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Biomedical subjects

J F Val-Bernal

Publications and source records attributed to J F Val-Bernal.

At least 55 records · Page 3Linked to original sources

Extravulvar subcutaneous cellular angiofibroma.

Cellular angiofibroma is a rare distinctive mesenchymal neoplasm of the vulva or perineal region. We report here one unique extravulvar case. A 43-year-old woman presented with an asymptomatic tumor, 7 cm in diameter, located in the subcutaneous tissue of the chest below the left submammary sulcus. Histologically, the lesion was composed of uniform spindled stroma cells, numerous thick-walled vessels, and scarce mature adipocytes. An additional feature was the presence of prominent perivascular lymphoid aggregates. The stromal cells were positive for vimentin and negative for CD34 and muscle, epithelial, myoepithelial, or neural markers. Although nasopharyngeal angiofibroma or the group of acral angiofibromas have a concurrent heading, cellular angiofibroma should not be mistaken with them. Differential diagnosis of this distinctive tumor especially includes aggressive angiomyxoma, angiomyofibroblastoma, superficial angiomyxoma, vascular myxolipoma, and other tumors with spindle cells reminiscent of those in angiofibroma.

Adult↗

Isolated fibrinoid vasculitis in renal angiomyolipoma.

Renal angiomyolipoma is a tumor composed of a mixture of thick-walled blood vessels, smooth muscle, and mature adipose tissue. It may present as a single unilateral lesion or as a multifocal uni- or bilateral neoplasm. The histologic spectrum of angiomyolipoma is wide but as far as we know intratumoral fibrinoid vasculitis has not been described in the tumor. This is the first report of isolated intratumoral fibrinoid vasculitis observed in two nodules of a unilateral multifocal renal angiomyolipoma. The vasculitis arose in a hypertensive 62-year-old woman who presented with a history of dull right flank and low back pain of 3 months' duration. There were no signs of generalized disease. Immunophenotyping of the vascular cellular infiltrate disclosed abundant T lymphocytes, significant numbers of histiocytes, and absence of B lymphocytes. The diagnosis of isolated intratumoral arteritis depends on the exclusion of systemic disease. It is important to distinguish cases like this one to avoid misdiagnosis and to prevent unnecessary treatment.

Angiolipoma↗

Spindle cell hemangioendotheliomas associated with multiple skeletal enchondromas: a variant of Maffucci's syndrome.

An unusual case of Maffucci's syndrome confined to an extremity with an additional superficial lymphangioma in the same limb is described. The patient, a 47-year-old woman, presented with several painful subcutaneous spindle cell hemangioendotheliomas (SCHs) and multiple lytic bone lesions, some calcified, in the left leg. She has been followed for two years and continues to do well. The combination of SCHs and multiple enchondromas (ENCHs) should be searched in any patient who presents with SCHs or multiple ENCHs, because there is a significant risk of malignant transformation.

Bone Neoplasms↗

Solitary neurofibroma of the gallbladder. A case report and review of the literature.

Benign non-epithelial neoplasms of the gallbladder are rare. A case of incidental neurofibroma of the gallbladder in a 44-year-old man without neurofibromatosis 1 is reported. The intramural tumor, located in the neck of the gallbladder, was associated with lithiasis, chronic cholecystitis and localized fundic adenomyomatosis. Seven cases including the present one are reviewed. The seven patients ranged in age from 44 to 77 years, with an average age of 61.6 years. Four of the seven patients were women. Five tumors appeared as mural nodules, and two showed intraluminal growth. The most common location was the body of the gallbladder. The size of the lesion varied from 0.3 to 5.3 cm, with a mean size of 1.3 cm. Five of the seven cases were incidentally found in a gallbladder resected for cholelithiasis. Using ultrasonography, two cases with no lithiasis were detected preoperatively, presenting as intracavitary growing lesions by ultrasonography. It is possible that neurofibroma of the gallbladder may be more common than the small number of known cases indicates.

