We report an autopsy case of Fahr\'s syndrome in an 85-year-old woman associated with asymptomatic hypoparathyroidism. The patient was diagnosed as having brain calcification at 65 years of age. She developed mild dementia at 75, parkinsonism at 76, and severe dementia at 82. Computed tomography revealed extensive, symmetric intracranial calcification, involving both sides of the basal ganglia and cerebellar dentate nuclei, and severe cerebral atrophy that developed afterwards. A neuropathological examination revealed intracranial calcification, particularly in the wall of the arterioles and capillaries having numerous calcium deposits. Severe vascular calcification and severe neuronal loss without α-synuclein accumulation were found in the substantia nigra. There were high-level neuropathological changes indicative of Alzheimer\'s disease. Although the colocalization of calcium and amyloid-β deposits in the same arterial wall was rare, both of them were located in a similar layer of the arterial wall. The vascular calcification in the basal ganglia spread continuously through the corona radiata into the selective cerebral areas along the medullary arteries, but did not involve the corpus callosum or insular region. Stone formation was observed at the corona radiata adjacent to the superolateral angles of the lateral ventricles. We hypothesized that there would be a stereotypical extension pattern of vascular calcification related to the arrangement of penetrating arteries in Fahr\'s syndrome.

Haemangiopericytoma (HPC) is an uncommon, vascular tumor derived from Zimmerman pericytes surrounding blood vessels. HPC constitute around 1% of all tumors of vascular origin and may appear anywhere, 5% of them can be situated in nasal cavity. Tumor location within the head and neck predispose to its benign character and improves prognosis. This case report presents the case of 33-year-old patient with haemangiopericytoma-like tumor of the nasal cavity, presented symptoms of impaired nasal breathing and recurrent epistaxis. Tumor was excised with 0 degree endoscope. The follow-up recurrence-free period was 2 year 6 months and shows this is effective way of treatment. Described in the literature late recurrences and metastases reminds that regular, life-long observation is mandatory.

BACKGROUND: Myofibroblastic sarcoma or myofibrosarcoma is a malignant tumor of myofibroblasts and known to develop rarely in the breast, but its underlying lesion and tumor cell origin have never been reported yet.
METHODS: A 61-year-old female presented with a gradually growing breast mass with well-demarcated ovoid nodular shape. The tumor was histologically characterized by fascicular-growing spindle cell proliferation with large areas of hyalinized fibrosis and focally ductal epithelial remnants embedded in myxoid stroma, mimicking a fibroadenomatous lesion. It had frequent mitoses of 5-16/10 high-power fields, hemorrhagic necrosis, and focally pericapsular invasion. The spindle cells were diffusely immunoreactive for fibronectin, smooth muscle actin, and calponin, which suggest a myofibroblastic origin. Multiple irregularly thickened vessels with medial or pericytic cell proliferation were found to be merged with the intrinsic tumor cells. The tumor could be diagnosed low-grade myofibroblastic sarcoma arising in an old fibroadenoma.
CONCLUSIONS: We report a case of a low-grade mammary myofibrosarcoma that showed a background lesion of fibroadenoma first in the worldwide literature and suggest the pericytes or medial muscle cells of the intratumoral vessels as the cell origin of the myofibroblastic sarcoma.

Ischemia is a leading cause of death in the western world. Regenerative medicine aims to improve healing of ischemic injury by complementing pharmacologic/interventional treatments. Navigating regenerative therapies from \'bench-to-bedside\' is a multistep time-consuming process, balancing cell expansion, purity, safety and efficacy while complying with regulatory guidelines. Studies started in academic laboratories unused to long-term planning often fail because of poor strategy design, lack of contingency plans or funding. We provide a strategic insight into our translation of saphenous vein-derived adventitial progenitor cells into a clinical grade product to treat angina. We discuss discovery phases, introduction of standard operating procedures and upgrade to clinical standards. We also examine contractual aspects of transferring to GMP-accredited facilities for clinical production and unexpected hurdles.

Pericytic tumours is a new category recently introduced in soft tissue pathology describing those entities sharing evidence of myoid differentiation and a tendency of spindle or more rounded cells to grow in a perivascular fashion. The great majority of pericytic tumours are benign but recurrence has been described as well as very rare malignant cases with an aggressive behaviour. Although pericytic tumours arise most commonly in the subcutaneous tissue of distal extremities in middle age patients, additional cases have been recently described in many other organs. Here we describe a case of a 57-year-old man with an 8.5 cm mass of the liver hilus diagnosed as myopericytoma, with particular reference to its biological potential and differential diagnosis. It is important for the pathologists to take this diagnosis into consideration as well for a better management of patient care.

