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1/95. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma.

    Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously.
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ranking = 1
keywords = stromal
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2/95. Malignant fibrous histiocytoma of the conjunctiva.

    We report the clinical and pathological findings in a case of malignant fibrous histiocytoma in the conjunctiva of a 60-year-old man. The patient initially had an atypical limbal lesion, resembling a pterygium, which was excised. Two local recurrences, noted during the following year, were treated by surgical excision followed by cryotherapy. Histopathologic examination of the conjunctival lesions showed a stromal neoplastic infiltrate composed of atypical spindle cells and histiocytelike cells. The immunohistochemical and ultrastructural studies suggested that the tumor was composed of various cellular elements: fibroblasts, myofibroblasts, and histiocytes.
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ranking = 1
keywords = stromal
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3/95. Unilateral hydronephrosis and recurrent endometrial stromal sarcoma with review of the literature.

    In this case we present a woman with arterial hypertension. Further examination showed an unilateral hydronephrosis caused by extrinsic compression. A tumoral mass, invading the caval inferior vein and the renal vein, is the very origin of the compression. This mass is a recidive of an endometrial stromal sarcoma for which she had a hysterectomy in 1984.
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ranking = 259.633618111
keywords = endometrial stromal, stromal
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4/95. Actin inclusions in stromal cells of fibroepithelial tumor of breast: immunohistochemical and ultrastructural studies.

    An uncommon occurrence of actin inclusions in the stromal cells of a benign fibroepithelial tumor of breast is reported. Histologically, many of the stromal cells contained round and eosinophilic intracytoplasmic inclusions identical to those seen in inclusion body fibromatosis. Ultrastructurally, these inclusions represented dense spherical clumps of microfilaments derived from rough endoplasmic reticulum. The literature was reviewed and follow-up data showed that the clinical course of these morphologically distinctive benign fibroepithelial tumors was relatively indolent if completely excised, in contrast to inclusion body fibromatosis, which commonly recurs. The pathogenesis may be related to abnormal production of truncated actin filaments or alteration in microenvironment.
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ranking = 6
keywords = stromal
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5/95. Low-grade endometrial stromal sarcoma with cardiovascular involvement--a report of three cases.

    BACKGROUND: In low-grade endometrial stromal sarcoma, it has been reported that vascular space involvement in surgical specimens is found in over 50% of patients. However, in contrast to intravenous leiomyomatosis, it has been thought that further tumor extension to large vessels is rarely observed. CASES: We present three cases of low-grade endometrial stromal sarcoma with cardiovascular involvement by recurrent tumors observed on imaging studies. Two cases demonstrated tumor infiltration inside the inferior vena cava while the other case showed tumor growth in the left ventricle. CONCLUSION: This report suggests that attention should be paid to the possibility of cardiovascular invasion during the entire course of this disease.
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ranking = 311.5603417332
keywords = endometrial stromal, stromal
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6/95. Infiltrative glomus tumor arising from a benign glomus tumor: a distinctive immunohistochemical pattern in the infiltrative component.

    Malignant glomus tumors (MGT) are rare. Although metastatic MGT has been reported, most MGT have only been locally aggressive, some with multiple local recurrences. We report an additional case of an infiltrative glomus tumor. In addition to the pattern of immunohistochemical staining for alpha-smooth muscle actin (SM-actin) previously described, we performed immunohistochemical stains for Ki-67 and CD34. The infiltrative component of the glomus tumor showed variably decreased staining with SM-actin and occasional tumor cells showed nuclear staining with Ki-67. CD34 staining occurred in stromal cells forming the pseudocapsule in the benign component of this tumor and in other benign glomus tumors. The infiltrative component showed increased CD34 stromal cells. Although Ki-67 staining showed only an occasional proliferative cell, the immunohistochemical staining pattern of CD34 and SM-actin raise the possibility that the infiltrative component of this tumor may have differences in the degree of differentiation from the circumscribed part and that local factors could support its spread from a conventional benign glomus tumor.
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ranking = 2
keywords = stromal
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7/95. hemoperitoneum is an initial presentation of recurrent granulosa cell tumors of the ovary.

