Cases reported "Pelvic Neoplasms"

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1/21. 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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2/21. Parotid lymph node metastasis from adenocarcinoma of the urachus.

    The parotid gland and its lymph nodes are frequent sites of metastases from head and neck cancers. However, metastasis from a distant primary below the clavicle is unusual. These originate from a variety of sites, most commonly the lung, kidney and breast. A case of a 59-year-old woman with parotid lymph node metastasis from an adenocarcinoma of the urachus, diagnosed on the basis of two discrete periparotid masses on CT and the patient's history, is presented.
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3/21. Multifocal aggressive angiomyxoma: a case report.

    A case of aggressive angiomyxoma in a 25 year old woman is presented. The patient was admitted to hospital with a history of hesitancy of micturation and pain in the right iliac fossa. She was found to have a left labial mass, which was clinically diagnosed to be a Bartholin gland cyst. A pelvic ultrasound revealed an additional mass in the right paravesical region. At surgery, two distinct masses were removed, one from the right perivesical space and the other from the left labium. Both masses were rubbery, white, and gelatinous and showed similar histopathology findings of thick and thin walled vascular channels set in a loose myxoid stroma. A diagnosis of multifocal aggressive angiomyxoma was made. This is the first reported case of aggressive angiomyxoma occurring as two distinct masses in one patient.
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4/21. 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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5/21. Pelvic lipomatosis associated with proliferative cystitis occurring in two brothers.

    Pelvic lipomatosis is a rare disorder of benign mature adipose tissue proliferation around the bladder and rectum. Most cases are associated with proliferative cystitis, in particular, cystitis glandularis. The etiology of pelvic lipomatosis and its association with proliferative cystitis are not well understood. This is the first reported case of familial pelvic lipomatosis. The pathogenesis and the possibility that this disorder could be secondary to genetic abnormalities of the HMG-IC (high mobility group) gene on chromosome 12 are discussed.
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6/21. A case of von hippel-lindau disease with bilateral pheochromocytoma, renal cell carcinoma, pelvic tumor, spinal hemangioblastoma and primary hyperparathyroidism.

    A rare case of von Hippel-Lindau (VHL) disease with bilateral pheochromocytomas, right renal cell carcinoma, right pelvic carcinoma, spinal hemangioblastoma and primary hyperparathyroidism is described. A 78-year-old woman had a history of hypertension from her forties. She suffered from headache and body weight loss. Abdominal CT revealed bilateral adrenal tumors and right external renal tumors enhanced in early stage. MIBG scintigraphy exhibited a high accumulation of tracer in both adrenal glands. On the basis of the radiographic findings and endocrinological results, the patient was diagnosed as having bilateral pheochromocytomas and right renal cell carcinoma. A bilateral adrenectomy was performed, followed by surgery for resection of the renal cell carcinoma. The other resected right kidney showed a clear cell subtype that was determined to be renal cell carcinoma, and proved that the pelvic tumor was transient cell carcinoma. Spinal MRI showed spinal hemangioblastoma. von Hippel-Lindau (VHL) gene mutation for the patient was found. We diagnosed the patient as VHL because of the existence of spinal hemangioma and a VHL disease gene. Parathyroid echo revealed a hypoechoic space on the back of the left lobe, and serum calcium and intact PTH to be elevated. The patient was diagnosed as primary hyperparathyroidism. We report the first case of a patient with VHL disease complicated with bilateral pheochromocytomas, right renal cell carcinoma, right renal pelvic carcinoma and primary hyperparathyroidism. The life expectancy of affected individuals has been less than 50 years. Since the prognosis may be improved by an early diagnosis, affected individuals with VHL complexes should undergo cranial, spinal MRI and abdomen CT. The families may benefit from presymptomatic detection of affected gene carriers and the exclusion of at-risk family members by negative test results.
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7/21. dermoid cyst in bony pelvis that coexists with pheochromocytoma: report of a case and review of the literature.

    A 42-year-old man presented with right lower quadrant abdominal pain and dysuria. The bladder was displaced to the right side of the pelvis in excretory urography. Abdominal CT revealed a mass in right adrenal gland, measuring 8 cm in diameter. There was also a cystic mass; filling left half of the bony pelvis and displacing bladder to the right, measuring 14.5 x 10, 5 x 7 cm. The patient underwent right adrenalectomy and pelvic mass excision. Pathologic examination showed that the adrenal mass was pheochromocytoma and pelvic mass was dermoid cyst. This case is the first one in literature that an intrapelvic dermoid cyst is not derived from an organ coexists with pheochromocytoma.
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8/21. Signet ring cell carcinoma of the breast as a source of pelvic floor metastatic mass. A case report.

    Primary signet ring cell carcinoma of the breast is a very rare tumour. We present a case with pure signet ring cell carcinoma of the breast, which was recognized as metastasis on the pelvic floor, before developing breast symptoms and signs. A 40-year old woman was admitted with abdominal pain. First diagnostic effort revealed a cystic mass on the pelvic floor, compressing the colon and other neighbouring organs. A biopsy of the pelvic mass was performed. The histopathological examination revealed metastatic signet-ring cell carcinoma. At the time of the first operation, the mammary glands were not suspicious. No other sources of primary tumour were evidenced. An inflammatory sign developed in right breast two months after biopsy of the pelvic metastasis. The histopathology of the breast incisional biopsy revealed primary pure signet ring cell carcinoma of the breast. Because the oestrogen and progesterone receptor were negative in the tumoral tissue, the patient underwent chemotherapy followed by modified radical mastectomy, chemotherapy, and palliative resection of the metastatic mass. The patient was followed up for eight months. To our knowledge, in English literature, we believe that this case is the first report of signet ring cell carcinoma of the breast presenting with pelvic floor metastasis without breast sign.
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9/21. Pelvic lipomatosis associated with cystitis glandularis and adenocarcinoma of the bladder.

    Pelvic lipomatosis is a rare condition characterized by an overgrowth of normal fat in the perivesical and perirectal spaces. cystitis glandularis, cystica or follicularis has been observed in 75% of the patients with pelvic lipomatosis. Although cystitis glandularis is widely regarded as premalignant few reports have documented its transition to adenocarcinoma. We describe a 41-year-old man with pelvic lipomatosis in whom primary adenocarcinoma of the bladder developed 6 years after a histological diagnosis of cystitis cystica and glandularis was established. To our knowledge this is the second case reported of pelvic lipomatosis associated with proliferative cystitis and adenocarcinoma of the bladder, indicating that intestinal metaplasia of the urothelium may be a precursor of malignancy in these patients.
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10/21. Extrauterine mesodermal (mullerian) adenosarcoma: a clinicopathologic analysis of five cases.

    Five extrauterine examples (three pelvic and two ovarian) of a distinctive mesodermal (mullerian) mixed tumor previously described to occur in the uterus and designated mullerian adenosarcoma are reported. The tumors were diagnosed during the fifth to eight decades of life. They usually formed very large, partly cystic masses, which had occasionally spread to adjacent organs by the time of operation. On microscopic examination the tumors consisted of benign-appearing neoplastic glands lying in a sarcomatous stroma. The glands were lined by a variety of mullerian epithelial cell types, and the stromal element resembled endometrial stromal sarcoma. Three patients had malignant courses manifested by intrabdominal recurrence, distant metastases, or both. This tumor should be clearly distinguished from the more common forms of mesodermal (mullerian) mixed tumor in which the epithilial as well as the stromal component is malignant and the prognosis is much more grave.
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