Cases reported "Rhabdomyosarcoma"

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1/16. Primary rhabdomyosarcoma of the central nervous system.

    A case of primary rhabdomyosarcoma in the brain stem is described in an 8 year old girl. The clinical data showed a right side hemiplegia then a total paralysis of the left sixth cranial nerve and a paraplegia which became lethal in 3 months. The necrospy revealed a tumoral nodule in the left medulla oblongata and pons with diffuse subarachnoidal extension from the cranial nerves to the cauda equine roots. Histologically the tumor appeared to be polymorph with numerous rhabdomyoblasts which had a clear cross striation and which were sometimes less differentiated without any neuronal or glial elements. Perivascular tumoral cells and blood vessels were closely linked, the Virchow-Robin spaces were clearly involved. The electron microscopic study confirmed that the less differentiated cells were of a rhabdomyoblastic nature. A review of the litterature indicates that these malignant neoplasias are highly exceptional, and can be classified within the group of primary tumors of the neuraxis with muscular elements. The histogenetic origin of these tumors appears to be the ectomesenchyme of neural crests.
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2/16. Jejunoileal alveolar rhabdomyosarcoma. A case report.

    Intestinal localization of rhabdomyosarcoma is exceptional, this case is the first to be published in the world literature. A 35-year-old patient with abdominal pain, fever, was found to have an infiltrative white-grey tumour, involving 20 cm the jejunoileal wall and also the surrounding mesenterium up to the origin of upper mesenterical vessels and lymph nodes. Histologic examination showed an alveolar type of rhabdomyosarcoma intricated with solid undifferentiated tumoral cells. The presence of multinucleated giant cells and the positivity of protein s 100 reaction was important for differential diagnosis, given the alveolar soft part sarcoma, malignant mesothelioma, malignant melanoma or papillary carcinoma.
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3/16. radiation retinopathy: electron microscopy of retina and optic nerve.

    A 4 1/2 year old female was treated for embryonal rhabdomyosarcoma of the left orbit in 1975 with radiation (59.5 Gy in 5 weeks), followed by chemotherapy. An electroretinogram (ERG) in March, 1988 revealed cone responses 3% of normal and no rod responses in the left eye, and normal responses in the right eye. The eye was enucleated in April 1988. In the fovea no choroidocapillaris was seen at the intact Bruch's membrane, and the pigment epithelium was preserved only in small patches. No photoreceptor cells were seen in the areas devoid of pigment epithelial cells. The parafoveal and peripheral (30 degrees eccentricity) retina was better preserved. The thickness of the layer of rods and cones and of Henle's fiber layer was reduced. Very few outer segments were present. macrophages had invaded the retinal tissue in moderate numbers. The retinal vessels were ensheathed by several layers of collagen fibrils. The spatial densities of pigment epithelial, cone, rod, and bipolar cells had been reduced. The optic nerve contained a total number of 1,022,000 nerve fibers.
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4/16. Structural fat grafting: more than a permanent filler.

    Grafted fat has many attributes of an ideal filler, but the results, like those of any procedure, are technique dependent. Fat grafting remains shrouded in the stigma of variable results experienced by most plastic surgeons when they first graft fat. However, many who originally reported failure eventually report success after altering their methods of harvesting, refinement, and placement. Many surgeons have refined their techniques to obtain long-term survival and volume replacement with grafted fat. They have observed that transplanted fat not only adjusts facial and body proportion but also improves surrounding tissues into which the fat is placed. They have noted not only the improvement in the quality of aging skin and scars but also a remarkable improvement in conditions such as radiation damage, chronic ulceration, breast capsular contracture, and damaged vocal cords. The mechanism of fat graft survival is not clear, and the role of adipose-derived stem cells and preadipocytes in fat survival remains to be determined. Early research has indicated the possible involvement of more undifferentiated cells in some of the observed effects of fat grafting on surrounding tissues. Of particular interest is the research that has pointed to the use of stem cells to repair and even to become bone, cartilage, muscle, blood vessels, nerves, and skin. Further studies are essential to understand grafted fat tissue.
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5/16. Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases.

    Myxofibrosarcoma (MFS) is one of the most common soft tissue sarcomas of elderly patients and has a predilection for the limbs. Herein, we report a previously undescribed variant of MFS showing epithelioid morphology. Seventeen cases diagnosed as epithelioid MFS were retrieved from the authors' files from among 570 cases of MFS. hematoxylin and eosin-stained sections were reexamined and immunostains for pan-keratin (15 cases), S-100 protein (15), desmin (15), and alpha-smooth muscle actin (13) were performed. Nine patients were men and 8 were women (age range 43 to 89 y; median 63.5). Fifteen patients presented with a mass, and in 2 of these there was also pain. Duration of symptoms varied from 1 to 24 months (median 3). Tumor size ranged from 2 to 15 cm (median 6.75). In 10 cases, the tumor was located in subcutaneous tissue and in 6 cases it was subfascial. The majority of the tumors were located on the limbs (8 lower extremities and 6 upper extremities) followed by neck (1), scalp (1), and trunk (1). Follow-up was available for 14 patients (range 2 to 240 mo; median 16). Twelve patients were treated by surgery followed by chemotherapy and/or radiation (8 cases). One patient received chemotherapy after an incisional biopsy and 1 patient was treated by surgery alone. Ten patients (71.4%) developed local recurrences. Seven patients (50%) developed metastases to lungs or retroperitoneum. Five patients (35.7%) have died of disease so far. Two patients were lost to follow-up. Morphologically, 14 cases were high grade, 2 were intermediate, and 1 was low grade. Tumors were characterized by a multinodular, infiltrating growth pattern with alternation of hypercellular and hypocellular myxoid areas; the latter showed prominent curvilinear vessels. Neoplastic cells were arranged singly and in small clusters in the myxoid areas or formed sheets in the hypercellular areas, where they showed epithelioid morphology with round nuclei, vesicular chromatin, prominent nucleoli, and moderate amounts of eosinophilic cytoplasm. The epithelioid areas were generally multifocal with admixed areas of conventional MFS. Immunostains were negative for all markers studied. Differential diagnosis included carcinoma, melanoma, myoepithelial carcinoma, pleomorphic liposarcoma, and pleomorphic rhabdomyosarcoma. In conclusion, epithelioid MFS is a rare variant of MFS, accounting for <3% of MFS in consultation material. Its natural history seems more aggressive than usual high-grade MFS, with approximately 70% local recurrence and 50% metastases, even within a relatively short follow-up period.
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6/16. A clinicopathological study on soft tissue tumors of the head and neck.

