Preoperative symptoms and postoperative sequelae of intrathoracic neurogenic tumors: a single institution's experience.

OBJECTIVE: Most intrathoracic neurogenic tumors are resected for therapeutic diagnosis; many adult tumors are benign. However, few studies have reported the preoperative symptoms, postoperative modalities, and sequelae of these tumors. We focused on and evaluated the diversity and postoperative prognosis of these tumors. METHODS: We assessed 31 consecutive cases of intrathoracic neurogenic tumors resected at Tsuchiura Kyodo General Hospital between 1988 and 2012. Two cases involved multiple tumors; therefore, complete resection or enucleation was performed only in the remaining 29 cases. The patients' clinical records were investigated retrospectively. RESULTS: All tumors were benign. Five cases (16.1 %) presented with preoperative symptoms; 2 cases with non-neurologic symptoms (dysphagia due to tumor oppression and a massive hemothorax with neurofibromatosis type 1) improved after surgery, but 3 others with neurologic symptoms (back pain, hand motor paralysis, and Horner's syndrome) did not. Ten cases (32.3 %) presented with postoperative modalities or sequelae. Eight cases presented with neurologic sequelae (Horner's syndrome, 4 cases; grip weakness, 3 cases; hypohidrosis, 3 cases; and hand numbness, 2 cases). All the patients presenting with neurologic sequelae had tumors proximal to the first or second thoracic vertebra; no tumors proximal to or under the third thoracic vertebra caused neurologic sequelae. Severe neurologic sequelae in daily life were observed in 2 cases, but they did not radically improve. CONCLUSIONS: While the non-neurologic symptoms caused by intrathoracic neurogenic tumors can be resolved by resection, the neurologic symptoms may not improve. Tumors proximal to the first or second thoracic vertebra can cause postoperative neurologic sequelae.

Spinal tumors in children.

Pediatric spine tumors encompass a diverse group of pathologic diagnoses that differ markedly based on the location and age of the child. Children can be affected by primary and metastatic tumors, making the differential diagnosis and treatment options extensive. This article discusses the features of spinal tumors in children based primarily on location: extradural, intradural-extramedullary, and intramedullary tumors. Because this article deals with such a broad topic, detailed descriptions and outcomes of surgical and nonsurgical treatments for each particular tumor are limited. Rather, the key clinical, diagnostic, and therapeutic features of each tumor are discussed.

Role of cerebrospinal fluid diversion in posterior fossa tumor surgery.

OBJECTIVE: To assess the role of cerebrospinal fluid diversion in posterior fossa tumor surgery. DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: This study was conducted at the Department of Neurosurgery, Pakistan Institute of Medical Sciences (PIMS), Islamabad from February 2000 to July 2002 over a period of two and-a-half years. SUBJECTS AND METHODS: Clinical, radiological and operative records of the patients who were operated for posterior fossa tumors were studied. Absolute and relative frequencies of the patients who were managed with external ventricular drainage (EVD) or ventriculoperitoneal shunt (VPS) were determined. Mean age and male to female ratio were also noted. RESULTS: There were 48 patients who were operated for posterior fossa tumors. Mean age was 23 years. Male to female ratio was 1.2:1. VPS was done in 14 patients (29%) pre-operatively, in one patient (2%) per-operatively and in 2 patients (4%) postoperatively. EVD was done in 33 patients out of whom 2 patients were shunted post-operatively. Sixty-five percent of the patients remained shunt-free. CONCLUSION: Although management of hydrocephalus secondary to posterior fossa tumors is controversial, majority of the patients need temporary cerebrospinal fluid diversion

[Mixed neuroglial tumor: description of a case]. / Tumore misto neurogliale: descrizione di un caso.

A case of neuroglial tumor in a 18-year-old man is presented. The neoplasm was composed by two cell types. One type showed features typical of neuronal cells, while the other resembled glial cells. The diagnosis was confirmed by immunohistochemistry results.

