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1. Kim WJ, Kim SR, Choe YH, Lee KY, Park SJ, Lee HB, Chung MJ, Jin GY, Lee YC: Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography. J Korean Med Sci; 2008 Dec;23(6):1121-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clear cell "sugar" tumor of the lung: a well-enhanced mass with an early washout pattern on dynamic contrast-enhanced computed tomography.
  • Clear cell tumor of the lung is a rare and very unusual benign pulmonary tumor.
  • As clear cell tumor of the lung contains abundant cytoplasmic glycogen, this tumor is called "sugar tumor".
  • We report a case of sugar tumor in a 64-yr-old man presenting as a round pulmonary nodule.
  • The patient underwent wedge resection for the tumor.
  • Pathologic examination, including immunohistochemical studies, revealed that the nodule was a benign clear cell tumor, so-called "sugar tumor".
  • Because only a small number of cases have been reported previously, clinical aspects, radiological characteristics on dynamic contrast-enhanced CT, and differential diagnosis of the tumor are not well established.
  • Herein we present a clear cell tumor of the lung and discuss the clinical, radiological, and pathological features of the tumor.
  • [MeSH-major] Lung Neoplasms / radiography. Perivascular Epithelioid Cell Neoplasms / radiography. Solitary Pulmonary Nodule / radiography. Tomography, X-Ray Computed
  • [MeSH-minor] Antigens, Neoplasm / metabolism. Diagnosis, Differential. Humans. Lung / radiography. Lung / surgery. Male. Melanoma-Specific Antigens. Middle Aged. Neoplasm Proteins / metabolism

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  • (PMID = 19119463.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ PMC2610653
  • [Keywords] NOTNLM ; Clear Cell Tumor / Dynamic Contrast-Enhanced CT / Lung / Sugar Tumor / Washout Pattern
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2. Mizobuchi T, Masahiro N, Iwai N, Kohno H, Okada N, Nakada S: Clear cell tumor of the lung: surgical and immunohistochemical findings. Gen Thorac Cardiovasc Surg; 2010 May;58(5):243-7
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  • [Title] Clear cell tumor of the lung: surgical and immunohistochemical findings.
  • We encountered a clear cell tumor of the lung (CCTL) that was located peripherally, adjacent to the visceral pleura.
  • The tumor could be directly observed during surgery.
  • The in vivo color of the tumor was red, suddenly changing to white after the tumor was clamped.
  • Immunohistochemistry revealed tumor cells positive for vimentin and melanocytic markers (HMB-45 and melan-A) and negative for epithelial membrane antigen and cytokeratin.
  • With the absence of clinical findings in both kidneys, the tumor was diagnosed as a benign CCTL.
  • [MeSH-major] Biomarkers, Tumor / analysis. Immunohistochemistry. Lung Neoplasms / chemistry. Lung Neoplasms / surgery. Perivascular Epithelioid Cell Neoplasms / chemistry. Perivascular Epithelioid Cell Neoplasms / surgery. Thoracic Surgery, Video-Assisted
  • [MeSH-minor] Aged. Antigens, Neoplasm / analysis. Female. Humans. Incidental Findings. MART-1 Antigen. Melanoma-Specific Antigens. Neoplasm Proteins / analysis. Tomography, X-Ray Computed. Treatment Outcome. Vimentin / analysis

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  • (PMID = 20449716.001).
  • [ISSN] 1863-6713
  • [Journal-full-title] General thoracic and cardiovascular surgery
  • [ISO-abbreviation] Gen Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / MART-1 Antigen; 0 / MLANA protein, human; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins; 0 / Vimentin
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3. Jha P, Moosavi C, Fanburg-Smith JC: Giant cell fibroblastoma: an update and addition of 86 new cases from the Armed Forces Institute of Pathology, in honor of Dr. Franz M. Enzinger. Ann Diagn Pathol; 2007 Apr;11(2):81-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • All cases had benign behavior, but almost half recurred.
  • The caveat was mistaking this tumor for a malignancy.
  • They proposed a relationship of this childhood tumor to dermatofibrosarcoma protuberans (DFSP).
  • Most remarkable is the peculiar perivascular lymphocytes in an onionskin pattern in GCF, not observed in DFSP.
  • Two cases of pure GCF recurred as a hybrid tumor with DFSP areas, one of these with hypercellular DFSP.
  • Additional observations of marked perivascular and onionskin-like chronic inflammation and consistent hemorrhage may aid in the diagnosis of this previously well-described tumor.
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Child. Child, Preschool. Female. History, 20th Century. Humans. Infant. Male. Middle Aged. Neoplasm Recurrence, Local / pathology. Registries

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  • (PMID = 17349565.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Biography; Historical Article; Journal Article; Portraits
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Personal-name-as-subject] Enzinger FM
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4. Bradshaw MJ, Folpe AL, Croghan GA: Perivascular epithelioid cell neoplasm of the uterine cervix: an unusual tumor in an unusual location. Rare Tumors; 2010;2(4):e56
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  • [Title] Perivascular epithelioid cell neoplasm of the uterine cervix: an unusual tumor in an unusual location.
  • Further review of the pathological material from the hysterectomy revealed a morphologically benign perivascular epithelioid cell neoplasm rather than a malignant melanoma.

