Eliane Maria Ingrid Amstalden

Sonographically guided core needle biopsy of bone and soft tissue tumors.

To determine the value of sonographically guided core needle biopsies of musculoskeletal tumors as a reliable alternative to fluoroscopy and computed tomography.

HLA-G Expression in the Skin of Patients with Systemic Sclerosis

Objective. To determine HLA-G expression in skin biopsies from patients with systemic sclerosis (SSc), and its association with epidemiological, clinical, and laboratory variables and survival. Methods. Paraffin-embedded skin biopsies obtained from 21 SSc patients (14 limited SSc, 7 diffuse SSc) and from 28 healthy controls were studied. HLA-G expression was evaluated by immunohistochemistry. Results. HLA-G molecules were detected in 57% of skin biopsies from patients with SSc (9 from limited SSc, 3 from diffuse SSc), whereas no control sample expressed HLA-G (p = 0.000004). In patients, HLA-G molecules were consistently observed within epidermal and some dermal cells. HLA-G expression was associated with a lower frequency of vascular cutaneous ulcers (p = 0.0004), telangiectasias (p = 0.008), and inflammatory polyarthralgia (p = 0.02). After a 15-year followup, SSc patients who exhibited HLA-G survived longer than patients who did not. Conclusion. HLA-G is expressed in skin biopsies from patients with SSc, and this is associated with a better disease prognosis. This suggests a modulatory role of HLA-G in SSc, as observed in other skin disorders.

Sporothrix schenckii infection presented as monoarthritis: report of two cases and review of the literature

Septic arthritis demands early diagnosis and correct treatment if the function of the joint is to be restored. Sometimes, as in fungal infection, signs and symptoms may be mild and the diagnosis delayed. Nevertheless, the outcome of fungal arthritis is severe and usually causes joint disability. The authors report two patients with chronic monoarthritis due to Sporothrix schenckii infection diagnosed by synovial fluid cultures. Their clinical presentation, laboratory and image findings, and their treatment and follow-up are analyzed and compared to previously reported cases. These cases illustrate the differential diagnosis of monoarthritis in immunocompetent adults and picture clinical features that could lead to early diagnosis and proper treatment.

Primary diffuse large B-cell lymphoma of bone displays preferential rearrangements of the c-MYC or BCL2 gene

We selected a series of 63 primary diffuse large B-cell lymphomas (DLBCLs) of bone collected in tissue microarrays from centers in France and Brazil. These cases were classified according to the expression of antigens associated with germinal center (GC; n = 42) or non-GC (n = 21) stages of B-cell differentiation. By fluorescence in situ hybridization, we found a substantial number of cases with a rearrangement of BCL2 (9/32) and c -MYC (n = 3), whereas the PAX5, BCL6, BCL1 cyclin D1, and ALK genes were in germline configuration. It is interesting that 1 case, with a GC phenotype, showed dual BCL2 and c -MYC rearrangement. The majority of the cases with rearrangements were of the GC phenotype. These results, associated with the lack of BCL6 rearrangement, suggest that bone DLBCL represents a specific group within extranodal B-cell lymphomas.

Congenital generalized fibromatosis causing spinal cord compression

An unusual case of congenital generalized fibromatosis in which involvement of the spinal dura mater was accompanied by flaccid paralysis in the lower limbs is presented.

Desmoid tumor in patients with familial adenomatous polyposis

CONTEXT Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.

Osteosarcoma in dogs: clinical-morphological study and prognostic correlation

Osteosarcoma is defined as a bone matrix-producing malignant mesenchymal tumor. It is relatively rare among domestic animals, but corresponds to 85% of all malignant bone tumors in dogs. To study canine osteosarcoma in animals living in Brazil and correlate the clinical- morphological findings with prognosis, forty-six cases of dogs with osteosarcoma were studied retrospectively out of a total of 56 malignant primary bone tumors on file. The animals were assessed in terms of age, sex, breed, topographic tumor distribution, and presence of metastases at diagnosis. The tumors were classified histologically by the method of Pool 1 . Osteosarcomas corresponded to 82% of primary malignant bone dysplasias in the sample studied. Age ranged from 6 months to 14 years (C = 8 y), sex: 32 F/18 M; breed: large dogs (86%), topographic location: appendicular skeleton (64%), axial skeleton (28.5%) and extraskeletal (7.5%); presence of metastases in 17.4%. Histopathology: osteoblastic (32.6%), chondroblastic (9.6%) and telangiectatic (5.8%) patterns; combined: 52%. Osteosarcoma mainly affects adult females. Breed is a predisposing factor, the appendicular skeleton is most often involved, and an osteoblastic histological pattern predominates. The lungs are a common site of metastasis, with pure- bred male dogs being most predisposed regardless of the morphological pattern of osteosarcoma.

