1/28. 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) |
2/28. A case of intravascular large B-cell lymphoma mimicking erythema nodosum: the importance of multiple skin biopsies.BACKGROUND: Intravascular lymphoma is a rare disease characterized by the proliferation of neoplastic monuclear cells within the lumens of small blood vessels. The neoplastic cells are usually of B-cell origin, and rarely of T-cell or histiocytic origin. Although this clinicopathological entity of lymphoma has not been listed in general pathological classifications such as REAL classification or the Working Formulation, it is recently in the WHO classification scheme, which is essentially an updated REAL scheme, and the EORTC classification scheme. methods: In this report, a 62-year-old woman with intravascular large B-cell lymphoma was observed by clinical, histopathological, immunohistochemical and molecular methods. RESULTS: A 62-year-old woman presented with large erythematous macules on the bilateral thighs and lower legs. The lesions were accompanied with hard, tender, intradermal or subcutaneous nodules mimicking erythema nodosum. Histopathological examination in the first biopsy revealed non-specific panniculitis compatible with erythema nodosum. The second biopsy revealed emboli of atypical lymphocytes within many of the dilated and proliferated vessels in the deep dermis and subcutaneous tissue. These cells were positive for L-26 and kappa light chain, and negative for lambda light chain, factor viii-related antigen, CD30, CD34, CD68 and UCHL-1. These findings confirmed the diagnosis of intravascular large B-cell lymphoma. A laboratory examination showed a high level of LDH and abnormal cells in the bone marrow. An MRI of the brain and computed tomographic (CT) scans of the chest and abdomen revealed no evidence of malignancy. Before the treatment, the size of the nodules decreased spontaneously by about 50% in one month and significantly in two months. Although combination chemotherapy, which consisted of CHOP, brought her partial remission, she experienced neurological symptoms 6 months after the initial treatment and died of brain metastasis 9 months after the treatment. CONCLUSIONS: This is a unique case for two following reasons: 1) the first biopsy revealed non-specific findings compatible with erythema nodosum; and 2) before the treatment, the nodules regressed spontaneously. Dermatologists should take multiple skin biopsies for EN lesions with the non-specific histopathological findings not to refute the existence of this disease.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
3/28. Primary leptomeningeal lymphoma with long-term survival: a case report.Primary leptomeningeal lymphoma (PLML) is a rare disease. The most common presentation is symptoms of increased intracranial pressure. confusion, dysarthria, hearing loss, paraparesis and lumbosacral spinal root symptoms have also been reported. Chemotherapy and radiotherapy have been tried, but its prognosis is usually poor. We experienced a case of PLML with a relatively benign course in an 18-year-old girl. Initial diagnosis was made as idiopathic intracranial hypertension. Lumbosacral shunt was done with good response for 3 years. When headache recurred, she was reevaluated and was correctly diagnosed as PLML.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
4/28. Primary malignant lymphoma of the ovary: an unusual presentation of a rare disease.Because the outcome of patients with primary ovarian non-Hodgkin's lymphoma (NHL) is controversial, we present the incidental finding of a primary malignant lymphoma of the ovary in a 50-year-old patient. Three and a half years following ablative surgery and adjuvant chemotherapy, the patient is alive and disease free. Ovarian lymphoma is a disease of reportedly poor prognosis. However, many previously reported cases of ovarian lymphoma actually represented ovarian involvement by a more diffuse lymphomatous process. If stringent criteria are used for case selection, true primary ovarian lymphoma is a very rare disease and usually carries a favorable prognosis.- - - - - - - - - - ranking = 1.25keywords = rare disease (Clic here for more details about this article) |
5/28. Primary pancreatic lymphoma: clinicopathological analysis of 19 cases from japan and review of the literature.Primary pancreatic lymphoma (PL) is an extremely rare disease, and large-scale studies are rarely performed even in Western countries, in which all cases of PL reported to date were of the B-cell type. Little information regarding PL is available in japan. Nineteen cases of PL were collected through a nationwide study in japan, and paraffin-embedded specimens were processed for staining with hematoxylin and eosin and by immunohistochemical procedures. Clinicopathological findings were reviewed and compared with those reported in Western countries. The patient population consisted of 13 men and 6 women, ranging in age from 46 to 84 (average 62) years. abdominal pain was the most common presenting symptom. Tumors were located in the pancreatic head (12 cases), tail (4 cases) and body (2 cases), and ranged in size from 4 to 17 cm. Clinical stage was I(E) in 9 cases and II(E) in 10. Radical resection was performed in 10 cases and bypass operation in 1, followed by chemotherapy in 8. Immunophenotypically, 15 cases were B-cell and 4 were T-cell lymphomas. Seventy-three percent of B-cell tumors were diffuse large B-cell lymphomas. The 1-year actuarial survival rate for B-cell lymphomas (51.9%) was better than that of T-cell lymphomas (0%). However, in japan the incidence of T-cell PLs was higher, and, partly as a consequence of this, prognosis was poorer than in Western countries.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
