Overexpression of p53 in disseminated superficial actinic porokeratosis with and without malignant degeneration.
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Biomedical subjects
Publications and source records attributed to X Matías-Guiu.
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BACKGROUND: Uterine papillary serous adenocarcinoma (UPSA) is a highly aggressive neoplasm with a great tendency for dissemination. p53 and c-erbB-2 immunoreactivity and DNA ploidy are considered to be indicators of prognosis for endometrial carcinomas. METHODS: Ten cases of patients with UPSA are reported. An attempt to relate pathologic findings with immunohistochemical stains for p53- and c-erbB-2-associated proteins, ploidy, and survival was made. RESULTS: Three patients were classified as having Stage I; three, Stage II; two, Stage III; and two, Stage IV. Myometrial invasion was present with nine tumors and involved over 50% of the myometrial thickness in five. Uterine lymph vessel invasion was detected in seven cases. Peritoneal spread occurred in six patients. Overexpression of p53 was observed in six tumors, immunoreactivity for c-erbB-2 in four, and aneuploidy in seven. However, only peritoneal spread correlated significantly with survival (P < 0.005). CONCLUSIONS: UPSA is a tumor with a high metastatic potential that exhibits immunoreactivity for p53 and c-erbB-2 and aneuploidy more often than that reported for conventional endometrioid adenocarcinomas.
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Invasive prolactinoma is a relatively infrequent variety of macroprolactinoma characterized by a fast and aggressive growth, with infiltration to adjacent structures, and whose management is frequently difficult. We present the case of a fatal invasive macroprolactinoma in whom resistance to different dopaminergic drugs developed.
The clinicopathologic features of 44 serous borderline tumors (SBT) of the ovary were evaluated. Nineteen were Stages II and III, and 9 had invasive peritoneal implants. All 19 patients received chemotherapy and 4, who had invasive implants, died of disease after 3, 4.3, 8, and 9 years. The other 25 patients were free of tumor 1-14 years (mean, 5.3 years) after surgery. Coexpression of low molecular weight keratins (AE1, CAM 5.2) and vimentin was found in all tumors and their implants. No significant differences were found between SBT with different volume-corrected mitotic indices (M/Vi) with respect to gross features, presence or absence of implants, stage, and survival. Cytometric DNA analysis also was performed on the primary ovarian tumors and the implants. Twenty-one primary tumors had diploid or tetraploid histograms, and 23 had aneuploid histograms. DNA ploidy of the primary ovarian tumors did not correlate with gross features, the presence or absence of implants, M/Vi, stage, and survival. The data from this study confirm that most SBT are clinically benign, but SBT with invasive implants may behave aggressively. Expression of intermediate filaments, M/Vi, and DNA ploidy evaluation of the primary ovarian tumors seem to be of no value in predicting prognosis. However, four of seven patients with aneuploid DNA implants died of tumor.
We report on the appearance of centrifugally spreading ulcers with undermined borders in two patients with chronic recurrent erythema elevatum diutinum controlled with dapsone. The ulcerated lesions were consistent on clinical and pathologic examination with the diagnosis of pyoderma gangrenosum. They eventually responded to treatment with oral corticosteroids. The addition of cyclosporine was required in one case. No associated disease was found in any of the patients. The possible pathophysiological mechanisms of this uncommon association are reviewed.
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The clinicopathologic features of five cases of verrucous carcinoma of the vulva and their staining pattern with antikeratin monoclonal antibodies AE1 and AE3 were compared with those of conventional squamous cell carcinoma. Two patients had local recurrences but none died of the tumor. AE1 and AE3 antibodies stained the entire epithelial thickness in both verrucous and squamous cell carcinoma, but in the former the positivity was uniform and homogeneous everywhere, while in squamous cell carcinoma the positivity was extremely disorganized and patchy. The pattern of expression of monoclonal antibodies AE1 and AE3 confirms that verrucous carcinoma is an extremely well-differentiated squamous neoplasm in contrast to squamous cell carcinoma, which is heterogeneous from a viewpoint of differentiation.
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Pigmented dermatofibrosarcoma protuberans, first described by Bednar under the term storiform neurofibroma, is an infrequent neoplasm accounting for 1 to 5% of cases of dermatofibrosarcoma protuberans. Bednar's tumor is composed of spindle shaped cells arranged in a distinctive storiform or carthwheel pattern, and melanin-containing dendritic cells scattered within the tumor. The differential diagnosis with (non-pigmented) dermatofibrosarcoma protuberans is based on the presence of this population of pigmented cells. Two cases of pigmented dermatofibrosarcoma protuberans (Bednar's tumor) are reported here in, and a discussion follows on the clinicopathological features of this neoplasm and the different hypotheses on its histogenesis.
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The autosomal dominant multiple endocrine neoplasia type 1 (MEN1) syndrome is characterized by neoplasia of parathyroids, anterior pituitary, and gastrointestinal and pancreatic neuroendocrine tissues. Recently the gene responsible for the MEN1 syndrome has been identified on chromosome region 11q13. Most of the described mutations are nucleotide substitutions and small deletions affecting exons 2 and 3, causing protein truncation. Only one mutation in exon 5 has been found, and this corresponds to a MEN1 sporadic case. Small insertions are also rare. We studied a MENI family composed of five members, two of whom were clinically affected. We found a new germline 1 basepair insertional mutation affecting the exon 5 of the MEN1 gene in the two members affected in this MEN1 family.