Meningeal hemangiopericytoma manifesting as massive intracranial hemorrhage--two case reports

Meningeal hemangiopericytoma is rare, and only seven cases have manifested as intracranial hemorrhage. We treated two patients with meningeal hemangiopericytoma manifesting as life-threatening massive intracerebral hemorrhage. Case 1: A 66-year-old woman presented with consciousness disturbance and left hemiparesis. Computed tomography showed a mass lesion in the right frontal parasagittal region and massive hematoma in the brain tissue at the medial border of the lesion. Immediate evacuation of the hematoma was performed. Postoperative magnetic resonance imaging revealed a well-enhanced mass lesion with small intratumoral hemorrhage. The tumor was removed totally through a bilateral frontal craniotomy. The postoperative course was uneventful. The histological diagnosis was meningeal hemangiopericytoma. Radiotherapy was not performed. Case 2: A 59-year-old man presented with consciousness disturbance. Computed tomography showed a mass lesion in the right frontotemporal convexity region and massive hematoma in the brain tissue at the medial border of the lesion. His neurological condition was refractory to any treatment and the clinical diagnosis of brain death was confirmed. Autopsy was performed and the histological diagnosis was meningeal hemangiopericytoma. Meningeal hemangiopericytoma manifesting as intracranial hemorrhage is quite rare, but carries the risk of life-threatening massive bleeding from the tumor.     

Paraganglioma in the frontal skull base--case report.

A 56-year-old female presented with a paraganglioma in the left anterior cranial fossa who manifesting as persistent headache. Computed tomography and magnetic resonance imaging showed a solid, enhanced tumor with a cystic component located medially. The tumor was attached to the left frontal base and the sphenoid ridge. Angiography demonstrated a hypervascular tumor fed mainly by the left middle meningeal artery at the left sphenoid ridge. The preoperative diagnosis was meningioma of the left frontal base. The tumor was totally resected via a left frontotemporal craniotomy. Histological examination revealed the characteristic cellular arrangement of paraganglioma generally designated as the "Zellbaren pattern" on light microscopy. Only 10 patients with supratentorial paraganglioma have been reported, seven located in the parasellar area. The origin of the present tumor may have been the paraganglionic cells which strayed along the middle meningeal artery at differentiation.     

Meningeal hemangiopericytoma: histopathological features, treatment, and long-term follow-up of 44 cases

Forty-four cases of meningeal hemangiopericytoma that were treated between 1938 and 1987 are reviewed. Fifty-five percent of these tumors occurred in men. The average age of the patients at diagnosis was 42 years. The average duration of preoperative symptoms was 11 months. Symptoms were related to tumor location, which was similar to that of meningioma. The operative mortality was 9% overall, and has been zero since 1974 (18 patients). The average time before the first recurrence was 47 months, with the recurrence rates at 1, 5, and 10 years after surgery being 15, 65, and 76%, respectively. Ten patients have developed extraneural metastasis, mostly to lung and bone, at an average of 99 months after the first operation. The 10- and 15-year rates of metastasis were 33 and 64%, respectively. The average survival period has been 84 months, with survival rates at 5, 10, and 15 years after surgery of 67, 40, and 23%, respectively. The histological diagnosis of the tumor was not related to survival or recurrence and did not change with recurrence. Tentorial and posterior fossa tumors tended to be more lethal. Total tumor resection favorably affected recurrence and survival, as opposed to subtotal resection. Metastasis adversely affected survival, and was followed by death at an average of 24 months after its diagnosis. Radiation therapy after the first operation extended the average time before first recurrence from 34 to 75 months, and extended survival from 62 to 92 months.     

Meningeal hemangiopericytoma: a retrospective study of 21 patients with special review of postoperative external radiotherapy

