Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.24.11 (CD10)
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We present a case of benign mixed tumor of the vagina with its typical clinicopathologic and expanded immunohistochemical features. The strong coexpression of the mesenchymal spindle cell component with epithelial markers such as CK7, together with strong CD10, Bcl2, and estrogen and progesterone receptors favors a mullerian derived tumor. The tumor is best labeled as benign mixed mullerian tumor as originally designated.
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PMID:Benign mixed tumor of the vagina: case report with expanded immunohistochemical profile. 1291 36

Mixed tumors of the vagina (MTsV) are rare benign neoplasms characterized by an admixture of well-differentiated epithelial cells and stromal-type cells in various proportions. In contrast to mixed tumors in other anatomic sites, the histogenesis of the vaginal tumors is unclear. We studied the immunohistochemical profile of 13 examples to explore their histogenesis and determine whether their immunohistochemical profile might be useful in the differential diagnosis. The panel of antibodies used and the number of cases studied were: AE1/3 (12), cytokeratin 7 (CK7) (13), cytokeratin 20 (CK20) (13), epithelial membrane antigen (EMA) (13), muscle actin (MA) (12), desmin (11), h-Caldesmon (13), CD10 (13), CD34 (11), CD99 (8), and S-100 (7). Eight out of 12 tumors were positive for AE1/3, 7/13 for CK7, 2/13 for CK20, and 6/13 for EMA. MA was positive in 11/12 mixed tumors, desmin in 10/11 tumors and h-Caldesmon in 5/13. All tumors were extensively positive for CD10; CD34 was positive in 7/11; and none out of eight tumors showed membranous CD99 staining. Focal S-100 immunoreactivity was seen in 1/7 tumors. These results show that MTsV coexpress epithelial and mesenchymal markers. The expression of muscle actin (usually extensive), and focal desmin and h-Caldesmon positivity suggests the presence of a smooth muscle or myoepithelial component; however, the S-100 negativity and diffuse CD10 expression argue against it. Positivity for muscle markers does not help distinguish MTsV from smooth muscle or skeletal muscle tumors. The frequent expression of CD10 negates its use in the differential diagnosis with endometrial stromal tumors, and the CD10 and CD34 expression suggests that mixed tumors may arise from a primitive pluripotential cell. MTsV are positive for h-Caldesmon and CD10, two markers that have been used in gynecologic pathology primarily to aid in establishing the smooth muscle or endometrial stromal phenotype of a neoplasm.
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PMID:Mixed tumors of the vagina: an immunohistochemical study of 13 cases with emphasis on the cell of origin and potential aid in differential diagnosis. 1515 10

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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PMID:A case of primary mucosa-associated lymphoid tissue lymphoma of the vagina. 1534 21

Prostatic tissue has rarely been described in the lower female genital tract. We describe 6 cases of ectopic prostatic tissue: 5 involving the cervix and 1 the vagina. The latter is the first reported example of benign prostatic tissue in the vagina. The age of the patients ranged from 21 to 65 years; and in all cases, the prostatic tissue was located within the cervical or vaginal stroma without involvement of the surface. In all cases, there were both glandular and squamous elements, which varied in prominence. In some cases, the squamous elements predominated to such an extent that the underlying glandular component was easily overlooked. In the glandular areas, a double cell layer of luminal and basal cells was focally apparent. There was little cytologic atypia or mitotic activity. Immunohistochemically, 3 of 6 cases were positive with prostate specific antigen (PSA) and all 6 cases marked with prostatic acid phosphatase (PSAP). In some of the positive cases, staining was focal. Positive staining with prostatic markers was confined to the glandular elements with no staining of the squamous areas. Immunohistochemical staining with the high molecular weight cytokeratin 34betaE12 highlighted the basal cell layer, which often extended into the center of the cellular islands, reminiscent of basal cell hyperplasia involving the prostate gland. All cases tested were CD10 positive (largely restricted to the basal cell layer), alpha-methylacyl-CoA racemase positive, and p16 negative. Estrogen receptor (ER) and progesterone receptor (PR) were negative in the glandular areas, but ER was positive in the squamous elements in all cases and PR was positive in 1 case. All cases tested were androgen receptor positive and exhibited a low MIB-1 proliferation index with only scattered positive nuclei. The presence of ectopic prostatic tissue in the lower female genital tract may be more common than is appreciated. Once the possibility is considered, the diagnosis is easily confirmed using immunohistochemistry, although staining with prostatic markers may be focal and PSA may be negative. Ectopic prostatic tissue in the lower female genital tract is almost certainly a benign condition, based on the morphology, including the presence of a double cell layer, although follow-up of larger numbers of cases is required. Possible theories of histogenesis include a developmental anomaly, metaplasia of preexisting endocervical glands, and derivation from mesonephric remnants.
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PMID:Ectopic prostatic tissue in the uterine cervix and vagina: report of a series with a detailed immunohistochemical analysis. 1643 95

