Cases reported "Vaginal Neoplasms"

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1/11. Successful treatment of malignant placental site trophoblastic tumor with combined cytostatic-surgical approach: case report and review of literature.

    OBJECTIVE: Although rare among gestational trophoblastic diseases, the clinical relevance of malignant placental site trophoblastic tumor (PSTT) derives from its potential malignancy associated with early systemic tumor cell dissemination and manifestation of fatal metastases. Because of the low number of cases reported so far worldwide, several treatment strategies have been under consideration, which will be debated following this case report. METHOD: We present the case of a 33-year-old female with PSTT and metastases to the vagina and lung. A 9-month delay in accurate diagnosis was caused by a misinterpretation of her symptoms as signs of a spontaneous abortion. Specialized pathological examination finally led to the diagnosis of PSTT. Primary surgical treatment consisting of abdominal hysterectomy and unilateral salpingo-oophorectomy was followed by multiple resections of recurrent vaginal disease. After the completion of six cycles of EMA/CO (etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine) chemotherapy, hCG titers stayed within the normal range. The patient is without evidence of disease 39 months after primary diagnosis. RESULT: This is the third case of documented long-term remission (>1 year) in metastatic PSTT after combined cryostatic-surgical treatment. CONCLUSION: Since the few previously reported cases with prolonged remission have been treated with the described combined cytostatic-surgical approach consisting of cytoreductive surgery and adjuvant chemotherapy, this approach may be recommended for metastatic PSTT.
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2/11. Primary small cell neuroendocrine carcinoma of the vagina. Marked effect of combination chemotherapy: a case report.

    Primary small cell neuroendocrine carcinoma of the vagina is extremely rare and very aggressive. Nineteen patients with this cancer have been reported in the English-language literature to date. We report a 51-year-old woman with this cancer. Punch biopsy specimens of the vaginal tumor were studied. Electron microscopy demonstrated neuroendocrine granules in the cytoplasm. Tumor cells were strongly stained for cytokeratin, neuron-specific enolase, chromogranin a and serotonin. These findings were consistent with small cell neuroendocrine carcinoma. The patient was treated with combination chemotherapy. The cancer mass completely disappeared after five cycles of the therapy. The patient is still alive without metastasis 41 months after diagnosis. This is the first report of such a remarkable effect of combination chemotherapy on small cell neuroendocrine carcinoma of the vagina.
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3/11. A case of primary mucosa-associated lymphoid tissue lymphoma of the vagina.

    We report the first case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the vagina, the diagnosis of which is supported by genetic and immunophenotypic studies. A 65-year-old, para 2 woman presented to our hospital in July 1997 with a history of prolonged vaginal discharge. Although cytologic examination suggested possible malignancy, a biopsy of the vaginal wall was diagnosed as chronic inflammation. In June 2000, she underwent gynecologic examination because of anuria. Excisional biopsy revealed subepithelial infiltration of atypical lymphoid cells that stained for CD20, CD79a, and BCL-2; stained weakly for IgM; and did not stain for CD3, CD5, CD7, CD10, CD56, CD23, and IgD, suggesting marginal zone B-cell lineage. Monoclonality was detected by Southern blot analysis, and this patient was finally diagnosed as having primary MALT lymphoma of the vagina. She received 3 cycles of chemotherapy (THP-COP) and concurrent radiation to the whole pelvis. The patient is alive and well 40 months after treatment. Because the vagina is one of the mucosa-associated tissues, MALT lymphoma, though rare, must be included in the differential diagnosis of the vaginal neoplasms.
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4/11. A case of metastasizing invasive hydatidiform mole. Is less--less good? review of the literature with regard to adequate treatment.

    BACKGROUND: patients with invasive hydatidiform moles (IHM) have a good prognosis. Even if disease has spread, monocytostatic treatment might be sufficient if the diagnosis has been histologically confirmed. Established classifications divide gestational trophoblastic disease (GTD) including choriocarcinoma into cases with "high" and "low" risk. Without respect to histology "high-risk" cases are recommended to obtain polychemotherapy. CASE: A 40-year-old nullipara underwent hysterectomy for persistent vaginal bleeding after she had already been treated with curettage for hydatidiform mole. An IHM was pathohistologically confirmed. There were no signs of pulmonary spread or other metastases at the time of surgery. Postsurgically persistent beta-hCG levels lead to thorough staging, which revealed multiple pulmonary metastases and a vaginal metastasis. Despite metastasizing GTD with poor prognosis criteria she was treated with single agent therapy. Eight cycles of two weekly methotrexate (MTX) were administered. All sites of metastases responded and our patient is still fine after one year of follow-up. CONCLUSION: With respect to this and other reports monochemotherapy can be a reasonable primary treatment for metastatic IHM.
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5/11. Primary malignant vaginal melanoma treated with adriamycin and ifosfamide: a case report and literature review.

