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Contiguous scalp, skull, and epidural hodgkin's disease
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Surg Neurol 1984;21:182-4
Contiguous Scalp, Skull, and Epidural Hodgkin's Disease David E. Tomaszek, M.D., George W. Tyson, M.D., Paul Stang, B.S., P.A., and Thomas Bouldin, M.D. Departments of Neurological Surgery and Pathology, University of North Carolina School of Medicine, Chapel Hill, North Carolina
Tomaszek DE, Tyson GW, Stang P, Bouldin T. Contiguous scalp, skull, and epidural Hodgkin's disease. Surg Neurol 1984;21:182-4. A patient with Hodgkin's disease of the nodular sclerosing subtype presented with recurrent disease at the site of a previous blunt trauma to the head. The tumor involved the scalp and subjacent skull and epidural space. This particular presentation of Hodgkin's disease is probably unique, and involvement of the skull and cranial cavity by this subtype of Hodgkin's disease is rare. KEYWORDS: Hodgkin's disease; Vascular invasion; Metastasis; Cranial involvement; Scalp; Skull; Epidural
Intracranial involvement by histologically verified Hodgkin's disease is rare. Recent reports cite an incidence of 0.5 % or less [ 14]. We report a case of recurrent nodular sclerosing Hodgkin's disease that presented in the form o f contiguous intracranial and extracranial tumors at the site o f a minor closed-head injury.
Case Report A 23-year-old male presented with a mass in the scalp in the right parietal area. One month earlier he had suffered a minor blow to the head in the same region, and was left with slight residual local pain. Several days later a mass in the scalp appeared at the site of the injury; it was initially diagnosed as a subgaleal hematoma, but neurosurgical consultation was sought because of a progressive increase in the size of the mass. One year prior to this admission, the patient had been diagnosed as having nodular sclerosing Hodgkin's disease. H e was treated with thoracic and abdominal irradiation as well as with methotrexate, vincristine, proAddress reprint requests to: David E. Tomaszek, M.D., Division of Neurological Surgery, 148 Burnett Womack CSB 229H, UNC at Chapel Hill, Chapel Hill NC 27514.
© 1984by ElsevierSciencePublishingCo., Inc.
carbazine, and prednisone (MOPP), and later with bleomycin, cyclohexyl chloromethyl nitrosourea (CCNU), adriamycin, and vinblastine (B-CAV). The scalp mass was the first sign of recurrence o f his disease (stage IV; Ann Arbor classification). The patient denied having fever, weight loss, night sweats, or any symptoms of neurological dysfunction. Physical examination revealed a well-developed white male with normal vital signs. Several large, firm, fixed, nontender lymph nodes were palpable in the right anterior cervical area, and a firm but nontender and not freely mobile mass measuring 6 × 8 × 4 cm was present in the right parietal area. The neurological examination was normal. A computed tomography scan (Figure 1) indicated that this latter mass involved the subgaleal as well as the epidural space. A biopsy of the scalp and epidural masses as well as of the intervening calvarium was performed. The superficial mass was found to lie beneath the pericranium. Microscopic examination (Figure 2) of the biopsy specimens revealed a pleomorphic cellular infiltrate interlaced by fibrous bands. R e e d - S t e r n b e r g cells were plentiful and the histologic pattern was characteristic of Hodgkin's disease of the nodular sclerosing subtype. The patient developed pleural seeding, disseminated herpes zoster, and severe bone-marrow depression within months of the biopsy procedure. H e died 2 years after the initial diagnosis, without ever developing any focal signs of cerebral dysfunction.
Discussion Hodgkin's disease characteristically affects multiple organ systems. Although neurological complications o f the disease are not uncommon (15% of cases show some neurological dysfunction), most are secondary to either compression of the spinal cord by epidural deposits or to peripheral nerve palsies [3,5,16]. Additionally, the remote manifestations of the disease can produce neurological signs and symptoms, among which granulomatous angiitis [2], herpes zoster [3], and demyelination 0090-3019/84/$3.00
Hodgkin's Disease of Scalp and Skull
Figure 1. Computed tomography scan showing scalp and intracranial mass.
