1/2. A case of Maffucci 's syndrome with pleural effusion: ten-year follow-up.INTRODUCTION: Maffucci 's syndrome (MS) is a congenital non-hereditary mesodermal dysplasia characterised by numerous mesenchymal neoplasias in the form of enchondromas with secondary bone deformities and multiple soft tissue haemangiomas that may have phlebolitis. CLINICAL PICTURE: A 23-year-old male patient presented with non-productive cough, dyspnoea, chest pain and back pain. Chest X-ray showed unilateral pleural effusion and multiple enchondromas of the ribs. On physical examination, there were mobile, multiple, bluish-coloured lesions probably cavernous haemangiomas on bilateral chest walls. In addition, there were multiple nodular lesions on the extremities especially accumulated on the fingers. The patient was diagnosed as Maffucci 's syndrome according to computed tomography (CT) of the thorax, conventional radiography of the skeletal system, magnetic resonance (MR) imaging, Th4-Th11 intercostal and right upper extremity angiography and physical examination findings. TREATMENT: As the patient rejected any diagnostic intervention, he was monitored with CT. OUTCOME: During the last 6 years of follow-up, the lesion that was detected on the rib adjacent to the basal segments of the left lung showed significant progression and was accepted as chondrosarcoma. CONCLUSION: To our knowledge, this is the first case of Maffucci ' s syndrome with pleural effusion. In this case report, the probable mechanism of pleural effusion was discussed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/2. Cricoid chondrosarcoma presenting as arytenoid hypertelorism.BACKGROUND: Arytenoid hypertelorism (arytenoid cartilages spaced too widely apart) appears to be the most common initial recognizable physical finding of cricoid chondrosarcoma. Nine cases from the Center for voice disorders are presented. With arytenoid hypertelorism caused by cricoid chondrosarcoma, usually the posterior larynx is open. In fact, patients with arytenoid hypertelorism caused by cricoid chondrosarcoma may be aphonic even though the anterior membranous vocal folds make contact during attempted phonation. methods: Between 1991 and 2002, nine patients were diagnosed with cricoid chondrosarcoma. patients' charts and video examinations were retrospectively evaluated for symptoms and the presence of arytenoid hypertelorism on endoscopic evaluation. Two were women, and seven were men, with a mean age of 70 (range 53-72) years at diagnosis. RESULTS: Eight (88%) patients had aphonia or dysphonia caused by arytenoid hypertelorism as their presenting symptom. Six were diagnosed after failed medialization laryngoplasty or other laryngeal rehabilitation surgery. All nine patients had a low-grade tumor. The initial treatment in eight patients was hemicricoidectomy, and one patient had a total laryngectomy. In four cases, a second procedure was needed 1 to 6 years later because of recurrence. Seven patients are alive without clinically significant disease; one is alive with moderate disease, and one has died from an unrelated cause. CONCLUSIONS: Severely dysphonic, elderly patients presenting with arytenoid hypertelorism of unknown cause should be evaluated by fiberoptic laryngoscopy and by computed tomography scan to rule out cricoid chondrosarcoma. Because these tumors behave so benignly, the authors recommend conservative surgery (unilateral hemicricoidectomy) as the diagnostic/treatment modality of choice.- - - - - - - - - - ranking = 0.5keywords = physical (Clic here for more details about this article) |