Significance of residual abdominal masses in children with abdominal Burkitt's lymphoma

Purpose: To evaluate the natural history of children with abdominal Burkitt's lymphoma who had complete clinical remission and residual abdominal mass after treatment. Material and methods: The charts and imaging findings of all children with abdominal Burkitt's lymphoma treated and followed at our medical center between 1988 and 1999 were reviewed for the presence, management, clinical course, and prognosis of residual mass. Results: Only children who achieved complete clinical remission were included. The study group consisted of 33 children (20 boys and 13 girls) aged 2.6–17.6 years (mean 7.2 years). Of these, seven (20.6 %) were found to have a residual abdominal mass. Two underwent second-look operation with no evidence of viable tumor on histology. The remaining five were followed by imaging studies for 2.2–9.1 years (mean 6.1 years); none relapsed. Conclusion: Residual mass is not uncommon in children with abdominal Burkitt's lymphoma. The presence of residual mass in a child with complete clinical remission does not alter the long-term prognosis. Therefore, in children with Burkitt's lymphoma and residual mass with no other signs of disease activity, expectant watching may be appropriate. Received: 10 December 2000 Accepted: 25 June 2001     

Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience.

BACKGROUND: Intraabdominal desmoplastic small round cell tumors (IDSRCT) are rare in children and predominantly affect male adolescents and young adults. We present our experience in the management of five children with diffuse IDSRCT, managed with aggressive chemotherapy, surgery, radiotherapy and peripheral blood stem cell transplantation. MATERIAL AND METHODS: During the last decade five patients, four males and one female (mean age 9.6 years), with diffuse IDSRCT were managed in our department. The main symptoms were abdominal distention, vague abdominal pain, and vomiting. Three patients with inoperable tumor on admission were submitted initially to open biopsy followed by aggressive chemotherapy. Regression of the tumor was followed by a second laparotomy and radical excision of any macroscopically distinguishable masses, followed by chemotherapy. In the remaining two patients a debulking procedure was done initially, followed by chemotherapy. The accurate diagnosis of the disease was established by immunohistochemistry, additionally confirmed in the last two patients by molecular analysis. RESULTS: Three patients who had radical excision of the tumor and adjuvant chemotherapy had recurrence after two to six months. In the remaining two patients, recurrence was evident after two and eighteen months, respectively, following debulking. In addition, one patient with recurrence received radiotherapy and two others underwent peripheral blood stem cell transplantation. All but one patient died within three years from diagnosis. The last patient, who was submitted to a debulking procedure, is still alive eight months after the operation. CONCLUSIONS: Intrabdominal desmoplastic small round cell tumor is a highly aggressive malignancy with a very poor prognosis. Multiagent chemotherapy usually leads initially to a temporary regression of the tumor, but recurrence is the rule. Radical surgical excision, radiotherapy and peripheral blood stem cell transplantation does not seem to improve prognosis significantly. Despite all therapeutic modalities the outcome is dismal and surgical efforts can be considered only as palliative.     

儿童伯基特和伯基特样淋巴瘤40例的临床研究

目的 认识儿童伯基特淋巴瘤(BL)和伯基特样淋巴瘤(BLL)的病理及临床特点,总结大剂量、短疗程化疗方案的疗效,探讨治疗相关的合并症及预后影响因素.方法 2003年2月-2006年4月住院治疗的淋巴瘤患儿40例,全部行活检病理形态及免疫组化检查,并按WHO新的病理分型标准进行分型,参照儿童非霍奇金淋巴瘤(NHL)的St.Jude分期标准进行分期.采用大剂量、短疗程的改良LMB89方案进行治疗.根据不同分期、不同危险因素及对治疗反应的评估结果,将患儿分成A、B、c三个治疗组.结果 临床特点:发病年龄2～14岁,中位发病年龄6.9岁;男33例,女7例,男:女=4.7:1;腹腔肿物及急腹症为最常见表现.病理示BL 30例、BLL 10例;7例(17.5%)在瘤组织中检测到EBER-1阳性细胞,19例血清中EB病毒抗体阳性.确诊时I～Ⅱ期患儿9例,Ⅲ～Ⅵ期患儿31例;伴中枢侵犯者3例,骨髓侵犯者2例.疗程2～8个月.随访率100%,中位追踪时间22.6个月.至2006年12月底,实际存活35例,1年生存率88.7%,预计3年无进展生存率81.8%.化疗中Ⅲ～Ⅳ度骨髓抑制的发生率在B组和C组分别为97.5%和100%,3例出现重症黏膜炎.5例死亡,其中2例为副作用相关死亡.单因素分析显示,临床分期为Ⅳ期、治疗3个月时评估有残留病灶、病程小于15 d与预后不良相关(P＜0.05).结论 采用大剂量、短疗程方案治疗BL和BLL疗效好,预计3年无进展生存率81.8%.病史小于15 d、诊断时为Ⅳ期、治疗3个月未达完全缓解者与预后不良相关.     

