Surg Neurol 1992;38:273-6
273
Meningioma in a Neonate: Case Report Hirohito Niida, M.D., Ryuichi Tanaka, M.D., Norio Takeda, M.D., Shigekazu Takeuchi, M.D., Kaoru Hinokuma, M.D., and Hitoshi Takahashi, M.D. Departments of Neurosurgery and Pathology, Brain Research Institute, Niigata University, Niigata, Japan
Niida H, Tanaka R, Takeda N, Takeuchi S, Hinokuma K, Takahashi H. Meningioma in a neonate: case report. Surg Neurol 1992;38:273-6.
A 10-day-old female with a parasagittal meningioma presenting as a subcutaneous tumor is reported. Meningiomas within the 1st month of life are rare. The clinical and pathological characteristics of congenital meningioma are reviewed. Meningioma; Congenital tumor; Extracranial meningioma; Neonate KEY WORDS:
Intracranial meningiomas that occur within the 1st month of life are rare. Solitare and Krigman [14] defined congenital tumors as tumors that present or produce symptoms within the 1st month of life. Only seven cases (excluding hemangiopericytoma) have been previously reported in the literature [1,3,5-7,11,15]. We report the case of a meningioma in a neonate that presented as a subcutaneous tumor in the midfrontal region 10 days after birth--a unique report of a congenital meningioma invading bone and extending subcutaneously.
Case R e p o r t A female infant was born on December 16, 1989, after a full-term pregnancy without complications. Her body weight was 3295 g, and her head circumference was 33 cm at birth. Ten days after birth, a small subcutaneous tumor was noted in the midfrontal region; it enlarged rapidly. On January 24, 1990, she was referred to the Department of Neurosurgery, Niigata University Hospital. The size of the subcutaneous tumor was 2.5 × 2.2 x 0.8 cm. On February 26, she was admitted to Niigata University Hospital for operation. Her body weight was 5800 g, and her head circumference was 38
Address reprint requests to: Hirohito Niida, M.D., Department of Neurosurgery, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata, 951, Japan. Received April 3, 1992; accepted June 1, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
cm. She presented no neurological signs and was in good general condition. Systemic examination revealed no abnormality. Routine laboratory examinations were within normallimits. An elastic hard 4 x 3 x 1-cm subcutaneous tumor was present behind the anterior fontanel; it was slightly tense. Plain skull films showed an osteolytic lesion with a slightly sclerotic border in the frontal region. Coronal CT demonstrated a large, contrast-enhanced extraaxial mass invading the parietal bone and extending subcutaneously (Figure 1). The parietal bone appeared to be completely destroyed, with a slightly osteoblastic edge. Tl-weighted magnetic resonance images showed a mass isointense to gray matter, with homogeneous enhancement after intravenous administration of gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA) in the frontal region (Figure 2). The superior sagittal sinus was not visualized under the tumor. Bilatet~alcommon carotid angiograms demonstrated a highly vascular tumor fed by both middle meningeal arteries (Figure 3). An operation was performed on March 8. A coronal incision was made over both parietotemporal regions. After retraction of the Skin flap, an elastic hard tumor was noticed. The parietal bone was completely destroyed, with the raised outer table at the border. The superior sagittal sinus was completely occluded. Since the tumor did not adhere to the arachnoid membrane, it was easily separated from the surrounding brain tissue. Almost all of the tumor was extirpated, but the posterior portion involving a large rolandic vein could not be resected. The tumor tissue consisted of a large number of spindle-shaped fibroblastic cells with oval nuclei and a small number of those with small nucleoli (Figure 4). There were parallel arrangement of the cells and interlacing bundles. Neither whorls nor psammoma bodies were seen. A small number of mitotic figures and some highly cellular areas were noted. No foci of necrosis were observed. Immunohistochemical examination showed many vimentin-positive tumor cells. From these findings, the tumor was diagnosed as a fibroblastic meningioma with mild atypical features. The postoperative course was uneventful. She underwent local radiation therapy for the residual tumor. A 0090-3019/92/$5.00
274
Surg Neurol 1992;38:273-6
A
Niida et al
Figure 2. Postcontrast Tl-weighted magnetic resonance sagittal image revealing a homogeneously enhanced mass located in the frontal region, both intracranially and extracranially.