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  • (PMID = 21234248.001).
  • [ISSN] 2036-3613
  • [Journal-full-title] Rare tumors
  • [ISO-abbreviation] Rare Tumors
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC3019591
  • [Keywords] NOTNLM ; gynecological neoplasms. / immunohistochemistry / melanoma / perivascular epithelioid cell neoplasm
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5. Laga AC, Tajirian AL, Islam MN, Bhattacharyya I, Cohen DM, Plamondon CJ, Robinson-Bostom L: Myopericytoma: report of two cases associated with trauma. J Cutan Pathol; 2008 Sep;35(9):866-70
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  • Myopericytoma is a rare, recently described tumor demonstrating a hemangiopericytoma-like vascular pattern.
  • Biopsy specimens of the lesions in both cases demonstrated a striking concentric perivascular proliferation of bland spindle-shaped pericytic cells characteristic of myopericytoma.
  • Despite sharing morphologic features with angioleiomyoma, myofibroma and glomus tumor, myopericytoma is thought to represent a distinct perivascular myoid neoplasm of skin and soft tissues.
  • The tumor is characterized by a radial and perivascular arrangement of ovoid, spindled to round neoplastic cells that are immunoreactive to alpha-smooth muscle actin, often for h-caldesmon as well as smooth muscle myosin-heavy chain, and usually negative for desmin antibodies.
  • Most cases of myopericytoma are benign, however, local recurrence and malignancy have recently been reported, Myopericytoma can be multifocal involving a single or multiple anatomic regions, and tends to occur in dermal and superficial soft tissues of adults primarily on the extremities.
  • [MeSH-minor] Actins / metabolism. Aged. Biomarkers, Tumor / metabolism. Calcium-Binding Proteins / metabolism. Female. Humans. Male. Microfilament Proteins / metabolism. Middle Aged. Mouth Mucosa / injuries. Myosin Heavy Chains / metabolism. Nose / injuries. Treatment Outcome

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  • (PMID = 18494828.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
  • [Chemical-registry-number] 0 / Actins; 0 / Biomarkers, Tumor; 0 / Calcium-Binding Proteins; 0 / Microfilament Proteins; 0 / calponin; EC 3.6.4.1 / Myosin Heavy Chains
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6. Parfitt JR, Bella AJ, Izawa JI, Wehrli BM: Malignant neoplasm of perivascular epithelioid cells of the liver. Arch Pathol Lab Med; 2006 Aug;130(8):1219-22
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  • [Title] Malignant neoplasm of perivascular epithelioid cells of the liver.
  • Neoplasms of perivascular epithelioid cells (PEComas) have in common the coexpression of muscle and melanocytic immunohistochemical markers.
  • Although this group includes entities with distinct clinical features, such as angiomyolipoma, clear cell sugar tumor of the lung, and lymphangioleiomyomatosis, similar tumors have been documented in an increasing diversity of locations.
  • While most reported PEComas have behaved in a benign fashion, malignant PEComas have occasionally been documented.
  • We present a case of hepatic PEComa with benign histologic features, which nonetheless presented with metastases to multiple sites nearly 9 years later.

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  • (PMID = 16879028.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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7. Juric-Sekhar G, Zarkovic K, Waeg G, Cipak A, Zarkovic N: Distribution of 4-hydroxynonenal-protein conjugates as a marker of lipid peroxidation and parameter of malignancy in astrocytic and ependymal tumors of the brain. Tumori; 2009 Nov-Dec;95(6):762-8
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  • The aim of this study was to evaluate the expression of HNE in 120 astrocytic and 40 ependymal tumors in relation to tumor type, grade of malignancy, angiogenesis, and presence of necrosis and apoptosis.
  • The incidence of HNE-immunopositive tumor cells increased with increasing grades of malignancy.
  • Significantly higher HNE expression was found in tumor cells of glioblastomas multiforme than in cells of pilocytic astrocytomas (P < 0.005), and in anaplastic ependymomas than in benign ependymomas (P < 0.01).
  • HNE-immunopositive tumor cells were distributed more diffusely than in perivascular locations (P < 0.05).
  • HNE was expressed in the endothelium of almost all tumor vessels, but its expression in the walls of the vessels was significantly higher in diffuse and anaplastic astrocytomas than in pilocytic astrocytomas and glioblastomas multiforme (P < 0.05).
  • [MeSH-major] Aldehydes / analysis. Astrocytoma / chemistry. Biomarkers, Tumor / analysis. Brain Neoplasms / chemistry. Brain Neoplasms / pathology. Ependymoma / chemistry. Lipid Peroxidation. Neoplasm Proteins / analysis

