Rare Benign Vascular Lesion Research Articles

Sclerosing angiomatoid nodular transformation of the spleen (SANT) in a patient with clear cell carcinoma of the uterus: a case report

BackgroundSclerosing angiomatoid nodular transformation of the spleen is a very rare benign vascular lesion recently described. Usually, sclerosing angiomatoid nodular transformation of the spleen is an incidental finding; the association with malignant tumors is extremely rare. To the best of our knowledge, we report the first case of sclerosing angiomatoid nodular transformation of the spleen associated with uterine clear cell carcinoma.Case presentationA 49-year-old Arabic woman presented to our institute with abdominal pain and distention. An abdominal computed tomographic scan was obtained, which showed a 14-cm uterine malignant tumor and a 4-cm isolated splenic nodule suggesting a metastatic lesion. The tumor was limited to the uterus but did not extend beyond. The patient underwent surgical treatment, and the histopathological examination of the resected uterine and splenic specimens disclosed invasive uterine clear cell carcinoma and sclerosing angiomatoid nodular transformation of the spleen, respectively. The patient had no signs of the disease 17 months after surgical treatment.ConclusionsSclerosing angiomatoid nodular transformation of the spleen is a very rare benign disease with a misleading presentation when associated with a malignant tumor. Pathological assessment of the resected spleen is the only way to achieve the correct diagnosis.

Splenic hamartoma with bizarre stromal cells: a case report and literature review

BackgroundSplenic hamartoma is a rare benign vascular proliferative lesion composed of unorganized sinusoid-like channels lined with plump or flat endothelial cells and characterized by a CD8-positive immunophenotype of the lining cells. Scattered bizarre stromal cells can be found in some splenic hamartomas. The presence of splenic hamartoma with bizarre stromal cells is extremely rare and these bizarre cells make it possible to be regarded as a malignancy. Recognition of this rare histologic variant will help to avoid diagnostic confusion and overtreatment of this benign entity.Case presentationWe report a case of a 40-year-old man with occasional left-sided waist back pain. A splenic space-occupying lesion was detected by ultrasound and magnetic resonance imaging. Microscopically bizarre large cells were scattered throughout the splenic hamartoma. The cells exhibited atypical nuclei, scarcely visible cytoplasm, and vesicular chromatin, and they did not form expansile clusters and lacked mitotic activity. An immunohistochemical panel was performed. The bizarre cells strongly expressed vimentin, and the Ki-67 index was very low. The lesion was diagnosed as a splenic hamartoma with bizarre stromal cells.ConclusionsTo the best of our knowledge, this is the first systematic review on a splenic hamartoma with bizarre stromal cells; only six cases have been described in the literature. Proper identification is important to secure adequate treatment.

Sclerosing Angiomatoid Nodular Transformation: Laparoscopic Splenectomy as Therapeutic and Diagnostic Approach at the Same Time

Introduction Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion with unknown etiopathogenesis and with definite features of imaging, histopathology, and immunohistochemistry. It was first described by Martel et al. in 2004, and to date, only 151 cases have been reported. Case Description We report a case of SANT of the spleen detected in a 66-year-old Caucasian, without comorbidities, presented to our department with epigastric pain. We, also, presented a review of the literature. Conclusions SANT is a benign incidentally vascular condition in the majority of cases. The wide age and gender distribution in our review is in accordance with that in previous studies in English literature. In our opinion, splenectomy is the choice treatment because it is at the same time diagnostic and therapeutic in a definitive way.

Gamma Knife surgery for a hemangioma of the cavernous sinus in an adult: Case report and short review of the literature

Cavernous sinus hemangiomas (CSH) are rare benign extra-axial vascular lesions. Both radiological and clinical aspects are important, for deciding a therapeutic modality, including medical treatment, radiation therapy or microsurgery. In the particular case of CSH, a radical removal of the tumor often cannot be achieved and is associated with a considerable risk for intraoperative uncontrollable bleeding. An alternative treatment modality is radiosurgery. Here we report the case of a 45-year-old patient, who presented with diplopia due to left abducens nerve palsy. A left-sided cavernous sinus lesion was found, initially considered to be a meningioma. However, after serial MR acquisitions, a progressive and heterogeneous enhancement was observed. In order to clarify the diagnosis between meningioma and hemangioma, a diagnostic Tc-99m labeled red blood cells (RBC) scintigraphy (Tc-99m RBC scintigraphy) was performed and showed a typical perfusion blood pool mismatch, with accumulation of the RBC at the level of the left CS, which is typical for a hemangioma. The patient underwent Gamma Knife surgery. The CSH showed a significant reduction in size starting 6 months after treatment and a full regression of the left abducens nerve palsy was observed within 1 year. These clinical and radiological results persisted over the next 3 years.

