Multiple cutaneous and uterine leiomyomas associated with gastric GIST.

BACKGROUND: There are a number of reports documenting familial cases of leiomyomatosis cutis associated with uterine leiomyomata. However, to our knowledge, the association of gastrointestinal stromal tumour (GIST) with this entity has not as yet been reported. We report an interesting case of cutaneous leiomyomatosis, metachronous uterine leiomyomata, and a gastric GIST in a 43-year-old woman. OBSERVATION: The patient had previously undergone two separate uterine myomectomies at ages 25 and 26 years, respectively, followed by a hysterectomy at 27 years. At 36 years she underwent partial gastrectomy for excision of GIST and this was followed by the development of extensive, symptomatic cutaneous leiomyomata at 43 years. In the report, we have documented histological, immunohistochemical and clinical observations and furthermore report on the therapeutic measures undertaken. CONCLUSION: We report an interesting association of cutaneous leiomyomatosis, uterine leiomyomas and GIST.

Localized and adaptive synoviocyte proliferation characteristics in rat knee joint contractures secondary to immobility.

OBJECTIVE: To investigate the proliferative activity of synoviocytes in joint contracture. DESIGN: Experimental controlled trial. SETTING: Laboratory in vivo study. ANIMALS: Adult male Sprague-Dawley rats (avg weight, 340g). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: We immobilized the knee joints of 24 rats, in 135 degrees of flexion, for up to 32 weeks. Controls were 24 sham-operated and 5 unoperated rats. On sagittal sections, synoviocytes that stained with a proliferating cell nuclear antigen antibody were counted over the anterior and posterior synovial intima. The length of the synovial intima was also measured. RESULTS: The absolute number of proliferating synoviocytes decreased markedly in the posterior capsule of knee joints immobilized for more than 2 weeks (2.4+/-1.0 vs 22.7+/-7.1 at week 16, P<.05), and so did the synovial intima length (1.4+/-0.1mm vs 8.6+/-0.5mm at week 16, P<.05). No change occurred anteriorly. CONCLUSION: A decreased number of proliferating synoviocytes and increased intima adhesion in the posterior capsule characterized joint contractures. The data further suggest that the synovial intima adapted to the new position of the joint. Phenomena of mechanotransduction could explain the fact that adaptations were restricted to the posterior synovial intima.

Isolated pleural PTLD after cardiac transplantation.

UNLABELLED: PREAMBLE: Epstein-Barr virus infection (EBV) and immunosuppression promote emergence of posttransplant lymphoproliferative disorders (PTLD) in patients undergoing organ transplantation. OBJECTIVE: We report a case of PTLD confined to the pleura. FINDINGS: The patient was a 62-year-old male who had undergone cardiac transplant in 1993 for ischemic heart disease. Seven years later, he presented with dyspnea and bilateral pleural effusions. The CT scan revealed left sided pleural base thickening. The cytology of the pleural fluid and fine needle aspirate of the pleura was both suggestive of PTLD. However, the tissue submitted for ancillary studies did not contain the diagnostic material. A clinical decision was made to withdraw immunosuppressive therapy and start rituximab. His clinical course was complicated by Pneumocystis carinii pneumonia and he died 4 months after the diagnosis of PTLD. Autopsy revealed bilateral pleural effusions with pleural nodules involving the visceral and parietal pleura of both lungs. Immunohistochemistry demonstrated B cell lineage with kappa/lambda ratio of 1. PCR studies done on the pleural nodules (postmortem specimen) revealed the presence of EBV DNA and absence of human herpes virus 8 (HHV8) DNA. In situ hybridization revealed positive staining for EBV RNA within the neoplasm. CONCLUSION: Pleural-based PTLD is rare. Cytology in conjunction with immunophenotyping and molecular studies can be useful for a definitive diagnosis. In our case, cytology sample was suggestive of PTLD. PCR studies performed on the antemortem specimen confirmed the presence of monoclonal IgH gene rearrangement, while the postmortem specimen revealed oligoclonal IgH gene rearrangement. The change from monoclonal to oligoclonal IgH gene rearrangement suggests reversion of monoclonal to polyclonal PTLD following rituximab and CHOP therapy. We also demonstrated EBV DNA and RNA in the tumor nodules, supporting EBV-induced PTLD.

Aneurysmal bone cyst associated with fibrous dysplasia.

We report the clinicopathologic features of a 22-year-old patient with aneurysmal bone cyst and fibrous dysplasia of the orbit. The patient was evaluated clinically with computed tomography of the orbit before surgery. An orbital biopsy specimen was examined histologically with conventional light microscopy. The lesion was treated with combined neurosurgical and orbital intervention. Clinical evaluation revealed axial and inferior displacement of the globe. Computed tomography revealed a cystic mass in the superotemporal left orbit with adjacent bone erosion. "Ground-glass" thickening of the adjacent frontal bone and sphenoid bone was observed. Microscopic examination showed fibrous stroma with giant cells and hemosiderin-laden macrophages with adjacent trabeculae of woven bone and osteoblast cells. A fronto-orbital craniotomy was performed, the cystic cavity was excised, and the hyperostotic bone was debulked. After treatment, the globe position and patient appearance have improved. There has been no sign of recurrence of the aneurysmal bone cyst. Aneurysmal bone cyst should be considered in patients with fibrous dysplasia that has a cystic component, or in patients with fibrous dysplasia who present with sudden expansion of their lesion.