Chondromatous Hamartoma of the Chest Wall: Description of 3 New Cases and Literature Review

Chondromatous hamartoma of the chest wall is an extremely rare, benign lesion that usually occurs in early infancy. It typically arises in the rib cage and produces a large mass. It is composed of a varying admixture of hyaline cartilage that has features resembling growth plate cartilage, fascicles of spindle cells, woven bone, and hemorrhagic cysts. Treatment consists mainly of surgical excision, which is usually curative. We present 3 new cases, which demonstrated interesting findings, including multicentricity, involvement of the sternum, and local recurrence, and we discuss these findings in the context of a literature review.

Magnetic resonance imaging of low-grade fibromyxoid sarcoma

. We present theMR imaging features of a surgically confirmed case ofLGFMS affecting the shoulder.A 30-year-old man presented with a 20-year history ofa painless slow-growing mass in the right shoulder. Mag-netic resonance images were obtained on a 2.0T scanner(Elscint, Haifa, Israel), demonstrating a well-defined soft-tissue mass measuring 12.0 x 7.0 x 9.0 cm located betweenthe deltoid muscle, rotator-cuff muscles, and proximal hu-merus. No peri-tumoral edema in adjacent subcutaneous tis-sues or muscles was noted. The signal intensity (SI) of thebone marrow was normal. The mass had an intermediate,heterogeneous SI on T1-weighted images [repetition time(TR) / time to echo (TE), 600/25] (Fig. 1a). Heterogene-ous low to high SI with multiple hypointense intralesionalnodules was seen on T2-weighted fast spin-echo images(TR/TE, 3500/90) (Fig. 1b). The mass enhanced heteroge-neously on T1-weighted fat-suppressed images (TR/TE,720/20) after intravenous injection of gadolinium (Fig. 1c),predominantly in corresponding T2-weighted hyperintenseareas.The lesion was surgically removed with negative mar-gins. The surgical specimen consisted of a soft-tissue tumor,measuring 14.5 x 9.5 x 5.0 cm and weighing 290 g. Thecut sections showed a firm, grossly circumscribed, andlobulated mass, with a yellow-white color and glisteningappearance secondary to the accumulation of myxoidground substance (Fig. 2a). Microscopically, the tumor hadlow to moderate cellularity composed of bland spindle-shaped cells with a fibroblast pattern, arranged mainly in

Analysis of angiogenic factors and cyclooxygenase-2 expression in cartilaginous tumors: clinical and histological correlation

OBJECTIVES: To study the role of angiogenesis and cyclooxygenase-2 expression in cartilaginous tumors and correlate these factors with prognosis. INTRODUCTION: For chondrosarcoma, the histological grade is the current standard for predicting tumor outcome. However, a low-grade chondrosarcoma can follow an aggressive course—as monitored by sequential imaging techniques—even when it is histologically indistinguishable from an enchondroma. Therefore, additional tools are needed to help identify the biological potential of these tumors. The degree of angiogenesis that is induced by the tumor could assist in this task. Angiogenesis can be quantified by measuring the expression of vascular endothelial growth factor and CD34, and cyclooxygenase-2 can induce angiogenesis by stimulating the production of pro-angiogenic factors. METHODS: In total, 21 enchondromas and 58 conventional chondrosarcomas were studied by examining the clinical and histopathological findings in conjunction with the immunostaining markers of angiogenesis and cyclooxygenase-2 expression. RESULTS: The significant variables that were associated with poor outcome were 1) higher-grade chondrosarcomas, 2) tumors that developed in flat bones, and 3) over-expression of CD34 (with a median count that was higher than 5.9 vessels in 5 high power fields). Moreover, CD34 expression (measured using the Chalkley method) revealed significantly higher microvessel density in flat bone chondrosarcomas. DISCUSSION: Previous studies have shown a positive correlation between Chalkley microvessel density and histological grade; however, in our sample, we found that the former is predictive of the outcome. Chondrosarcomas in flat bones have been shown to correlate with a poor prognosis. We also found that CD34 microvessel density values were significantly higher in flat-bone chondrosarcomas. This could explain—at least in part—the more aggressive biological course that is taken by these tumors. CONCLUSIONS: These results provide evidence that CD34 microvessel density in chondrosarcomas can be helpful in predicting patient outcome and may add to our understanding of chondrosarcoma pathogenesis.