Sclerosing angiomatoid nodular transformation (SANT) of the spleen: review of the literature / Nodular angiomatoide esclerosante transformación (SANT) de la bazo: revisión de la literatura

We present a sclerosing angiomatoid nodular transformation (SANT) case report in a 60 year-old-woman. SANT is an extremely rare benign disease of the spleen that it is radiologically similar to malignant tumors, and clinically difficult to differentiate from other splenic diseases. Splenectomy is both diagnostic and therapeutic in symptomatic cases. The analysis of the resected spleen is necessary to achieve the final diagnosis of SANT.(AU)

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

OBJECTIVE: The 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 MATERIALS: Seven 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. RESULTS: The 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. RESULTS: Apart from the progressive spoke-wheel enhancing pattern, DWI and ADC findings will add value to the diagnosis of SANT.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen: review of the literature.

We present a sclerosing angiomatoid nodular transformation (SANT) case report in a 60 year-old-woman. SANT is an extremely rare benign disease of the spleen that it is radiologically similar to malignant tumors, and clinically difficult to differentiate from other splenic diseases. Splenectomy is both diagnostic and therapeutic in symptomatic cases. The analysis of the resected spleen is necessary to achieve the final diagnosis of SANT.

Angiomatoid fibrous histiocytoma in the spinal canal of T3-T4: a case report and literature review.

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumour that occurs in the superficial tissue of extremities of children and young adults. A painless mass in the deep dermis and subcutaneous tissue is the main clinical manifestation. AFH also occurs infrequently in the central nervous system and is relatively common in the cranium. However, spinal canal AFH has not been described yet. We report a rare case of AFH in the cervical canal of a 20-year-old male patient. Microsurgical gross total resection of the tumour was performed, and the diagnosis was confirmed by postoperative pathology. To our knowledge, this is the first case of AFH in the spinal canal.

Dermoscopy of haemosiderotic/aneurysmal dermatofibroma: A morphological study of 110 cases.

BACKGROUND: Haemosiderotic and aneurysmal dermatofibromas are uncommon and frequently misdiagnosed lesions, which can be considered as different histopathological stages of the same tumour. A dermoscopic diagnosis testing accuracy has not been performed for these tumours to date. OBJECTIVES: To determine the diagnostic significance of dermoscopic structures and patterns associated with haemosiderotic/ aneurysmal dermatofibromas in a large series. METHODS: Dermoscopic images of histopathologically proven cases of 110 haemosiderotic/ aneurysmal dermatofibromas and 501 other tumours were collected. The frequency, sensitivity, specificity, positive predictive value and negative predictive value of the dermoscopic structures and patterns associated with these lesions were calculated. RESULTS: Haemosiderotic/ aneurysmal dermatofibromas are mostly symmetric lesions (86.5%), and a prominent homogeneous area was present in 100% of them. The presence of vascular structures was very common (86.4%), and dotted vessels were predominant (58.2%). Shiny white structures were seen in 85.5% of lesions, while a peripheral delicate pigment network was present in 69.1%. The most significant pattern was the one composed of a prominent homogeneous area and peripheral delicate pigment network, which showed a specificity of 100% with a relatively good sensitivity (69.1%). All the patterns containing a peripheral delicate pigment network showed very good specificities, positive predictive values and negative predictive values. Those patterns without a peripheral delicate pigment network showed the highest sensitivities, but they showed a significant overlap with other tumours, mainly with melanoma. CONCLUSIONS: Dermoscopy is helpful in improving the diagnostic accuracy of haemosiderotic/ aneurysmal dermatofibromas. However, there is a considerable dermoscopic overlap between these tumours and melanoma, specifically when the peripheral delicate pigment network is absent.

Benign fibrous histiocytoma of the oral and maxillofacial region: A systematic review.

OBJECTIVE: The aim of the present study was to carry out a systematic review of available data regarding case reports and case series of oral and maxillofacial benign fibrous histiocytoma (BFH). STUDY DESIGN: A search strategy was performed using the PubMed, Web of Science, Scopus, and EMBASE electronic databases. RESULTS: Male individuals (56.89%) were more affected, with a mean age of 34.55 ± 20 years. The buccal mucosa (20.33%) represented the most common site, with the clinical presentation of a painless (86.95%) nodule (98.03%). The clinical hypothesis of a fibroma was reported in most cases (31.57%). Radiographic presentation of intraosseous lesions showed multilocular radiolucent images (55.55%). Regarding the histopathologic features, the biphasic population of fibroblastic and histiocytic cells was seen in 21 cases (39.62%), and a spindle-shaped fibroblastic cell population organized into a storiform pattern was observed in 25 cases (47.16%). CD68 (n = 26) and vimentin (n = 25) showed immunoreactivity in all BFH cases in which they were used. All cases were treated with a surgical resection, and 8.10% recurred. CONCLUSIONS: The current systematic review demonstrated that BFH represents a rare lesion that mainly affects the buccal mucosa of male individuals, and the treatment is mainly surgical with a good prognosis.

Sclerosing angiomatoid nodular transformation of the spleen: a case report.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is an uncommon primary benign condition with specific histopathological characteristics. The majority of the patients are usually asymptomatic and present for treatment after an incidental finding on a routine abdominal imaging. The differential diagnosis includes other benign tumours, as well as primary malignancy or metastases of unknown origin. We report a 55-year-old woman who was presented to the surgical team of our hospital for splenectomy after the investigation of an incidental splenic lesion led to the diagnosis of SANT. The pathology report of the specimen repeated the initial diagnosis. The patient has not yet presented any complication or recurrence of the disease 8 months after the operation. From such infrequent cases, the most valuable conclusion which can be yielded is that the more the number of patients reported, the more the knowledge about the disease is broadened.