A RARE CASE OF ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILIA IN ORAL MUCOSA

The angiolymphoid hyperplasia with eosinophilia (ALHE) is considered a rare benign vascular lesion that mainly affects the skin and subcutaneous tissue of the head and neck, but is uncommon in the oral cavity. Its etiology remains unclear and it is described as a reactive vascular proliferation, vascular malformation, or neoplasia. Kimura’s disease is its main differential diagnosis. Here, we report a rare case of ALHE present on the lip of a 50-year-old male patient that exhibited size of approximately 3 cm with 7 years of evolution. After excisional biopsy, the histopathologic examination showed a well-encapsulated and multilobulated lesion with proliferation of blood capillaries that showed endothelial cells of epithelioid appearance, diffuse inflammatory infiltration with lymphocytes, plasma cells, numerous eosinophils, and presence of lymphoid follicles were also found. Immunohistochemical analysis revealed positivity for markers CD34, Bcl-2, and Ki-67 that, together with morphologic examination, support the diagnosis of ALHE.

Sclerosing angiomatoid nodular transformation of the spleen: A case report and literature review.

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign splenic vascular lesion. Since it was first defined in 2004, a total of 132 cases of SANT have been reported in ~50 studies in the English literature. However, it remains difficult to form a definitive pre-operative differential diagnosis of SANT compared with other splenic tumors or malignant lesions. The present study reports a pathologically proven case of SANT in a 29-year-old man who initially presented with left upper quadrant and back discomfort. The study also provides a review of the current knowledge on the condition, including the clinical profile, imaging features, cytological features, differential diagnosis and treatment of SANT. The most important distinguishing features of SANT are its typical vascular character and lack of other features that are typical of a granuloma. A splenectomy is required and the diagnosis is based on pathological analysis.

Oral Intravascular Papillary Endothelial Hyperplasia Associated with an Organizing Thrombus: Case Report and Immunohistochemical Analysis.

Intravascular papillary endothelial hyperplasia (IPEH) is a benign lesion of the skin and mucosa of vascular origin characterized by reactive proliferation of endothelial cells. A 76-year-old woman was referred presenting a painless nodule on the lip. Intraoral examination revealed bluish submucosal nodular proliferation, measuring 10 × 5 × 5 mm, affecting the lower labial mucosa. The lesion had a firm consistency and it was not fixed to the adjacent tissues. The main differential diagnoses were mucocele/mucus retention cyst, sialolith, or salivary gland neoplasia. An incisional biopsy was performed and during the intraoperative procedure an encapsulated red-bluish nodular mass was observed. Microscopic analysis revealed papillary endothelial proliferation in the center of the lesion and fibrin admixed with inflammatory cells in organization peripherally. There was no nuclear atypia, mitotic figures, or necrosis. The endothelial cells were CD34 positive, with low Ki-67 proliferation index (4%). α-SMA highlighted the vessel walls, whereas negativity for D2-40 excluded lymphatic origin. Final diagnosis was IPEH associated with an organizing thrombus. Dentists should be aware about this rare benign vascular lesion, whose final diagnosis is achieved only after histopathology analysis. Surgical removal is the treatment of choice and no recurrence is expected.

Epithelioid Hemangioma (Angiolymphoid Hyperplasia With Eosinophilia) of the Heart With Peripheral Eosinophilia and Nephrotic Syndrome

Epithelioid hemangioma (EH) is a rare benign vascular lesion, characterized by endothelial cells with epithelioid/histiocytoid appearance. Heart involvement is extremely rare. We present an unusual case of cardiac EH in a young woman with supraventricular arrhythmia, nephrotic syndrome (membranous glomerulopathy), and peripheral eosinophilia after a pregnancy and normal partum resembling Kimura disease. Echocardiogram showed a large tumor mass in the right cardiac ventricle. The cardiac tumor was removed and the histopathology revealed an endothelial proliferation associated with abundant eosinophils. The neoplastic endothelial cells were eosinophilic and polygonal with epithelioid/histiocytoid morphology. Lymphoid nodules were occasionally seen. The neoplastic cells were positive for CD34, CD31, ERG, and factor VIII and negative for CK. A diagnosis of EH was rendered. The patient was alive and well after surgical resection. EH and Kimura disease represent separate entities, but clinical and/or histological overlapping can be observed. Epithelioid/histiocytoid endothelial cells constitute the hallmark feature that favors a final diagnosis of EH.

