Cases reported "Angiomyolipoma"

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1/22. Giant retroperitoneal angiomyolipoma. A case report.

    We discuss a case of a giant renal angiomyolipoma. A 28 year-old woman presented with a history of a painless and swelling abdominal mass. A computerized tomography (CT) scan of the abdomen demonstrated a 27 x 13 x 11 cm tumor in the right retroperitoneum. At laparotomy, a wide excision of the mass was performed and the histopathological examination of the resected specimen revealed the presence of an angiomyolipoma. The clinical, radiological and pathological findings of this case are reported with a review of the literature.
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2/22. Arteriovenous shunting in a giant renal angiomyolipoma. A rare condition.

    We report a case of a 33-year-old woman with tuberous sclerosis and bilateral angiomyolipomas. She suffered from acute left flank pain due to retroperitoneal haemorrhage. During renal arteriography an arteriovenous shunting was found in the left tumour. angiomyolipoma is a rare cause of angiographically demonstrable arteriovenous shunting.
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3/22. Giant polypoid tumor of the esophagus.

    A patient with a giant polypoid tumor of the esophagus, measuring 22 cm in length is described in this report. The patient presented with cough attacks and respiratory distress. Diagnostic and therapeutic intervention required aggressive airway management, radiographic and endoscopic evaluation, and definitive surgical treatment. Benign esophageal tumors are rarely seen and originate from the upper third of esophagus, frequently close to the cricopharyngeus muscle. They may attain giant proportions. A variety of clinical presentations are described, the most serious being asphyxia secondary to laryngeal obstruction. We observed a giant esophageal tumor which was interpreted as angiofibromyolipoma that caused laryngeal obstruction. We present the clinical picture and histopathological findings of the tumor.
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4/22. Giant angiomyolipoma associated with marked pulmonary lesions suggesting lymphangioleiomyomatosis in a patient with tuberous sclerosis.

    The association between tuberous sclerosis (TS) and angiomyolipoma (AML) is well known. A patient with TS and giant AML mimicking Renal Cell carcinoma (RCC), measuring 29 x 18 x 11 cm, weighing 4700 gr is presented. Imaging studies revealed coexistent pulmonary lymphangioleiomyomatosis and concurrent renal and pulmonary involvement is extremely rare in patients in TS. We believe that the growth potential of this hamartomatous lesion may reach to a life threatening size.
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5/22. Nephron-sparing nephrectomy for giant renal angiomyolipoma associated with lymphangioleiomyomatosis.

    Renal angiomyolipomas are benign tumors known to occur sporadically and in association with genetic syndromes, including tuberous sclerosis and lymphangioleiomyomatosis. Surgical removal or radiographic embolization of angiomyolipomas larger than 4 cm is usually indicated because of an increased risk of spontaneous hemorrhage. We describe successful nephron-sparing surgery for a giant angiomyolipoma and discuss relevant management issues in a patient with lymphangioleiomyomatosis who had previously undergone bilateral pulmonary transplantation.
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6/22. Secondary parathyroid hyperplasia in tuberous sclerosis: report of a case with large eosinophilic ganglion-like cells similar to those of subependymal giant cell astrocytoma, tubers, and atypical angiomyolipoma.

    We report a case of secondary parathyroid hyperplasia in a 49-year-old man with tuberous sclerosis. Two parathyroid glands had collections of large, eosinophilic ganglion-like endocrine cells that to our knowledge have not been previously described at this site. These cells are morphologically similar to those of subependymal giant cell astrocytoma, tubers, and atypical angiomyolipoma, all of which may arise in the setting of tuberous sclerosis. These large, eosinophilic ganglion-like cells found in different affected organs appear to be distinctive of tuberous sclerosis. We suggest these large eosinophilic cells arise from a common stem cell precursor that acquires variable phenotypes according to alterations in the cellular microenvironment.
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7/22. Giant bleeding renal angiomyolipoma: diagnosis and management.

