Cases reported "Pelvic Neoplasms"

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1/29. Unusual late extrapulmonary metastasis in osteosarcoma.

    The major site of metastasis from osteosarcoma is the lung, and over 90% of fatalities in patients with this disease die from pulmonary metastases. Extrapulmonary disease is developing in an increasing proportion of patients, usually after pulmonary metastasis. This study reports three cases of patients with osteosarcoma that metastasized to the brain, mediastinum, intramuscular site, and pelvic cavity. The physician must be aware that extrapulmonary metastases may be present at the time a pulmonary metastasis becomes evident.
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2/29. Multilocular cystic adenomyoma of the pelvic cavity: A rare clinical and histological entity in a young man.

    We report a large complex cystic pelvic mass encountered in a 16-year-old man complaining of perineal discomfort. A large and elastic hard mass was palpated on the right anterior wall of the rectum with no discernible prostate on digital rectal examination. Prostatic tumor markers were elevated. Excretory urography and urethrocystography showed a filling defect with a smooth edge on the right side of the bladder. magnetic resonance imaging demonstrated a mass with a mosaic pattern between the right side of the bladder and the rectum. We performed ultrasound-guided transrectal needle core biopsy of the mass. The pathologist suspected hyperplastic glandular epithelium of prostatic origin with focal inflammatory cell infiltration, but there was no sign of malignancy. We thought that the tumor arose from the prostate. Surgery was successfully performed. The tumor was located on the right side of the pelvic cavity and adhered to the right lobe of the seminal vesicle. En bloc excision was performed. This lesion histologically proved to be a multilocular prostatic tissue with a seminal vesicle component, without communication to the surrounding tissue. Such a lesion has not previously been reported in the literature.
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3/29. Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location.

    A peripheral primitive neuroectodermal tumor arising from the abdominopelvic cavity is reported in a 24-year-old young male without any previous remarkable pathology. He was admitted to our hospital with complaints of urinary symptoms (acute urinary retention) and mild intestinal occlusion that had been present for three months. physical examination and CT scan revealed a pelvic mass occupying the entire pelvic cavity. The diagnostic workup included a CT-guided biopsy which defined the tumor as a sarcomatous type. Radical surgery was performed including tumor resection, pelvic exenteration (bladder and prostate gland) and urinary and fecal diversion. Adjuvant chemotherapy (VAIA) was delivered once the histology was confirmed. We reviewed the available literature focusing on the varied nomenclature of this tumor (peripheral neuroepithelioma, Askin's tumor, Ewing's extraosseous tumor, peripheral adult neuroblastoma, peripheral primitive extracranial neuroectodermal tumor (PPNET), the clinical features, the role of diagnostic imaging techniques, pathologic assessment and controversial therapeutic management. In addition, the prognosis and survival of this rare condition were analyzed.
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4/29. Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.

    The perivascular epithelioid cell has been proposed to be the unifying proliferating cell type in a number of lesions such as angiomyolipoma, lymphangiomyomatosis, clear cell "sugar" tumor and renal capsuloma. With the exception of rare examples of angiomyolipoma, they are non-metastasizing. We report four examples of a new member of this family of perivascular epithelioid cell neoplasms that occur in abdominopelvic location and show metastatic properties. The patients, all women, were aged 19 to 41 years (mean, 32), and presented with a tumor mass involving the serosa of the ileum, uterus or pelvic cavity. Morphologically, the tumors were composed of sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm and moderately pleomorphic nuclei, traversed by a delicate vasculature, mimicking clear cell carcinoma. There were areas of coagulative necrosis and occasional mitotic figures. Intracytoplasmic brown pigment was present in two cases. Spindly cells, smooth muscle and fat were absent. Lymphovascular invasion was present in all, lymph node metastasis was documented in two and metastasis to the ovary was present in one case. Two patients developed widespread metastatic disease after 10 and 28 months from diagnosis. One patient showed the clinical signs of tuberous sclerosis. In spite of the epithelial-like appearance, the tumor cells were negative for epithelial markers but were strongly positive with the melanogenesis-related marker HMB45. Another melanogenesis marker (MART-1) was positive in two cases. Other markers including S-100 protein, vimentin, muscle-specific actin, desmin and chromogranin a were negative. Thus, these tumors are not readily classifiable in the existing schema of known entities, and show overlapping morpho-phenotypic features of clear cell "sugar" tumor of the lung and epithelioid angiomyolipoma. We consider them as sarcomas composed of a pure population of uncommitted perivascular epithelioid cell, that lack modulation toward smooth muscle or adipose cells.
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5/29. Late intracaval and intracardiac leiomyomatosis following hysterectomy for benign myomas treated by surgery and GnRH agonist.

    BACKGROUND: The aim of this study was to report an exceptional case of a patient presenting with intracaval and intracardiac leiomyomatosis treated by combined surgical and medical treatment. CASE: A 48-year-old presented with intracaval and intracardiac leiomyomatosis (IL) discovered 6 years following a total hysterectomy with ovarian conservation for myomas. Surgical resection of the pelvic myomas and intracaval leiomyomatosis was performed during the same surgical procedure. Given the presence of a small tumor residuum in the pelvic cavity, postoperative medical treatment based on a gonadotropin-releasing hormone (GnRH) agonist was delivered for 1 year. The patient was followed-up using clinical examination and systematic CT scan. Ten months following the end of medical treatment, she is still in good health and the pelvic residuum has stabilized. CONCLUSIONS: patients with pelvic tumor combined with IL could be treated using a one-stage surgical procedure. In cases of incomplete surgical resection, medical treatment based on GnRH agonist could be successfully delivered.
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6/29. Pelvic lipomatosis.

    Three cases of pelvic lipomatosis are presented. Excretory urogram revealed characteristic elevation and elongation of urinary bladder base and relative hyperlucency of pelvic cavity. Associated varying degrees of hydronephrosis and hydroureters are seen secondary to distal ureteral obstruction. barium enema showed elongation and elevation of rectosigmoid colon.
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7/29. Well-differentiated papillary mesothelioma in the pelvic cavity. A case report.

    BACKGROUND: Well-differentiated papillary mesothelioma (WDPM) is considered to be a distinct subtype of peritoneal mesothelioma. It occurs in the peritoneum, is most commonly seen in young women and is found incidentally at laparotomy for other indications. Clinically, WDPM is considered to be benign or to have low malignancy potential. CASE: A 48-year-old female with no history of asbestos exposure presented with hypermenorrhea. An operation was performed for adenomyosis, and six papillary nodules, 2 cm or less, were found in the serosa of the pelvic cavity. peritoneal lavage fluid and imprint material from the tumor were obtained for cytologic examination. The cytologic specimens showed many scattered cells and sheetlike clusters and some papillary clusters. These cells had abundant, polygonal, cyanophilic cytoplasm; clearly outlined borders; and slitlike intercellular spaces. The cell arrangement was orderly. The nuclei were uniform in size, with a single centrally located nucleolus, and there were no binucleated forms or mitosis. There was no increase in chromatin. On the luminal surface of the cells, a brush border was observed. CONCLUSION: It is important to differentiate WDPM from diffuse malignant mesothelioma or other peritoneal malignant tumors to avoid treating them as malignant tumors.
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8/29. Malignant hemangiopericytoma in the pelvic cavity successfully treated by combined-modality therapy: report of a case.

    A 55-year-old Japanese woman underwent extirpation of a malignant hemangiopericytoma in the pelvic cavity, followed by postoperative irradiation. An abdominal computed tomography scan 3 years later revealed a local recurrent tumor, 12 cm in diameter, in the pelvic cavity, for which transarterial embolization was done, followed by excision of the tumor employing Hartmann's procedure. Although an unresectable part of the recurrent tumor remained, postoperative irradiation reduced its size remarkably. The patient is still alive 7 years 2 months after her first operation, but with more recurrent tumors in the abdominal wall and around the bilateral iliac arteries. Because hemangiopericytoma often recurs or metastasizes after a prolonged disease-free interval, close long-term follow-up is necessary after the operation. Combined-modality therapy against the recurrent or unresectable disease may result in a good prognosis.
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9/29. Peripheral medulloepithelioma: an immunohistochemical, ultrastructural, and cytogenetic study of a rare, chemotherapy-sensitive, pediatric tumor.

    A case of peripheral medulloepithelioma, a rapidly growing tumor involving the pelvic cavity of a 12-year-old girl, is presented. The diagnosis was supported by expression of vimentin, nestin, alpha-internexin, neurofilaments, and microtubule-associated protein 5 and by characteristic ultrastructure that included absence of cilia or microvilli. trisomy of chromosomes 2 and 8 was the only detectable chromosomal abnormality. Combination chemotherapy resulted in complete remission. Because some of these rare tumors are sensitive to chemotherapy, their recognition and separation from other neuroectodermal tumors are advisable for better understanding of their biology and determination of optimal treatment.
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10/29. Modified gluteus maximus V-Y advancement flap for reconstruction of perineal defects after resection of intrapelvic recurrent rectal cancer: report of a case.

    Technical advances in myocutaneous flap preparation have resulted in primary reconstruction now being generally indicated for malignant tumors extensively infiltrating the pelvic cavity and perineum. Pelvic tumor resection can dramatically improve the health-related quality of life (QOL) of patients with locally recurrent rectal cancer complicated by infection and pain. However, the removal of a wide area of perineum by these two procedures often leaves a large dead space. A gluteal thigh muscle, rectus abdominis muscle, or pedicle myocutaneous flap is usually made to reconstruct such extensive perineal defects. The subject of this case report was a 76-year-old woman with recurrent rectal cancer in the pelvis after abdominoperineal resection. The large pelvic tumor, which was causing severe pain, was resected and the extensive perineal defects were reconstructed using a modified maximus V-Y advancement flap. The operating time was approximately 30 min, and the pain after surgery was much less severe. Moreover, she could walk the day after surgery and returned to normal daily life without requiring prolonged bed rest. No infection developed in the intrapelvic dead space postoperatively. This technique proved very useful for improving the patient's QOL.
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