Surgical management, outcomes, and recurrence rate of orbital lymphangiomas

Orbital lymphangiomas are rare benign vascular lesions of the orbit. Due to their intimate relationship to the orbital contents, gross total resection can be difficult, resulting in a relatively high recurrence rate. Between May 2002 and June 2013, eight patients (five male, three female) underwent surgical resection for orbital lymphangiomas at our institution. Of the seven patients with follow-up, six experienced improvement in presenting symptoms at a mean of 5.3years (range 1–23years). Recurrence was seen in five of the seven patients (71.4%). Recurrence developed at a mean of 7.2years (range 1–23years) after the initial procedure. New or worsening cranial nerve deficits were seen postoperatively in three of eight patients (37.5%) with follow-up. All postoperative deficits were resolved at last follow-up. Orbital lymphangiomas are challenging surgical lesions in which gross total resection is frequently not possible. Subtotal resection is safe and effective for symptomatic relief.

Laproscopic partial splenectomy : A rare surgery for unusual diagnosis

Laproscopic partial splenectomy is a safe, minimally invasive technique for the treatment of solitary space occupying lesion (SOL) in splenic pole. Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion, first described in the literature under this name in 2004. We report a case of upper pole splenic SOL diagnosed as SANT on HPE successfully managed by Laparoscopic partial splenectomy for the first time at INHS Asvini. The patient presented with pain left hypochondrium of 02 year duration. Ultrasonography (USG) and Contrast enhanced Computerized tomography (CECT) abdomen revealed a splenic mass in upper pole. The laproscopic partial splenectomy has proved to be both diagnostic and therapeutic.

Angiomatosis of breast - an extremely rare benign vascular lesion of the breast: a case report

Angiomatosis of the breast extremely rare benign vascular tumor. Very few cases reported in the literature. Complete excision and examination is required for the diagnosis of any benign vascular lesion in the breast. If the diagnosis of a benign vascular lesion is made on a partial excision, it must be stated to be contingent upon complete excision. These tumors are prone to local recurrence. Complete excision or simple mastectomy is the treatment of choice.

Sclerosing angiomatoid nodular transformation of spleen (SANT) – case report and literature review

Introduction Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of spleen of unknown aetiology. Case history A 74-year-old female presented with a 6 month history of iron deficiency anaemia and raised C-reactive protein. A full body CT scan showed an exophytic mass involving the anterior pole of the spleen measuring up to 6 cm in maximum dimension. No other abnormalities or relevant past history was noted. The clinical differential diagnoses were lymphoma or metastatic tumour, and thus a splenectomy was performed. Macroscopically, the spleen showed a well-demarcated tumour with red-brown nodules and central speckled area of fibrosis. The histology revealed a lesion composed of complex slit- like vascular spaces interspersed with spindle to ovoid cells and numerous inflammatory cells. On immunohistochemistry, all the nodules expressed CD31, CD34, SMA and CD30. EBER and IgG4 stains were negative. The lesion was finally diagnosed as a SANT. Discussion SANT is thought to be a reactive process rather than a true neoplasm. They may represent the final common pathway of a variety of splenic lesions including inflammatory pseudotumours. SANT may be related to IgG4 disease or EBV virus. They show upregulation of CD30 with respect to normal spleen. Splenectomy is usually the standard curative treatment.

