Intracranial Hodgkin's disease. A report of 12 cases and review of the literature

Intracranial involvement by Hodgkin's disease is rare. Of the 30 previously reported cases, in many of which central nervous system (CNS) disease was documented postmortem, no associated risk factors have been elucidated, and response to treatment has been poor. This report cites 12 additional cases treated at the Stanford University Medical Center, five of whom have enjoyed prolonged freedom from intracranial relapse.     

Intracranial presentation of systemic Hodgkin's disease

Intracranial involvement by Hodgkin's disease is rare. We report a patient with Hodgkin's disease who had intracranial disease at presentation. We also review the literature pertaining to intracranial Hodgkin's disease. Using the key words "Hodgkin's disease" and "central nervous system (CNS) disease", we searched the Pubmed and Cancerlit databases. References were systematically reviewed and data regarding the following variables was extracted: age, gender, signs and symptoms at presentation, histology of Hodgkin's disease, cerebrospinal fluid analysis, stage and treatment. Only 36 cases of intracranial Hodgkin's disease were identified in the literature. Intracranial Hodgkin's disease at presentation is even more uncommon with only 8 reported cases. Most cases of intracranial involvement by Hodgkin's disease occur at the time of relapse. The most common presenting feature of intracranial Hodgkin's disease is a cranial nerve palsy with brain parenchyma being the most common intracranial site of involvement. Mixed cellularity histology is the most frequent subtype of Hodgkin's disease among these patients and the median survival following intracranial presentation is 46 months. Treatment has varied extensively but includes whole brain radiation with or without combination chemotherapy. Our literature review suggests that the prognosis is not dismal with appropriate treatment.     

Hodgkin's disease in children. Pathologic study of 87 cases

From 1963 to 1977 at the Istituto Nazionale Tumori at Milan, 112 patients below the age of 16 years with Hodgkin's disease (HD) were observed, representing 13.2% of all the cases of this disease seen during the stated time interval. Eighty-seven of these cases are the subject of the present study. Fifty-nine patients were males and 28 females (2.1:1 ratio). The age range varied from 2 years 10 months to 15 years 10 months (median 10 years). Forty-three (49.4%) children, of whom 35 were males and 8 females, were below the age of 10 years at the onset of their disease. The clinical staging resulted in 34 patients as stage I, 33 as stage II, 13 as stage III and 7 as stage IV. The histologic type was nodular sclerosis (NS) in 49 cases (56.3%), lymphocytic predominance (LP) in 15 cases (17.2%), mixed cellularity (MC) type in 9 cases (10.3%) and lymphocytic depletion (LD) in 8 cases (9.2%). In the remaining 6 cases the histologic classification was not applicable. LP type in 15/15 (100%) patients was associated with stages I and II, and NS in 38/49 (77%) patients was related to stage I and stage II. The latter was also the istologic type most often encountered in patients with stage II disease (23/33 or 70%). Eleven patients have died, and their survival varied from 6 to 47 months (median 30 months). The histologic type was LD in 4 cases, NS in 3 cases, MC in 1 case, and LP in 1 case. In the other 2 nonsurvivors, the histologic type was not identifiable. Of the 23 patients with more than a 5-year survival, 14 (60.8%) had NS HD. As in adults, LP and NS were associated with early stages of the disease and with long survival.     

Childhood Hodgkin's disease in Israel. A study of 17 cases.

A study of 17 children suffering from Hodgkin's disease (H.D.) is presented. The special ethnic distribution with predominance in Arab and Oriental Jewish children is noted. A high incidence of mixed cellularity histology in Arab children is emphasized. A conservative approach to therapy, i.e. extended field regional radiotherapy only, resulted in a long first complete remission period in lymphocyte predominance and nodular sclerosis H.D. Stages IA and IIA. All children in Stage IIIB and IV were treated by six courses of combined chemotherapy, and were maintained either on chlorambucil and vinblastine or on MOPP. Patients with mixed cellularity H.D. Stage IIA and all patients in Stage IIIA were treated by four courses of MOPP after the completion of extended regional radiotherapy. The 5 children who did not receive the schedules treatment died. The remaining 12 are well, with a survival of 8 to 104 months.     

Primary pulmonary Hodgkin's disease. A clinicopathologic study of 15 cases

Fifteen cases of primary pulmonary Hodgkin's disease are reported. This presentation of Hodgkin's disease occurred more frequently in women (2:1) and older individuals, and B symptoms were relatively common (7 of 13). The majority of patients had nodular lesions on chest radiographs. An unfavorable prognosis was linked to B symptoms, older age (greater than 60 years) and multiplicity and bilaterality of lung lesions. The histologic differential diagnosis is discussed, particularly in regard to Wegener's granulomatosis and non-Hodgkin's lymphomas.     

