Gastric metastasis of malignant fibrous histiocytoma presenting as gastrointestinal bleeding.

Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. With only 4 cases described in the available English literature up to now, gastric metastasis of MFH is extremely rare. Among them only one case has been reported to lead to gastrointestinal bleeding. We report the case of a 55-year-old woman who underwent total gastrectomy, 14 months after resection of an MFH from the right side of retroperitoneum. The neoplasm was detected at the time of diagnostic workup for upper gastrointestinal bleeding. The resected specimen contained multiple polypoid nodular lesions which were located in the greater curvature. The clinical and pathological characteristics of gastric metastasis of MFH are presented herein with review of literature.

Myofibroma of the external auditory canal: report of an adult case.

UNLABELLED: Myofibroma of the external auditory canal: report of an adult case. OBJECTIVE: Myofibroma is a benign fibrous proliferative disease generally reported to occur in neonates and children. Similar lesions have been reported in adults, but solitary tumours affecting the external auditory canal are extremely rare. Only one such case has been reported in the literature. CASE REPORT: The case of solitary myofibroma occurring in the external auditory canal of a 42-year-old woman is presented. The diagnosis of myofibromatosis was made on the basis of its histological appearance and corroborated by immunohistochemical staining. CONCLUSIONS: Clinicians and pathologists must be aware that myofibromatosis may occur in adults at extremely rare locations. Myofibroma should be included in the differential diagnosis of fibrous lesions in the external auditory canal.

Prognostic value of proliferating cell nuclear antigen immunostaining in pediatric rhabdomyosarcomas.

BACKGROUND: The levels of proliferating cell nuclear antigen (PCNA) are almost negligible in long-term quiescent cells and increase dramatically during the cell cycle. Recently, the monoclonal antibodies to PCNA have been used to demonstrate the proliferative component of paraffin-embedded tumor tissues. It has been shown to be available as a simple histological marker of proliferative activity and the PCNA labeling index has been correlated with the prognosis of several malignant neoplasms. METHODS: Formalin fixed, paraffin embedded tissue specimens of 29 primary pediatric rhabdomyosarcomas were immunostained by using an anti-PCNA monoclonal antibody (DAKO PCNA PC10). The relationship between the PCNA index and prognosis, clinicopathological features and survival were assessed retrospectively. RESULTS: The mean PCNA index for the whole series was 54%. There was no correlation between PCNA index and any of the clinicopathological characteristics. However, patients having tumors with a high (> 54%) PCNA index demonstrated significantly lower survival rates than tumors with a low (< 54%) PCNA index (P = 0.01). Moreover, there were significantly more patients with relapse or progressive disease in the high PCNA index group (P = 0.005). CONCLUSION: The PCNA labeling index can be a useful prognostic factor and a good indicator of recurrence and/or survival in patients with rhabdomyosarcoma.

Multiple vascular leiomyomas involving bilateral adrenal glands, spleen, and epicardium, associated with bilateral testicular microlithiasis and empty sella turcica.

Leiomyomas can arise from any tissue having smooth muscle as one of its constituents, especially gastrointestinal and genitourinary systems, less frequently in the skin, and rarely in deep soft tissue. An unusual case of multiple vascular leiomyomas involving bilateral adrenal glands, spleen, and epicardium, associated with bilateral testicular microlithiasis, and empty sella turcica is reported in a 15-year-old boy. The adrenal masses were incidentally discovered by ultrasonography. These three findings, taken individually are rare, and the constellation of the three, to the best of our knowledge, is the first recorded example. Furthermore, bilateral involvement of the adrenal glands with leiomyomas is, by itself, very uncommon. The tumors considered benign were surgically removed. Orchiectomy wasn't performed. The boy is well after 1.5-year follow-up under cortisone, 9alpha-fludrocortisone, and testosterone therapy.

Immunohistochemical detection of p53 protein in rhabdomyosarcoma: association with clinicopathological features and outcome.

PURPOSE: Alteration in the p53 tumor suppressor gene is the most common tumor specific genetic change identified in most major cancer types including rhabdomyosarcomas. To investigate the overexpression of p53 and its relation to clinical features and outcome in patients with rhabdomyosarcoma (RMS), an immunocytochemical study was performed. METHODS: Formalin-fixed paraffin embedded tissue sections obtained from 42 cases of RMS were immunostained with a mouse monoclonal antibody p53-D07. Staining was assessed by evaluating the percentage of p53 immunopositive cancer cell nuclei. RESULTS: Nuclear accumulation of p53 protein was detected in 8 of 42 (19%) samples. Clinical analyses of patients demonstrated no correlation between positive staining and age, sex, histological subtype, stage and overall survival. This analysis, however, was limited by the small number of patients who demonstrated p53 immunostaining. Nonetheless, a statistically significant association was observed between p53 expression and adverse outcome. Nuclear p53 expression was associated with disease progression or recurrence (p <0.001) and with a worse event free survival (p = 0.0015). CONCLUSION: The nuclear p53 immunoreaction rate is low in RMS, but p53 expression appears to correlate with poor prognosis.

Syndrome of congenital cutis laxa with ligamentous laxity and delayed development: report of a brother and sister from Turkey.

Congenital cutis laxa with ligamentous laxity and delayed development has recently been defined as a distinct entity of autosomal recessive inheritance. Here we report on 2 new cases of this syndrome. With severe manifestations in the male, X-linked dominant inheritance is discussed. Results of ultrastructural studies of skin and biochemical studies are reported.

Long-term results of cryotherapy on malignant epithelial tumors of the conjunctiva.

We studied the results of combined treatment with excision and cryotherapy with the nitrous oxide probe in 22 eyes of 20 patients with intraepithelial tumors or squamous cell carcinomas of the conjunctiva. Patients were followed up for five to 12 years. Only two recurrences (9%) were seen, both of which occurred within the first two years. Over the long term, excision combined with cryotherapy decreased the recurrence rate of intraepithelial tumors and squamous cell carcinomas of the conjunctiva.