OBJECTIVE: To specify that postoperative radiotherapy is useful for preventing local recurrence and neuraxis recurrence of surgically treated meningeal hemangiopericytomas. METHODS: We retrospectively studied 21 patients with meningeal hemangiopericytoma who were followed in our department during a 34-year period. In 17 patients, the meningeal hemangiopericytoma was intracranial, and in 4 there was an intradural extramedullary localization. These groups were studied separately. RESULTS: Of the 17 patients with intracranial hemangiopericytoma, all underwent surgery; 8 also underwent radiotherapy (5,000-6,400 rads) (Group I), and 9 did not (Group II). The mortality rate was zero for Group I patients and 55% for Group II. The mean local recurrence rate was 52% (12.5% in Group I and 88% in Group II; P < 0.05). Neuraxis recurrences occurred in two patients in Group II, and none occurred in Group I (P = 0.4). Peripheral metastasis took place in two patients (22%) in Group II and in one patient (12.5%) in Group I (P = 0.5). Of the four patients with intradural extramedullary hemangiopericytoma, all underwent surgery. Two patients received 4000 rads of radiotherapy after intervention. No patient in this group had a recurrence. CONCLUSION: For patients with intracranial meningeal hemangiopericytoma, surgical removal followed by external radiotherapy reduced the risk of local recurrence. It was not demonstrated that postoperative radiotherapy protected against neuraxis metastasis. Radiotherapy did not protect against peripheral metastasis, which can occur up to several years after the first operation. It appears that radiotherapy after surgery for local or neuraxis recurrence did not avoid further recurrence. Radiosurgery is indicated for recurrent tumors measuring less than 25 mm in greatest diameter. For intradural extramedullary localizations, the value of postoperative radiotherapy is more questionable.     

Intracranial meningeal hemangiopericytoma metastatic to the scapula

Meningeal hemangiopericytomas are rare vascular tumors that have a propensity for recurrence and metastasis. Intracranial hemangiopericytomas are rare vascular tumors. They account for 0.5% of primary central nervous system tumors and 2% of meningiomas. Unlike usual benign meningiomas, which rarely metastasize extracranially, meningeal hemangiopericytoma has a high rate of local recurrence and distant metastasis. The treatment paradigms for hemangiopericytomas and meningiomas differ based on their biological behaviors. Hemangiopericytomas have higher rates of recurrence and metastasis compared with meningiomas. Intracranial meningeal hemangiopericytoma is characterized by clinically repeated local recurrences at the primary site. Bone, liver, lung, central nervous system, and abdominal cavity are the most commonly reported sites of metastasis in hemangiopericytomas.This article describes a case of bone metastasis with extensive involvement of the scapula from intracranial hemangiopericytoma. Bone metastasis can be seen in a relatively late phase of the disease, with metastasis to other organs. Although radiation therapy is effective in controlling pain from bone metastases in unresectable disease and those with extensive visceral metastases, aggressive local surgical control of a solitary bone metastasis may be an option for patients with limited distant disease. The diagnosis may be initially confused with clear cell meningioma and benign meningiomas. The management of bone metastasis is not well reported in the orthopedic literature.     

Multiple extracranial metastases from a meningeal hemangiopericytoma and severe hypoglycemia: a case report

Extracranial multiple metastases from meningeal hemangiopericytoma have been reported only rarely. The authors describe the case of a 61-year-old woman, who was previously diagnosed as having primary meningeal hemangiopericytoma with its multiple metastases to the liver, lumbar spine, etc. and who suffered from a sudden attack of hypoglycemia. Considering the history of her present illness, this hypoglycemic shock was most likely brought on by the remarkable metastatic tumors to the liver. Recent literature pertinent to hypoglycemia suggests that insulin-like growth factor-II (IGF-II) produced by tumor is strongly suspected to be involved in the development of hypoglycemia.     

A case report of adjacent tumor of sphenoid ridge meningioma and GH producing pituitary adenoma

A case of sphenoid ridge meningioma and pituitary adenoma adjacent in the brain is reported. A 70-year-old female was admitted to our hospital with headache. She had no neurological deficit but did have acromegalic change. Hormonal examination showed elevation of plasma levels of HGH (19.0 ng/ml), with normal levels of the other hormones. CT examination revealed a tumor with calcification in the inner third of the sphenoid ridge and another in the pituitary fossa with suprasellar expansion. MRI showed flow void of ICA between these tumors. Intensity of the T1-weighted image of the tumor in the sphenoid ridge was homogeneously iso intensity, and low intensity in the pituitary fossa. The diagnosis of adjacent tumors in the sphenoid ridge meningioma and pituitary adenoma had been made preoperatively. Left front-temporal craniotomy and removal of these tumors were performed. These tumors were close to each other, but were separated by the internal carotid artery and anterior cerebral artery. Pathological examination demonstrated meningotheliomatous meningioma in the sphenoid ridge and sparsely granulated somatotroph adenoma in the pituitary fossa. Fourteen cases showing association of meningioma and pituitary adenoma, which had no history of radiation and trauma, have been reported previously. Although GH producing pituitary adenoma may stimulate adjacent dura and arachnoid cells resulting in the formation of meningioma, the possibility of coincidental occurrence of the two tumors cannot be ignored.     

