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Prenatal multicarcinogenesis by ethylnitrosourea in mice.

The Role of prenatal age, sex, and the maternal background upon the incidence, multiplicity, and spectrum of tumors induced by ethylnitrosourea has been studied in the offspring of reciprocal hybrids of the same genotype. The first generation (F1) offspring of C57BL/6J X C3HeB/FeJ and the reciprocal hybrids were observed throughout their life-span for tumor development following single i.p. injections of ethylnitrosourea (60 microng/g) given to pregnant mothers at 12, 14, 16, or 18 days of gestation. Animals exposed to ethylnitrosourea survived on the average for only 90 weeks. They developed by that age tumors in lungs, livers, ovaries, nervous system, and forestomach. Control mice killed at 90 weeks were essentially free of tumors. The fetal age at the time of administration of the carcinogen was one of the most significant modulators of tumor development in lung, liver, ovaries, and nervous system. The sex of the animals influenced the rate of development of liver tumors, whereas maternal background affected the multiplicity of lung tumors.

Animals↗

[Solid tumors in the child. New advances and the importance of histological sub-classifications. II. Tumors of the peripheral nervous system, soft tissue, liver and pancreas].

According to immunohistochemical, ultrastructural features of neural cells, and identical 11; 22 chromosome translocation, at least some extra osseous Ewing's sarcoma, as well as the malignant small cell tumor of the thoracopulmonary region (Askin's tumor) are actually classified as peripheral neuroepitheliomas. Embryonal rhabdomyosarcoma, including the botryoid variant, is now, when treated with appropriate chemotherapy, a tumor of relatively favorable histology. Its prognosis is still primarily related to clinical stage and location of tumor. The alveolar subtype of rhabdomyosarcoma (including its solid variant) has a less favorable prognosis. Hepatoblastoma (epithelial or mixt variants) have the same long term survival, also mainly related to stage. Pancreatoblastoma is a tumor with well differentiated cytopathology and prolonged course, compared with other pancreatic tumors.

Carcinoma, Hepatocellular↗

CT and MR imaging of melanocytic schwannomas; report of three cases.

Melanocytic Schwannomas are rare tumours which can arise in soft tissues, spinal nerve roots and in the central nervous system. The literature suggests that they have a malignant behaviour with local recurrence after surgery. We present three patients with this lesion, two in the thoracic spine and one in the head of the pancreas. The clinical outcome in these cases has been disappointing, since two patients died from complications due to local spreading of the tumour in spite of surgery and radiotherapy. CT could not distinguish these tumours from other neurogenic neoplasms. When these tumours occur near the vertebral body, CT could only detect early bone erosion. MR findings show promising features, with a high signal on T1-weighted images, due to melanin. However, more experience is necessary to establish the possible specific features of melanocytic Schwannomas in MRI.

Aged↗

Neurological manifestations of neoplastic and radiation-induced plexopathies.

Metastatic plexopathy is often a disabling accompaniment of advanced systemic cancer and may involve any of the peripheral nerve plexuses. Brachial plexopathy most commonly occurs in carcinoma of the breast and lung; lumbosacral plexopathy is most common with colorectal and gynecologic tumors, sarcomas, and lymphomas. Regardless of the location, neoplastic plexopathy is often characterized by severe, unrelenting pain. Later, weakness and focal sensory disturbances occur in the distribution of plexus involvement. In previously treated patients, the main differential diagnostic consideration is radiation-induced plexopathy. Treatment of metastatic plexopathy is palliative and includes radiotherapy to the tumor mass, chemotherapy, and symptomatic treatment. In selected cases, subtotal surgical resection of the tumor may be warranted. The response to therapy is modest and generally short-lived. Efforts should be made to provide adequate pain control, maximize remaining neurological function, and prevent complications of immobility produced by the neuromuscular dysfunction.

Brachial Plexus Neuropathies↗

Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases.