Surgical aspects of intussusception due to lymphoma in children]

BACKGROUND: Intussusception due to lymphoma is a challenging condition for pediatric surgeons. The aim of this study is to report seven cases of this entity and to discuss its management. CASE REPORT: Six boys and one girl, 3-15-years-old, were admitted for intussusception secondary to a lymphoma. All patients underwent laparotomy: biopsy of massive abdominal tumor 6 and 8 weeks following resection of an intussusception (two cases), ileal resection of non-reductible intussusception (one case), right hemicolectomy for tumor of the appendix (one case), tumorectomy of localized ileal tumor (two cases), enlarged mesenteric lymph node biopsy associated with simple reduction of intussusception (one case). All children were successfully treated with protocol chemotherapy with a 15-month to 13-year follow-up. No relapse was observed. CONCLUSION: Surgeons should be aware of operative sights of ileal lymphomas. Diagnosis of lymphoma may be difficult after manual reduction of intussusception. A sample of any abnormality (mesenteric lymph node, peritoneal fluid) should be taken. Intestinal resection allows to reduce the intensity of chemotherapy but must be as limited as possible: ileal resection in cases of complicated intussusception, tumorectomy "in sano" in cases of ileal parietal isolated tumor. Reduction of intussusception alone (with no resection of ileal tumor) seems to be effective if diagnosis of lymphoma is possible from peripheral samples (peritoneal fluid, pleural effusion, mesenteric lymph node, bone marrow biopsy...).     

An abdominal Burkitt's lymphoma in acute phase. Case report

Burkitt's lymphoma is a highly malignant, aggressive and rapidly growing B-cell neoplasm, which has low long-term survival rates. Abdomen is the most frequent onset site of nonendemic Burkitt's lymphoma. The rapidity of volumetric doubling of this neoplasm frequently justifies an abdominal acute presentation, that may mime other less rare diseases. Symptoms are often misleading and make diagnosis difficult. The aim of this work is to report a case of a 13-year-old boy affected by terminal ileum Burkitt's lymphoma with hepatic metastasis, which initially was mistaken for acute appendicitis complicated by hepatic abscesses and, following a second surgical operation, for terminal-ileum inflammatory bowel disease. The rapidity of growth of this neoplasm justifies the finding, during the second surgical operation, of a mass that was not clinically manifested during first operation, carried out only a week ago. Clinical signs and instrumental investigations were not diagnostic, as well as the literature reports. The role of surgery remains controversial, and is usually limited to collection of specimens for histological diagnosis or to management of acute complications, as in our case report. Mostly treatment protocols are based on chemotherapy, because of the high sensibility of this neoplasm.     

