1/6. Angiomyomatous hamartoma and associated stromal lesions in the right inguinal lymph node: a case report.Angiomyomatous hamartoma is a rare disease with a predisposition for the inguinal lymph nodes. A 51-year-old male patient visited a local hospital because of a right inguinal mass, measuring 3 x 4 cm in size, which was resected. The resected specimen showed irregularly distributed thick-walled vessels in the hilum, extending into the medulla and focally into the cortex of the node, eventually becoming more dispersed and associated with smooth muscle cells splaying into sclerotic stroma. These findings are compatible with an angiomyomatous hamartoma. Another tumor-like mass appeared shortly after the resection at the same location, but was not an angiomyomatous hamartoma, rather it was composed of edematous stromal tissue with proliferating smooth muscle cells. The stromal component included thick-walled blood vessels and lymphatics. Although it could not be determined whether these associated changes in the surrounding stroma are a cause or an effect of angiomyomatous hamartoma, they indicate the clinical difficulty in determining an appropriate area of resection and may provide clues to the pathogenesis of angiomyomatous hamartoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/6. On the pathogenesis and clinical course of mesenteric lymph node cavitation and hyposplenism in coeliac disease.BACKGROUND: Coeliac disease is a disorder characterised by malabsorption related to abnormal small bowel structure and intolerance to gluten. There are several reports of an increased risk for malignancy in coeliac disease and its relation to gluten-free, reduced gluten, or normal diet. While a normal diet is associated with an excess of cancer of the mouth, pharynx, oesophagus, and also of lymphoma, treatment with a gluten-free diet restores the cancer risk back to normal. PATIENT: In the present study, we report on a 63-year-old female patient with a history of coeliac disease for twenty years who presented with persistent diarrhoea, weight loss, and an abdominal mass. RESULTS: The gastroenterological work-up revealed small bowel mucosal atrophy, absence of functional splenic tissue, and evidence for an involution of a mesenteric lymph node, termed cavitation. DISCUSSION: This triad has been previously described to represent a rare disease entity related to coeliac disease. We report a two-year follow-up and a review of the literature on the pathogenesis, prognosis, and therapeutical implications of this disease entity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/6. leiomyomatosis of mesenteric lymph nodes associated with duodenal adenocarcinoma.leiomyomatosis of lymph nodes is an extremely rare disease. Only a few cases have previously been reported in pelvic lymph nodes. They were related to a benign uterine leiomyoma, a metastasizing uterine leiomyoma, an endometrial adenocarcinoma, and an ovarian endometrioid carcinoma. We report on a case of leiomyomatosis of the mesenteric lymph nodes associated with a duodenal adenocarcinoma with no history of uterine leiomyoma or any gynecological malignancy. The patient, a 56-year-old woman, was found to have an adenocarcinoma of the duodenum. All mesenteric lymph nodes removed showed leiomyomatosis, which was verified by immunohistochemical study showing positive immunostaining for smooth muscle actin, desmin, and vimentin, but negative staining for HMB-45. It is necessary to make a differential diagnosis from other examples of spindle cell proliferation involving lymph nodes such as a hemorrhagic spindle cell tumor with amianthoid fibers (palisade myofibroblastoma), angiomyolipoma, lymphangiomyomatosis, inflammatory pseudotumor, and Kaposi's sarcoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/6. Infantile periarteritis nodosa or mucocutaneous lymph node syndrome. A report on four cases and diagnostic considerations.Coronary artery aneurysm in childhood is a rare disease and has in most cases been ascribed to infantile periarteritis nodosa (IPN). In recent years a mucocutaneous lymph node syndrom (MLNS) has been found almost exclusively in japan first described by Kawasaki 1967; this disease frequently involves the coronary arteries and myocardium. Four cases with coronary aneurysms are presented from sweden and seem to be first described from scandinavia. Three of these patients died a sudden death with cardiac arrest. Since MLNS and IPN have identical clinical and pathological features, we suggest that MLNS and IPN constitute a pathologic entity and that to separate them on a clinical or histological basis is nonsensical. The risk of coronary aneurysm and possible sudden death must be considered in patients with uncharacteristic symptoms including prolonged fever, conjunctivitis, exanthema, lesions in the oral mucosa, elevated sedimintation rate, and leukocytosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/6. actinomycosis in neck glands.A middle aged woman presented with right submandibular swelling, fever off and on and anemia. Excisional biopsy of the gland showed actinomycosis. It is a rare disease in the neck. Patient was treated with high doses of parenteral pencillins followed by three months course of oral Doxycyclines. The patient was followed up to six months and she was symptom free. In this case report the disease is elaborated and literature is reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/6. 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) |