[Notochordal tumors : Benign notochordal tumors and chordomas]. / Notochordale Tumoren : Benigne notochordale Tumoren und Chordome.

Benign notochordal tumors (BNCT) and chordomas are primary bone tumors of the spine with a predominant localization in the sacrum and clival region followed by the vertebral bodies. Besides the most common variant (NOS [not otherwise specified] with hepatoid or renal carcinoma cell-like differentiation) chordomas with chondroid, and polymorphic to anaplastic morphology are described. An unfavorable variant are pediatric chordomas with a loss of INI-1. BNCT and chordomas are characterized by the following immunohistological profile: vimentin+, cytokeratin+/-, epithelial membrane antigen (EMA)+/-, S100 protein+/-, brachyury+. This profile helps to distinguish these tumors from other lesions such as chondrosarcoma, chordoid meningioma, and metastases of carcinoma.

A Rare Case of Isolated Cerebral Sarcoidosis Presenting as Suprasellar Mass Lesion with Salt-Wasting Hypopituitarism.

Background Sarcoidosis is a systemic disorder of unknown origin characterized by noncaseating granulomas. Clinical symptoms due to central nervous system (CNS) involvement occur in 5 to 7% of all cases; subclinical involvement is more frequent. Sole CNS involvement is very rare. Case Report A 25-year-old man presented with increasing polyuria and polydipsia over 8 weeks. Magnetic resonance imaging (MRI) revealed a supra- and infra-chiasmatic pre-thalamic mass lesion 1.0 × 1.4 × 1.4cm in diameter. Microsurgical biopsy verified a necrotizing noncaseating epithelioid cell tumor indicative for neurosarcoidosis. All symptoms dissolved within 3 months under stringent corticoid therapy. Conclusion Intracranial mass lesions as the primary and only manifestation of neuronal sarcoidosis are rare. Because conservative treatment is safe and effective, surgery is limited to biopsy and the alleviation of pressure-related symptoms to preserve neurologic function.

[Novel molecular aspects of chordomas]. / Neue molekulare Aspekte der Chordome.

Chordomas are rare and slowly growing malignant bone tumors which mostly occur in adults. These bone tumors are characterized by epithelial and mesenchymal aspects. It is suggested that they arise from remnants of the notochord because they are found along the axial skeleton (e.g. clival, spinal and sacrococcygeal locations). It appears that cytogenetic aberrations are not randomly found in this tumor group. Loss of chromosomal material (e.g. 1p, 3p, 10q, 13q and 14q) is more frequently found than gain of material (e.g. 7q, especially 7q33). Several studies demonstrated brachyury expression (T; 6q27) as a possible candidate gene in the oncogenesis of chordomas (e.g. knock down in the chordoma cell line U-CH1). So far therapy consists of complete resection and irradiation, e.g. with carbon ions. Targeting therapy is not yet established in routine protocols but phase II studies with tyrosine kinase inhibitors have shown partial response of tumors and, in some studies stabilization of the disease has been described.

[A rare manifestation of sarcoidosis]. / Seltene Manifestation einer Sarkoidose.

This article reports the case of a 14-year-old boy who was presented in the case conference with symptoms of decreased visual acuity, scintillating scotomas and photophobia. Physical examination revealed right facial paralysis, parotid gland swelling, high fever and poor general condition. Ophthalmoscopy revealed anterior and posterior uveitis including macular edema and chorioretinal infiltrates. Angiography revealed a dense pattern of hyperfluorescent lesions and these observations resulted in the diagnosis of Heerfordt syndrome. Under systemic prednisolone therapy, symptoms were reduced and visual acuity recovered.

[Classification of solid soft tissue tumours by ultrasonography]. / Sonografische Klassifikation solider Weichteiltumoren.

PURPOSE: Malignant soft tissue tumours appear infrequently in comparison to benign lesions. Clinical misdiagnosis leads to inadequate or delayed therapy in many cases of soft tissue sarcoma. The present study explores the question if ultrasonography as a widely-used diagnostic tool allows for a discrimination of benign and malignant soft tissue tumours. MATERIALS AND METHODS: In a prospective study over a period of 8 years 224 histologically ascertained solid soft tissue tumours, thereof 120 sarcomas and 27 aggressive benign lesions were investigated by B-mode and colour Doppler sonography. The echotexture was analysed computer-based using the parameters echogenicity, homogeneity and vascularisation in all lesions. RESULTS: Different tumour groups showed typical patterns of echotexture, which enabled a classification using 6 categories, distinguishing homogenous hyperechoic, heavily inhomogeneous and homogenous hypoechoic lesions, each group linked to an elevated or low vascularisation. Implementation of the proposed classification revealed a sensitivity in the detection of soft tissue sarcomas and aggressive benign lesions of 94.4 % with a specificity of 79.7 % and an accuracy of 89.7 %. CONCLUSION: Ultrasonography allows for a determination of the diagnostic and therapeutic procedure in soft tissue tumours. Due to the fact that soft tissue sarcomas present hypervascularised almost exclusively, predominantly homogenous hypoechoic, rarely homogenous hyperechoic, and aggressive benign tumours present homogenous hypoechoic predominantly, such patterns require a biopsy prior to further surgical therapy. However, in homogenous hyperechoic lesions displaying a low blood flow either a primary resection or a conservative treatment with follow-up examinations can be discussed depending on clinical findings and history of the patient. Although the group of heavily inhomogeneous tumours within our collective consisted of benign lesions exclusively, biopsy should be recommended in theses cases in order to exclude a soft tissue sarcoma.