Association of Helicobacter pylori and childhood lymphoma

We aimed to estimate the frequency of association between non-Hodgkin lymphoma (NHL) with abdominal, gastric, or intestinal involvement and Helicobacter pylori in childhood. Between February 2003 and June 2006, we evaluated 15 children with newly diagnosed NHL who were diagnosed and treated at the Pediatric Oncology Department of Hacettepe University. Patients who were given chemotherapy previously or who received H. pylori eradication therapy were excluded from the study. Routine procedures were done for staging. Pathologic diagnosis was made by examining the biopsy samples. The presence of H. pylori was confirmed by H. pylori IgG serology with urea breath test (UBT) in cooperated children. Endoscopic examination was also planned for patients with positive test results. Twelve male and 3 female patients, with a median age of 7 (range: 3 to 16), were evaluated. They had extensive abdominal, gastric, and/or intestinal involvement. Six had stage IV characteristics, whereas another 9 patients had stage III disease. Ten had high-grade B-cell lymphoma. Only 3 patients had H. pylori IgG and UBT positivity (20%). First patient had T-cell lymphoma and stage IV disease with involvement in stomach, mediastinum, peripheral lymph nodes, and bone marrow. The second one had anaplastic large cell lymphoma exclusively in abdominal lymph nodes. Last patient had Burkitt lymphoma and stage IV disease, with primary tumor localization in abdominal lymph nodes, liver, and kidneys. The H. pylori IgG and UBT were both positive in 3 patients on admission. We did not find any positive test results in the other 12 patients with intestinal, stomach, or abdominal disease. Preliminary results of our study suggest that H. pylori may not be the responsible agent for NHL involved the abdomen in childhood.     

Early diagnosis of intestinal non-Hodgkin lymphoma by endoscopy

The early diagnosis of intestinal non-Hodgkin lymphoma, stages I and II, lymphoblastic of Burkitt's type, was made by colonoscopy combined with selective radiologic visualization of the tumor in two children, ages 4 and 5. This technique is recommended in patients with abdominal pain, iron-deficiency anemia, intestinal blood loss, and an elevated sedimentation rate, when conventional radiologic and ultrasound techniques fail.     

Burkitt's lymphoma presenting as obstructive jaundice

A 12-year-old boy presented with a 5-month history of yellowness of the eyes, progressive painless abdominal swelling and weight loss. Physical examination revealed a grossly wasted child with marked jaundice and non-tender hepatomegaly. Liver function tests and abdominal ultrasound suggested obstructive liver disease. Tissue biopsy at laparotomy showed histological findings consistent with Burkitt's lymphoma. He was commenced on cytotoxic chemotherapy and, after two courses, the jaundice disappeared and he remained well. Although ante-mortem presentation of Burkitt's lymphoma as hepatic disease is rare, this condition should be included in the differential diagnosis of a child with obstructive jaundice.     

The efficacy of rituximab in high-grade pediatric B-cell lymphoma/leukemia: a review of available evidence

PURPOSE OF REVIEW: This review evaluates whether rituximab has efficacy in high-grade pediatric B-cell lymphoma/leukemia. Current pediatric protocols for CD20+ B-cell lymphoma/leukemia significantly improve survival, but with major morbidity. To assess whether rituximab has efficacy in very high-grade pediatric disease, all published data on rituximab therapy for Burkitt's lymphoma/B acute lymphoblastic leukaemia (B-ALL) and pediatric patients with relapsed/refractory large B-cell lymphoma were reviewed. RECENT FINDINGS: Three trials in adult Burkitt's/B-ALL showed a significant survival advantage when rituximab was added to standard chemotherapy. Minimal pediatric data have been published, but 19 children with mature B-cell lymphoma/B-ALL received rituximab, alone or in combination with chemotherapy, as salvage therapy, after failure of intensive chemotherapy. Fifteen of 19 (79%) responded, 12 (63%) remained alive in continuous complete remission at 5+ to 48+ months of follow-up. Two patients were alive in partial remission. Five patients died, four of progressive disease. Only one patient had no response to rituximab. SUMMARY: Rituximab has demonstrated efficacy in Burkitt's disease in adults. Although positive reporting bias is suspected, it appears that rituximab, even as monotherapy, has efficacy in heavily pretreated pediatric patients with high-grade B-lymphoma/B-ALL. Rituximab use can be justified in a prospective controlled chemotherapy dose-reduction study.     

Successful therapy of Clostridium septicum sepsis in a child with Burkitt's lymphoma

The association between Clostridium septicum sepsis in patients with malignant disease has been frequently documented. A presentation with fever, neutropenia, vomiting, and an acute abdomen is characteristic of this anaerobic infection which has been uniformly fatal in children with acute leukemia. We report the unusual course and the successful treatment of an adolescent with an abdominal Burkitt's lymphoma with leukemic transformation and clostridium septicum sepsis and cellulitis.     

