1/20. Malignant lymphoma of the cervix. An unusual presentation and a rare disease.Malignant lymphomas arising in the uterus are uncommon and are more commonly seen in the cervix than the corpus. Involvement of the cervix as part of a systemic lymphoma is more common than primary lymphoma, but the cervix as the site of presentation is unusual. We report two cases of malignant lymphoma of the cervix. The first patient, a 52-year-old woman, was referred to colposcopy following persistent low grade dyskaryosis on cervical cytology. At colposcopy a Lletz biopsy was performed and a diagnosis of CIN 1 and focal CIN 2 was made. In addition the subepithelial zone revealed a non-Hodgkin's (NHL) B-cell follicular lymphoma. The patient was subsequently staged as NHL Stage 3E. The second patient, a 35-year-old woman, was referred to the gynaecology department with a history of abnormal vaginal bleeding and two abnormal smears. Subsequent cervical biopsy revealed a high grade, large cell, malignant lymphoma, diffuse, B-cell. The patient was staged as Stage IE. Primary lymphoma of the uterine cervix as illustrated in the second case is very unusual. One case had negative cytology and one case had abnormal cells of uncertain origin. This highlights the difficulty of diagnosing cervical lymphoma, a rare but treatable malignancy, on cytology and suggests that cervical biopsy is needed for the confirmation of the diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/20. Staphylococcal pyomyositis in a patient with non-Hodgkin's lymphoma.pyomyositis is a rare disease, encountered mainly in tropical climates. The diagnosis of this entity is difficult, if not misdiagnosed, because of its rarity and its subacute presentation. We report of a 42-year-old man, in whom pyomyositis developed while he was receiving the standard chemotherapy for T-cell non-Hodgkin's lymphoma (NHL). Three months following splenectomy, multiple abscesses occurred in the muscles of both thighs while the patient was receiving the third course of the CHOP regimen. A purulent exudate was aspirated from the abscesses under computed tomographic guidance. coagulase-positive staphylococcus aureus was cultured in the aspirate. pyomyositis was completely resolved following the surgical drainage and the antistaphylococcal antibiotic treatment. This patient has shown that immunosuppression due to splenectomy, NHL, and chemotherapy, especially when using steroids, could be risk factors for pyomyositis in nontropical or semitropical countries.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
3/20. Primary renal lymphoma does exist: case report and review of the literature.Primary renal lymphoma (PRL) is a controversial and rare disease and there is still no agreement on its existence. Many cases have been reported in the literature, but clear diagnostic criteria have not yet been established. Most of the reported cases are questionable because of incomplete staging or the presence of extrarenal disease. Here we report a new case and a review of the literature based on a critical examination of the diagnostic procedure. Thus, probably only 29 cases, ours included, should be recognized as PRL, because only these cases fulfil the three diagnostic criteria and underwent complete diagnostic screening, including renal biopsy, bone marrow biopsy and thoraco-abdominal computerised tomography (CT).- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
4/20. Nasal natural killer-cell lymphoma: a disease with very poor prognosis.Nasal Natural Killer-Cell lymphoma is an aggressive subtype of NHL even when it presents with localized disease. It is more common in Oriental than Western population. We report the case history of a white Caucasian male patient with this disease who died 5 months from diagnosis despite aggressive treatment with 2 different chemotherapy regimes and radiotherapy. We discuss the diagnosis, presentation, treatment and prognosis of this rare disease.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
5/20. An unusual primary renal lymphoma.A case of primary renal lymphoma (PRL) in a 78-year-old man is reported. The tumor was found by computed tomography during a check-up for hematuria and weight loss. Histologic and immunohistochemical analyses of this tumor revealed features typical of low-grade lymphoma with localized amyloid deposition. PRL is a rare disease and only a few cases have been reported previously. To our knowledge, there have been no other reports of PRL with localized amyloid deposition.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
6/20. Primary breast lymphoma: an uncommon but curable disease.Primary malignant breast lymphoma (PBL) is a rare disease with an incidence of 0.04-0.5% of all malignant breast neoplasms. The majority of cases are B-cell lymphomas and the most common histologic type is diffuse large B-cell lymphoma (DLCL). In this study, we report our experience with three cases of PBL. The treatment was the same currently indicated for early stage aggressive NHL, i.e. anthracycline based chemotherapy followed by the involved field radiation therapy. Unfortunately, two patients underwent mastectomy to carry out correct diagnosis. The three patients are alive without any evidence of relapse after 24, 67 and 135 months of follow-up. Considering that aggressive NHL is very sensitive to chemotherapy, mastectomy should be avoided to preserve the quality of life of these patients, once surgery does not change the good prognosis of PBL.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
7/20. PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging.We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
8/20. Primary non-Hodgkin's malignant lymphoma of the breast: long-term follow-up.A 56-year-old woman patient was treated for non-Hodgkin's lymphoma in the left breast and subsequently for four recurrences over the next 109 months. This case illustrates the importance of close and long-term follow-up after the initial treatment of this rare disease.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
9/20. Autoimmune hemolytic anemia in a patient with primary ovarian non-Hodgkin's lymphoma.The primary ovarian lymphoma is a rare disease with poor prognosis. The incidence of autoimmune hemolytic anemia in patients with non-Hodgkin's lymphoma is estimated at 3%. However, a substantial portion of the previously reported cases of ovarian lymphoma actually represented ovarian involvement by more diffuse lymphomatous process. If stringent criteria are used for case selection, true primary ovarian lymphoma usually carries a favorable prognosis. We present a primary malignant lymphoma of ovary accompanied by autoimmune hemolytic anemia in a 29-yr-old patient. After ablative surgery, the hemoglobin level and the reticulocyte count were normalized. One year following surgery and chemotherapy, the patient is alive and disease free.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
10/20. Primary non-Hodgkin's lymphoma of the common bile duct presenting as obstructive jaundice.Primary non-Hodgkin's lymphoma of the extrahepatic bile duct presenting as obstructive jaundice is an extremely rare disease. At this writing, a review of the medical literature disclosed 17 reported cases of primary non-Hodgkin's lymphoma arising from the extrahepatic bile duct. We, herein, report an additional case of obstructive jaundice caused by primary non-Hodgkin's lymphoma of the common bile duct, in a 21-year-old woman. Our patient showed clinical evidence of obstructive jaundice, and endoscopic retrograde cholangiopancreatography and abdominal magnetic resonance imaging demonstrated a long strictured segment of the common bile duct with proximal bile duct dilatation. These clinical and radiological findings resembled those of cholangiocarcinoma. Resection of the common bile duct tumor, cholecystectomy, lymph node dissection, and Roux-en-Y hepaticojejunostomy were carried out. histology and immunohistochemistry of the resected specimen confirmed a diffuse large B-cell-type malignant lymphoma involving the common bile duct. She received four courses of combination chemotherapy, including cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP), and 3060 cGy external irradiation. She has been well, without evidence of tumor recurrence, 17 months after the surgery. In summary, first, primary non-Hodgkin's lymphoma of the extrahepatic bile duct, despite its rarity, should be considered in the differential diagnosis of causes of obstructive jaundice. Second, an accurate histopathologic diagnosis and surgical resection, if feasible, combined with chemotherapy with or without radiotherapy may be the approach to offer a chance for cure.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
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