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.     

Small round and spindle cell sarcoma with neuronal differentiation and oncocyte-like features of the thoracic wall: a case report with histological, immunohistochemical and ultrastructural examinations

A case of small round and spindle cell sarcoma with neuronal differentiation and oncocyte-like features is presented. The tumor was encountered in a 32 year old Japanese woman with an initial presentation of palpable tumor in the left lateral region of the thorax. The resected tumor was a partially well encapsulated whitish medullary one and consisted of small round and spindle tumor cells, together with so-called rhabdoid cells in the small round cell area. Although pseudorosettes were often observed, true rosette formation could not be detected anywhere. Ultrastructurally, despite a histologic variety of tumor cells, most tumor cells possessed numerous mitochondria, some of which occasionally contained abnormal filamentous or crystalloid structures. Various amounts of microfilaments were present in most tumor cells and microtubules were present in a few. A minority of small round cells possessed a small number of neurosecretory granules, especially in short cytoplasmic processes. A positive immunoreaction for neuron specific enolase was found by immunohistochemical examination in several small round tumor cells and for neurofilaments in lesser numbers. Despite the lack of S-100 protein, MB2 was detected in both small round and spindle cells. On the basis of these findings, the tumor of the present case corresponds to malignant peripheral nerve sheath tumor with neuronal differentiation and oncocytic features.     

Lipoteichoic acid as a target for antimicrobial action

Fifteen patients with unilateral functioning arteriovenous fistula were assessed clinically and electromyographically to identify local neurologic changes. Ten of the patients were symptomatic (motor and/or sensory) and 5 were asymptomatic. Clinically, 11 patients had signs of a mild polyneuropathy, 2 patients of ulnar neuropathy, and 1 patient had signs of median neuropathy. A decrease of the above-elbow ulnar conduction velocity was noted in the study group on the side of the functional fistula, and in the symptomatic patients only on the side of the nonfunctional/nonexisting fistula. We suggest that ulnar nerve vulnerability should be taken into consideration during construction of the fistula, as well as during dialysis.     

Ossifying epithelioid hemangioendothelioma

A case of small epithelioid hemangioendothelioma encapsulated by an egg-shaped bony shell composed of newly formed mature lamellar bone is presented. A nodule measuring 10 x 5 x 5 mm was excised from inside of the right cheek muscle of a 46-year-old man. Histopathologically, the nodule consisted mainly of round-to-short spindled epithelioid cells with round nuclei and occasional cytoplasmic vacuoles associated with some foci of osseous metaplasia. Characteristically, egg shell-like lamellar bone enclosed this lesion showing well-circumscribed appearance. The main component cells were immunohistochemically stained positive for factor VIII-related antigen, CD 34, UEA-1, and vimentin, and proved to be vascular endothelial cells. The term ossifying epithelioid hemangioendothelioma is proposed for this unique lesion. This type of epithelioid hemangioendothelioma has not been previously reported to our knowledge, and differentiation from ossifying fibromyxoid tumor of soft parts is considered to be important.     

Aging is associated with a decrease in synaptosomal glutamate uptake and an increase in the susceptibility of synaptosomal vitamin E to oxidative stress

We report a case of epithelioid leiomyosarcoma that developed on the nose of a 55-year-old Korean male over a one year period. The lesion was a pea sized, firm, erythematous, painless nodule with erosion in the center. Histologic examination revealed short spindled cells with blunt-ended nuclei and pleomorphic round to oval epithelioid cells with abundant eosinophilic cytoplasm that were perivascular and densely packed in the dermis. Immunostaining for desmin was negative, although stains for vimentin and smooth muscle actin were both strongly positive.     

"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.     