Abdominal manifestations of non-Hodgkin's lymphomas

25 children (18 boys and 7 girls) were treated for non-Hodgkin's lymphomas (NHL) during 1982-1990. The age at diagnosis was 2-15 years. Five of the 25 children died. Of the 25 children 6 patients had the diagnosis of intraabdominal NHL. The presenting abdominal symptoms for these 6 patients were abdominal pain in 4 cases, nausea 3, abdominal distention 2, icterus 1 and diarrhoea 1. Four of the 6 patients with abdominal NHL underwent laparotomy but the diagnosis was unknown before surgery. The indication for explorative laparotomy was in all cases a tumor of unknown etiology combined with ascites in 2 cases and icterus in 1 case. At surgery there was a tumor localized around the porta hepatitis in 1 case and in the right ovarium in 1 case. Furthermore, spread infiltrative growth of tumor was found in 3 cases, with infiltration of the peritoneum in 2 of these cases. A specimen for peroperative histological examination was taken and gave the conclusive diagnosis of NHL in 4 of 6 cases. Staging laparotomy, palliative tumor resection and radical surgery are preferably avoided. For proper treatment an adequate biopsy is important. The management of children with NHL is a multidisciplinary approach.     

小儿血液系统恶性疾病合并外科急腹症的诊治分析

目的探讨小儿血液系统恶性疾病合并外科急腹症的诊断与治疗,以提高对小儿恶性血液病并发急腹症的认识,改善该类患者的预后。方法对13例恶性血液病并发急腹症患者进行回顾性分析。13例中,急性淋巴细胞白血病6例,MDS转化的急性髓细胞白血病1例,慢性粒细胞白血病1例,红白血病1例,再生障碍性贫血2例,淋巴瘤2例。7例行手术治疗,6例予保守治疗。结果7例手术患儿中,4例急腹症获治愈,待内科疾病缓解后出院;1例急腹症治愈,后死于内科疾病;2例术后早期自动出院,其中1例死亡。6例保守治疗患儿中1例死亡,5例临床症状缓解。结论各种恶性血液病在疾病的任何阶段均可并发多种急腹症,手术治疗是挽救患儿生命的重要手段之一。应根据原发病所处的阶段以及急腹症的类型和严重程度,给予及时诊断和恰当治疗,以利于改善该类患者的预后。     

Primary follicular lymphoma of the testis: excellent outcome following surgical resection without adjuvant chemotherapy

The authors report a case of follicular lymphoma of the testis in a 3-year-old boy. Follicular lymphoma of childhood is rare, accounting for less than 5% of all non-Hodgkin lymphomas in the pediatric population. Children with follicular NHL have typically been described as presenting with localized disease, with a preponderance of male patients. The authors conducted a comprehensive review of the literature and found that this patient is the only child reported to have localized follicular lymphoma of the testis successfully treated without systemic chemotherapy. In all previously reported cases, a favorable outcome was seen following surgical resection and chemotherapy. This patient has remained disease-free for more than 2 years after surgical resection alone. The successful outcome in this case following surgical resection, without postoperative adjuvant chemotherapy, suggests that localized follicular non-Hodgkin lymphoma in children may not be associated with occult systemic disease and that subsequent chemotherapy may not be necessary for cure.     

Burkitt's Lymphoma in 63 Turkish Children Diagnosed Over a 10 Year Period

Sixty-three Turkish children with Burkitt's lymphoma (BL) diagnosed over a 10-year period in a single institution were retrospectively analyzed. Burkitt's lymphoma included 41.7% of non-Hodgkin's lymphomas and 17.2% of all childhood malignant solid tumors diagnosed in our department in this duration. The patients studied with BL were aged between 3 and 14 years (mean 5.9 years), with a male to female ratio of 2:1. While the age distribution in our patients was similar to that in African BL (endemic), the predominance of abdominal involvement and the frequency of bone marrow infiltration and pleural effusion were reminiscent of American BL (sporadic). The incidence of jaw involvement (15.9%) in our group was higher than in American BL, however, and was not as high as in African BL. Most of the patients were of a lower socioeconomic status. Significant growth retardation was found in the children with BL compared with 40 age-matched children without malignancy, nor chronic or endocrinologic disorders, who were of a similar socioeconomic status. A serological study for Epstein-Barr virus (EBV) was performed in 18 children, and the IgG-type antibody to the viral capsid antigen of EBV was found to be positive in all of them. As a result, BL seems to include a considerable proportion of all childhood malignant solid tumors in Turkey. The epidemiological and clinical presentation and course indicate that BL appears in Turkish children in a form that is between the African and American types of the disease. Further molecular and chromosomal studies in Turkish children with BL are needed.     