Angiosarcoma arising in a solitary schwannoma (neurilemoma) of the sciatic nerve.

Angiosarcomas rarely develop within a peripheral nerve or a peripheral nerve sheath tumor. We describe an epithelioid angiosarcoma that arose in a benign schwannoma (neurilemoma) of the right thigh in a 65-year-old man who did not have von Recklinghausen's disease. Histologically, the resected tumor was a high-grade undifferentiated sarcoma that was predominantly arranged in solid sheets or nests and composed of epithelioid cells. The endothelial origin of the tumor was suggested by Factor VIII R-ag, Ulex europaeus-I, CD34, CD31, BNH9, and vimentin immunoreactivity, along with the ultrastructural evidence of occasional Weibel-Palade bodies. In this location, epithelioid angiosarcoma should be distinguished from malignant transformation of a schwannoma with epithelioid changes. This observation stresses the importance of immunohistochemical and ultrastructural analysis in the differential diagnosis of vascular tumors with features of epithelioid sarcoma.

Tumors in Rubinstein-Taybi syndrome.

The 14 tumors reported in Rubinstein-Taybi syndrome since 1989, when added to the 22 previously reported, are beginning to show a pattern of neural and developmental tumors, especially of the head, which is malformed in the syndrome. Among the neoplasms were 12 of the nervous system: 2 each of oligodendroglioma, medulloblastoma, neuroblastoma, and benign meningioma, a pheochromocytoma, and 3 other benign tumors; 2 of nasopharyngeal rhabdomyosarcoma; and 1 each of leiomyosarcoma, seminoma, and embryonal carcinoma. Among the other benign tumors were an odontoma, a choristoma, a dermoid cyst, and 2 pilomatrixomas.

Neurogenic tumors of the thorax.

We have conducted a retrospective review of 134 cases of neurogenic tumors of the thorax, including 66 in infants and children and 68 in adults. Nerve cell tumors were the majority in infants and children (84.8%) and were mostly malignant (67.2%). Nerve sheath tumors were more frequent in adults (73.5%). Nerve cell tumors were the most frequent tumors in males and nerve sheath tumors in females. Nineteen tumors were associated with von Recklinghausen's disease. The tumors were symptomatic in 76.4% of children and 36.7% of adults. Spinal cord compression was observed in 8 infants and children and in 2 adults. Neo-adjuvant treatment was administrated to 3 patients. Resection was complete for 80 benign tumors out of 86 (93%) and for 26 malignant tumors out of 48 (54%). Postoperative chemo- or radiotherapy (or both) was administrated to 17 children and 8 adults. The mean followup periods were 11 years for the infants and children and 8 years, 6 months for the adults. There was one postoperative death. There were no late deaths related to benign tumor. The prognosis of spinal cord compression depended on the malignancy and staging of the tumors. At 5 years postoperatively, 21 children out of 28 with neuroblastomas and 8 out of 9 with ganglioneuroblastomas were alive. The possibility of maturation of neuroblastomas and survival with hepatic metastases was confirmed. The prognosis in cases of chemodectoma depended on the extension. Patients with malignant schwannomas had very poor prognoses, especially when associated with von Recklinghausen's disease.

Heterotopic neuroglial tissue of the face. Report of six cases and review of the literature.

We report six cases of heterotopic neuroglial tissue of the face. All cases appeared at birth with a laterofacial mass and, in most cases, a respiratory disorder. The initial diagnosis has frequently been lymphangioma. But the rapidly growing character of the mass with resistance to high dose corticoid therapy, the fluid aspect at computerized tomography imaging with no connection to the central nervous system, and the presence of cerebrospinal fluid at the needle puncture were the most helpful features in narrowing the differential diagnoses. Patients were treated by surgical removal of the mass. The pathologic study showed the presence of neuroglial tissue. The management of the patients is discussed. The thirteen previous cases reported in the literature and our six cases lead to a discussion on the embryologic origin of this rare entity--the cephalic neural crest.

