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We report a rare case of tanycytic ependymoma arising from the cerebral hemisphere. A 59-year-old man was admitted to our hospital because of the incidental detection by MRI of a tumor lesion in the right temporooccipital paratrigonal region. The mass showed low-to iso-intensity on T1-weighted images and high intensity on T2/proton-weighted images. Partial resection was performed using a transsulcal approach to avoid compromising the visual field. Most of the tumor cells showed elongated spindle shapes arranged in dense fascicles. A few true ependymal rosettes and perivascular pseudorosettes were visible. The tumor cells were positive for GFAP, S-100, and vimentin, but negative for synaptophysin, EMA, and keratin. The MIB-1 labeling index was approximately 1%. Ultrastructurally, the tumor cells had ependymal cell features, such as microvilli and cilia. From these findings, a pathological diagnosis of tanycytic ependymoma was made.
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PMID:A case of tanycytic ependymoma arising from the cerebral hemisphere. 1809 25

Neurenteric cysts are extremely rare congenital anomalies, often presenting in the first 5 years of life, and are caused by an incomplete separation of the notochord from the foregut during the third week of embryogenesis. They are frequently accompanied with spinal or gastrointestinal abnormalities, but the latter may be absent in adults. Although usually located in the thorax, neurenteric cysts may be found along the entire spine. We present a 24-year-old woman admitted for epigastric pain, nausea, vomiting, low grade fever and leucocytosis. She underwent cystgastrostomy for a loculated cyst of the distal pancreas at the age of 4 years, which recurred when she was at the age of 11 years. Ultrasound and computer tomograghy (CT) scan revealed a 16 cm multiply 15 cm cystic mass in the body and tail of pancreas, with a 6-7 mm thickened wall. Laboratory data and chest X-ray were normal and spinal radiographs did not show any structural abnormalities. The patient underwent a complete cyst excision, and after an uneventful recovery, remained symptom-free without recurrence during the 5-year follow-up. The cyst was found to contain 1200 mL of pale viscous fluid. It was covered by a primitive single-layered cuboidal epithelium, along with specialized antral glandular parenchyma and hypoplastic primitive gastric mucosa. Focal glandular groups resembling those of the body of the stomach were also seen. In addition, ciliary respiratory epithelium, foci of squamous metaplasia and mucinous glands were present. The wall of the cyst contained a muscular layer, neuroglial tissue with plexogenic nerve fascicles, Paccini corpuscle-like structures, hyperplastic neuroganglionar elements and occasional psammomatous bodies, as well as fibroblast-like areas of surrounding stroma. Cartilagenous tissue was not found in any part of the cyst. Immunohistochemistry confirmed the presence of neurogenic elements marked by S-100, GFAP, NF and NSE. The gastric epithelium showed mostly CK7 and EMA immunoexpression, and the respiratory epithelium revealed a CK8 and CK18 immunoprofile without CK 10/13 positive elements, though neither CEA or AFP positive cells were found. To our knowledge, this is the first reported case of an abdominally located neurenteric cyst with no associated spinal anomalies.
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PMID:Abdominal neurenteric cyst. 1859 46

Ceruminous gland tumours are rare neoplasms. We describe a case of a ceruminous tumour with complex morphology characterised by fibrous hyaline stroma bilayered epithelial ductal structures and nodules of tightly arranged clear cells with abundant Pas-positive cytoplasm. Within nodules among clear cells delicate apocrine ducts were found. Stromal tongues infiltrated with lymphocytes invaginated into nodules producing a lymphadenomatous pattern. Among clear cells, there are also numerous eosinophilic, Pas-positive refractile crystalline inclusions that appeared as floral petals (gerbera) or as a firework-like pattern. By immunohistochemistry, ductal structures were reactive for CK pan, CK7, CK18, CK19, EMA and GCDFP-15. Epithelial ductal basal cells were reactive for CK5, p63, calponin and SMA. Clear cells were weakly positive for CK18 and strongly positive for vimentin; they also displayed S100 protein and focal GFAP immunoreactivity. Interestingly clear cells lacked immunostaining for calponin, p63, caldesmone, SMA and MSA. This result supports the myoepithelial nature of clear cells, which have lost some antigenic specificities, and the diagnosis of adenomyoepithelioma of the ceruminous gland. The lesion appears morphologically benign. The patient is a 47-year-old woman with no evidence of disease after 3 years of follow-up.
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PMID:[Clear cell adenomyoepithelioma of the ceruminous gland]. 1884 27