[1,4] of the cerebrum and cerebellum are all well documented. Beyond this viral and fungal encephalitis as well as infectious meningitis show an increased incidence in patients with Hodgkin's disease [3,11]. Moreover, the clinical course of the disease can be complicated by the spread of lymphomatous cells through the CSF and meninges. It is also possible for the treatment of Hodgkin's disease to produce such neurological sequelae as radiation necrosis of the brain or spinal cord. Metastasis of Hodgkin's disease to the intracranial compartment is uncommon. Dujovny et al categorized intracranial Hodgkin's disease into four morphologic types [5]. In the most common of these there is metastasis to the dura mater, with subsequent compression or infiltration of the brain. Only about 10 cases of isolated, histologically verified intracerebral Hodgkin's disease have been reported. Spreading of the disease from bones of the cranial vault to the dura mater and brain has also been reported, but is likewise exceedingly rare [6,10]. Furthermore, even when intracranial involvement does occur, it can be asymptomatic and discovered only at autopsy [8,10]. Radiographic evidence of involvement of the bony cortex by Hodgkin's disease occurs in some 15% of cases [7]. However, the true incidence of bony involvement by the disease is difficult to document, and de-
Surg Neurol 1984;21:182-4
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pends on the clinical criteria used in making the diagnosis; thus, an additional 10% of bone lesions not revealed radiographically can be discovered at autopsy [15]. Calvarial lesions occur in only 5% of patients with skeletal metastases [7]. Scalp, calvarial, and intracranial lesions are caused by the hematogenous dissemination of Hodgkin's disease. Naeim et al noted a 75% incidence of vascular invasion in the lymphocyte-depleted subtype of the disease (lymphosarcoma in the older terminology) [5], and one-third of cases of the mixed-cellularity and 13 % of the nodular sclerosing subtypes (which were grouped together as lymphogranuloma in the older literature) showed this feature. No vascular invasion was noted in the lymphocyte-predominant subtype. Scheithauer [6] found that hematogenous spread of the disease to extracranial organs is common when intracranial metastases are present. This was true in our patient who developed pulmonary involvement. Scheithauer has provided the only other report of histologically verified intracranial Hodgkin's disease of the nodular sclerosing subtype. We are unaware of any reports of lymphocyte-predominant Hodgkin's disease (paragranuloma) presenting with either scalp or intracranial metastasis. Although the hematogenous spread of Hodgkin's disease is relatively common, particularly in patients with more aggressive histologic subtypes of the disease, our case is unusual in several respects. First, an enlarging scalp mass has not previously been reported as a presentational manifestation of primary or recurrent Hodgkin's disease. Second, the nodular sclerosing subtype of the disease is only rarely the source of intracranial metastasis, and cranial involvement is similarly uncommon. The fact that the foci of scalp, skull, and epidural involvement were all contiguous suggests that they originated from a single hematogenous metastatis (perhaps to the skull). Third, and most unusual, was the temporal and topographic relationship of this patient's tumor to the focal blunt trauma to the head that he had previously experienced. This case represents another example of a possible relationship between trauma and the development of tumors involving the brain and its coverings. However, as in most cases involving other types of neoplasia, the relationship between trauma and tumor development remains obscure.
References 1. Bruin L, Wilkinson M. Subacute cerebellar degeneration associated with neoplasm. Brain 1965;88:465-78. 2. Cravioto H, Feigin I. Non-infectious granulomatous angiitis with a predilection for the nervous system. Neurol (Minneap~ 1959;9:599-608. 3. Diamond H. Hodgkin's disease, neurologic sequelae. Missouri Med 1957;53:945-56.