life and classified them into three categories: "definitely congenital" tumors, those present or producing symptoms at birth; "probably congenital" tumors, those present or producing symptoms within the 1st week o f life; and "possibly congenital" tumors, those present or producing symptoms within the 1st m o n t h o f life. Congenital meningiomas are extremely rare. Drake
Figure 3. Lateralviewoftheleftcommoncarotidangiogramdemonstrating a marked vascular sunburstlike stain fed by the middle meningeal artery.
B
Figure 1. (A) Postcontrast coronal CT scan showing an enhanced mass in the midfrontal area. (B) Bone algorithm showing complete osteolysis of the parietal bone with some osteoblastic change at its border. 10-meV electron irradiation was given at the rate o f 9.0 G y / w e e k in five fractions, up to a total dose of 50.4 Gy. She was discharged without neurological deficit on May 9. She has been doing well without evidence o f tumor regrowth or brain atrophy on magnetic resonance images 2 years after the surgery (Figure 5). Discussion Solitare and Krigman [ 14] reviewed 4 5 cases o f neonatal brain tumors that developed within the 1st month of
Neonatal Meningioma
Surg Neurol 1992;38:273-6
275
F i g u r e 4. Meningioma consisting of spindle-shapedfibroblastic cells. (He-
matoxylin & eosin stain, original magnification × 270.)
and H o f f m a n [4] reported 13 meningiomas out o f 1283 tumors in children less than 19 years o f age. N o n e of these cases occurred within the 1st year o f life. Raimondi and Tomita [13] reported 39 patients less than 1 year old among 341 children with intracranial tumors, but no meningiomas were reported. Matson [10] reported three meningiomas in children under 14 years o f age among 750 intracranial tumors. T h e r e was no case o f meningioma in a child under 1 year o f age. Buetow [2] reported one meningioma out o f 45 intracranial tumors diagnosed within 2 months o f birth. Only seven meningioma cases within the 1st month o f life have previously been reported in the literature. We have reviewed the clinical and pathological findings o f the eight congenital meningiomas, including our case (Table 1). Four cases are male, and four are female. Four cases are "definitely congenital," presenting with signs o f increased intracranial pressure or with enlargement of the head at birth. T h r e e cases are "probably congenital," presenting with vomiting, exophthalmos, or seizures. Only our case is "possibly congenital." Tumors were located mainly in the supratentorial region. Dural attach-
F i g u r e 5. Postcontrast Tl-weighted magnetic resonance sagittal image 2 years after surgery showed no evidence of tumor re~rowth or brain atrophy.
ment and cyst formation were noticed in six and two o f the cases, respectively. Histologically, five were fibroblastic, two were angioblastic, and one was angiomatous. T h e r e was no case of meningothelial meningioma, which is c o m m o n in adults. In our case, a parasagittal meningioma invaded the parietal bone and formed a subcutaneous tumor. T h e r e has been no case of a congenital meningioma presenting as a subcutaneous tumor in the literature; Meningioma with extracranial extension is uncommon. In our neurosurgical department, only five cases o f 192 meningiomas in the past 12 years extended extracranially. Some meningiomas seem to extend through bone at places o f decreased resistance such as sutures and foramina [8]. Because the parasagittal meningioma was located behind the anterior fontanel in our case, it appeared to extend through the sagittal suture.
Table 1. Summary of Eight ReportedCases of Congenital Meningioma • Source
Sex
Symptom
Onset
Location
Cuneo & Rand [3] Florin & Reid [7] Fessard [6] Mendiratta et al [ 11] Sosnik & Wrzesczynski [ 15] Endo & Aihara [5] Benli et al [1] Present case
F M M M F F M F
Vomiting, rigidity Enlargement of head Enlargement of head Seizure Enlargement of head Vomiting Exophthalmos Subcutaneous tumor
At birth At birth At birth 7 d At birth 3 d 5 d 10 d
Convexity Tentorial Convexity Convexity Basofrontal Convexity Tentorial Parasagittal
Abbreviations: F, female; M, male.