Extrahepatic Hodgkin's disease with intrahepatic cholestasis: report of two cases.

Liver is involved in about 5-8% of newly diagnosed Hodgkin's disease (HD) cases. The incidence reaches up to 50-60% in postmortem studies. In the literature only a few cases of idiopathic cholestatic jaundice have been described without an apparent cause and a paraneoplastic etiology has been suggested. We report 2 cases with HD presenting with obstructive jaundice without obvious liver involvement. The first case died soon after diagnosis; the second case received chemotherapy and radiotherapy, and she is well at 26 months' follow-up. Extrahepatic HD with intrahepatic cholestasis is an extremely rare situation without an established approach. Such cases like the present ones may help to understand the pathogenesis of the liver involvement of HD and determine the best management of these cases.     

Ultrastructural and immunocytochemical localization of immunoglobulin synthesis in tumor cells in Hodgkin's disease.

An immunocytochemical and ultrastructural study using the Fab fragment of an anti-human Ig antibody labelled with peroxidase was carried out on affected lymph nodes from five Hodgkin's disease patients. The tumor cells (Reed-Sternberg cells and Hodgkin cells) showed an exclusively hyaloplasmic granular staining. By comparing these grains with ribisome staining. By comparing these grains with ribosome staining of the endoplasmic reticulum of plasma cells it could be suggested that they are free risobomes. This ribosomal Ig synthesis is a major argument for the B lymphocyte nature of Reed-Sternberg and Hodgkin cells. The total absence of vacuole staining allows us to conclude that these cells are not histiocytic or macrophage derivatives.     

Blood eosinophilia in Hodgkin's disease. A follow-up of 25 cases in Venezuela.

Twenty-five patients with Hodgkin's disease and high eosinophil counts were observed for an average of 90 months. Fluctuations in the levels of eosinophils were important in the course of observation. No relation with stages, histologic type, or evolution was noticed. Steroid-containing regimens and severe premortem conditions seemed to lower the counts. Relapse-free survival was shorter in our 25 patients than in a control group of 50 patients with Hodgkin's disease and no eosinophilia who had approximately the same stage, histologic type, and treatment of disease. However, the overall survival was somewhat better for the eosinophilic patients with stages IIIB and IV (0.1 greater than P greater than 0.05).     

Hodgkin's disease in childhood.

A retrospective study of 116 children with Hodgkin's disease diagnosed in the period 1935-1970 was undertaken to assess the prognostic role of histopathologic classiciation and clinical extent of the disease. The ages of the 80 boys and 36 girls ranged from 2.5 years to 15.0 years (mean, 10.0 years). The histopathologic diagnosis by lymph node biopsy revealed lymphocyte predominance in 22, nodular sclerosis in 67, mixed cellularity in 24, and lymphocyte depletion in 3. Within the subgroup of nodular sclerosis, 47 biopsies had classic well-developed collagenous bands, whereas 20 were in the cellular phase (10 without collagenous bands and 10 with minimal collagen). The clinical extent of disease was determined. There were 33 patients with Stage I disease, 38 with Stage IIA, 12 with Stage IIB, 24 with Stage III, and 9 with Stage IV. Survival correlated with histopathologic type and clinical stage, but not with age or sex. Survival was not dependent on the degree of collagenization in nodular sclerosis. There were 28 patients who survived for more than 10 years. Four of these 29 subsequently died owing to acute myelomonocytic leukemia, carcinoma of the breast, sepsis, and progression of Hodgkin's disease, respectively. Neoplasms developed in two other long-term survivors (thyroid carcinoma in one, and multiple basal cell carcinomas in the other).     

Hodgkin's disease in Kenya.

A total of 136 patients with Hodgkin's disease (HD) seen at a national referral hospital in Kenya over a 15-year period were studied. The overall male-to-female ratio was 2.6:1, with 65% of patients younger than 20 years of age. The bimodal incidence of HD frequently seen in the developed nations was absent, and the histologic pattern of the disease was similar to that seen in other African countries. Fifty percent of patients had advanced-stage disease when first seen. Over two thirds of patients treated had a clinical remission, and the relapse rate in all stages was approximately 30%. Therapy-related complications were common, with frequent myelosuppression and younger patients more susceptible.     

The management of Hodgkin's disease.

This paper presents a destillation of the authors' current views concerning the optimal management of Hodgkin's disease in relation to site(s) and stage of disease, constitutional symptoms, histopathology, and prior treatment, based on experience with a series of controlled clinical trials under way at Stanford University Medical Center since 1962.