1/4. Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk.BACKGROUND: Diagnosis of pulmonary artery sarcoma is difficult and frequently delayed owing to the insidious, non-specific nature of the symptoms. We present our experience with surgery for this rare disease. methods: Between 1995 and 1998, four patients underwent surgery for pulmonary trunk sarcomas. They presented with similar symptoms and were initially treated for thromboembolic disease of the lung. Preoperative diagnosis was established using computer tomography and echocardiography. Postoperatively, two patients received radiotherapy and one combined radio-/chemotherapy. RESULTS: One patient underwent thrombendarteriectomy of the trunk with adjuvant radiotherapy and remains tumor-free after three years and one showed complete regression of metastases after chemotherapy and remains well after more than one year. The other two patients died within two months of surgery, one due to a tumor mass in the inferior caval vein and the other because of cerebral metastases. CONCLUSIONS: According to the literature, mean survival rate is 1.5 months without resection and 10 months with resection, with survival up to four years following combined resection and adjuvant therapy. Our findings are in agreement with this.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/4. Senile angioma-like eruption: a skin manifestation of intravascular large B cell lymphoma.Intravascular large B cell lymphoma (IVL) is a rare disease characterized by intravascular proliferation of neoplastic mononuclear cells within the vascular lumina. The diagnosis requires skin, liver, renal, meningeal, or brain biopsy, but is rarely made antemortem. We report a 67-year-old man in whom the histological examination of senile angioma-like eruption enabled us to establish the diagnosis of IVL. In a suspected case of IVL, a skin biopsy of the senile angioma-like eruption may be beneficial for an early diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/4. Cutaneous reactive angiomatosis occurring in erythema ab igne can cause atypia in endothelial cells: potential mimic of malignant vascular neoplasm.erythema ab igne (EAI), an old and rare disease, is an erythematous, often pigmented, reticular, macular dermatosis that occurs at the site of repeated exposure to moderate heat. Reported herein is an unusual case of EAI occurring in a 33-year-old woman with a very broad lesion of reticular erythema and pigmentation on the lower extremities. The patient frequently put her lower extremities close to a heater in the wintertime to alleviate chill. The lesion started a decade ago, and it gradually became conspicuous. Microscopic findings showed a proliferation of small blood vessels in a thickened papillary dermis, not as typical as seen in EAI, but as seen in cutaneous reactive angiomatosis. They were arranged as small lobules and associated with hyalinization, edema and delicate fibroplasia. Many vessels were lined by plump endothelial cells, some of which had enlarged hyperchromatic nuclei. Many of these cells were multinucleated. Similar-appearing cells were associated with concentric foci of hyalinization without vascular lumina. A few atypical mitoses were observed. The lesion became much less conspicuous after the patient started avoiding close exposure to a heater, without any other special treatments. The aforementioned changes may be confused with malignant vascular neoplasm because of unusual cytological atypia and atypical mitoses in the endothelial cells.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/4. Neurological presentation of intravascular lymphoma: report of two cases and discussion of diagnostic challenges.About a third of patients with intravascular lymphoma (IVL) present to the neurologist with symptoms mimicking thromboembolic events. Diagnosis is difficult, and often made postmortem. As remission may be induced in almost half of patients with combination chemotherapy, early diagnosis of this rare disease is essential. We report two cases of IVL. A 62-year-old male presented with hyperacute myelopathy followed by cortical ischaemic events. The diagnosis was reached with frontal cortical and meningeal biopsy. A 56-year-old female had symptoms of transient ischaemic events, subacute dementia, weight loss and fever. As the disease progressed, she developed nephrotic syndrome and thrombocytopenia. Diagnosis was made postmortem. Our cases illustrate that IVL should be considered in the differential diagnosis of cerebral and systemic vasculitis and subacute bacterial endocarditis. literature suggests IVL can also mimic Creutzfeld-Jakob disease and paraneoplastic encephalomyelitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |