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In this report we describe a primary giant cell tumor (GCT) of soft tissues located in the left dorsal wrist of a 52-year-old man. Plain radiographs did not reveal any lesion in his carpal or hand bones. Although the tumor was clinically considered a ganglion initially, the microscopic features were identical to those found in classic GCT of bone. Light microscopy showed a lesion composed of a homogeneously mixed proliferation of spindle and polygonal mononucleated stromal cells and evenly distributed multinucleated, osteoclast-like giant cells. Whereas most bone tumors have an extraosseous counterpart, only 13 cases of GCT in soft tissues had been published until 1998. Moreover, 64 new cases have been reported in three series. Nevertheless, most major reviews and textbooks do not consider this tumor as a specific entity and regard it as a low grade variant of malignant GCT of soft tissue. We describe the clinical, histologic, and immunohistochemical features of this rare benign neoplasm emphasizing the differential diagnosis with its malignant soft tissue counterpart, an ominous tumor.  相似文献   

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Tenosynovial giant cell tumors are a group of generally benign intra-articular and soft tissue tumors with common histologic features. They can be roughly divided into localized and diffuse types. Localized types include giant cell tumors of tendon sheath and localized pigmented villonodular synovitis, whereas diffuse types encompass conventional pigmented villonodular synovitis and diffuse-type giant cell tumor. Localized tumors are generally indolent, whereas diffuse tumors are locally aggressive. Recent developments indicate that tenosynovial giant cell tumors are clonal neoplastic tumors driven by overexpression of CSF1. Herein, I report a case of intra-articular, localized tenosynovial giant cell tumor (or localized pigmented villonodular synovitis) and review the classification, histopathology, and recent developments regarding its pathogenesis.  相似文献   

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Idiopathic orbital inflammatory pseudotumor is a chronic neoplasm-like inflammatory reaction, usually affecting the orbital tissues of both eyes and orbit. Isolated optic nerve involvement by the idiopathic inflammatory process is a rare finding and very few reports are available. Here a case of an isolated orbital nerve inflammatory pseudotumor presenting with gradually progressive unilateral loss of vision is described. It showed dramatic response to surgery and steroid treatment and its differential diagnoses are discussed.  相似文献   

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Renal cell carcinoma with syncytial giant cells (SGCs) is rare; to our knowledge, there are only 3 cases reported in the English literature. We report on a case that, unlike previous reports, was associated with higher grade renal cell carcinoma and an aggressive behavior. A 63-year-old man with gross hematuria was found to have a large left renal mass and no evidence of metastasis. The nephrectomy specimen contained a 14.8-cm mass that was confined within the renal capsule. Microscopically, a 1.8-cm area of SGCs was noted in a background of clear cell renal cell carcinoma of Fuhrman nuclear grades 2 to 4. The SGCs had voluminous cytoplasm, multiple large nuclei with prominent nucleoli, and focal emperipolesis. Identical to the background mononuclear tumor cells, the SGCs were positive for AE1/AE3, vimentin, and epithelial membrane antigen and negative for CD68, beta-human chorionic gonadotropin, and mucin. Three months after surgery, the patient developed metastases in the liver, right kidney, and lungs.  相似文献   

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Giant cell reparative granuloma (GCRG) is a non-neoplastic hyperplasia of bones that mostly happens in the mandible and maxilla in any age group but has a predilection for children and young adults. GCRGs that cause bone destruction are of very low frequency. Orbital-involved cases have been rarely reported since 1981, especially in children. We now report a 1-year-old girl with a rapidly enlarging post-traumatic orbital mass. CT scan and surgical resection showed a well-defined mass occupying the upper right orbit, causing bone destruction. Microscopically there was a proliferation of histocytes and some osteoclast-like multinucleated giant cells with hemosiderin, finally confirmed to be GCRG. 22 months’ follow up showed no evidence of recurrence. This case suggests infant orbital GCRG can be locally aggressive.  相似文献   

