AUTHOR(S): Itoh, Hiroji, M.D.; Waga, Shiro, M.D.; Kojima, Tadashi, M.D.; Hoshino, Tamotsu, M.D. Department of Neurosurgery, Mie University School of Medicine and Hospital, Tsu, Mie, Japan Neurosurgery 30; 295-298, 1992 ABSTRACT: A rare case of a solitary eosinophilic granuloma in the brain is reported. The mass, located in the right frontal lobe, mimicked a glioma not only grossly, but also by neuroimaging. The lesion was confirmed to be an eosinophilic granuloma by electron microscopy and immunohistochemical staining for S-100 protein and HLA-DR.
preoperative diagnosis was a low-grade astrocytoma. Operation A right parietal craniotomy was performed. The dura appeared entirely normal, and the brain surface was edematous. After an ultrasonography-guided corticotomy, a well-demarcated, firm, grayish tumor was identified and removed totally. Postoperative course After a tentative pathological diagnosis of an eosinophilic granuloma, radiation therapy (20 Gy) was given to the local area of the brain. Bone scintigraphy was negative for the entire skeletal system. The patient's seizure activity has been well controlled by the administration of anticonvulsants, and no recurrence of the lesion has developed 2 years after the operation.
INTRODUCTION Central nervous system (CNS) involvement of histiocytosis X is unusual, and, to our knowledge, a solitary eosinophilic granuloma in the cerebral hemisphere has been reported only occasionally (1,3,6, 7,12-14) . Computed tomography (CT) scans of solitary eosinophilic granulomas show a low-density mass with surrounding edema and marked enhancement (3, 6,7,12,14) . Magnetic resonance imaging (MRI) reveals a lesion with low intensity or isointensity on T1weighted images and high intensity on T2-weighted images (13). We report a case of a solitary eosinophilic granuloma in the frontal lobe, review the CT and MRI findings of previous cases, and add some new findings with respect to neuroimaging.
Histological examination Paraffin-embedded materials were fixed in 10% formalin, and sections were stained with hematoxylin and eosin and Ziehl-Neelsen and avidin-biotin complex immunohistochemical methods for S-100 protein (DAKO, Copenhagen, Denmark), HLA-DR (Nichirei, Tokyo, Japan), and lysozyme (DAKO). Another surgical specimen was used for electron microscopic study. Staining with hematoxylin and eosin revealed various types of inflammatory cells such as histiocytes, eosinophils, lymphocytes, and plasma cells (Fig. 3, upper left). The results of the ZiehlNeelsen staining were negative. Immunohistochemical staining showed nuclear and cytoplasmic positivity for S-100 protein (Fig. 3, upper right) and cytoplasmic positivity for HLA-DR in the histiocytes (Fig. 3, lower left). On the other hand, staining for lysozyme was negative in these cells (Fig. 3, lower right). Electron microscopy demonstrated trilaminar intracytoplasmic Birbeck granules in the histiocytes (Fig. 4).
CASE REPORT A 7-year-old boy with a history of generalized convulsions was admitted to our hospital. A CT scan taken at another hospital had demonstrated a right frontal mass. There was no past history of diabetes insipidus, exophthalmos, otitis media, or lymphadenopathy. A physical examination revealed a widespread atopic dermatitis. Laboratory data revealed eosinophilia (6.1%). A neurological examination demonstrated only left hemiparesis. A CT scan without contrast enhancement revealed a small high-density area with a large zone of surrounding edema in the right frontal lobe, and a contrast-enhanced CT scan demonstrated a homogeneous lesion, larger than the high-density area (Fig. 1). MRI showed the mass to be a slightly low-intensity or isointense lesion on T1-weighted images and a high-intensity one on T2-weighted images with homogeneous enhancement after the administration of gadolinium diethylene-triaminepentaacetic acid (Gd-DTPA) (Fig. 2). The tentative
DISCUSSION Histiocytosis X is a disease complex that includes Letterer-Siwe disease, Hand-Schüller-Christian disease, and eosinophilic granulomas. It is a nonneoplastic chronic disease of a granulomatous nature and unknown cause. Bone is the tissue affected most frequently, although the skin, liver, spleen, and reticuloendothelial system also can be involved. CNS involvement is unusual and frequently associated with bone lesions (2); typically, it occurs late in the course of the disease (2,6,14). CNS lesions are classified into two forms: cerebral and meningeal (10). The Gagel hypothalamic granuloma is a well-known cerebral form (11), but, to our knowledge, a solitary eosinophilic granuloma in the cerebral hemisphere has been reported in only eight patients including ours (1,3,6,7,12-14); only four of these were of the cerebral type (1,13,14). In these cases, CT scans showed a low-density mass, except for one which was isodense, with surrounding edema and markedly homogeneous
KEY WORDS: Brain tumor; Eosinophilic granuloma; Histiocytosis X; Neuroimaging
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 February 1992, Volume 30, Number 2 295 Solitary Eosinophilic Granuloma in the Frontal Lobe: Case Report Case Report
ACKNOWLEDGMENT The authors thank Miss T. Sugiura for preparing the pathological specimens. Received for publication, May 30, 1991; accepted, July 16, 1991. Hiroji Itoh, M.D., Department of Neurosurgery, Mie University School of Medicine and Hospital, 2174 Edobashi, Tsu, Mie, Japan 514. REFERENCES: (1-14) 1.
