Sclerosing Angiomatoid Nodular Transformation Research Articles

Sclerosing Angiomatoid Nodular Transformation (SANT) Involving Spleen: A Case Report

Background: Sclerosing angiomatoid nodular transformation (SANT) also known as multinodular haemangioma is a recently recognized, rare non-neoplastic vascular sclerosing mass-like lesion of the spleen with uncertain etiology, presents most commonly as an asymptomatic splenic mass found incidentally on imaging studies but may cause abdominal pain or discomfort, leading to splenectomy, as the risk of rupture and malignancy cannot securely be ruled out. Diagnosis is made postoperatively on histology. Case report: We report a case of 64-years old man, with incidentally detected splenic lesion on imaging who underwent elective splenectomy and was diagnosed postoperatively on histological examination. The post-operative course was uneventful and the patient was discharged on POD9. Conclusion: SANT of the spleen although rare, is to be considered as a differential diagnosis of splenic vascular lesions. Further research should focus on clinical and radiological characteristics and consideration of observation with serial imaging as an alternative approach. Open or laparoscopic splenectomy is still known to be curative as it is both therapeutic and diagnostic.

Sclerosing angiomatoid nodular transformation of spleen with calcifying fibrous pseudotumor: A rare case with unusual association

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen can be safely diagnosed by a core percutaneous ultrasound-guided biopsy.

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign lesion of the spleen. SANT cannot be distinguished from other benign or malignant splenic tumors based on imaging findings. So, diagnosis relies on histopathologic examination. Although splenectomy is frequently considered as an option, core needle biopsy tissue analysis is safe and accurate to avoid surgery.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: Radiological Findings and Radiological-pathological Correlation.

The objective of this study was to describe the CT and MRI features of sclerosing angiomatoid nodular transformation (SANT) of the spleen with pathologic correlation. Materials and Methods： Ten patients with surgically resected and pathologically confirmed SANTs were included. Clinical history was reviewed, and gross pathologic, histologic, and immunohistochemical findings were recorded. CT and MRI examinations were evaluated by two radiologists. Patients included seven men and three women, with a mean age of 42.9±16.7 years. Pathologic features of SANTs involved multiple angiomatous nodules in a radiating pattern with a central stellate fibrous scar and evidence of hemosiderin deposition. 9 cases showed a lobulated demarcated margin, 8 cases a slight hypoattenuating, 1 isoattenuating, and 1 case with two lesions demonstrated a slight hyperattenuating margin, respectively. Multiple scattered punctate calcifications were involved in 2 cases. 5 cases manifested hypointensity on in-phase imaging, 1 iso-intensity, and 4 iso-hypointensity on out-of-phase imaging. Progressive and centripetal enhancement were exhibited in 10 cases, spoke-wheel pattern in 3 cases, and nodular enhancement in 4 cases, respectively. The central fibrous scar was identified in 8 cases during delayed enhancement. Characteristics of SANTs on CT/MRI reflected the underlying pathology. Hypointensity on DWI and T2WI, and change of signal on T1 chemical-shift imaging were found to be due to hemosiderin deposition and fibrous tissue. Typical features were a solitary, round, lobulated mass with a fibrous scar. Progressive and centripetal enhancement, spoke-wheel pattern, nodular enhancement, and delayed enhancement of central fibrous scar were observed.

Sclerosing angiomatoid nodular transformation, a rare splenic tumor with increasing incidence.

SANT (sclerosing angiomatoid nodular transformation) tumor is a rare splenic tumor of unknown etiology and vascular lineage, first described in 2004. Most cases are asymptomatic, although cases of anemia or abdominal pain in association with growth have been described. Spontaneous ruptures have not been described. Radiologically it presents a radial pattern with centripetal filling in dynamic MRI, being a characteristic feature, but not pathognomonic. It may present hypermetabolism in PET-CT. Its incidence is increasing since its description as an independent clinical and histopathological entity, especially in the oncological patients follow-up. Due to its radiological resemblance to metastatic lesions and its growth despite being a vascular lesion, splenectomy is indicated following the principles of oncologic surgery until a definitive diagnosis is made. It presents a benign behavior, requiring neither treatment nor specific subsequent surveillance. Two diagnosed cases of SANT are presented, as well as a review of the clinical, radiological and histopathological characteristics of this little-known splenic lesion.

