Bilateral primary renal lymphoma with orbital metastasis in a child

Primary renal lymphoma (PRL) is a rare condition and bilateral PRL even rarer. Most of these bilateral PRL have been reported in adults. We describe a 3‐year‐old male with bilateral primary renal B cell lymphoma with orbital metastases. We discuss the difficulties in diagnosis and management of this rare presentation of lymphoma. Pediatr Blood Cancer 2009;52:539–541. © 2008 Wiley‐Liss, Inc.     

Primary renal lymphoma presenting with hypertension

Primary renal lymphoma (PRL) is a rare lymphoma which usually presents with hematuria, flank pain, abdominal mass, and weight loss. PRL is more diagnosed in adults than children. We describe an asymptomatic child who presented with hypertension and was subsequently diagnosed with primary renal lymphoma. This case represents an atypical presentation for PRL.     

Primary B-cell lymphoma of bone in children

Primary lymphoma of bone (PLB) is a rare entity of extranodal non-Hodgkin lymphoma (NHL). We report on two children with PLB focussing on diagnostic evaluation and treatment strategy. Clinical and radiographic presentation in both children suggested a primary bone tumour such as Ewing sarcoma. A 13-year-old girl showed osteolytic tumours in the right 7th rib and right iliac crest. Additional skeletal lesions were found by whole-body positron emission tomography. A 6-year-old boy presented with an isolated, osteolytic lesion of the left distal femur. In both patients staging procedures excluded any organ involvement besides the skeletal tumours. Tumour biopsy and immunohistological studies revealed lymphoblastic non-Hodgkin lymphoma of B-cell lineage in both children. They received a polychemotherapy for B-cell lymphoma according to the NHL-BFM 95 protocol and are in complete remission with a follow up of 24 and 18 months respectively. Conclusion Isolated, primary lymphoma of bone in children may clinically and radiographically impose as primary bone tumour. Multiple therapeutic strategies have been applied in the treatment of this malignancy, however, treatment modalities are not well focussed on immunological patterns in the case of primary lymphoma of bone. Staging techniques should include immunophenotyping to initiate specific cell lineage treatment. Received: 1 March 2000 / Accepted: 7 November 2000     

Primary Burkitt lymphoma presenting as a solitary rectal polyp in a child

Non-Hodgkin lymphoma (NHL) accounts for approximately 60% of all lymphomas in children and adolescents. It represents 8-10% of all malignancies in children aged between 5 and 19 years. Primary rectal lymphoma in childhood is extremely rare. This report focuses on the importance of considering the possibility of malignancy in rectal polyps. We report a 5-year-old girl with fresh rectal bleeding who was admitted in our clinic. In physical exam, we found a single pedicled polyp on the posterior wall of the rectum. Surgical removal under general anesthesia involved polyp and its pedicle. Dimensions of the extirpation material were 1.5 x 1.0 x 0.8 cm. Histopathological examination and immunohistochemistry study of the polyp revealed a high-grade B-cell lymphoma (Burkitt lymphoma). These studies also showed lymphoma cells on the surgical border. The patient was referred to pediatric oncology center for chemotherapy. Primary rectal lymphoma in childhood is extremely rare; therefore, the possibility of malignancy in rectal polyps should be considered in the pediatric patients.     

Primary leptomeningeal histiocytic lymphoma in a young child

A 20-month-old boy had an 8-week history of vomiting, lethargy, generalized muscle weakness, and seizures. There was no history or clinical signs of an underlying systemic disease or an immunodeficiency. Cerebrospinal fluid (CSF) had 99 nucleated cells/cu mm, malignant cells, high protein and normal glucose. CT and MRI scans showed diffuse meningeal enhancement around the brain and spinal cord, but no parenchymal involvement. Biopsy of the leptomeninges showed malignant cells with marked nuclear pleomorphism and prominent clear to eosinophilic cytoplasm. The immunohistochemical studies were positive for histiocyte-macrophage markers and were negative with T and B cells, Ki-1, neural and glial cell antibodies. Multiple tests revealed no other site of disease. The patient died 3 months after onset of treatment despite intensive IV and intrathecal chemotherapy. We have not found any other reported case of primary histiocytic leptomeningeal lymphoma in a young child. © 1996 Wiley-Liss, Inc.     

