumor size changes and clinicopathological variables. Five-year metastasis-free survival and overall survival were evaluated using univariate and multivariate analyses. Results  A total of 71 patients (55.9%) showed a decrease in tumor volume, and 56 patients (44.1%) showed an increase. An increase in tumor volume after neoadjuvant chemotherapy was found to be positively correlated with a poor histological response and subsequent metastasis. Univariate analysis identified the following parameters as poor prognostic factors: age ≤15 years (P = 0.03), American Joint Committee on Cancer (AJCC) stage IIB (P = 0.02), a subtype other than osteoblastic (P < 0.01), a poor histological response (P < 0.001), and increased tumor volume after preoperative chemotherapy (P < 0.0001). Multivariate analysis revealed that AJCC stage IIB (P = 0.006) and an increase in tumor volume after preoperative chemotherapy (P < 0.001) both independently shortened metastasis-free survival. However, a poor histological response lost its prognostic significance (P = 0.34). Conclusions  Increased tumor volume after neoadjuvant chemotherapy independently shortened metastasis-free and overall survival in AJCC stage II osteosarcoma patients. Tumor volume changes may serve as a basis for risk-adapted therapy when used in combination with other prognostic factors.     

Repeat nephron-sparing surgery for children with bilateral Wilms tumor

Background

Renal insufficiency is a significant complication of Wilms tumor treatment in the 5% with bilateral disease. Nephron-sparing surgery (NSS) is recommended after neoadjuvant chemotherapy initially. However, the role of NSS in recurrent disease is unknown. We reviewed our experience to assess the feasibility and oncologic and functional outcomes of repeat NSS for children with recurrent disease.

Methods

A retrospective review was performed of all children treated at our institution for bilateral, favorable histology (FH) Wilms tumor. Patients undergoing repeat NSS for locally recurrent disease were identified. The outcomes evaluated included tumor recurrence, renal function, and patient survival.

Results

Since 2001, 36 children with bilateral FH Wilms tumor have been treated at our institution. Eight patients (22%) underwent repeat NSS for locally recurrent disease. Two patients had a second local recurrence and underwent a third NSS. Six patients are alive without disease (75%) with an average follow-up of 4.5 years. Two patients have died, each with blastemal-predominant histology at repeat NSS. The surviving patients have normal renal function, although two patients require medical management of hypertension.

Conclusions

Our experience suggests that repeat NSS for local recurrence of FH bilateral Wilms tumor is feasible and affords acceptable oncologic outcome with preservation of renal function. However, more aggressive therapy may be required for patients whose recurrence has blastemal-predominant histology, given the poor outcome for these patients in our series.     

Induction Chemo-Radiotherapy for Squamous Cell Carcinoma of the Thoracic Esophagus: Long-Term Results of a Phase II Study

Background: This study was done to evaluate the results of the combined use of chemo- and radiotherapy before surgery in a group of patients with squamous cell esophageal carcinoma after a median follow-up period of more than 5 years.Methods: Between June 1987 and January 1995, 111 patients with squamous cell carcinoma of the thoracic esophagus were submitted to a preoperative course of radiotherapy (3000 cGy) and chemotherapy (cisplatin and 5-FU) before surgery in the First Division of General Surgery at the University of Verona.Results: The neoadjuvant treatment was completed in 90.9% of the cases (101/111). After an average of 29 days, 87 patients underwent surgery (operability rate: 78.3%) and, of these, 80 underwent esophagectomy (resectability rate: 91.9%). Histopathologic studies showed no residual disease in the specimen (T0) in 17 cases (21.2%), only microscopic clusters of neoplastic cells within the esophageal wall (Minimal Residual Disease, MRD) in 14 cases (17.5%) and in 5 cases the tumor did not extend beyond the submucosal layer (T1). The median overall survival time of the 111 patients who were eligible for the study protocol was 14 months, and the 2- and 5-year survival rates were 32.0% and 17.5%, respectively. Kaplan-Meier determination of survival showed a statistically significant difference between the good responders (T0, T1, and MRD) to the neoadjuvant treatment and the remaining cases. The 2- and 5-year survival rates were 50.3% and 34.9%, respectively, in the good responder group compared with 26.7% and 10.7%, respectively, in the other cases, with a median survival time of 24 months vs. 13 months, respectively.Conclusions: The neoadjuvant treatment showed promising results, especially in the group of patients that had a good response. The identification of these patients may be the key to selecting which patients should be submitted to preoperative radio- and chemotherapy.     

Abdominal apoplexy: A rare case of spontaneous rupture of the superior mesenteric artery in a hypertensive patient

INTRODUCTIONSpontaneous rupture of an intra-abdominal visceral artery is an exceptionally rare and potentially fatal cause of abdominal apoplexy.PRESENTATION OF CASEWe present a case of a 54-year-old hypertensive male who developed hypovolemic shock in our Emergency Department after presenting with abrupt onset of abdominal pain and diarrhea. Intra-operative findings revealed rupture of the superior mesenteric artery with massive hemoperitoneum. The bleeding vessel was ligated and the patient made a full recovery after 3 weeks in the Intensive Care Unit.DISCUSSIONHigh index of suspicion is necessary for early preoperative diagnosis and must be considered in any patient with a history of hypertension presenting with abrupt abdominal pain, signs of peritoneal irritation and unexplained hypovolemic shock. Immediate resuscitation and prompt surgical control of bleeding is paramount in patient prognosis.CONCLUSIONThe seemingly unpredictable nature of abdominal apoplexy must be noted, a precipitating cause in most cases is untraceable and early diagnosis relies solely on awareness of the condition.     