Dural attachment + + Unknown + + + +
Cyst + + -
Histology Angioblastic Angioblastic Fibroblastic Fibroblastic Fibroblastic Fibroblastic Angiomatous Fibroblastic
276
Surg N e u r o l 1992;38:273-6
It is generally accepted that meningiomas in children tend to grow rapidly in spite of benign histological findings. In our case, the subcutaneous tumor grew rapidly, which indicated a possible biologically malignant character. Postsurgical radiation therapy was performed because a small amount of tumor remained. No tumor recurrence has been noticed 2 years after surgery. Although radiation therapy is an effective treatment for tumor control, cranial radiotherapy to young children has not been established because it may cause damage to immature brain [12,16]. In this case, we considered that brain damage would be minimal because of its localized and superficial field. However, when the tumor is located in the deep region, radiation dose should be reduced to avoid cerebral damage. There is general agreement that complete surgical resection is the therapeutic procedure of choice in cases with meningioma. If a radical resection cannot be performed in infantile meningioma, additional radiation therapy should be recommended because of the possibility of rapid growth [9].
References 1. Benli K, Cataltepe O, Oge HK, Onol B. Giant congenital meningioma in a newborn. Childs Nerv Syst 1990;6:462-4. 2. Buetow PC, Smirniotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. AJNR 1989;11:793-9.
Niida et al
3. Cuneo HM, Rand CW. Brain tumors in childhood. Springfield, Ill.: CC Thomas, 1952:116-25. 4. Drake JM, Hoffman HJ. Meningiomas in children. In: AI-Mefty O, ed. Meningiomas. New York: Raven Press, 1991:145-52. 5. Endo S, Aihara H. Intracranial meningioma of the newborn: a case report. No To Hattatsu 1978;10:248-51 (in Japanese). 6. Fessard C. Les tumeurs c6r6brales des 2 premieres ann6es de la vie (66 observation anatomocliniques). Ann Pediatr 1966;13:289-302. 7. Florin RE, Reid ND. Congenital angioblastic meningioma: review of literature and report of case. Bull Los Angeles Neurol Soc 1961 ;26:51-6. 8. Lopez DA, Silver DN, Helwig EB. Cutaneous meningiomas: a clinicopathological study. Cancer 1974;34:728-44. 9. Lopez MJ, Olivares JL, Ramos F, Redondo JA, Cajal SR, Banzo J, Legido A. Giant meningioma in a 5-month-old infant. Childs Nerv Syst 1988;4:112-5. 10. Matson DD. Neurosurgery of infancy and childhood, 2nd ed. Springfield, Ill.: CC Thomas, 1969:624-31. 11. Mendiratta SS, Rosenblum JA, Strobos RJ. Congenital meningioma. Neurology 1967;17:914-8. 12. Oi S, Kokunai T, Ijichi A, Matsumoto S, Raimondi AJ. Radiationinduced brain damage in children. Neurol Med Chir (Tokyo) 1990;30:36-42. 13. Raimondi AJ, Tomita T. Brain tumors during the first year of life. Childs Brain 1983;10:193-207. 14. Solitare GB, Krigman MR. Congenital intracranial neoplasm: a case report and review of the literature. Neuropathol Exp Neurol 1964;23:280-92. 15. Sosnik H, Wrzesczynski K. Wrodzony oponiak przedniego dolu czaszkowego. Patol Pol 1972;23:503-7. 16. SpunbergJJ, Chang CH, Goldman M, Auricchio E, BellJJ. Quality of long-term survival following irradiation for intracranial tumors in children under the age of two. Int J Radiat Oncol Biol Phys 1981;7:727-36.
