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1/142. Villoglandular papillary adenocarcinoma of the uterine cervix: report of a case.

    Villoglandular papillary adenocarcinoma is a recently described form of adenocarcinoma of the uterine cervix, which apparently affects young women and seems to have a favorable course with an excellent prognosis. We report on a case of villoglandular papillary adenocarcinoma in a 26-year-old woman. The patient was treated by conization alone and is disease free after a 40-month follow-up.
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2/142. Exfoliative cytology of a lymphoepithelioma-like carcinoma in a cervical smear. A case report.

    BACKGROUND: Lymphoepithelioma-like carcinoma of the cervix (LELC) is cytologically identical to its counterparts at other sites, such as the nasopharynx. LELC can be suspected on a cervical cytologic smear. The differential diagnosis includes nonkeratinizing squamous cell carcinoma with prominent stromal inflammation, carcinoma with intense stromal eosinophilia, glassy cell carcinoma, malignant lymphoma (especially lymphoepitheloid-Lennerts lymphoma) and metastatic Schmincke-Regaud tumor. CASE: A 55-year-old female presented with an ulcerated endophytic tumor in the cervix. Exfoliative cytology showed uniform, large tumor cells, often associated with inflammatory cells, with round or oval nuclei and one or more prominent nucleoli. The cytoplasm was finely granular to flocculent, and the nuclei were uniformly vesicular. The chromatin was peripherally marginated. The cell borders were indistinct. There was no evidence of dyskeratotic or keratinized cells, koilocytes or glandlike formations. These findings were highly suspicious for LELC and were confirmed by biopsy. flow cytometry showed dna aneuploidy, with a dna index of 1.08. in situ hybridization was negative for human papillomavirus 16 and 18. CONCLUSION: LELC of the uterine cervix has cytologic features that are sufficiently characteristic for a specific cytologic diagnosis. The diagnosis, nevertheless, has to be proven by histology.
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3/142. Multiple primary cancers and HPV infection: are they related?

    Multiple primary cancers have been reported with increasing frequency in recent years, but the presence of foreign dna sequences of infectious agents in tumours arising in the same patient has so far not been investigated. We report a case of a patient with Hodgkin's lymphoma, an "in situ" cervix carcinoma and an adenocarcinoma of the right and left mammary gland. In all the tumour samples we detected the presence of dna genomic sequences of Papillomavirus type 16. Our results suggest that HPV infection may be an exogenous risk factor even in second primary tumours of non-epithelial origin.
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4/142. Exfoliative cytology of atypical polypoid adenomyoma. A case report.

    BACKGROUND: An atypical polypoid adenomyoma (APA) is a well-defined entity. It occurs in the endometrium, lower uterine segment and endocervix. It is usually composed of atypical complex glands with squamous metaplasia admixed with myofibromatous stroma. CASE: A 35-year-old female presented with one-year history of irregular menstrual periods. A diagnosis of adenocarcinoma in situ was rendered on her cervicovaginal smear. Pelvic examination revealed an enlarged uterus due to a leiomyoma. Colposcopic examination revealed a 0.6-cm, sessile, polypoid mass at the junction of the endocervix and ectocervix. A cone biopsy of the lesion showed irregular, endometrial-type glands embedded in a prominent myofibromatous stroma. The atypical glandular component of the mass demonstrated varying degrees of architectural complexity, ranging from simple to complex hyperplasia. In tissue sections the lesion was diagnosed as APA of the cervix. The patient underwent a hysterectomy for the leiomyoma. The hysterectomy specimen showed an 8.5-cm leiomyoma. The cervix and uterine corpus revealed no residual APA. CONCLUSION: APA of the cervix should be considered among the differential diagnoses of atypical glandular cells of undetermined significance. The diagnosis of APA cannot be made on cytology; the final diagnosis requires histologic confirmation.
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5/142. A new observation of the Carney's triad with long follow-Up period and additional tumors.

    The etiology of the Carney's triad (gastrointestinal stromal tumors, pulmonary chondromas, and paragangliomas) is unknown, and only 57 cases have been reported since its identification in 1977. We report the clinical course of a female with the complete triad and some additional tumors. Bilateral vagal paragangliomas were treated surgically and with radiotherapy between the ages of 24 and 26 years. Subsequently she underwent surgery for a gastric leiomyosarcoma (27 years), a pleomorphic adenoma of the parotid gland (49 years) and a multifocal breast cancer with axillary spread (50 years). A calcified lesion was also noticed in the left lung, the radiologic diagnosis of which was consistent with chondroma. A mediastinal paraganglioma, detected at 56 years on a control X-ray of the chest, was partially excised at 63 years. At the last control, performed at 66 years, the patient was alive with residual cervical and mediastinal paraganglioma. Her younger brother was affected by Hirschsprung's disease and died at 54 years of rectal cancer. Her daughter is 33 and has been suffering since birth with severe constipation. In conclusion, this is one of the longest followed-up patients with Carney's triad. Her case illustrates the need for early recognition of the setting in order to detect the component tumors at a stage when surgery may be curative, and careful and life-long follow-up, both because the multicentricity of the classic components tends to manifest metachronously and because of the tendency to develop other tumors, some of which may be malignant. Furthermore, the presence of Hirschsprung's disease in the patient's family, coupled with the alleged common origin of two component lesions from derivatives of the neural crest, open new avenues for the understanding of this disorder.
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6/142. Villoglandular papillary adenocarcinoma of the uterine cervix.