Primary Burkitt's lymphoma of the colon--an uncommon cause of acute constipation and abdominal pain

Constipation is a common problem in children and adolescents, accounting for over 25% of all visits to pediatric gastroenterologists. Approximately 95% of childhood constipation is functional in nature. Primary gastrointestinal malignancies are rare in childhood and adolescence. When present in the gastrointestinal tract, Burkitt's lymphoma is typically located in the ileocecal region. We present a case of primary Burkitt's lymphoma of the transverse colon in an adolescent presenting with acute exacerbation of constipation, abdominal pain, and later, rectal bleeding and progressive weight loss.     

儿童腹腔的非何杰金淋巴瘤

为了解儿童原发于腹腔的非何杰金淋巴瘤早期诊断及正确治疗，分析５例不同阶段的儿童原发于腹腔的非何杰金淋巴瘤。１例为急腹症，早期行剖腹探查并作肿块完全切除，加化疗，预后较好。２例因淋巴结活检确诊，未作手术，直接化疗，预后次之。另２例因诊断不明，延误手术，术中作肿块部分切除，且放弃化疗，预后最差。儿童原发于腹腔的非何杰金淋巴瘤治疗的关键是早期取得病理诊断。治疗原则以肿块完全切除＋化疗最好。     

儿童成熟B细胞淋巴瘤并发急性肿瘤溶解综合征18例临床分析

目的 探讨儿童成熟B细胞淋巴瘤并发急性肿瘤溶解综合征(ATLS)的临床特点.方法 总结患儿临床资料,评估化疗前肿瘤负荷,收集化疗后第8天、3个月及停药前各项评估结果并追踪预后.统计ATLS在B细胞淋巴瘤的发生情况及其与高肿瘤负荷、肾脏侵犯的关系.全部患儿给予大剂量水化碱化、利尿、别嘌呤醇等常规治疗,予血液透析等治疗.结果 (1)103例成熟B细胞淋巴瘤患儿中诊断ATLS者18例(17.5%),病理类型均为伯基特型,18例患儿均为Ⅲ～Ⅳ期且并发巨大瘤块,骨髓幼稚细胞＞25%者7例(38.9%),乳酸脱氢酶≥1000 U/L者11例(61.1%),肾脏侵犯者11例(61.1%);(2)18例(100%)患儿有恶心、呕吐等胃肠道反应;7例(38.9%)发生抽搐(5例为低钙惊厥,2例头颅磁共振提示为双侧枕叶脑白质病变);6例(33.3%)存在严重的少尿或无尿;消化道出血1例(5.6%);(3)全部患儿均有血尿素氮和(或)肌酐升高,高尿酸、高磷及高钾血症、低钙血症;(4)经常规治疗缓解者9例(50.0%),经血液透析和(或)尿酸氧化酶治疗缓解者8例(44.5%);本组接受规律化疗者13例(72.2%),10例至今无事件存活(55.6%),3例停药后复发导致死亡(16.7%);治疗初期放弃治疗或死亡者共5例(27.8%).结论 (1)儿童成熟B细胞淋巴瘤中伯基特型易发生ATLS;(2)高肿瘤负荷、肾脏侵犯是ATLS发生的高危因素;(3)常规应用水化碱化、别嘌呤醇、利尿是治疗ATLS的有效措施,重症者应用血液透析等治疗是有效的缓解手段.

