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Enlarged extraocular muscles from metastatic signet ring carcinoma.

Enlarged extraocular muscles usually occur from inflammatory conditions, but in rare instances metastatic tumors that invade the muscles will require a biopsy and specific treatment. The authors report what they believe to be the third case of metastases to the extraocular muscles from signet ring cell adenocarcinoma of an unknown primary site.

Antineoplastic Agents↗

Intravenous leiomyomatosis with cardiac extension: successful surgical management and histopathologic study.

A case of intravenous leiomyomatosis with cardiac extension was treated successfully with a two-stage operation: resection of the intracardiac and intracaval mass for the first stage and total hysterectomy with bilateral salpingo-oophorectomy for the second. Tumors were located either within or in continuity to dilated uterine veins. A leiomyomatous protrusion of the wall of a small uterine vein was found in two remote sections. The involved veins were located alone in the layer of apparently normal uterine smooth muscles. The findings suggest that intravenous leiomyomatosis originates from the smooth-muscle component of uterine veins.

Aged↗

Myxoid leiomyosarcoma of the liver.

This is the first report of a myxoid leiomyosarcoma arising in a cirrhotic liver. The tumor was resected from a 64-year-old man. On gross examination, it was soft and hemorrhagic. The tumor was composed of deceptively benign-looking smooth muscle cells with clear cytoplasm suspended in a myxoid stroma with foci of hemorrhage. Immunohistochemistry and electron microscopy confirmed that this was a smooth muscle cell neoplasm. The abundance of glycogen and ultrastructural signs of smooth muscle differentiation were considered consistent with an immature smooth muscle cell phenotype consistent with the diagnosis of myxoid leiomyosarcoma. Since myxoid leiomyosarcomas are aggressive tumors, it is important to recognize them histologically and also bear in mind that these tumors can occur even in unusual extrauterine locations such as a cirrhotic liver.

Biomarkers, Tumor↗

Sinonasal fibrosarcomas, malignant schwannomas, and "Triton" tumors. A clinicopathologic study of 67 cases.

BACKGROUND: Sinonasal fibrosarcomatous neoplasms are uncommon tumors and there are no previous studies of a large number of such cases. The clinical and histologic features of 67 fibrosarcomatous neoplasms of the nasal cavity and paranasal sinuses are reported. METHODS: Multiple clinical and histologic parameters (including immunostain results) were analyzed to characterize the features important for histologic recognition of the tumors and for correlation with patient outcomes. RESULTS: Some tumors could be classified as malignant schwannomas or malignant "Triton" tumors, but their behavior was similar to that of the fibrosarcomas. Histologically, most tumors were very low-grade malignant neoplasms; however, 22% of patients died of their tumors. Factors that correlated with death were mitotic rate (greater than 4 mitoses per 50 high-power fields), increased tumor cellularity, and male sex. CONCLUSIONS: Many tumors originally were diagnosed as benign (by others), probably causing initial undertreatment of some patients. Proper recognition and histologic evaluation of the tumor are important to ensure the best therapy and optimal patient survival.

Adolescent↗

Concordance of histopathologic and cytologic grading in musculoskeletal sarcomas: can grades obtained from analysis of the fine-needle aspirates serve as the basis for therapeutic decisions?

