Search

Perivascular epithelioid cell tumors (PEComas) include different morphological entities originating from perivascular epithelioid cells. Their clinical behavior is not predictable, and there are no strict histologic criteria for malignancy, although larger tumors with infiltrative growth, hypercellularity, cellular atypia, atypical mitoses, and necrosis generally have a malignant course. Pediatric PEComas are rare, with less than 40 cases reported, mostly in children older than 5 years. We describe a case of malignant PEComa of the ligamentum teres in a 2-year-old girl, characterized by the occurrence of local relapse after primary treatment with chemotherapy and surgery and poor response to imatinib mesilate and temsirolimus used after further analyses confirmed p70S6K expression involved in the mTOR pathway. The girl was eventually treated with a debulking surgical procedure and is now alive with disease 6 years after diagnosis. Literature data of children affected by PEComas were also analyzed, trying to identify pathologic characteristics that could predict their course and therapeutic options. Histologically, they may be differentiated in 3 prognostic categories: (1) benign, lacking unfavorable morphological markers; (2) with uncertain malignant potential, carrying 1 unfavorable marker; and (3) malignant, with at least 2 unfavorable markers. In the literature, 9% of cases occurred as a second malignancy probably because of genomic instability related to treatment. Their different biology and the potential value of targeted therapies remain to be explored. The indolent evolution in our patient was similar to that reported in some other cases in the literature. In terms of treatment, the present case suggests a minor response to temsirolimus compared with the adult population., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: Open biopsy has been the mainstay for definitive diagnosis of neuroblastoma in pediatric patients. However, needle core biopsy may represent a faster, less invasive, and safer alternative to open biopsy in children. The purpose of this study was to compare safety and efficacy between needle core and open biopsy in the diagnosis of patients with intermediate- and high-risk neuroblastoma at our institution., Methods: We retrospectively reviewed the medical records of children with intermediate- and high-risk neuroblastoma who underwent open or needle core biopsies from 2002 to 2010. Data collected included patient demographics, tumor size, sample adequacy for diagnosis and risk stratification (histology and cytogenetics), length of hospital stay, time to initiate chemotherapy after biopsy, need for repeat biopsy, and both intraoperative and postoperative complications. Mann-Whitney U and Fisher's exact tests were used for statistical analysis., Results: During the study period, 7 patients underwent needle core primary biopsies (5 intermediate-risk primary tumors and 2 high-risk primary tumors), and 4 patients underwent needle core biopsy for metastatic tumors, whereas 21 patients had open biopsies (10, intermediate risk; 11, high risk). Median age at biopsy and median tumor size were similar in both groups. There was no significant difference in adequacy of biopsy, need for repeat biopsy, time to initiate chemotherapy, length of stay, or minor complications. The rate of major complications differed significantly between the 2 groups with 0% after needle core biopsy vs 48% after open biopsy (P = .027)., Conclusions: In children, needle core biopsy is comparable in efficacy with open biopsy in the diagnosis of intermediate- and high-risk neuroblastoma with significantly lower rates of major postoperative complications. These findings warrant a larger scale evaluation of diagnostic needle core biopsies in pediatric patients with solid tumor., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Hyperthermic intraperitoneal chemotherapy (HIPEC) is a well-established therapy for carcinomas, mesotheliomas, and some sarcomas. However, HIPEC has not been reported in melanomatosis or nephroblastomatosis. Here we present 2 unique cases of cytoreductive surgery and HIPEC. Cytoreductive surgery followed by HIPEC was performed in a 4-year old with melanomatosis and a 12-year old with nephroblastomatosis. A 4-year-old girl presented with leptomeningeal melanoma that metastasized from a congenital nevus. She had a ventricular peritoneal shunt placed as an infant. Melanomatosis involving the meninges and peritoneal surfaces was identified by positron emission tomographic scan imaging. Extensive plaques of melanoma were removed at the time of surgery, followed by HIPEC. She had no significant postoperative complications. Her abdomen remained free of disease 7 months after treatment. A 13-year-old girl presented with a Wilms' tumor at age 10 years. She then presented to us at the second local recurrence with multiple intraabdominal tumor implants (ie, nephroblastomatosis). We performed cytoreductive surgery followed by HIPEC. She had no postoperative complications and, at 1 year post-HIPEC, had no abdominal recurrence. We conclude that HIPEC can be performed safely for rare diseases such as melanomatosis and nephroblastomatosis. Although abdominal disease control can be realized, further systemic treatment options are necessary. A review of indications for HIPEC is included., (Published by Elsevier Inc.)

