Cases reported "Mesenchymoma"

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1/14. Phosphaturic mesenchymal tumor, mixed connective tissue variant (oncogenic osteomalacia).

    A case of tumor-induced phosphaturic osteomalacia in a 54 year old man is reported. The patient was admitted because of progressive muscle spasms with pain and weakness in the bilateral thighs. Laboratory data showed hypophosphatemia, decreased tubular resorption of phosphate (TRP), a low 1,25-dihydroxyvitamin D level, and a high serum alkaline phosphatase level. Radiologic examinations revealed multiple lesions of osteomalacia in the ribs, and a small mass in the lower posterior mediastinum. After removal of the tumor, clinical symptoms disappeared and hypophosphatemia, decreased TRP, and the 1,25-dihydroxyvitamin D level were corrected. Microscopical examination revealed that the tumor was composed of mature adipose tissues, osseous tissues, and primitive stromal zones including osteoclast-like giant cells, non-mineralized woven bone, and various sized blood vessels. Patho-physiologic observations suggested that the tumor secreted some humoral substances inhibiting 25-hydroxyvitamin D-1 alpha-hydroxylase activity, renal phosphate resorption, and parathyroid hormone production.
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2/14. radiation-induced chorioretinal degeneration: a clinicopathological report of three cases.

    BACKGROUND: With the shift in radiotherapy toward the posterior segment and with the use of lead screens to shield the anterior segment, posterior segment lesions have become more readily recognized. The purpose of this study is to highlight the effects of ionizing radiation on the choroid and to demonstrate how this can result in visual loss, particularly if the macula is involved. methods: Histopathological study of three enucleated eyes of three patients who had received ionizing radiation: a 27-year-old woman who had received radiation as a child for a hemangioma of the left side of the face, a 16-year-old girl who had received radiation at age 11 years for a malignant mesenchymoma of the right maxilla, and a 4-year-old girl who had received radiation at age 1 year for a retinoblastoma of the right eye. RESULTS: Histopathological examination of the three globes showed extensive chorioretinal degeneration, among other ocular findings. In all cases the fellow eye did not show similar chorioretinal lesions. As all three patients were relatively young, the degree of chorioretinal degeneration was considered to be secondary to radiation treatment. INTERPRETATION: Vascular damage from ionizing radiation is not limited to the retina. It can also affect the choroid in the form of chorioretinal degeneration. Since most of the intraocular circulation arises from the uveal vessels, chorioretinal degenerative lesions may be extensive and may even involve the macula.
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3/14. Extraluminal gastrointestinal stromal tumour in the second portion of the duodenum.

    The preoperative diagnosis of extraluminal gastrointestinal stromal tumours in the duodenum is difficult to establish due to their rare occurrence and the lack of pathognomonic signs. This report describes the case of a 61-year-old woman who suffered from an immunohistologically confirmed gastrointestinal stromal tumour in the second portion of the duodenum. Preoperative, abdominal, multislice computed tomography showed an extraluminal but intramural tumour located between the head of the pancreas and the duodenum. Rapid postprocessing analysis by three-dimensional, volume-rendered images revealed a strong arterial blood supply and an early draining vessel into the superior mesenteric vein during the portal-venous phase. The combination of endoscopic ultrasonography and non-invasive multislice computed tomography provided an early suggestion of gastrointestinal stromal tumour.
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4/14. August 2003: 47-year-old female with a 7-year history of osteomalacia and hypophosphatemia.

    The August 2003 COM. A 47-year-old woman presented with a long history of muscle pain, weakness, and visual disturbances. Over the last year, she developed diplopia and left sixth nerve palsy. No other neuro-ophthalmologic abnormalities were found. Past medical and family history was unremarkable. Laboratory investigation disclosed hypophosphatemia, phosphaturia, elevated serum alkaline phosphatase activity, and normal serum calcium levels. CT scans showed a lobulated mass arising on the meningeal surface of the cavernous sinus, measuring 3x 2 x 2 cm. The lesion was partially resected and microscopic examination revealed a highly vascularized tumor composed of primitive mesenchymal cells arranged whether in a patternless-pattern or surrounding thin-walled, branching vascular spaces and thick-walled hyalinized vessels. Other eye-catching features were microcystic areas, multinucleated osteoclastic-like giant cells, scattered islands of mature adipocytes, foci of hemorrhage, thrombosed medium-sized-to-large vessels, and hemosiderin-laden macrophages. After surgery, the patient recovered from the muscle pain and weakness, with a significant improvement of serum calcium and alkaline phosphatase levels and phosphate blood levels. Taken together, the clinical and pathologic features were consistent with a diagnosis of phosphaturic mesenchymal tumor (mixed connective tissue variant) of the cavernous sinus in a setting of oncogenic osteomalacia. No further treatment was carried out. The patient has been followed for the last 4 years with no evidence of recurrent disease. Oncogenic osteomalacia is a rare clinical entity characterized by hypophosphatemia, phosphaturia, and a low concentration of 1,25-dihydroxyvitamin D(3) caused by a neoplasm. Pathologists should be aware of this entity, because surgical excision of the tumor is usually curative.
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5/14. Benign mesenchymoma of the round ligament. A report of two cases with immunohistochemistry.

