Multiple schwannomas: report of two cases

In this paper authors present two cases of multiple schwannomas without the features of neurofibromatosis (NF). The authors retrospectively reviewed the hospital charts, radiology films, operative notes and pathology slides of these two patients. There was no family history of neurofibromatosis. The two patients had contrast enhanced MRI, which was negative for vestibular schwannomas. Both underwent surgical excision of symptomatic lesions. Histopathology confirmed these lesions as schwannomas. Molecular genetic analysis in case 1 demonstrated two distinct mutations of the NF2 gene in two different schwannomas, with concomitant loss of heterozygosity in both tumours. In contrast peripheral blood lymphocytes did not reveal mutations of NF2. The authors recommend surgery for symptomatic lesions. Asymptomatic tumours can be monitored. Regular follow up is essential as they may develop fresh lesions at any time. The relevant literature is discussed.

     

Facial nerve schwannomas: report of two cases

Although schwannoma may arise from any true cranial nerve, it is well known that the acoustic and trigeminal nerves are frequent origins. Schwannomas of the facial nerve are rare. In this communication, two cases of facial nerve schwannoma arose from the horizontal portion are reported. Case 1. A 44 year-old male was admitted to our clinic complaining of left hearing loss and facial asymmetry. About three years prior to the admission, he first noticed left hearing disturbance which was gradually deteriorated and was transiently accompanied by left tinnitus during the progression. Then muscle spasm developed on the left eyelid which resolved spontaneously in a few weeks, then asymmetry of the face developed. Neurological examination on admission revealed left hearing loss and left peripheral facial palsy. As a result of neuro-otological examinations, left hearing impairment and left facial palsy were thought to be due to retrocochlear and suprageniculate lesions respectively. Plain skull radiograms and tomograms revealed marked destruction of left pyramis and enlarged internal auditory canal. Computed tomography of the brain showed low-density mass in the left middle fossa and defect of the tip of the left pyramis. After bolus injection of contrast material, peripheral portion of the middle fossa mass was enhanced non-homogeneously and enhanced mass extended to the posterior fossa. Left middle and posterior fossas were explored by carrying out a osteoplastic temporal flap and suboccipital craniectomy. A large extradural mass was noted to have filled the middle fossa which extended to the posterior fossa through destroyed pyramis and enlarged internal auditory canal. The tumor was removed subtotally.(ABSTRACT TRUNCATED AT 250 WORDS)     

Retroperitoneal giant schwannomas: report on two cases and review of the literature

The occurrence of massive retroperitoneal schwannomas is extremely rare and their presence may only be expressed by insidious onset of non-specific and misleading symptoms with a predominance of lower back pain. MRI scan as the imaging procedure of choice will demonstrate the tumour location and its relation to the surrounding structures, but due to heterogeneity and degeneration in some tumours, it may mimic malignancy. Hence tissue sampling through needle biopsies are essential to verify the diagnosis prior to surgery. Tumour excision in toto is considered the treatment of choice, but it can be hazardous especially if the tumour is adherent to the presacral venous plexus. Severe bleeding complications due to the damage of venous structures have to be encountered, and establishing lasting haemostasis may pose considerable difficulties. Hence surgery should be attempted with full precautions, and preoperative counseling of the patient. If malignancy can safely be excluded, laparoscopic piecemeal excision should be considered as an alternative treatment as recurrence is unlikely. Definition of the originating nerve might not always be possible and a minor degree of neurological impairment has therefore to be anticipated.     

Intrasellar tuberculoma: case report

We report a unique case of exclusively intrasellar tuberculoma. The patient was operated on by the transsphenoidal approach, which allowed total removal of the lesion and prevented cerebrospinal fluid contamination. After histological diagnosis, the patient was treated with isoniazid, 300 mg daily for 3 months, as prophylaxis against tuberculous meningitis.     

Dumbbell C2 schwannomas involving both sensory and motor rootlets: report of two cases

OBJECTIVE AND IMPORTANCE: Intradural-extradural dumbbell C2 schwannomas are rare. This report concerns two such cases with the intradural compartment located ventral to the spinal cord and involving both sensory and motor rootlets. CLINICAL PRESENTATION: One patient was a 57-year-old woman with sensory disturbances in the right extremities and hyperreflexia in the left extremities. The other patient was a 73-year-old man who presented with tetraparesis, walking disability, atrophy of the nuchal and bilateral shoulder muscles, and pain in the right C2 dermatome. INTERVENTION: The extradural component of the tumor was removed first; next, the intradural component was removed successfully via the posterior approach combined with a C1-C2 laminectomy. The patients experienced symptomatic improvement without further deficits except for sensory impairment of the C2 dermatome in one of the patients. CONCLUSION: Intradural-extradural dumbbell C2 schwannomas can be satisfactorily managed with a posterior approach. Removal of the extradural component and opening of the dural ring of the C2 nerve root are necessary for safe extraction of the intradural ventrally located component after debulking. These tumors may arise extradurally within the nerve sheath, extend intradurally and ventrally toward the spinal cord, and involve both sensory and motor rootlets.     