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  • (PMID = 20210242.001).
  • [ISSN] 0300-8916
  • [Journal-full-title] Tumori
  • [ISO-abbreviation] Tumori
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Aldehydes; 0 / Biomarkers, Tumor; 0 / Cross-Linking Reagents; 0 / Neoplasm Proteins; K1CVM13F96 / 4-hydroxy-2-nonenal
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8. El Jack AK, Tomaszewski JE, Haller DG, Siegelman ES: Metastatic PEComa arising from renal angiomyolipoma: MRI findings. J Magn Reson Imaging; 2007 Jul;26(1):159-61
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The perivascular epithelioid cell tumor (PEComa) is a neoplasm composed primarily of HMB-45-positive epithelioid cells with a perivascular distribution.
  • Both benign and malignant PEComas have been reported in diverse anatomic locations.

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  • [Copyright] Copyright 2007 Wiley-Liss, Inc.
  • (PMID = 17659554.001).
  • [ISSN] 1053-1807
  • [Journal-full-title] Journal of magnetic resonance imaging : JMRI
  • [ISO-abbreviation] J Magn Reson Imaging
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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9. Walsh SN, Sang├╝eza OP: PEComas: a review with emphasis on cutaneous lesions. Semin Diagn Pathol; 2009 Aug;26(3):123-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • The connection between angiomyolipoma (AML) of the kidney, clear cell sugar tumor (CCST) of the lung, and pulmonary lymphangioleiomyoma (LAM), was progressively discovered because of the histologic and immunophenotypic similarities between the three tumors and their frequent association with tuberous sclerosis complex (TSC).
  • Morphologically, analogous lesions found in other locations are composed of the unifying cell, the perivascular epithelioid cell (PEC).
  • Most reported cutaneous PEComas follow a benign course, however, a malignant case has been reported.
  • [MeSH-major] Desmin / metabolism. Epithelioid Cells / pathology. Perivascular Epithelioid Cell Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Actins / metabolism. Antigens, Neoplasm / metabolism. Biomarkers, Tumor / metabolism. Female. Humans. Melanoma-Specific Antigens. Middle Aged. Neoplasm Proteins / metabolism

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  • [CommentIn] Am J Dermatopathol. 2016 Feb;38(2):165-6 [26825164.001]
  • (PMID = 20043511.001).
  • [ISSN] 0740-2570
  • [Journal-full-title] Seminars in diagnostic pathology
  • [ISO-abbreviation] Semin Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Desmin; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
  • [Number-of-references] 65
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10. Arai A, Sasayama T, Tamaki M, Sakagami Y, Enoki E, Ohbayashi C, Kohmura E: Rosette-forming glioneuronal tumor of the fourth ventricle--case report. Neurol Med Chir (Tokyo); 2010;50(3):224-8
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  • [Title] Rosette-forming glioneuronal tumor of the fourth ventricle--case report.
  • Rosette-forming glioneuronal tumor (RGNT) of the fourth ventricle is a rare tumor included as a novel glioneuronal neoplasm in the 2007 World Health Organization classification of brain tumors.
  • The tumor was gross totally resected.
  • The neurocytic component consisted of uniform neurocytes forming neurocytic rosettes and perivascular pseudorosettes.
  • The prognosis is benign, but relatively aggressive behaviors such as tumor growth, recurrence, and acute deterioration due to intratumoral hemorrhaging can occur.

