Perineurioma with ossification: a case report with immunohistochemical and ultrastructural studies

We evaluated the elbow flexion test in 216 elbows without compression of the ulnar nerve at the cubital tunnel and without other neuropathies. We used Rayan's four positions as our test. The percentage of positive tests was only 3.6% at one minute, whereas evaluating the responses at three minutes we saw positive results in 16.2%. Therefore we find that if the test is performed for one minute it may be useful to help in diagnosing ulnar nerve compression at the cubital tunnel.     

Malignant peripheral nerve sheath tumor: analysis of treatment outcome

Although previous studies have examined the functional role of the neurons in the area ventrolateral to the hypoglossal nucleus (perihypoglossal neurons) in the trigemino-hypoglossal reflex, no convincing evidence for the direct connection from the perihypoglossal neurons to the hypoglossal motoneurons has yet been provided. In addition, the role of the perihypoglossal neurons in swallowing has not been studied. The purpose of this study was to investigate (1) the input-output relationship of the perihypoglossal neurons and (2) whether the afferent feedback was essential for their swallowing-related activity in chloralose-anesthetized cats. Before and after the cats were paralyzed, single-unit activities were recorded extracellularly from 30 perihypoglossal neurons during swallowing elicited by electrical stimulation of the superior laryngeal nerve. These perihypoglossal neurons responded with spike potentials after short latencies to stimulation of the inferior alveolar and hypoglossal nerves. The neurons also responded with spike potentials to single shocks applied to the superior laryngeal nerve, but were activated transiently at the initial phase of repetitive stimulation of the nerve and kept silent until the occurrence of swallowing before and after the animal was paralyzed. They showed burst activities in coincidence with swallowing. Averaging of intracellular potentials of a hypoglossal motoneuron by simultaneously recorded extracellular spikes of a perihypoglossal neuron revealed monosynaptic inhibitory post-synaptic potentials. We conclude that, in the region ventrolateral to the hypoglossal nucleus, there are neurons which relay trigeminal, hypoglossal, and vagal afferents. Furthermore, some of these perihypoglossal neurons are inhibitory hypoglossal premotor neurons that are involved in the central programming of swallowing.     

Primary lung neoplasm of rare incidence: giant cell carcinoma

Primary giant-cell carcinoma of the lung is an infrequent observation. It is distinguished clinically solely its fairly rapid development but otherwise it does not seem to have any predilection for localization, age or sex. Histologically, differential diagnosis with othe tumours such as angiosarcoma, rhabdomyosarcoma, chorioepithelioma and others is of interest. Three cases of primary giant-cell carcinoma of the lung are reported and the various clinical and anatomopathological aspects discussed.     

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.     

Dermoid cyst of the pancreas: a rare cystic neoplasm

Dermoid cysts of the pancreas, also called cystic teratomas, are a rare entity and are included in the group of neoplasms with a germ cell origin. Only twelve cases have been described in the world literature. The symptomatology is due to tumor compression of the neighboring tissues. Ultrasonography and computed tomography may be helpful, but there are no pathognomonic data for their preoperative recognition. The differential diagnosis should include all other cystic tumors of the pancreas. Complete surgical removal is mandatory. We describe the diagnostic and surgical procedures in a 74 year-old man with a pre-operatively unsuspected dermoid cyst. We review the previously published cases and emphasize the appropriate therapeutical management.     

Malignant peripheral nerve sheath tumors: the St. Jude Children's Research Hospital experience

BACKGROUND: Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon in young patients. To contribute to the understanding of these tumors, we reviewed the records of all patients treated for PNSTs at one institution over a 30-year period. METHODS: We reviewed the records of eight patients treated for benign PNSTs and 28 patients treated for 29 MPNSTs. We focused on the latter group, statistically testing several clinical factors for their significance in affecting survival. RESULTS: Five-year survival in patients with MPNSTs was 39%. The most significant prognostic factor was gross tumor resectability (p = 0.0004). Five-year survival for patients with resectable tumors was 65%, whereas no patient with unresectable disease survived > 25 months. Tumor grade, site, and patient race were also significant factors by univariate analysis but were not significant when adjusted for resectability. CONCLUSION: Gross tumor resection is crucial in treating malignant PNSTs. Supplemental radiation therapy is recommended for positive microscopic margins. More effective treatment is still being sought for unresectable disease.     

