Cases reported "Sarcoma"

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1/111. Granular cell traumatic neuroma: a lesion occurring in mastectomy scars.

    BACKGROUND: Granular cell changes can be observed in a variety of benign and malignant tumors, and are seen more commonly in granular cell tumors, which in about 5% of cases develop in the breast. Granular cells also have been observed in sites of previous trauma, such as surgery, and are found to be inflammatory reactions of histiocytic origin. methods AND RESULTS: We investigated, morphologically and immunohistochemically, 2 granular cell lesions occurring in mastectomy scars after surgery for carcinoma. Both lesions were composed of strands and nests of large granular cells, haphazardly set in a background of fibrous tissue, with sparse inflammatory infiltrates. Several tortuous hypertrophic nerve bundles were also embedded in the fibrous tissue. A few of these nerve bundles showed degenerative changes and contained granular cells. Immunohistochemically, granular cells were positive for S100 protein, neuron-specific enolase, vimentin, and CD68 antigen. CONCLUSIONS: We consider these proliferative lesions of peripheral nerves to have the features of both granular cell tumor and traumatic neuroma. These cases indicate that traumatic neuroma can undergo extensive granular cell changes and constitute a previously unrecognized entity, which we provisionally label granular cell traumatic neuroma. Granular cell traumatic neuroma has to be taken into consideration when evaluating lesions occurring at mastectomy scars and should be differentiated from malignant tumors with granular cells, such as apocrine carcinoma and alveolar soft part sarcoma.
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2/111. Gastrointestinal autonomic nerve sarcoma presenting as a giant intra-abdominal cyst.

    Gastrointestinal autonomic nerve sarcomas are rare tumours. A 36-year-old man presented with abdominal pain, distension and constipation. Abdominal ultrasound and CT scanning demonstrated a giant cystic intra-abdominal mass. laparotomy confirmed a large cystic mass arising from the ileum with multiple metastases. Immunohistochemical staining was positive for vimentin, neuronal-specific enolase and PGP9.5. This is the first reported case to present as a giant intra-abdominal cyst. Specialist histopathological and immunohistochemical analysis is essential to establish the diagnosis of this rare tumour.
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3/111. Plexosarcoma: endoscopic ultrasound and electron-microscopic characteristics of a stromal tumor.

    Endoscopic ultrasound is useful for managing submucosal masses; however, some of these lesions can be difficult to classify except with full histological and electron microscopic evaluation. A 72-yr-old woman was seen with upper GI bleeding. endoscopy showed a 1.7-cm sessile ulcerated submucosal mass in the duodenal bulb. Endoscopic ultrasound revealed an echolucent submucosal mass arising from the fourth echolayer, the muscularis propria of the duodenal wall. These findings suggested that the lesion was a leiomyoma. The patient eventually had the lesion resected because of recurrent bleeding. Histologically it was a spindle cell tumor that on electron microscopy showed neuronal elements consistent with a plexosarcoma, or gastrointestinal autonomic nerve tumor. These lesions account for some one third of all gastrointestinal stromal tumors. Despite their low grade malignant histologic appearance, local recurrence or hepatic metastases occur in about 70% of patients.
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4/111. Follicular dendritic cell tumor presenting in the lung: a case report.

    An example of extranodal follicular dendritic cell sarcoma (FDCS) presenting in the lung, a heretofore unreported site, is described. Macroscopically, a 9.5-cm, tan-white, dominant mass and multiple smaller parenchymal and pleural nodules were identified. Microscopically, the tumor was composed of spindled cells with uniform cytologic features arranged in short, intersecting fascicles and intermixed small lymphocytes and plasma cells. One of 4 peribronchial and hilar lymph nodes evaluated microscopically was focally involved by the process. Immunohistochemically, the neoplastic spindled cells expressed complement receptors CD21 and CD35 and low-affinity nerve growth factor receptor but did not express keratin (AE1/AE3 and CAM5.2), CD45 (leukocyte common antigen), CD20 (L26), S-100 protein, muscle-specific actin, or gp100 protein (HMB45). Ultrastructurally, the tumor cells have complex interdigitating cell surface processes and desmosomes. Epstein-Barr virus (EBV) was not detected in the tumor cells by in situ hybridization for EBV-encoded rna or by polymerase chain reaction for viral dna. FDCS should be considered in the differential diagnosis of any spindled-cell tumor with interspersed chronic inflammatory cells occurring in the lung. An immunohistochemical panel, including anti-CD21 and -CD35, can assist in its diagnosis, especially with small bronchial biopsy specimens. 2001 by W.B. Saunders Company.
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5/111. An unusual association between oral sarcoma and bulbar palsy.

