Das neurogene Sarkom im Kopf-Halsbereich mit Kontakt zur Sch�delbasis

Abstract

The neurogenic sarcoma is a neoplasm found seldom. It descends from the sheaths of peripheric nerves. If this tumor develops from a solitary or multiple neurofibroma, it might also be called a neurofibrosarcoma. All other synonyms, especially that of the malignant schwannoma, should better be abandoned for histogenetic reasons. Today the classification of all tumors of the peripheral nervous system is based on a suggestion of the WHO from 1969. The tabular summary of the literature shows that only a little more than a hundred cases of neurogenic sarcoma of the head and neck were reported during the last 50 years. Differential diagnosis of this malignant tumor includes nearly all other malignant neoplasms of soft tissue, sometimes even anaplastic carcinoma. Histopathology, including electron microscopy, is described in general and also in detail with regard to three cases. Three forms of micro-morphological variants are dealt with, too. For diagnosis histological examination will be absolutely successful only in cases of neurofibromatosis or in cases where the nerve from which the tumor originates may be identified. Otherwise, conventional and computerized X-ray tomography may be helpful. Finally, some therapeutic and prognostic principles are discussed.

A case report of cervical neurofibrosarcoma: Clinical presentation, treatment and outcome

“…Neurofibrosarcoma is a rare clinical entity most often reported as case report. During the past 50 years, only about a hundred cases of head and neck neurofibrosarcomas were reported [ 4 ], often associated with NF1. The degeneration of a benign tumor into malignancy usually occurs in large and diffuse tumors in NF1 [ 3 ] like in our patient.…”

A rare case of a solitary peripheral nerve sheath sarcoma of the scalp

“…Even then, diagnosis may be difficult and only suggestive but not conclusive in cases where these examinations are not decisive. References in the literature to this type of tumor are very rare [3,9]. From these findings, this tumor is considered to be an extremely rare nerve sheath sarcoma.…”

“…Histological subclassifications, which are practically achieved, reproducible and uniquely predictive, are of great value. As new methods are utilized and new clinical data are reported, an accurate subclassification is of great importance [3,9,11 ], since the importance of histological subclassification lies principally in the correlation between nosology and tumor biology, including response to therapy.…”

“…Perusal of the literature shows that this neoplasm (MPNSS) of the head and neck has been seldom reported during the last 72 years [3,5,6,9]. Since its prognosis is grim and it appears in the young, we report an illustrative case with a review of the current literature in order to determine its prognosis, subclassification, biological behavior and possible treatment regimen [2].…”