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.     

Dystrophin binding to nonmuscle actin

AIMS: Plexiform schwannoma (PS) is a rare variant of benign schwannoma characterized by a multinodular and plexiform growth pattern. In contrast to plexiform neurofibroma. PS is not associated with neurofibromatosis 1 (NF-1; von Recklinghausen's disease) and has no propensity for malignant transformation. The purpose of this study was to clarify the relationship between PS and the entities of neurofibromatosis 2 (NF-2; bilateral acoustic neurofibromatosis) and schwannomatosis. METHODS AND RESULTS: Six cases of PS associated with NF-2 or meningioma were retrospectively studied clinicopathologically and immunohistochemically. Four cases of PS were found among the patients with NF-2, and all of these had multiple PSs; three cases also had multiple schwannomas of the spinal nerve roots and two of these had meningioma. Two other patients had meningioma, but not NF-2. Four patients were male and two were female. The ages ranged from 18 to 52 years (mean 29.6 years). Histologically, PS showed the histological features that have been previously described, i.e. schwannoma composed of a predominant Antoni A-type component with a plexiform growth pattern. Immunohistochemically, the tumour cells were positive for S100 protein. Each nodule was surrounded by perineural cells which were positive for epithelial membrane antigen. CONCLUSIONS: It is important to recognize that PS could be associated with NF-2 or meningioma. The combination of PS and meningioma may be a 'formes frustes' of NF-2, and is clinically overlapped with schwannomatosis.     

Malignant triton tumour of the spine: a case report

A malignant triton tumour is a malignant schwannoma with rhabdomyoblastic differentiation, and is a very rare occurrence. We describe the case of a 33-year-old man with neurofibromatosis type 1 who presented with paraplegia. Pathological examination of an intradural, extramedullary tumour excised at lumbar laminectomy demonstrated a malignant schwannoma with rhabdomyoblastic differentiation. We believe this is the first documented case of a malignant triton tumour of the spine.     

Cutaneous-splanchnic neurofibromatosis

BACKGROUND: Segmental neurofibromatosis (NF V) is ten times less frequent than Recklinghausen disease. Would the risk of visceral involvement in this uncommon form of neurofibromatosis warrant systematic imaging procedures? CASE REPORT: A 31-year-old man consulted for a voluminous plexiform neurofibroma in the left lumbar area. More ventrally, on the left side, there was also a café au lait spot. There were no Lisch nodules. The chest and abdominopelvic computed tomography and magnetic resonance imaging showed intramuscular tumoral extension, two neurofibromas in the 9th intercostal space and a voluminous 5-cm tumor situated in the left adrenal area. After resection pathology examination of the surgical specimen confirmed the diagnosis of ganglioneuroma. DISCUSSION: In this patient, all the neurofibromas and the café of lait spot developed in the territories of the left T10 and adjacent spinal roots. This was also true for the ganglioneuroma which developed on the deep sympathetic ramus to the adrenal gland which originates essentially from roots T8 to T11. This would place this case in the second subgroup of NF V in Roth's classification. Only six other cases have been reported in the literature. Such deep localizations are very likely to be underestimated, raising the problem of their detection and the correct protocol to follow asymptomatic forms, especially to detect disease progression to malignant degeneration which has a poor prognosis. Patients with a NF V should receive genetic counselling with a search for a family history, other signs of neurofibromatosis and Lisch nodules. In young patients, the risk of deep asymptomatic spread underlines the importance of regional computed tomographic or magnetic resonance explorations.     

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.     

Retrovesical leiomyosarcoma responsive to preoperative chemoradiotherapy: a case report

A 70-year-old man with dysuria was referred to our hospital. Computed tomography scan and magnetic resonance imaging demonstrated a large solid tumor in the retrovesical space. Transurethral needle biopsy revealed leiomyosarcoma. Since the size of the tumor decreased markedly by intraarterial chemotherapy with cisplatin, methotrexate and pirarubicin, in combination with radiotherapy (40 Gy), surgical extirpation of the tumor was performed. Neither infiltration to the adjacent organs or lymph node metastasis was recognized. The patient had been free of recurrence for 12 months after operation.     

Creutzfeldt-Jakob disease in a husband and wife

A 53-year-old man died of sporadic Creutzfeldt-Jakob disease (CJD) after a 1.5-year clinical course. Four and a half years later, his then 55-year-old widow died from CJD after a 1-month illness. Both patients had typical clinical and neuropathologic features of the disease, and pathognomonic proteinase-resistant amyloid protein ("prion" protein, or PrP) was present in both brains. Neither patient had a family history of neurologic disease, and molecular genetic analysis of their PrP genes was normal. No medical, surgical, or dietary antecedent of CJD was identified; therefore, we are left with the unanswerable alternatives of human-to-human transmission or the chance occurrence of sporadic CJD in a husband and wife.     