    A 90 year old male presented with symptoms and signs of right lower cranial nerves palsy. A small mass was found on the right side at the back of his mouth. light microscopy and histoimmunochemical studies of the biopsy of the mass showed an alveolar soft part sarcoma arising from the right myohyoid muscle. The unusual location and presentation of this rare tumor is discussed.
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6/111. Orthopaedic implant-related sarcoma: a study of twelve cases.

    Sarcoma developing in association with a metallic orthopaedic prosthesis or hardware is an uncommon, but well recognized complication. We review 12 cases of sarcomas arising in bone or soft tissue at the site of orthopaedic hardware or a prosthetic joint. Nine patients were male, and three were female. Their ages ranged from 18 to 85 (mean 55) years at the time of diagnosis of the malignancy. Five patients had undergone hip arthroplasty for degenerative joint disease, four had been treated with intramedullary nail placement for fracture, two had staples placed for fixation of osteotomy, and one had hardware placed for fracture fixation followed years later by a hip arthroplasty. The time interval between the placement of hardware and diagnosis of sarcoma was known in 11 cases and ranged from 2.5 to 33 (mean 11) years. The patients presented with pain, swelling, or loosening of hardware and were found to have a destructive bone or soft tissue mass on radiography. Two sarcomas were located primarily in the soft tissue and 10 in bone. Seven patients developed osteosarcoma, four malignant fibrous histiocytoma, and one a malignant peripheral nerve sheath tumor. All sarcomas were high grade. Three patients had metastatic disease at the time of diagnosis. Follow-up was available on eight patients: five patients died of disease 2 months to 8 years (mean 26 months) after diagnosis; two patients died without evidence of disease 7 and 30 months after diagnosis; and one patient is alive and free of disease 8 years after diagnosis. Sarcomas that occur adjacent to orthopaedic prostheses or hardware are of varied types, but are usually osteosarcoma or malignant fibrous histiocytoma. They behave aggressively and frequently metastasize. Clinically, they should be distinguished from non-neoplastic reactions associated with implants, such as infection and a reaction to prosthetic wear debris.
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7/111. thymoma with pseudosarcomatous stroma.

    thymoma with psuedosarcomatous stroma is a recently described, rare variant of thymomas that are characterized by having a biphasic histologic pattern which consists of both an epithelial and a spindle cell stromal component. So far only 11 cases having similar histologic findings have been reported worldwide. At this time we report a case of this rare variant of thymoma which occurred in a 53-year-old Korean man. This previously healthy patient presented with coughing and an anterior mediastinal mass was then detected radiographically. Mediastinal exploration revealed a 9 x 8 x 8 cm-sized well- encapsulated, ovoid, cystic mass. Histological examination showed a biphasic neoplasm composed of anastomosing nests of epithelial cells and somewhat cellular stromal spindle cells that had advanced degenerative changes. Immunohistochemical staining using the antibodies for cytokeratins, EMA, e-cadherin, and p75NGFR showed a strong expression of these markers in the epithelial component but no expression in the spindle stromal cells. The epithelial tumor cells showed no reactivity to CD5 and L26 and a high proportion of the infiltrated lymphocytes were the cortical type that expressed CD99 and terminal deoxynucleotidyl transferase. Ultrastructural examinations revealed tonofilaments in the spindle cells. Follow up has been done for 5 years after the surgical excision and the patient has been free of disease during that period. Similar to previous reports, this patient had a benign clinical course that was unassociated with myasthenia gravis which appears to be a characteristic of this histologic variant of thymoma. However, our case also showed advanced degenerative features and we could demonstrate ultrastructural evidence of epithelial differentiation in the stromal spindle cells that were not mentioned in the previously reported cases. Based on the results of our studies, we suggest this entity is a distinct type of organotypic thymoma that shows cortical differentiation and abundant cellular stroma.
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8/111. Histological and immunohistochemical characterization of extranodal diffuse large-cell lymphomas with prominent spindle cell features.

    AIMS: To describe five cases of diffuse large-cell lymphoma with prominent spindle cell components involving skin, nasal-ocular mucosa, and soft tissue. Because of the spindle cell morphology, such cases must be differentiated from true sarcomas arising in or metastasizing to soft tissue, skin, bone, lymph node, or other organs and sites. methods AND RESULTS: Formalin-fixed paraffin-embedded archival tissue from five consultation cases of diffuse large-cell lymphoma with prominent spindle cell features involving the skin, nasal-ocular mucosa, and soft tissue in three male and two female patients was studied by histology and immunohistochemistry. Clinicopathological findings were also reviewed for all the patients. By morphology, initial evaluation of the cases suggested spindle cell sarcoma in two cases, inflammatory pseudotumour in one case, large-cell lymphoma in another case, and one case was considered suspicious for malignant lymphoma. immunohistochemistry demonstrated a B-cell lineage in four of the spindle cell lesions, with a diagnosis of primary cutaneous CD30 anaplastic large cell lymphoma made for the fifth case. Four of five cases also showed actin reactivity. CONCLUSIONS: Although extremely rare, lymphomas with prominent spindle cell morphology can be encountered in daily surgical pathology practice, and should be included in the differential diagnosis of spindle cell lesions in skin and soft tissue. The observed actin reactivity in four of the five spindle cell lymphomas may lead to a misdiagnosis of leiomyosarcoma if lymphoid markers are not included in the immunohistochemical panel.
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9/111. Polypoid sarcomas of the esophagus. A rare but potentially curable neoplasm.

    Five patients with polypoid esophageal sarcoma are reported. All had dysphagia similar to that occurring in epithelial carcinoma of the esophagus. The clinical diagnosis of sarcoma was suspected when barium swallow showed a large polypoid lesion. biopsy was often inconclusive concerning the nature of the lesion except to identify it as a neoplastic process. In spite of their large size, the tumors remained superficial within the esophageal wall. Nodal or distant organ metastasis was absent in 4 of the 5 patients. The histology of these tumors suggests that so-called carcinosarcoma is an epithelial carcinoma of the esophagus with varying amounts of spindle cell features and should be considered separately from sarcoma arising from mesenchymal tissue. Unlike carcinoma, esophageal sarcoma has a favorable prognosis following radical resection, and recent advances in esophageal surgery have decreased the mortality and morbidity of esophagectomy.
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10/111. Primary epithelioid sarcoma of the dura: case report.

    OBJECTIVE AND IMPORTANCE: Epithelioid sarcomas are rare mesenchymal neoplasms that occur most often in the extremities of young adults. Despite isolated reports of epithelioid sarcomas arising in the head and neck region, these lesions have not been described previously, to our knowledge, in the central nervous system. CLINICAL PRESENTATION: We present the case of an 18-year-old woman with a unique dural sarcoma that arose in the right frontotemporal region. As visualized on magnetic resonance imaging studies, the 4.5-cm tumor focally traversed the cranium to penetrate the galea, the temporal muscle, and subcutaneous tissue. No brain invasion was noted. INTERVENTION: Despite gross total removal and postoperative radiotherapy (59 Gy), a large recurrence was noted 5 months after surgery. Histologically, the partly necrotic tumor consisted of epithelioid and spindle cells showing widespread vimentin and variable cytokeratin as well as epithelial membrane antigen immunoreactivity. Ultrastructurally, the cohesive cells featured various organelles, intermediate filaments, junctions, and filopodia-containing intercellular spaces. CONCLUSION: With the inclusion of epithelioid sarcoma, the spectrum of central nervous system sarcomas continues to expand.
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