Adult↗

Laryngeal melanosis: report of four cases and literature review.

OBJECTIVE: Laryngeal melanosis is a rare condition defined by the presence of melanocytes within the laryngeal epithelial lining. Our aims were (1) to review our cases together with those in the literature, and (2) to determine whether melanocyte incidence is increased with exposure to irritant stimuli such as tobacco. METHODS: A retrospective study of all cases diagnosed with laryngeal melanosis in our hospital from January 1, 1990, to December 31, 1996, was accomplished. To determine the melanocyte incidence in the normal larynx as well as the influence of tobacco in development of laryngeal melanosis, 16 age-matched controls, 8 of whom were smokers and 8 of whom were not, were chosen, and a histochemical and immunohistochemical study was performed. The following antibodies were used: S-100 protein, CD1a, and HMB-45. A comparative study of the melanocyte incidence between patients with laryngeal melanosis and the controls was carried out. Also, a comparative study between smoking and nonsmoking patients was performed. RESULTS: Laryngeal melanosis was diagnosed in 4 patients at our hospital during this period of time. In the comparative study, the number of melanocytes in the 4 patients with laryngeal melanosis was higher than in the 8 smoking (p < 0.01, Mann-Whitney U test) and 8 nonsmoking (p < 0.01) controls, and there was a trend toward a higher number of melanocytes in the 8 smoking patients than in the 8 nonsmoking (p = 0.064) controls. CONCLUSIONS: Laryngeal melanosis was more frequent in smoking men older than 50 years. Our observations underline the association of LM with larynx carcinoma and its relation to a stimulus such as tobacco. In fact, we have found activated melanocytes in our cases of laryngeal melanosis. They were identified by immunoreactivity for HMB-45.

Aged↗

Lipoleiomyosarcoma of the mediastinum.

A case is described of a mediastinal soft tissue sarcoma, in a 76-year-old man, characterized microscopically by the intimate combined features of liposarcoma and leiomyosarcoma. The lipomatous component consisted of well differentiated liposarcoma with myxoid areas. The smooth muscle component was characterized by fascicles of spindle cells showing nuclear atypia and scattered mitoses. The spindle cells displayed intense reactivity for alpha smooth muscle actin (ASMA), desmin and vimentin. Differential diagnoses included dedifferentiated liposarcoma, spindle cell liposarcoma, angiomyolipoma and myolipoma. To the best of our knowledge no case of sarcoma in the mediastinum with combined features of liposarcoma and leiomyosarcoma has been reported. The dual lineage differentiation of the lipoleiomyosarcoma may be difficult to identify in small biopsy samples which may not represent the tumor as a whole. Lipoleiomyosarcoma should be included in the differential diagnosis of soft tissue tumors arising in the mediastinum.

Actins↗

Giant intravenous glomus tumor.

We report a painful intravenous glomus tumor located in the right forearm of a 79-year-old woman. The tumor originated from the wall of a vein, protruded into its lumen and was completely excised. The largest dimension of the tumor occluding the vein was 14 cm. Tumor cells were characterized immunohistochemically by the presence of vimentin, alpha-smooth-muscle actin, and collagen IV. Intravascular spread of the glomus tumor is rare and has been described in the stomach and subcutaneous tissue. An entirely intravenous glomus tumor has been reported only three times. However, a huge intravenous growth as in our case appears never to have been reported. A review of the intravascular cases showed that the average age of presentation is 61.5 years (range 40-79 years) and the most frequent location is the forearm. Despite this intravascular growth, there is no evidence of aggressive clinical behavior, recurrence or metastasis. The pathologist must be aware of this variant of glomus tumor to avoid misdiagnosis and unnecessary additional treatments.

Actins↗

Chromophobe cell renal carcinoma: DNA flow cytometry and proliferation status in 9 cases.