Conventional and contrast-enhanced ultrasound of scleroring angiomatoid nodular transformation of the spleen.

Sclerosing angiomatoid nodular transformation (SANT) is an uncommon non-tumorous disease of the spleen. The low morbidity and non-specific clinical symptoms of SANT might cause a misdiagnosis. The present study reported a case of a 31-year-old female with a SANT of the spleen. Findings on clinical manifestation and examinations, especially on contrast-enhanced ultrasound (CEUS), were carefully analyzed, and relevant literatures have also been reviewed.

Sclerosing angiomatoid nodular transformation of the spleen: multimodality imaging features and literature review

ObjectiveThe purpose of this study was to evaluate the CT and MRI findings, clinicopathologic features, and differential diagnosis of Sclerosing angiomatoid nodular transformation (SANT).Methods and materialsSeven men and seven women with pathological diagnoses of SANT were included in this retrospect study. Patients underwent at least one radiological examination before surgery. The number, shape, margin, size, attenuation, signal intensity, homogeneity, and enhancing pattern of the lesion were evaluated by two abdominal radiologists independently. Immunohistochemistry reports were available for 11 patients. The immunoreactivity to the vascular markers CD8, CD31, and CD34 was assessed.ResultsThe 14 SANT patients (7 men, 7 women; mean age, 43.5 years; age range, 24–56 years) presented with a single lesion and showed no specific clinical symptoms. Among 14 patients, 12 patients underwent MR scan, 5 patients underwent CT scan and 3 patients underwent PET-CT. On CT, all 5 lesions showed hypodensity on non-contrast images and spoke-wheel enhancing pattern after contrast administration, and calcification was observed. On T2WI, 10 cases(83.3%)showed hypointensity and 2 cases (16.7%) showed hyperintensity with central hypointensity. On T1WI, 10 cases (83.3%) were isointense and 2 cases (16.7%) were slightly hypointense. 10 cases (83.3%) showed hypointensity on DWI and 2 cases (16.7%) showed slightly hyperintensity on DWI. After contrast administration, all 12 lesions showed progressive enhancement. 18 F-fluorodeoxyglucose (FDG) uptake in the tumor was seen in all three cases that underwent PET-CT. The maximum standardized uptake value (SUVmax) was 4.5, 5.1, and 3.8 respectively.ResultsApart from the progressive spoke-wheel enhancing pattern, DWI and ADC findings will add value to the diagnosis of SANT.

A rare case of sclerosing angiomatoid nodular transformation of the spleen – a case report

Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion arising in the spleen. Although this lesion has characteristic radiological findings on both computerised tomography (CT) and magnetic resonance imaging (MRI) scans, it may pose a difficulty in distinguishing this rare benign entity from other lesions of the spleen both clinically and radiologically. We report a case of 46-year-old man with a splenic soft tissue lesion found incidentally on ultrasonography of the abdomen.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Diagnostic Conundrum.

A splenic lesion often represents a diagnostic challenge due to relative scarcity and the broad differential diagnosis. Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is usually encountered only incidentally. Although benign, patients with SANT often receive splenectomy, due to its rarity, diagnostic uncertainty and sometimes intimidating imaging morphology and size. Imaging features on computed tomography, magnetic resonance and positron emission tomography have a high diagnostic value for SANT and help differentiate this entity from other splenic lesions. When the imaging parameters are matched with core needle biopsy tissue analysis, further watchful waiting can be recommended in order to avoid splenectomy.