273
Meningioma in a Neonate: Case Report Hirohito Niida, M.D., Ryuichi Tanaka, M.D., Norio Takeda, M.D., Shigekazu Takeuchi, M.D., Kaoru Hinokuma, M.D., and Hitoshi Takahashi, M.D. Departments of Neurosurgery and Pathology, Brain Research Institute, Niigata University, Niigata, Japan
Niida H, Tanaka R, Takeda N, Takeuchi S, Hinokuma K, Takahashi H. Meningioma in a neonate: case report. Surg Neurol 1992;38:273-6.
A 10-day-old female with a parasagittal meningioma presenting as a subcutaneous tumor is reported. Meningiomas within the 1st month of life are rare. The clinical and pathological characteristics of congenital meningioma are reviewed. Meningioma; Congenital tumor; Extracranial meningioma; Neonate KEY WORDS:
Intracranial meningiomas that occur within the 1st month of life are rare. Solitare and Krigman [14] defined congenital tumors as tumors that present or produce symptoms within the 1st month of life. Only seven cases (excluding hemangiopericytoma) have been previously reported in the literature [1,3,5-7,11,15]. We report the case of a meningioma in a neonate that presented as a subcutaneous tumor in the midfrontal region 10 days after birth--a unique report of a congenital meningioma invading bone and extending subcutaneously.
Case R e p o r t A female infant was born on December 16, 1989, after a full-term pregnancy without complications. Her body weight was 3295 g, and her head circumference was 33 cm at birth. Ten days after birth, a small subcutaneous tumor was noted in the midfrontal region; it enlarged rapidly. On January 24, 1990, she was referred to the Department of Neurosurgery, Niigata University Hospital. The size of the subcutaneous tumor was 2.5 × 2.2 x 0.8 cm. On February 26, she was admitted to Niigata University Hospital for operation. Her body weight was 5800 g, and her head circumference was 38
Address reprint requests to: Hirohito Niida, M.D., Department of Neurosurgery, Brain Research Institute, Niigata University, 1 Asahimachi, Niigata, 951, Japan. Received April 3, 1992; accepted June 1, 1992.
© 1992 by Elsevier Science Publishing Co., Inc.
cm. She presented no neurological signs and was in good general condition. Systemic examination revealed no abnormality. Routine laboratory examinations were within normallimits. An elastic hard 4 x 3 x 1-cm subcutaneous tumor was present behind the anterior fontanel; it was slightly tense. Plain skull films showed an osteolytic lesion with a slightly sclerotic border in the frontal region. Coronal CT demonstrated a large, contrast-enhanced extraaxial mass invading the parietal bone and extending subcutaneously (Figure 1). The parietal bone appeared to be completely destroyed, with a slightly osteoblastic edge. Tl-weighted magnetic resonance images showed a mass isointense to gray matter, with homogeneous enhancement after intravenous administration of gadolinium diethylenetriaminepentaacetic acid (Gd-DTPA) in the frontal region (Figure 2). The superior sagittal sinus was not visualized under the tumor. Bilatet~alcommon carotid angiograms demonstrated a highly vascular tumor fed by both middle meningeal arteries (Figure 3). An operation was performed on March 8. A coronal incision was made over both parietotemporal regions. After retraction of the Skin flap, an elastic hard tumor was noticed. The parietal bone was completely destroyed, with the raised outer table at the border. The superior sagittal sinus was completely occluded. Since the tumor did not adhere to the arachnoid membrane, it was easily separated from the surrounding brain tissue. Almost all of the tumor was extirpated, but the posterior portion involving a large rolandic vein could not be resected. The tumor tissue consisted of a large number of spindle-shaped fibroblastic cells with oval nuclei and a small number of those with small nucleoli (Figure 4). There were parallel arrangement of the cells and interlacing bundles. Neither whorls nor psammoma bodies were seen. A small number of mitotic figures and some highly cellular areas were noted. No foci of necrosis were observed. Immunohistochemical examination showed many vimentin-positive tumor cells. From these findings, the tumor was diagnosed as a fibroblastic meningioma with mild atypical features. The postoperative course was uneventful. She underwent local radiation therapy for the residual tumor. A 0090-3019/92/$5.00
274
Surg Neurol 1992;38:273-6
A
Niida et al
Figure 2. Postcontrast Tl-weighted magnetic resonance sagittal image revealing a homogeneously enhanced mass located in the frontal region, both intracranially and extracranially.
