Lymphangiomas are rare benign cystic tumors of the lymphatic system.Retroperitoneal lymphangiomas account for 1%of all lymphangiomas,and approximately 186 cases have been reported.They may clinically present as a palp...Lymphangiomas are rare benign cystic tumors of the lymphatic system.Retroperitoneal lymphangiomas account for 1%of all lymphangiomas,and approximately 186 cases have been reported.They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors,including those arising from the liver,kidney and pancreas.This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient.The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention,abdominal pain,anorexia,fever,nausea and diarrhea.Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis.Surgical removal of the cyst was accomplished without incident.A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.展开更多
Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been...Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. Importantly, it can cause fatal complications such as volvulus or involvement of the main branch of the mesenteric arteries, requiring emergency surgery. Moreover, the gross and histopathologic findings may resemble benign multicystic mesothelioma and lymphangiomyoma. Immunohistochemical study for factor Ⅷ-related antigen, D2-40, calretinin and human melanoma black-45 (HMB-45) are essential for diagnosis. Factor Ⅷ-related antigen and D2-40 are positive in lymphangioma but negative in benign multicystic mesothelioma. HMB-45 shows positive study in the smooth-muscle cells around the lymphatic spaces of the lymphangiomyoma. We report a case of smallbowel volvulus induced by mesenteric lymphangioma in a 2-year-and-9-mo-old boy who presented with rapid abdominal distension and vomiting. The abdominal computed tomography scan showed a multiseptated mass at the right lower quadrant with a whirllike small-bowel dilatation, suggestive of a mesenteric cyst with midgut volvulus. The intraoperative findings revealed a huge, lobulated, yellowish pink, cystic mass measuring 20 cm × 20 cm × 10 cm, that was originated from the small bowel mesentery with small-bowel volvulus and small-bowel dilatation. Cut surface of the mass revealed multicystic spaces containing a milky white fluid. The patient underwent tumor removal with small-bowel resection and end-to-end anastomosis. Microscopic examination revealed that the cystic walls were lined with flat endothelial cells and comprised of smooth muscle in the walls. The flat endothelial cells were positive for factor Ⅷ-related antigen and D2-40 but negative for calretinin. HMB-45 showed negative study in the smooth-muscle cells around the lymphatic spaces. Thus, the diagnosis was lymphangioma of the small bowel mesen展开更多
文摘Lymphangiomas are rare benign cystic tumors of the lymphatic system.Retroperitoneal lymphangiomas account for 1%of all lymphangiomas,and approximately 186 cases have been reported.They may clinically present as a palpable abdominal mass and can cause diagnostic dilemmas with other retroperitoneal cystic tumors,including those arising from the liver,kidney and pancreas.This report describes the rare case of a cystic retroperitoneal lymphangioma in a 54-year-old male patient.The lymphangioma had progressed to the point of inducing clinical symptoms of abdominal distention,abdominal pain,anorexia,fever,nausea and diarrhea.Radiological imaging revealed a large multiloculated cystic abdominal mass with enhancing septations involving the upper retroperitoneum and extending into the pelvis.Surgical removal of the cyst was accomplished without incident.A benign cystic retroperitoneal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains.
文摘Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. Importantly, it can cause fatal complications such as volvulus or involvement of the main branch of the mesenteric arteries, requiring emergency surgery. Moreover, the gross and histopathologic findings may resemble benign multicystic mesothelioma and lymphangiomyoma. Immunohistochemical study for factor Ⅷ-related antigen, D2-40, calretinin and human melanoma black-45 (HMB-45) are essential for diagnosis. Factor Ⅷ-related antigen and D2-40 are positive in lymphangioma but negative in benign multicystic mesothelioma. HMB-45 shows positive study in the smooth-muscle cells around the lymphatic spaces of the lymphangiomyoma. We report a case of smallbowel volvulus induced by mesenteric lymphangioma in a 2-year-and-9-mo-old boy who presented with rapid abdominal distension and vomiting. The abdominal computed tomography scan showed a multiseptated mass at the right lower quadrant with a whirllike small-bowel dilatation, suggestive of a mesenteric cyst with midgut volvulus. The intraoperative findings revealed a huge, lobulated, yellowish pink, cystic mass measuring 20 cm × 20 cm × 10 cm, that was originated from the small bowel mesentery with small-bowel volvulus and small-bowel dilatation. Cut surface of the mass revealed multicystic spaces containing a milky white fluid. The patient underwent tumor removal with small-bowel resection and end-to-end anastomosis. Microscopic examination revealed that the cystic walls were lined with flat endothelial cells and comprised of smooth muscle in the walls. The flat endothelial cells were positive for factor Ⅷ-related antigen and D2-40 but negative for calretinin. HMB-45 showed negative study in the smooth-muscle cells around the lymphatic spaces. Thus, the diagnosis was lymphangioma of the small bowel mesen