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  • (PMID = 20339273.001).
  • [ISSN] 1349-8029
  • [Journal-full-title] Neurologia medico-chirurgica
  • [ISO-abbreviation] Neurol. Med. Chir. (Tokyo)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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11. Batinac T, Zamolo G, Coklo M, Hadzisejdic I: Possible key role of granzyme B in keratoacanthoma regression. Med Hypotheses; 2006;66(6):1129-32
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • However, in some cases, otherwise typical keratoacanthoma can behave aggressively showing the signs of perineural and perivascular invasion and metastases in regional lymph nodes.
  • Within the past few years, it has become evident that the molecular events regulating cell survival and apoptosis are important contributors to the overall kinetics of benign and malignant cell growth.
  • Immunological mechanisms have been implicated in a phenomenon of spontaneous tumor regression.
  • Recent studies suggested that the tumor regression is dependent mainly on the immune response mediated by cytotoxic T lymphocytes (CD8+), together with helper T cells (CD4+).
  • Cytotoxic T lymphocytes can kill tumor cells and mediate tumor regression in vivo through two distinct molecular mechanisms: Fas/Fas ligand and granzyme B/perforin mediated pathways.
  • Tumor cells are capable of developing different escape mechanisms in order to overcome their sensitivity to apoptotic signals.
  • However, granzyme B, contained in cytolytic granules released upon target cell recognition, can also cause tumor cell death and consequently tumor regression by direct damage to non-nuclear structures through a caspase-independent pathway.
  • Therefore, we propose a key role of plasticity in the granzyme B mediated cell death pathway in the killing of changed tumor cells, resulting in keratoacanthoma regression through apoptosis or direct damage of tumor cells.
  • [MeSH-major] Carcinoma, Squamous Cell / immunology. Keratoacanthoma / immunology. Models, Immunological. Neoplasm Regression, Spontaneous / immunology. Serine Endopeptidases / immunology. Signal Transduction / immunology. T-Lymphocytes / immunology

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  • (PMID = 16497444.001).
  • [ISSN] 0306-9877
  • [Journal-full-title] Medical hypotheses
  • [ISO-abbreviation] Med. Hypotheses
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Scotland
  • [Chemical-registry-number] EC 3.4.21.- / GZMB protein, human; EC 3.4.21.- / Granzymes; EC 3.4.21.- / Serine Endopeptidases
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12. Sato K, Ueda Y, Miwa S, Yokogawa A, Ozaki M, Katsuda S: Low-grade malignant soft-tissue perineurioma: interphase fluorescence in situ hybridization. Pathol Int; 2008 Nov;58(11):718-22
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Perineuriomas are usually benign soft-tissue tumors that arise from perineurial cells of the peripheral nerve sheath.
  • This report describes a case of low-grade malignant perineurioma in a 60-year-old man who presented with a growing tumor on the dorsal side of his left wrist.
  • The tumor was surgically excised and showed no adhesion to the surrounding muscle and no continuity with nerves.
  • Histology indicated that the tumor contained hypercellular and hypocellular areas with spindle-shaped cells proliferating in storiform patterns or perivascular whorling.
  • On immunohistochemistry tumor cells were found to be positive for epithelial membrane antigen, glucose transporter protein 1, and claudin-1.
  • Approximately 18.4% of tumor nuclei were labelled for Ki-67.
  • [MeSH-minor] Biomarkers, Tumor / analysis. Cell Nucleus / chemistry. Cell Nucleus / pathology. Chromosome Aberrations. Chromosomes, Human, Pair 13. Claudin-1. DNA, Neoplasm / analysis. Glucose Transporter Type 1 / analysis. Humans. In Situ Hybridization, Fluorescence. Ki-67 Antigen / analysis. Male. Membrane Proteins / analysis. Middle Aged. Mitosis. Mucin-1 / analysis. Treatment Outcome. Wrist / surgery

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  • (PMID = 18844938.001).
  • [ISSN] 1440-1827
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / CLDN1 protein, human; 0 / Claudin-1; 0 / DNA, Neoplasm; 0 / Glucose Transporter Type 1; 0 / Ki-67 Antigen; 0 / Membrane Proteins; 0 / Mucin-1
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13. Hussein MR, Elsers DA, Fadel SA, Omar AE: Immunohistological characterisation of tumour infiltrating lymphocytes in melanocytic skin lesions. J Clin Pathol; 2006 Mar;59(3):316-24
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • We hypothesise that the transition from normal skin to benign naevi (BN) to melanocytic dysplastic naevi (MDN) to radial growth phase cutaneous malignant melanoma (RGP-CMM) to vertical growth phase cutaneous malignant melanoma (VGP-CMM) is associated with alterations in TIL.
  • RESULTS: Histologically, the transitions from normal skin to BN to MDN to RGP-CMM to VGP-CMM was associated with a gradual increase in the numbers of TIL (total, parenchymal, stromal, perivascular, and epidermal TIL, as well as TIL at the base of the lesions).
  • [MeSH-major] Biomarkers, Tumor / analysis. Lymphocytes, Tumor-Infiltrating / pathology. Melanocytes / pathology. Melanoma / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Analysis of Variance. Antigens, CD20 / analysis. Antigens, CD3 / analysis. Antigens, CD45 / analysis. Case-Control Studies. Disease Progression. Humans. Immunohistochemistry / methods. Immunophenotyping. Lymphocyte Count. Neoplasm Staging. Nevus / immunology. Nevus / pathology. T-Lymphocytes, Cytotoxic / pathology