    Benign mesenchymomas are tumors composed of an admixture of two or more mature mesenchymal tissue types. Clinically and histologically they are benign lesions. However, they are seldom encapsulated and therefore may recur locally if incompletely excised. The authors report on two benign mesenchymomas composed of mature fat, thin-walled small and medium size vessels, and spindle cells demonstrated to be smooth muscle by immunohistochemical studies. They were diagnosed in women in their 50s. Both were located in the round ligament of the uterus. One was discovered incidentally during hysterectomy, and the other was unusually large, being diagnosed preoperatively as a retroperitoneal liposarcoma. Most round ligament tumors are leiomyomas or fibromas. To the authors' knowledge, this is the first report of benign mesenchymomas occurring in this location.
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6/14. Clinicopathologic features of two rare cases of mesenchymal metastatic tumors in the pancreas: review of the literature.

    OBJECTIVES: A clinicopathologic presentation of 2 unusual cases of metastatic mesenchymal neoplasms in the pancreas. methods: The first case concerns a 26-year-old man with a history of intracranial mesenchymal chondrosarcoma (since the age of 17), 2 left lung operations, and 3 right thigh operations. Distal pancreatectomy and splenectomy was performed because of suspicious mass in the pancreas. The second case concerns a 66-year-old woman with a history of uterus leiomyosarcoma (10 years ago) with left axillary and right femoral metastases. She underwent distal pancreatectomy and splenectomy because of suspicious mass measuring 4 x 4 cm, in the pancreatic body. RESULTS: In the first case, the pathological examination revealed a tumor measuring 3.8 x 3.5 cm and histologically compatible with mesenchymal chondrosarcoma, developing in a vessel lumen and invading into the pancreatic parenchyma. In the second case, the pathological examination showed metastatic leiomyosarcoma of high-grade malignancy. The incidence of metastatic pancreatic tumors has been reported to be only 1.6% to 3%. Most of these tumors were of epithelial origin, and the most common sites of the primary lesions were the lung, kidney, and gastrointestinal tract. CONCLUSIONS: The cases of metastatic uterus leiomyosarcoma and the metastatic intracranial chondrosarcoma are, to our knowledge, the first to be described.
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7/14. Benign mesenchymoma of the tongue. Report of a case, with review of the literature.

    A benign mesenchymoma of the dorsal midtongue in an 88-year-old woman is presented. The tumour was excised under local anaesthesia, and microscopic examination revealed cartilage, bone, striated muscle, peripheral nerve, adipose and myxoid tissue, and blood vessels in addition to fibrous tissue. Eight cases including ours were found in the literature.
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8/14. Mesenchymal hamartoma in a 10-month-old infant: appearance by magnetic resonance imaging.

    A previously well 10-month-old infant boy presented with a large hepatic tumor. Its sonographic and computed tomographic appearances were similar to those reportedly found in mesenchymal hamartomas. Magnetic resonance (MR) imaging showed a multiseptated mass with fluid-filled compartments and some displacement of major intra-abdominal vessels. This appears to be the first report of MR imaging of a mesenchymal hamartoma.
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9/14. Cerebral necrosis following radiotherapy of extracranial neoplasms.

    We have examined 6 patients with delayed cerebral necrosis following irradiation of extracranial neoplasms. Four of the 6 patients received 1,760 rets (or less) tumor dose. The initial symptoms attributable to radiation necrosis appeared 4 to 31 months after irradiation and were those of a focal supratentorial mass. cerebral angiography delineated an avascular frontal or temporal lesion in all 6 patients; in 1 case a magnification study revealed narrowing, irregularity, and occlusion of small cortical vessels. Four of our 6 patients underwent craniotomy with partial or complete surgical extirpation of necrotic brain tissue. Two operated patients are alive and without disabling neurological symptoms 30 and 25 months, respectively, after the operation. The characteristic neuropathological features of delayed radiation necrosis of brain suggest that vascular injury rather than neuronal or glial damage is of primary pathogenetic significance.
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10/14. Recurring thoracal mesenchymoma.

    A four-times-recurring thoracal mesenchymoma is presented. Each tumor evolved for 2-5 years before resection, the general evolution of the case being of about 30 years. Histologically, the tumors, which involved the thoracal wall presented at the same time the predominating fibroblasto-fibrocytic structure sometimes forming whirls, different mesenchymal derivatives as vessels and angiomatous formations, osseous trabeculae, chondroblastic areas, and more or less dense zones with leiomyocytes sometimes forming myomatous and myofibromatous aspects. Mitoses and nuclear atypias were very rare. The diagnosis of benign recurring mesenchymoma of the thoracal wall and pleura is discussed.
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