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Surg N e u r o l 1984;21:182-4
Figure 2. A frozen section of the epidural mass reveals the polymorphous infiltrate typical of Hodgkin's disease. A binucleated Reed-Sternberg cell is in the center of the illustration. (H & E; x 800).
4. Dolman G, Cairns A. Leukoencephalopathy associated with Hodgkin's disease. Neurology 1961;11:349-53. 5. Dujovny M, McBride D, Segal R. Intracranial manifestations of Hodgkin's disease. Surg Neurol 1980;13:258-65. 6. Fein S, Newell V. Cerebral Hodgkin's disease: case report of Hodgkin's granuloma with cerebral invasion. Am J Med 1954;17:291. 7. Fucilla I, Hamann A. Hodgkin's disease in bone. Radiology 196;77:53-9. 8. Goldman L. Hodgkin's disease; an analysis of 212 cases. JAMA 1940;114:1611-6.
Tornaszek et al
9. Griffin J, Thompson R, Mitchinson M, Kiewiet J, Welland, F. Lymphomatous leptomeningitis. Am J Med 1971;51:200-8. 10. Jackson H, Parker F Jr. Hodgkin's disease lI. Pathology. N Engl J Med 1944;231:35-44. 11. Jackson M, Parker F, Jr. Hodgkin's disease V. Involvment of certain other organs. N Engl J Med 1945;233:369-474. 12. Naeim E, Waisman J, Coulson W. Hodgkin's disease. The significance of vascular invasion. Cancer 1955;8:629-33. 13. Scheithauer B. Cerebral metastasis in Hodgkin's disease, Arch Pathol Lab Med 1979;103:284-7. 14. Todd I. Intracranial lesions in Hodgkin's disease. Proc Psychiatr Soc Med 1967;60:734-5. 15. Uehlinger E. User Knocken--lymphogranulomatose. Virchows Arch Pathol Anat 1933;288:36-118. 16. Williams H, Diamond H, Craver L, Pausars H. Neurological complications of lymphomas and leukemias. Springfield, Ill: Charles C Thomas, 1959;44-72.
Surg Neurol 1984;21:182-4
Contiguous Scalp, Skull, and Epidural Hodgkin's Disease David E. Tomaszek, M.D., George W. Tyson, M.D., Paul Stang, B.S., P.A., and Thomas Bouldin, M.D. Departments of Neurological Surgery and Pathology, University of North Carolina School of Medicine, Chapel Hill, North Carolina
Tomaszek DE, Tyson GW, Stang P, Bouldin T. Contiguous scalp, skull, and epidural Hodgkin's disease. Surg Neurol 1984;21:182-4. A patient with Hodgkin's disease of the nodular sclerosing subtype presented with recurrent disease at the site of a previous blunt trauma to the head. The tumor involved the scalp and subjacent skull and epidural space. This particular presentation of Hodgkin's disease is probably unique, and involvement of the skull and cranial cavity by this subtype of Hodgkin's disease is rare. KEYWORDS: Hodgkin's disease; Vascular invasion; Metastasis; Cranial involvement; Scalp; Skull; Epidural
Intracranial involvement by histologically verified Hodgkin's disease is rare. Recent reports cite an incidence of 0.5 % or less [ 14]. We report a case of recurrent nodular sclerosing Hodgkin's disease that presented in the form o f contiguous intracranial and extracranial tumors at the site o f a minor closed-head injury.
Case Report A 23-year-old male presented with a mass in the scalp in the right parietal area. One month earlier he had suffered a minor blow to the head in the same region, and was left with slight residual local pain. Several days later a mass in the scalp appeared at the site of the injury; it was initially diagnosed as a subgaleal hematoma, but neurosurgical consultation was sought because of a progressive increase in the size of the mass. One year prior to this admission, the patient had been diagnosed as having nodular sclerosing Hodgkin's disease. H e was treated with thoracic and abdominal irradiation as well as with methotrexate, vincristine, proAddress reprint requests to: David E. Tomaszek, M.D., Division of Neurological Surgery, 148 Burnett Womack CSB 229H, UNC at Chapel Hill, Chapel Hill NC 27514.