We describe a case of primary neuroendocrine carcinoma arising from the anterior vaginal wall of a 67-year-old woman. Primary neuroendocrine carcinoma of the vagina is a rare entity with only 25 previously reported cases in the literature. In previous reports, these tumors have not been distinguished from primary neuroendocrine carcinoma of the skin (Merkel cell carcinoma). The tumor was composed of cells that showed neuroendocrine-type nuclear features with hyperchromasia, nuclear molding, occasional small nucleoli, and a chromatin pattern that was finely granular. The tumor cells were positive for cytokeratin 20 (CK20), neuron specific enolase, pancytokeratin, epithelial membrane antigen, and chromogranin A expression. Ki-67, a marker of proliferation, was also positive in>90% of cells. The tumor cells showed intense expression of Bcl-2 oncoprotein and mild to moderate expression of c-KIT. Synaptophysin, neurofilament, CD45, CD56, CD10, S-100, HMB-45, cytokeratin 7, and thyroid transcription factor 1 were negative. This pattern of staining is consistent with a Merkel cell carcinoma. This is the first report of a primary neuroendocrine carcinoma of the vagina with a Merkel cell phenotype. Previous studies have not distinguished primary neuroendocrine carcinoma of the vagina from Merkel cell carcinoma of the skin. Positive expression of CK20 in primary small cell carcinoma of the vagina might represent a Merkel cell carcinoma subtype of this tumor.
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PMID:Primary neuroendocrine carcinoma of the vagina with Merkel cell carcinoma phenotype. 1653 63

Mesonephric adenocarcinoma (MA) is a rare tumor of the female genital tract, mainly in the cervix and vagina, which is usually associated with mesonephric remnants or mesonephric hyperplasia. In the uterus corpus, MA is as rare as mesonephric structures, and only a few cases have been previously reported. Here, we report a rare case of MA of the uterine corpus. A 73-year-old woman presented with multiple nodules in the bilateral lung. Abdominal computed tomography scan confirmed a uterine tumor measuring 8.6 cm. All tumor markers, including CA125, were within normal limits. A total hysterectomy and bilateral salpingo-oophorectomy were performed. The tumor was confined to the myometrium and showed strong resemblance to cervical MA despite the absence of mesonephric hyperplasia or remnants. The most striking pattern consisted of large sheets of small round tubules, often with densely eosinophilic secretions in the lumen. In addition, the ductal pattern simulating endometrioid adenocarcinoma was also noted. A mixture of tubular and ductal patterns, most predominantly seen, formed more complex tubules and cribriform structures. Other elements consisted of a retiform pattern, serous adenocarcinoma-like papillary budding, and glomeruloid morphology. Immunohistochemically, the tumor cells were positive not only for cytokeratin, epithelial membrane antigen, and vimentin but also for CD10 and calretinin. No staining was identified for estrogen receptor, progesterone receptor, or androgen receptor. Adjuvant chemotherapy was begun for the patient, who is alive with disease 28 months later. We review the previously published cases of MA and discuss the principal differential diagnosis of MA in the uterine corpus.
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PMID:Mesonephric adenocarcinoma of the uterine corpus: a case report and review of the literature. 1858 Mar 12