The local control of neuroblastoma is a very important treatment consideration. We describe a patient who received high-dose rate 60Co remote after loading system treatment for local control of recurrent neuroblastoma and discuss the efficacy of high-dose rate 60Co remote after loading system treatment., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background/purpose: Image-defined vessel encasement is a significant risk factor for surgical complications and incomplete resection for intermediate-risk tumors. We sought to examine the impact of vessel encasement on complications or resectability in intermediate-risk or high-risk patients after neoadjuvant chemotherapy., Methods: We retrospectively reviewed 207 consecutive patients with circumferential encasement of the renal vessels, celiac axis, and/or superior mesenteric artery (SMA) who underwent resection between 1991 and 2009. Specifically, we evaluated resection rates, complications, and outcome., Results: Median age at diagnosis was 3.0 years, and 79% of patients had stage 4 disease. Of known MYCN status, 23.4% had MYCN amplification. Vessel encasement included renal vessels, celiac axis, or SMA alone in 107, 7, and 4 patients, respectively. Both the renal vessels and celiac axis were encased in 5 patients, renal vessels and SMA in 7 patients, and celiac axis and SMA in 14 patients. Sixty-three patients had all 3 vessels encased. The gross total resection (GTR) rate was 94%. No operative or postoperative deaths occurred. The overall complication rate was 34.8% (n = 72). Overall 5-year survival (± SEM) was 67.4% (± 7.4%)., Conclusion: Encasement of major visceral arteries in patients with neuroblastoma who have received chemotherapy does not preclude gross total resection., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy with distinctive histologic and immunohistochemical features occurring in a young population with a male predominance. The tumor appears to arise as masses in the abdominal cavity without a clear visceral origin. Five patients with DSRCT were treated as usual with combined chemoradiation and surgery. In addition, in our center, patients underwent autologous bone marrow transplant (BMT), which is a novel approach to this disease., Methods: Charts of 5 patients (4 males, mean age of 11 years) treated between 2000 and 2007 were reviewed. The diagnosis of DSRCT was made on the basis of clinical examination, computed tomographic scan, and explorative laparotomy with biopsy, and biochemical markers were negative. All patients were treated with aggressive chemoradiation and surgery. Three patients also had autologous BMT., Results: Three patients (BMT recipients) responded to treatment. The responding patients had surgery with the intent of removing all disease. Two patients died of their cancer, neither of whom underwent BMT., Conclusion: The patients DSRCT are sensitive to an aggressive combination of chemotherapy, surgical debulking, and radiation therapy, followed by autologous BMT. It appears that this new multifaceted treatment offers good palliation, which may prolong survival and a possible cure.

Desmoplastic small round cell tumor (DSRCT) is a rare disease of children, adolescents, and young adults that begins and spreads on the peritoneal surfaces. Desmoplastic small round cell tumor usually presents with diffuse abdominal metastatic disease similar in gross appearance to carcinomatosis. To date, very aggressive treatment programs have yielded dismal outcomes. Here we present 2 cases of DSRCT that were treated with aggressive surgical excision followed by intraoperative continuous hyperthermic peritoneal perfusion using cisplatin. These are the first pediatric case reports of DSRCT being treated with continuous hyperthermic peritoneal perfusion, a procedure usually used in treatment of adult carcinomatosis.