Intrasellar rhabdomyosarcoma: case report

OBJECTIVE AND IMPORTANCE: There has been only one reported case of an intrasellar rhabdomyosarcoma, the origin of which was in the para-nasal sinus. The authors encountered a patient with an intrasellar rhabdomyosarcoma with no evidence of tumor at any additional sites. CLINICAL PRESENTATION: A 28-year-old otherwise healthy man complaining of headache exhibited left abducent nerve palsy and left visual disturbance. The patient was diagnosed as having a sellar tumor invading the left cavernous sinus. INTERVENTION: Near total removal of the tumor was achieved via a trans-sphenoidal approach. Histologically, the tumor was composed of small, round-to-elongated undifferentiated cells and large spindle cells with myoblastic features. Immunohistochemically, tumor cells were positive for antibodies against desmin, myoglobin, and alpha-smooth muscle actin. The tumor was identified as an embryonal rhabdomyosarcoma on the basis of the above pathological findings. Systemic investigation, including the nasal and para-nasal regions, failed to detect any additional tumors. Postoperative local radiation therapy and chemotherapy with the use of ifosfamide, etoposide, and vincristine brought about complete initial remission. CONCLUSION: Rhabdomyosarcoma should be considered in the differential diagnosis of a primary intrasellar neoplasm.     

Intrasellar schwannoma: case report

An unusual case of an intrasellar schwannoma radiographically simulating a pituitary tumor is reported. The literature on unusual intracranial locations of schwannomas and their possible origin is discussed.     

Intrasellar pleomorphic xanthoastrocytoma: case report

OBJECTIVE AND IMPORTANCE: A patient with a primary intrasellar pleomorphic xanthoastrocytoma is described. CLINICAL PRESENTATION: A 78-year-old woman experienced slowly progressing bilateral visual disturbance. Analysis of magnetic resonance imaging scans indicated a well-enhanced tumor occupying intra- and suprasellar spaces with displacement of the pituitary gland anteriorly. INTERVENTION: Partial resection of the tumor via the transsphenoidal route brought about improvement of the patient's visual disturbance. The tumor was conspicuously pleomorphic and composed of plump to spindle-shaped large and bizarre cells with single or multiple nuclei and lipid-laden foamy cytoplasm. The neoplastic cells were positive for glial fibrillary acidic protein, S-100 protein, and vimentin. The MIB-1 antibody labeling index was very low (<1%) CONCLUSION: Pleomorphic xanthoastrocytoma should be included in the differential diagnosis of tumors arising in the posterior hypophysis.     

Brachytherapy of vestibular schwannomas: report of three cases.

OBJECTIVE: Gamma knife and multileaf collimator LINAC have recently gained notoriety in the treatment of vestibular schwannomas. Without the use of gamma knife or LINAC, we have successfully pursued the 125-iodine interstitial irradiation of three vestibular schwannomas. CASE REPORT: Up to March 2004, there was a 57 and 45 month follow-up period in two cases. One patient died of heart insufficiency 15 months after the brachytherapy. At the end of the follow-up period audiometric examinations revealed slight improvements of hearing in patients 1 and 2. In patient 1, the tumor volume measured was 4.06 mL on the control CT indicating a 40 % shrinkage in comparison to the 6.74 mL target volume at the brachytherapy. In patient 2, the tumor volume measured on the control MRI was 6.64 mL, indicating a 42 % shrinkage of the 11.45 mL target volume at the time of brachytherapy. Eleven months after the brachytherapy in patient 3 we found 10 % tumor shrinkage, a post-irradiation cyst developed inside the tumor and reached up to 35 % of the tumor volume. INTERVENTION: In the treatment of three vestibular schwannomas we have used CT and image-fusion guided 125-iodine stereotactic brachytherapy. CONCLUSION: Due to financial considerations, gamma knife and LINAC are not available for many countries and neurosurgical institutes. In the absence of the above-mentioned radiosurgical methods, we have shown brachytherapy as a new alternative and solution in the treatment of the three patients studied with vestibular schwannoma.     