Eruptive epithelioid hemangioendothelioma with spindle cells

A 39-year-old white man presented with four discrete dermal nodules in his right upper arm. Biopsy revealed superficial dermal well-circumscribed nodules composed of solid areas and vascular spaces lined by epithelioid endothelial cells and a similar nodule composed of spindle and epithelioid cells. A moderate mitotic count of 3-4 mitoses/10 hpf was present. Multiple lesions erupted 1 month later distally and proximally to the original lesions. Magnetic resonance imaging of the right arm demonstrated a lesion in the humerus. Biopsy of the humerus showed a vascular tumor with similar histologic features to the overlying skin lesions. The differential diagnosis included epithelioid vascular tumors, bacillary angiomatosis, pyogenic granuloma, and Kaposi sarcoma. Vascular lesions containing epithelioid and spindle cells span a spectrum from benign to malignant. We believe these tumors belong in the category of hemangioendothelioma and propose the name eruptive epithelioid hemangioendothelioma with spindle cells. Our case emphasizes that eruptive cutaneous vascular lesions do not always suggest immunosuppression or malignancy. Additionally, it highlights the association between epithelioid vascular lesions of the skin and bone.     

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.     

Acute bacterial endocarditis following burns: case report and review

A 64-year-old man was admitted to our hospital because of abnormal shadows on chest X-ray films. Malignant mesothelioma was suspected. However a CT scan revealed a large mass in the right kidney and many nodules in the liver and pleura. This suggested that primary renal cell carcinoma had metastasized to the liver and pleura. The patient was treated with transarterial embolization (TAE), alpha and gamma interferon, and UFT, but died of respiratory failure caused by massive bleeding from the pleural metastases. At autopsy, renal cell carcinoma, clear cell subtype, was noted. The many pleural lesions were of the same histologic type, which suggested that they were metastases from the kidney. Renal cell carcinoma frequently metastasizes to the lungs or bones via the arteries. However, many pleural metastases without lung metastasis is rare. We report a case of renal cell carcinoma with many pleural metastases via Batson's venous plexus.     

Dermatoepidemiology. II: Causal inference established by a rule called "ASSOCIATED"

Two cases of endometrial low-grade stromal sarcoma with ovarian sex cord-like differentiation occurring in a 39-year-old woman and a 42-year-old woman are presented. Both tumors, which were intramyometrial and measured 7.5 cm and 7.0 cm in greatest diameter, respectively, showed a multinodular, ill-demarcated, and yellowish white cut-surface. Histologically, most parts of the tumors were composed of trabecular, cord-like, or plexiform arrangements that were reminiscent of the growth pattern seen in ovarian sex cord tumors. Features of conventional endometrial low-grade stromal sarcoma were only focally observed. The tumors showed infiltrative margins and lymphatic invasion. The tumor cells were positive for vimentin, desmin, alpha-smooth muscle actin, and muscle actin (HHF35). The tumors were also positive for both estrogen and progesterone receptors. Both tumors were DNA diploid as determined by flow cytometry. One patient had recurrences, including osteolytic lesions in the pelvic bones, but had no evidence of recurrence or metastasis 11 months after the last surgery. The other patient had no evidence of tumor in a limited follow-up. Familiarity with the neoplasm and other uterine mesenchymal tumors with ovarian sex cord-like differentiation by gynecologists and pathologists is essential in avoiding misdiagnosis because adjuvant hormonal therapy may be effective in treating low-grade stromal sarcomas.     

Two patients with polymyositis or dermatomyositis complicated with massive pleural effusion

Two patients with polymyositis (PM) or dermatomyositis (DM) complicated with massive pleural effusion are reported here. Both patients presented a high-grade fever, pleural effusion prominent on the right, and good response to steroid therapy. In a 50-year-old woman with PM, combined process of pleural inflammation, cardiomyopathy and coexisting hypothyroidism were considered to be responsible for the accumulation of the massive pleural effusion. However, in a 34-year-old man with DM, pleural inflammation associated with interstitial pneumonia or pleural microvasculopathy in DM was considered to be responsible for the accumulation of the massive pleural effusion.     

Angiosarcoma (angioendothelioma) of the scalp. An unusual case of scarring alopecia

Angiosarcoma with initial involvement of the scalp is a rare, malignant neoplasm. We present the case of a 77-year-old woman in whom extensive scarring alopecia developed that was proved histologically to be due to angiosarcoma. She had a good initial response to treatment with electron-beam therapy, but after seven months, extensive nodules and indurated areas on the face beyond the borders of the radiation field developed. Subsequently, massive cervical adenopathy and metastases to the lungs, liver, and spleen developed. Angiosarcoma must be differentiated from other malignant blood vessel tumors and should be considered in the differential diagnosis of scarring alopecia of the scalp in elderly patients.     