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  • (PMID = 16505286.001).
  • [ISSN] 0021-9746
  • [Journal-full-title] Journal of clinical pathology
  • [ISO-abbreviation] J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD20; 0 / Antigens, CD3; 0 / Biomarkers, Tumor; EC 3.1.3.48 / Antigens, CD45
  • [Other-IDs] NLM/ PMC1860334
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14. Ma L, Kowalski D, Javed K, Hui P: Atypical angiomyolipoma of kidney in a patient with tuberous sclerosis: a case report with p53 gene mutation analysis. Arch Pathol Lab Med; 2005 May;129(5):676-9
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  • Angiomyolipoma (AML) is the most common benign mesenchymal tumor of the kidney.
  • It belongs to the family of perivascular epithelioid cell tumors and is typically composed of blood vessels, adipose tissue, and smooth muscle- like cells, which are characteristically positive for HMB-45.
  • The tumor consisted of mostly epithelioid cells with marked nuclear pleomorphism and frequent mitoses and was positive for HMB-45.
  • [MeSH-minor] Adult. Biomarkers, Tumor / analysis. Cytoplasm / ultrastructure. DNA Mutational Analysis. DNA, Neoplasm / genetics. Female. Humans. Immunohistochemistry. Intercellular Junctions / ultrastructure. Nephrectomy. Polymerase Chain Reaction. Polymorphism, Single-Stranded Conformational. Treatment Outcome


15. Mentzel T, Dei Tos AP, Sapi Z, Kutzner H: Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases. Am J Surg Pathol; 2006 Jan;30(1):104-13
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  • Perivascular neoplasms comprise traditionally glomus tumor and hemangiopericytoma (HPC).
  • Whereas glomus tumor represents a well-defined entity, the existence of HPC as a separate entity has been questioned because a number of neoplasms of different lines of differentiation are characterized by a HPC-like vascular growth pattern.
  • Histologically, in all cases, numerous thin-walled vessels and a concentric, perivascular arrangement of ovoid, plump spindled to round myoid tumor cells was seen.
  • Despite overlapping morphologic features to angioleiomyoma and myofibroma, myopericytoma represents a distinct perivascular, myoid neoplasm of skin and soft tissues, characterized by a broad morphologic spectrum of concentrically, perivascularly growing myoid tumor cells that stain positively for ASMA and often for h-caldesmon, whereas desmin is usually negative.
  • Most cases of myopericytoma behave in a benign fashion, but local recurrences and rarely metastases may occur in atypical and malignant neoplasms.
  • [MeSH-major] Biomarkers, Tumor / analysis. Neoplasms, Vascular Tissue / pathology. Skin Neoplasms / pathology. Soft Tissue Neoplasms / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Angiomyoma / pathology. Diagnosis, Differential. Female. Glomus Tumor / pathology. Hemangiopericytoma / metabolism. Hemangiopericytoma / pathology. Humans. Immunohistochemistry. Male. Middle Aged. Myofibroma / pathology

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  • (PMID = 16330949.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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16. Yang YP, Zhu YL, Wu WP, Wang ZM, Zhang JM: [Perivascular epithelioid cell tumor of uterus: report of 5 cases and literature review]. Zhonghua Bing Li Xue Za Zhi; 2007 May;36(5):302-6
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  • [Title] [Perivascular epithelioid cell tumor of uterus: report of 5 cases and literature review].
  • OBJECTIVE: To study the pathologic features, diagnosis, differential diagnosis and biologic behavior of uterine perivascular epithelioid tumor.
  • METHODS: Five cases of uterine perivascular epithelioid cell tumor were studied by light microscopy and immunohistochemistry.
  • Immunohistochemically, the tumor cells demonstrated positive staining for melanocytic markers (HMB45 and/or Melan-A), desmin and smooth muscle actin.
  • CONCLUSIONS: Perivascular epithelioid cell tumor is a rare mesenchymal tumor of uterus, with distinctive histologic and immunohistochemical features.
  • Positivity for melanocytic markers (especially HMB45) plays an important role in the diagnosis of this tumor.
  • In general, the tumor is categorized as benign, with uncertain malignant potential and malignant.
  • [MeSH-major] Antigens, Neoplasm / metabolism. Neoplasm Proteins / metabolism. Perivascular Epithelioid Cell Neoplasms / pathology. Uterine Neoplasms / pathology