Neoadjuvante Therapie im oberen Gastrointestinaltrakt

While primary surgical resection with systematic lymphadenectomy remains the treatment of choice for locoregional Barrett’s cancer, neoadjuvant chemotherapy is an increasingly accepted treatment modality for patients with locally advanced tumors and patients with extensive lymphatic spread. In contrast to neoadjuvant radiochemotherapy preoperative chemotherapy alone does not seem to increase peri-operative complications and mortality. Responders to pre-operative treatment clearly have a survival advantage as compared to those who do not respond. The use of positron emission tomography to measure changes in glucose metabolism of the primary tumor can predict response early after initiation of neoadjuvant chemotherapy and thus help to select patients who will or will not benefit from this approach. The best treatment strategy for non-responders to neoadjuvant therapy remains to be defined.     

An enhanced recovery after surgery protocol in children who undergo nephrectomy for Wilms tumor safely shortens hospital stay

BackgroundPediatric unilateral renal tumors in the US are treated with upfront nephrectomy and surgical staging. We applied enhanced recovery after surgery (ERAS) principles in care of children after Wilms nephrectomy.MethodsWe reviewed records of pediatric unilateral nephrectomies for Wilms tumors, and analyzed tumor stage, surgical approach, length of operation, use of anesthesia adjuncts and catheters, diet advancement, hospital length of stay (LOS), and complications. Our ERAS protocol includes: parental education regarding discharge criteria and anticipated LOS, avoiding thoraco abdominal incisions, avoiding routine nasogastric tubes, clear liquids starting day of surgery, minimizing opiates, routine IV ketorolac use, and avoiding routine ICU stay. We examined the effects of our protocol on postoperative hospital LOS and complication rates.ResultsSixty six children (31 boys, mean age 3.8y, range 0–11.9) underwent unilateral total nephrectomy for Wilms tumor. Mean nephrectomy duration was 2.7 h. Post operatively, seven (11%) had temporary gastric tubes and 24 (36%) had epidural catheters. Ten (15%) recovered in the ICU. Patients were given regular diets mean of 1.9 days post op. Mean LOS was 3.7 days, with 56% of patients being discharged within 2–3 days. Presence of tumor thrombus, longer epidural catheter duration, delayed diet advancement, and total IV narcotic usage were associated with longer LOS. Routine use of IV ketorolac was associated with shorter LOS.ConclusionsUse of an ERAS protocol in children undergoing nephrectomy for Wilms tumor is safe, resulting in rapid return to regular diet and compared to the published literature, shorter postoperative LOS without an increase in complications or return to ED/OR.Level of evidenceLevel III     

Comparative analysis of three vs. four cycles of neoadjuvant gemcitabine and cisplatin for muscle invasive bladder cancer

PurposeBecause the optimal number of cycles of neoadjuvant gemcitabine and cisplatin chemotherapy (GC) is unclear, we aimed to compare disease response and survival outcomes of patients receiving either 3 or 4 cycles of neoadjuvant GC for muscle-invasive bladder cancer (MIBC).MethodsA total of 166 patients who were treated with neoadjuvant GC and radical cystectomy for clinical stage T2-4N0M0 were identified. Response and effectiveness of different cycle counts were assessed using downstaging (complete pathologic and partial pathologic response), cancer-specific survival (CSS), and overall survival (OS). Response and survival outcomes were examined with adjusted logistic regression and Cox regression models. Statistical significance was defined as P < 0.05.ResultsOf 166 patients who received neoadjuvant GC, 107 (64.5%) received 3 cycles and 59 (35.5%) received 4 cycles. Age, insurance, comorbidity, tumor histology (pure urothelial carcinoma, urothelial with divergent differentiation, variant histology), and tumor stage were similar between the 2 treatment groups. Rates of complete response or any downstaging were similar between groups (21.5% and 40.2% in the 3-cycle group and 20.3% and 44.1% in the 4-cycle group, respectively). While disease response was similar (OR 1.03, 95% CI 0.43–2.45), both cancer-specific survival (HR 1.69, 95% CI 0.87–3.26) and overall survival (HR:1.88, 95% CI:1.02–3.48) were more favorable among patients managed with 4 cycles of neoadjuvant chemotherapy compared to those who received 3 cycles in adjusted models.ConclusionsOur analysis demonstrated that survival outcomes tended to be better among patients who received 4 cycle of neoadjuvant GC compared to those treated with 3 cycles. Although potential benefits of omission of fourth cycle may include expedited time to surgery, reduced chemotherapy-associated toxicity, and lower treatment costs, continuation of treatment with a fourth cycle of neoadjuvant GC chemotherapy may benefit patients with muscle-invasive bladder cancer and further improve disease outcomes.