Background/purpose: Intussusception as a presenting feature of Burkitt lymphoma may be associated with early stage disease, which is curable with less intensive therapy. We determined the incidence, presentation, stage, and outcome of children with Burkitt lymphoma presenting with intussusception., Methods: The medical records of patients with Burkitt lymphoma treated at our hospital from 1962 to 2005 were reviewed, and the patients presenting with intussusception were then further analyzed., Results: Of 189 patients with primary abdominal Burkitt lymphoma, 33 (17.5%) presented with intussusception. Their median age at diagnosis was 10 years (range, 3-19 years). Most patients presented with abdominal pain (88%) and/or nausea/vomiting (42%). Twenty-three (70%) of these 33 patients as compared with 10 of the other 156 patients with abdominal lymphoma could have complete resection of their tumor (P < .0001) and hence had low stage disease (stage II). Only 10 patients with intussusception had stage III (n = 7) or stage IV (n = 3) unresectable disease. Twenty-five of the patients remained alive in continuous complete remission for 3 months to 31 years (median, 14 years)., Conclusion: Pediatric patients with Burkitt lymphoma presenting with intussusception often have completely resectable disease and are older than general pediatric patients with intussusception.

Background/purpose: Gross total resection of the primary tumor in treatment of high-risk neuroblastoma remains controversial. Furthermore, there are few reports of the effect of primary tumor resection on local control as opposed to overall survival. The authors reviewed their institutional experience to assess the effect of primary tumor resection on local control and overall survival., Methods: A total of 141 patients were treated on protocol between November 1, 1979 and June 25, 2002 and are the subject of this report. Gross total resection was assessed by review of operative notes, postoperative computerized axial tomograms, and postoperative meta-iodobenzyl guanidine (MIBG)1 scans when available., Results: The median age was 3.3 years, and all patients were International Neuroblastoma Staging System (INSS) stage 4 with 79% having metastases to cortical bone. The primary site was the adrenal gland in 74%, the central abdominal compartment in 13%, the posterior mediastinum in 7%, and other sites in 6%. Gross total resection was accomplished in 103 (73%) but was more than 90% for the last 3 protocols. Five kidneys were lost overall. The probability of local progression was 50% in unresected patients compared with 10% in patients undergoing gross total resection (P <.01). Overall survival rate in resected patients was 50% compared with 11% in unresected patients (P <.01)., Conclusions: Our data indicate that local control and overall survival rate are correlated with gross total resection of the primary tumor in high-risk neuroblastoma. Gross total resection should be part of the management of stage 4 neuroblastoma in patients greater than 1 year of age.

Purpose: The authors examined whether recombinant human endostatin (rhEndostatin), an antiangiogenic agent, is effective against a human neuroblastoma cell line (designated TNB9) using a human neuroblastoma xenograft model and investigated whether continuous infusion is more effective than intermittent administration., Methods: In the first experiment, when tumors on the back of nude mice reached a weight of 90 to 95 mg, rhEndostatin, 10 mg/kg/d mouse weight, was administered subcutaneously to the mice (n = 5) every day for 10 consecutive days. In the second experiment, the same daily dose of rhEndostatin was administered continuously to the TNB9-bearing mice (n = 6) via subcutaneous infusion pumps for 3 consecutive days with total dose being 30% of that in the first experiment. Nestin and factor VIII expression levels were studied immunohistochemically to elucidate whether histologic evidence of the effects of rhEndostatin was present on day 4 in the second experiment., Results: In the first experiment, relative tumor weight in treated mice (n = 5) was significantly less than that in controls (n = 12) on day 2 only after treatment initiation (P <.05). The maximum inhibition rate (MIR) of TNB9 xenograft growth by rhEndostatin was 46.4%, indicating lack of efficacy. In the second experiment, the effects of rhEndostatin were much more marked than those in the first experiment, with an MIR of 60.7%. The mean relative tumor weight in the treated group (n = 6) in the second experiment was significantly less than that in controls (n = 10) on days 2, 4, and 6 (P <.01) as well as on days 8 and 10 (P <.05). Nestin staining in the endothelium of control tumors (n = 2) was marked, whereas it showed a loss of fibrillar structure in rhEndostatin-treated tumors (n = 2). The number of vessels immunostained with antifactor VIII antibody was markedly reduced in tumors (n = 2) from rhEndostatin-treated mice compared with that in tumors from control animals (n = 2)., Conclusions: Continuous administration of rhEndostatin resulted in more significant tumor regression than intermittent administration of the agent in the same model. This indicates that rhEndostatin, if administered in continuous fashion, could become an effective agent for treating patients with neuroblastoma in the future.