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  • (PMID = 17706136.001).
  • [ISSN] 0529-5807
  • [Journal-full-title] Zhonghua bing li xue za zhi = Chinese journal of pathology
  • [ISO-abbreviation] Zhonghua Bing Li Xue Za Zhi
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Desmin; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
  • [Number-of-references] 24
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17. Ghazali N, Cascarini L, Norris P, Barrett AW, Lavery KM: Perivascular epithelioid cell tumor (PEComa) of the cheek. Oral Surg Oral Med Oral Pathol Oral Radiol Endod; 2010 Jul;110(1):e26-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Perivascular epithelioid cell tumor (PEComa) of the cheek.
  • We present the unusual case of a perivascular epithelioid cell tumor (PEComa) occurring within the cheek of a 32-year-old woman.
  • Other possible diagnoses considered included benign mesenchymal tumors of smooth muscle or neural origin.
  • The tumor was completely excised, but in view of uncertainty as to how this entity would behave in an unusual location, lifelong follow up is recommended.
  • [MeSH-major] Cheek / pathology. Facial Neoplasms / diagnosis. Perivascular Epithelioid Cell Neoplasms / diagnosis. Soft Tissue Neoplasms / diagnosis
  • [MeSH-minor] Actins / analysis. Adult. Antigens, Neoplasm / analysis. Arterioles / pathology. Diagnosis, Differential. Epithelioid Cells / pathology. Female. Follow-Up Studies. Humans. MART-1 Antigen. Muscle, Smooth, Vascular / pathology. Neoplasm Proteins / analysis

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  • [Copyright] Copyright (c) 2010 Mosby, Inc. All rights reserved.
  • (PMID = 20610292.001).
  • [ISSN] 1528-395X
  • [Journal-full-title] Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics
  • [ISO-abbreviation] Oral Surg Oral Med Oral Pathol Oral Radiol Endod
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, Neoplasm; 0 / MART-1 Antigen; 0 / MLANA protein, human; 0 / Neoplasm Proteins
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18. Oba M, Sasaki M, Ii T, Hoso M, Ajisaka H, Matsuki N, Miwa K: A case of lymphocytic mastopathy requiring differential diagnosis from primary breast lymphoma. Breast Cancer; 2009;16(2):141-6
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  • Lymphocytic mastopathy is a benign breast disease characterized by dense fibrosis, lobular atrophy, and aggregates of lymphocytes in a periductal and perivascular distribution.
  • [MeSH-minor] Diagnosis, Differential. Female. Fluorodeoxyglucose F18. Humans. Magnetic Resonance Imaging. Mammography. Middle Aged. Neoplasm Staging. Positron-Emission Tomography. Prognosis. Radiopharmaceuticals. Tomography, X-Ray Computed. Ultrasonography, Mammary

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  • (PMID = 18769994.001).
  • [ISSN] 1880-4233
  • [Journal-full-title] Breast cancer (Tokyo, Japan)
  • [ISO-abbreviation] Breast Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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19. Zimmermann A, von der Brelie C, Berger B, Kappeler A, Candinas D: Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity. Histol Histopathol; 2008 10;23(10):1185-93
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  • [Title] Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: hepatic PEComa as an emerging entity.
  • Primary perivascular epithelioid cell tumor (PEComa) of the liver is a very rare example of an emerging family of hepatic PEC tumors.
  • We report the case of a large but benign hepatic PEComa in a 53-year-old man without signs of tuberous sclerosis.
  • In contrast to recently described PEC-derived liver tumors in children and young adults, this neoplasm was not related to the hepatic ligaments but had developed deeply within the liver substance.
  • The unique relationship of myoid tumor cells to the adventitia of blood vessels prompted us, in comparison with published findings obtained with angiomyolipomas, to comment on the possible origin of the still enigmatic perivascular epithelioid cells.

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  • (PMID = 18712670.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Spain
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20. Chaplin A, Conrad DM, Tatlidil C, Jollimore J, Walsh N, Covert A, Pasternak S: Primary cutaneous PEComa. Am J Dermatopathol; 2010 May;32(3):310-2
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  • Microscopic examination of the excisional specimen revealed a dermal tumor composed of nests of epithelioid cells exhibiting clear cytoplasm.
  • The tumor showed an infiltrative border.
  • Immunohistochemical evaluation revealed that the tumor cells were positive for HMB-45 and microftalmia associated transcription factor (MITF).
  • The tumor cells were negative for S-100 protein, alfa smooth muscle actin, HHF-35, and various cytokeratins.
  • Pecomas are rare, recently described mesenchymal tumors composed of perivascular epithelioid cells.
  • They constitute a spectrum of lesions in different organs including angiomyolipoma of the kidney and liver, sugar tumor of the lung, lymphangiomatosis, and lymphangiomyoma.
  • Follow-up data is limited but they appear to behave in a benign fashion.
  • We report an additional case with the goal of alerting dermatopathologists to this distinctive unusual neoplasm.
  • [MeSH-major] Perivascular Epithelioid Cell Neoplasms / pathology. Skin Neoplasms / pathology
  • [MeSH-minor] Antigens, Neoplasm / analysis. Biomarkers, Tumor / analysis. Biopsy. Desmin / analysis. Female. Humans. Leg. Melanoma-Specific Antigens. Microphthalmia-Associated Transcription Factor / analysis. Middle Aged. Neoplasm Proteins / analysis. Treatment Outcome