Papillary thyroid carcinoma with metastasis to the frontal skull is extremely rare. We report a case of unsuspected papillary thyroid carcinoma with frontal skull metastasis. The patient was a 62-year-old African American woman with presentation of a 4-cm firm, painless, immobile, ill-defined mass at the right forehead. Ultrasound and computer tonography detected a hypervascular and osteolytic tumor involving the skull and overlying skin. Fine-needle aspiration was performed followed by surgical biopsy. Cytologic examination revealed the presence of hypercellular and bloody material. The neoplasm showed glandular features and was composed of clusters of round to oval cells with pinkish squamoid cytoplasm, oval nuclei and inconspicuous nucleoli on smears and sections of cell block. With immunocytochemical stain, the neoplastic cells were positive for pancytokeratin and vimentin and focally positive for EMA, while they were negative for S100, HMB45, Melan-A, CD34, GFAP, CD10, LCA, RCC and CD138. The diagnosis was a metastatic carcinoma. Clinical follow up with surgical biopsy was recommended. Surgical biopsy demonstrated histological and cytological features of papillary thyroid carcinoma including prominent papillae, nuclear overlapping, grooves, and intranuclear pseudoinclusions. Thus, a diagnosis of metastatic papillary thyroid carcinoma was rendered. Though skull metastasis of thyroid carcinoma is rare, it should be considered in the differential diagnosis when a skull mass lesion is encountered.
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PMID:Papillary thyroid carcinoma with metastasis to the frontal skull. 1945 56

By analogy to gliosarcoma, the term "ependymosarcoma" has recently been coined to thematize the rare phenomenon of a malignant mesenchymal component arising within an ependymoma. We report on an example of this paradigm, involving tanycytic ependymoma as the host tumor in a 40-year-old female who underwent two tumor extirpation procedures at one-year interval. She first presented with severe headaches, and was seen by imaging to harbor a moderately enhancing mass 2.5cm in diameter at the rostral septum pellucidum accompanied by occlusive hydrocephalus. Microscopically, the tumor consisted of solid, wavy fascicles of elongated cells that were occasionally interrupted by vague perivascular pseudorosettes. Mitotic activity was absent, and less than 1% of nuclei immunoreacted for MIB-1. A histological diagnosis of tanycytic ependymoma (WHO grade II) was rendered, and no adjuvant therapy given. At recurrence, the lesion was 3.5cm in diameter, intensely enhancing, and had already seeded into the subarachnoid space. Histology showed a biphasic glial-sarcomatous architecture with remnants of the original ependymoma now displaying hypercellularity and atypical - yet not frankly anaplastic - features. The sarcomatous moiety consisted of spindle and epithelioid cells densely interwoven with reticulin fibers. While the ependymal component was GFAP and S100 protein positive, and featured punctate staining for EMA, none of these markers was expressed in the adjacent sarcoma. Instead, the latter reacted for vimentin and smooth muscle actin. To the best of our knowledge, this is the first documentation of tanycytic ependymoma undergoing malignant transformation, one driven by a highly anaplastic mesenchymal component, corresponding to "ependymosarcoma".
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PMID:Rapid spontaneous malignant progression of supratentorial tanycytic ependymoma with sarcomatous features - "Ependymosarcoma". 1985 84

Chordoid meningioma is a rare variant of meningioma with histological features resembling those of chordoma. This tumor has a great risk of recurrence and aggressive growth (WHO grade II). This study was done to document the clinical and pathological features of ten patients with chordoid meningioma who submitted to surgery at the National Institute of Neurology and Neurosurgery in Mexico City. Clinical, histological and immunohistochemical features were examined. The age range was from 30 to 67 years old (mean, 34.2 years). Seven patients were female and three male. The duration of symptoms varied from 3.5 months to 5 years (mean, 14.1 months). No systemic symptoms were noted. The tumor was localized in eight cases in the supratentorial compartments. Histologically, the tumors were characterized by strands and cords of meningothelial cells arranged in a mucinous stroma. Two of the ten tumors showed metaplasic changes, and seven showed brain invasion. Tumor cells demonstrated CK7, EMA and focal S-100 protein and Ep-CAM. Cytokeratin AE1/AE3, GFAP and synaptophysin were negative. The MIB-1 proliferative index was from 6 to 9% (mean 7.8). PCNA Li was 6 to 20% (mean, 14), and microvascular density was 6-16 (mean, 14.5). The mean rate of the MIB-1 labeling index in recurrences was 7.1% versus 6.33% for no tumor recurrence. Chordoid meningioma, World Health Organization grade II, is an uncommon variant of meningioma with a propensity for aggressive behavior and increased likelihood of recurrence. Chordoid meningiomas are predominantly tumors of young adults with a predilection for the supratentorial location. Intraventricular location and absence of systemic manifestations, despite the presence of abundant B-lymphocytes, mast cells and low MIB-1 LI, are some of the interesting findings in the present series that need further study. Hence, a larger number of cases with adequate follow-up data need to be studied further to establish the clinical relevance of this variant.
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PMID:Chordoid meningioma: a report of ten cases. 2009 74

