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Central Nervous System
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Previously thought of as a variant of meningioma, this tumor is now believed to be indistinguishable from hemangiopericytoma in other sites, where it is usually designated as solitary fibrous tumor. It has been thought to derive from the meningeal capillary pericyte, although the true histogenesis remains uncertain. It is a rare tumor that tends to arise in a younger age group than meningioma and is more common in men. These tumors arise in the dura as highly vascular, lobulated masses. They are densely cellular and often highly mitotic and may be graded WHO 2 or 3 on this basis. These tumors carry fusions of NAB2 and STAT6, and staining for STAT6 to identify the fusion event is recommended to confirm diagnosis. Hemangiopericytomas have a marked tendency to recur after surgery alone and to metastasize within and outside the CNS, particularly to bone. Maximal surgery is essential treatment, but post-operative high-dose radiotherapy (55–60 Gy) is often recommended to reduce local recurrence and metastasis. Median survival is around 5 years.
Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
The presence of any soft tissue strands should suggest the possibility of liposarcoma (130). Liposarcomas are not homogeneously fatty like lipomas, but contain islands of soft tissue (134). Poorly differentiated liposarcomas may not demonstrate any visible fatty component on imaging studies (134). The prognosis of these tumours depends on the grade and extent of local invasion. The most common CT finding of lymphangioma includes a smoothly-marginated cystic mass with homogeneous water density (135,136). Multiple locations may be seen within the mass in approximately one-third of patients. Haemorrhage into a lesion may lead to an increase in size and attenuation values on CT. The findings on MRI are variable and non-specific. Haemangiomas on CT are often heterogeneous in attenuation on unenhanced images, and fat may occasionally be seen within them. Heterogeneous enhancement is typical after contrast-medium administration, but is not always present (137). Enhancement may be dense, multifocal, or diffuse, and central or peripheral. Hemangiopericytomas are inhomogeneous in appearance with central areas of low attenuation and peripheral rim enhancement after intravenous administration of contrast material (138). On MRI, they are heterogeneous in signal intensity in T1-weighted (T1W) and T2-weighted (T2W) spin-echo images (138). Although some hemangiopericytomas are truly benign, many invade locally at the time of diagnosis and may recur after surgical excision (139).
Primary Pleuropulmonary Synovial Sarcoma: Report of Two Cases and a Comprehensive Review of the Literature
Published in Cancer Investigation, 2022
Neda Khalili, Elham Askari, Nastaran Khalili, Aboulghasem Daneshvar-Kakhki, Makan Sadr, Sara Haseli, Mihan Pourabdollah Toutkaboni
Histologically, SSs are divided into four categories: monophasic fibrous, monophasic epithelial, biphasic and poorly differentiated. More precisely, poorly differentiated tumors can actually develop in either the monophasic or biphasic subtype, and can be regarded as a form of tumor progression rather than a distinct subtype (34). The biphasic tumor type could be readily diagnosed since it consists of two components: epithelial cells and spindle-shaped cells. Monophasic fibrous SS, the most common subtype, exhibits uniform spindle cell fascicles arranged in a herringbone or interweaving pattern that may be accompanied by small areas of cells with epithelioid-like morphology. Poorly differentiated tumors generally have hemangiopericytoma-like vascular patterns. In addition, these tumors may have high mitotic counts, and may present with three prototypic forms: (1) a large cell or epithelioid pattern constituting rounded nuclei of varying size with prominent nucleoli, (2) a small cell pattern with nuclear features similar to other small round cell tumors and (3) a high-grade spindle cell pattern composed of spindle-shaped cells with high-grade nuclear features and a high mitotic rate, often accompanied by necrosis (34,35). According to the results of this review, the most frequently observed subtype was the monophasic fibrous, which constituted about 70% of all reported cases. The two latter subtypes were less often reported, and no case was documented as monophasic epithelial SS.
Solitary fibrous tumor/hemangiopericytoma of the external auditory canal
Published in Acta Oto-Laryngologica Case Reports, 2019
Jefferson Rijo-Cedeño, Luz Martín-Figueiro, Harold Tavárez, Rafael Ramírez-Camacho
Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm first described in 1931 by Klemperer and Rabin in the pleura [1]. In the past, it has been classified as a different but related entity to hemangiopericytomas, both having intermediate malignant potential, as described by the World Health Organization (WHO) classification scheme [2]. Currently, they have been categorized as different manifestations of the same pathology under the combined term ‘solitary fibrous tumor/hemangiopericytoma’ (SFT/HPC) by the WHO classification of tumors of the central nervous system in 2016 [3]. We report a case of a solitary fibrous tumor in the external auditory canal (EAC) in a 60-year-old male. To the best of our knowledge, there have been only three cases reported in the past by Izumaru et al. [4], Rezk et al. [5], and Lee et al. [6]. We describe the fourth case of SFT in the EAC and a review of the literature.
Intracranial hemangiopericytoma after radiation treatment: first case in the literature
Published in British Journal of Neurosurgery, 2023
Gulden Demirci Otluoglu, Emre Zorlu, Baran Yılmaz, Akın Akakın, Ozlem Yapıcıer, Turker Kilic
A twenty-five-year-old male patient was admitted with sudden onset headache, upward gaze palsy and altered consciousness. Magnetic Resonance (MR) showed a heterogeneously enhancing lesion in the pineal region and hydrocephalus. He had a VP shunt surgery first and then a left occipital craniotomy and a gross total excision of the tumor. Histology was of a pineoblastoma. In the post-operative period, he had radiotherapy with IMRT (Intensity Modulated Radiation Treatment) method (6000 cGy in 30 fractions). Forty-one month later he had a new extra-axial lesion heterogeneously enhancing in the right parietal parafalcine area. Second surgery was performed via right parietal craniotomy and gross total excision was achieved (Figure 1). Histology was of cellular mesenchymal tumor composed of fibroblastic type cells showing a rich thin-walled branching staghorn-like vessels. Tumor showed a delicate and rich network of reticulin fibers typically investing individual tumor cells. A mitotic count of 3 mitoses per 10 high-power fields was seen. Necrosis was not identified. Immunohistochemical analysis was carried out. Epithelial membrane antigen (EMA), progesterone receptor (PR) and Glial fibrillary acidic protein (GFAP) immunostains were negative. Tumor cells were only positive for CD34 and STAT6. The diagnosis was consistent with hemangiopericytoma, grade II (David N. Louis6) (Figure 2). He had adjuvant radiotherapy again with IMRT (Intensity Modulated Radiation Therapy) method to the second surgical excision area (5000 cGy in 30 fractions). He is now under follow up without any new intracranial lesion or extracranial metastasis 42 months since his last surgery.