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Petrification of the auricle results in a rigid and immalleable ear. The etiology of such a finding is usually ectopic calcification. The condition has been associated with injurious processes, such as cold injury, and with various endocrinopathies, including Addison disease. In a significant number of cases, ossification occurs without knowledge of the precipitating cause or event. True auricular ossification is a rare occurrence, with only 12 histologically confirmed cases in the literature. We herein present the clinical and pathologic findings of another case. A 60-year-old man with diet-controlled diabetes presented with a 10-year history of slowly and insidiously stiffened auricles. He denied any precipitating historical events. Routine testing did not demonstrate systemic abnormalities. Radiographic examination revealed opacities consistent with bony structure in the auricles of the ears, with the right more prominent than the left. Histologic sampling demonstrated ossification with deposition of trabecular bone in proximity to normal elastic cartilage.  相似文献   

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Schwannomas are usually benign tumors that arise from well-differentiated Schwann cells. They rarely occur in the retroperitoneum. Here, we present a case of a 60-year-old man with a giant retroperitoneal pelvic mass. Imageological diagnosis suggested a large heterogeneous mass of 16 cm in diameter located in the abdominopelvic retroperitoneum. Complete intralesional enucleation was achieved without any adjacent organs injury except a severe bleeding which was ceased as we applied the bilateral inferior vesical artery embolization. Final histopathological result showed the tumor was a low malignant Schwannoma. The patient’s symptoms were greatly improved after operation. Unfortunately, a local recurrence was detected at the six-month follow-up appointment with consequent losing to follow up.  相似文献   

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We present a case of metastatic malignant melanoma in a patient initially diagnosed with glioblastoma multiforme, giant cell variant. A forty year old female presented to our institution for a re-resection of a recurrent right parietal lobe mass, presumed to be recurrent glioblastoma multiforme. PET scan during preoperative evaluation revealed a 3 cm left lower lobe lung mass. Metastatic glioblastoma to lung was considered in the differential diagnosis. Resection of the brain mass revealed a highly pleomorphic giant and spindle cell lesion with an immunophenotype strongly supportive of melanoma. Immunostains for melanocytic markers were subsequently performed on the lung biopsy specimen, and demonstrated diffuse staining of the atypical cells, supporting the diagnosis of malignant melanoma in the lung. This case demonstrates the importance of considering melanoma in the differential in any tumor with high grade features.  相似文献   

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Liposarcoma was mainly occurring in the lower extremities and deep retroperitoneal soft tissues, but rarely occurred in the thoracic cavity. Most cases were reported in the literature, and most of them were middle-aged and elderly people. It was even more rare in adolescents with tumors occupying the entire thoracic cavity. Recently, one case of myxomatous liposarcoma was admitted and treated in our hospital. This paper reports this case and discusses the diagnostic method, pathological type and treatment of giant liposarcoma in the chest, in order to provide a reference for the clinical diagnosis and treatment of this rare tumor.  相似文献   

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The localized form of tenosynovial giant cell tumor or pigmented villonodular synovitis is rarely intraarticular in the knee. We reported a 40-year-old woman with a tenosynovial giant cell tumor arising from posterior cruciate ligament (PCL). She suffered sudden knee pain and locking without any reason for two days. A mass with a size of 1.7×0.8×0.7 cm in the fossa intercondyloidea was detected on the MRI. After one time hyperextension physical examination the patients felt sudden pain relief. During the arthroscopy examination, a loose soft tissue mass was found under the lateral meniscus. Only the synovium tissue lesion on the proximal PCL was detected. The mass had a conceivable thin pedicel and the shape matched well with the tumor bed on the PCL. The histopathology of the mass demonstrated a tenosynovial giant cell tumor. At six weeks follow-up, no clinical evidence of recurrence was noted. A Literature Review of tenosynovial giant cell tumor or pigmented villonodular synovitis arising from the PCL is present.  相似文献   