BACKGROUND: The epithelioid variant of malignant peripheral nerve sheath tumor (MPNST) is a rare tumor with poor prognosis that sometimes involves the head and neck. The diagnosis is based principally on the histological examination, and it is generally very difficult to reach the correct diagnosis. CASE: An 84-year-old Japanese woman presented with a tumor mass of 2 week's duration in the right medial canthal region. OBSERVATIONS: Although the tumor was excised surgically, metastases occurred three times on her face and head, and the patient died of distant systemic multiple metastases. In the histopathological analysis, the tumor showed a composite pattern comprising spindle or polygonal cells arranged in irregular bands, and a population of larger epithelioid cells in solid sheets and nests. In the immunohistochemical analysis, the tumor cells were positive for S-100 protein, vimentin, and nerve growth factor receptor (NGFR), and negative for cytokeratin and HMB 45 (melanoma-associated antigen). These findings confirmed the diagnosis of MPNST. CONCLUSIONS: Epithelioid MPNST has complex histopathological findings and histopathological features similar to other epithelioid tumors, especially malignant melanoma. Immunohistochemical examination using NGFR and HMB-45 is important in the differential diagnosis.

Aged↗

Perineural invasion by ductal adenocarcinoma of the pancreas.

BACKGROUND AND OBJECTIVES: The correlation between various levels of perineural invasion by pancreatic carcinoma and the patient's prognosis has never been cleared. The authors carried out a histopathologic study of resected pancreatic carcinoma to elucidate the significance of a new histologic finding concerning perineural invasion, which we designated "intrapancreatic, extratumoral perineural invasion (nex)," and also to determine its predictive value for prognoses of patients after surgical removal of the tumor. METHODS: We investigated 90 patients with pancreatic adenocarcinoma who had undergone pancreatic resection. The prognoses of all patients were explored, and correlations between survival and pathologic factors were statistically examined for neural invasion. RESULTS: Nex was found in more than 50% of resected pancreases. A statistically significant association was found between the presence of nex and the grade of intrapancreatic neural invasion or the presence of extra-pancreatic neural plexus invasion. Nex was also found to be associated with patient survival after removal of the tumor. CONCLUSIONS: Nex appears to be an element predicting pancreatic cancer infiltration to the extrapancreatic nerve plexus and also to be a factor influencing postoperative survival of patients with pancreatic carcinoma.

Adult↗

Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: a clinicopathologic study of 25 patients and 26 tumors.

BACKGROUND: To determine the effects of anatomic site on the presentation and diagnosis of malignant peripheral nerve sheath tumors (MPNSTs) and on the treatment and outcomes of the patients, the authors initiated a study of these tumors at different sites. An earlier report described MPNSTs of the buttock and lower extremity, and the current series analyzes those presenting at intrathoracic (IT) and subdiaphragmatic (SD) paraspinal sites. METHODS: The authors reviewed data on patients with paraspinal MPNSTs who were seen at Memorial Hospital during the period 1960-1995 and for whom histologic slides were available. Various clinicopathologic parameters and their effects on patient outcomes were examined. RESULTS: Twenty-five patients with 26 tumors were evaluated. Seven tumors were IT and 19 were SD; 60% of the patients had neurofibromatosis type 1 (NF1). Most patients presented with pain, and a diagnostic delay (of 3 months to 2 years) was often noted. Mean tumor sizes for SD and IT tumors were 14.3 cm and 6.6 cm, respectively. Most MPNSTs were composed of spindle cells in fascicles. Twenty-seven percent exhibited divergent differentiation. Twenty-four tumors were high grade, and a low grade component was identified in 8 tumors. Surgical resection was attempted for 23 tumors (88%), but complete resection was achieved in only 6 cases (23%). Eighty percent of the patients died of their tumors, 2-year and 5-year survival rates were 35% and 16%, and median survival was 8.5 months. Significant prognostic factors were tumor size <5 cm, the presence of a low grade component, and complete tumor resection. CONCLUSIONS: Paraspinal MPNSTs have more aggressive behavior than peripherally located tumors, mainly because of the difficulty encountered in resecting them completely. Prognoses of patients with MPNST at this site appear to be affected by resection status, tumor size, and tumor grade.

Adolescent↗

Malignant peripheral nerve sheath tumor: a fine-needle aspiration biopsy study.

