ABSTRACT
Sclerosing angiomatoid nodular transformation (SANT) is a splenic lesion composed of angiomatoid/vascular nodules surrounded by hyalinized/sclerotic stroma, fibroblasts, myofibroblasts, and inflammatory cells. The endothelium within the nodules has a phenotype resembling splenic sinusoids, capillaries, and small veins. Martel et al. (Am J Surg Pathol 28:1268-1279, 2004) suggested that SANT may represent the final pathway of a variety of splenic lesions including inflammatory pseudotumors (IPTs). Epstein-Barr virus (EBV) has a role in the genesis of some splenic IPTs, but its presence in SANT has not been investigated. Six cases of SANT are reported. All were stained for CD31, CD34, CD8, CD68, smooth muscle actin, muscle-specific actin, and CD30 and were tested for EBV by in situ hybridization (EBER). All cases showed angiomatoid nodules with complex expression of CD31, CD34, and CD8, with focal CD68. Expression of CD30 by endothelial cells was also seen. One case had small diffuse areas lacking nodules resembling an IPT and was positive for EBV. The inflammatory cells and the normal spleen were negative for CD30 and EBER. In conclusion, SANT shows upregulation of CD30 with respect to normal spleen. The presence of EBV in the stromal cells of a case supports the notion that a subset of SANT may be related to IPT.
Subject(s)
Angiomatosis/pathology , Herpesvirus 4, Human/isolation & purification , Ki-1 Antigen/analysis , RNA, Viral/analysis , Spleen/pathology , Splenic Diseases/pathology , Adult , Aged , Angiomatosis/virology , Female , Granuloma, Plasma Cell/pathology , Humans , Immunohistochemistry , In Situ Hybridization , Male , Middle Aged , Sclerosis , Spleen/chemistry , Spleen/virology , Splenic Diseases/virologyABSTRACT
A 41-yr-old patient with a history of von Hippel-Lindau (VHL) disease with previously removed bilateral pheochromocytomas and renal cell carcinoma presented with progressive obstructive jaundice due to multiple lesions in the pancreas. The pancreatectomy specimen showed a range of endocrine lesions including islet hyperplasia, nesidioblastosis, microadenomas, and endocrine carcinoma. In addition, some of the non-tumorous islets displayed peliosis. The endocrine carcinoma showed a biphasic pattern composed of typical endocrine cells and oncocytes. The oncocytic component showed widespread lymphovascular invasion and lymph node metastasis. Immunohistochemistry and electron microscopy confirmed that the oncocytic cells were endocrine. Focal areas contained cells with foamy cytoplasm, a feature that is associated with pancreatic endocrine tumors in VHL. This case expands the spectrum of lesions seen in the pancreas of VHL patients. There is some overlap with lesions encountered in multiple endocrine neoplasia type I. In addition, the endocrine lesions were composed of two main cell types (typical and oncocytic cells) with the oncocytic component invading lymphatic channels and spreading to regional lymph nodes.