6/28. Clinicopathologic and cytogenetic analyses of three cases of primary uterine non-Hodgkin's lymphoma.Primary uterine non-Hodgkin's lymphoma (NHL) is an extremely rare disease. To accumulate more information on clinical data, we report three cases of primary uterine NHL with apparently the first demonstration of karyotypic analysis. Histological diagnosis was diffuse large B cell type in all patients. Two of them with advanced stage showed chemoresistance and a short survival. The remaining case with early stage showed an uneventful course following operation. No common chromosomal abnormality was detected. The therapeutic strategy for uterine NHL might therefore be similar to that for other types of aggressive NHL, although a larger study is needed.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
7/28. A case of primary bilateral adrenal lymphoma (PAL) with central nervous system (CNS) involvement.Primary adrenal lymphoma (PAL) is a very rare disease and usually does not have disease elsewhere, and if present, it is frequently extranodal. Several cases of PALs, with central nervous system (CNS) involvement, gastrointestinal tract or other endocrine organs involvement, have been reported in Western literature. We experienced a case of PAL with CNS involvement, which was previously unreported in korea. The patient, a 61 year-old male, was admitted with left abdominal pain. After imaging study and needle aspiration biopsy (NAB), a bilateral primary adrenal lymphoma (large B-cell type), with bilateral hemorrhage, was confirmed. Combination chemotherapy, with CEOP regimen, was used. A follow-up abdomino- pelvic CT scan, after the second CEOP chemotherapy, showed the previous adrenal masses had nearly disappeared. However, about 2 months after diagnosis (day 52), he showed recent memory loss, obtundation, disorientation and drowsy mental status. The brain MRI revealed multifocal scattered lesions with increased signal intensity. The result of a brain biopsy was diffuse large B-cell lymphoma. After 5(1/2) cycles of chemotherapy, with CEOP regimen, and whole brain radiotherapy, he is still surviving 6 months later, and has become alert with nearly normalized cognitive function.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
8/28. Primary breast lymphoma, contralateral breast cancer, and bilateral Brenner tumors of the ovary.BACKGROUND: Primary lymphoma of the breast is an unusual clinical entity. Its presence with invasive breast cancer and bilateral Brenner tumors of the ovary is very rare. CASE: We report a 62-year-old woman referred for further evaluation of a palpable mass in her breast. She was diagnosed and treated for simultaneous primary lymphoma of the right breast, contralateral invasive ductal carcinoma, and bilateral Brenner tumors of the ovary. One year after treatment, she is free of recurrence or progression. CONCLUSION: Compared with breast carcinoma, primary breast lymphoma is a rare disease but should be considered in the differential diagnosis of breast masses. The presence of both breast malignancies presents a challenge in treatment decisions.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
9/28. 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) |
10/28. An unusual cause of new-onset atrial flutter: primary cardiac lymphoma.Primary cardiac lymphoma is a rare disease with a high mortality rate due to the advanced stage of myocardial involvement at presentation. The diagnosis is extremely difficult to make because of the rarity of the disease, variability of clinical manifestations, limited noninvasive diagnostic techniques available, and difficulties and/or delays in the use of invasive measures. The incidence of the disease is increasing, especially among immunocompromised patients, with those suffering from acquired immunodeficiency syndrome accounting for the greatest increase. We report the case of an immunocompetent 76-year-old black woman who presented with near-syncopal episodes. Transthoracic echocardiogram revealed a right atrial mass. Surgical resection was performed, and a diagnosis of large B-cell non-Hodgkin's lymphoma was made. Primary cardiac lymphoma should be considered in any patient with a cardiac mass. Prompt diagnosis and treatment of primary cardiac lymphoma is imperative for survival.- - - - - - - - - - ranking = 0.25keywords = rare disease (Clic here for more details about this article) |
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