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  • (PMID = 20139753.001).
  • [ISSN] 1533-0311
  • [Journal-full-title] The American Journal of dermatopathology
  • [ISO-abbreviation] Am J Dermatopathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Desmin; 0 / MITF protein, human; 0 / Melanoma-Specific Antigens; 0 / Microphthalmia-Associated Transcription Factor; 0 / Neoplasm Proteins
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21. Bougrine F, Bacha D, Chouchane O, Laabidi B, Yeades M, Bouziani A: [Intracerebral schwannoma: case report]. Neurochirurgie; 2007 Nov;53(5):387-90
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  • The tumor was removed through a right parietal craniotomy.
  • The most popular hypothesis argues that these tumors arise from the proliferation of Schwann cells in the perivascular nerve plexii.
  • CONCLUSIONS: Intracerebral schwannoma is an extremely rare benign tumor.
  • The importance of recognizing this tumor is stressed, particularly in younger patients, given its benign nature, radiological resemblance to other tumors and favorable response to resection without toxic treatment.
  • [MeSH-minor] Adult. Female. Humans. Immunohistochemistry. Intracranial Hypertension / etiology. Magnetic Resonance Imaging. Neoplasm Metastasis. Neurosurgical Procedures. Seizures / etiology

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  • (PMID = 17884108.001).
  • [ISSN] 0028-3770
  • [Journal-full-title] Neuro-Chirurgie
  • [ISO-abbreviation] Neurochirurgie
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] France
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22. Nonomura A, Enomoto Y, Takeda M, Tamura T, Kasai T, Yosikawa T, Nakamime H: Invasive growth of hepatic angiomyolipoma; a hitherto unreported ominous histological feature. Histopathology; 2006 Jun;48(7):831-5
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  • The other consisted of portal or perivascular invasive growth along blood vessels.
  • However, among about 180 reported hepatic AMLs, including the present cases, only one hepatic AML with distant metastasis has been reported to date and nearly all hepatic AMLs behave in a benign fashion.
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Antigens, Neoplasm. Female. Humans. Immunohistochemistry. Male. Melanoma-Specific Antigens. Middle Aged. Neoplasm Invasiveness. Neoplasm Proteins / analysis

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  • (PMID = 16722932.001).
  • [ISSN] 0309-0167
  • [Journal-full-title] Histopathology
  • [ISO-abbreviation] Histopathology
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
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23. Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW: Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol; 2005 Dec;29(12):1558-75
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  • [Title] Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.
  • PEComas, occasionally associated with the tuberous sclerosis complex, are defined by the presence of perivascular epithelioid cells that coexpress muscle and melanocytic markers.
  • This family of tumors includes angiomyolipoma (AML), clear cell sugar tumor of the lung (CCST), lymphangioleiomyomatosis (LAM), and very rare tumors in other locations.
  • Recurrence and/or metastasis was strongly associated tumor size > median size (8 cm), mitotic activity greater than 1/50 HPF, and necrosis.
  • We conclude that PEComas of soft tissue and gynecologic origin may be classified as "benign," "of uncertain malignant potential," or "malignant."
  • Small PEComas without any worrisome histologic features are most likely benign.
  • [MeSH-major] Biomarkers, Tumor / analysis. Epithelioid Cells / pathology. Genital Neoplasms, Female / pathology. Neoplasms, Connective and Soft Tissue / pathology
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Disease-Free Survival. Female. Follow-Up Studies. Humans. Immunohistochemistry. Immunophenotyping. Male. Middle Aged. Mitosis. Neoplasm Metastasis. Neoplasm Recurrence, Local. Retrospective Studies. Survival Analysis. Time Factors. Treatment Outcome. Tumor Burden

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  • (PMID = 16327428.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 56
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24. Rousseau A, Kujas M, van Effenterre R, Boch AL, Carpentier A, Leroy JP, Poirier J: Primary intracranial myopericytoma: report of three cases and review of the literature. Neuropathol Appl Neurobiol; 2005 Dec;31(6):641-8
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  • Myopericytoma is a benign tumour generally arising in the subcutaneous and superficial soft tissues of the extremities.
  • Histological analysis revealed oval-to-spindle shaped myoid-appearing cells with a striking tendency for concentric perivascular growth.
  • The lesional cells showed apparent differentiation towards perivascular myoid cells as witnessed by smooth muscle actin expression.
  • Myopericytoma is a recently described neoplasm, and it is likely that reappraisal of intracranial haemangiopericytoma with which it shares many histopathologic features will lead to more case reports of primary intracranial myopericytoma.

