ABSTRACT
BACKGROUND: Sarcomas count among the very rare malignancies of the orbit. Epithelioid sarcomas typically occur in the subcutaneous tissue of younger patients; an affected orbit constitutes a rarity. OBJECTIVES: Only three cases of this extremely rare disease of the orbit, which is associated with marked aggressiveness and a very poor prognosis, have been analysed in the worldwide current literature. PATIENT AND METHODS: We present a case report together with a literature review. A rapidly progressing painful proptosis with visual loss occurred in a 30-year-old patient. Imaging and invasive diagnostic procedures were initiated as a consequence of this. RESULTS: Diagnostic imaging revealed an infiltrative process of the right orbit. Following endonasal decompression of the orbit and probe acquisition, histological and immunohistochemical tests showed the presence of a proximal type epithelioid sarcoma. Therapy consisted of orbit exenteration together with implantation of titanium miniplates for orbital prosthesis. During the course of disease, osseous metastases developed. The patient died during palliative chemotherapy, 14 months after the initial diagnosis. CONCLUSION: Epithelioid sarcomas are extremely rare, difficult to treat tumours in the head and neck region. The associated mortality rates are high. Aggressive surgical therapy with intensive follow-up is recommended. The prognosis depends upon the resection status.
Subject(s)
Blindness/etiology , Exophthalmos/etiology , Orbital Neoplasms/diagnosis , Sarcoma/diagnosis , Adult , Blindness/diagnosis , Diagnosis, Differential , Exophthalmos/diagnosis , Humans , Magnetic Resonance Imaging , Male , Orbital Neoplasms/complications , Sarcoma/complicationsABSTRACT
Of the 721 documented patients in the German Registry for Adamantiades-Behçet disease (registered charity), 258 were of German and 308 of Turkish descent, along with 30 other countries of origin. The prevalence in Germany is 0.9:100,000. Manifestation of the disease was predominantly in the third decade of life (median age: 26.5 years) and in 10.7% the disease onset was under 16 years of age. The full clinical picture developed on average in 2.9 years (median 3 months). Patients of Turkish descent showed androtopism in contrast to those of German descent (female:male 1.9:1), which was also detected in the whole collective in a ratio of 1.4:1. In 12.4% there was a family history with differences between German and Turkish patients (3.8% versus 14.6%) as well as in patients with disease onset in young and adult age (25.0% versus 7.3%). Most frequent features included oral aphthae (98.5%), cutaneous lesions (81%), genital ulcers (64.7%), ocular manifestations (51.6%), arthritis (52.4%) and positive pathergy test (30.8%). Turkish patients suffered significantly more often from eye manifestations compared with Germans, while in German patients prostatitis/epididymitis and gastrointestinal involvement were more frequently documented. As serious complications arose blindness 6.8%, meningoencephalitis 4.0%, severe arthritis 2.6%, fatal outcome 1.2%, hemoptysis 1.1% and gastrointestinal perforation 0.5%. The HLA-B5 antigen was positive in 58.1% and showed an association with eye manifestations. The relative risk of HLA-B5 positive individuals is high in both Germans (6.57) and Turks (5.81).
Subject(s)
Behcet Syndrome/diagnosis , Behcet Syndrome/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Child , Female , Germany/epidemiology , Humans , Male , Middle Aged , Prevalence , Registries , Risk Factors , Turkey/ethnology , Young AdultABSTRACT
Epidemiological studies demonstrate a prevalence of Adamantiades-Behçets disease (MAB) in the range of 0.12-420 per 100,000 inhabitants with the highest rates in Istanbul, Turkey and the lowest rates in the USA. Ophthalmological data on the prevalence of ocular involvement are limited for MAB in Germany because most epidemiological studies are based on rheumatological or dermatological data. Berlin is the city with the highest number of non-native German inhabitants and its multiethnic character renders it uniquely appropriate for epidemiological studies on MAB. This article summarizes the most important epidemiological data of 140 patients (63 female and 77 male) with a mean follow-up of 6.4 years (range 0.5-22 years) which we have recently published. The mean age was 23 years at the first manifestation and 32 years when the fully developed disease was recorded. The mean age at onset of ocular involvement was 30 years, 56% of patients developed ocular involvement, which was the first manifestation in 8.6% and the second manifestation in 19.3%. More than half the patients developed ocular involvement. The calculated prevalence of ocular involvement in MAB is 1.77/100,000 inhabitants for the population of Berlin.
