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1/43. Pleomorphic hyalinizing angiectatic tumor of soft parts: immunohistochemical study including the expression of vascular endothelial growth factor.

    We report morphologic, flow cytometric, and immunohistochemical findings in two cases of pleomorphic hyalinizing angiectatic tumor of soft parts. Both patients were middle-aged women with subcutaneous lesions located in the lower extremity. The tumors consisted of sheets of spindled and pleomorphic cells with frequent intranuclear pseudoinclusions associated with clusters of ectatic vessels surrounded by prominent perivascular hyaline material. Numerous, nonhyalinized vessels were also present, mostly in the peripheral areas of the lesions. Some of these vessels had their walls permeated by numerous small capillaries. Immunostaining for vascular endothelial growth factor (VEGF), a secreted protein that has been implicated in tumor-associated angiogenesis, demonstrated positive staining in both tumoral and endothelial cells. Tumor cells were also reactive to vimentin and CD34. Focal positivity for CD99 and factor xiiia was also present. flow cytometry yielded a diploid dna histogram with S-phase fraction of 7%. Our findings corroborate those from previously reported cases. They further suggest that angiogenesis and the angiogenic factor VEGF may play a role in the development of this peculiar tumor.
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2/43. Myxoid malignant fibrous histiocytoma of the uterus: a case with immunohistochemical, ultrastructural and tumor cell culture studies.

    The clinical and pathological features, including ultrastructural and immunohistochemical findings, of a primary myxoid malignant fibrous histiocytoma of the uterus in a 60-year-old woman are reported. Microscopically, the principal feature of the tumor was a hypocellular area with diffuse degeneration, containing thin-walled curvilinear vessels, in which hyperchromatic small spindle and stellate cells, sometimes with vacuolated cytoplasm, were found. The transplanted tumor of primary cultured cells in nude mice presented as a prominent myxoid stroma confirming the histological structure of the primary tumor. Immunohistochemically, the presence of epithelial or heterogenous mesenchymal tumor components or cells of smooth muscle derivation were excluded and the tumor cells were positive for vimentin, CD 68, alpha 1-antitrypsin and alpha 1-antichymotrypsin. Ultrastracturally, pseudopodia and filopodia at the cell membrane and intracytoplasmic lysosomal granules were common. The patient had debulking surgery but died 38 days after the primary onset with the tumor occupying the entire abdomen and the pelvis.
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3/43. Primary and secondary microvascular reconstruction of the upper extremity.

    Tissue defects of the upper extremity may result from trauma, tumor resection, infection, and congenital malformation. Restoration of anatomy and functional integrity may require microsurgical free flap transfer for coverage of bones, nerves, blood vessels, or tendons. Microsurgical tissue transfer also may be required prior to secondary reconstruction, such as tendon transfers or nerve or bone grafts. This article addresses indications for upper extremity reconstruction using microsurgical tissue transfer flap selection and strategies including primary and secondary reconstruction.
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4/43. Benign isolated fibrohistiocytic tumor arising from the central nervous system. Considerations about two cases.

    Benign fibrous histiocytomas (BFHs) are tumors with fibroblastic and histiocytic components without histological anaplasia. Intracerebral lesions are exceptional and to our knowledge a spinal location was not yet described. We describe 2 cases of BFHs of the neural axis: the first, a 22-month-old boy with Down's syndrome, presented with a paraparesis and the magnetic resonance (MR) of the spine disclosed an intradural extramedullary, thoracic mass, totally resected; the second, a 13-year-old boy with left partial motor seizures, in whom the MR of the brain showed an intracerebral, right frontal tumor, also surgically removed. Both patients are free of recurrence, 6 years and 15 months after surgery, respectively. Histological examination and immunoreactivity for vimentin and histiocytic markers favored the diagnosis of BFH. It is likely that these tumors may originate from spinal dura mater mesenchymal stem cells and from the intracerebral perivascular pial sheath or the brain vessel walls themselves, respectively. Other benign, isolated, intracranial fibrohistiocytic neoplasms, namely the juvenile xanthogranuloma, can harbor a clinical, morphological and immunohistochemical profile overlapping the one of the BFH. Intracranial germ cell tumors may be associated with Down's syndrome, although harboring an unusual, non-pineal and non-chiasmatic location. One can speculate that a similar, still unknown genetic mechanism responsible for this association, could also induce the growth of other type of tumors in patients with this syndrome. BFHs should be added to the differential diagnosis of intracerebral or spinal dural attached tumors. Furthermore, we propose to name these intracranial tumors "benign isolated fibrohistiocytic tumors of the CNS".
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5/43. Malignant fibrous histiocytoma of the lung.

