Cases reported "Anus Neoplasms"

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1/35. Extramammary Paget's disease.

    Extramammary Paget's disease (EMPD) is an uncommon but distinctive tumor. The lesion is defined as an intra-epidermal neoplasm. The lesion may be accompanied by an invasive adenocarcinoma or in situ adenocarcinoma of apocrine glands. Visceral carcinoma may also coexist or develop. Definitive diagnosis requires biopsy of the lesion and immunohistochemical staining. In most cases of noninvasive or minimally invasive EMPD, surgical resection with clear margins and careful follow-up are recommended, since the recurrence rate is high. We review the literature and report two cases of EMPD, one involving the perineal-scrotal area and the other involving the perianal area.
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2/35. Anal duct carcinoma: case report and review of the literature.

    This report details the clinical course of two patients with true anal duct carcinoma. The incidence of this malignancy is low. The tissues of origination are the glands of the anal duct. The features that differentiate this tumor from the usual rectal carcinoma are prominent ductal structures, abundant mucin production with organized mucinous pools, and infiltration into the perirectal soft tissue. The clinical management of anal duct carcinoma remains a surgical challenge. The extent of surgical resection must be radical because of the infiltrative nature of the tumor. This report describes treatment of two patients with anal duct carcinoma. The first patient was a black woman with no previous history of rectal disease. Her operative procedure was an abdominoperineal resection with posterior vaginectomy. Nine months after initial surgery a local recurrence was resected. The second patient was a white man with a previous history of hemorrhoidectomy and anal fissure. He underwent an abdominoperineal resection but had positive dermal skin margins on permanent sections despite wide perirectal soft tissue resection. A secondary resection with confirmed clear margins of the skin was performed 2 weeks postoperatively. One management aspect of anal duct carcinoma that needs emphasis is the need for wide local excision of the perirectal soft tissues.
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3/35. Anal duct carcinoma: report of case and a survey of the experience of the American Osteopathic College of Proctology.

    Anal duct carcinoma, also known as anal gland carcinoma or adenocarcinoma of the anal canal, is an unusual anal cancer that accounts for approximately 0.1% of all gastrointestinal cancers. Delays in diagnosis most likely account for the poor prognosis associated with this cancer. Presenting symptoms often mimic those of more common benign anorectal pathologic processes. Multimodality treatment that includes surgery, chemotherapy, and radiation therapy is often recommended. The authors describe a typical case of anal duct carcinoma and its management. They also discuss the findings of a survey of the combined experience of members of the American Osteopathic College of Proctology and review the literature.
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4/35. Hidrosadenoma of the anal canal: a case report with review of the literature.

    Hidrosadenoma of the anal canal is an extremely rare tumour. Only nine cases with similar histologic structure have been described in the literature, most representing tumours resected from the anal or rectal mucosa. We present a case of anal hidrosadenoma with immunohistochemical staining features identifying it as a true sweat gland tumour.
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5/35. Perianal endometrioma: report of five cases.

    One of the gynecologists' commonest findings at pelvic laparotomy is endometriosis. Despite the fact that the presence of endometrial tissue in ectopic locations is commonplace, the finding of a perianal endometrioma is no more than a surgical curiosity. Very few cases have been reported. The present report describes five such verified cases. Interestingly enough, only two of the five cases were diagnosed preoperatively. The pathogenesis of endometriosis has been, and continues to be, controversial. The prevailing theories include 1) transtubal regurgitation of menstrual blood, 2) the coelomic metaplasia doctrine, 3) lymphatic dissemination, and 4) hematogenous spread. Pathologically the lesions may vary grossly from red-blue to yellowish-brown implants, ranging in size from microscopic to 1-2 cm in diameter. The definitive histologic diagnosis requires two of the following three features-glands, stroma, and hemosiderin pigment. The clinical manifestations depend upon the functional activity of the involved tissue and may range from an asymptomatic mass to the classic presentation of a mass increasing in size and becoming acutely painful during menstruation but subsiding in size and decreasing in tenderness between menstrual periods. These protean manifestations are readily illustrated by the cases presented. Anatomically the lesions are usually found in old episiotomy scars. Because these lesions are usually readily accessible, and because their exact nature is frequently not known preoperatively, the treatment of choice is local excision. Complementary hormonal therapy has been suggested, but the value of such treatment is not yet proven. Ovarian ablative therapy is also considered.
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6/35. Synchronous squamous and glandular neoplasia of the anal canal.

    A 48 year old man presented with invasive adenocarcinoma in the wall of a non-healing anal fistula. The subsequent abdomino-perineal resection specimen showed residual invasive carcinoma coexisting with in situ carcinoma of anal glands as well as in situ squamous carcinoma of the anal canal. The epithelium of the anal canal had koilocytotic features. dna hybridisation studies by the dot blot technique showed weak positivity for human papillomavirus (HPV) subtypes 16, 18. This case illustrates a number of important points--namely, anal fistulas, particularly non-healing fistulas should be biopsied to exclude malignancy; some adenocarcinomas of the anal arise in anal glands; the coexistence of glandular and squamous carcinoma with evidence of HPV infection is highly reminiscent of similar synchronous lesions of the uterine cervix and suggests that HPV may have an aetiological role in both squamous and glandular carcinomas of the anal canal.
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7/35. Perianal Paget's disease: report of five cases.

