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Primary central nervous system lymphoma (PCNSL) is a rare subtype of non-Hodgkin lymphoma (NHL) in childhood and adolescence, with an increased risk among patients with immunodeficiency.1-5 Given the rarity and lack of any prospective trials, reliable data on PCNSL are lacking, with most data coming from case reports or small series. Thus, 2 of the largest childhood NHL consortia, the European Intergroup for Childhood Non-Hodgkin Lymphoma (EICNHL) and the international Berlin-Frankfurt-Munster (i-BFM) Group, as well as selected North American centers, designed a retrospective multinational study on pediatric PCNSL. Here, we present data from the largest series of pediatric patients with PCNSL reported to date.

Burkitt and Burkitt-like lymphomas (BL) account for 30–40 % of childhood NHL [1, 2]. It is the most common childhood cancer in equatorial Africa [3]. About 2.5 new cases per one million individuals occur every year worldwide. There is a definite predominance of boys over girls, with the ratios ranging from 1.3 to 8.8:1 [2, 4]. The peak age is between 4 and 7 years [4].

Diffuse large B-cell lymphoma (DLBCL) is a mature B-cell neoplasm, which accounts for approximately 10–20 % of paediatric NHL [1]. In fact, this definition represents a morphologically, immunologically and clinically heterogeneous group of lymphoid neoplasms, rather than one entity [2]. In contrast to other childhood NHL, there is no specific genetic feature for DLBCL. The recent WHO classification delineated some newly defined entities on the basis of distinctive clinical, pathologic or biologic features.

Non-Hodgkin’s lymphomas (NHL) occur much less frequently in childhood than in adulthood. The histological subtypes are also restricted to some entities. The more frequent include Burkitt’s lymphoma (BL) (about 50-60% of cases), followed by the lymphoblastic lymphoma (LBL) (25-35%), the diffuse large B-cell lymphoma (DLBL) (f&12%), and the anaplastic large cell lymphoma (ALCL) (8-12%). The proportion depends mainly on the geographical origin of the patients and the upper limit of the age, DLBL being more frequent in adolescents. Although the proportion of these different subtypes is lower in adults, their absolute number might not be very different from that in children. Due to the predominance of extranodal primary sites, the usual staging classification in children is St. Jude’s classification (Table l), which is different from the Ann Arbor classification used in adult NHL. Many advances have been made in childhood NHL, thanks to co-operative national studies with cure rates higher than 70%. Some of these studies might be of benefit in the treatment of adult NHL. The lessons learned from the large co-operative trials can be summarised as follows.

Based on the Swiss Pediatric Oncology Group (SPOG) cancer registry data during 1981–1991, a high average incidence of 8 new NHL per million children younger than 15 years per year was found. Of 162 children with NHL registered in 1976–1991, 120 were study patients, i.e., officially registered and treated according to SPOG or Pediatric Oncology Group (POG) protocols, while 42 were non-study patients, i.e., patients not officially enrolled on protocols. Overall, 91 of 120 (76%) study patients remained alive. Seventy-nine study patients were treated according to older SPOG protocols, and 53 (67%) of these survived, while 38 of 41 (93%) study patients treated according to newer POG protocols remained alive (P = 0.0068). Only 22 (52%) of the 42 non-study patients survived (P = 0.0001). There was no improvement if the survival of non-study patients before and since 1986 was compared. Population-based treatment results in Switzerland were similar to those in the United Kingdom. They provided an important base for the development of future treatment strategies. © 1995 Wiley-Liss, Inc.

The relative frequencies of the various histopathologic types of lymphomas are generally similar among Asian countries. Hodgkin's disease and follicular lymphomas are relatively rare in Asia. Among NHL, the Asians have a higher rate of aggressive NHL, as compared with the NCI data. Immunologic analysis revealed that PTCL is common in Asia. The relative frequency of PTCL is comparable among Chinese in Taiwan, the east coast of China, and Hong Kong, as well as in adult T-cell leukemia/lymphoma (ATLL) nonendemic areas in Japan. The increased rate of T-cell lymphomas in Asia is attributed to the low incidence of follicular lymphomas. The similar patterns of distribution in histopathologic and immunologic subtypes of NHL in Asia suggest that a common ethnic or geographic factor exists. To elucidate it, further detailed epidemiologic studies are needed. Primary extranodal NHL is slightly more prevalent in Asia than in the United States; the most frequent primary site is Waldeyer's ring in Japanese patients and the GI tract in Chinese patients. Primary small intestinal lymphoma in Asia showed the pattern of the Western type. Primary cutaneous lymphomas are rare in Asia. The clinical features of PTCL in Asia are comparable with those described in the United States, except for a predilection for the nasal/paranasal region. In Asia, outside Japan, ATLL has been reported only in Taiwan. The seroepidemiologic survey of carriers of ATLL showed the rate of seropositivity for HTLV-I in Taiwan was similar to that in nonendemic areas in Japan. The clinicopathologic features of ATLL in Taiwan and Japan are essentially identical. In children in Japan and Taiwan, Hodgkin's disease is much less frequent than in the West. However, the relative frequencies of the histopathologic and immunologic subtypes of childhood NHL in Japan and Taiwan do not differ significantly from those of the West. Although Burkitt's lymphoma in Japan and Taiwan is of nonendemic type, in India it may comprise both endemic and nonendemic types in almost equal number.