    Primary malignant fibrous histiocytoma (MFH) of the lung is very rare. To date, only 32 reports of 63 cases of primary MFH of the lung have appeared in English, excluding tumors arising from the pulmonary arteries and pleura. We describe a patient with primary MFH of the lung who developed brain metastasis and involvement of pulmonary great vessels. In addition, we reviewed previously reported cases to establish the clinical characteristics and most appropriate management of primary pulmonary MFH. When disease is sufficiently limited, complete resection remains the mainstay of treatment.
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6/43. Malignant fibrous histiocytoma arising from the superficial femoral artery.

    Primary sarcomatous arterial tumors are rare. We have reported a case of malignant fibrous histiocytoma in an elderly woman successfully treated by resection of the involved femoral vessel and reconstruction with a PTFE graft. The long-term result in this patient is presently uncertain.
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7/43. Case report: recurrence of soft tissue MFH in bone due to minute intravenous tumor emboli detected by MRI.

    We recently encountered a case with local recurrence of malignant fibrous histiocytoma (MFH) in the bone after wide resection, caused by minute intravenous tumor emboli which were retrospectively detected in MR imaging. The patient was a 69-year-old woman who initially noticed a mass in her left thigh. The tumor was diagnosed to be MFH, therefore a wide resection was performed; although the tumor was closely attached to the periosteum, it was not difficult to dissect the tumor subperiosteally from the cortex of the femur. The patient received postoperative brachytherapy, but no chemotherapy. Two years later, the tumor recurred with bony destruction of the femur. We reviewed the pre-operative films obtained by various imaging modalities, as well as the histology of the primary tumor, and found minute intravenous tumor emboli in the MR imaging obtained before surgery. Tumor emboli were also observed histologically in the small vessels of the surgically resected tumor. Such intravenous tumor emboli have recently been implicated in the development of regional bone metastasis near the site of the primary lesion in cases of malignant soft tissue tumors. Therefore, we concluded that the tumor recurrence in our case was caused by small tumor emboli invading the perforating veins of the femur. It is therefore emphasized that MR images should be carefully reviewed for the presence of such intravenous tumor emboli before surgery in cases of high-grade malignant sarcomas. As at the time of writing, our patient remains alive and disease-free, with no evidence of any local recurrence or distant metastasis after wide tumor resection for the recurrent tumor.
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8/43. Recurrent aneurysmal fibrous histiocytoma.

    Aneurysmal fibrous histiocytoma is a rare variant of cutaneous fibrous histiocytoma that results from blood vessel proliferation and haemorrhage into a fibrous histiocytoma. The resulting lesion has a very different clinical appearance, hence the potential confusion with other skin lesions. This report describes the case of a 48 year old woman with a recurrent fibrous histiocytoma with prominent vasculature, which over a three year period recurred on two occasions, showing more progressive features of the aneurysmal variant. In addition, squamous lined cysts were present within this tumour, a finding that has not been described previously. The histological features of this rare lesion and the importance of the differential diagnosis from other similar appearing malignant lesions will be discussed.
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9/43. Retroperitoneal malignant fibrous histiocytoma mimicking pheochromocytoma.

    We report the case of a 52-yr-old man with a mass in the area of the left adrenal. The clinical features, MIBG uptake, and elevated urinary dopamine levels suggested the diagnosis of pheochromocytoma. He presented with unstable hypertension, tachycardia, weight loss, and the "inflammatory syndrome" (fever, leukocytosis, and high sedimentation rate). Clinical findings, preoperative radiographic (sonography, CT scan, [131I]MIBG scintigraphy), and endocrine evaluations (elevated 24-h urinary dopamine) were suggestive of a dopamine-secreting adrenal tumor. The mass was resected and on histologic examination showed the characteristic features of a malignant fibrous histiocytoma (MFH). The tumor cells were immunopositive for neuron-specific enolase (NSE), vimentin, CD-68, S-100, desmin, and immunonegative for chromogranin a, synaptophysin, neurofilament protein, and low-molecular-weight keratin, indicating that this tumor was not able to synthesize catecholamines. The prolonged retention of the tracer (MIBG) was interpreted as a consequence of obstructive hydronephrosis, while elevated urinary dopamine levels were assumed to be due to compression of the renal vessels by the large retroperitoneal mass.
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10/43. Atrophic dermatofibroma.

    Atrophic dermatofibroma, a newly proposed entity in recent times, is thought to be a specific variant of dermatofibroma. We report a typical case of atrophic dermatofibroma on the thigh of a 69-year-old female. The lesion consisted clinically of a light brown, intracutaneous nodule with a central crateriform depression, and histologically of fibrohistiocytic components in the thinning dermis. On elastica van Gieson stain, loss of elastic fibres and dense accumulation of elastic fibres around medium-sized vessels were observed in the lesion.
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