Use of cryoprecipitate coagulum to control tumor-bed bleeding. Case report

The case is presented of a 44-year-old woman who underwent reoperation under cardiac standstill for a recurrent left sphenoid wing meningeal hemangiopericytoma. Because of persistent accumulations of clot with clinical deterioration and shift on computerized tomography scanning, the patient was returned to the operating suite twice. At the second reoperation, hemostasis was finally achieved through the instillation of admixed cryoprecipitate, calcium, and activated thrombin.     

Hemangiopericytoma: A 20-year single-institution experience

Background: Hemangiopericytoma is an uncommon soft tissue sarcoma. We sought to evaluate the long-term outcome of a consecutively treated patient cohort with hemangiopericytoma. Methods: The study involved 36 adult patients (older than 16 years) with hemangiopericytoma treated at The University of Texas M. D. Anderson Cancer Center between July 1975 and July 1995. Data on clinicopathologic parameters, surgical treatment, adjuvant therapy, disease recurrence, and survival were obtained from a review of medical records. Results: The median follow-up was 57 months. Twenty-eight patients (78%) underwent complete and potentially curative resection of their primary disease. Of the nine patients (32%) who had local recurrences, four (57%) had epidural tumors and three (43%) had retroperitoneal tumors, but none had extremity tumors. Extremity tumors were associated with a significantly prolonged local recurrence-free survival compared to tumors at nonextremity anatomic sites (P<.05). Ten patients had recurrences at distant sites. Of the 13 patients who experienced any form of disease recurrence, four had recurrences after a disease-free interval of more than 5 years. The 5-year actuarial survival rate for the entire group of 36 patients was 71%. Noncurative surgical treatment (P=.007) and development of distant metastatic disease (P=.013) were associated with shortened survival. Conclusion: Extended survival is common in hemangiopericytoma patients treated with curative intent. However, local and distant recurrences may occur after a prolonged disease-free interval, emphasizing the need for long-term follow-up. Retroperitoneal and meningeal tumors were associated with higher local recurrence rates; therefore, adjuvant therapies should be considered and evaluated for tumors at these sites. Presented at the 48th Annual Meeting of the Society of Surgical Oncology, Boston, Massachusetts, March 1995.     

The surgical treatment strategy and results of parasellar meningiomas in the era of radiosurgery

We evaluated the surgical treatment results of parasellar meningiomas in the era of radiosurgery. We treated 24 patients of parasellar meningiomas surgically. The median age was 60 yrs (ranging from 29 to 82 yrs). The most common tumor location was the sphenoid ridge in 12 patients and the tuberculum sellae in 7 patients. The pterional approach using fronto-temporal craniotomy was performed for all patients. The residual or recurrent tumors were treated by gamma knife radiosurgery (GKS). We are able to follow up these cases for a median of 3.8 yrs (ranging from 1 to 8 yrs) after the operations. The radicality of tumor resection was Simpson grade II in 13 patients (54%), grade III in 3 patients (13%) and grade IV in 7 patients (33%). Clinical improvement was achieved in 81% of the patients. Of the patients who had visual disturbance preoperatively, 8 patients (73%) showed improvement, but 3 patients suffered deterioration postoperatively. None of the patients died. One patient suffered transient memory disturbance and one patient suffered mild facial numbness postoperatively. Boost radiosurgery for the residual tumors was performed for six patients and tumor growth control was able to be achieved in all patients, with a median of 3.1 years follow-up period (ranging from 0.5 to 6 yrs). Five patients with tumor regrowth or recurrence were treated by GKS. We recommend fronto-temporal craniotomy with nonradical resection for parasellar meningiomas and radiosurgery for residual and recurrent tumors. This strategy will achieve good functional outcome with long-term tumor growth control.     

Hemangiopericytoma on the intradural thoracic spinal cord: a case report

Hemangiopericytoma develops from many organs. In the central nervous system, most tumors arise in the intracranial portion, and tumors originating from the spinal cord are rare. Its clinical course and neurological characteristics have not been disclosed. We present a case of a 51-year-old woman with gradually progressing paraparesis. Magnetic resonance (MR) images of the thoracic spine demonstrated an intradural tumor at the 6 and 7 thoracic vertebral body level. The patient underwent total excision of the tumor. The histological diagnosis was hemangiopericytoma. MR images after the operation showed no residual tumor and the patient was followed up without adjuvant therapy. However, 5 years later, the patient complained of back pain and gait disturbance again, and MR images showed a recurrence of the tumor. We resected the tumor under motor evoked potential (MEP) monitoring and removed the extradural part of the tumor, but the part of the tumor which had infiltrated the spinal cord was left due to the lowering of MEP amplitude. The operation resulted in partial resection. Spinal intradural hemangiopericytoma is very rare, and only 15 cases including the present case have been reported. This paper will discuss the clinical characteristics and treatment for this tumor.     