2.
3.
4.
5.
Cerdá-Nicolas M, Broseta J, Peydrò-Olaya A, Barberá J, Barcia-Salorio JL, Llombart-Bosch A: Primary eosinophilic granuloma of the frontal lobe. Virchows Arch [A] 388:221-228, 1980. Elian M, Bornstein B, Matz S, Askenasy HM, Sandbank U: Neurological manifestations of general xanthomatosis. Arch Neurol 21:115120, 1969. Eriksen B, Janinis J, Variakojis D, Winter J, Russel E, Marder R, DalCanto MC: Primary histiocytosis X of the parieto-occipital lobe. Hum Pathol 19:611-614, 1988. Goldberg R, Han JS, Ganz E, Roessman U: Computed tomography demonstration of multiple parenchymal central nervous system nodules due to histiocytosis X. Surg Neurol 27:377-380, 1987. Graif M, Pennock JM: MR imaging of histiocytosis X in the central nervous system.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
enhancement (3,4,6,7,12-14). In our patient, however, CT scans revealed a small high-density area in the center of the mass, which we think is a unique feature. MRI of histiocytosis X in CNS has been reported rarely. According to Graif and Pennock (5) and Penar et al. (13) , the lesions showed low intensity or isointensity on T1-weighted images and high intensity on T2weighted images, but they were not enhanced by GdDTPA. In our patient, both T1- and T2-weighted images were the same as those reported previously, but the lesion showed marked homogeneous enhancement after the administration of Gd-DTPA. These CT and MRI findings may represent a wide range of abnormalities evident in the neuroimaging of eosinophilic granulomas involving the CNS. The pathological diagnosis was based on characteristic hematoxylin and eosin staining, immunohistochemical staining, and electron microscopy. Of these examinations, the best evidence of histiocytosis X is the ultrastructural demonstration of Birbeck granules in the proliferating histiocytes. Moreover, recent immunohistochemical studies have demonstrated a high degree of specificity for histiocytosis X cells; staining for S-100 protein shows strong nuclear and cytoplasmic positivity in paraffin sections (3,7-9,12,13), whereas staining for HLADR in paraffin sections and for OKT 6 in frozen sections shows strong cytoplasmic positivity in histiocytosis X cells (3,8). On the other hand, staining paraffin sections for lysozyme gave uniformly negative results in histiocytosis X cells.
Figure 2. Left, T1-weighted MRI shows a slightly low-intensity or isointense mass in the right frontal lobe. Right, the mass is homogeneously enhanced by Gd-DTPA.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 1. Left, CT scan without contrast enhancement shows a small high-density area with a large zone of surrounding edema in the right frontal lobe. Right, the lesion is enhanced homogeneously to a greater degree than the high-density area.
Figure 4. Left, electron micrograph shows a large cell with a filopoidal surface, abundant cytoplasm, and indented nucleus. Birbeck bodies, so-called tennis racket appearance (arrows), in the cytoplasm are characteristic (×16,000). Right, high-magnification view of a Birbeck body (×80,000).