Background/purpose: This randomized study examined survival (S) and event-free survival (EFS) rates using high-or standard-dose cisplatin-based combination chemotherapy and surgical resection for this subset of germ cell tumors., Methods: Twenty-six of 317 patients enrolled on the POG 9049/COG 8882 intergroup study for malignant germ cell tumors had abdomen or retroperitoneum as the primary site. Twenty-five of 26 were eligible for inclusion (n = 25). Patients had biopsy or resection at diagnosis and randomization to chemotherapy including etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB). In patients with initial biopsy, delayed resection was planned., Results: Median age was 26 months. There were 14 girls and 11 boys. There were 3 stage I to II, 5 stage III, and 17 stage IV patients. Surgical management included primary resection in 5, resection after chemotherapy in 13, and biopsy or partial resection in 7 patients. Overall 6-year EFS rate was 82.8% +/- 10.9%, and 6-year survival rate was 87.6% +/- 9.3%. By group, 6-year survival rate was 90.0% +/- 11.6% for PEB and 85.7 +/- 14.5% for HDPEB. Deaths include one from sepsis, one from malignant tumor progression, and one from bulky disease caused by benign components despite response of the malignant elements to chemotherapy., Conclusions: Malignant germ cell tumors arising in the abdomen and retroperitoneum have an excellent prognosis despite advanced stage in most children. Aggressive resection need not be undertaken at diagnosis, but a concerted attempt at complete surgical removal after chemotherapy is important to distinguish viable tumor from necrotic tumor or benign elements that will not benefit from further chemotherapy., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Background/purpose: The importance of primary tumor resection in stage IV neuroblastoma is controversial. The authors analyzed prospectively the role of surgery in a multicentric series of stage IV neuroblastoma patients., Methods: Patients were studied according to the International Neuroblastoma Staging System (INSS) recommendations. Age, sex, location of the tumor, type of metastases, time of resection (initial or delayed), extension of resection, surgical complications, pathology, N-myc and Shimada classification results, relapses, and outcome were studied. After diagnosis, children received induction chemotherapy followed by delayed surgery and autologous stem cell transplantation or maintenance chemotherapy. Resection was classified as complete (C), greater than 90% (P1), greater than 50% (P2), less than 50% (P3), and biopsy (B)., Results: Ninety-eight stage IV children were admitted in the study from June 1992 to July 1999. Seventy-six were older than one year, and in 78 the primary tumor was abdominal. Bone was the most common metastatic site followed by bone marrow. Initial biopsy was performed in 74 patients, and resection in 6, with one complication in each group. N-myc was amplified in 20 of 80 tumors, and Shimada was unfavorable in 45 of 67. Delayed surgery was performed in 70 cases, achieving gross total resection in 55 (79%); there were minor complications in 10%. Mean survival rate time was 50 months. Event-free survival rate (EFS) at 5 years for the entire series is 0.32, but 0.0 for children having biopsy only, 0.25 for less than 50% resection, 0.31 for 50% to 90% resection, 0.44 for greater than 90% resection, and 0.33 for complete resection. Differences were statistically significant only when compared with the biopsied group. EFS rate for infants was 0.56, but, again, there was no difference in relation to the type of resection. There were 46 relapses, 12 of them local, 7 of 20 N-myc-amplified tumors, and 4 of 60 not amplified (P <.005)., Conclusions: Biopsies of stage IV neuroblastoma allow safe assessment of N-myc and other biological factors on tumor tissue. Delayed surgery after chemotherapy is performed with a low rate of complications, achieving a good local control of disease. N-myc-amplified tumors have higher local relapse rates than nonamplified and therefore would need more intensive local treatment. The final outcome in these patients is determined more by metastatic relapses than by the degree of resection., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Intestinal obstruction in a 2-year-old girl with a histologically proven diagnosis of retroperitoneal yolk sac carcinoma developed after the second course of anticancer chemotherapy. Nonoperative treatment was not effective. Because the patient had fallen into a state of chemotherapy-induced myelosuppression, surgery was ruled out. Thus, hyperbaric oxygen therapy was the next treatment of choice. It was performed twice under hyperbaric oxygen conditions at 2.8 atmospheric pressure for 111 minutes. After the procedure, her general status recovered well. The air-fluid level disappeared on the radiograph, and no adverse effects were observed. Later, a surgical removal of the primary tumor was performed successfully, but an intestinal resection was not required. This is the first instance in which we performed hyperbaric oxygen therapy on a child in the management of an intestinal obstruction. Based on the successful outcome in this case, hyperbaric oxygen therapy is suggested to be a useful adjunct to nonoperative therapy for intestinal obstruction when a patient's overall state does not allow operative intervention.

