Gastric schwannoma

A rare case of gastric schwannoma is reported. A 36-year-old woman whose endoscopy showed a mucosa tumor, biopsy findings were suggestive for leiomyoma. Diagnosis was made only with postoperative histology that revealed the benign schwannoma. Immunohistochemical staining showed positivity for S-100 protein and the neuron specific enolase, schwannoma is often associated with Von Recklinghausen's disease. Surgical resection is recommended as their malignant potentiality. Gastric and small intestine localizations are often with bleeding. They also represent almost the 24% of all gastrointestinal stromal tumors (GIST) and the 4% of all primary retroperitoneal tumors.     

Bilateral, perisylvian and rolandic cortical dysplasia in trisomy 13 syndrome

A 25-year-old woman presented with left progressive deafness due to an intracranial tumor of the cerebello-pontine angle. Biopsy and subsequent total surgical excision indicated origin from the acoustic nerve. Histologically, the tumor showed typical feature of Schwannoma associated with a population of epithelioid cells. Three years after surgery no local invasion, no relapse, and no metastasis were noted. Immunohistochemistry showed a strong positivity for S100 protein in the cytoplasm of Schwannian and epithelioid tumoral cell components. This positivity in the epithelioid cells correspond to a well differentiated cell population derived from a Schwann cell precursor explaining the benign course of the tumor. In the absence of similar cases of epithelioid Schwannoma of the acoustic nerve in literature, we will discuss the differential diagnosis, potential evolutivity, and origin of the epithelioid cells.     

Extensive malignant schwannoma of the sciatic nerve. Contribution of imaging techniques

The authors reports a case of malignant schwannoma of sciatic nerve. Clinically, this tumor was manifested by a progressive tumefaction of the leg, and moderate pain, without functional signs. The origin of the sciatic nerve has been suspected by computed tomography, because its location, the vascular contacts, and the lesion's extension. Biopsy leads to confirm the nerve tumor, and precised the histologic type: malignant schwannoma. Local, regional and general extension did not premise treatment.     

Unattached intracranial extra-axial schwannoma

We describe an intracranial extra-axial petroclival schwannoma which did not arise from any cranial nerve, nor was it attached to brain parenchyma or dura. The possible pathogenesis is discussed.     

Light microscopic and ultrastructural observations of a metastasizing malignant epithelioid schwannoma

The light microscopy and ultrastructure of a malignant epithelioid schwannoma are described. Characteristic cells resembling perineural elements with various degrees of differentiation were observed. Primitive epithelioid cells contained scant ergastoplasm, and few tubules and filaments, but did have abundant free ribosomes and Golgi membranes. Also noted were junctional complexes and focal fusion of plasma membranes, basal laminae were absent. Better differentiated cells were completely limited by a well-developed basal membrane and had an abundance of intracytoplasmic filaments and multiple pinocytotic vesicles. The intercellular ground substance was composed of numerous fine collagen fibrils and amorphous, basement membrane-like, electron-dense material. A striking ultrastructural similarity of the tumor cells to those encountered in ethylnitrosourea-induced malignant schwannomas in rats was noteworthy.     

Hemiatrophy of the tongue due to hypoglossal schwannoma shown by MRI

Schwannomas account for 8.5% of all intracranial tumours; more than 90% arise from the 8th cranial nerve. Only 42 cases of schwannoma of the hypoglossal nerve have been reported. A 59 year-old woman developed right hemiatrophy of the tongue, clearly demonstrated on MRI, as was a small hypoglossal schwannoma. High signal was seen in the atrophic side of the tongue on both T1- and T2-weighted images, as described in the literature.     

Adenocarcinoma combined with malignant peripheral nerve sheath tumor of the gallbladder

Adenocarcinoma of the gallbladder combined with a malignant peripheral nerve sheath tumor (MPNST) in the gallbladder in an 81-year-old woman is reported. The resected gallbladder showed two distinct tumor components, the epithelioid type of MPNST and adenocarcinoma with areas of mucin production. Although the immediate postoperative course was uneventful, a pathologic fracture of her right upper femur developed 4 months after the cholecystectomy. The pathology was determined to be a feature of metastatic MPNST rather than of adenocarcinoma. A whole body bone scan revealed multiple metastases, including the left parietal skull, left ninth rib, seventh thoracic vertebra, and right upper third of the femur. Despite cholecystectomy and postoperative irradiation therapy, she died 6 months after diagnosis of the tumor. Without an autopsy the primary site of the MPNST was unknown. We found that the prognosis was very poor in patients with distal metastatic MPNST, especially in older patients.     