Primary cardiac Burkitt lymphoma in a child

Primary cardiac lymphoma (PCL) is a rare and usually fatal malignancy, seldom reported in children. This report describes the case of a 10-year-old boy who presented with multiple intracardiac masses which, when biopsied, proved to be small non-cleaved cell (Burkitt's) lymphoma. The first two cycles of chemotherapy according to the LMB 96 protocol were given under close cardiological supervision, with good response. The treatment was then continued with full-dose chemotherapy, without any cardiological complication. The patient who was treated by chemotherapy alone remains in complete remission 36 months after the end of treatment and can presently be considered as cured, without late cardiac effect.     

儿童原发性皮肤前B淋巴母细胞性淋巴瘤2例并文献复习

目的了解儿童原发性皮肤前B淋巴母细胞性淋巴瘤的临床、病理特点以及治疗和预后,提高对本病的认识。方法对2例患儿资料进行临床病例分析及文献复习。结果 2例患儿分别以左膝关节及右颌下皮肤皮疹起病,均伴有局部淋巴结肿大,早期被误诊为皮肤感染,抗感染治疗无效,经皮肤活检病理确诊为前B淋巴母细胞性淋巴瘤,骨髓活检病理提示骨髓受累,就诊时临床分期即为Ⅳ期,按BCH-LBL-2003方案规律化疗,分别随诊26及31个月,至今完全缓解无复发。结论该病早期仅有皮肤受累,易误诊或漏诊,怀疑本病应尽早做活检病理检查确诊,并进行全面评估分期,骨髓活检对该病分期诊断具有重要意义,多药联合化疗预后较好。     

儿童伴IRF4 基因重排大B 细胞淋巴瘤临床病理分析

目的探讨儿童伴IRF4基因重排大B细胞淋巴瘤的临床及病理特点。方法回顾分析3例儿童伴IRF4基因重排的大B细胞淋巴瘤患儿的临床资料。结果 3例患儿均为男性,发病年龄5岁4月龄至7岁10月龄,发病部位分别为扁桃体、回盲部及颈部淋巴结。3例患儿手术后组织病理形态及免疫组织化学检查显示,肿瘤细胞呈结节样或弥漫性分布,瘤细胞均表达CD20、MUM1、BCL-6和BCL-2,2例表达CD10。FISH检测IRF4基因,2例断裂阴性、1例阳性。均诊断为伴IRF4基因重排的大B细胞淋巴瘤。3例患儿均按CCCG-NHL-2016方案治疗,其中2例按CCCG-NHL-2016方案R2组执行,1例按R3组执行。2例患儿已经结束化疗1年余,随访至今无复发;1例颈部淋巴结起病的R2组患儿仍在治疗中,目前已获得完全缓解。结论儿童伴IRF4基因重排的大B细胞淋巴瘤少见,行FISH检测IRF4基因有助诊断,可按CCCG-NHL-2016方案治疗。     

Granulomatous reaction in mediastinal B-cell non-Hodgkin lymphoma and intracardiac thrombosis

Epithelioid cell granulomas may be associated with several neoplasms. Lymphomas may mimic or are associated with epithelioid granulomas. In this article the authors report a child with granulomatous reaction in B cell non-Hodgkin lymphoma and intracardiac thrombosis. Although cancer alone is a risk factor for thromboembolism, thrombosis is a multifactorial disorder with both hereditary and acquired risk factors. This is the first reported case of intracardiac thrombosis with MTHFR A1298C and factor XIII V34L mutations together with granulomatous reaction in non-Hodgkin lymphoma.     

