Hepatocellular carcinoma with central nervous system metastasis: A case report and literature review

In the United States and western Europe, hepatocellular carcinoma is not generally regarded as a distantly metastasizing tumor. The ability of hepatocellular carcinoma to metastasize to the brain is illustrated by the example of a 65-year-old white American man with no identifiable risk factors for primary liver cancer. Though hepatocellular carcinoma rarely metastasizes to the central nervous sytem, such metastases are reported to have led to tumor diagnosis or signaled tumor relapse.     

Long-term remission of children with relapsed and secondary anaplastic large cell non-Hodgkin lymphoma (ALCL) following treatment with pulsed dexamethasone and low dose etoposide

Anaplastic large cell lymphoma (ALCL) accounts for approximately 15% of childhood NHL. Relapsed ALCL represents a formidable challenge because outcome is poor despite the use of high-dose chemotherapy regimens. We report two patients with relapsed T-type and 0-type ALCL who achieved long-term 3rd and 4th remissions with 4-weekly oral dexamethasone (DEX) and etoposide pulses for 2 years. This regimen also induced and maintained remission in a third patient with Nijmegen breakage syndrome (NBS) with secondary T-type ALCL. These patients demonstrate that low-intensity oral chemotherapy can induce long-term remissions and offer a curative perspective in refractory, relapsed and secondary ALCL.     

Results of AIEOP LNH-97 protocol for the treatment of anaplastic large cell lymphoma of childhood

Background

Anaplastic large cell lymphoma (ALCL) represents approximately 15% of all pediatric non‐Hodgkin lymphomas (NHL). It has distinct clinical features, including frequent involvement of extranodal sites and rare localization to the central nervous system (CNS). Despite varying treatment approaches the outcome of patients with ALCL has not significantly improved during the last two decades.

Procedure

From October 1997 to beginning of 2000, newly diagnosed ALCL patients were enrolled into AIEOP LNH‐97 protocol for ALCL. Thereafter and until 2007, only CNS positive patients were included. AIEOP LNH‐97 was based on the BFM‐95 schema for ALCL and included six high‐dose chemotherapy courses. CNS prophylaxis was obtained with one intrathecal injection of chemotherapy in each course, whereas treatment of CNS involvement included three intrathecal injections without irradiation.

Results

Thirty‐two patients were eligible for the study. Lymph‐node disease was the most frequent localization (69% of the cases), followed by mediastinal (25%), CNS (22%), bone marrow (16%), and skin (13%) involvement. Probabilities of overall survival (OS) and of event‐free survival (EFS) at 5 years for the whole population were 87% (SE 6%) and 68% (SE 8%), respectively.

Conclusions

This study confirmed that short pulse chemotherapy is an efficacious treatment option for first line therapy of pediatric ALCL, and that dose intensity may have some relevance for outcome, but not in all of the patients. Refinement and optimization of therapy strategies for ALCL may originate from a combination of clinical and biological prospective studies, as those in the pipeline of current international collaboration. Pediatr Blood Cancer 2012; 59: 828–833. © 2012 Wiley Periodicals, Inc.     

Central nervous system involvement at diagnosis in a case of pediatric CD30+ anaplastic large cell lymphoma

Central nervous system (CNS) involvement in Ki-1/CD30 lymphoma is extremely rare, in contrast to the frequent involvement in other types of pediatric non-Hodgkin's lymphoma. No mechanism has yet been proposed to explain the sparing of the blood brain barrier in Ki-1/lymphoma. We present a 2-year-old boy who was admitted to the Department of Pediatric Hemato-Oncology due to lethargy, progressive breathing difficulties, massive diffuse lymphadenopathy, hepatosplenomegaly, and ichthyosis-like skin involvement with epidermolysis. A lymph node biopsy was compatible with Ki-1/CD30 anaplastic large cell lymphoma (ALCL). Bone marrow aspirate and biopsy demonstrated reactive hyperplasia. Cytogenetic analysis displayed hyperdiploid cells with 1p(−) in most cells. Cerebrospinal fluid examination showed pleocytosis with CD30+ cells. Possible mechanisms which could enable CNS involvement in this unusual case are discussed. Med. Pediatr. Oncol. 28:132–135 © 1997 Wiley-Liss, Inc.     

Isolated central nervous system (CNS) relapse in a case of childhood systemic anaplastic large cell lymphoma without initial CNS involvement

Central nervous system (CNS) involvement in cases of anaplastic large cell lymphoma (ALCL) has been described only rarely. The authors describe an 11-year-old girl with ALCL who developed isolated CNS relapse but had no CNS disease at initial diagnosis and had received CNS-prophylactic treatment. The patient achieved a second remission following intensive treatment of the relapse and continues to be in remission at the time of writing. This case serves to emphasize that isolated CNS relapse without detectable initial CNS involvement can arise even after CNS-prophylactic treatment in pediatric ALCL cases.     