Cytology of malignant schwannoma metastatic to the lung. Report of a case with diagnosis by fine needle aspiration biopsy.

The use of fine needle aspiration biopsy (FNAB) to diagnose lung tumors has been very successful in regard to epithelial neoplasms. However, there is much less experience in diagnosing primary and metastatic sarcomas of the lung with this technique. We describe an unusual case of malignant schwannoma metastatic to the lung diagnosed by FNAB in a young female with von Recklinghausen's disease. The usefulness of this technique and the additional aid of S-100 staining and electron microscopic studies are discussed.

Pancreatic stromal tumor of nerve sheath origin treated by pancreatoduodenectomy.

A pancreatic sarcoma of nerve sheath origin is reported in a 28-year-old female patient, who presented with melaena. Preoperative imaging showed an 8.5 cm diameter mass in the head of pancreas. There was bleeding from the papilla of Vater at endoscopy and a highly vascular lesion on arteriography. The patient was submitted to proximal pancreatoduodenectomy and remains symptom-free at 1 year follow-up.

Painful ophthalmoplegia caused by neurotropic malignant melanoma.

Progressive cranial nerve palsies and painful ophthalmoplegia developed in a 76-year-old man with lentigo maligna of the forehead 18 months after the excision of a spindle cell tumour of the forehead that proved on immunohistochemical study to be a malignant melanoma. The signs and symptoms were caused by neurotropic spread of the malignant melanoma into the cavernous sinus and subsequent neurosarcomatous transformation of the orbital metastasis.

Neuroblastoma and DiGeorge anomaly.

DiGeorge anomaly is a rare disorder felt to be a complex neurocristopathy. We report the unusual association of congenital neuroblastoma arising in an infant with the DiGeorge anomaly.

Central nervous system lymphomas and immunodeficiency.

Primary lymphomas of the central nervous system (CNS) may sometimes be associated with some immunological abnormalities, including renal or cardiac transplants, some congenital and acquired immunodeficiencies, immunoinflammatory diseases and immunosuppressive treatments. A relatively high incidence of cerebral lymphomas has been particularly noticed in renal or cardiac transplantation patients and in those with acquired immune deficiency syndrome (AIDS), two conditions which are today observed with increasing frequency. The different congenital and acquired immunodeficiencies associated with cerebral lymphomas and the pathogenetic connections between the two conditions are discussed from a large review of the literature.

Optic glioma with progressive occlusion of the aqueduct of Sylvius in monozygotic twins with neurofibromatosis.

Monozygotic twins sisters with optic glioma "in mirror image" (one with involvement of the left optic nerve and the other with the right optic nerve) and hydrocephalus secondary to progressive stenosis of the aqueduct have been found in a series of 128 cases below 14 years of age with neurofibromatosis. The optic glioma was diagnosed in each of the twins at 2 years of age. In one twin the tumor involved only the optic nerve but in the other the glioma affected the optic nerve and spread to the homolateral zone of the optic chiasm. First symptoms of hydrocephalus appeared at 8 years and 11 years of age respectively but ventriculo-peritoneal shunting procedures were performed to relieve intracranial hypertension at 11 years and 15 years of age respectively. At 2 years of age both twins had pneumoencephalography which demonstrated normal air passage through the aqueduct and cerebral ventricles of normal size and morphology. Posterior studies with CT-scan demonstrated progressive obstruction of the aqueduct with very slow progression of the hydrocephalus in each twin, although it was not observed simultaneously. The increased intracranial pressure was tolerated for many years in each twin without obvious symptoms which could be attributed to the slow progression of the aqueduct obstruction.

Chronic syndrome of inappropriate secretion of antidiuretic hormone in childhood.

Chronic forms of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) occur infrequently in childhood. We describe three pediatric patients with chronic SIADH. In two patients the persistent SIADH was associated with neoplasms of the central nervous system while in the third patient it was due to congenital anomalies of the central nervous system. We describe the clinical course and management of each patient, including the long-term use of furosemide taken orally once a day.