Spindle cell hemangioma

Spindle cell haemangioma (SCH) is a slow growing, benign vascular lesion with a preference for the distal extremities. Its occurrence in the oral cavity is rare. Clinically, it presents as solitary or multiple subcutaneous nodules, therefore, it could be considered in the differential diagnosis of benign soft tissue tumours. Microscopically it mimics some malignant vascular tumours and it is necessary to differentiate it from other malignant vascular lesions. We report a case of SCH in anterior mandibular region of a young male in his 20s...

Composite hemangioendothelioma (CHE) is an extremely rare vascular neoplasm that is characterized by an admixture of benign, low-grade malignant, and malignant vascular components. It is usually located superficially in the dermis and subcutis of the extremities, and other sites involved include the head and neck region, oral mucosa, and viscera of the kidney and spleen. CHE has a low-grade malignant potential because it is locally aggressive. Here, we report a case of CHE in the heart in a 46-year-old man, who presented with a palpable mass arising from his right ventricle...

Spindle cell hemangioma is a rare benign hemangioma. It is mainly found as small soft tissue nodules in the distal extremities, especially in the hands. The manifestation in bone is extremely rare. We present a case of spindle cell hemangioma of bone in the frontal bone. We also reviewed the literature to find the common imaging findings of intraosseous hamangiomas. The main manifestations of X-ray and CT were osteolytic lesions with soap bubble-like changes, and soft tissue mass formation. Magnetic resonance imaging mainly showed a lobulated mass with clear boundary, uneven hyperintense on T2WI, and obvious enhancement on contrast-enhanced scan...

Maffucci syndrome (MS, OMIM 166000) is an extremely unusual, nonhereditary, multisystemic disorder that is characterized with multiple enchondromas and vascular lesions, most of which are spindle cell hemangiomas. Complications of MS, such as bone deformities and dysfunction caused by enchondromas, usually increase during childhood and adolescence. Malignant transformation of enchondromas and other malignancies are the most severe complications. MS is caused by somatic mosaic IDH1/2 mutations, 65% of which are the IDH1 p...

'Intracranial mesenchymal tumor, FET-CREB fusion-positive' occurs primarily in children and young adults and has previously been termed intracranial angiomatoid fibrous histiocytoma (AFH) or intracranial myxoid mesenchymal tumor (IMMT). Here we performed genome-wide DNA methylation array profiling of 20 primary intracranial mesenchymal tumors with FET-CREB fusion to further study their ontology. These tumors resolved into two distinct epigenetic subgroups that were both divergent from all other analyzed intracranial neoplasms and soft tissue sarcomas, including meningioma, clear cell sarcoma of soft tissue (CCS), and AFH of extracranial soft tissue...

INTRODUCTION: Little information about clinical presentation of mesenchymal tumors of the lower gastrointestinal (GI) tract due to their extreme heterogeneity is available for clinical management. Usually, small solitary asymptomatic polyps are accidently found during a screening colonoscopy performed for hematochezia, abdominal pain, constipation, diarrhea, and bowel obstruction. In this case series, we illustrate our experience with mesenchymal tumors of the lower GI tract, which are a group of unusual and quite challenging lesions...

Spindle cell hemangioma (SCH), a non-neoplastic reactive vascular lesion, rarely locates in bones. We herein report a successful case of intralesional curettage for an infant with SCH of fibula. An 11-month-old boy was admitted to our center with a painless mass in the right proximal calf. Preoperative digital radiograph demonstrated a massive vascular lesion with an irregular bone destruction of proximal fibula. The lesion was removed via the intralesional curettage approach and pathologically diagnosed as SCH...

Maffucci syndrome is a rare, highly variable somatic mosaic condition, and well-known cancer-related gain-of-function variants in either the IDH1 or IDH2 genes have been found in the affected tissues of most reported individuals. Features include benign enchondroma and spindle-cell hemangioma, with a recognized increased risk of various malignancies. Fewer than 200 affected individuals have been reported; therefore, accurate estimates of malignancy risk are difficult to quantify and recommended surveillance guidelines are not available...

Extraskeletal osteosarcoma is a highly aggressive malignant osteoid forming mesenchymal neoplasm arising from soft tissues which accounts for 1% of all soft tissue sarcomas. We report the case of a 46-year-old female with no significant past medical history presenting to an emergency department with a right lateral thigh mass following minor trauma. She was eventually found to have high grade extraskeletal osteosarcoma with rapid progression of disease resulting in patient demise. Differentiation of these lesions from alternative processes relies on specific imaging and pathologic features...

Symplastic hemangiomas (SH) are benign vascular lesions that show atypia in vascular smooth muscle and interstitial cells with sparing of endothelial cells. We present four cases of this rare tumor. The patients (two males; two females) ranged in age from 57 to 83 years (median 74); lesions were located on the leg (n = 3) and back (n = 1), and ranged from 6 to 8 mm. SH were well-circumscribed and dermal-based, often with an epidermal collarette (3/4). They were characterized by the presence of variably atypical, hyperchromatic/pleomorphic epithelioid to spindled cells within vascular walls (3/4) and/or perivascular stroma (4/4)...

A simple bone cyst (SBC) is a cystic bone lesion predominantly affecting young males. The cyst is lined by a fibrous membrane and filled with serosanguinous fluid. EWSR1/FUS-NFATC2 rearrangements were recently identified in SBC. We here report exactly the same rearrangement in 3 lesions diagnosed as vascular malformations of 2 elderly patients. In total, through Archer FusionPlex, fluorescence in situ hybridization and/or reverse transcriptase-polymerase chain reaction the EWSR1-NFATC2 rearrangement was identified in 6 of 9 SBC, 3 of 12 benign vascular tumors, and none of 5 aneurysmal bone cyst lacking USP6 fusion...

Pulmonary sclerosing pneumocytoma is a rare benign pulmonary tumor of primitive epithelial origin. Because of the unspecific radiological features mimicking malignancies and its histological heterogeneity, the differential diagnosis with adenocarcinoma and carcinoid tumors is still challenging. We report our experience of two cases of sclerosing pneumocytoma, as well as a review of the literature. Immunohistochemical findings showed intense staining of the cuboidal epithelial cells for cytokeratin-pool and TTF-1, with focal positivity for progesterone receptors...

Objective: To investigate the clinical features, diagnoses and treatments of head and neck occupying lesions in newborns. Methods: All newborns with head and neck occupying lesions admitted to Neonatel Intensive Care Unit of the First Affiliated Hospital of Zhengzhou University form January 2014 to November 2019 were included. There were 23 males and 17 females, admission age was from 2 d-28 d, and the clinical manifestations, examinations, treatments and outcomes were evaluated. Results: Among 40 newborns with head and neck occupying lesions, 22 cases were admitted with dyspnea, 15 cases with masses in oral cavity or head and neck, 2 cases with fever as the first symptom, and 1 case with hoarseness as the first symptom...

Neuroendocrine tumors (NETs) account for approximately 1-2% of all gastrointestinal tumors. In particular, primary hepatic NETs (PHNETs) are extremely rare. A 42-year-old female patient was given a diagnosis of liver tumor after a medical check-up with ultrasonography. An enhanced CT scan and MRI were suggestive for a hepatic hemangioma or adenoma, but the possibility of hepatocellular carcinoma could not be denied. Moreover, this tumor grew larger, so we performed a laparoscopic partial hepatectomy for the diagnosis and treatment of the patient...

Primary malignant vascular neoplasms of the liver, angiosarcoma and epithelioid hemangioendothelioma, are extremely rare entities in the pediatric population. International Society for the Study of Vascular Anomalies classification system is recommended for the pathologic diagnosis of hepatic vascular lesions in this age group. In this article, we highlight the clinicopathologic characteristics of hepatic angiosarcoma and epithelioid hemangioendothelioma in the pediatric population. Hepatic angiosarcoma in children shows a slight female predominance with an average age of 40 mo at diagnosis...

BACKGROUND: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment...

Spindle cell hemangioma is a benign vascular tumor typically occurring in the dermis or subcutis of distal extremities as red-brown lesions that can grow in both size and number over time. They can be very painful and potentially disabling. A family history of cancer or previous history may be relevant and must be taken into consideration. Juxtaglomerular cell tumor (reninoma) is an extremely rare cause of secondary hypertension diagnosed mostly among adolescents and young adults. Excessive renin secretion results in secondary hyperaldosteronism...

Spindle cell hemangioma (SCH) is a benign unusual vascular neoplasm. It does not have gender predilection and can occur at all ages. The disease affects dermis and subcutis of distal extremities predominantly; head and neck involvement is very rare, paranasal sinus involvement has not been reported before. Herein we present a 4-month-old infant with nasal obstruction since two weeks of age due to a mass in ethmoid sinus obliterating the nasal passage. After the histopathological diagnosis of SCH, the tumor was partially resected...

INTRODUCTION: Spindle cell hemangioma (SCH) is a rare vascular tumor which was first described in 1986. It affects mostly the distal extremities. The head and neck are rarely involved. This article reports the first case of SCH in the infratemporal fossa. PRESENTATION OF CASE: A 41-year-old woman presented with an 8-month history of right cheek swelling. Facial CT scan and MRI showed an intensely and heterogeneously enhancing tumor of the infratemporal fossa suggesting an angiomatous neoplasm...

Intracranial mesenchymal tumors with FET-CREB fusions are a recently described group of neoplasms in children and young adults characterized by fusion of a FET family gene (usually EWSR1, but rarely FUS) to a CREB family transcription factor (ATF1, CREB1, or CREM), and have been variously termed intracranial angiomatoid fibrous histiocytoma or intracranial myxoid mesenchymal tumor. The clinical outcomes, histologic features, and genomic landscape are not well defined. Here, we studied 20 patients with intracranial mesenchymal tumors proven to harbor FET-CREB fusion by next-generation sequencing (NGS)...