Diffuse cavernous hemangioma of the rectum: magnetic resonance imaging features: a case report

Diffuse cavernous hemangioma of the rectum: magnetic resonance imaging features: a case report Diffuse cavernous hemangioma of the rectum (DCHR) is a rare benign vascular lesion usually seen in young adults. The presenting symptom is painless recurrent rectal bleeding. Herein, we report a 5 yearold male patient with a history of DCHR. He had been diagnosed with DCHR at his birth. The definite diagnosis was made on the basis of biopsy wich was performed during colonoscopic examination. The patient underwent magnetic resonance imaging (MRI) examination which revealed characteristic signal intensity features of the tumor and also determined the exact location and extent of the lesion. The tumor had only minimally progressed in size compared to the last MRI examination. Currently, the patient is on medical treatment and being followed up by MRI. DCHRs are rarely encountered and often misdiagnosed because they can mimic internal hemorrhoids, inflammatory bowel disease, carcinoma and various other pathological conditions. In conclusion, we can emphasize that despite its rarity, DCHR should be considered in the differential diagnosis of young patients presenting with

Epithelioid Hemangioma of Lingual Alveolar Mucosa: An Immunohistochemical Case Report

Epithelioid hemangioma is a rare benign vascular lesion that presents as a nodular lesion in the skin of head and neck region. It is a superficial vascular entity which can either be due to tumor or reactive lesion, but the exact etiology is still unknown. We hereby present a rare case which has been reported with the history of small nodular-like growth on mandibular buccal and lingual area. The excisional biopsy was performed and tissue was submitted for histopathological diagnosis. The immunohistochemistry was performed to check the expression of CD31 marker which proved that origin of epithelioid cells was vascular.

An unusual case of sclerosing angiomatoid nodular transformation: radiological and histopathological analyses

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign primary vascular lesion of the spleen. Its etiology is still debated. Radiological characteristics are less known, although there are some reports regarding histopathological features ofSANT. A 21-year-old male patient was admitted to our hospital with fatigue, weight loss, and abdominal pain for 4 months. Physical examination, complete blood count, andbiochemical parameters were unremarkable. Dynamic contrast-enhanced computed tomography (CT) of the abdomen was performed. A heterogeneous well-defined hyperdense nodular lesion 3 cm in diameter was detected during the arterial phase. The detected lesion was seen as isodense with the spleen parenchyma during the portal venous and late venous phases. Magnetic resonance imaging (MRI) showed an isointense-hypointense nodular lesion on T1- and T2-weighted images. Intraabdominal LAM or splenomegaly was not detected. Microscopically, it was composed of angiomatoid nodules separated by central stellate fibrous stroma and fibrous septa. The contrast enhancement pattern was described as centrally hypovascular, radially progressive centripetal vascular contrast enhancement, called a spoke-wheel pattern in previously reported cases. We present CT and MRI findings and their correlation with histopathological findings of a case of unusual symptomatic SANT.

Orbital Lymphangiomas

Orbital lymphangiomas are rare benign vascular lesions of the orbit. Due to their intimate relationship to the orbital contents, gross total resection can be difficult, resulting in a relatively high recurrence rate. Between May 2002 and June 2013, eight patients (five male, three female) underwent surgical resection for orbital lymphangiomas at our institution. Of the seven patients with follow-up, six experienced improvement in presenting symptoms at a mean of 5.3 years (range 1–23 years). Recurrence was seen in five of the seven patients (71.4%). Recurrence developed at a mean of 7.2 years (range 1–23 years) after the initial procedure. New or worsening cranial nerve deficits were seen postoperatively in three of eight patients (37.5%) with follow-up. All postoperative deficits were resolved at last follow-up. Orbital lymphangiomas are challenging surgical lesions in which gross total resection is frequently not possible. Subtotal resection is safe and effective for symptomatic relief.

Myometrial cavernous hemangioma with pulmonary thromboembolism in a post-partum woman: a case report and review of the literature

IntroductionCavernous hemangiomas of the uterus are rare benign vascular lesions. Nine cases of diffuse cavernous hemangioma of the gravid uterus have been reported, most of which diffusely involved the myometrium. These vascular malformations are clinically significant, and may cause pronounced bleeding resulting in maternal or fetal demise. Thrombosis of cavernous hemangiomas of the uterus has been previously reported. We here report the first case in which a thrombosed cavernous hemangioma of the myometrium resulted in a fatal pulmonary embolism in a post-partum woman.Case presentationA 25-year-old obese African-American woman who had one pregnancy and was delivered of twins by cesarean section was admitted 1 week after the successful delivery. The 12-day clinical course included ventilator-dependent respiratory failure, systemic hypertension, methicillin-resistant Staphylococcus aureus in the sputum, leukocytosis and asystole. A transabdominal ultrasound examination showed heterogeneous thickened and irregular products in the endometrial canal. The laboratory values were relevant for an increased prothrombin time, activated partial thromboplastin time, ferritin and a decrease in hemoglobin. The clinical cause of death was cited as acute respiratory distress syndrome. At autopsy, a 400g spongy, hemorrhagic uterus with multiple cystic spaces measuring approximately 0.5 × 0.4cm filled with thrombi within the myometrium was identified. Immunohistological examination with a CD31 stain for vascular endothelium associated antigen confirmed several endothelium-lined vessels, some of which contained thrombi. These histological features were consistent with cavernous hemangioma of the myometrium. A histological examination of the lungs revealed multiple fresh thromboemboli in small- and medium-sized pulmonary arteries in the right upper and lower lobes without organization, but with adjacent areas of fresh hemorrhagic infarction.ConclusionThis case underscores the importance of a high index of suspicion in a pregnant or post-partum woman presenting with respiratory symptoms. Thrombosis of the cavernous hemangiomas of the gravid or post-partum uterus is a rare entity. This case is of interest because it indicates that this condition can be fatally complicated by embolization of the thrombi in the cavernous myometrial hemangiomas. Although delivery by conservative methods, as well as cesarean section, is possible without resorting to hysterectomy, occasionally, the consequences could be fatal as in this case.

Testicular Capillary Hemangioma Presenting as an Incidental Contralateral Lesion in a Child With Cryptorchidism

Testicular capillary hemangioma is a rare benign vascular lesion. Appropriate recognition is paramount to ensuring testicular preservation. The authors present a case with an atypical presentation along with a review of the literature. Classic epidemiologic, clinical, and pathologic characteristics, as well as recommended surgical intervention are provided.

Sclerosing angiomatoid nodular transformation of the spleen: clinical and radiologic characteristics

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign primary vascular lesion of the spleen. Although there have been many reports about the pathology of SANTs of the spleen, there have been no comprehensive descriptions of the imaging findings of SANTs of the spleen. To determine the clinical characteristics and imaging findings of SANTs of the spleen. We retrospectively evaluated seven patients with pathologically confirmed SANT, who underwent CT (n = 7), MRI (n = 4), ultrasonography (n = 4), and PET/CT (n = 3). Follow-up CT examinations were obtained in five patients. Clinical characteristics such as symptoms and concurrent disease were assessed. CT and MRI findings were evaluated by two radiologists, including the number, border, signal intensity, enhancement pattern, hemorrhage, and cystic change or necrosis. The longest diameter of each tumor was measured on CT. Echogenicity on ultrasonography and standardized uptake value on PET/CT were also evaluated. No specific symptom was associated with SANT. Two patients had a history of malignancy, one with cervical cancer and the other with early gastric cancer. Tumor growth was observed in four of five patients. On CT, all seven SANTs appeared as single, well-demarcated masses. CT showed a heterogeneous enhancement in seven patients. MRI showed centripetal progressive enhancement and absence of cystic change or necrosis in four patients, with three of these patients showing evidence of old hemorrhage. Ultrasonography showed heterogeneous hypoechoic mass in four patients. PET/CT showed increased standardized uptake value, ranging from 2.0 to 2.8, in three patients. SANT of the spleen is a single, well-demarcated solid mass without cystic change or necrosis. Increased FDG activity and tumor growth on follow-up imaging are common.

Laparoscopic splenectomy for splenic sclerosing angiomatoid nodular transformation: A case report

Introduction: Sclerosing angiomatoid nodular transformation (SANT) is a rare benign and proliferative vascular lesion arising within the red pulp of the spleen. The lesion is often identified incidentally on imaging and the diagnosis is confirmed on pathologic assessment of the resected spleen. The lesion can be cured by splenectomy. Until now, little has been published on the laparoscopic approach for treatment of SANT. Case Report: A 5­cm splenic tumor was discovered during a routine examination of an asymptomatic 38­ year­old man. We were initially suspicious of fibrous hamartoma, but could not rule out malignancy. Consequently, laparoscopic splenectomy was performed, and histopathological findings showed that the tumor was a sclerosing angiomatoid nodular transformation. Conclusion: We report a rare case of sclerosing angiomatoid nodular transformation, completely resected by laparoscopy. In such cases, laparoscopic splenectomy seems to be a feasible, safe, and effective treatment.