A rare case of sclerosing angiomatoid nodular transformation (SANT) of the spleen in a 13-year old girl

Sclerosing Angiomatoid Nodular Transformation (SANT) of spleen is a benign, rare, vascular disease. We report a case of 13-year old female with left upper abdominal pain, ultrasound revealing a hypoechoic lesion in spleen; laparoscopic splenic resection was performed. The definitive diagnosis of SANT was based on histopathological examination and immunohistochemistry.

Reappraisal of surgical decision-making in patients with splenic sclerosing angiomatoid nodular transformation: Case series and literature review.

BACKGROUNDMany clinicians and surgeons are unfamiliar with the sclerosing angiomatoid nodular transformation (SANT), which is gaining recognition as a benign splenic tumor. We challenge that SANT is rare and whether surgical intervention could be avoided through critical imaging review.AIMTo evaluate the incidence of SANT among splenic tumors and the decision-making process of SANT management.METHODSTwenty hospitalized patients who underwent splenectomy in 2018 and 2019 in a tertiary university hospital were retrospectively reviewed, and their data on imaging, diagnosis, surgical indications, and courses were recorded. All pathology results were confirmed by pathologist. Discriminative features differentiating SANT from other non-SANT splenic tumors were descriptively analyzed in this case series.RESULTSFourteen out of 20 patients who underwent splenectomy had splenic tumors, including 3 SANTs (21% splenic tumors), 6 non-SANT benign lesions (43%), 2 metastatic tumors, and 3 lymphomas. Hypointensity on T2-weighted magnetic resonance imaging (MRI), spoke wheel enhancing pattern in contrasted computed tomography or MRI, and cold spot (low fluorodeoxyglucose uptake) in positron emission tomography (PET) scan helped establish the diagnosis of SANT. Lymphoma, presenting with a hot spot on the PET scan were differentiated from SANT. Surgical indications were reformatted for splenic tumors. Splenectomy need not be performed in patients with typical imaging features of SANT.CONCLUSIONSANT is not a rare disease entity in clinical practice. Splenectomy should not be routinely indicated as the only management option for SANT with typical imaging features.

Clinicopathological features of sclerosing angiomatoid nodular transformation of the spleen

PurposeTo explore the clinicopathological features of sclerosing angiomatoid nodular transformation (SANT) of the spleen. MethodsThe clinicopathological data of 26 SANT patients were analyzed. ResultThere were 15 men and 11 women, aged 23–62 years (mean: 43.9 years; median: 43 years). Twenty patients were found during health check-ups. Magnetic resonance imaging had significantly higher specificity than other imaging modes in the diagnosis of SANT. Macroscopically, the lesions were gray–red and gray–white, along with well-demarcated nodules. Microscopy showed multiple angiomatoid nodules embedded in hyperplastic fibrous tissues and dense collagen fiber; the angiomatoid structures inside the nodules had varied morphology. Patchy and nodular fresh and old hemorrhages were observed in each lesion. Proliferative fibroblasts were seen in the stroma, along with infiltration of a few mixed inflammatory cells. Serum tumor markers were negative. Fourteen patients (53.8 %) had benign or malignant lesions in other parts of the body, including the liver, kidneys, and adrenal and pituitary glands which were similar to von Hippel–Lindau (VHL) syndrome. The reasons for occurrence of SANT may be as follows: hemangioma/lymphangioma or splenic congestion with extensive hemorrhage and secondary changes. ConclusionsSANT is a rare benign vascular lesion with some clinical manifestations similar to VHL syndrome. Patients have good prognosis after tumor removal.

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.