Mullerian adenosarcomas are rare mixed tumors of low malignant potential that occur mainly in the uterus and also in extrauterine locations. Microscopically, they may be difficult to distinguish from adenofibromas. In this clinicopathologic study of 55 adenosarcomas, the mean patient age was 50 years (range: 13 to 83 y). Thirty-seven tumors were of the uterine corpus, 11 of the cervix, 4 of the ovary, and 1 each of the fallopian tube, vagina, and Douglas peritoneum. Abdominal pain and vaginal bleeding were the usual complaints. Treatment was known in 50 patients: 10 had polypectomy, 1 cone biopsy, and 39 hysterectomy, which was accompanied by bilateral salpingo-oophorectomy in 24 and lymphadenectomy in 4. Five patients had radiotherapy and 2 of them had chemotherapy. Stage was known in 41 cases. Of 30 tumors of the uterine corpus, 17 were stage IA, 11 stage IB, 1 stage IC, and 1 stage IIIC. Four cervical tumors were stage IB. Three of the 4 ovarian tumors were stage IA and the other was stage IIIC. The tumor of the fallopian tube was stage IC, and the tumors of the vagina and recto-uterine pouch were confined to their site of origin. Most uterine tumors were polypoid masses ranging from 1 to 20 cm (mean: 6.5 cm). Microscopically, sarcomatous overgrowth was found in 18 cases (33%), heterologous elements in 13 (24%), and sex cordlike differentiation in 7 (13%). Fourteen of 30 uterine tumors (47%) had myometrial invasion that was minimal in 5, involved one-third of the myometrial thickness in 7, and more than 50% in 2. Of 4 cervical tumors, 2 were endocervical polyps, 1 invaded one-third of the cervical wall, and the other invaded its full thickness. Follow-up information (2 mo to 18 y; average: 7.5 y) was available in 29 patients. Six developed metastases and 5 of them died of tumor. Four had adenosarcomas with sarcomatous overgrowth; however, the other 2 patients had typical low-grade adenosarcomas of the uterine corpus and cervix, respectively, exhibiting only mild nuclear atypia of the stromal component and </=2 mitotic figures/10 high power fields. Both were initially underdiagnosed as adenofibromas. The finding of such cases, which raises the controversy of whether or not adenofibroma exists as a tumor entity, prompted us to make a comparative immunohistochemical analysis of 23 typical adenosarcomas, 8 adenosarcomas with sarcomatous overgrowth, and 29 benign and malignant related lesions, including 7 clinically benign adenofibromas. Adenosarcomas with sarcomatous overgrowth showed strong immunoreaction for Ki-67 and p53 and loss of CD10 and progesterone receptors immunostaining; in contrast, the immunoreaction for these tumor markers in typical adenosarcomas without sarcomatous overgrowth was similar to that of adenofibromas associated with favorable outcome and other benign lesions such as endometrial polyps and endometriosis. These findings suggest that some of the tumors currently classified as adenofibromas, on the basis of their low mitotic count and lack of significant nuclear atypia, are, in fact, well-differentiated adenosarcomas.
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PMID:Mullerian adenosarcoma: a clinicopathologic and immunohistochemical study of 55 cases challenging the existence of adenofibroma. 1894 2

