This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Chim, C. S. Articles by Liang, R. Search for Related Content PubMed PubMed Citation Articles by Chim, C. S. Articles by Liang, R. Social Bookmarking                            What's this?

Case 2: Meningeal Relapse in Hodgkin’s Disease

C. S. Chim, T. W. Shek, G. C. Ooi, R. Liang

Queen Mary Hospital, University of Hong Kong, Hong Kong

CASE 2: MENINGEAL RELAPSE IN HODGKIN’S DISEASE

A 23-year-old woman with clinical stage IIA nodular sclerosing Hodgkin’s disease involving bilateral cervical lymph nodes achieved complete remission (CR) in Canada after mantle-field irradiation (40 Gy). She remained in CR until she developed progressive headache, blurred vision, and mental obtundation. A computed tomography (CT) scan showed a large lobulated mass in the right parieto-occipital region, measuring 7 cm in the greatest dimension. It had a broad base abutting the dura in the right parietal vertex with midline shift (Fig 1A, an unenhanced axial CT scan of the brain, shows a hyperdense slightly lobulated mass with peritumoral edema, while Fig 1B, an enhanced scan, shows homogeneous enhancement of the lesion; note the enhancing dural tail [arrowheads]). Craniotomy showed a meningeal tumor, and complete excision of the tumor was performed. Histologic examination of the excised tumor showed extensive replacement of the meninges by classical Hodgkin’s disease, which was characterized by the presence of an abnormal lymphoid infiltration with numerous lacunar and Reed-Sternberg cells in a background of mixed reactive inflammatory cells, including eosinophils (Fig 2). She did not have any B symptoms. One month later, she developed a left cervical lymph node. Excisional lymph node biopsy showed recurrent Hodgkin’s disease. A complete blood count showed a hemoglobin level of 12.8 g/dL (normal range, 11 to 16 g/dL), platelet count of 230 x 10⁹/L (normal range, 150 to 400 x 10⁹/L), and leukocyte count of 8 x 10⁹/L (normal range, 4 to 11 x 10⁹/L) with normal differential. Her serum lactate dehydrogenase level was 260 µL (normal, < 400 µL). Serology results for HIV were negative. A CT scan of the thorax and abdomen, a bipedal lymphangiogram, and a gallium scan did not show disease elsewhere. Bilateral trephine biopsies were negative for disease. She was treated with cranial irradiation (20 Gy) at the site of disease and six courses of cyclophosphamide, vincristine, procarbazine, and prednisolone. She remains in CR with no clinical evidence of neurologic deficit as of August 1999.

View larger version (76K): [in this window] [in a new window]   Fig. 1. No caption available.

View larger version (178K): [in this window] [in a new window]   Fig. 2. No caption available.

Most patients with CNS Hodgkin’s disease present with headache, cranial nerve palsy, motor deficits, and seizures caused by the mass effects. Dural involvement is more common than intracerebral lesions. The majority of the cases are of classical Hodgkin’s disease, nodular sclerosis type,^1-3 which constitutes more than half of the cases.⁴ Our patient had nodular sclerosing Hodgkin’s disease and developed meningeal relapse after a remission of 8 years.

The main differential diagnosis in our case was a meningioma, based on the CT findings of a hyperdense, extra-axial, dural-based mass, with homogeneous contrast enhancement. Because of the rarity of CNS Hodgkin’s disease, most published radiologic series have almost exclusively been on CNS non-Hodgkin’s lymphoma.^5-7 The CT appearances for both primary and secondary CNS non-Hodgkin’s lymphoma are identical; most occur as solitary, homogeneously enhancing lesions in thecentral grey matter/corpus callosum that may encroach on an ependymal or meningeal surface.^5,7 Close contact with the meninges may mimic a meningioma.^6,7 In our case, two imaging features were atypical of a meningioma: the absence of adjacent skull vault hyperostosis and the presence of marked peritumoral edema.^6,7 Our findings suggest that the imaging features of intracranial Hodgkin’s disease may be comparable to those of non-Hodgkin’s lymphoma. Histologic examination was, however, essential in our case for definitive diagnosis. The presence of Reed-Sternberg and lacunar cells, together with the history of Hodgkin’s disease, clinched the final diagnosis of meningeal relapse in Hodgkin’s disease.

Response to therapy is often poor,^3,8 with survival reported to range from 6 to 180+ months after intracranial presentation.^1,4 However, when combined-modality treatment (neurosurgery if indicated, irradiation and combination chemotherapy) is given with curative intent, prolonged disease-free survival may be achieved.^1,4 Indeed, our patient had curative surgical excision of the tumor followed by combination chemotherapy and radiotherapy and remained in CR for 6 years after the intracranial relapse.

REFERENCES

1. Sapozink MD, Kaplan HS: Intracranial Hodgkin’s disease: A report of 12 cases and review of the literature. Cancer 52:1301-1307, 1983[Medline]

2. Burke JS: Hodgkin’s disease: Histopathology and differential diagnosis, in Knowles DM (ed): Neoplastic Hematopathology. Baltimore, MD,Williams & Wilkins, 1992, pp 497-533

3. Cavalli F: Rare syndromes in Hodgkin’s disease. Ann Oncol 9:s109-s113, 1998 (Suppl 5)

4. Anselmo AP, Prioa A, Cartoni C, et al: Meningeal localization in patient with Hodgkin’s disease: Description of a case and review of the literature. Ann Oncol 7:1071-1075, 1996 [Abstract/Free Full Text]

5. Clifford RJ, O’Neill BP, Banks PM, et al: Central nervous system lymphoma: Histologic types and CT appearance. Radiology 167:211-215, 1988[Abstract/Free Full Text]

6. Herkes GK, Partington MD, O’Neill BP: Neurological features of cranial vault lymphomas: A report of two cases. Neurosurgery 29:898-901, 1991[Medline]

7. Gutman J, Kendall B: Unusual appearances of primary central nervous system non-Hodgkin’s lymphoma. Clin Radiol 49:292-702, 1994

8. Kaplan HS: Patterns of anatomic distribution, in Kaplan HS (ed): Hodgkin’s Disease (ed 2). Cambridge, MA,Harvard University Press, 1980, pp 280-339

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Chim, C. S. Articles by Liang, R. Search for Related Content PubMed PubMed Citation Articles by Chim, C. S. Articles by Liang, R. Social Bookmarking                            What's this?