© 1984by ElsevierSciencePublishingCo., Inc.
carbazine, and prednisone (MOPP), and later with bleomycin, cyclohexyl chloromethyl nitrosourea (CCNU), adriamycin, and vinblastine (B-CAV). The scalp mass was the first sign of recurrence o f his disease (stage IV; Ann Arbor classification). The patient denied having fever, weight loss, night sweats, or any symptoms of neurological dysfunction. Physical examination revealed a well-developed white male with normal vital signs. Several large, firm, fixed, nontender lymph nodes were palpable in the right anterior cervical area, and a firm but nontender and not freely mobile mass measuring 6 × 8 × 4 cm was present in the right parietal area. The neurological examination was normal. A computed tomography scan (Figure 1) indicated that this latter mass involved the subgaleal as well as the epidural space. A biopsy of the scalp and epidural masses as well as of the intervening calvarium was performed. The superficial mass was found to lie beneath the pericranium. Microscopic examination (Figure 2) of the biopsy specimens revealed a pleomorphic cellular infiltrate interlaced by fibrous bands. R e e d - S t e r n b e r g cells were plentiful and the histologic pattern was characteristic of Hodgkin's disease of the nodular sclerosing subtype. The patient developed pleural seeding, disseminated herpes zoster, and severe bone-marrow depression within months of the biopsy procedure. H e died 2 years after the initial diagnosis, without ever developing any focal signs of cerebral dysfunction.
Discussion Hodgkin's disease characteristically affects multiple organ systems. Although neurological complications o f the disease are not uncommon (15% of cases show some neurological dysfunction), most are secondary to either compression of the spinal cord by epidural deposits or to peripheral nerve palsies [3,5,16]. Additionally, the remote manifestations of the disease can produce neurological signs and symptoms, among which granulomatous angiitis [2], herpes zoster [3], and demyelination 0090-3019/84/$3.00
Hodgkin's Disease of Scalp and Skull
Figure 1. Computed tomography scan showing scalp and intracranial mass.
[1,4] of the cerebrum and cerebellum are all well documented. Beyond this viral and fungal encephalitis as well as infectious meningitis show an increased incidence in patients with Hodgkin's disease [3,11]. Moreover, the clinical course of the disease can be complicated by the spread of lymphomatous cells through the CSF and meninges. It is also possible for the treatment of Hodgkin's disease to produce such neurological sequelae as radiation necrosis of the brain or spinal cord. Metastasis of Hodgkin's disease to the intracranial compartment is uncommon. Dujovny et al categorized intracranial Hodgkin's disease into four morphologic types [5]. In the most common of these there is metastasis to the dura mater, with subsequent compression or infiltration of the brain. Only about 10 cases of isolated, histologically verified intracerebral Hodgkin's disease have been reported. Spreading of the disease from bones of the cranial vault to the dura mater and brain has also been reported, but is likewise exceedingly rare [6,10]. Furthermore, even when intracranial involvement does occur, it can be asymptomatic and discovered only at autopsy [8,10]. Radiographic evidence of involvement of the bony cortex by Hodgkin's disease occurs in some 15% of cases [7]. However, the true incidence of bony involvement by the disease is difficult to document, and de-
Surg Neurol 1984;21:182-4
183