    Villoglandular papillary adenocarcinoma of the uterine cervix was recently (1989) described by three main histological features: exophytic proliferation, papillary architecture and mild to moderate cellular atypicality. The authors report a case of villoglandular papillary adenocarcinoma, clinical stage IB, which was peculiar because of its association with a co-existing and simultaneously discovered invasive squamous cell carcinoma. These two patterns were juxtaposed and not intermingled. The patient was treated with radical hysterectomy followed by vaginal radiation therapy. She remains without evidence of recurrence after 12 months of follow-up. Five main clinicopathological features of the villoglandular papillary adenocarcinoma could be stressed: rare histological variant (72 described cases), young age of patients (25-45 years old), superficial stromal invasion, usual association with other tumoral patterns (in situ or invasive adenocarcinoma as well as in situ or invasive squamous cell carcinoma) and excellent prognosis. For selected cases, a conservative surgical approach (cervical conization) was possible.
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7/142. Florid endocervical glandular hyperplasia with intestinal and pyloric gland metaplasia: worrisome benign mimic of "adenoma malignum".

    We describe three cases of florid endocervical glandular hyperplasia with intestinal and pyloric gland metaplasia, which can be a benign mimic of adenoma malignum. In two cases, adenoma malignum was seriously considered preoperatively because of watery vaginal discharge and the results of imaging studies. The three cases shared common histopathological features, i.e., (i) proliferating endocervical glands surrounded by clusters of smaller glands, resembling the pyloric glands of the stomach; (ii) occasional intestinal metaplasia; (iii) bland nuclear features; and (iv) predominantly PAS-positive neutral mucin in the glandular epithelium. In two cases, glands were densely and irregularly arranged in some areas. immunohistochemistry disclosed that the intracytoplasmic mucin of the metaplastic epithelium was positive for M-GGMC-1 (HIK1083), which reacts with mucin of pyloric glands. Monoclonal CEA was negative in all cases. This pseudoneoplastic benign condition should be recognized by both gynecologists and pathologists, although it might be difficult to establish a definite diagnosis preoperatively even with deep cone biopsy.
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8/142. Multicystic autoimmune thyroiditis-like disease associated with hiv infection. A case report.

    BACKGROUND: Human immunodeficiency virus (hiv) infection and resulting acquired immunodeficiency syndrome (AIDS) may involve virtually every organ system, including the endocrine glands. Thyroid dysfunction most commonly reflects advanced disease and generally resembles euthyroid sick syndrome. Rarely do opportunistic infections, hemorrhage, neoplasms and drugs account for alterations in thyroid tissue. Multiple lymphoepithelial cysts of parotid gland and thymus have been identified, but similar findings in thyroid gland have not been reported. CASE: A 41-year-old, hiv-seropositive woman, asymptomatic for seven years, developed a squamous cell carcinoma of the cervix with local-regional extension. At the same time, bilateral complex thyroid cysts and high titers of antimicrosomal antibodies (1/6,400) were detected. Ultrasound-guided fine needle aspiration biopsy of the thyroid showed a heterogeneous lymphocytic population with a reactive appearance and occasional groups of epithelial cells with an immature squamous pattern, along with cytologic features of autoimmune thyroiditis. Immunocytochemistry was positive for CD20, CD3 and CD5. Immunoglobulin heavy chain gene rearrangement by polymerase chain reaction from cytologic material showed a polyclonal lymphoid population. External radiotherapy resulted in a significant reduction in the pelvic lesion. Four months after diagnosis, abdominal ultrasound displayed multiple hepatic metastasis, the patient's condition rapidly deteriorated, and she died about a month later. CONCLUSION: This case had unique features and probably represented an AIDS-related lesion and distinct entity.
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9/142. Uterine adenomyoma of endocervical type.

    A uterine adenomyoma of endocervical type was presented histopathologically in a 44-year-old woman. She had a mural tumor of the uterine endocervix, which showed a well-circumscribed margin and multiple cysts filled with mucin. Histologically, the tumor was composed of a proliferation of endocervical glands and smooth muscle. The glandular epithelial components were cystic and occasionally papillary, but neither component showed cytological atypia. A differential diagnosis of the uterine endocervical tumor should include adenomyoma of endocervical type.
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10/142. Villoglandular papillary adenocarcinoma of the cervix. Beware of a wolf in sheep's clothing.

    Villoglandular papillary adenocarcinoma of the cervix is a well differentiated form of cervical adenocarcinoma with a favourable prognosis and a conservative procedure is suggested. We present three cases of villoglandular papillary adenocarcinoma of the cervix. Histological examination of a biopsy of each cervix showed well differentiated villoglandular papillary adenocarcinoma, stage Ib according to FIGO classification. In all cases the disease was limited to the cervix. Nevertheless, histopathological examination of the surgical specimen revealed an infiltrating component with squamous differentiation in one case, while in a second case histopathological examination revealed a moderately differentiated papillary adenocarcinoma with a superficially infiltrating growth-pattern besides the villoglandular papillary adenocarcinoma. Before conservative therapy is considered, careful evaluation of the presence of poor prognostic features must be made. One should consider whether conservative therapy is sufficient because of the predominance of concomitance of other carcinoma besides the villoglandular papillary adenocarcinoma.
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