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1/10. 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.
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2/10. Neuroendocrine small cell carcinoma of the uterine cervix: what disease? What treatment? Report of ten cases and a review of the literature.

    Neuroendocrine small cell carcinoma of the uterine cervix (NESCCC) is an entity with very aggressive behaviour. The optimal initial therapeutic approach to this rare disease has not yet been clearly defined. We reviewed our experience of this disease over the past 10 years with regard to chemosensitivity. Since 1988, ten patients (mean age 33 years; range 24-47) have been diagnosed with NESCCC and treated in our institutions. disease stage at presentation was IA (one), IB (five, two with lymph node involvement), IIB (one), IIIB (one), and IV (two). One patient had metastatic disease at presentation; three developed metastases during initial treatment. Eight patients underwent surgery and eight received radiation therapy. Six patients received pre- or postoperative cisplatinumvepeside (PE) combination chemotherapy, either alone or concurrently with radiation therapy. PE alone as primary chemotherapy led to disease stabilization in the two patients so treated; concurrent PE and radiation therapy resulted in a pathological complete response in one patient. Eight patients relapsed within 16 months and died of their disease within 29 months from the initial diagnosis. Two patients are alive with no evidence of disease at 13 and 53 months. Our series confirms the previously described very poor prognosis of NESCCC, despite initial aggressive multidisciplinary treatment. It may be that the introduction of chemotherapy, especially combined primary chemoradiotherapy, might allow patients to do a little better, although at the moment there is no good evidence one way or the other.
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keywords = rare disease
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3/10. Von Recklinghausen's disease with urogenital manifestation.

    Neurofibromatosis or Von Recklinghausen's disease is a rare disease that affects the urogenital system in its visceral form. We report on one case where the patient had a recurring mass located in the vesicouterine space that caused irritative urinary symptoms, which was confirmed as uterine cervix neurofibroma following surgery and immunohistochemical study. We stress the importance of a wide resection of such tumors and the need for long-term follow-up due to the high recurrence index and the risk of malignant transformation.
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4/10. Primary malignant lymphoma of uterine cervix.

    Primary malignant lymphoma of the uterine cervix is a rare disease. Malignant lymphoma can be clinically and histopathologically misdiagnosed for the infrequent presentation in this are. A case of 56-year-old woman with uterine cervical tumor with infiltration to both parametria is presented. A biopsy was performed and histopathological studies reported a large cell B lymphoma. After the diagnosis CT abdominal, pelvic and thoracic scan was performed and shows infiltration to posterior bladder without evidence of disease in lymph nodes or another organ. The patient was treated with chemotherapy and radiotherapy. Six month after finish the treatment is well and free of disease.
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5/10. Successful conservative management of primary nonmetastatic cervical choriocarcinoma.

    Primary cervical choriocarcinoma is a rare disease; since 1915 only about 60 cases have been published. The case presented here can be defined as primary cervical choriocarcinoma since it fulfills all the criteria delineated previously.
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6/10. Detection of human papillomavirus type 16 in plasma cells.

    Chronic benign plasma cell tumor of the cervix, also called chronic plasma cell cervicitis, is a rare disease of unknown etiology, characterized by a heavy infiltration of plasma cells forming granulation tissue. To identify infectious agents associated with this disease, we extracted and analyzed dna from the 17-year-old paraffin section of the original case report and from granulation tissue surgically removed from a patient at our institution with a chronic benign plasma cell tumor. The dna from both patients was shown by a polymerase chain reaction (PCR) technique to contain a human papillomavirus 16 (HPV 16) sequence. Genomic Southern analysis of the fresh-frozen tissue confirmed the initial PCR finding. in situ hybridization further demonstrated that the HPV 16 was present in the plasma cells and not a contaminant from the surrounding epithelial tissue. The etiological role of HPV 16, an oncogenic virus associated with cervical carcinoma, in this disease is not yet clear. Our results, however, suggest that the types of cells that are infected by HPV may include cells of lymphoid origin, and that HPV may be associated with chronic benign plasma cell tumors of the cervix.
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keywords = rare disease
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7/10. Adenoid cystic carcinoma of the cervix in a young patient.

    Adenoid cystic carcinoma of the cervix is a rare disease. It usually occurs in an elderly age group. A case who presented at a younger age (30 years) is being reported. The chief complaint was excessive vaginal bleeding for 2 months. Per vaginum examination revealed a growth. She was staged FIGO IIIb. She was treated with radical radiotherapy. disease regressed slowly and complete local response appeared after six months of treatment. Presentation of adenoid cystic carcinoma in younger age group and effect of radiotherapy in advanced stage is reviewed.
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keywords = rare disease
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8/10. The suitable treatment for adenocarcinoma in situ of uterine cervix: a report of four cases.

    adenocarcinoma in situ (AIS) of the uterine cervix is a rare disease. Some authors suggested that conization is a suitable treatment for AIS as for squamous cell carcinoma in situ. However, others did not agree. Four cases of AIS, from 1984 to 1994, have been diagnosed in the Hospital of National taiwan University. If the diagnosis of the conization specimen showed AIS, abdominal total hysterectomy was performed days thereafter. The remaining uterine cervix was checked by histopathology to determine whether it was free of cancer lesions. AIS lesions remained in the uterine cervix, as shown by conization in two cases. AIS is not like lesion as squamous cell carcinoma in situ lesion which is easy to be removed by conization. It is suggested that conization is not an adequate treatment for AIS, rather, a total hysterectomy should be performed.
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keywords = rare disease
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9/10. MR imaging of primary uterine lymphoma.

    Primary malignant lymphoma of the uterus is a rare disease. We present the MR findings in three cases where the uterus was the initial site. MR findings wer retrospectively evaluated. Although the intact junctional zone is a specific finding for lymphoma, diffuse enlargement of the uterus and relatively homogeneous signal intensity on MR imaging in spite of large tumor size are helpful for diagnosing uterine lymphoma.
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10/10. Sarcoma botryoides of the uterine cervix: a case report.

    rhabdomyosarcoma of the uterine cervix is a very rare disease. We present a case of rhabdomyosarcoma (botryoide type) arising from the introitus of the uterine cervix, and make a discussion on the diagnosis, treatment and involving factors of prognosis.
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