pends on the clinical criteria used in making the diagnosis; thus, an additional 10% of bone lesions not revealed radiographically can be discovered at autopsy [15]. Calvarial lesions occur in only 5% of patients with skeletal metastases [7]. Scalp, calvarial, and intracranial lesions are caused by the hematogenous dissemination of Hodgkin's disease. Naeim et al noted a 75% incidence of vascular invasion in the lymphocyte-depleted subtype of the disease (lymphosarcoma in the older terminology) [5], and one-third of cases of the mixed-cellularity and 13 % of the nodular sclerosing subtypes (which were grouped together as lymphogranuloma in the older literature) showed this feature. No vascular invasion was noted in the lymphocyte-predominant subtype. Scheithauer [6] found that hematogenous spread of the disease to extracranial organs is common when intracranial metastases are present. This was true in our patient who developed pulmonary involvement. Scheithauer has provided the only other report of histologically verified intracranial Hodgkin's disease of the nodular sclerosing subtype. We are unaware of any reports of lymphocyte-predominant Hodgkin's disease (paragranuloma) presenting with either scalp or intracranial metastasis. Although the hematogenous spread of Hodgkin's disease is relatively common, particularly in patients with more aggressive histologic subtypes of the disease, our case is unusual in several respects. First, an enlarging scalp mass has not previously been reported as a presentational manifestation of primary or recurrent Hodgkin's disease. Second, the nodular sclerosing subtype of the disease is only rarely the source of intracranial metastasis, and cranial involvement is similarly uncommon. The fact that the foci of scalp, skull, and epidural involvement were all contiguous suggests that they originated from a single hematogenous metastatis (perhaps to the skull). Third, and most unusual, was the temporal and topographic relationship of this patient's tumor to the focal blunt trauma to the head that he had previously experienced. This case represents another example of a possible relationship between trauma and the development of tumors involving the brain and its coverings. However, as in most cases involving other types of neoplasia, the relationship between trauma and tumor development remains obscure.
References 1. Bruin L, Wilkinson M. Subacute cerebellar degeneration associated with neoplasm. Brain 1965;88:465-78. 2. Cravioto H, Feigin I. Non-infectious granulomatous angiitis with a predilection for the nervous system. Neurol (Minneap~ 1959;9:599-608. 3. Diamond H. Hodgkin's disease, neurologic sequelae. Missouri Med 1957;53:945-56.
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Surg N e u r o l 1984;21:182-4
Figure 2. A frozen section of the epidural mass reveals the polymorphous infiltrate typical of Hodgkin's disease. A binucleated Reed-Sternberg cell is in the center of the illustration. (H & E; x 800).
4. Dolman G, Cairns A. Leukoencephalopathy associated with Hodgkin's disease. Neurology 1961;11:349-53. 5. Dujovny M, McBride D, Segal R. Intracranial manifestations of Hodgkin's disease. Surg Neurol 1980;13:258-65. 6. Fein S, Newell V. Cerebral Hodgkin's disease: case report of Hodgkin's granuloma with cerebral invasion. Am J Med 1954;17:291. 7. Fucilla I, Hamann A. Hodgkin's disease in bone. Radiology 196;77:53-9. 8. Goldman L. Hodgkin's disease; an analysis of 212 cases. JAMA 1940;114:1611-6.
Tornaszek et al
9. Griffin J, Thompson R, Mitchinson M, Kiewiet J, Welland, F. Lymphomatous leptomeningitis. Am J Med 1971;51:200-8. 10. Jackson H, Parker F Jr. Hodgkin's disease lI. Pathology. N Engl J Med 1944;231:35-44. 11. Jackson M, Parker F, Jr. Hodgkin's disease V. Involvment of certain other organs. N Engl J Med 1945;233:369-474. 12. Naeim E, Waisman J, Coulson W. Hodgkin's disease. The significance of vascular invasion. Cancer 1955;8:629-33. 13. Scheithauer B. Cerebral metastasis in Hodgkin's disease, Arch Pathol Lab Med 1979;103:284-7. 14. Todd I. Intracranial lesions in Hodgkin's disease. Proc Psychiatr Soc Med 1967;60:734-5. 15. Uehlinger E. User Knocken--lymphogranulomatose. Virchows Arch Pathol Anat 1933;288:36-118. 16. Williams H, Diamond H, Craver L, Pausars H. Neurological complications of lymphomas and leukemias. Springfield, Ill: Charles C Thomas, 1959;44-72.