    A case of a giant bleeding renal angiomyolipoma is presented. The patient was a 40-year-old Egyptian male with no clinical or radiological evidence of tuberous sclerosis. The radiological features and management, including the role of angiography are briefly discussed and the medical reviews on this subject are briefly considered.
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8/22. Imprint cytology of epithelioid angiomyolipoma in a patient with tuberous sclerosis. A case report.

    BACKGROUND: angiomyolipoma composed predominantly of epithelioid cells has been referred to as epithelioid angiomyolipoma. As this subtype shows considerable cellular atypia, it may be erroneously diagnosed as malignant epithelioid tumor, such as renal cell carcinoma and hepatocellular carcinoma. So far, only one report describing the cytologic findings of epithelioid angiomyolipoma has been documented, and epithelioid angiomyolipoma occurring in the peritoneal cavity has not been reported. CASE: Eleven years after resection of a renal epithelioid angiomyolipoma in a 34-year-old male with tuberous sclerosis, a tumor appeared in the peritoneal cavity and three masses in the liver. The intraoperative smears imprinted from part of the peritoneal mass revealed many large, atypical cells. The well-preserved atypical cells showed abundant, round to polyhedral, granular cytoplasm. Bizarre, giant nuclei with hyperchromasia and huge nucleoli were occasionally seen. Intranuclear cytoplasmic inclusions and mitotic figures were occasionally observed. As the epithelioid cells were markedly pleomorphic, we could not rule out hepatocellular carcinoma, cytologically and histologically, in the intraoperative consultation. In permanent sections the tumor was composed predominantly of epithelioid cells showing an alveolar pattern or sheetlike arrangement. Mitotic counts were zero to one per 10 high-power fields. Immunohistochemically, the epithelioid tumor cells were positive for vimentin, alpha-smooth muscle actin and HMB-45, consistent with epithelioid angiomyolipoma. MIB-1-labeling index was 1.6%. CONCLUSION: When one sees atypical epithelioid tumor cells in a tuberous sclerosis patient during an intraoperative consultation, one must consider epithelioid angiomyolipoma.
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9/22. Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis.

    OBJECTIVE: To report a case of bilateral giant renal angiomyolipoma associated with tuberous sclerosis, with successful treatment, and to review the literature concerning angiomyolipoma treatment. CASE REPORT: Patient with tuberous sclerosis and angiomyolipoma diagnosed by ultrasonography during her pregnancy. At that time, the angiomyolipoma on the right side was 9 cm in diameter. Conservative management was selected during her pregnancy. The patient returned 7 years later, with a 24.7 x 19.2 x 10.7 cm tumor on the right side and another of 13 x 11.5 x 6.5 cm on the left side, in addition to multiple small angiomyolipomas. A nephron-sparing surgery with tumoral enucleation was performed on the right side, and after 3 months, the tumor on the left side was removed. Renal function in the post-operative period was preserved, and contrast medium progression was uniform and adequate in both kidneys. CONCLUSION: We conclude that an angiomyolipoma larger than 4 cm should be removed surgically, since they have a greater growth rate and pose a risk of hemorrhage. Resection of smaller tumors is safe and has decreased morbidity. Tumoral enucleation is an effective treatment method that preserves kidney function.
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10/22. Giant extrarenal retroperitoneal angiomyolipoma: a case report and literature review.

    Extrarenal angiomyolipoma is an uncommon disease entity and is rare in the retroperitoneum. We report a case of giant extrarenal retroperitoneal angiomyolipoma mimicking liposarcoma. A 35-year-old woman presented with a history of increased abdominal circumference and urinary frequency. Abdominal computerized tomography demonstrated a 24 x 21 x 16 cm retroperitoneal fatty tumor displacing the right kidney to the left upper quadrant of the abdomen. At laparotomy, the tumor was widely excised and the right kidney was successfully preserved. Final histologic examination identified the tumor as an angiomyolipoma. At 4-month postoperative follow-up, no evidence of tumor recurrence was found and the right kidney was fully functional.
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