Gastrointestinal autonomic nerve tumor of the jejunum. Case report and review of the literature

Gastrointestinal autonomic nerve tumor is very rare and it is difficult to distinguish this tumor from other gastrointestinal tumors due to the absence of clinical, instrumental and macroscopic features which allow pre- or intraoperative diagnosis. Our aim was to recognize the characteristic features (preoperative, intraoperative, pathological) that would allow diagnosis of gastrointestinal autonomic nerve tumor. A case of gastrointestinal autonomic nerve tumor of the jejunum is reported. Surgical specimen was routinely processed. Immunohistochemical staining was performed according to modified immunoperoxidase Avidin-Biotin-Peroxidase Complex method. An electron microscopy study was also performed. The tumor mass showed some characteristic pathological findings: histologically, it was composed of spindle cells and epithelioid cells; immunohistochemically, a focal positivity for Neuron Specific Enolase was shown, and finally, ultrastructural examination showed neuron-like cells with long cytoplasmic processes containing microtubules and bulbouns synapse-like structures with dense core neurosecretory-type granules. Preoperatively gastrointestinal autonomic nerve tumor of the jejunum must be considered and treated as a malignant tumor. A correct diagnosis is possible only with immunohistochemical and ultrastructural studies. It is probable that this tumor is more common than previously thought.     

Proximal median and ulnar resections. Results of primary and secondary repairs

PURPOSE OF THE STUDY: Recovery after median and ulnar nerve proximal repair is widely appreciated. The place and time for secondary functional reconstruction remains controversial. MATERIAL AND METHOD: From January 1983 to January 1990, 66 patients suffering from proximal injury of the median or ulnar nerves underwent nerve repair. Forty-five patients had a postoperative follow-up of more than 24 months: 24 isolated ulnar nerve lesions, 12 isolated median nerve lesions, and 9 combined median and ulnar nerve lesions. Ten patients were given a primary microsurgical nerve suture in our department. Thirty-eight patients underwent a delayed or secondary nerve repair of one or both nerves: 8 secondary nerve sutures, and 35 nerve grafts in 31 patients. RESULTS: Muscular strength, sensitivity, motion, and pain were better after primary nerve sutures (when technically possible) or after shortly delayed secondary sutures, although 40 per cent of patients treated with nerve grafts get final "good" or "very good" results. The time between the injury and nerve repair was the most significant prognosis factor. Results of ulnar nerve repairs at the elbow were statistically better with anterior transposition as compared to in situ repairs (p < 0.005). Fourteen patients required secondary functional reconstruction. Tendon transfers were performed at least 24 months after nerve repair. DISCUSSION: Nerve repair of proximal lesion to the median or ulnar nerves depends on the type of injury, but is advised even when delayed. Residual deficit following nerve repair should require functional transfers depending on hand sensitivity and extrinsic function.     

Surgical treatment of the ulnar nerve entrapment neuropathy: submuscular anterior transposition or simple decompression of the ulnar nerve? Long-term results in 79 cases

The surgical treatment of the ulnar nerve entrapment neuropathy at the elbow is controversial. None of the presently advocated procedures (simple decompression of the ulnar nerve, medial epicondylectomy, subcutaneous or submuscular anterior transposition of the ulnar nerve) has proven optimal regarding long-term results. We studied the outcome in 79 patients whose ulnar nerve had been operated on for the first time, either by simple decompression (31 cases) or by submuscular anterior transposition (48 cases). The mean follow-up was 76 months. Patients were classified according to McGowan pre- and postoperatively; we also applied a more detailed scoring system of our own. Preoperatively, the patients were distributed almost equally between the three McGowan classes. Postoperatively, about one out of three patients in both treatment groups experienced a distinct improvement, i.e. was upgraded to a better McGowan class. Using our own scoring system, the overall rate of objective improvement was 73% after transposition and 55% after simple decompression. Irrespective of the surgical method, roughly 90% of the patients considered their postoperative condition to be improved. However, one specific group of patients (people with habitual ulnar luxation or subluxation of the ulnar nerve) experienced a distinctly better result when treated by anterior transposition than by simple decompression. Our results show that simple decompression of the ulnar nerve can be recommended in all patients without cubital (sub)luxation of the nerve, whereas people with a tendency of cubital (sub)luxation of the ulnar nerve should be treated by submuscular anterior transposition.     