Aetiology, cost of antimicrobial therapy and outcome in neutropenic patients who developed bacteraemia during antimicrobial prophylaxis: a case-control study

The authors describe one case of purely epithelioid schwannoma of the ulnar nerve and discuss the therapeutic management. A 44 year-old man was referred to us for a fusiform, movable mass in the left ulnar nerve and was removed by a wide en bloc excision. Intraoperative nerve action potentials were performed both prior to and following excision of the lesion. Histologically, the tumor was composed of round or polygonal cells arranged in necrotic clusters and anastomosing cords. There were areas of spindle cells. The epithelioid cells were round with abundant cytoplasm. Mitoses were frequent. S100 protein immunoreactivity was present diffusely in tumor cells (both nuclear and cytoplasmic), whereas cytokeratin, NSE, and anti-melanoma reactions gave negative results. After a 13 months, the patient's neurological conditions are excellent and there are no signs of either recurrence or metastasis.     

Survey of minor surgery in general practice in the west of Scotland

BACKGROUND: Noncutaneous psammomatous melanotic schwannoma has recently been reported as an unusual component of Carney's complex (myxomas, spotty pigmentation, and endocrinopathy). The most common locations for this lesion include peripheral nerve roots and the gastrointestinal tract. OBSERVATIONS: A cutaneous psammomatous melanotic schwannoma is reported in a patient with known Carney's complex. This neoplasm was pseudoencapsulated, with epithelioid to spindle-shaped cells and no nuclear atypia. Immunostaining was positive for S100 protein and vimentin, as well for HMB-45 antibody. Electron microscopy showed melanosomes in cytoplasmic processes of cells that were ensheathed by layers of reduplicated basal lamina. CONCLUSIONS: Location in the superficial soft tissues is extremely unusual for psammomatous melanotic schwannomas. Recognition of this new cutaneous marker as a part of this complex may aid in identification of individuals at risk for cardiac myxomas. It is also important that this lesion is not mistaken for melanoma, given the strong HMB-45 positivity.     

Malignant schwannoma of the parapharyngeal space

We present a case of malignant schwannoma (MS) of the parapharyngeal space which completely encircled the internal carotid artery in close proximity to the skull base and required resection of the internal carotid artery along with the excision of the tumour. There have been three previous case reports in the literature of malignant schwannoma of the parapharyngeal space of which one case was associated with neurofibromatosis. Our patient did not exhibit any feature of neurofibromatosis.     

Primary mesenchymal tumors of the urinary bladder. A histological and immunohistochemical study of 30 cases

The light microscopic and immunohistochemical features of 30 primary mesenchymal neoplasms of the urinary bladder are reported. Half of the cases represented smooth and striated muscle tumors (five leiomyomas, seven leiomyosarcomas including epithelioid and myxoid subtypes, one rhabdomyoma, one embryonal rhabdomyosarcoma and one alveolar rhabdomyosarcoma). One third of the tumors were of fibrohistiocytic origin (one fibrous histiocytoma and eight malignant fibrous histiocytomas including fascicular and storiform, inflammatory and pleomorphic subtypes). In addition, a malignant epithelioid schwannoma, a round cell liposarcoma, two hemangiomas and two mixed mesodermal tumors were observed. The morphology of the vesical mesenchymal tumors was identical to that of their counterparts known to occur in other sites, particularly in the soft tissue. Muscle-specific actin, alpha-1-antichymotrypsin, S-100-protein and neuron-specific enolase proved to be useful and reliable immunomarkers for differential diagnosis of poorly differentiated leio- and rhabdomyosarcomas, malignant fibrous histiocytomas and malignant schwannomas. Since some tumors coexpressed several classes of intermediate filaments, diagnostic immunocytochemistry should only be used considering a larger panel of antibodies and in close correlation with the histological and cytological features of the neoplasms.     

Drug resistant typhoid fever

BACKGROUND: Malignant schwannomas are rare malignant mesenchymal tumors often associated with neurofibromatosis. They occur less frequently in the head and neck than in other regions. PATIENT: A case history of a primary malignant schwannoma of the head and neck area in a 27-year-old man is reported. The tumor was located in the left submandibular region. The patient did not have any functional deficits. The tumor was totally removed. There have been no signs of either recurrence or metastasis within the two years following diagnosis and surgery. DISCUSSION: The microscopic and immunohistochemical findings are presented, and the importance and therapy of this very rare malignant tumor of the head and neck area are discussed. CONCLUSION: Malignant schwannoma in the head and neck region is rare. Radical resection is the treatment of choice.     