    Ovarian sex cord-stromal tumors account for less than 5% of all ovarian carcinoma, of which granulosa cell tumors account for 70%. These tumors have a propensity for indolent growth and late recurrence; they may even occur 25 years after initial treatment. We report a 44-year-old woman with hemoperitoneum (acute abdomen) after initial treatment 10 years earlier for granulosa cell tumor of the ovary. This case re-emphasizes the need for long-term follow-up in patients with stromal cell tumors of the ovary and considers the possibility of recurrence when presented with acute abdomen after conservative treatment.
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ranking = 2
keywords = stromal
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8/95. Fine-needle aspiration cytology of desmoplastic malignant melanoma metastatic to the parotid gland: case report and review of the literature.

    We report a case of desmoplastic malignant melanoma metastatic to the parotid gland initially evaluated by fine-needle aspiration. The cytologic findings consisted of scattered spindle cells in a background of heterogeneous lymphoid cells. The spindle cells were scant and displayed mild cytologic atypia. In addition, rare stromal fragments were also present. Cytoplasmic pigment and intranuclear cytoplasmic inclusions were not seen. The initial impression was that of a reactive lymph node with fibrosis. In retrospect, rare spindle cells displayed moderate atypia. In addition, the stromal fragments were cellular and contained spindle cells with mild atypia. These cytologic findings along with a known history of malignant melanoma should provide clues to the correct diagnosis of desmoplastic malignant melanoma. Diagn. Cytopathol. 2000;22:97-100.
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ranking = 2
keywords = stromal
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9/95. Warty (condylomatous) squamous cell carcinoma of the penis: a report of 11 cases and proposed classification of 'verruciform' penile tumors.

    Within the spectrum of penile squamous cell carcinomas, those that we descriptively refer to collectively as the "verruciform" lesions are particularly difficult to subclassify. In a review of 50 such tumors, we found 11 distinctive neoplasms with condylomatous features conforming to the appearance of so-called "warty (condylomatous) carcinoma." The average patient age was 55 years and the average duration of disease was 19 months. The primary tumor involved multiple anatomic sites (glans, coronal sulcus, and foreskin) in seven cases and a single site (glans or foreskin) in four cases. Grossly, white to gray cauliflower-like tumors typically measuring approximately 5 cm were noted. Histologically the tumors were mainly papillomatous with acanthosis and hyperkeratosis. The papillae had prominent fibrovascular cores. The most conspicuous microscopic findings were striking nuclear atypia of koilocytotic type and clear cytoplasm. The interface between tumor and stroma was irregular in the majority of cases; deep invasion of corpus cavernosum was noted in five cases. The differential diagnosis included verrucous carcinoma, low-grade papillary squamous cell carcinoma, not otherwise specified, and giant condyloma acuminatum. Among other differences, the first two lesions show no koilocytotic changes and the last lacks malignant features and irregular stromal invasion. Metastatic spread occurred in two patients; both are alive with evidence of recurrent disease 12 and 72 months after initial diagnosis. A third patient was alive with recurrent disease 12 months after diagnosis. Five patients were free of disease 8, 12, 24, 52, and 108 months after diagnosis. Three patients were lost to follow up. Warty (condylomatous) carcinomas of the penis are morphologically distinctive verruciform neoplasms with features of human papillomavirus-related lesions and should be distinguished from other verruciform tumors so that differences in behavior, if any, between these tumors will become established.
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ranking = 1
keywords = stromal
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10/95. diagnosis of recurrent uterine carcinosarcoma by fine-needle aspiration cytology: report of a case.

    Uterine carcinosarcomas are uncommon, aggressive neoplasms usually afflicting postmenopausal women. Histologically, they are characterized by the presence of both malignant epithelial and stromal cells. The latter may be either homologous or heterologous in nature. The cytologic diagnosis of carcinosarcoma via fine-needle aspiration (FNA) was previously described for primary tumors arising in the parotid gland, breast, lung, and ovary. Although the diagnosis of uterine carcinosarcoma via cervicovaginal, endometrial, and peritoneal fluid cytology has been described, the FNA cytology diagnosis of recurrent uterine carcinosarcoma has only been rarely described. We present a case of recurrent uterine carcinosarcoma in a 59-yr-old woman, diagnosed by ultrasound-guided FNA cytology. Cytologic smears showed a biphasic neoplasm consisting of both malignant columnar epithelial and spindle cells, in a background of tumor diathesis. This case illustrates the diagnostic utility of aspiration cytology in the investigation of recurrent carcinosarcoma in clinically suspicious lesions arising postsurgery.
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ranking = 1
keywords = stromal
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