Sclerosing angiomatoid nodular transformation in a child

Abstract Sclerosing angiomatoid nodular transformation (SANT) is a spleen-specific benign mass-forming vascular lesion. Although there have been several reports of cases in the adult population, data in the pediatric population are exceedingly rare. Herein, we present a case of SANT with hypersplenism and investigate the unique features of this disease in pediatric patients. A 14-year-old girl visited a nearby physician with fatigue that had lasted for several months and was diagnosed with severe anemia and thrombocytopenia. Computed tomography revealed marked splenomegaly and a mass on the spleen. A malignant tumor could not be ruled out by blood and imaging tests, and splenectomy was therefore performed with laparotomy. Histopathologically, hemangiomatous nodules with a mixture of multiple vascular components were found, resulting in a diagnosis of SANT. The patient recovered and experienced no recurrence of her previous symptoms in the 18 months following the operation. Accumulation of further cases is desired to improve diagnosis and treatment for children with this extremely rare condition.

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 of the Spleen: Analysis of Clinical and Pathological Features in Five Cases.

Objective: We aimed to summarize the clinical and pathological features of sclerosing angiomatoid nodular transformation (SANT) in spleen among five cases.Methods: Five cases (male: 3; female: 2; mean age: 47.6 years) with SANT confirmed by pathological analysis between July 2010 and November 2019 in our hospital were included in this study. The clinical, imaging, and pathological data were analyzed retrospectively.Results: Three patients presented with mild abdominal pain or discomfort, while the other two were symptom free. Two patients received ultrasonography (US), and all patients underwent a computerized tomography (CT) scan in our hospital. The typical “spoke wheel” pattern was seen in two cases, and central calcification was detected in one case on the CT scans. All patients indicated peripheral enhancement around the SANT lesion during the arterial phase. Open or laparoscopic splenectomy was performed for treatment. No patient showed recurrence in the follow-up. The pathological characteristics of our cases were in line with those of previous literatures.Conclusions: Peripheral enhancement around the SANT lesion during the arterial phase should be taken into consideration for the diagnosis of SANT as an imaging sign on CT scans. Special attention should be paid to the splenic integrality during the laparoscopic approach, due to the probability of malignancy and the fragility of the spleen.

CT and MRI features of sclerosing angiomatoid nodular transformation of the spleen: A report of 18 patients with pathologic correlation

PurposeThe purpose of this study was to describe the computed tomography (CT) and magnetic resonance imaging (MRI) features of sclerosing angiomatoid nodular transformation (SANT) of the spleen and correlate imaging features with those obtained at histopathologic analysis. Materials and methodsA total of 18 patients (9 men, 9 women; mean age, 42.2±10.7 [standard deviation (SD)] years; range, 23–59 years) with histopathologically confirmed SANT were retrospectively evaluated. The presenting symptoms, gross pathologic changes, and histopathologic and correlative immunohistochemical results were recorded. CT (n=8) and MRI (n=12) features were analyzed by two radiologists and included number, size, shape, boundary, attenuation, signal intensity, and enhancement patterns. ResultsSeventeen patients (17/18; 94%) had a single SANT without specific clinical symptoms and one patient (1/18; 6%) had multiple SANTs with left-upper-quadrant bloating and pain. The largest lesion diameter exceeded 3cm. On plain CT images, SANTs were slightly hypoattenuating in seven patients (7/8; 88%), isoattenuating in one patient (1/8; 13%), and contained calcification in two patients (2/8; 25%). On T2-weighted MR images, SANTs displayed hypointensity in ten patients (10/12; 83.3%), isointensity in one patient (1/12; 8%) and hyperintensity in one patient (1/12; 8%). On T2-weighted images, stellate or scattered fibrous scars were observed in all patients (12/12; 100%). On diffusion-weighted images, SANTs appeared as heterogenous or homogeneous hypointense in 12 patients (12/12; 100%). Compared to out-of-phase images, SANTs displayed decreased local signal intensity on in-phase images in 12 patients (12/12; 100%). On enhanced CT and MRI images, SANTs had clear boundaries (17/18; 94%), oval (7/18; 39%) or lobular (7/18; 39%) shape, displayed progressive centripetal enhancement (18/18; 100%), spoke-wheel pattern (14/18; 78%), nodular enhancement (11/18; 61%), or delayed enhancement of central fibrous scar (9/18; 50%). ConclusionsSANT of the spleen predominantly manifests as a solid, single, oval or lobular, and well-defined lesion with a fibrous scar and occasional calcification. Typical enhancement characteristics include progressive and centripetal enhancement, spoke-wheel pattern, nodular enhancement, and delayed enhancement of central fibrous scar. Hypointensity on T2- and diffusion-weighted images are due to hemosiderin deposition and fibrous tissue.