Abstract：

Objective To investigate risk factors associated with acute tumor lysis syndrome (ATIS) in children with B-cell lymphoma and to explore feasible means for the prophylaxis and treatment.Method Data from 18 children with ATLS in B-cell lymphoma were collected to assess their tumor burden at diagnosis and before chemotherapy. Evaluation was performed at the 8th day, 3 month, and the end of chemotherapy and follow up. The incidence of ATLS in B-cell lymphoma, and the relationship between the incidence of ATLS and whether the kidney was involved and large tumor burden were analyzed respectively.All patients received hydration, alkalinization and received allopurinol routinely. Urate oxidase and hemodialysis treatment were administered in some cases. Result Of the 103 children with B-cell lymphoma, 18 were diagnosed as having ATLS (17.5%). All the 18 cases with ATLS were histopathologically confirmed as having Burkitt's lymphoma. All the patients were at stage Ⅲ or Ⅳ and all had large tumor sizes, and 7 were found to have blasts in the bone marrow ＞ 25% ( 38. 9% ). Lactate dehydrogenase (LDH) levels ≥1000 U/L were found in 11 (61.1%) cases. All patients had developed metabolic abnormalities, including hyperuricemia, hyperphosphatemia, hypocalcemia , and uremia. In terms of clinical features and prognosis, all cases had nausea, vomiting, anorexia, oliguria, and anuria at different levels. One had gastrointestinal bleeding, 7 patients experienced seizures. The etiology in five was hypocalcemia and two had reversible posterior encephalopathy syndrome and all responded well to treatment.Nine cases of ATLS responded to supportive care, 4 required hemodialysis, and the other 4 responded to urate oxidase. Ten cases survived and 8 died. The major cause of death was severe complications and treatment was given up in 5 cases and recurrence occurred in 3 cases. Conclusion ATLS was commonly seen in Burkitt's subtype of B-cell lymphoma. Higher LDH and large tumor sizes and kidney involvement were important risk factors for the development of ATLS in children with B-cell lymphoma. Treatments with hydration, alkalinization and allopurinol were safe and effective. Urate oxidase and hemodialytic treatments should be given timely.     

Development of diffuse large B cell lymphoma during the maintenance therapy for B-lineage acute lymphoblastic leukemia

Non-Hodgkin lymphoma (NHL) is a very rare complication of acute lymphoblastic leukemia (ALL). A Japanese boy presented with B-lineage ALL at the age of 2.5. He was treated with chemotherapy for standard-risk ALL. While he was receiving maintenance treatment 2 years and 9 months after the diagnosis of ALL, diffuse large B cell lymphoma (DLBL) was diagnosed from a biopsy of an abdominal mass. DLBL was treated by surgical resection followed by chemotherapy for 6 months. The patient has been free from the recurrence of ALL or DLBL for 16 months after the development of DLBL.     

Hospital observation for right lower quadrant abdominal pain with questionable acute appendicitis in children

920 children below the age of 12 years were admitted with complaints of pain in the right lower abdomen and a suspected diagnosis of acute appendicitis. In 720 patients, clinical diagnosis was made and immediate operation was performed. In 644 of them (89.5%) an intraabdominal lesion was found but in 76 (10.5%) no disease was encountered. Rest 200 patients were observed in the ward and progression was noted at regular intervals. Eight of these patients did not improve while on observation and they were operated. Five others did not have acute appendicitis but in them definite medical diagnosis was made. However in remaining 187 observed patients abdominal signs gradually resolved and needed no surgery but no definite diagnosis also could be made. They appeared to have non-specific abdominal pain. The conclusion of the study was that inhospital observation of patients with right lower quadrant abdominal pain and questionable appendicitis upto three days was a safe way to reduce the rate of negative appendicectomies and unnecessary surgical exploration.     

A complete ileo-tumor-colic fistula in a child with Burkitt's lymphoma

A boy with abdominal Burkitt's lymphoma developed rectal bleeding, abdominal distension, pain, and fever three weeks after diagnosis. Imaging studies revealed a necrotic tumor mass allowing a fistulous pathway from the ileum to the proximal colon. A laparotomy was performed, with resection of the large necrotic tumor. The child recovered, and has had no further evidence of Burkitt's disease. The radiological evaluation performed in this case ensured proper medical management and surgical intervention during a life-threatening event. the possibility of bowel perforation as a complication of therapy for abdominal lymphoma must be recognized.