Glomus tumors are rare benign neoplasms. They typically present with a triad of severe pain, point tenderness, and cold sensitivity. Most glomus tumors will form well-circumscribed nodules, and excisional biopsy of masses detected by ultrasonography or magnetic resonance imaging of the lesion can relieve the symptoms. We describe a 30-year-old female patient with nontumorous glomus cell proliferation with clinical symptoms similar to those of glomus tumors. The lesion was treated by exploration and excision, with a satisfactory clinical outcome.

We report a case of malignant myopericytoma arising in the left atrium with brain, skeletal, and liver metastases, which, to our knowledge, is the first report of this rare entity in this anatomic location. A 52-year-old man presented with progressive blackening of his left field of vision. Magnetic resonance imaging and a computed tomography scan of the brain and thorax showed a heterogeneous mass in the right occipital lobe and a large left atrial floor mass. Excision of the atrial mass showed a circumscribed but unencapsulated malignant spindled neoplasm with a perivascular concentric cellular arrangement punctuated by sheets of tumor necrosis. The cells were round to spindled with eosinophilic cytoplasm and indistinct borders. Focally, the tumor infiltrated cardiac muscle. By immunohistochemistry, the cells were positive for smooth muscle actin and negative for desmin, H-Caldesmon, S-100, HMB-45, and Melan-A. The features were prototypic for malignant myopericytoma. Eight months after initial presentation, the patient is alive with metastatic disease.

Myopericytoma arising in the visceral organs is rare and only 1 case of renal myopericytoma has been reported in the literature to date. We report the second case of myopericytoma arising in the kidney in a 40-year-old Hispanic woman who presented with pain on the left side of the abdomen and frequent urination. Abdominal computed tomography scan showed an exophytic left-sided renal mass. Partial nephrectomy was performed. The patient remains free of disease at 24 months after diagnosis. Our case is histologically distinct from the previously described case as it lacks the \"hemangiopericytic/glomangiopericytoma\" pattern. The tumor in our case showed the characteristic pattern of myopericytoma and an additional glomus tumorlike pattern. The tumor cells showed diffuse reactivity for vimentin, smooth muscle actin, smooth muscle myosin heavy chain, and muscle-specific actin in both morphologic patterns and strong diffuse CD34 expression in glomus tumorlike focus. This case report adds to the morphologic heterogeneity of myopericytomas.

Myopericytoma is a benign neoplasm consisting of cells that appear to have a distinct differentiation towards presumed perivascular myoid cells. Amongst myopericytoma, an intravascular variant appears to have been reported only rarely. A 67-year-old man presented with a 15-year history of a painful, slow growing 3 × 3.5 cm sized mass in the subcutis of his right lateral thigh. Histopathological studies showed a subcutaneous mass entirely within the lumen of a vein. The tumor was composed of spindle-shaped myoid-appearing cells in a concentric arrangement, intimately associated with thin-walled vascular channels. Tumor cells were diffusely positive for smooth muscle actin, focally positive for CD34, and negative for desmin and CD31. From these findings, we diagnosed this lesion as intravascular myopericytoma. Unlike previous reports, our case showed a relatively large painful subcutaneous mass, although this tumor has an intravascular nature.

Primary cardiac synovial sarcoma is a rare disease. A 51-year-old man visited our hospital with the chief complaint of palpitations and shortness of breath while exercising. Copious bloody pericardial effusion and a multicystic intrapericardial tumor were detected. A primary cardiac malignant tumor was suspected, an open-chest tumor resection was performed with the objectives of diagnosis and treatment. Histologically, the tumor cells were uniformly spindle-shaped with an ovoid or oval nucleus, they had proliferated in fascicular fashion. In addition myxoid degeneration, a hemangiopericytomatous vascular pattern and pseudorosette formation were seen in some areas of the tumor. Based on the histopathological and immunohistochemical findings and reverse transcription polymerase chain reaction detection of SS18-SSX1 fusion transcripts, a monophasic fibrous type synovial sarcoma was diagnosed. Postoperative radiation therapy was administered and there had been no recurrence 9 months after the surgery.