Sixth nerve schwannomas. Report of two cases

Two cases of sixth cranial nerve schwannoma are presented with a review of four other cases from the literature. The clinical spectrum, neuroradiological findings, and surgical outcome of the six cases are discussed. There are two distinct clinical presentations for sixth cranial nerve schwannomas. Type I sixth nerve schwannomas present with sixth nerve palsy and diplopia and arise from the cavernous sinus. In contrast, type II sixth nerve schwannomas have a more severe presentation with obstructive hydrocephalus, raised intracranial pressure, sixth nerve palsy, and diplopia. This type arises along the course of the sixth cranial nerve in the prepontine area. Cavernous sinus involvement in either type may preclude total surgical excision and indicate an increase possibility for recurrence.     

Orbital schwannomas: report of nine cases and review of the literature

The authors report 9 cases of orbital schwannoma out of a total of 148 orbital tumors operated. The computed tomography (CT) scan is considered superior to other methods used in the radiological work-up of endoorbital tumors because, in addition to being noninvasive, it gives direct visualization of the lesion and more precise localization both with respect to the muscular cone and tridimensionally. Unfortunately, preoperative diagnosis is not always possible solely on the basis of the CT scan because the enhancement of the tumor is not constant, thus making differential diagnosis with meningioma and cavernous angioma problematic. In addition, CT enables the surgeon to choose the best route for tumor removal. Operative results are good and are influenced by the use of microsurgical techniques.     

Contiguous conventional and plexiform schwannomas. Report of two cases

The authors present clinical, imaging, and histological features of two adult patients found to harbor a conventional schwannoma contiguous with a deep plexiform schwannoma. One patient had neurofibromatosis (NF) Type 2 (NF2), and both intracranial (bilateral oculomotor, trigeminal, acoustic, and hypoglossal schwannomas as well as meningiomas) and intraspinal (schwannomas and meningiomas) lesions. The proximal forearm lesions consisted of a conventional schwannoma and an underlying plexiform component. The second patient, who did not have NF2, presented with a similar enlarging mass in the distal arm; two contiguous lesions were resected. Both patients exhibited distinct, readily identifiable, magnetic resonance imaging features. Plexiform schwannomas are rare lesions that occur sporadically or, on occasion, in association with NF2 or meningiomas with or without multiple schwannomas. The authors believe that a more careful examination of patients with NF2 may show that these people have a higher incidence of plexiform schwannoma than previously thought. Pathological confirmation is crucial in distinguishing plexiform schwannomas from plexiform neurofibromas, because the latter are directly associated with NF1 and have a significant tendency to undergo malignant transformation. To the best of the authors' knowledge, this is the first report of a conventional schwannoma contiguous with a deep plexiform schwannoma.     

Intrasellar hydatid cyst. Case report

The authors report a case of hypopituitarism due to a cystic lesion in the sella turcica which was revealed by neuroradiological investigations. A hydatid cyst was found and removed by the transsphenoidal route. The transsphenoidal approach avoided intracranial spillage of fertile daughter cyst and scolices.     

Intrasellar cysticercosis. Case report

Cysticercosis of the pituitary fossa is a rare finding. A case is described in which intra- and suprasellar cysticercosis produced disturbances in both visual acuity and visual fields. On clinical examination, angiography, and computerized tomography, it simulated an intrasellar tumor with suprasellar extension. The diaphragma sellae was absent.     

Intrasellar cavernous hemangioma. Case report

The authors present a rare entity, an intrasellar cavernous hemangioma that on neuroimages mimicked a nonfunctioning pituitary macroadenoma in a patient with a known orbital hemangioma. Such lesions can grow extraaxially within the dural sinuses, particularly the cavernous sinus, and present like tumors. A better understanding of the neuroimaging. clinical, and anatomical features of these lesions may prevent difficulties in management.     

Intrasellar mucocele simulating pituitary adenoma: case report

Mucoceles occupying the sella turcica with suprasellar extension are quite rare. A case of intrasellar mucocele is reported. The preoperative clinical and computed tomographic appearance could not be differentiated from that of pituitary adenoma.     

Gallbladder volvulus: two cases report

The authors report two cases of gallbladder volvulus. The diagnosis of this rare pathology is mainly identified preoperatively. Ultrasonographic findings include a "floating gallbladder" with thickened hypoechoic walls. The treatment is emergency cholecystectomy.     

Spontaneous pneumomediastinum: report of two cases

Spontaneous pneumomediastinum (SPM) is an uncommon, benign, self-limited disorder that usually occurs in young adult males without any apparent precipitating factor or disease. SPM responds extremely well to conservative treatment, without recurrence in the great majority of cases. In this report, two patients who were admitted for SPM are discussed together with the associated literature.