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Breast carcinoma with osteoclastic giant cells (OGCs) are uncommon. Here, we report a 46-year-old woman with a painless lump in her left breast that has been proved clinically and radiographically. Microscopical examination showed OGCs accompanying invasive ductal carcinoma. Immunohistochemical assay revealed that OGCs derived from macrophages. Despite positive lymph node metastasis, the patient has been well without evidence of recurrence or metastasis one year after the operation. To date, the influence of OGCs on the prognosis of patients is still controversial. Our case may provide insights into further understanding beast carcinoma with OGCs.  相似文献   

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We report a case of idiopathic portal hypertension associated with prominent polyclonal hyperimmunoglobulinemia and plasmacytosis in the bone marrow and the spleen. Microscopic examination of the liver showed chronic inflammation in the portal area and abnormal vasculature adjacent to the portal tracts. In the spleen, polyclonal plasma cell proliferation was demonstrated histochemically in addition to the presence of remarkable sinus hyperplasia and periarterial fibrosis. In the present case, a chronic inflammatory state such as connective tissue disease was strongly suspected to exist in the liver, since pancytopenia and hyperimmunoglobulinemia persisted even after splenectomy. It is suggested that the pathogenesis in the present case may have been due to this chronic inflammatory state.  相似文献   

17.
Noonan syndrome with multiple giant cell lesions (NS/MGCL) was recently shown to be a phenotypic variation within the syndromes of the Ras/MAPK pathway and not an independent entity as previously thought. Here we report on a 13-year-old boy with a typical phenotype of NS including atrial septal defect, pulmonic stenosis, short stature, and combined pectus carinatum/excavatum, pronounced MGCL of both jaws, and a de novo mutation in PTPN11, c.236A>G (which predicts p.Q79R). Mutations in PTPN11 are the most frequent cause of NS and p.Q79R is a recurrent mutation in exon 3. Including this patient, 24 patients with molecularly confirmed NS, LEOPARD, or CFC/MGCL syndrome have been reported to date, of these 21 patients have PTPN11, SOS1, or RAF1 mutations and three have BRAF or MAP2K1 mutations, confirming that MGCL is a rare complication of the deregulated RAS/MAPK pathway. In all patients, the lesions of the mandible and to a lesser extent of the maxilla were first noted between ages 2 and 19 years (median 11 years), and were combined with enlargement of the jaws in 11/24 patients (46%). In this case and, with one exception (mutation not reported), all previous cases the NS/MGCL was caused by known mutations in the PTPN11, SOS1, RAF1, BRAF1, and MAP2K1 genes that were previously reported with RASopathies without MGCL. ? 2012 Wiley Periodicals, Inc.  相似文献   

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Giant cell arteritis of the breast: case report and literature review   总被引:1,自引:0,他引:1  
P B Clement  H Senges  A R How 《Human pathology》1987,18(11):1186-1189
The clinical and pathologic features of a case of giant cell arteritis presenting in the breast and those of seven previously reported similar cases are described. A unique finding in the present case was a coincidental in situ and infiltrating ductal carcinoma in the same biopsy specimen. All the patients were postmenopausal women who presented with a firm mass in one or both breasts that mimicked a carcinoma on physical examination. There was no definite clinical evidence of temporal artery involvement in any patient, and two patients had normal temporal arteries on histologic examination. Five patients, however, had systemic manifestations, arteritis in another site, or both. Giant cell arteritis presenting in the breast may occur as an isolated finding or represent a manifestation of more widespread disease.  相似文献   

20.
Nati S  Monico S  Gentili A 《Pathologica》1998,90(6):798-800
We describe a case of multiple granular cell tumour (GCT) in 41 years old man. It manifested as subcutaneous synchronous multiple lesions. GCT is, usually, a benign lesion that is histologically diagnosed after biopsy or surgical excision. It raises doubts about his benignity in relation to multiple and distant localizations as in the present case.  相似文献   

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