life and classified them into three categories: "definitely congenital" tumors, those present or producing symptoms at birth; "probably congenital" tumors, those present or producing symptoms within the 1st week o f life; and "possibly congenital" tumors, those present or producing symptoms within the 1st m o n t h o f life. Congenital meningiomas are extremely rare. Drake
Figure 3. Lateralviewoftheleftcommoncarotidangiogramdemonstrating a marked vascular sunburstlike stain fed by the middle meningeal artery.
B
Figure 1. (A) Postcontrast coronal CT scan showing an enhanced mass in the midfrontal area. (B) Bone algorithm showing complete osteolysis of the parietal bone with some osteoblastic change at its border. 10-meV electron irradiation was given at the rate o f 9.0 G y / w e e k in five fractions, up to a total dose of 50.4 Gy. She was discharged without neurological deficit on May 9. She has been doing well without evidence o f tumor regrowth or brain atrophy on magnetic resonance images 2 years after the surgery (Figure 5). Discussion Solitare and Krigman [ 14] reviewed 4 5 cases o f neonatal brain tumors that developed within the 1st month of
Neonatal Meningioma
Surg Neurol 1992;38:273-6
275
F i g u r e 4. Meningioma consisting of spindle-shapedfibroblastic cells. (He-
matoxylin & eosin stain, original magnification × 270.)
and H o f f m a n [4] reported 13 meningiomas out o f 1283 tumors in children less than 19 years o f age. N o n e of these cases occurred within the 1st year o f life. Raimondi and Tomita [13] reported 39 patients less than 1 year old among 341 children with intracranial tumors, but no meningiomas were reported. Matson [10] reported three meningiomas in children under 14 years o f age among 750 intracranial tumors. T h e r e was no case o f meningioma in a child under 1 year o f age. Buetow [2] reported one meningioma out o f 45 intracranial tumors diagnosed within 2 months o f birth. Only seven meningioma cases within the 1st month o f life have previously been reported in the literature. We have reviewed the clinical and pathological findings o f the eight congenital meningiomas, including our case (Table 1). Four cases are male, and four are female. Four cases are "definitely congenital," presenting with signs o f increased intracranial pressure or with enlargement of the head at birth. T h r e e cases are "probably congenital," presenting with vomiting, exophthalmos, or seizures. Only our case is "possibly congenital." Tumors were located mainly in the supratentorial region. Dural attach-
F i g u r e 5. Postcontrast Tl-weighted magnetic resonance sagittal image 2 years after surgery showed no evidence of tumor re~rowth or brain atrophy.
ment and cyst formation were noticed in six and two o f the cases, respectively. Histologically, five were fibroblastic, two were angioblastic, and one was angiomatous. T h e r e was no case of meningothelial meningioma, which is c o m m o n in adults. In our case, a parasagittal meningioma invaded the parietal bone and formed a subcutaneous tumor. T h e r e has been no case of a congenital meningioma presenting as a subcutaneous tumor in the literature; Meningioma with extracranial extension is uncommon. In our neurosurgical department, only five cases o f 192 meningiomas in the past 12 years extended extracranially. Some meningiomas seem to extend through bone at places o f decreased resistance such as sutures and foramina [8]. Because the parasagittal meningioma was located behind the anterior fontanel in our case, it appeared to extend through the sagittal suture.
Table 1. Summary of Eight ReportedCases of Congenital Meningioma • Source
Sex
Symptom
Onset
Location
Cuneo & Rand [3] Florin & Reid [7] Fessard [6] Mendiratta et al [ 11] Sosnik & Wrzesczynski [ 15] Endo & Aihara [5] Benli et al [1] Present case
F M M M F F M F
Vomiting, rigidity Enlargement of head Enlargement of head Seizure Enlargement of head Vomiting Exophthalmos Subcutaneous tumor
At birth At birth At birth 7 d At birth 3 d 5 d 10 d
Convexity Tentorial Convexity Convexity Basofrontal Convexity Tentorial Parasagittal
Abbreviations: F, female; M, male.