BACKGROUND: Utilization of fine-needle aspiration (FNA) for the diagnosis of musculoskeletal lesions has been de-emphasized by many clinicians because of concerns regarding the technique's ability to supply all information necessary for prognostication and appropriate selection of therapy. Paramount among the concerns is the belief that FNA is incapable of supplying precise subtyping and grading in many cases. Secondary concerns regarding the use of FNA involve its perceived inability to supply adequate tissue for ancillary studies including immunohistochemistry and molecular diagnostic analysis. The authors investigated the ability of FNA to accurately subtype and grade a series of 107 primary sarcomas of the musculoskeletal system. METHODS: The files of the Departments of Pathology at Duke University Medical Center and the University of California at Los Angeles and the private consultation files of one of the authors were searched for all fine-needle aspirates of sarcomas arising within the musculoskeletal system. A total of 107 cases were obtained and reviewed by three board-certified cytopathologists. Each cytopathologist independently assigned subtype when possible and generated a grade for each of the sarcomas. Corresponding surgical material was available for 77 cases. The surgical material was re-reviewed for accuracy of diagnosis and assignment of grade independently of the cytologic examination. Correlation of cytologic grade with histopathologic grade was made and analyzed by the kappa test. In addition, agreement on grade between cytopathologists was analyzed, and accuracy of histologic subtype prediction by cytologic analysis was studied. RESULTS: Most of the sarcomas were cytologically graded as Grade 1 or 2 but were assigned a histologic grade of 2 or 3. Cytopathologist A graded 93% of the sarcomas as either Grade 1 or 2, cytopathologist B graded 89% of cases as Grade 1 or 2, and cytopathologist C graded 94% of cases as Grade 1 or 2. Histologic evaluation demonstrated 82% of cases to be Grade 2 or 3. Correlation of cytologic and surgical grade varied among the observers. Cytopathologist A showed the highest degree of correlation between cytologic and surgical grade with an r value of 0.5. The corresponding r values for cytopathologists B and C were 0.46 and 0.41, respectively. Correlation between cytologic and surgical grade was significant for all three observers (P < 0.001). Only nuclear grade showed a consistent correlation in predicting final surgical pathology grade. Cellularity, mitotic rate, and the presence of necrosis were not statistically significant for predicting histopathologic grade. Accurate exact subtyping by cytologic examination was achieved in approximately 55% of cases. Prediction of histopathologic subtype by cytologic analysis was most successful when a distinctive stroma was present or high-grade features indicative of malignant fibrous histiocytoma were observed. There was little agreement as to histopathologic type as predicated by cytology for low-grade spindle cell sarcomas. CONCLUSIONS: Although there was a statistically significant correlation between cytologically assigned grade and final histopathologic grade, statistical analysis revealed only a moderate correlation between the two with an overall r value of approximately 0.57. Cytologic analysis tended to undergrade in comparison to final histopathologic grading. Only analysis of nuclear atypia showed good correlation with final surgical grade. Fine-needle aspiration was only moderately successful at predicting histopathologic subtype for musculoskeletal sarcomas in this series.

Adult↗

Granular cell tumor: a clinicopathologic study of 110 patients.

The clinicopathologic features of 118 granular cell tumors (GCT) encountered at two affiliated hospitals were reviewed. A total of 110 patients were affected over this 32-year period of study (71 men, 39 women), and in 5% GCT were multiple. Patients ranged in age from 16 to 58 years (average 32 years) and were symptomatic for an average duration of 11 months prior to diagnosis. There was a greater than expected frequency of GCT among black patients (29%). Although tongue was the single most common anatomic site involved, relatively more GCT (44%) occurred in skin or subcutaneous tissue. Less common locations were breast parenchyma (10 cases), rectal mucosa and anus (6), vulva (4), esophagus and larynx (2 cases each). The correct preoperative diagnosis of this protean tumor was made in only three patients. GCT were surgically treated with the average diameter of resected tumor being 1.2 cm (range 0.2--3.5 cm). Pseudoepitheliomatous hyperplasia was noted in 11 tumors and in one vulvar GCT there was overlying in situ squamous cell carcinoma. Tumors were incompletely excised in 24 of 56 patients having adequate followup; only five of these 24 patients experienced a local recurrence of tumor. Malignant behavior was not observed. Results of histochemical and ultrastructural study are briefly discussed. The precise histogenesis of GCT is uncertain but Schwann cell origin is favored in most cases.

Adenofibroma↗

Development of a metastatic human colon cancer xenograft model in the nude mouse.

The objective of our experimental protocols was to develop a metastatic model for a human colon carcinoma xenograft in congenitally athymic nude mice. This model would be useful in evaluating the efficacy of radiolabeled monoclonal antibodies for detection and treatment of regional and distant metastases. The LS-174T human colon carcinoma line was used to establish primary subcutaneous tumors in nude mice. Mice were killed at varying time intervals to establish the incidence of spontaneous metastases. Only lung micrometastases were observed during the 2-month observation period. To increase the metastatic rate, the site of primary implantation was varied and/or surgical manipulations were performed. Excision of small primary tumors resulted in a low incidence of local recurrence and no distant metastases. However, with excision of large primary tumors, a high local recurrence rate was noted and over 30% of mice had gross metastases. Mice bearing hind footpad tumors underwent excision when tumors were at least 1 cm in size. There were no local recurrences, but by 8 weeks over 40% had large pulmonary metastases. The LS-174T tumor was also established as a primary implant in the spleen from which 10 to 15% of the mice developed liver or lung metastases. The LS-174T tumor can metastasize in the nude mice and the latter two models may prove very useful in imaging and therapy studies.

Adenocarcinoma↗