Background: Histopathologic findings of advanced neuroblastoma after intensive induction chemotherapy have not been studied well., Methods: In the present study, all of the surgical specimens from 19 patients who had advanced abdominal neuroblastoma and were pretreated intensively with the protocol of the Study Group of Japan were reviewed. The authors found that dissection of the contralateral lymph nodes is mandatory in advanced neuroblastoma when the goal is the complete dissection of the abdominal disease. Effects of chemotherapy were graded histologically according to the ratio of viable residual neuroblastoma tissue to total areas of the tumor, including neuroblastoma, ganglioneuroblastoma, ganglioneuroma, hemorrhage, necrosis and fibrosis, in five ranks from ( ) to (-)., Conclusions: The newly introduced, highly cytotoxic regimen of the Japanese protocol, designated "A3," appears to be more effective histologically than the conventional regimen, designated "A1" or "new A1." Effects designated ( ) or (++) were prerequisites for survival in stage IV disease, but some stage III patients with the (+) effect survived.

Obstructive jaundice is an unusual initial manifestation of non-Hodgkin's lymphoma in children. Two pediatric patients with primary intraabdominal non-Hodgkin's lymphoma causing obstructive jaundice are presented. Although percutaneous transhepatic cholangial drainage (PTCD) is frequently used to treat obstructive jaundice, there was no definite rule for management of PTCD during chemotherapy, which may rapidly resolve the obstruction. Biliary peritonitis occurred in both patients soon after removal of the PTCD tube after chemotherapy. It was speculated that secure formation of the fistula for PTCD was impaired because of chemotherapy for a considerably long period. The PTCD tube that was already placed for obstructive jaundice in a non-Hodgkin's lymphoma patient should be maintained during chemotherapy with great care for displacement of the tube. PTCD can result in complications, and is probably unnecessary when lymphoma has been diagnosed before PTCD.

The role of surgery in intraabdominal Burkitt's lymphoma is a controversial subject, and different views are expressed. In the authors' institution, 41 children (30 boys and 11 girls) have been treated who had intraabdominal Burkitt's lymphoma. A study was undertaken to assess the limitations of surgery in the treatment of intraabdominal Burkitt's lymphoma. An uniform policy was made to resect the tumor if possible or to debulk tumor irrespective of the spread of disease, and if this was not possible to perform only a biopsy. Thirty-nine children had laparotomy and various operative procedures, and in 2 children no laparotomy was performed, but tissue was obtained for histopathologic examination from cervical lymph nodes and jaw. Twelve children underwent complete resection of the intraabdominal tumor. There were no deaths in this group, and 10 of 12 children are long-term survivors. In 15 patients, debulking of the tumor was carried out. In 4, the tumor had perforated the bowel and caused widespread intraperitoneal deposits and sepsis. There were 3 deaths during the immediate postoperative period, 2 caused by peritoneal sepsis and 1 caused by surgical trauma in advanced disease. Three of 15 patients have survived for more than 2 years. Lastly, in 14 children only a biopsy was performed. Notable features in this group were that 6 patients had extraabdominal disease and another 6 presented initially with features of intestinal obstruction. In the latter group, intestinal bypass procedures were performed but no debulking was attempted. Five of 14 patients died after surgery, whereas 3 were long-term survivors. Patients were administered a chemotherapy regimen consisting of cyclophosphamide, vincristine, Adriamycin, and prednisolone.(ABSTRACT TRUNCATED AT 250 WORDS)