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma)

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle-aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer-like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and "pericytoma." The epithelioid leiomyoma, the benign form, often arises in the mid- and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, assoicated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.     

Compression of the ulnar nerve in Guyon's canal by a traumatic aneurysm. A case report

A patient developed after an occupational trauma with fall on the ulnar edge of his right hand pain and swelling in the ulnar area with reduced sensation in the fourth and fifth finger. After examination performed by a neurologist, ulnar nerve-entrapment was diagnosed and operative treatment was indicated. The release of the ulnar tunnel showed a traumatic aneurysma of the ulnar artery compressing the superficial branch of the ulnar nerve. The tumor was removed and end-to-end anastomosis ensued. Four weeks after surgery, examination showed complete recovery. Various reasons of ulnar tunnel syndrome are discussed.     

Urological aspects of intra-abdominal desmoplastic small round cell tumor of childhood: a preliminary report

To our knowledge intra-abdominal desmoplastic round cell tumor has not previously been described in the pediatric urological literature. This lesion has only recently been recognized as a clinicopathological entity with predilection for adolescent boys. We report on 2 patients who presented with urological symptoms and a large abdominal or pelvic tumor that was biopsied at exploratory laparotomy. Complete surgical excision was impossible in both patients, who subsequently underwent multiagent chemotherapy. Histological and immunohistochemical staining are distinctive in this condition. The tumor is associated with a poor prognosis, despite multidisciplinary, multimodal therapy.     

Comparison of dose standard units for drug utilisation studies

Primary malignant melanoma of the esophagus is rare. We identified one patient over a period of 15 years. This patient was a 80 years-old caucasian man. No association was found with tobacco or ethanol use, nor was there a personal or family history of malignant melanoma. Symptoms were related to obstruction. This tumor was polypoid in its upper part and ulcero-infiltrant in its lower part. Histologically the melanoma had epithelioid spindle cells. The neoplasm was immunoreactive for S-100 protein and non reactive with anti-cytokeratins. This patient was treated by Garlock type, esophagectomy with excision of 13 cm of esophagus and 2 cm of stomach. The survival was of only 3 days, because he developed acute respiratory and cardiac disease syndrome and died.     

Glomangiosarcoma of the hip: report of a highly aggressive tumour with widespread distant metastases

Malignant glomus tumour is an extremely rare neoplasm, and only a few cases with local invasive or aggressive features have been described to date. We present a distinctive case of a malignant glomus tumor arising on the skin of the left hip of a 47-year-old woman. The primary tumour was small, with a maximum diameter of 1 cm, but 2 years after the tumour was excised, multiple lung and skin metastases appeared. Post-mortem examination also revealed widespread metastases in almost all of the visceral organs. Histological examination showed that the primary tumour was composed of a sheet-like proliferation of epithelioid round or short spindle cells surrounding small venules. In addition, there was a proliferation of long spindle cells with eosinophilic fibrillary cytoplasm, which mimicked a leiomyosarcoma. The tumour cells were intensely immunopositive for actin and vimentin but were negative for desmin. This is an exceptional case of malignant glomangiomyoma with widespread metastases, which represents a distinct variant of de novo glomangiosarcoma.     

Bisphosphonate therapy of reflex sympathetic dystrophy syndrome

Four cases of post-operative ulnar nerve mononeuropathy are reported. In all the cases a severe sensory and motor loss was strictly limited to ulnar nerve territory. The electrophysiological examination: needle examination, motor and sensory nerve conduction studies and even more somatosensory potential evoked from ulnar nerve after stimulation above elbow allowed to eliminate a lesion at the elbow and to asses the lesion at wrist, arm, axilla or plexus. Full recovery occurred once and partial recovery twice. We considered that these ulnar lesions are neuralgic amyotrophies of Parsonage and Turner according to the epidemiological, clinical, evolutive and electrophysiological data.     