Data pertaining to primary and secondary osseous involvement in pediatric non-Hodgkin's lymphoma (NHL) are scarce in English literature. Fifty-nine cases of childhood NHL over a period of 3·5 years were reviewed out of which eight had bone involvement, the incidence of skeletal involvement being 13·6%. There were seven males (87·5%) and mean age was 9·9 years (range: 1–15 years). Two patients (25%) had primary bone lymphoma and six cases (75%) were classified as secondary bone lymphoma. Six patients who opted for treatment received chemotherapy; 4/6 (67%) patients are in complete remission at a median follow-up of 41 months (range 19–44 months). Bone involvement was more common in relapsed cases in comparison to de novo presentations.

In this study an attempt is made to propose a working clinical classification and staging of adult non-Hodgkin's lymphoma (NHL) which brings out both primary site and extent of disease. Unlike childhood NHL, where histopathology is uniformly of unfavourable type, this clinical staging system seems to have a prognostic value when applied with reference to different histology groups of working formulation. Based on the experience of staging of childhood NHL as proposed earlier, 304 cases of adult NHL above 14 years of age seen at Kidwai Memorial Institute of Oncology, Bangalore, India, over a period of 5 years (1981-1985) are first categorized according to primary site (initial bulky site at presentation): (1) peripheral nodal (n = 181; (2) extranodal (excluding gastrointestinal tract; n = 48); (3) abdominal (including gastrointestinal tract; n = 46), and (4) mediastinal (n = 29). Each group is further staged according to the extent of the disease. Once categorized into various clinical groups, the Ann Arbor Clinical staging fits very well only with the peripheral nodal group, the major group, although not suitable for other clinical groups.

The types of childhood non-Hodgkin lymphoma (NHL) differ considerably from Hodgkin lymphoma and NHL seen in adults, both pathologically and clinically. Essential to understanding these differences is a knowledge of the three major histologic subtypes (undifferentiated, lymphoblastic, and large cell) that account for the vast majority of cases of pediatric NHL. Each of these subtypes has typical imaging and clinical features. The most common subtype, undifferentiated NHL, usually shows intraabdominal disease. Lymphoblastic tumors most frequently manifest as a mediastinal mass, perhaps with respiratory or circulatory compromise. Large cell tumors show heterogeneous clinical and imaging features but tend to spare the anterior mediastinum. Knowledge of the appropriate imaging modality to be used in evaluation of these tumors is also important. Computed tomography (CT) is the primary imaging modality for staging childhood NHL. Magnetic resonance imaging is best for examination of the central nervous system and bone involvement. Ultrasonography may be useful as a complementary study to abdominal CT; gallium scintigraphy also plays an adjunctive role to CT. Familiarity with typical and atypical patterns of tumoral behaviors and optimal imaging methods aid in the diagnosis and appropriate follow-up of these tumors.

The childhood non-Hodgkin’s lymphomas (NHL) are histologically, immunologically, and clinically a heterogeneous group of diseases. Recent advances in our understanding of NHL have demonstrated the similarities between childhood NHL and childhood acute lymphoblastic leukemia (ALL). Treatment strategies utilizing systemic chemotherapy and modeled after successful treatment programs for ALL have resulted in a dramatic improvement in prognosis for children with NHL, and the majority of affected children are now curable.

During the past decade an evident increase in the curability of children with non-Hodgkin lymphoma (NHL) has been achieved. Intensive multidrug therapy regimens involving local radiotherapy and prophylaxis of the central nervous system have been used with notable success [1–3]. The increasing efficiency of modern therapy of childhood NHL has stimulated further studies. It has been demonstrated that the remarkable heterogeneity of NHL in the relapse risk is related to the primary location of the tumor and its dissemination, histology, and immunology (4–9). During its 6-year observation of children with non-Hodgkin lymphoma treated according to the modified LSA2L2 protocol, the Polish Children’s Leukemia and Lymphoma Study Group has accumulated experience which, together with other studies, could be the background for further refinment of the treatment. This paper summarizes the long-term results in the treatment of NHL with the modified LSA2L2 protocol. The evaluation of the early effects of therapy of disseminated NHL with two other regimens, COAMP [5] and Murphy-Bowmann [4], also is reported.

Twenty-five children with previously untreated stage IV non-Hodgkin's lymphoma (NHL) were studied. At the time of evaluation 16 patients were disease-free (64%), with a median observation time of 23 months. Intensive chemotherapy for childhood NHL provides a better outlook for these patients, including those who would be considered high-risk acute lymphoblastic leukemia (ALL).

Treatment of children with non-Hodgkin’s lymphoma (NHL) with intensive combined modality regimens, incorporating combination chemotherapy, involved field radiotherapy and prophylaxis of the central nervous system, reproducibly cures the majority, as demonstrated by a number of reports [1–4]. Cure of a majority of cases ought not to mask however the considerable heterogeneity characteristic of NHL, either in adult or pediatric age group. While virtually all lymphomas in children are high grade malignancies, significant differences in the relapse hazard with modern therapy are related to the primary site, stage and immunopathologic subtype (T or B). A rational strategy for success in management of childhood NHL therefore requires consideration of these factors. The review will outline such a strategy, rationale and recent results.

The histopathologic classification of non-Hodgkin’s lymphoma (NHL) is discussed in relation to age and sex of 62 children under the age of 15 yr. NHL was less common than Hogkin’s lymphoma in the ratio of 6:7. Male patients were six times commoner than female patients. This male preponderance was seen in all histologic subtypes. The youngest child was 6 months of age The peak incidence was in 6–10 yr age group. Nodular lymphomas were rare constituting only 3.2% of total NHL Diffuse poorly differentiated lymphocytic lymphcma was the commonest subtype in all age groups. Childhood NHL showed greater male preponderance and diffuse pattern as compared to adult NHL Starry-sky appearance in non-Burkitt NHL was noted and was related to soft tissue infiltration.