Giant plexiform schwannoma: a report of two cases with soft tissue and visceral involvement

We report two cases of massive, solitary, plexiform schwannoma. One was a 9-cm subcutaneous lesion on the hip of a 72-year-old man who had become aware of the slow-growing tumor 50 years earlier; the other is the first reported plexiform schwannoma to arise in a visceral organ: it arose in the ascending colon of a 54-year-old man and exhibited a dumbbell configuration with submucosal and subserosal components. Neither patient had neurofibromatosis or schwannomatosis. Both tumors were well-circumscribed and multinodular, and both showed a plexiform architecture. Microscopically, the nodules were composed primarily of Antoni A tissue, replete with nuclear palisading and Verocay bodies. Examination by immunohistochemistry and electron microscopy demonstrated the features of well-differentiated Schwann cells; nodules were surrounded by attenuated, residual perineurium. Both patients followed a benign clinical course, without recurrence or metastasis. Neither the large tumor size nor the unusual locations affected the biologic behavior of these neoplasms. A massive plexiform schwannoma must be distinguished from a malignant peripheral nerve sheath tumor and from a plexiform neurofibroma, a tumor prone to malignant transformation.     

Thoracic outlet compression syndrome caused by a schwannoma of the C7 nerve root

This is the first report of a schwannoma originating from the C7 nerve root causing thoracic outlet compression syndrome. The patient was a 30-year-old woman with a 3-year history of numbness on the radial side of the left hand, left arm tiredness, nocturnal pain in the left forearm and pain in the left elbow, shoulder and neck. Conservative treatment and previous operations, including carpal tunnel release and first rib resection, provided no relief. A left scalenectomy was performed. During the removal of the anterior scalene muscle, a mass approximately 3 cm long and 1.5 cm in diameter was noted under the anterior scalene muscle involving the C7 nerve root. The tumour was encapsulated and covered with attenuated and stretched nerve fascicles. It was completely excised without disturbing the nerve fascicles. The clinical impression was schwannoma, which was confirmed on pathological examination.     

Transverse and T fractures with fracture of the posterior wall of the acetabulum. Results of orthopedic and surgical treatment. Apropos of 52 cases

A patient developed delayed facial nerve palsy at the level of House-Brackmann grade I to grade III 10 days after vestibular schwannoma surgery by the suboccipital transmeatal approach. The palsy had completely recovered after one month. Immunological study showed reactivation of herpes simplex and magnetic resonance (MR) imaging demonstrated an abnormal enhancement pattern of the facial nerve; intense enhancement of the distal intracanalicular segment and labyrinthine segment, similar to the MR findings for Bell's palsy. A prospective control study on the enhancement pattern of the functionally preserved facial nerve after vestibular schwannoma surgery in six cases showed a similar pattern to that of the normal facial nerve. Based on these findings, we propose the hypothesis that herpes simplex reactivation is an underlying cause of delayed facial palsy after vestibular schwannoma surgery.     

Research and the problems of litter and medical wastes on the UK coastline

OBJECTIVE AND IMPORTANCE: Symptomatic cerebral vasospasm can occur after resection of tumors in or adjacent to the basal cisterns, causing delayed neurological deterioration. This potentially treatable condition may go unrecognized. Delay in its recognition will adversely affect the outcome of the patients. There has been a few cases of vasospasm after tumoral resection reported in the literature, mostly in adults. We report a case of vasospasm after resection of a third nerve schwannoma in a pediatric patient. This is the youngest patient reported to date with vasospasm after resection of a brain tumor. CLINICAL PRESENTATION: A six years old girl presented with sudden onset diplopia. Radiological work-up revealed a third nerve mass. She underwent a craniotomy for resection of her mass. Pathological findings were consistent with a third nerve schwannoma. One week postoperatively, her mental status deteriorated. A CT scan revealed a diffuse hypodense area involving the right frontal and temporal lobes in the middle cerebral artery distribution as well as the midbrain. The absence of these findings on the MRI imaging performed on the first postoperative day made us evoke a vascular etiology. A cerebral angiogram was performed and revealed vasospasm in the right internal carotid artery and in the right middle and posterior cerebral arteries. Hyperdynamic hypervolemic hemodilutional therapy was instituted. CONCLUSION: Delayed clinical deterioration from vasospasm is a potentially reversible condition, if recognized early. A high index of suspicion should be maintained in case delayed clinical deterioration occurs after surgery of tumors in the basal cisterns. Cerebral angiography will confirm the diagnosis. Early institution of hyperdynamic hypervolemic hemodilutional therapy and angioplasty may reverse the deficit and improve outcome.     

Identification and characterization of two thrombin-activatable fibrinolysis inhibitor isoforms