Benign schwannoma of the breast: report of a case

We report herein the case of a 62-year-old woman who presented to our hospital for investigation of occasional pain in her left breast. Although there was no mass palpable in her left breast, mammography and ultrasonography revealed a round tumor in the upper outer quadrant of the right breast. Although the mammography findings indicated that the tumor was benign, the possibility of a malignant neoplasm could not be ruled out by the ultrasonographic images. A final diagnosis of schwannoma was established by histopathological examination of the excised mass.     

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.     

Pleural malignant mesothelioma with osseous cartilaginous and rhabdomyogenic differentiation

A 63-year-old man, who was formerly an asbestos factory worker who had been followed due to asbestosis, and was admitted to our hospital with left chest pain and dyspnea on exertion. A chest X-ray and chest computed tomogram (CT) on admission revealed a large tumor in the left lung field. Percutaneous needle biopsy determined that the tumor was a sarcoma. No clinical response was obtained by systemic chemotherapy. The autopsy revealed diffuse malignant mesothelioma of sarcomatous type with osseous, cartilaginous and rhabdomyogenic differentiation. Osseous and cartilaginous differentiation in a malignant mesothelioma is rare, and the presence of a malignant rhabdomyogenic component is the first to be described in the Japanese literature.     

A surgical case of cardiac malignant lymphoma in right atrium

We reported a case of malignant lymphoma originating from the right atrium. The patient was a 71-year-old man who had no symptoms associated with heart failure and arrhythmias. At the time of admission the patient was suggested the presence of a tumor in the right atrium by an echocardiogram accidentally. Coronary angiography revealed a feeding artery to a tumor. Although cytological confirmation was not obtained, diagnostic extirpation of tumor was performed under cardiopulmonary bypass. The histological diagnosis was malignant lymphoma of B-cell origin. His postoperative course was uneventful and no recurrence had been observed one year postoperatively without chemotherapy.     

Ruffling membrane, stress fiber, cell spreading and proliferation abnormalities in human Schwannoma cells

Schwannomas are peripheral nerve tumors that typically have mutations in the NF2 tumor suppressor gene. We compared cultured schwannoma cells with Schwann cells from normal human peripheral nerves (NHSC). Both cell types expressed specific antigenic markers, interacted with neurons, and proliferated in response to glial growth factor, confirming their identity as Schwann cells. Schwannoma cells frequently had elevated basal proliferation compared to NHSC. Schwannoma cells also showed spread areas 5-7-fold greater than NHSC, aberrant membrane ruffling and numerous, frequently disorganized stress fibers. Dominant negative Rac inhibited schwannoma cell ruffling but had no apparent effect on NHSC. Schwannoma cell stress fibers were inhibited by C3 transferase, tyrphostin A25, or dominant negative RhoA. These data suggest that the Rho and Rac pathways are abnormally activated in schwannoma cells. Levels of ezrin and moesin, proteins related to the NF2 gene product, merlin, were unchanged in schwannoma cells compared to NHSC. Our findings demonstrate for the first time that cell proliferation and actin organization are aberrant in schwannoma cells. Because NF2 is mutant in most or all human schwannomas, we postulate that loss of NF2 contributes to the cell growth and cytoskeletal dysfunction reported here.     

CT imaging in adults with neurofibromatosis-1: frequent asymptomatic plexiform lesions

OBJECTIVE: The authors examined the incidence and radiologic characteristics of plexiform neurofibromas in neurofibromatosis-1 (NF-1) to define a cohort at greatest risk for malignant nerve-sheath tumors. BACKGROUND: Plexiform neurofibromas are a frequent complication of NF-1. They can impair function, produce disfigurement, and be the site for the development of malignant nerve-sheath tumors. The incidence and natural history of plexiform neurofibromas is unknown. METHODS: CT imaging of the chest, abdomen, and pelvis was performed in 91 of 125 consecutive adults (age, > or = 16 years) with NF-1. RESULTS: Twenty percent of patients had plexiform neurofibromas of the chest in the paraspinal, mediastinal, or supraclavicular area. Approximately 40% of patients had abnormal abdominal/pelvic scans. The paraspinal, sacral plexus, sciatic notch, and perirectal regions were the most common sites. Most plexiform neurofibromas were asymptomatic. Imaging also revealed a number of tumors, including malignant nerve-sheath tumors, adrenal tumors, carcinoids, and schwannomas. CONCLUSIONS: The frequency of plexiform lesions and other tumors in NF-1 indicates that clinicians should monitor young adults carefully; however, imaging characteristics alone cannot reliably distinguish benign from malignant lesions.