白血病阶段的小细胞亚型间变性大细胞淋巴瘤1例并文献复习

目的探讨间变性大细胞淋巴瘤(ALCL)白血病阶段的临床特点、诊断、治疗及相关进展。方法对一例白血病阶段的ALK阳性的ALCL患者的诊治经过进行回顾性分析及相关文献复习。结果 4岁男童因淋巴结肿大24 d入院,院外按淋巴结炎抗感染治疗无效。入院时患儿高热,精神差,肝、脾、淋巴结肿大,白细胞81.99×10~9/L。根据病理、FISH、骨髓流式细胞术、细胞因子检查确诊为小细胞型ALCL的白血病阶段,合并高细胞因子血症。给予血浆置换使患者一般状态得到改善后立即给予化疗,期间合并骨髓抑制及感染,家长放弃治疗。结论小细胞病理亚型的ALCL侵袭性强,可以合并白血病,易和感染性疾病及其他小细胞肿瘤相混淆,需引起临床医生的注意。患者可合并高细胞因子血症,致病情危重,治疗风险大。     

Isolated cutaneous anaplastic large cell lymphoma progressing to severe systemic disease with myocardial involvement and central nervous system infiltration

Anaplastic large cell lymphoma (ALCL) is a rare tumor comprising around 10-15% of childhood lymphomas. We describe the case of a female who initially presented with localized skin disease associated with an insect bite. However, she subsequently relapsed with widespread systemic ALK-positive ALCL that included lymphoma deposits in the myocardium, a very rare manifestation. Her disease responded well to chemotherapy but she later developed a fatal relapse in the CNS. We also present data on an immune response to ALK, demonstrating a fluctuation in the levels of circulating antibodies to ALK corresponding to the different phases of her illness.     

Infant anaplastic lymphoma: case report and review of the literature

Anaplastic large cell lymphoma (ALCL) is a well-known entity, but there are no data on prognosis according to the age of the patient, especially in infants. A 2-month-old girl was admitted with a 2-week history of coughing, fever, and lymphadenopathy. Physical examination revealed mild respiratory distress, an erythematous macular rash on her trunk, massive cervical lymphadenopathy, splenomegaly, and very mild ascites. Chest radiograph showed bilateral pulmonary infiltrates, pleural effusion, and a mediastinal mass. CBC count showed WBC: 172,000/μL (PMN 40%, lymphocytes 47%, monocytes 3%); hemoglobin concentration: 8.7 g/dL; platelets: 390,000/μL. Cervical lymph node biopsy revealed anaplastic lymphoma with positive staining to ALK 1 and TIA 1. Immunophenotypic analysis of peripheral and bone marrow lymphoid cells showed an aberrant T-cell immunophenotype, including expression of CD3, CD45R0⁺, CD43⁺, and CD30⁺. Cytogenetic analysis performed on blood and bone marrow samples demontrated the translocation t(2;5) (p23;q35), and trisomy 47. After leucophoresis, the child received chemotherapy according to the ALCL-99-EICNHL protocol, and was started on corticosteroids and cyclophosphamide, which resulted in marked improvement. After the second course, WBC decreased to 6000/μL without tumor lysis syndrome, but the child developed bacterial and fungal disseminated infections and died of septic shock with multiorgan failure. This report is of a rare case of infant anaplastic lymphoma and excellent response to treatment. Unfortunately, she did succomb to overwhelming infection. More reports of similar cases may determine the cause and prognosis of such children, helping to tailor therapy according to the age of the child and other prognostic factors, especially bone marrow involvement.     

Childhood intrathoracic Hodgkin lymphoma with hypertrophic pulmonary osteoarthropathy: a case report and review of the literature

Hypertrophic osteoarthropathy (HOA) is characterized by clubbing, periosteal new bone formation and polyarthritis. The pathogenesis of clubbing involves an increased expression of platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) from the digitally lodged platelet clumps, which bypass the pulmonary capillary network as a result of various systemic disorders. Intrathoracic neoplasms are rare causes of HOA in children. We report here a 14-year-old boy with digital clubbing, who eventually received the diagnosis of intrathoracic Hodgkin lymphoma (HL) and HOA. Eight cases previously reported with these two diagnoses are reviewed to emphasize the prognostic significance of HOA in childhood HL. Conclusion: Given the pathogenesis of clubbing and the prognostic significance of HOA, intrathoracic disease should be considered when HOA is detected in a child with a known or suspected malignant disease, and the occurrence of HOA during follow-up should alert the physicians for possible recurrence of the neoplastic disease or intrathoracic involvement. There is no conflict of interest associated with this report nor any sponsors.     

NHL in adolescents and young adults: A unique population

Non‐Hodgkin lymphoma (NHL) is a heterogeneous group of lymphoid malignancies with high incidence in adolescents and young adults (AYAs). The most common diseases include diffuse large B‐cell lymphoma, anaplastic large cell lymphoma, Burkitt lymphoma, lymphoblastic lymphoma, and primary mediastinal large B‐cell lymphoma. In comparison to younger children and adults, AYAs (15–39 years) with NHL present a specific set of challenges including variations in tumor biology, inconsistent treatment, pharmacodynamics, and psychosocial barriers. While survival of AYAs with NHL has improved, cure rates remain suboptimal. Incorporation of novel agents into pediatric‐inspired treatment regimens specifically designed for NHL in AYAs has led to improved outcomes. Consideration of AYAs as a distinct population in the diagnosis and treatment of NHL is encouraged.