Dural attachment + + Unknown + + + +
Cyst + + -
Histology Angioblastic Angioblastic Fibroblastic Fibroblastic Fibroblastic Fibroblastic Angiomatous Fibroblastic
276
Surg N e u r o l 1992;38:273-6
It is generally accepted that meningiomas in children tend to grow rapidly in spite of benign histological findings. In our case, the subcutaneous tumor grew rapidly, which indicated a possible biologically malignant character. Postsurgical radiation therapy was performed because a small amount of tumor remained. No tumor recurrence has been noticed 2 years after surgery. Although radiation therapy is an effective treatment for tumor control, cranial radiotherapy to young children has not been established because it may cause damage to immature brain [12,16]. In this case, we considered that brain damage would be minimal because of its localized and superficial field. However, when the tumor is located in the deep region, radiation dose should be reduced to avoid cerebral damage. There is general agreement that complete surgical resection is the therapeutic procedure of choice in cases with meningioma. If a radical resection cannot be performed in infantile meningioma, additional radiation therapy should be recommended because of the possibility of rapid growth [9].
References 1. Benli K, Cataltepe O, Oge HK, Onol B. Giant congenital meningioma in a newborn. Childs Nerv Syst 1990;6:462-4. 2. Buetow PC, Smirniotopoulos JG, Done S. Congenital brain tumors: a review of 45 cases. AJNR 1989;11:793-9.
Niida et al
3. Cuneo HM, Rand CW. Brain tumors in childhood. Springfield, Ill.: CC Thomas, 1952:116-25. 4. Drake JM, Hoffman HJ. Meningiomas in children. In: AI-Mefty O, ed. Meningiomas. New York: Raven Press, 1991:145-52. 5. Endo S, Aihara H. Intracranial meningioma of the newborn: a case report. No To Hattatsu 1978;10:248-51 (in Japanese). 6. Fessard C. Les tumeurs c6r6brales des 2 premieres ann6es de la vie (66 observation anatomocliniques). Ann Pediatr 1966;13:289-302. 7. Florin RE, Reid ND. Congenital angioblastic meningioma: review of literature and report of case. Bull Los Angeles Neurol Soc 1961 ;26:51-6. 8. Lopez DA, Silver DN, Helwig EB. Cutaneous meningiomas: a clinicopathological study. Cancer 1974;34:728-44. 9. Lopez MJ, Olivares JL, Ramos F, Redondo JA, Cajal SR, Banzo J, Legido A. Giant meningioma in a 5-month-old infant. Childs Nerv Syst 1988;4:112-5. 10. Matson DD. Neurosurgery of infancy and childhood, 2nd ed. Springfield, Ill.: CC Thomas, 1969:624-31. 11. Mendiratta SS, Rosenblum JA, Strobos RJ. Congenital meningioma. Neurology 1967;17:914-8. 12. Oi S, Kokunai T, Ijichi A, Matsumoto S, Raimondi AJ. Radiationinduced brain damage in children. Neurol Med Chir (Tokyo) 1990;30:36-42. 13. Raimondi AJ, Tomita T. Brain tumors during the first year of life. Childs Brain 1983;10:193-207. 14. Solitare GB, Krigman MR. Congenital intracranial neoplasm: a case report and review of the literature. Neuropathol Exp Neurol 1964;23:280-92. 15. Sosnik H, Wrzesczynski K. Wrodzony oponiak przedniego dolu czaszkowego. Patol Pol 1972;23:503-7. 16. SpunbergJJ, Chang CH, Goldman M, Auricchio E, BellJJ. Quality of long-term survival following irradiation for intracranial tumors in children under the age of two. Int J Radiat Oncol Biol Phys 1981;7:727-36.