    BACKGROUND: Vaginal melanoma is a very rare but highly malignant gynecological disease, usually diagnosed in postmenopausal woman. The prognosis tends to be poor and it is associated with high rate of recurrence and short survival rates. CASE: The following paper describes a case report regarding a 72-year-old woman with a locally advanced malignant melanoma. The previous erroneous histopathological diagnosis was leiomyosarcoma. She underwent chemotherapy with 3 courses of doxorubicin and ifosfamide. The diagnosis of malignant melanoma was obtained after a repeated biopsy and further pathological investigations. She later underwent radical surgery and 2 additional cycles of the same chemotherapy. At present, 7 months after the last cycle, the patient was locally disease-free, but developed brain metastases, requiring chemotherapy treatment. CONCLUSION: In view of poor survival, this chemotherapy regimen may be an interesting alternative to the traditional treatment of vaginal melanoma.
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6/11. Successful treatment of an endodermal sinus tumor of the vagina by chemotherapy alone: a rare case of an infant diagnosed by pathological examination of discharged tumor fragment.

    A 7-month-old infant was noted to have vaginal bleeding that was accompanied by a discharged tumor fragment. The histological diagnosis was endodermal sinus tumor. Her serum alpha-fetoprotein (AFP) was increased to 358.7 ng/mL, and magnetic resonance imaging showed a 1.8 x 1.0 cm tumor in the vagina. She received combination chemotherapy with cyclophosphamide, pirarubicin, carboplatin, and etoposide. The tumor in the images disappeared and the serum level of AFP returned to the normal range after 2 cycles. Treatment was complete without surgical or radiological therapy. More than 45 months after the completion of chemotherapy, she is alive without signs of recurrence.
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7/11. endodermal sinus tumor of the infant vagina treated exclusively with chemotherapy.

    endodermal sinus tumor (EST) of the vagina is a rare malignancy usually diagnosed before 3 years of age. Historically, the approach to therapy has included radical surgical resection, with adjuvant irradiation, and chemotherapy. An infant presented with vaginal bleeding, imaging evidence of a vaginal mass and an elevated alpha-fetoprotein level. Examination under anesthesia with vaginal biopsies confirmed the diagnosis of an EST (yolk sac) tumor of the vagina. After 5 cycles of chemotherapy, the alpha-fetoprotein had normalized and repeat vaginal biopsies for suspected residual disease was negative for malignancy. To allow preservation of sexual and reproductive function, chemotherapy as a sole modality of treatment for EST should be considered.
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8/11. melanoma relapse of the upper third of the vagina treated with BCG and beta-interferon.

    Faced with a vaginal melanoma relapse in an old cardiac patient operated in May 1985 for melanoma in the upper 3rd of the vagina, we had to solve a therapeutic problem in view of the impossibility of excising a lesion larger than the preceding one and because the patient refused every type of surgery. So we then chose the combined treatment of intralesional BCG and intravenous beta-Interferon. Monthly intravenous administration of beta-Interferon has reached a total of 8,000,000 I/U, while the BCG has been inoculated intralesionally in the submucosa in doses of 0.1 ml after observing the negative result of the PPD test. Our aim was to achieve double immunological stimulation by combining two drugs that are often used separately with satisfactory results. Before and after every therapy cycle, the immunological status was established, and showed a small but constant and permanent increase in t-lymphocytes, both helper and suppressor. Histological results before and after therapy and immunological responses are reported, and the case distinguishes itself by its brilliant results.
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9/11. Phase II trial of methotrexate, vinblastine, doxorubicin, and cisplatin in advanced/recurrent carcinoma of the uterine cervix and vagina.