Burkitt lymphoma in a child with Joubert syndrome

Joubert syndrome is a rare disorder, characterized by hypoplasia, or aplasia of the cerebellar vermis, hypotonia, ataxia, and psychomotor retardation. The molecular basis underlying the disease is still unknown. There are various syndromes, which are associated with malignancies. Previously known associations between Joubert syndrome and tumors, are benign soft tissue tumors of the tongue and laryngeal lymphangioma. This report describes a 17-year-old boy known with Joubert syndrome, who was diagnosed with Burkitt lymphoma. The boy received chemotherapy, which successfully induced complete remission.     

Recurrent pancreatitis after renal transplantation in a child

Abstract:  A five-yr-old girl, who was a renal transplant recipient, presented with nausea, vomiting, epigastric discomfort, papules, and vesicles on her body. She was diagnosed with acute pancreatitis and varicella zoster infection because her serum amylase and lipase levels were positive. Fourteen months later, she was readmitted with nausea, vomiting, and epigastric pain similar to the previous symptoms and was diagnosed with acute pancreatitis. This case report indicates that acute pancreatitis can be one of a number of complications following pediatric renal transplantation and can recur because of various causes.     

Arthritis as a presenting feature of non-Hodgkin's lymphoma

Leukaemia can present with joint swelling in the absence of abnormal haematological findings. Arthritis as a presenting sign of lymphoma, however, is extremely rare. Three children with non-Hodgkin''s lymphoma who had joint swelling at the onset of their disease are reported. Two cases showed histological features of anaplastic large cell lymphoma (Ki-l/CD30 positive), and one of angioimmunoblastic T cell lymphoma. In all patients the unusual presentation delayed correct diagnosis.     

Extrarenal rhabdoid tumour presenting as a congenital subcutaneous mass

Extrarenal malignant rhabdoid tumour is a rare neoplasm of childhood. A congenital example has only been reported once previously, presenting as a facial mass. We report a second congenital case with the tumour over the right shoulder in which postoperative investigations revealed multiple hepatic metastases proving fatal at 51 days, with adjuvent therapy felt inappropriate.     

Cavernous transformation of the portal vein in a child with non-Hodgkin's lymphoma

We present an 11 year old boy who developed collateral vessels in the portal hepatis with non-visualization of the portal vein 9 months after treatment for large cell lymphoma. This “cavernous transformation of the portal vein” may lead to varices with subsequent gastrointestinal hemorrhage. Med. Pediatr. Oncol. 29:143–145, 1997. © 1997 Wiley-Liss, Inc.     

African histoplasmosis presenting as a facial tumour in a child

A case of African histoplasmosis with an unusual presentation in a 13-year old Nigerian school boy is described. The diagnosis was established by histological examination of biopsy material and treatment with amphotericin B was successfully undertaken.     

儿童成熟B细胞非霍奇金淋巴瘤临床特点及预后分析

目的 了解儿童成熟B细胞非霍奇金淋巴瘤（B-NHL）的临床特征和治疗结局。方法 28例患儿均以CCCG-B-NHL 2010方案化疗,其中20例联合美罗华治疗,随访31（4~70）个月。回顾性分析患儿的临床特征,以Kaplan-Meier法进行生存分析,并且进行预后因素的单因素分析。结果 28例患儿中伯基特淋巴瘤（BL）17例（61%）,弥漫大B细胞型淋巴瘤（DLBCL）8例（29%）,3 例（11%）未能进一步分类。首发表现为颈部肿物的13例（46%）,颌面部肿物10例（36%）,肝脾大9例（32%）,腹部包块5例（18%）,突眼5例（18%）。LDH<500 U/L者14例,500~1 000 IU/L者3例,≥1 000 IU/L者11例。化疗2疗程后,21例完全缓解、7例部分缓解,截至随访末期,24例持续完全缓解、4例复发。2年无事件生存率为85.7±6.6%。骨髓活检提示骨髓浸润、LDH≥500 IU/L、骨髓肿瘤细胞 > 25%的成熟B-NHL患儿2年累积生存率较低。结论 CCCG-B-NHL 2010方案联合美罗华治疗儿童B-NHL疗效满意,骨髓活检发现的骨髓浸润与不良预后相关。