Although the histopathologic features of malignant peripheral nerve sheath tumors (MPNST) have been well-described, the cytologic features are less well-characterized. We retrospectively reviewed 4 aspiration specimens from 3 patients with histologically-proven MPNST. The biopsy specimens were obtained from the primary tumor (1 case), a local recurrence (2 cases), and a pulmonary metastasis (1 case). The patients were women, aged 42, 61, and 70 yr. The primary tumors arose from the left neck and elbow, and from a neurofibroma in the right medial thigh. The latter patient had von Recklinghausen's disease. Cardinal cytomorphologic features included aggregates and single cells with ovoid to elongated, and comma to serpentine shapes, as well as hyperchromatic nuclei surrounded by delicate, tapering cytoplasm. Nuclear pleomorphism ranged from slight to marked. Multinucleated giant cells were seen in 2 cases. In general, the degree of nuclear variability within each individual case exceeded that typically observed with synovial sarcoma or fibrosarcoma. Although the cytomorphologic features may strongly suggest MPNST, clinical correlation is necessary and immunocytochemistry (i.e., S-100 protein) may help to distinguish MPNST from other spindle-cell sarcomas. Origin from a nerve trunk or preexisting neurofibroma, or occurrence in a patient with von Recklinghausen's disease, confirms the diagnosis of MPNST.

Adult↗

Peripheral nerve tumors involving paranasal sinuses: a case report and review of the literature.

A 45-year-old man, afflicted with von Recklinghausen's disease, with symptomatic schwannomas and plexiform neurofibromas involving the right maxillary antrum, a rare site for peripheral nerve tumors, is presented. After a literature review, 15 cases were considered adequate for a clinicopathologic study. Twelve schwannomas, 2 plexiform neurofibromas, 2 neurofibromas, and 1 probable malignant schwannoma were included. Epistaxis was common in tumors of the ethmoid sinus and nasal fossae, while pain was related to lesions of the maxillary sinus. Two benign schwannomas recurred and were cured by surgery alone. The importance of early diagnosis and thorough surgical excisions is emphasized, especially for those tumors associated with von Recklinghausen's disease. The difficulties involved in the diagnosis of primary malignant peripheral nerve tumors are discussed.

Adult↗

Malignant melanoma with a myxoid stroma: a diagnostic pitfall on fine-needle aspiration biopsy.

Malignant melanoma (MM), both primary and metastatic, may be associated with a prominent myxoid stromal reaction causing diagnostic confusion on fine-needle aspiration biopsy (FNAB), most often with sarcomas that demonstrate a myxoid stroma, particularly malignant peripheral nerve sheath tumor (MPNST). We present a case of a 32-yr-old man with no past medical history who presented with a unilateral neck mass clinically suspicious for lymphoma. FNAB produced a specimen composed of large sheets of anaplastic cells encased in a myxoid stroma that was S100 and vimentin-positive but HMB-45-negative. A diagnosis of MPNST was made. Excision demonstrated a metastatic MM of unknown primary, with a prominent myxoid stromal reaction. A repeat HMB-45 was again negative. Electron microscopy demonstrated intracytoplasmic melanasomes and cisternae of rough endoplasmic reticulum with intracisternal parallel tubules, confirming the diagnosis. Although HMB-45 is typically negative in both tumors, S100 should be strongly positive in myxoid MM and only focal in MPNST.

Adult↗

Actinomycotic granuloma masquerading as an infraorbital nerve neoplasm.

Actinomycotic infections of the head and neck region are rare and frequently present a confusing clinical picture. We document a case of actinomycosis that simulated a neoplasm of the infraorbital nerve, both clinically and surgically. To the best of our knowledge, this is the first report of actinomycosis involving this nerve. Correct diagnosis of actinomycotic infection in the head and neck relies most heavily on clinical suspicion. It is, therefore, imperative for the head and neck surgeon to be familiar with the etiology of this infection and its various modes of presentation as well as its appropriate management.

Actinomyces↗

Peripheral nerve sheath tumors of the paranasal sinuses.

Only 4% of peripheral nerve sheath tumors of the head and neck occur in the paranasal sinuses. The most commonly involved sinus is the maxillary antrum and the most common histologic type is benign schwannoma. Two new cases of schwannoma occurring in the sphenoid and maxillary sinuses are reported. The clinical pathologic, and radiographic features of these lesions are reviewed.

Aged↗