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 3. Staining with hematoxylin and eosin reveals a number of different inflammatory cell types such as histiocytes, eosinophils, lymphocytes, and plasma cells (upper left). Immunohistochemical staining shows nuclear and cytoplasmic positivity for S-100 protein (upper right) and cytoplasmic positivity for HLA-DR in histiocytes (lower left). Staining for lysozyme is negative in these histiocytes (lower right) (all figures, ×200).
preoperative diagnosis was a low-grade astrocytoma. Operation A right parietal craniotomy was performed. The dura appeared entirely normal, and the brain surface was edematous. After an ultrasonography-guided corticotomy, a well-demarcated, firm, grayish tumor was identified and removed totally. Postoperative course After a tentative pathological diagnosis of an eosinophilic granuloma, radiation therapy (20 Gy) was given to the local area of the brain. Bone scintigraphy was negative for the entire skeletal system. The patient's seizure activity has been well controlled by the administration of anticonvulsants, and no recurrence of the lesion has developed 2 years after the operation.
INTRODUCTION Central nervous system (CNS) involvement of histiocytosis X is unusual, and, to our knowledge, a solitary eosinophilic granuloma in the cerebral hemisphere has been reported only occasionally (1,3,6, 7,12-14) . Computed tomography (CT) scans of solitary eosinophilic granulomas show a low-density mass with surrounding edema and marked enhancement (3, 6,7,12,14) . Magnetic resonance imaging (MRI) reveals a lesion with low intensity or isointensity on T1weighted images and high intensity on T2-weighted images (13). We report a case of a solitary eosinophilic granuloma in the frontal lobe, review the CT and MRI findings of previous cases, and add some new findings with respect to neuroimaging.
Histological examination Paraffin-embedded materials were fixed in 10% formalin, and sections were stained with hematoxylin and eosin and Ziehl-Neelsen and avidin-biotin complex immunohistochemical methods for S-100 protein (DAKO, Copenhagen, Denmark), HLA-DR (Nichirei, Tokyo, Japan), and lysozyme (DAKO). Another surgical specimen was used for electron microscopic study. Staining with hematoxylin and eosin revealed various types of inflammatory cells such as histiocytes, eosinophils, lymphocytes, and plasma cells (Fig. 3, upper left). The results of the ZiehlNeelsen staining were negative. Immunohistochemical staining showed nuclear and cytoplasmic positivity for S-100 protein (Fig. 3, upper right) and cytoplasmic positivity for HLA-DR in the histiocytes (Fig. 3, lower left). On the other hand, staining for lysozyme was negative in these cells (Fig. 3, lower right). Electron microscopy demonstrated trilaminar intracytoplasmic Birbeck granules in the histiocytes (Fig. 4).
CASE REPORT A 7-year-old boy with a history of generalized convulsions was admitted to our hospital. A CT scan taken at another hospital had demonstrated a right frontal mass. There was no past history of diabetes insipidus, exophthalmos, otitis media, or lymphadenopathy. A physical examination revealed a widespread atopic dermatitis. Laboratory data revealed eosinophilia (6.1%). A neurological examination demonstrated only left hemiparesis. A CT scan without contrast enhancement revealed a small high-density area with a large zone of surrounding edema in the right frontal lobe, and a contrast-enhanced CT scan demonstrated a homogeneous lesion, larger than the high-density area (Fig. 1). MRI showed the mass to be a slightly low-intensity or isointense lesion on T1-weighted images and a high-intensity one on T2-weighted images with homogeneous enhancement after the administration of gadolinium diethylene-triaminepentaacetic acid (Gd-DTPA) (Fig. 2). The tentative
DISCUSSION Histiocytosis X is a disease complex that includes Letterer-Siwe disease, Hand-Schüller-Christian disease, and eosinophilic granulomas. It is a nonneoplastic chronic disease of a granulomatous nature and unknown cause. Bone is the tissue affected most frequently, although the skin, liver, spleen, and reticuloendothelial system also can be involved. CNS involvement is unusual and frequently associated with bone lesions (2); typically, it occurs late in the course of the disease (2,6,14). CNS lesions are classified into two forms: cerebral and meningeal (10). The Gagel hypothalamic granuloma is a well-known cerebral form (11), but, to our knowledge, a solitary eosinophilic granuloma in the cerebral hemisphere has been reported in only eight patients including ours (1,3,6,7,12-14); only four of these were of the cerebral type (1,13,14). In these cases, CT scans showed a low-density mass, except for one which was isodense, with surrounding edema and markedly homogeneous
KEY WORDS: Brain tumor; Eosinophilic granuloma; Histiocytosis X; Neuroimaging
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Neurosurgery 1992-98 February 1992, Volume 30, Number 2 295 Solitary Eosinophilic Granuloma in the Frontal Lobe: Case Report Case Report
ACKNOWLEDGMENT The authors thank Miss T. Sugiura for preparing the pathological specimens. Received for publication, May 30, 1991; accepted, July 16, 1991. Hiroji Itoh, M.D., Department of Neurosurgery, Mie University School of Medicine and Hospital, 2174 Edobashi, Tsu, Mie, Japan 514. REFERENCES: (1-14) 1.
