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[CT morphology of malignant schwannoma].

Thirty patients with histologically confirmed malignant schwannomas were examined. In five patients the lesions were malignant neurofibromas, as part of von Recklinghausen's disease, the others were solitary malignant schwannomas. CT appearances of malignant schwannomas are variable. Shape, structure and attenuation vary considerably. Most malignant schwannomas present with the CT criteria of benign tumours. Altogether neither the solitary schwannomas, nor those associated with von Recklinghausen's disease show specific diagnostic features, whether for the primary tumour or for recurrences. Reliable information on the nature of these lesions is possible in a few cases only.

Adult↗

EUS-FNA of recurrent postoperative extraluminal and metastatic malignancy.

BACKGROUND: EUS-guided FNA is safe and accurate for the diagnosis of benign or malignant neoplasia and lymphadenopathy; however, its role in the diagnosis of recurrent malignancy is not well described. METHODS: A prospectively updated EUS-guided FNA cytology database was used to identify patients in whom a diagnosis of postoperative, recurrent, extraluminal, or metastatic malignancy was made over a 5-year period. Only patients with a positive EUS-guided FNA were included in the analysis. All had undergone surgery for the primary malignancy and were in clinical and/or radiographic remission before the initial suspicion of tumor recurrence. RESULTS: Twenty-one patients underwent EUS-guided FNA of 21 lesions (19 masses, 2 lymph nodes) because of a suspicion of recurrent malignancy based on CT (n = 17) or EUS (n = 4) findings. Median time from the initial diagnosis to recurrence was 26 months (range 5-276 months). Lesions were located in the pancreas (9 patients), mediastinum (7), liver (3), perigastric region (1), and liver hilum (1). EUS-guided FNA (mean number of needle passes, 4.5; range 2-8) obtained diagnostic material for recurrent malignancy in all patients as follows: esophageal (6 patients), renal cell (6), pancreatic (2), breast (2), colon (2), bile duct (1), Ewing's sarcoma (1), and lung (1) cancer. No complication was encountered. Transgastric EUS-guided FNA (4 patients), distal, or transesophageal EUS-FNA (2) proximal to a surgical anastomosis was required to confirm recurrence in all 6 patients with esophageal cancer. The initial cytologic diagnosis of recurrent malignancy was made by EUS in 20 of 21 (95%) patients. One patient with recurrent breast cancer had CT-guided FNA of a right lung mass preceding EUS-guided FNA of an AP window lymph node. CONCLUSIONS: EUS-guided FNA can detect and safely diagnose recurrent malignancy in the mediastinum, retroperitoneum, and liver. When possible, correlation between EUS-guided FNA cytology and original tumor histopathology/cytology, or the use of immunostaining to confirm the diagnosis, is recommended.

Adenocarcinoma↗

New endoureteral double-J stent resists extrinsic ureteral compression.

The disadvantages of highly flexible endoureteral (double J) stents in the face of tumor-induced extrinsic ureteral compression are a consequence of insufficient cross-sectional stability, leading to stent compression and thus to hydronephrosis or pyonephrosis. The newly developed tumor stent, which is used in cases of tumor-induced ureteral compression, is available in sizes from 6F to 8F in diameter and 24 to 32 cm in length. The shaft consists of a combination of high-stability plastics that presents sufficient elasticity in length. Both ends are made of extremely elastic J parts, guaranteeing stable fixation. Compared with common double-J stents with the same outside diameter, the tumor stent possesses a comparable interior diameter and compared with available stents promises sufficient interior flow in the face of extrinsic diseases. The application can be undertaken using well-known endoscopic techniques, needs no special instrumentation, and entails no learning curve. To date, 49 stents were placed at our urologic departments without any problems, the latest stent remaining for 15 months. Tumor-induced compression or a higher rate of encrustation were not seen. All patients tolerated these stents well. In our opinion, the new stabilized endoureteral stent can be seen as a better solution than percutaneous nephrostomy or frequent stent changing to tumor-induced extrinsic ureteral compression.

Constriction, Pathologic↗

Management of ureteral obstruction secondary to pelvic malignancies.