    The present study consists of 651 cases of soft tissue tumors originating in the head and neck area. Among these tumors 628 were benign (96%), while there were only 23 malignant cases (4%). In the benign group hemangiomas showed the highest incidence (47%) followed by tumors of adipose tissue (19%) and those of peripheral nerves (12%). Rare tumors consisted of nodular fascitis, hibernoma, lipoblastomatosis, glomus tumor, granular cell tumor, and chondroma. The majority of cases in the malignant group were either those of muscle origin or blood vessel origin. Excepting for two cases of rhabdomyosarcoma of the embryonal type and two cases of angiosarcoma, all showed recurrence and/or metastasis and terminated in death. Three cases of liposarcoma of either well-differentiated or myxoid type had a good prognosis with all three living for 3 or more years without any recurrence. Other malignant tumors consisted of malignant schwannoma, fibrosarcoma, malignant fibrous histiocytoma, alveolar soft part sarcoma, and chordoma. The age, sex, anatomical location, and differential diagnosis have also been described.
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7/16. Malignant "triton" tumor with metastatic hemangiopericytoma in a patient associated with von Recklinghausen's disease.

    An autopsy case of malignant schwannoma in a 31-year-old female associated with von Recklinghausen's disease with unusual elements is reported. The patient who was diagnosed as von Recklinghausen's disease from a biopsy specimen of a subcutaneous tumor in her shoulder and multiple cafe au lait spots in her chest and back, complained of gradual increase in pain in her lower abdomen. A large mass measuring about 18 cm in diameter was located in her left retroperitoneum. Malignant schwannoma associated with von Recklinghausen's disease was indicated by the cellularity of the tumor, intersecting bundles of spindle cells, alternating cellular and less cellular myxoid areas in the peripheral portion of the tumor, whereas a large part of the tumor had a hemangiopericytoma-like arrangement with gaping blood vessels. In addition, however, foci of rhabdomyosarcomatous differentiation and of angiosarcomatous components were found in some areas of this tumor. Moreover, intravascular schwannian growth, which was presumably related to von Recklinghausen's disease, was observed in the heart and thyroid. This tumor should be classified as a malignant triton tumor because of the presence of rhabdomyoblasts.
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8/16. Pseudosarcoma botyroides in pregnancy: report of a case with ultrastructural observations.

    A case of pseudosarcoma botryoides of the vagina diagnosed in advanced pregnancy is described. The cellular characteristics (atypical cells and abnormal mitoses) suggested malignancy, but ultrastructural studies demonstrated the fibroblastic nature of the cells, thus confirming the diagnosis of pseudosarcoma. Local excision and subsequent cesarean section was the method of treatment. Three years later, another pregnancy was terminated by repeat cesarean section and uncontrollable hemorrhage requiring emergency hysterectomy. The histologic study of the bleeding area revealed very edematous areas and lacunaelike endothelium-lined vessels. No cellular abnormalities were observed.
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9/16. Gliomyosarcoma. Report of a case of rhabdomyosarcoma arising in a malignant glioma.

    The development of sarcoma within a glioblastoma is a well recognised complication: in the majority of cases it is a fibro-sarcoma originating in the tissues of the proliferating hyperplastic blood vessels. In the case described here the tumour is a rhabdomyosarcoma, apparently derived from abnormal prolific vascular mesenchyme. Only one comparable example has been recorded previously.
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10/16. Transaxillary latissimus dorsi flap reconstruction in head and neck cancer. Limitations and refinements in 56 cases.

    The transaxillary latissimus dorsi musculocutaneous flap is suitable whenever a large volume of tissue is required for head and neck reconstruction. Fifty-six transaxillary latissimus dorsi musculocutaneous flap reconstructions were performed in 55 patients. There were two cases of complete flap necrosis and eight cases of partial flap necrosis. The latissimus dorsi vascular pedicle is separate from the irradiated field. The pedicled latissimus dorsi flap provides coverage of the orbitocranium, including the supraorbital region and central portion of the upper face. In the event that the pedicled latissimus dorsi flap does not reach far enough cephalad, the nutrient vessels may be separated from the axillary artery and anastomosed to vessels in the neck. Combined defects of the esophagus, mandibulofacial region, and neck may be reconstructed with a single large latissimus dorsi flap. Hairless skin particularly suitable for oral cavity reconstruction is usually available. Aesthetic and functional deficits are minimal after latissimus dorsi reconstruction. Disadvantages of this technique include repositioning of the patient, increased blood loss, and longer operating time. Permanent brachial plexus injury may occur. The latissimus dorsi musculocutaneous flap should not be used when defects can be reconstructed by simpler methods.
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