    A phase II combination chemotherapy protocol combining methotrexate, vinblastine, doxorubicin, and cisplatin was designed to evaluate tumor response and survival in patients with advanced/recurrent cervix and vaginal cancer. Twenty-nine patients with advanced/recurrent cervix cancer and three patients with advanced vaginal cancer who had not previously received cytotoxic chemotherapy were assigned to chemotherapy treatment at 4-week intervals with methotrexate 30 mg/m2 i.v., Day 1, vinblastine 3 mg/m2 i.v., Days 2, 15, and 22, doxorubicin 30 mg/m2 i.v., Day 2, and cisplatin 70 mg/m2 i.v., Day 2. After a median of 4 cycles (maximum number 2 cycles beyond complete regression; 6 cycles with stable regression); we observed objective regressions in all 3 patients with vaginal cancer and 19 patients (66%, 95% CI = 46.82) with cervix cancer including complete regression in 6 patients (21%, 95% CI = 8.40) and partial regression in 13 patients (45%, 95% CI = 26.64). Median overall survival was 11.5 months (range 1.1-54 ). Median survival of responders was 12.8 months (range 3.6-54 ). Toxicity included neutropenia, alopecia, nausea, emesis, and stomatitis. Although grade 3 and 4 neutropenia was observed in over half of the patients, there were no treatment-related deaths. In conclusion, MVAC is a highly active outpatient chemotherapy regimen in patients with advanced/recurrent cervix cancer, achieving a high complete and partial response rate with moderate hematologic toxicity. These results need to be confirmed by phase III trial in advanced disease patients and MVAC may be a suitable regimen for investigation in neoadjuvant chemotherapy trials in poor prognosis, previously untreated patients.
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10/11. Comparison of angiomyofibroblastoma and aggressive angiomyxoma in both sexes: four cases composed of bimodal CD34 and factor xiiia positive dendritic cell subsets.

    Aggressive angiomyxoma (AA) is a distinctive, locally aggressive, fibromyxoid tumor of the pelvic and genital soft tissues. AA is of unknown histogenesis but the cytologically bland spindled tumor cells, which surround characteristic variegated blood vessels, show fibroblastic or myofibroblastic features. AA may be related to angiomyofibroblastoma (AMF), another cytologically bland fibromyxoid genital spindle cell tumor with variable myoid differentiation that does not, as a rule, recur. Recently, CD34 primitive fibroblasts and factor xiiia dendritic histiocytes have been found in varying combination in many fibrovascular, fibrohistiocytic, and myxoid soft tissue tumors. Both cells belong to the microvascular unit, a tissue responsible for stromal repair and remodeling and angiogenesis. To determine if these ubiquitous stromal cells participate in the histogenesis of AA and AMF, we examined two scrotal tumors, one AA with multiple recurrences and one AMF, for the presence of CD34 and FXIIIa dendritic cell subsets. For comparison, a vaginal AMF and a pararectal AA in a woman were included. We also studied actins and desmin to detect myofibroblastic differentiation, and, through double labeling studies, assessed hormone receptors and the cell cycle marker Ki 67 in the different cell subsets. The AA showed unusual cytologic atypia and was initially diagnosed as liposarcoma. It massively recurred four times over 12 years, the first time after seven years. The histologic appearance was fairly constant over the years. The scrotal AMF was a circumscribed 6 cm mass in a 37 year old man. In both cases, most tumor cells were wavy and fibrillar, spindled, stellate, or polygonal fibroblast-like CD34 dendritic cells. Depending on the area examined, a 20-50% subset of dendritic cells showed both nuclear and cytoplasmic staining for FXIIIa. Actin cells were rare but vessels had actin myopericytes, although a small focus of the initial male AA was desmin positive. The recurring AA expressed androgen receptors and had Ki 67 index of 10-20% in "hot spots" of the primary and up to 30% in recurrent tumors. The scrotal AMF widely expressed androgen and progesterone receptors with focal estrogen receptor positivity and the Ki 67 index was 10%. Both CD34 fibroblasts and FXIIIa histiocytes were present in the Ki 67 cycling fraction in both the male AA and AMF and both cell types expressed androgen receptors. The female pararectal AA had more focal CD34 reactivity, particularly in perivascular fibroblasts and these cells were admixed with small FXIIIa cells. The vaginal AMF was strongly desmin and variably to weakly CD34 with 20% FXIIIa dendritic cells and Ki 67 index of 2%. The vaginal AMF strongly expressed estrogen, progesterone, and androgen receptors. In conclusion our data suggest that at least some AA and AMF are myxoid fibrohistiocytic tumors composed of CD34 fibroblasts and FXIIIa dendritic histiocytes. In our tumors, neoplastic CD34 dendritic fibroblasts showed predominantly myxo-collagenous differentiation with prominent myofibroblastic differentiation in only one desmin vaginal AMF. Our results support the notion that AMF and AA are part of a morphologic and histogenetic continuum of myxofibrous and myoid tumors that may arise due to interactions between microvascular CD34 fibroblasts and FXIIIa histiocytes. CD34 and FXIIIa reactivity may be underappreciated in these tumors and is more important when considered histogenetically and biologically rather than in classifying individual neoplasms. Hormonal stimulation of proliferating pelvico-gential microvascular dendritic cells appears to play a role in the morphogenesis of both tumors.
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