Splenic Primary Solid Tumors : Does a Preoperative Histopathology Diagnosis Really Matter?

The present study aims to present a case series of patients who underwent splenectomy for splenic primary solid tumors without preoperative histopathologic diagnosis. From 2013 to 2019, 12 patients underwent splenectomy for solid primary splenic tumors at 3 different academic medical centers. All electronic medical records were retrospectively reviewed. Seven (58.3%) patients were women, and 5 (41.6%) were male. The median age was 48 years (range: 25-72 years). In 8 (66.6%) patients, a conventional approach was performed. In 2 (16.6%), a hand-assisted laparoscopic surgery procedure was completed, and in other 2 (16.6%) patients, a laparoscopic approach was auspiciously achieved. Median operative time was 135 minutes (range: 60-210 minutes), and median blood loss was 500 mL (range: 200-1500 mL). Procedure-related morbidity was found in 2 (16.6%) patients, and the mortality rate was 0%. The final histopathologic diagnosis was lymphoma in 5 (41.6%) patients, lymphangioma in 3 (25%) patients, hamartoma in 2 (16.6%) patients, angiosarcoma, and sclerosing angiomatoid nodular transformation (SANT) in 1 (8.3%) case each. Splenectomy should be the treatment of choice when encountering a primary splenic tumor without the need for preoperative fine-needle aspiration biopsy, avoiding the complications this technique entails.

Treatment options for sclerosing angiomatoid nodular transformation of spleen

BackgroundTo summarise the clinical features of Sclerosing angiomatoid nodular transformation (SANT) of the spleen and to compare the efficacy of three different surgical treatments. MethodsWe performed a retrospective analysis of patients with SANT of spleen treated at our center from 2009 to 2018. We compared the efficacy and safety of three different types of surgical procedures. ANOVA and the chi-square test were used for statistical analysis. ResultsA total of 37 patients were included. Most (35/37; 94.6%) were asymptomatic. A number presented as obscure boundary lesions such that malignancy could not be excluded. Open splenectomy was performed for 12 patients, laparoscopic splenectomy for 12 patients and laparoscopic partial splenectomy for 13 patients. Operation time (P = 0.355), blood loss (P = 0.135), length of hospital stay after operation (P = 0.271) and postoperative complications (P = 0.502) were comparable between the three groups. Duration of drainage tube placement was significantly longer in laparoscopic partial splenectomy patients (P = 0.006). Peak platelet count after operation was significantly lower in laparoscopic partial splenectomy patients (P < 0.001). ConclusionLaparoscopic partial splenectomy appears to be a technically feasible and therapeutically effective approach for SANT.

A rant about a SANT: A rare mimic of splenic haemangioma

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare, benign, vascular lesion with characteristic histological features. Often an incidental finding, full excision of the lesion has been shown to be curative. We describe a case of SANT in a 35-year-old woman who presented with a purported splenic haemangioma with atypical imaging features including an uncharacteristic enlargement over a two-year period. Gross examination of the splenectomy specimen revealed a circumscribed 42 mm white, lobulated mass with prominent central sclerosis and dilated vascular spaces at the periphery. Histological examination showed a hyalinised lesion with a nodular architecture imparted by nodules of small capillary channels with red cell extravasation, and scattered admixed chronic inflammatory cells. A rare lesion of indeterminate aetiology, SANT remains a curiosity and an essential diagnostic consideration among vascular lesions of the spleen with features not typical of haemangioma.