    Paget's disease of the anus is a rare perianal disorder. The condition is often associated with underlying invasive carcinoma. The prognosis is poor when rectal adenocarcinoma is present. Five own cases of perianal Paget's disease are presented. In two of our cases an underlying adenocarcinoma was found in the anorectum. adenocarcinoma is sweat gland ducts was found in one case. One patient developed an adenocarcinoma in the anorectal junction four years after the Paget diagnose. In only one of our cases no underlying adenocarcinoma was found.
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8/35. anal canal neuroendocrine carcinoma with Pagetoid extension.

    A case of anal canal neuroendocrine carcinoma with Pagetoid intraepithelial extension is presented. An 80-year-old man was admitted to hospital with a complaint of pain in the anorectal region. Clinical examination revealed a hard and fixed mass in the anal canal, and subsequent biopsy of the lesion showed it to be a carcinoma. The surgically resected specimen showed a solid tumor measuring 3.4 x 3.2 cm within the area from the surgical anal canal to the anatomical anal canal. Tumor cells proliferated predominantly with compact nests. Many tumor cells had a high nuclear-to-cytoplasmic ratio, dispersed chromatin, and conspicuous nucleoli. Additionally, neoplastic cells focally formed a glandular structure. Some polygonal neoplastic cells were small with round nuclei. A rosette-like arrangement was also focally observed. In addition, tumor cells exhibited Pagetoid extension into the overlying epithelium of the histological anal canal. Both the underlying original neoplastic cells and the Pagetoid spreading tumor cells showed cytoplasmic granules positive for Grimelius staining and immunopositivity for carcinoembryonic antigen, synaptophysin and cytokeratins 7 and 20. These findings are highly suggestive of neuroendocrine differentiation of adenocarcinoma cells. To the best of our knowledge, this is the first case of anal canal neuroendocrine carcinoma with Pagetoid extension into the overlying epithelium of the histological anal canal.
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9/35. Pseudoangiomatous stromal hyperplasia in lesions involving anogenital mammary-like glands.

    Pseudoangiomatous stromal hyperplasia (PASH), first reported in 1986, is nowadays a well-recognized change in the breast. We present three cases of lesions involving anogenital mammary-like glands demonstrating this feature. All patients were females (ages, 42, 43, and 53 years). Each presented with a solitary, 1.5- to 2-cm asymptomatic nodule. Locations included the perianal area, perineum, and labium majus. Histopathologically, one lesion was classified as low-grade phyllodes tumor, another as fibroadenoma, and in the remaining case PASH was found in the background of mild hyperplasia of anogenital mammary-like glands and substantial lipomatous metaplasia. In all lesions, PASH had an identical appearance to that in the breast, that is open, slit-like, often anastomosing channels devoid of erythrocytes and lined by discontinuous, often attenuated, inconspicuous cells without atypia or mitotic activity set in a hyalinized collagenous stroma. Quantitatively, PASH ranged in the above cases, forming a relatively small focus in the fibroadenoma and being quite extensive in the remaining two cases. In the phyllodes tumor, PASH areas exhibited focal hypercellularity and presence of myoid cells. In none of the cases were there cells with intranuclear inclusions or multinucleated cells. The lesions were surgically excised. Two patients with follow-up were disease-free at one and three years after the operation. As to our knowledge, PASH has not been previously described in the anogenital area, this feature seems to have been either overlooked or is genuinely rare in this location. It may occur in a preexisting lesion of anogenital mammary-like glands or may apparently by itself produce a clinically detectable lesion. The clinicopathologic features of PASH in the anogenital area seem to be identical to those in the breast.
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10/35. Basaloid squamous carcinoma of the anal canal with an adenoid cystic pattern: histologic and immunohistochemical reappraisal of an unusual variant.

    Two cases of a distinctive variety of basaloid squamous carcinoma (BSC) of the anal canal are described. Both occurred in female patients who presented with bleeding per rectum. Histologic evaluation of the tumors showed lobules and aggregates of medium-sized basaloid cells with distinctive peripheral palisading and focal areas of central, comedo-necrosis. Accompanying dysplasia of the overlying squamous mucosa was absent. However, the microscopic pattern was dominated by the presence of eosinophilic, hyaline, paucicellular basement membrane-like material around and within tumor nests. This appearance together with microcystic spaces simulated that of an adenoid cystic carcinoma. immunohistochemistry of the tumors revealed the following profile: CK7, CK5/CK6, 34betaE12 positive, CK14 focally positive but CK20 negative. The following were all negative: EMA, CEA, smooth muscle and muscle-specific actin, calponin, and S-100. The tumor cells exhibited diffuse nuclear positivity with p63. The eosinophilic basement membrane hyaline material was positive for collagen type iv and also for laminin. BSC of the anal canal with an adenoid cystic pattern is an infrequently encountered and reported variant, although it is seen more often in the aerodigestive tract. There may be an increased propensity for BSC with an adenoid cystic pattern to metastasize to the liver, but the number of cases encountered are too small to be definitive. The histologic differential diagnosis is true salivary gland-type adenoid cystic carcinoma and basal cell adenocarcinoma. immunohistochemistry and awareness of this unusual pattern of BSC will facilitate the correct diagnosis being reached.
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