Alloimmune neonatal neutropenia: flow cytometry study of the 1st patient described in Spain with identification of an anti-NA1

We developed a Micro-neurostimulator that is suitable for intracranial electrical stimulation of the facial nerve in excision of acoustic neurinomas. The tips of our Micro-neurostimulator were thinner and finer than those of commercially-available bipolar forceps and therefore it was possible to stimulate tumor tissue and the facial nerve selectively. This facilitated precise excision of tumor tissue from the facial nerve without residual tumor tissue around it. It was emphasized that the shape of the tips of stimulating electrodes and the stimulus intensity were the most important factors in obtaining reliable responses in preserving the facial nerve using intracranial electrical stimulation and evoked electromyographic responses.     

Epithelioid cells from foreign-body granuloma selectively express the calcium-binding protein MRP-14, a novel down-regulatory molecule of macrophage activation

The S-100 proteins MRP-8 and MRP-14 are expressed by cells of the myelomonocytic lineage, either alone or simultaneously during certain stages of cellular differentiation. We demonstrate that MRP-14 but not MRP-8 was detected by immunostaining in the cytoplasm of epithelioid cells on the surface of round coverslips implanted for 14 days into the subcutaneous tissue of mice. Using this experimental model, our laboratory has previously shown that epithelioid macrophages are poor phagocytic cells that release a macrophage-deactivating factor (MDF) in short-term cultures. The full chemical characterization of MDF has not been achieved so far. We provide evidence that the calcium-binding protein MRP-14 was also released by epithelioid macrophages in short-term cultures and that its neutralization from the culture medium after addition of monoclonal antibody anti-MRP-14 abolished the MDF activity of the conditioned medium. Purified or recombinant human MRP-14 but not MRP-8 inhibits the respiratory burst of BCG-activated macrophages. Recombinant mouse MRP-14 also down-regulate macrophage activation in vitro, and PMA does not revert the inhibitory effect induced by MRP-14. It is thus concluded that epithelioid cells from foreign-body granuloma express and release MRP-14 in short-term cultures and that this molecule is endowed with MDF activity.     

The role of pain from veins for the formation of perivenous edema in humans

To test the hypothesis that nociception from veins plays a role in the formation of perivenous edema, we looked at edema along hand veins of humans during painful noxious stimulation in the presence and absence of nerve conduction block. Pain from vascularly isolated hand vein segments was evoked by perfusion with hyperosmolar saline and rated with the help of an electronically controlled visual analogue scale. Perivenous edema measured as changes in skin altitude was continuously recorded by means of infrared reflection. To alternately block the innervation of skin and vein segment we used a perivenous block (vein but not skin numbed), a distal ulnar nerve block (skin but not vein numbed), and a proximal ulnar nerve block (both vein and skin numbed). Without nerve block, hyperosmolar saline always evoked both pain and a continuous increase in perivenous edema to a maximum of 2.0-3.2 mm after 30 min. On painless control perfusions with isoosmolar saline, edema increased slightly (0.2-0.8 mm) to a plateau which was maintained until the end of perfusion. When the vein was denervated by perivenous or proximal ulnar nerve block, hyperosmolar saline evoked a slight increase in edema which resembled that of control perfusions in both extent and time course. On distal ulnar nerve block, which numbed the skin but not the vein, both pain and substantial edema were evoked. These observations show that nociception from veins is a prerequisite for perivenous edema to occur.     

Effect of anxiety on the rate of gastric emptying of liquids

We studied the morphology and distribution of nerve endings in the human triangular fibrocartilage complex using both silver staining and immunohistochemical staining using a protein specific to nerve fibres. Free nerve endings were found in the ulnar side of the triangular fibrocartilage complex, especially in the ulnar collateral ligament, meniscus homologue and the adjacent collagen fibre area of the peripheral part of the ulnar side of the articular disc. Meissner's and Krause's corpuscles were observed in the ulnar collateral ligament and meniscus homologue. The fact that free nerve endings were observed in the meniscus homologue and adjacent collagen fibre area of the peripheral part of the ulnar side of the articular disc suggests that this disc may be a source of wrist pain. The presence of nerve end bulbs in the triangular fibrocartilage complex also suggests a possible role for corpuscles as mechanoreceptors.