We report a case of primary dural based osteosarcoma in the right fronto-temporal convexity in a 43-year-old female who presented with a short history of seizure and headache. Radiologic evaluation revealed a well defined brightly enhancing extra-axial lesion in the right fronto-temporal region with a dural tail around the sylvian fissure. The overlying bone was uninvolved. Paraffin section of the tumor showed plump cells with moderate nuclear and cellular pleomorphism with eosinophilic extracellular material (osteiod) between the cells. At a few places, lace like osteiod was seen encasing individual cells signifying osteiod being formed by tumor cells. Immunohistochemistry for EMA was focally positive and negative for S-100 protein and GFAP. A final histopathological diagnosis of dural based primary osteosarcoma of the right fronto-temporal region was rendered. To the best of our knowledge this will be the eighth such case in literature.
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PMID:Dural based primary osteosarcoma in right fronto-temporal region with review of literature. 2022 82

We describe the clinicopathologic characteristics of 55 oral palisaded encapsulated (solitary circumscribed) neuromas (PEN/SCN). Fifty-five cases of PEN/SCN in 54 patients were reviewed. Lesions were categorized according to their histologic pattern, partial or complete encapsulation, presence of Verocay bodies and presence of a parent peripheral nerve. In 13 selected cases immunohistochemical evaluation for neuronal markers (S-100, GFAP, NFP, EMA) was performed. When immunoreaction with EMA was weak, claudin-1 and glut-1 stains were utilized. Thirty-eight patients were men and 16 were women. Mean patient age was 48 years (SD: +/-14). The vast majority involved the masticatory (palate and gingiva) mucosa (76.4%) followed by the labial mucosa, the tongue and buccal mucosa. Recurrence was recorded in only one case. Histologically, 34 lesions had a lobular pattern, 10 were plexiform, 7 fungating and 4 multilobular. Stroma was limited, but focal myxoid changes were seen at the periphery of the lobules. Only one predominantly myxoid lesion was encountered. The number of intralesional axons varied, but the ratio of Schwann cells to axons was generally less than 1:2. Most lesions (89%) were only partially surrounded by perineurium. Tumor cells were S-100 positive and GFAP negative. The parent nerve was identified in 50% of the cases. Overlying epithelium was generally atrophic. Peritumoral connective tissue was generally unremarkable, but chronic inflammation was present in five cases. PEN/SCN is a relatively common peripheral nerve sheath tumor. Generally, its diagnosis is simple. GFAP may be of help to distinguish PEN/SCN from other peripheral nerve sheath tumors (schwannoma, neurofibroma, traumatic neuroma) in cases where histomorphologic features may be confusing. Finally, pathologists should be aware of the occurrence of plexiform and multilobular PEN/SCN variants, to avoid misinterpretation as plexiform neurofibroma or schwannoma.
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PMID:Palisaded encapsulated ("solitary circumscribed") neuroma of the oral cavity: a review of 55 cases. 2023 84

Angiocentric gliomas (AG) have only recently been described. We encountered a 25-year-old woman with AG who had a history of epilepsy for two years. MRI revealed that there was a solid tumor in the hippocampus. The tumor was totally removed. Histologically, the spindle tumor cells proliferated around small parenchymal vessels with perivascular pseudorosettes. The tumor cells of the hippocampus surface umbilicated forming rosettes. Immunohistochemistry demonstrated positivity for GFAP, Vimentin and S-100, but were negative for neurofilament protein, Syn, CgA and P53. EMA had "dot-like" positive staining. The proliferation index was less than 1%. The location of the tumor and the pathological findings confirm that the diagnose was AG. Epilepsy disappeared after the operation. When fully resected these tumors have a good prognosis.
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PMID:A 25-year-old woman with a mass in the hippocampus. 2043 70

Nerve sheath myxoma (NSM) is a benign peripheral nerve sheath tumor that rarely occurs in the oral cavity. Among the 23 reported intraoral cases, no lesion has previously been reported on the gingiva. In this report, we describe the first gingival case of oral neurothekeoma with histopathologic and immunohistochemical characteristics. The patient, a 32 year old female presented with a slowly growing gingival mass diagnosed clinically as an epulis. The lesion was surgically excised. Histopathologically, the lesion presented as a submucosal multinodular mass composed of spindle and stellate-shaped cells with a myxoid background. Immunohistochemically, the tumor cells were sporadically positive for S-100 and NSE and negative for GFAP, EMA, SMA, CD68 and HMB45. The immunoprofile of this lesion confirmed a Schwann cell origin. The lesion was followed up for 10 months with no reports of recurrence.
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PMID:Nerve sheath myxoma (neurothekeoma) of the gingiva, a case report and review of the literature. 2050 96


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