Transitional cell neoplasm of the bladder in childhood: presentation of a clinical case

The authors report their experience with the use of loco-regional anaesthesia for the surgical treatment of inguinal and femoral hernias. Their series includes 1730 operations, 235 of which for hernia relapses, performed exclusively in the outpatient department. In the authors opinion, with loco-regional anaesthesia it is possible to treat any type of inguinal-femoral hernia, reducing the risks related to total anaesthesia and the complications arising from bedrid. Excellent results were achieved especially in the elderly and high risk patients like cardiopathic or those affected by respiratory and hepatorenal insufficiency with a complete absence of mortality and morbidity.     

Liver hamartoma in an adult: report of a rare case

Liver hamartoma is a rare type of benign tumor which usually occurs in the first few years of life. Although it is the second most common benign tumor in childhood, only about 100 cases have been reported in the English literature. Thus, it is extremely rare to encounter liver hamartoma in adults. This report details the clinical presentation and surgical management of a 24-year-old woman with a rapidly growing liver hamartoma. A right hepatic lobectomy was successfully performed and the patient was discharged in good health on the 10th postoperative day.     

Primary papillary meningioma of the optic nerve sheath: a case of unique location and benign pathology

I report here a case of primary papillary meningioma of the optic nerve sheath which developed in the left eye of a 54-year-old woman over a period of 20 years. A review of 123 primary papillary meningiomas, including the current instance, disclosed that they tended to occur in young patients of both sexes at an average age of 35 years, with one-fourth of them occurring in the first two decades of life, and with a female/male ratio of 3:2. These meningiomas were most frequent in the supratentorial compartment of the cranial cavity, especially on the cerebral convexity and in the parasagittal region. They were uncommon subtentorially, intraspinally, and outside the central nervous system. Most reported papillary meningiomas were claimed to be malignant, but the tumor of this particular woman was benign pathologically and clinically.     

Closed rupture of a finger extensor following the Sauvé-Kapandji procedure: a case report

A case of closed rupture of the ring extensor digitorum tendon following performance of the Sauvé-Kapandji procedure is presented. This complication is not rare following performance of the Darrach procedure. The operative findings indicated that the rupture was caused by an impingement of the tendon between the proximal stump of the ulna and the extensor retinaculum. The patient was successfully treated with shortening of the proximal ulnar stump and tendon transfer.     

A resected case of malignant peripheral nerve sheath tumor (MPNST) necessary to distinguish from Askin tumor

The aim of this study was to compare the morpho-functional modifications of the right cardiac sections of the athlete's heart, with those of sedentary healthy control subjects. We studied 24 endurance athletes (mean age 28.17 +/- 7.28 years), 21 power athletes (mean age 25.86 +/- 4.96 years), and 20 sedentary healthy control subjects (mean age 33.22 +/- 6.67 years). We examined the right cavities by standard echocardiographic projections and the following parameters were evaluated: right ventricular longitudinal diameter; under tricuspid valve and medium ventricular transversal diameter immediately under the tricuspid plane and at medium ventricular level; right atrial transversal and longitudinal diameters. All parameters were corrected for body surface area. Our data showed that the right ventricle presents morphological adaptations to endurance exercise; modification is represented mainly by an increase in the mean transversal ventricular diameter with a consequent reduction in the transversal/longitudinal diameter ratio accompanied by modification of the ventricular geometry. In addition the data showed an increase in longitudinal and transversal diameters of the right atrium. On the contrary, the power athletes did not show statistical modification of the right ventricle and atrium. The different modifications of the right heart side diameter are probably due to the different hemodynamic loading, which is involved in the endurance and power training respectively.     

Granular cell tumor of the duodenum: a case report

Granular cell tumor (GCT) in the duodenum is an extremely rare disease: only one case has been listed in a review, to date. We reported a 47-yr-old Japanese male case with GCT of the duodenum. Clinically, melena caused by bleeding from the tumor was the only symptom. The tumor cells showed abundant, granular eosinophilic cytoplasm. Although this tumor was clinically and histologically benign, highly developed tumor microvessels were demonstrated both angiographically and histologically, suggesting malignant potential of the tumor.     