Mullerian adenosarcoma is an uncommon, but not rare, mixed tumor containing a neoplastic but benign or mildly atypical epithelial element and a sarcomatous, usually low-grade, stromal component. The most common site is the uterine corpus but adenosarcoma also occurs in the cervix and ovary and more rarely in the vagina, fallopian tube, arising from peritoneal surfaces, or outside the female genital tract, for example in the intestine. Most uterine cases have a polypoid gross appearance, sometimes resulting in the formation of multiple polyps. Characteristic histologic features include a low power "phyllodes-like" architecture with leaf-like projections lined by a variety of benign Mullerian type epithelia, sometimes with squamous metaplasia. Intraglandular stromal protrusions are a characteristic feature. The stroma may be uniformly cellular but there is typically increased cellularity around the epithelial elements, resulting in the formation of a cambium layer. Using the World Health Organization definition, stromal mitotic activity of 2 or more per 10 high-power fields is required for a diagnosis of adenosarcoma but in practice the diagnosis is made with stromal mitotic activity less than this if the characteristic architecture and cambium layer is present. The stromal component is usually morphologically "low-grade" and of endometrial stromal or fibroblastic type (hormone receptor and CD10 positive). Sometimes it is high grade, resembling undifferentiated sarcoma. Additional features sometimes present include heterologous stromal elements or sex cord-like differentiation. Uterine adenosarcomas are, in general, low-grade neoplasms capable of local recurrence after polypectomy or hysterectomy and much less commonly distant metastasis. The 2 most important adverse prognostic factors, which sometimes coexist, are deep myometrial invasion and sarcomatous overgrowth; the latter is usually associated with morphologically "high-grade" stromal elements with loss of expression of hormone receptors and CD10. Adenosarcoma may be confused with a variety of lesions and one of the main differential diagnoses is adenofibroma in which the stromal component is, by definition, morphologically benign. However, occasional adenofibromas recur or even metastasize. As such, it has been suggested that all adenofibromas should be classified as adenosarcomas, albeit with low-malignant potential. Ovarian adenosarcomas are much more likely to exhibit malignant behavior than their uterine counterparts, probably due to the lack of an anatomic barrier to peritoneal dissemination.
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PMID:Mullerian adenosarcoma of the female genital tract. 2017 34

A 27-year-old nulliparous woman presented with large finger-like projections protruding from her vagina. Intraoperatively there were deposits in the pouch of Douglas. Clinical presentation and history of vaginal bleeding suggested malignancy. Histopathology of both the vaginal and pouch of Douglas masses showed endometrial glands and stroma. There was no architectural complexity or cytological atypia of glands or stroma. Immunohistochemistry for oestrogen receptor, progesterone receptor and CD10 was positive. Based on morphological and immunohistochemical findings, multifocal polypoid endometriosis was diagnosed; this is a recently described entity having a clinical presentation and age range completely different from conventional or non-polypoid endometriosis. Although an association between tamoxifen use, unopposed oestrogen therapy and polypoid endometriosis has been suggested, the patient had no history of tamoxifen or oestrogen intake. Polypoid endometriosis should be part of the differential diagnosis in young women presenting with vaginal growth.
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PMID:Multifocal polypoid endometriosis in a young woman simulating vaginal and pelvic neoplasm. 2029 88

Female sexual arousal disorder (FSAD) is the inability to attain or maintain an adequate lubrication-swelling response of sexual excitement. The potentiation of vascular responses leading to increased blood flow in clitoris and vagina has represented the main focus in the pharmacological treatment of FSAD, including the evaluation of the type 5 phosphodiesterase (PDE5) inhibitors. However, due to a lack of clear efficacy, there is no approved pharmacotherapy for FSAD to date. In the present issue of the British Journal of Pharmacology, Wayman et al. show that the administration by intravenous or intravaginal routes of a novel neutral endopeptidase inhibitor, UK-414,445, results in enhanced genital blood flow responses to pelvic nerve stimulation in female rabbits, without significantly affecting blood pressure. Neutral endopeptidase inhibition, by preserving vasoactive peptides such as vasoactive intestinal polypeptide, raises the possibility of a new pharmacological approach to the treatment of FSAD.
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PMID:Neutral endopeptidase inhibition: could it have a role in the treatment of female sexual arousal disorder? 2041 68


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