Traumatic lesions of the bilateral middle meningeal arteries--case report

A 44-year-old man presented with traumatic injuries of the bilateral middle meningeal arteries after a traffic accident. Neurological examination found left visual impairment due to left optic nerve injury. Computed tomography demonstrated a small amount of left epidural hemorrhage and bilateral skull fractures. Left external carotid angiography revealed a pseudoaneurysm of the left middle meningeal artery at the sphenoid ridge. Right external carotid angiography demonstrated a dural arteriovenous fistula fed by the right middle meningeal artery colocated with the right frontal convexity fracture. Transarterial embolization of the left middle meningeal artery pseudoaneurysm with four fibered platinum coils and transarterial embolization of the right dural arteriovenous fistula with poly(2-hydroxyethyl methacrylate-co-methyl methacrylate) were performed, resulting in complete obliteration of both lesions. Angiographic cure was obtained and the postoperative course was uneventful.     

Craniopharyngioma with erosion and drainage into the nasopharynx. An autobiographical case report

This physician had a craniopharyngioma which was treated by radiation therapy in 1938, with relief of headaches and return to professional activity. Later, penetration of the tumor through the sphenoid sinus into the nasopharynx resulted in the intermittent drainage over 30 years of cystic parts of the tumor into the nasopharynx and from there to the oropharynx without any meningeal rupture. Radiation therapy was the initial mode of treatment, later combined with steroid and thyroid replacement therapy. A professional career was possible in spite of the patient's health problems.     

Multiple extra-axial adenocarcinomas mimicking meningiomas: a case report

We present the case of multiple meningeal tumours in the right sphenoid ridge and left parafalcine region. Magnetic resonance imaging disclosed homogenous enhancement. The histopathological examination revealed metastatic adenocarcinoma of both lesions. Metastatic adenocarcinoma should be considered in the differential diagnosis of dural-base lesions and the definitive diagnosis should only be established after the histopathological report.     

Malignant hemangiopericytoma--a soft tissue and bone tumor

Hemangiopericytomas are very rare tumors that mostly develop in the soft tissues. In rare cases, however, they may occur as primary bone tumors which are extremely rare. In case of such an intraosseous tumor, a bone metastasis of such a soft tissue sarcoma must be considered first and has to be excluded. In a 62 year old female, a hemangiopericytoma of the left femoral neck was diagnosed that had induced a pathological bone fracture. A resection of the femoral head and neck was performed. Anamnestical investigations, however, have shown that 4 years before, such a tumor of the pelvic soft tissues was removed. Thus, the lesion of the femoral neck had to be classified as bone metastasis of a malignant hemangiopericytoma. It has to be emphasized that this special tumor always shows a questionable prognosis and, by histological investigations, it cannot be decided if we are dealing with a benign or malignant tumor growth. Diagnostic managements and clinical problems with hemangiopericytomas are discussed in detail.     

Case of large sphenoid ridge meningioma treated by 2-stage surgery

Large skull base meningiomas frequently encase the major cerebral vessels and cranial nerves, and receive blood supply from the branches of the internal carotid artery. One-stage resection of these tumors is difficult due to the long time needed for surgery and profuse bleeding from the tumor. We report herein a case of large sphenoid ridge atypical meningioma that was successfully resected using a combination of two-stage surgery and irradiation. A 56-year-old man was referred to us with mild left hemiparesis and visual deterioration. Computed tomography and magnetic resonance imaging showed a large sphenoid ridge meningioma. Angiography showed blood supply from the branches of both external and internal carotid arteries, and pial blood supply from the middle cerebral artery. In the first surgery after embolization of feeder vessels from the external carotid artery, the tumor was still hemorrhagic and was partially resected with 2,374 ml of blood loss. Symptoms were improved after the first surgery. Pathological diagnosis was atypical meningioma. In the second surgery after 40 Gy of irradiation, the remnant tumor was no longer hemorrhagic and was totally resected. Staged surgery with irradiation is one treatment option for large vascular skull base meningiomas, particularly for atypical meningiomas.     

