Diagnosis Of Littoral Cell Angioma Research Articles

S3385 Diagnosis of Littoral Cell Angioma of the Spleen With Endoscopic Ultrasound-Guided Fine Needle Aspiration Biopsy

S3385 Diagnosis of Littoral Cell Angioma of the Spleen With Endoscopic Ultrasound-Guided Fine Needle Aspiration Biopsy

Extramedullary hemopoiesis and littoral cell angioma of the spleen: our experience and review.

Littoral cell angioma (LCA) is a rare primary vascular neoplasm of the spleen. A 54-year-old man was referred to our emergency department for abdominal pain. A CT scan showed multiple round hypodense lesions of various sizes throughout the spleen. The spleen was increased in volume. An MRI confirmed the lesion with a suspect of multiple angiomas vs. amartomas. The haematologists excluded any haematological disease. After a collegial discussion, we decided to perform laparotomic splenectomy. Histologically, the multiple lesions consisted in anastomosing vascular channels lined by plump cells. There was an increased number of dysmorphic megakaryocites inside the splenic parenchyma and along the tumour's border, known signs of extramedullary hemopoiesis, whose etiology in our patient was unexplained. To the best of our knowledge this is the third description of the association between littoral cell angioma and extramedullary hemopoiesis. LCA is a rare primary splenic vascular tumour that originates from the splenic littoral cells. The diagnosis of littoral cell angioma is confirmed histologically and on immunohistochemistry. This case report underlines the rarity of this type of benign splenic neoplams, but since the malignant potential of LCA, we recommend close clinical follow- up of patients with LCA of the spleen.

Littoral cell angioma in main and accessory intrapancreatic spleen presenting as splenic rupture

We report the incidental finding of a nodular mass in the pancreatic tail on a contrast-enhanced computed tomography scan preinterventional to emergency laparotomy for splenic rupture. Because of the past surgical history and radiologic appearance, differential diagnosis included atypical lymphoma in the spleen and regional lymph node, pancreatic adenocarcinoma with splenic metastasis, and intrapancreatic metastase of malignant melanoma; the patient underwent both splenectomy and pancreatic tail resection. A diagnosis of littoral cell angioma in main and accessory intrapancreatic spleen was made. To our knowledge, this is the first description of littoral cell angioma of the spleen involving both main and accessory organ presenting as splenic rupture.

Diffuse sinusoidal hemangiomatosis of the spleen: A case report with enzyme-histochemical, immunohistochemical, and electron-microscopic findings

Diffuse hemangiomatosis of the spleen is a very rare benign tumor in which the whole spleen is permeated by neoplastic blood vessels. It is occasionally accompanied by severe disturbances of blood coagulation. The histogenesis of this tumor remains obscure. No systematic investigations of the immunophenotype of the neoplastic endothelium have been published. We describe a case of isolated benign diffuse hemangiomatosis of the spleen in which the enzyme-histochemical and immunohistochemical findings suggested an origin in the splenic sinus endothelial cells. Some of the tumor endothelial cells reacted with UEA-1, BMA 120, antibodies against the von Willebrand factor, CD34, and CD8, an antigen which, in man, is expressed only by suppressor/cytotoxic T cells and the endothelial cells of the splenic sinuses. Enzyme-histochemical investigations revealed reactivity for nonspecific esterase and lack of reactivity for alkaline phosphatase--a pattern typical of the sinus endothelial cells. The tumor could be distinguished from other tumors/tumor-like lesions of the spleen that exhibit endothelium with characteristics typical of the splenic sinuses (peliosis, splenoma, littoral cell angioma) on the basis of its histological features. The lack of expression of histiocytic antigens by the tumor endothelium is also evidence against a diagnosis of littoral cell angioma, which also derives from the sinus endothelium. Thus, this tumor could not be identified as any of the recognized tumors/tumor-like lesions of the spleen and it is therefore proposed that it should be designated diffuse sinusoidal hemangiomatosis.