PURPOSE: To assess the efficacy of urinary diversion (internal v external) in the management of ureteral obstruction secondary to pelvic malignancies and the patients' quality of life after diversion. PATIENTS AND METHODS: Thirty-seven patients presented with malignant ureteral obstruction secondary to primary neoplasms of the pelvis or metastatic disease of the pelvis and retroperitoneum and underwent urinary diversion. Patients were categorized into two groups according to the success (Group I) or failure (Group II) of internal stent drainage. Successful drainage was defined according to either radiologic study or the serum creatinine concentration in the case of a solitary kidney. "Useful life" was defined as satisfying four criteria: (1) little or no pain; (2) no complications; (3) ability to return home for at least 2 months; and (4) full mental capacity. RESULTS: Of the total patient population, 58% ultimately failed internal diversion. Nearly all (92%) of the cervical cancer patients required external drainage. Complications were seen in 10% of the stented patients and 13% of the patients with a percutaneous nephrostomy tube, but no procedure-related deaths occurred. Useful life was achieved by 84% of all patients. CONCLUSION: Antegrade drainage should be considered initially in patients who are likely to fail internal drainage (i.e., those with cervical cancer). The majority of these patients have a reasonably good quality of life, and intervention is most often warranted.

Adult↗

Diffuse malignant mesothelioma of pleura: diagnostic criteria based on an autopsy study.

Sixteen unequivocal, diffuse malignant mesotheliomas ( DMM ) of pleura and eight diffuse pleural tumors simulating DMM , all from autopsied cases, were studied with hematoxylin and eosin, mucicarmine, diastase-periodic acid-Schiff ( DPAS ), colloidal iron, carcinoembryonic antigen, and keratin immunoperoxidase stains. Collagen production by tumor cells when identifiable was diagnostic of DMM and was found not only in sarcomatous and "biphasic" mesotheliomas but also focally in "epithelial" variants. In "epithelial" areas, a constant nuclear/cytoplasmic (N/C) ratio with variable cell size often imparted a distinctive appearance of regularity, and mucin-negative DPAS -negative vacuoles or ground-glass cytoplasmic zones always could be found. Most metastatic carcinomas featured a high N/C ratio; some but not all had mucicarmine-positive or DPAS -positive vacuoles. Stains for carcinoembryonic antigen and keratin did not discriminate between DMM and metastatic carcinoma. Distant metastases were present in 12 of the 16 DMM cases but were clinically occult in 11 cases.

Aged↗

Inflammatory fibrous histiocytoma (? anthogranuloma). Report of two cases with ultrastructural observations in one.

Two cases of inflammatory fibrous histiocytoma are presented, one occupying the pleural space, and the other the retroperitoneum. One of the cases was studied with the electron microscope and confirmed the histiocytic and xanthomatous nature of the lesion. It is suggested that the term xanthogranuloma be replaced by fibrous histiocytoma, and that when a considerable inflammatory component is present in the stroma, a guarded prognosis be entertained. Ultrastructurally there are no distinctive features for the inflammatory variant of the fibrous histiocytoma.

Histiocytoma, Benign Fibrous↗

Inflammatory fibrosarcoma of the mesentery and retroperitoneum. A tumor closely simulating inflammatory pseudotumor.

We report 38 cases of inflammatory fibrosarcoma occurring in 23 females and 15 males, 2 months to 74 years of age (median, 8.5 years; mean, 15 years) with symptoms of abdominal pain (17 cases), anemia (21 cases), fever (14 cases), mass (16 cases), and gastrointestinal obstruction (7 cases). Primary tumor sites included mesentery and retroperitoneum (31 cases), omentum (two cases), mediastinum (two cases), liver (one case), diaphragm (one case), and abdominal wall (one case). Sizes ranged from 2.4 cm to 20 cm (mean, 9.6 cm). Follow-up data in 27 cases revealed local recurrences in 10 patients, with multiple local recurrences in three and histologically proven distant metastases to lung (two cases) and brain (one case). Five patients died from their disease (median, 20 months). All tumors, including metastases, consisted of fibroblasts, myofibroblasts, and plasma cells, with variable degrees of fibrosis and calcification. Immunostains indicate myofibroblastic differentiation; 18 of 20 (90%) stained for actin, 15 of 18 (83%) for vimentin, and 10 of 13 (77%) for keratin (primarily in a submesothelial location). Ultrastructural studies also disclosed myofibroblastic features. The locally aggressive, recurrent nature of these neoplasms, as well as the occurrence of metastases and tumor deaths, indicate that they are potentially malignant neoplasms that we believe are better classified as inflammatory fibrosarcomas, not as cellular inflammatory pseudotumors.