Dermoscopy of dermatofibromas: A new perspective.

AIM: In dermoscopic studies on dermatofibromas, some publications divide the appearance of lesions into standardised patterns, and some publications classify the clinical appearance of the lesions by comparing them with existing dermatological lesions. This study aims to re-evaluate the dermoscopic findings and patterns of dermatofibromas from a different perspective. MATERIALS AND METHODS: In this study, 142 lesions of 72 patients were evaluated dermoscopically and their patterns were schematised. RESULTS: In our study, a total of 15 patterns consisting of main and sub-patterns were created. The most common patterns we detected were pattern 1 (1a: 13.4%, 1b: 8.5%), pattern 8 (8a: 10.6%, 8b: 4.2%) and pattern 2 (2a: 9.2%, 2b: 4.2%), respectively. CONCLUSION: Patterns of DFs were reclassified while preserving basic patterns. We think that the new sub-patterns and schematisation with this study can contribute to a better understanding of DFs.

Clinicopathological features of sclerosing angiomatoid nodular transformation of the spleen.

PURPOSE: To explore the clinicopathological features of sclerosing angiomatoid nodular transformation (SANT) of the spleen. METHODS: The clinicopathological data of 26 SANT patients were analyzed. RESULT: There 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. CONCLUSIONS: SANT is a rare benign vascular lesion with some clinical manifestations similar to VHL syndrome. Patients have good prognosis after tumor removal.

Can ultrasound distinguish between dermatofibroma and subcutaneous epidermal tumors? - Imaging features and reproducibility.

BACKGROUND: We evaluated ultrasound (US) features of dermatofibroma and epidermal tumor (ET). We also evaluated the reproducibility of each US findings. MATERIALS AND METHODS: We retrospectively assessed US findings of 30 surgically confirmed dermatofibromas and 44 ETs, measuring the lesion (i.e., the depth and the length of the lesion) and calculating the depth to length ratio (DLR). We then determined the sharpness and the shape of the lesion. Finally, we assessed whether the lesion appeared with a "more-than-half sign" (involving more than 50% of the dermal layer) or a "submarine sign" (focal projection to skin). We used Pearson's chi-squared test to analyze the US findings (i.e., presence of more-than-half sign or submarine sign and margin sharpness) between dermatofibroma and ET. Inter-observer agreement of the image findings was assessed using kappa statistics. RESULTS: The submarine sign and margin sharpness of ET lesions and the more-than-half sign and submarine sign in dermatofibroma, respectively, showed almost perfect agreement. Also, the more-than-half sign and shape of the lesion in ET and the margin sharpness and shape of the lesion in dermatofibroma showed substantial agreement. The measurements of DLRs showed excellent reproducibility. There were no significant differences of DLRs between two groups (p values = 0.512, 0.671, respectively). The more-than-half sign was observed more frequently in the context of dermatofibroma to a statistically significant degree (p < 0.001), while the submarine sign was statistically more common in relation to ET together with greater margin sharpness (p = 0.021 and <0.001, respectively). CONCLUSIONS: More-than-half signs are common in dermatofibroma while submarine sign and sharp margin are common in ET. Each finding showed high reproducibility and can be useful as a sonographic marker for differentiation between ET and dermatofibroma.

A rare case of atrophic dermatofibroma with dermoscopic findings.

BACKGROUND: Dermatofibroma, also known as cutaneous benign fibrous histiocytoma, is a common skin tumour. AIM: The aim of this paper was to present a rare variant of dermatofibroma, atrophic dermatofibroma, emphasizing histopathological and dermoscopic features. PATIENTS/METHODS: A case of atrophic dermatofibroma in a female patient with the characteristic histopathological features and newly demonstrated dermoscopic findings is presented. RESULTS: A 54-year-old female presented with a depressed reddish lesion on the back showing histopathological findings of atrophic dermatofibroma. The dermoscopy of the lesion revealed a peripheral pigment network surrounding a pink-reddish colouration around a central whitish scar-like patch with white-yellow scales which was not an exact match with the description in the literature. CONCLUSION: Atrophic dermatofibroma is a rare variant that presents as an atrophic, depressed skin lesion which can easily be overlooked. Atrophic dermatofibroma should be considered in the differential diagnoses of atrophic, depressed lesions on the upper body of middle-aged women. The case of atrophic dermatofibroma presented here showed typical histopathologic findings with atypical dermoscopic features.

Primary intracranial angiomatoid fibrous histiocytoma: a case report and literature review.

Angiomatoid fibrous histiocytoma (AFH) is a tumour primarily occurring in the extremities which can very rarely occur as an isolated intracranial lesion. We report a case of a 22-year-old woman presenting with generalized seizure and visual field deficit due to an occipital mass, which immunohistochemistry showed to be an AFH.

Endoscopic Resection of a T-Shaped Benign Fibrous Histiocytoma at the Skull Base in a Pediatric Patient.

BACKGROUND: Benign fibrous histiocytoma (BFH) is a benign tumor composed of cells with characteristics of histiocytes and with fibroblastic components. BFHs are rare lesions, especially in the skull base. CLINICAL PRESENTATION: Here, the authors report the case of a 9-year-old girl presented with reduced binocular vision for 2 months. The computerized tomography (CT) of the tumor appearance was high-density annular sclerosis. The tumor was removed via nasal endoscopic approach. Final pathologic diagnosis was benign fibrous histiocytoma. The post-operative period was uneventful after 8 months, and the visual acuity was improved to some extent. DISCUSSION AND CONCLUSION: This is the first case of BFH in a pediatric patient that was removed by endoscope. For similar case, endoscopic resection might be the first choice, including pediatric patient. Moreover, the CT feature and literature review may provide further insight into the diagnosis and management.