Case Of Sclerosing Angiomatoid Nodular Transformation Research Articles

Unusual Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen in an Adolescent Patient: Case Report and Literature Review

Sclerosing Angiomatoid Nodular Transformation (SANT) is a rare and benign lesion arising from the red pulp of the spleen, with an unknown etiopathogenesis. These tumors are usually asymptomatic and are found incidentally on radiographic examination. Therefore, high clinical suspicion is of great importance for the diagnosis. Splenectomy provides complete cure, and no recurrence and/or malignant transformation was reported to date. In this study, a rare case of SANT was reported in aadolescent male, and was discussed with the relevant literature.

Loss of CTNNB1 exon 3 in sclerosing angiomatoid nodular transformation of the spleen.

Sclerosing angiomatoid nodular transformation (SANT) is a rare vascular lesion of the spleen. Although several hypotheses have been suggested, the etiopathogenesis of SANT remains unknown. It is also unclear whether SANT is a reactive or a neoplastic lesion. Since CTNNB1 (β-catenin gene) exon 3 mutations were frequently detected in some rare fibrovascular lesions, we aimed to investigate the presence of oncogenic CTNNB1 mutations in SANT cases. For this purpose, 7 cases of SANT with typical histopathological features were retrieved. First, the presence of CTNNB1 exon 3 alterations was examined with a recently described immunohistochemistry-based method. Then, the findings were confirmed with polymerase chain reaction (PCR), reverse transcription PCR (RT-PCR), and Sanger sequencing. In all cases, immunochemistry of β-catenin gave a staining pattern that was suggestive of exon 3 alteration; however, no missense mutations were found in any case at the CTNNB1 exon 3 hotspot region. Subsequently, we screened for large interstitial deletions of CTNNB1 exon 3 which revealed short PCR products in three cases. Sequencing confirmed that these cases had large interstitial deletions, resulting in loss of the entire exon 3 of CTNNB1. In the remaining four cases, loss of exon 3 was documented at the cDNA level, although genomic deletion was not identified. These results demonstrate that loss of CTNNB1 exon 3 and stabilization of β-catenin with activation of Wnt signaling pathway might have a significant role in the pathogenesis of SANT. Through this study, we provided important evidence for the neoplastic nature and pathogenesis of this disorder.

Sclerosing Angiomatoid Nodular Transformation (SANT) in Spleen - A Case Report -

Sclerosing angiomatoid nodular transformation (SANT) of spleen is a rare inflammatory tumor-like vascular lesion composed of angiomatoid nodules in a fibrosclerotic background. We report here - in on a case of SANT in the spleen with its pathologic features, and review the related literature. A 50-year-old woman presented with mild left upper quadrant discomfort and tenderness and she showed a 6 cm-sized solitary splenic mass on computed tomography. She underwent laparo- scopic splenectomy. Grossly, the spleen showed a well circumscribed round-shaped solid mass with multinodular hemorrhagic surfaces. Microscopically, the mass consisted of multiple angio- matoid nodules surrounded by collagen bundles with fibroblasts and a lymphoplasma cell infiltra - tion. Immunohistochemically, the cells of the angiomatoid nodules were positive for CD31, CD30, CD34, alpha-smooth muscle actin, and VWF-VIII, but they were negative for CD8, anaplastic lym- phoma kinase protein, and D2-40. The patient has been under close follow-up without recur- rence.

Sclerosing angiomatoid nodular transformation of the spleen masquerading as a sarcoma metastasis

We report a case of sclerosing angiomatoid nodular transformation (SANT) of the spleen presenting as an incidental splenic mass in a patient with a history of retroperitoneal spindle cell sarcoma. Imaging studies and preoperative fine needle biopsy failed to differentiate this lesion from other vascular splenic lesions or a metastatic focus of a prior sarcoma. The patient was treated with splenectomy, which has proved both diagnostic and therapeutic in this and other cases of SANT. Although histology can lead to the diagnosis of vascular tumor, immunohistochemistry is the only way to confirm the diagnosis of SANT. The etiology of SANT is unknown. SANT of the spleen is a benign lesion that does not recur after splenectomy.

Sclerosing angiomatoid nodular transformation of the spleen masquerading as a sarcoma metastasis

We report a case of sclerosing angiomatoid nodular transformation (SANT) of the spleen presenting as an incidental splenic mass in a patient with a history of retroperitoneal spindle cell sarcoma. Imaging studies and preoperative fine needle biopsy failed to differentiate this lesion from other vascular splenic lesions or a metastatic focus of a prior sarcoma. The patient was treated with splenectomy, which has proved both diagnostic and therapeutic in this and other cases of SANT. Although histology can lead to the diagnosis of vascular tumor, immunohistochemistry is the only way to confirm the diagnosis of SANT. The etiology of SANT is unknown. SANT of the spleen is a benign lesion that does not recur after splenectomy.

La transformation angiomatoïde nodulaire sclérosante de la rate

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a complex vascular lesion recently described. We present a case of SANT occurring in a 49-year-old woman who was treated for choroid melanoma. The computed tomography abdominal scan revealed an asymptomatic mass in the spleen. Splenectomy was performed due to the risk of metastasis from the melanoma. The histopathologic examination revealed an angiomatoid lesion composed of three types of vessels recapitulating the normal composition of the red pulp of the spleen. In this report, we discuss the pathology of this lesion and the differential diagnosis of SANT with other lesions such as hemangiomas, hamartomas and inflammatory pseudotumors.

Detection of Epstein–Barr virus-encoded small RNA-expressed myofibroblasts and IgG4-producing plasma cells in sclerosing angiomatoid nodular transformation of the spleen

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare inflammatory tumor-like lesion composed of vascular nodules and non-neoplastic stroma including spindle cells and inflammatory cells. The focus of our study was on the stromal proliferating process in SANT. Nine cases of SANT were examined. All cases showed alpha-smooth muscle actin (alpha-SMA) and vimentin on the spindle cells but not CD21, CD31, CD34, CD68, desmin, S100, human herpes virus-8, or anaplastic lymphoma kinase-1. In one case, 20-30% of the myofibroblasts in Epstein-Barr-virus (EBV)-positive spindle cells were detected using double-labeling immunohistochemistry for alpha-SMA and EBV-encoded small RNA in situ hybridization. A quantitative analysis of IgG and IgG4-positive plasma cells (pPCs) in SANT was performed. The median densities of IgG-pPCs and IgG4-pPCs in SANT were approximately four-fold and 13-fold higher than those in the normal spleens, respectively. In addition, there was a statistically significant increase of IgG4/IgG-pPCs ratio in SANT in comparison to the control specimens. In conclusion, the fibrogenesis in a subset of SANT may be associated with EBV-infected myofibroblasts in an overlapping immune reaction indicated by the presence of infiltrating IgG4-pPCs. Further investigation is needed to elucidate the association between SANT and IgG4-related sclerosing disease.

CD30 and Epstein–Barr virus RNA expression in sclerosing angiomatoid nodular transformation of spleen

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.