Adolescent↗

Retroperitoneal xanthogranuloma in a patient with Erdheim-Chester disease.

A case of Erdheim-Chester disease with retroperitoneal and renal sinus xanthogranuloma that occurred in a 50-year-old woman is presented. The 12 previously reported cases of Erdheim-Chester disease associated with retroperitoneal xanthogranuloma are reviewed and compared with 13 sporadic cases of retroperitoneal xanthogranuloma. Retroperitoneal xanthogranuloma is distinguished from inflammatory malignant fibrous histiocytoma by its lack of neutrophils, inconspicuous vascularity, lack of nuclear atypia, and abundant collagen. It is distinguished from inflammatory fibrosarcoma by its numerous foamy histiocytes, relative lack of plasma cells, and lack of nuclear atypia; it is distinguished from retroperitoneal fibrosis principally by its many foamy histiocytes, lack of plasma cells, and lack of vasculitis.

Bone Diseases↗

Lipomatous hemangiopericytoma. A histologic, ultrastructural and immunohistochemical study of a unique variant of hemangiopericytoma.

We report three cases of a unique, previously undescribed soft tissue tumor composed of mature adipocytes and hemangiopericytomatous areas, for which we propose the term lipomatous hemangiopericytoma. The tumors occurred in adults and were located in the sinonasal area, the soft tissue of the shoulder, and the retroperitoneum. The tumors ranged in size from 4 to 10 cm in greatest diameter and grossly were solid and ranged from tan to yellow. Histologically, they were composed of a variable admixture of benign lipomatous and hemangiopericytomatous components. Immunohistochemically, they stained with antibodies to vimentin and not to alpha-smooth-muscle actin, muscle-specific actin, desmin, S-100 protein, glial fibrillary acidic protein, epithelial membrane antigen, or keratin. Ultrastructurally, the cells constituting the hemangiopericytomatous areas had the features of pericytes, and no lipoblasts or transitional forms between lipocytes and pericytes were found. The histologic differential diagnosis of this neoplasm includes spindle-cell lipoma, angiolipoma, liposarcomas, tumors showing smooth muscle and adipocytic differentiation, and hemangiopericytoma infiltrating fat. Because of the small number of cases and the limited follow-up, we cannot be certain of their biologic behavior, although we expect that they are benign. Lipomatous hemangiopericytoma represents a distinctive pathologic entity that should be recognized and studied further.

Adult↗

Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant.

Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients. We analysed the clinicopathologic features in a series of 75 patients. All patients were adults (range, 22-91 years; median, 66 years) with an approximately equal incidence in men and women. Thirty-five tumors arose in the lower and 25 in the upper extremities, nine on the trunk, two each in the retroperitoneum and the head and neck region, and one each in the pelvis and penis. Forty-eight cases (69.5%) were located in dermal or subcutaneous tissues. Distinctive histologic features included the following: a commonly nodular growth pattern; a myxoid matrix containing elongated, curvilinear capillaries; and fusiform, round or stellate tumor cells with indistinct cell margins, slightly eosinophilic cytoplasm, and hyperchromatic atypical nuclei. These lesions varied from a hypocellular, mainly myxoid, and purely spindle-cell appearance (low-grade neoplasms) to high-grade, pleomorphic (malignant fibrous histiocytoma-like) lesions with multinucleated giant cells, high mitotic activity, and areas of necrosis. Immunohistochemistry in 44 cases revealed only vimentin and occasional actin positivity. Ultrastructurally, tumor cells had a fibroblastic phenotype. DNA flow cytometry and proliferation analysis showed an association between aneuploidy and histologic grade. An average follow-up of 45 months (range, 5-300 months) in 60 cases has revealed local recurrence in 33 cases (54%). Thirteen patients developed metastases, and 13 tumor-related deaths occurred. A short interval to first local recurrence was associated with poor clinical outcome. The rate of local recurrence was independent of histologic grade, but only intermediate and high-grade neoplasms metastasized. The depth of the primary lesion did not influence the incidence of local recurrence. However, in deep-seated neoplasms, the incidence of metastases was higher and the percentage of tumor-related deaths was twice as high as in superficially located lesions, reflecting the fact that deep-seated lesions tended to be higher-grade, larger tumors. Myxofibrosarcoma tends to become progressively higher grade in recurrences, as demonstrated in five cases in our series. The poorly recognized low-grade myxofibrosarcoma is emphasized, as proper diagnosis and treatment and scrupulous follow-up are mandatory to avoid local recurrence and gradual tumor progression to a higher-grade neoplasm that may then metastasize.

Adult↗

Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors.

Solitary fibrous tumor (SFT), first described as a pleural lesion, has been reported at numerous extrathoracic sites over the past 10 years. About 10% to 15% of intrathoracic SFTs are histologically or clinically malignant, but such cases have very rarely been described at other locations. Among 92 cases of extrathoracic SFT in our files, we identified 10 that either had recurred (2 cases) or had a least one atypical histologic feature (8 cases). The ten tumors occurred in five men and five women, 32 to 81 years old (median 56), measured 1.9 cm to 20 cm (median 11.5 cm), and were located in the abdomen/pelvis (4 cases), retroperitoneum (3 cases), groin, trunk, and upper arm. Nuclear atypia (8 cases), markedly increased cellularity (6 cases), areas of necrosis (4 cases), and greater than 4 mitoses/10 HPFs (3 cases) were seen in addition to the typical histologic features of SFT. Six tumors had at least two of these atypical histologic features. Nine cases were positive for CD34, six were positive for O-13, and one was focally positive for smooth muscle actin. Eight were excised completely. Subsequent follow-up revealed tumor relapse in eight cases (follow up 6-180 months, median 24). Four patients had local recurrence at 12 to 168 months. Distant metastasis developed at 1 to 6 years in five cases with spread to lung (2 cases), liver (4 cases), and bone. Metastasis or local recurrence developed within 2 years in five patients. To date, no patient has died of their tumor. These findings demonstrate that nuclear atypia, hypercellularity, greater than 4 mitoses/10 HPFs, and necrosis may be seen in up to 10% of extrathoracic SFTs, and are associated with, but are not by themselves predictive of, aggressive clinical behavior. In addition, our findings confirm that the behavior of extrathoracic SFTs is unpredictable, entirely comparable to that of their better known pleural counterparts, and confirm that patients with SFTs in all locations require careful, long-term follow up. It is probably unwise to regard any such lesion as definitely benign.

12E7 Antigen↗

Metastasizing splenic littoral cell hemangioendothelioma.

Littoral cell angioma is a unique splenic tumor that is generally considered to be benign. We present a case of a low-grade littoral cell splenic tumor that metastasized to the liver and retroperitoneum 4 years after splenectomy. Although the splenic lesion showed the typical morphology of a littoral cell angioma, it also contained areas with unusual solid nests of cytologically bland, plump cells with clear cytoplasm. The liver was diffusely infiltrated exclusively by cells with similar clear cell features. Both splenic and liver lesions demonstrated identical immunophenotypes, typical of littoral cell angioma, expressing CD31, CD68, CD21, and CD163, although negative for CD8 and CD34. A single prior description of a littoral cell hemangioendothelioma showed nuclear atypia and necrosis, and this is the first case report of a splenic littoral cell hemangioendothelioma with a completely bland histologic appearance. This case suggests that the presence of solid areas of clear cells in a littoral cell angioma may be a marker of low-grade malignant potential in these tumors.

Aged↗