The first successful excision of a neuroblastoma took place in 1916. For many years there was no other form of treatment, and the outlook remained dismal. The use of radiotherapy (1928) and subsequently combination chemotherapy (1965) had a modest impact. More accurate staging has allowed a more coherent approach to diagnosis and treatment. Surgery has not been standardized to the same degree. A review of the author's experience with 129 patients over a 10-year period confirms that surgery is the mainstay of treatment for localized nonadvanced disease (stages I and II). Fourteen of 15 such patients are alive and disease-free. Combination chemotherapy may shrink advanced tumors (stages III and IV) and ablate metastases. Subsequent complete surgical excision does not improve survival. Approximately two thirds of those with stage III disease and one third of those with stage IV disease survive regardless of whether excision is complete. The present results do not support the value of complete surgical excision.

To determine the appropriate role of surgical intervention in non-Hodgkin's lymphoma primary to the abdomen, we analyzed the effect of multiple prognostic determinants on event-free survival for patients entered into the CCG-551 study. Eighty-four patients were identified with abdominal lymphoma and of these adequate data for analysis was available on 68 (81%). Variables of interest included: extent of disease at diagnosis, completeness of resection, use of bowel resection, radiation to the primary site, and sex/age/race. The median age on study was 8 years; 79% of patients were white and 85% were male. Thirty-three patients (49%) presented with localized disease. Laparotomy was performed in 67 children (99%) with complete gross resection in 28 (42%). Of the 10 reported surgical complications, 8 occurred in those with extensive disease who were incompletely resected. Radiation to the primary site was given in 60% of patients with median dose of 2,000 cGy. Analysis was performed both for the overall group and for the subgroup receiving optimal chemotherapy for histopathology. Variables with significant predictive effect on event-free survival in univariate analysis included extent of disease (P less than or equal to .001), complete resection (P less than or equal to .002), and use of bowel resection (P less than or equal to .004). However, in multivariate analysis, only extent of disease was an independent predictor of outcome. The data support a role for complete operative excision of localized lymphomas especially when accomplished with bowel resection. Aggressive attempts at debulking extensive retroperitoneal or mesenteric lymphomas are contraindicated.

The abdomen is the most frequent site of involvement in nonendemic Burkitt's lymphoma (small noncleaved cell). Some authors have proposed a role for extensive surgical resection or "second look" laparotomy in these patients. We retrospectively reviewed our series of 53 patients with Burkitt's lymphoma (1977 to 1990) to assess the role of surgery in their treatment. Patients were 2.5 to 21 years of age (median, 9.5 years) and 44 were males. The primary site of disease was the abdomen (38), head and neck (12), axilla (1), and bone marrow (2). Twenty-four of the 38 patients with abdominal primaries underwent laparotomy. Twelve of these patients presented with acute abdominal symptoms (right lower quadrant pain or intestinal obstruction) and at exploration underwent resection of the primary tumor. Ten of these 12 patients achieved grossly complete excision of tumor (9 had disease limited to the ileocecal area and adjacent mesentery and one had exophytic tumor adherent to the liver, which was excised). Of note, only 1 of these 12 patients had metastatic disease outside of the abdomen. The remaining 12 patients who underwent laparotomy had an incisional biopsy performed. Of the 14 patients who did not have a laparotomy, the diagnosis was made by bone marrow biopsy (6), and/or cytology of pleural fluid or ascites (6), lymph node biopsy (1), testicular biopsy (1), tibial biopsy (1), and percutaneous biopsy (1). Murphy staging for these 38 patients was: stage II (10), stage III (19), stage IV (5), and B cell acute lymphoblastic leukemia (ALL) (4). All patients received cyclophosphamide-containing combination chemotherapy regimens and stage III/IV/B cell ALL patients received central nervous system (CNS) prophylaxis.(ABSTRACT TRUNCATED AT 250 WORDS)