Study of meningeal carcinomatosis: diagnosis and treatment]

Thirty-four patients with meningeal carcinomatosis were treated at The Center for Adult Diseases, Osaka, from 1984 through 1990. The major origin of meningeal carcinomatosis was carcinoma of the lung (14 cases) and breast (8 cases). The most frequent histological type of carcinoma was adenocarcinoma (23 cases). Twenty cases still had active lesions and/or primary lesions in the lung, liver or bone at the time of diagnosis of the meningeal carcinomatosis. The primary lesions were treated surgically in 22 cases, with chemotherapy in 4 cases and with radiotherapy in 2 cases. Metastatic brain tumors were treated by surgical removal followed by chemo-radiotherapy in 12 cases, and with radiotherapy alone in 2 cases. The interval from the time of diagnosis of the primary lesion to the time of diagnosis of the meningeal carcinomatosis varied from one month to eight years, and the interval between surgical removal of metastatic brain tumors and the diagnosis of meningeal carcinomatosis ranged from 0 to 3 years. With regard to the diagnosis of meningeal carcinomatosis, positive cytology was seen in initial standard lumbar puncture in 32 of the 34 patients. CT scan showed abnormal findings in 28 of the 34 patients, including ventricular dilatation only (12 cases), and small enhancing lesions along the CSF space (12 cases). The EEG showed abnormal patterns in 14 of 15 cases. Intrathecal chemotherapy with methotrexate alone or combined methotrexate with cytosine arabinoside via the Ommaya reservoir or standard lumbar puncture alternately with or without radiation therapy was successful as treatment, and 22 of 29 treated patients showed symptomatic improvement of the meningeal irritation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Primary meningeal osteosarcoma: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Primary meningeal osteosarcomas are extremely rare lesions; before this case report, only five cases had been reported in the literature. We encountered a patient with a meningeal osteosarcoma but no evidence of additional tumor at another remote site. CLINICAL PRESENTATION: A 56-year-old man presented with progressive weakness of the right leg and the right arm. Computed tomography and magnetic resonance imaging revealed an extracerebral frontoparietal tumor on the left side. INTERVENTION: The patient underwent complete resection of the tumor and subsequent radiotherapy of the tumor region. The histological examination showed evidence of the osteosarcoma, which was confined to the meninges. Postoperative adjuvant radiotherapy was administered. There was no tumor recurrence during 3 years of postoperative follow-up. Because the patient had no risk factors or further osteosarcomas, primary meningeal development was assumed. CONCLUSION: Despite their rarity, osteosarcomas must be considered in the differential diagnosis of meningeal tumors.     

Measurements of CSF biochemical tumor markers in patients with meningeal carcinomatosis]

CSF beta--glucuronidase, polyamines and carcinoembryonic antigen (CEA) were analyzed in 16 patients with meningeal carcinomatosis from solid tumors in systemic organs, 27 with benign brain lesions, 11 with primary brain tumors, 14 with metastatic brain tumors and 5 with leptomeningeal dissemination of other malignant diseases. beta--glucuronidase levels in all cases of meningeal carcinomatosis, meningeal gliomatosis and meningeal lymphoma were higher than 100 micrograms/dl/hr. On the other hand, levels in all cases of benign brain lesions were below 100 micrograms/dl/hr. Levels of beta--glucuronidase in the cases of metastatic brain tumors returned to normal levels after tumor resection. Levels of beta--glucuronidase and polyamines were not high in the cases with positive cytology in CSF after tumor resection. The polyamine level seemed to be dependent on the growth rate of the disease and was shown to below 0.05 nmol/ml in all cases after resection of the metastatic brain tumors. Cystic fluid of both benign and malignant tumor showed high levels of beta--glucuronidase and polyamines except for spermidine and spermine levels in a suprasellar cyst. Some cases of meningeal carcinomatosis with high levels of serum CEA did not always show high levels of CSF CEA. In the surgical cases with a metastatic brain tumor, the cases with leptomeningeal, especially dural attachment showed high levels of beta--glucuronidase and CEA preoperatively, but they returned to normal after surgery. In 2 cases of meningeal carcinomatosis treated by intrathecal chemotherapy with MTX and Ara-C, CSF beta--glucuronidase and CEA showed clinical condition better than the cell count in CSF decreased rapidly following chemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)     

Hemangiopericytoma of the maxillary sinus and skull base

Since 1949, only 52 cases of hemangiopericytoma (HPC) of the nose and paranasal sinuses have been reported in the literature. Of the latter, only 2 originated from the maxillary sinus. This article reports 2 new cases. One arose in the maxillary sinus and involved the pterygopalatine fossa, skull base, and infratemporal fossa. The second tumor was located in the sphenoethmoidal recess. A new combination of techniques for resection of hemangiopericytoma of the skull base is described. Prognostic criteria for such tumors are reviewed, in conjunction with histological diagnosis.