OBJECT: The indications, operative findings, and outcomes of vestibular schwannoma microsurgery are controversial when it is performed after stereotactic radiosurgery. To address these issues, the authors reviewed the experience at two academic medical centers. METHODS: During a 10-year interval, 452 patients with unilateral vestibular schwannomas underwent gamma knife radiosurgery. Thirteen patients (2.9%) underwent delayed microsurgery at a median of 27 months (range 7-72 months) after they had undergone radiosurgery. Six of the 13 patients had undergone one or more microsurgical procedures before they underwent radiosurgery. The indications for surgery were tumor enlargement with stable symptoms in five patients, tumor enlargement with new or increased symptoms in five patients, and increased symptoms without evidence of tumor growth in three patients. Gross-total resection was achieved in seven patients and near-gross-total resection in four patients. The surgery was described as more difficult than that typically performed for schwannoma in eight patients, no different in four patients, and easier in one patient. At the last follow-up evaluation, three patients had normal or near-normal facial function, three patients had moderate facial dysfunction, and seven had facial palsies. Three patients were incapable of caring for themselves, and one patient died of progression of a malignant triton tumor. CONCLUSIONS: Failed radiosurgery in cases of vestibular schwannoma was rare. No clear relationship was demonstrated between the use of radiosurgery and the subsequent ease or difficulty of delayed microsurgery. Because some patients have temporary enlargement of their tumor after radiosurgery, the need for surgical resection after radiosurgery should be reviewed with the neurosurgeon who performed the radiosurgery and should be delayed until sustained tumor growth is confirmed. A subtotal tumor resection should be considered for patients who require surgical resection of their tumor after vestibular schwannoma radiosurgery.     

Benign neural sheath tumours of major nerves: characteristics in 119 surgical cases

Peripheral benign nerve sheath tumours are infrequent tumours and affect major nerve trunks. Some authors have indicated a high and prohibitive incidence of neurological injury in resection of these lesions. The authors describe their findings in a retrospective study comprising 119 patients with spontaneous benign nerve sheath tumours of the peripheral nervous system. Seventy-three patients had a schwannoma, 41 had neurofibroma and 5 had plexiform neurofibroma; 25 of the 119 patients suffered from neurofibromatosis. All schwannomas were excised completely and the outcome of patients was 41.0% improved, 6.8% worsened, 52.0% unchanged. Twenty-eight neurofibromas were excised completely and 13 subtotally; the outcome for patients was 19.5% improved, 19.5% worsened and 61% unchanged. All plexiform neurofibromas were removed subtotally and the outcome for patients was 20% improved and 80% unchanged. The best surgical results at average follow-up of 6 years were observed in the patients with schwannoma, the worst in those with plexiform neurofibroma. Our results demonstrated that it is often possible to remove schwannomas as well as neurofibromas with an acceptable risk of injury to the nerve.     

"Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series

Eighteen examples of an unusual malignant soft-tissue neoplasm, the morphology of which ranged from that of "atypical" epithelioid sarcoma to that of a rhabdoid tumor or undifferentiated carcinoma (with transitional forms) are described. Patients included 11 males and seven females; their median age was 35.5 years with most patients aged 20 to 40 years. Development of a mass was the main presenting symptom. Six tumors developed in the pelvis and perineal region, four in the pubic region and vulva, three in the buttocks, one in the deep soft tissues of the left hip, one on the penis, one in left forearm, one in left axilla, and one on the occiput. Tumor size ranged from 1 to 20 cm (median, 4 cm). On microscopic examination, the tumor cells invaded the subcutaneous or deep soft tissues, had prominent epithelioid or rhabdoid features, had marked cytologic atypia, and grew in a multinodular pattern in half of the cases. Areas of necrosis were often seen. A granuloma-like pattern reminiscent of that observed in classic epithelioid sarcoma was observed in only two cases. Immunohistochemically, positivity for cytokeratin, epithelial membrane antigen, and vimentin was seen in all but one of the cases. Of 16 cases, 10 and eight tumors reacted with desmin and CD34, respectively; five of 15 reacted at least focally with smooth-muscle actin, whereas three of 13 and one of 10 reacted for HMB-45 and carcinoembryonic antigen, respectively. S-100 protein and CD31 yielded negative results. Seven tumors were investigated at the ultrastructural level, four of which showed prominent intracytoplasmic intermediate filament aggregates, often accumulating into paranuclear whorls, which is in keeping with the rhabdoid phenotype. Five tumors showed features of epithelial differentiation (i.e., tonofilament-like structures or desmosomes or both), whereas one tumor displayed features of myofibroblastic differentiation. Differential diagnoses include mainly conventional epithelioid sarcoma, extrarenal malignant rhabdoid tumor, epithelioid malignant peripheral nerve sheath tumor, melanoma, rhabdomyosarcoma, and undifferentiated carcinoma. Follow-up information on 14 patients (range, 4 months to 8 years; median, 19 months) revealed local recurrence in one case and metastatic dissemination in six patients, leading to death in five. In our opinion, the above-described neoplasms represent a usually "proximal-type" of epithelioid sarcoma. In contrast to the conventional, "distal-type" epithelioid sarcoma, the proximal variant is characterized by a predominantly large-cell, epithelioid cytomorphology, marked cytologic atypia, frequent occurrence of rhabdoid features, and lack of a granuloma-like pattern in most cases. It appears to be somewhat more aggressive (or at least metastasizes earlier) than usual epithelioid sarcoma.