2.
3.
4.
5.
Cerdá-Nicolas M, Broseta J, Peydrò-Olaya A, Barberá J, Barcia-Salorio JL, Llombart-Bosch A: Primary eosinophilic granuloma of the frontal lobe. Virchows Arch [A] 388:221-228, 1980. Elian M, Bornstein B, Matz S, Askenasy HM, Sandbank U: Neurological manifestations of general xanthomatosis. Arch Neurol 21:115120, 1969. Eriksen B, Janinis J, Variakojis D, Winter J, Russel E, Marder R, DalCanto MC: Primary histiocytosis X of the parieto-occipital lobe. Hum Pathol 19:611-614, 1988. Goldberg R, Han JS, Ganz E, Roessman U: Computed tomography demonstration of multiple parenchymal central nervous system nodules due to histiocytosis X. Surg Neurol 27:377-380, 1987. Graif M, Pennock JM: MR imaging of histiocytosis X in the central nervous system.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
enhancement (3,4,6,7,12-14). In our patient, however, CT scans revealed a small high-density area in the center of the mass, which we think is a unique feature. MRI of histiocytosis X in CNS has been reported rarely. According to Graif and Pennock (5) and Penar et al. (13) , the lesions showed low intensity or isointensity on T1-weighted images and high intensity on T2weighted images, but they were not enhanced by GdDTPA. In our patient, both T1- and T2-weighted images were the same as those reported previously, but the lesion showed marked homogeneous enhancement after the administration of Gd-DTPA. These CT and MRI findings may represent a wide range of abnormalities evident in the neuroimaging of eosinophilic granulomas involving the CNS. The pathological diagnosis was based on characteristic hematoxylin and eosin staining, immunohistochemical staining, and electron microscopy. Of these examinations, the best evidence of histiocytosis X is the ultrastructural demonstration of Birbeck granules in the proliferating histiocytes. Moreover, recent immunohistochemical studies have demonstrated a high degree of specificity for histiocytosis X cells; staining for S-100 protein shows strong nuclear and cytoplasmic positivity in paraffin sections (3,7-9,12,13), whereas staining for HLADR in paraffin sections and for OKT 6 in frozen sections shows strong cytoplasmic positivity in histiocytosis X cells (3,8). On the other hand, staining paraffin sections for lysozyme gave uniformly negative results in histiocytosis X cells.
Figure 2. Left, T1-weighted MRI shows a slightly low-intensity or isointense mass in the right frontal lobe. Right, the mass is homogeneously enhanced by Gd-DTPA.
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 1. Left, CT scan without contrast enhancement shows a small high-density area with a large zone of surrounding edema in the right frontal lobe. Right, the lesion is enhanced homogeneously to a greater degree than the high-density area.
Figure 4. Left, electron micrograph shows a large cell with a filopoidal surface, abundant cytoplasm, and indented nucleus. Birbeck bodies, so-called tennis racket appearance (arrows), in the cytoplasm are characteristic (×16,000). Right, high-magnification view of a Birbeck body (×80,000).
Downloaded from https://academic.oup.com/neurosurgery/article-abstract/30/2/295/2753011 by UB Frankfurt/Main user on 05 March 2018
Redistribution of this article permitted only in accordance with the publisher’s copyright provisions.
Figure 3. Staining with hematoxylin and eosin reveals a number of different inflammatory cell types such as histiocytes, eosinophils, lymphocytes, and plasma cells (upper left). Immunohistochemical staining shows nuclear and cytoplasmic positivity for S-100 protein (upper right) and cytoplasmic positivity for HLA-DR in histiocytes (lower left). Staining for lysozyme is negative in these histiocytes (lower right) (all figures, ×200).
