1/9. Extra-abdominal fibromatosis: a case report.head and neck fibromatosis is a rare disease with a variable clinical picture. We report the case of a 40-year-old woman who had a 1-week history of a painless mass overlying her right neck. histology confirmed a diagnosis of extra-abdominal fibromatosis. Two unusual features of this case were the site of the lesion and its rapid growth. In this article, we discuss the clinical picture of head and neck fibromatosis, its etiology and pathology, and current opinion regarding its treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. fibrosarcoma of the infratemporal fossa in childhood: a challenging problem.fibrosarcoma in childhood is a rare disease. An interesting new case involving the infratemporal fossa in a three-year-old girl is reported. The literature, the symptomatology and the diagnostic features are reviewed. The difficult management decisions and the thorny problem of reconstruction of head and neck malignancies in growing children are discussed. Suggestions are made for management of similar cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. Pyogenic thyroiditis due to branchial pouch sinus.Suppurative thyroiditis due to branchial pouch sinus is a rare disease, affecting children and young adults. It presents as an abscess in the lower lateral neck, usually on the left side. Suppurative thyroiditis can be suggested by ultrasound, radionuclide scintigraphy or CT scan, but the diagnosis can only be established by barium swallow showing the sinus from the apex of the pyriform sinus to the perithyroid region. Complete surgical excision of the sinus is essential. Two patients are presented and the role of the various imaging modalities in the diagnosis is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Retinal vascular tortuosity and recurrent retinal haemorrhages in a patient with non-chromaffin paraganglioma.The present report describes the history of a 66-year-old man suffering from recurrent retinal haemorrhages combined with retinal vascular tortuosity. He also developed a non-chromaffin paraganglioma in the neck, and the coincidence of these two rare diseases is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. Madelung's disease (benign symmetric lipomatosis).Madelung's disease, benign symmetric lipomatosis, is a rare disease of undetermined cause characterized by symmetric deposits of diffuse adipose tissue on the cheeks, the neck, and the upper trunk. This article outlines our experience with four patients and presents a case report of a specifically remarkable affectation that agrees with previous data linking male alcoholics with this disease. The treatment in all cases was palliative surgical removal of excess fat from the neck and paracervical regions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Recurrent suppurative thyroiditis due to fourth branchial pouch sinus.Acute suppurative thyroiditis is a rare disease in childhood. The etiology of this condition is not always obvious. The description of a pharyngeal pouch fistula extending from the pyriform sinus to the thyroid gland or perithyroid area, high-lighted the etiopathogenesis of some of the thyroiditis cases. Presented here are the clinical, radiological and pathological aspects of an internal pharyngeal fistula manifesting with recurrent suppurative thyroiditis, until it was completely removed. The clinical features of caudal branchial pouch remnants and their role in the development of acute suppurative thyroiditis is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. Pigmented villonodular synovitis of the temporomandibular joint.Pigmented villonodular synovitis ( PVNS ) is a benign disease of unknown etiology arising from the synovial membranes of joints, bursae, and tendon sheaths. Though histologically benign, it is a very aggressive lesion, capable of bone destruction and widespread infiltration of surrounding tissues. It is a rare disease, usually confined to large joints, particularly the knee. Its propensity for recurrence is emphasized in this report. Involvement of the temporomandibular joint (TMJ) is unusual. It most commonly presents in the TMJ region as a left pre-auricular mass. Symptoms of TMJ dysfunction are usually present, but may be subtle. These may consist of painful mastication, clicking, trismus, malocclusion, and deep pain in the TMJ. Radiological evidence of bone destruction is frequently present, and together with the pre-auricular mass, should suggest the diagnosis. Surgical excision is the preferred mode of treatment. While cosmesis is a consideration, radical excision, including involved bone, is mandatory to prevent recurrence. The effect of radiotherapy on this disease in the head and neck region is documented.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. Sinus histiocytosis with massive lymphadenopathy. A case report.A case of sinus histiocytosis is reported. This apparently rare disease was first described in 1969. The patient presented with a large, painless, bilateral cervical adenopathy which regressed completely over a 16-month period. Histological examination of a lymph node specimen showed sinuses distended with foamy histiocytes. Intervening lymphoid tissue showed a tremendous increase in plasma cells which distorted the normal architecture of the lymph node. The importance of accurate pathological diagnosis is again noted.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. Tumefactive fibroinflammatory lesion of the head and neck: a management strategy.Tumefactive fibroinflammatory lesions are rare, benign, fibrosclerosing masses in the head and neck that appear clinically as malignancies. Twenty-one cases have been described since first reported in 1975. While all authors agree on the lesion's histopathologic characteristics, multiple variations in appearance, treatment, and results have been reported. We review a case and compile all previously reported cases. Common sites of involvement were sinonasal tract (41%), neck (37%), and parotid (23%). Symptoms frequently reported were pain (50%) and hard mass (55%). Five patients (23%) eventually developed extra-head and neck fibrosclerosing lesions. Treatments have included surgery, radiation, and corticosteroids, occasionally in combination. Results have varied depending on size and location of lesion. Based on this review, a discussion of this rare disease and recommendations regarding diagnosis and treatment are given.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |