Spindle cell hemangioma

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)...

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The fifth edition of the World Health Organization Classification of Tumors of Soft Tissue and Bone was published in early 2020. The revisions reflect a consensus among an international expert editorial board composed of soft tissue and bone pathologists, geneticists, a medical oncologist, surgeon, and radiologist. The changes in the soft tissue tumor chapter notably include diverse, recently described tumor types (eg, atypical spindle cell/pleomorphic lipomatous tumor, angiofibroma of soft tissue, and CIC-rearranged sarcoma), new clinically significant prognostic information for a variety of existing entities (eg, dedifferentiated liposarcoma and solitary fibrous tumor), and a plethora of novel genetic alterations, some of practical diagnostic relevance (eg, NAB2-STAT6 in solitary fibrous tumor, FOSB rearrangements in epithelioid hemangioma and pseudomyogenic hemangioendothelioma, and SUZ12 or EED mutations in malignant peripheral nerve sheath tumor, leading to loss of H3K27 trimethylation)...

Composite hemangioendothelioma (CHE) is an extremely rare locally aggressive vascular neoplasm comprising various benign, intermediate, and malignant vascular components. It is usually located superficially, in the dermis and subcutis of the extremities. Herein, we report a first case of CHE arising from the skeletal muscle in a 67-year-old woman who presented with a palpable mass on her right forearm. Magnetic resonance imaging revealed a 3.0 × 2.7-cm intramuscular mass with high-signal intensity on contrast-enhanced T2-weighted images...

Composite hemangioendothelioma is a rare vascular tumor morphologically comprising several distinct vascular components and exhibits a borderline malignant potential. We described the case of a 53-year-old female who presented with an infiltrative mass in the paravertebral soft tissue. The tumor showed discrete nests of synaptophysin-expressing epithelioid cells accompanied by rich vasculature, features highly reminiscent of sympathetic paraganglioma. Further analysis revealed areas resembling spindle cell hemangioma, retiform hemangioendothelioma, cavernous hemangioma/lymphangioma, and epithelioid hemangioendothelioma without the myxohyaline matrix in the tumor, and a final diagnosis of composite hemangioendothelioma with synaptophysin expression was made...

Recent molecular discoveries have refined vascular bone tumor classification. To investigate the clinical relevance of these refinements, we reviewed all cases of primary vascular bone tumors treated at our Institute. On the basis of morphology, cases were assessed immunohistochemically and molecularly. A total of 427 cases of primary vascular tumor of bone with available follow-up and histologic material were retrieved and reclassified according to the most recent diagnostic criteria as follows: 289 hemangiomas, 38 epithelioid hemangiomas, 21 epithelioid hemangioendotheliomas, 2 retiform hemangioendotheliomas, 1 intraosseous papillary intralymphatic angioendothelioma, 24 pseudomyogenic hemangioendotheliomas, and 52 angiosarcomas (of these, 45 were epithelioid angiosarcomas and 7 spindle cell secondary angiosarcoma)...

Objective: To investigate the clinicopathological features, diagnosis, differential diagnosis and immunohistochemical (IHC) characteristics of paraganglioma of urinary bladder (PUB). Methods: The clinical and pathological data of 23 cases of PUB were collected at the Second Affiliated Hospital of Fujian Medical University (7 cases); Fujian Provincial Hospital (8 cases); Fujian Medical University Union Hospital (6 cases); and First Affiliated Hospital of Fujian Medical University (2 cases) from May 2010 to November 2018...

BACKGROUND: Mammalian target of rapamycin (mTOR) inhibitors have been shown to have excellent effects in the management of kaposiform hemangioendothelioma (KHE); however, the mechanism of action is unclear. This study identified the expressions of mTOR pathway-related proteins in different vascular tumors to provide insight into the pathogenesis of KHE. METHODS: We retrospectively reviewed the pathologic specimens of 30 patients (KHE, 15; tufted angioma [TA], 5; infantile hemangioma [IH], 5; and lymphatic malformation [LM], 5)...

Maffucci syndrome is an extremely rare sporadic disease, characterized by multiple enchondromas and associated with multiple hemangiomas and/or lymphangiomas. First case was reported in 1881, and fewer than 200 case have been reported to date. Potential sarcomatous malignant transformation have been noticed in previous cases. This report describes the case of 21 years-old male complain of right sided nasal obstruction for 10 months, and right sided aural fullness. Examination revealed multiple asymptomatic nodules in both hands since childhood, that have not been investigated and a large nasopharyngeal mass...

In this study, we aimed to present the clinicopathologic and molecular features of a distinct group of hemangioma with GNA mutations that exhibited prominent thrombosis and organization changes with florid intravascular endothelial cell proliferation that we provisionally termed "thrombotic hemangioma with organizing/anastomosing features." Twenty-six cases were included. No sex predilection was seen (male:female=13:13). Patients' age ranged from 17 to 89 years (median: 51 y). All but 1 occurred in the skin whereas the remaining tumor involved the neck soft tissue...

BACKGROUND: Infantile hemangioma (IH) is the most common childhood benign vascular tumor. Recently, propranolol has been found to be an effective therapy for IH, but its mechanism of action is not yet understood. Hemangioma stem cells (HemSCs) have a mesenchymal morphology, robust proliferation, and multilineage differentiation (into adipocytes). Therefore, we hypothesized that propranolol could accelerate the transdifferentiation of HemSCs and prevent the growth of proliferating IH. In this study, the fibrofatty tissue of IH that received therapy with propranolol appeared much earlier than without the treatment...

Objective: To analyze the clinicopathological features, immunohistochemical (IHC) phentotype,diagnosis and differential diagnosis of infantile/congenital fibrosarcoma (IFS/CFS) with unusual histological features. Methods: Five IFS/CFS at Children's Hospital of Fudan University from March 2014 to July 2018 were analyzed for their diagnosis and differential diagnosis. Results: Two cases were males, three cases were females. The clinical manifestation of IFS/CFS was a rapidly-growing and painless mass. There were no specific radiologic features...

BACKGROUND: Spindle cell hemangioma (SCH) was an extremely rare benign tumor which typically arised in the subcutis of the distal extremities of young people. In this study, we reported a case of multiple spindle cell hemangioma in both lungs. CASE PRESENTATION: A 19-year-old HIV-negative female was found to have multiple lung nodules by the chest X-ray during the physical examination. Her chest CT scan revealed multiple round-like pulmonary nodules in both lungs...

INTRODUCTION: The type of soft tissue lesions seen in children differs from that seen in adults. The role of fine needle aspiration (FNA) cytology in their diagnosis is not well documented. AIM: To study the cytopathological spectrum of paediatric soft tissue tumours to highlight uncommon benign and malignant lesions and the challenges in their diagnosis. METHODS: A 3-year retrospective audit of all paediatric soft tissue FNA cytology cases from 2015 to 2017 was performed...

RATIONALE: Spindle cell hemangioma (SCH) is considered a benign vascular lesion. It typically develops as a solitary nodule or multiple masses located in the dermal or subcutaneous layers of the distal extremities. To the best of our knowledge, there are no prior reports of SCH in the spleen. PATIENT CONCERNS: A 41-year-old male was admitted to our hospital with recurrent headaches, nausea, and vomiting persisting for 5 days. Ultrasound, computed tomography, and magnetic resonance imaging revealed multiple space-occupying lesions in the spleen, and the biggest lesion was 4...

In this review, we provide an update of the recently discovered, diagnostically significant genetic aberrations harbored by a subset of vascular neoplasms. From benign (epithelioid hemangioma, spindle cell hemangioma), to intermediate (pseudomyogenic hemangioendothelioma), to malignant (epithelioid hemangioendothelioma, angiosarcoma), each neoplasm features a mutation or gene fusion that facilitates its diagnosis by immunohistochemistry and/or molecular ancillary testing. The identification of these genetic anomalies not only assists with the objective classification and diagnosis of these neoplasms, but also serves to help recognize potential therapeutic targets...

Calcifying nested stromal epithelial tumor is a rare entity that has gone by a variety of names in the literature: ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor of the liver. To our knowledge, approximately 38 cases have been reported in the literature. The histogenesis is still largely unknown but histopathologically is characterized by nests of spindle and epithelioid cells in an organoid arrangement surrounded by a prominent dense myofibroblastic stroma with occasional psammomatous calcification and focal heterotopic ossification...

Background: Intramuscular myxomas are rare, benign mesenchymal tumors in the musculoskeletal system, and usually, the tumors arise in the large muscles of the thigh, buttocks, shoulder, and upper arm. However, a tumor of the forearm is very rare. Herein, we describe the case of an intramuscular myxoma in the supinator muscle of a 56-year-old female patient. Case Presentation: Magnetic resonance imaging showed a well-defined mass that was hypointense with the peritumoral fat ring sign...

Although some soft tissue and bone tumors can be identified based on histologic features alone, immunohistochemistry plays a critical diagnostic role for most mesenchymal tumor types. The discovery of recurrent genomic alterations in many benign and malignant mesenchymal neoplasms has added important biologic insights and expanded the spectrum of some diagnostic subgroups. Some tumors are defined by unique genomic alterations, whereas others share abnormalities that are not tumor-specific and can be observed in a sometimes broad range of biologically unrelated neoplasms...

Spindle cell hemangioma (SCH) is a unique benign vascular lesion. We present a case of SCH in the upper lip of a 41-year-old woman. A submucosal nodular mass 30 × 20 mm in size was observed in the left upper lip. The mass developed 5 years earlier and enlarged after repeated ethanol injections. The mass was elastic firm, mobile, bluish in color, and well demarcated in magnetic resonance imaging. Under the clinical diagnosis of hemangioma, surgical excision was performed under local anesthesia. Microscopically, the lesion was composed of irregular cavernous spaces and multiple solid cellular areas...