Nine cases of chromophobe cell renal carcinoma are reported, 2 in pregnant women. One neoplasm progressed and the patient died of disease, and this tumor presented the highest mitotic index and focal positivity to Ki-67 antibody. DNA flow-cytometric study discovered 89% of aneuploidy, with 3 tetraploid cases. We conclude that chromophobe cell renal carcinoma is a relatively indolent but clearly malignant neoplasm. We suggest that the mitotic index could be the best method to predict malignant behavior. The high incidence of aneuploid cell lines may be due to structural anomalies; it is not indicative of malignant behavior.

Adult↗

Prognostic analysis of astrocytic gliomas correlating histological parameters with the proliferating cell nuclear antigen labelling index (PCNA-LI).

Eighty out of 250 cases of astrocytic glioma collected from a practice served by a single clinical team over a 15-year period were studied using a full complement of clinical, follow up, histopathological analysis and proliferating cell nuclear antigen (PCNA) immunostaining for the obtention of the PCNA-labelling index (LI). A statistical evaluation and discriminant analysis were carried out with the aim of clarifying the importance of various parameters as predictors of tumor behaviour. Data are correlated with survival (with a 10-year follow up). A significant correlation with survival was found when histological grouping and the PCNA-LI were studied with the Cox test. Most significant features were histological as detected using classical techniques including histological grading. The utilization of objective values (mitosis, cellular density and necrosis) appears to be useful in grading astrocytic tumors. Our results emphasize the importance of cytological, histological and PCNA-LI parameters as predictors of tumor behaviour.

Adolescent↗

Diffuse expansive cavernous hemangioma of the gallbladder.

Although hemangiomas are common hepatic tumors, their occurrence in the gallbladder is extremely rare. In the present report, we describe a diffuse expansive cavernous hemangioma of the gallbladder. The patient was a 50-year-old woman presenting with a 24-hour history of persistent right upper abdominal quadrant pain. The lesion caused a voluminous enlargement of the gallbladder creating a clinical impression of a cystic mesenteric tumor. The vascular origin of the tumor was confirmed by immunohistochemistry. Only 4 cases, including the present one of cavernous hemangioma involving the gallbladder have been reported in the English literature. The average age of presentation is 52.5 years (range 43-60 years), and the female to male ratio is 3:1. The lesions can be symptomatic and may have confusing radiographic appearances. Lithiasis has not been associated with the cavernous hemangioma of the gallbladder.

Female↗

Sarcomatoid chromophobe cell renal carcinoma: immunohistochemical and lectin study in one case.

We present one case of sarcomatoid chromophobe cell renal carcinoma with an indolent clinical course and assume that the carcinomatous component may affect the biologic behavior. The patient was a 61-year-old man who underwent right radical nephrectomy for a 11.2 cm tumor in the lower pole. The immunohistochemical findings demonstrate that EMA and cytokeratins 8 and 18 are useful markers for the sarcomatoid fraction, and the lectin study shows a loss of surface blood antigen. Chromophobe cell carcinoma may convert into sarcomatoid carcinoma. The existence of sarcomatoid renal cell carcinoma as a distinct entity should be re-considered.

Adenocarcinoma↗

Renal perineurioma in childhood.

Perineurioma of the kidney in a 7-year-old girl is described. The tumor in the upper pole was discovered during the evaluation of a urinary tract infection. The neoplasm measured 2.6 cm and radiographically it mimicked a nephroblastoma. The diagnosis of perineurioma was confirmed with histologic, immunohistochemical, and ultrastructural studies. These revealed findings typical of perineurioma of other locations. Histopathologically, differential diagnosis may include nephroblastoma, mesoblastic nephroma, neurofibroma, schwannoma, malignant peripheral nerve sheath tumor with perineural cell differentiation, and other benign and malignant mesenchymal tumors with spindle cell pattern. To the best of our knowledge, we report a unique renal perineurioma in a child.

Biomarkers, Tumor↗