[Idiopathic palmar vein thrombosis of the fingers - rare but relevant]. / Idiopathische Thrombosen der palmaren Fingervenen - differenzialdiagnostisch selten aber relevant.

Idiopathic thrombosis of palmar finger veins is rare and women suffer from it almost exclusively. Synovial cysts, epidermoid inclusion cysts, giant cell tumours and haemangiomatous lesions should be considered in the process of diagnosis. We present a 56-year-old woman with idiopathic and symptomatic thrombosis of palmar finger veins. Using the palmar approach the painful veins were identified and excised completely. An uncomplicated wound healing has followed with completely unrestricted and painless range of motion. Surgical excision of the finger vein thrombosis should be considered if there is continuing pain.

[A young patient with paraganglioma syndrome type I. An interdisciplinary challenge]. / Junger Patient mit Paragangliomsyndrom Typ 1. Eine interdisziplinäre Herausforderung.

A 33-year old patient presented at our outpatient department with pulse-synchronous tinnitus in the left ear of several years' standing. Examination of the left ear showed a neoplasm at the base of the meatus in front of the ear drum. In addition, there was paresis of the right hypoglossal nerve and right vocal cord. CT scan showed multiple paraganglioma in the head and neck region. Molecular genetic screening analysis showed a mutation of the SDHD gene and thus the diagnosis of paraganglioma syndrome type 1 was made. In the treatment concept presented here the main goal was preservation of function of the left cranial nerves due to the functional loss of the right cranial nerves X and XII. This goal could be achieved by means of a staged procedure in collaboration with radiotherapists.

[Salivary gland tumors: pathology and prognosis]. / Speicheldrüsenkarzinome: Pathologie und Prognose.

Due to their infrequency, diagnosis of salivary gland tumors remains a challenge for the practicing pathologist with respect to subtyping, grading and estimating patient prognosis. In a 3 center collaborative study of 288 cases with clinical follow-up information, it could be demonstrated that apart from clinical parameters like high patient's age, male sex and high tumor stage, the application of a 3-tiered grading system incorporating the histological tumor type is of high prognostic relevance. In immunohistochemical and molecular analyses overexpression of epidermal growth factor receptor (EGFR), high expression of human epidermal growth factor receptor 2 (HER2), amplification and gains of EGFR and HER2, absent expression of C-KIT, loss of maspin and of O(6)-methylguanine DNA-methyltransferase (MGMT) as well as MGMT promoter methylation turned out to be negative prognostic factors. In mucoepidermoid carcinomas the presence of a translocation t(11;19) was less relevant than recognizing different subtypes (classical type, eosinophilic, clear-cell and squamoid variants). Acinic cell carcinoma with mixed acinar-ductular differentiation as judged by expression of CK7 was characterized by a higher frequency of early relapse. The presented results underline the pathologist's role in the diagnosis and management of patients with salivary gland carcinomas.

[Hereditary head and neck tumors]. / Hereditäre Tumoren des Kopf-Hals-Bereichs.

Hereditary paraganglioma, Gorlin-Goltz syndrome and Fanconi anemia are among the rare hereditary tumor syndromes of the head and neck. Patients with hereditary paraganglioma often develop multiple tumors of the glomus caroticum and glomus jugulotympanicum. The corresponding genetic defects of the mitochondrial succinate dehydrogenase complex induce autonomous tumor cell growth. In patients with Gorlin-Goltz syndrome basal cell carcinomas and keratocystic odontogenic tumors usually occur much earlier than in patients with sporadic tumors. The associated germline mutations are located in the patched gene which is a modulator of the cell cycle. Fanconi anemia represents a chromosomal instability syndrome which is characterized by early onset of pancytopenia, i.e. bone marrow failure and subsequent development of acute myeloid leukemia and/or squamous cell carcinomas, especially of the head and neck. A total of 13 different gene clusters have been identified in 2 DNA associated complexes which play an important role in DNA repair mechanisms.