Langerhans cell histiocytosis of the clavicle: a case report

A case of Langerhans cell histiocytosis in a 47-year-old male presenting as an aggressive appearing lesion of the clavicle is reported. It illustrates the difficulties of the radiological diagnosis of a solitary bone lesion.     

Formation and maintenance of the myelin sheath in the peripheral nerve: roles of cell adhesion molecules and the gap junction protein connexin 32

Based on previous in vitro studies, the cell adhesion molecules L1, N-CAM, MAG, and P0, which all belong to the immunoglobulin (Ig)-superfamily, have been suggested to mediate myelin formation in the peripheral nervous system. Unexpectedly, studies in mice deficient for the corresponding molecules revealed that only P0 plays pivotal roles during the formation of peripheral nerve myelin in vivo, while L1-, N-CAM-, and MAG-deficient mice develop myelin of normal ultrastructure. However, MAG turned out to be important for the maintenance of myelin, as reflected by degeneration of myelin and axons in MAG-deficient mice older than 6 months. The MAG-mediated maintenance of myelin is backed up by N-CAM, since mice deficient in both MAG and N-CAM show an earlier and more prominent myelin degeneration than MAG single mutants. Another peripheral nerve component involved in the maintenance of myelinated fibers is connexin 32 (Cx32), a gap junction channel protein that does not belong to the Ig-superfamily. Mice deficient in Cx32 initially form normal myelin, which then develops blown-up periaxonal collars and abnormally shaped non-compacted regions followed by myelin and axonal degeneration. Our findings strongly support the view that very few myelin components are necessary for myelin formation whereas the maintenance of myelin is much more sensitive to molecular alterations. In addition, it became evident that myelin molecules can fulfill functionally overlapping roles that ensure that myelination takes place even under conditions in which there is a deficiency in the normal molecular components of myelin.     

A rare case of hypoglossal nerve palsy complicating a head injury

We describe a 68-year-old man who showed a photosensitive drug eruption induced by flutamide. The minimal erythema dose with ultraviolet A light was reduced to 2 J/cm2 under administration of flutamide, which recovered to over 16 J/cm2 after cessation of the medication, without changing the reactivity to ultraviolet B. The absorption spectrum of flutamide was not altered after ultraviolet A irradiation, suggesting that flutamide is photostable with regard to ultraviolet A. In addition, bovine serum albumin was not covalently photocoupled with flutamide under ultraviolet A. Thus, flutamide seems to have low potency to act as a photohapten, and it is likely that a non-photohaptenic mechanism operates in this photosensitivity or its active metabolite may work as a photosensitizer.     

Anomalous relationship of the facial nerve and the retromandibular vein: a case report

A 72-year-old man suffering from non-Hodgkin's lymphoma is described. After failing to respond to chlorambucil-prednisone regimen for three months, he was treated by splenic irradiation (total dose 500 cGY) in 10 consecutive daily doses. Two days after the last irradiation, the patient developed acute tumor lysis syndrome (TLS) with extreme blood levels of uric acid (33.7 mg/dl), phosphorus (13.7 mg/dl), potassium (6.08 mEq/l), and calcium (6.8 mg/dl). It should be remembered that the acute TLS, which usually occurs following chemotherapy, can also be radiation-induced.     

Granulomatous reaction in peripheral T-cell proliferative disease: a case report

A 52-year-old male presented with a prolonged fever, anemia, weight loss, hepatosplenomegaly, and elevated serum level of alkaline phosphatase. The patient developed septicemia, metabolic acidosis, and died 3 days after admission. Liver necropsy, tissue showed group C, peripheral T-cell proliferative disease. Epstein-Barr virus genomes were demonstrated in the nuclei of these abnormal T-cell lymphocytes. Granulomatous reaction was observed in the liver and bone marrow. Stains and cultures for mycobacteria and fungi gave negative results. Granulomatous reaction is believed to be induced by cytokines which were released from the T-cell lymphocytes that infiltrated in the liver and bone marrow.