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  • (PMID = 16281913.001).
  • [ISSN] 0305-1846
  • [Journal-full-title] Neuropathology and applied neurobiology
  • [ISO-abbreviation] Neuropathol. Appl. Neurobiol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 24
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25. Rathi KR, Jena J, Dash BM, Mitra D, Patnaik PK, Basu AR: Extradigital glomus tumor as a cause of chronic perianal pain. Indian J Pathol Microbiol; 2009 Jul-Sep;52(3):414-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Extradigital glomus tumor as a cause of chronic perianal pain.
  • Glomus tumor is a rare perivascular benign tumor arising from the Sucquet-Hoyer canal of the normal glomus body, most commonly in the digital areas.
  • We report a serving soldier with such a tumor in an atypical site, the perianal region, presenting with episodic shooting pain.
  • Histopathology revealed a well-circumscribed tumor composed of clusters of monotonous polygonal cells surrounding capillary-sized blood vessels.
  • Tumor cells also showed immunopositivity for smooth muscle antigen and vimentin.
  • [MeSH-major] Anus Neoplasms / diagnosis. Anus Neoplasms / pathology. Glomus Tumor / diagnosis. Glomus Tumor / pathology

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  • (PMID = 19679979.001).
  • [ISSN] 0974-5130
  • [Journal-full-title] Indian journal of pathology & microbiology
  • [ISO-abbreviation] Indian J Pathol Microbiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Vimentin
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26. Strzelczyk JM, Durczynski A, Szymanski D, Jablkowski M, Dworniak D, Sporny S: Primary perivascular epithelioid cell tumor (PEComa) of the liver: report of a case. Surg Today; 2009;39(10):916-21
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  • [Title] Primary perivascular epithelioid cell tumor (PEComa) of the liver: report of a case.
  • PEComa is very rare mesenchymal neoplasm which is formed by perivascular epithelioid cells and is characterized by dual melanocytic and myoid differentiation.
  • We herein present a patient who underwent a right hemihepatectomy for a huge tumor which could not be identified by imaging investigations.
  • A final histopathologic examination revealed a benign epithelioid tumor with a solid growth pattern, abundant vascularity, and frequently dilated vascular channels.
  • Immunohistochemically, the tumor cells were strongly positive for HMB-45, moderately positive for actin, and faintly positive for S-100, respectively.
  • Based on the above findings, a diagnosis of a primary clear cell "sugar" tumor was established.
  • [MeSH-major] Liver Neoplasms / pathology. Perivascular Epithelioid Cell Neoplasms / pathology

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  • (PMID = 19784736.001).
  • [ISSN] 1436-2813
  • [Journal-full-title] Surgery today
  • [ISO-abbreviation] Surg. Today
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Japan
  • [Number-of-references] 24
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27. Zhi L, Bing L, Yang L, Bo-ning L, Quan H: Cystic papillary meningioma with subarachnoid dissemination: a case report and review of the literature. Pathol Res Pract; 2009;205(8):582-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Meningiomas usually present as benign tumors corresponding to WHO grade I.
  • The tumor exhibits a marked peritumoral cyst, with contrast enhancement on magnetic resonance imaging (MRI) in accordance with type 2 of Zee's classification of cystic meningioma.
  • Histologically, the tumor displays a classical perivascular pseudopapillary pattern with focal necrosis and subarachnoid space dissemination.
  • Tumor cells are diffusely positive for epithelial membrane antigen (EMA) and vimentin, but lack immunoreactivity for cytokeratin (CK) and glial fibrillary acidic protein (GFAP).
  • MIB-1 labeling is high, accounting for 5% of tumor focally.
  • In addition, the biological behavior and the clinical outcome of this tumor are also discussed.
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Female. Humans. Magnetic Resonance Imaging. Mucin-1 / metabolism. Neoplasm Staging. Treatment Outcome. Vimentin / metabolism. Young Adult

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  • (PMID = 19307065.001).
  • [ISSN] 1618-0631
  • [Journal-full-title] Pathology, research and practice
  • [ISO-abbreviation] Pathol. Res. Pract.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Mucin-1; 0 / Vimentin
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28. Edelweiss M, Gupta N, Resetkova E: Preoperative diagnosis of clear cell "sugar" tumor of the lung by computed tomography-guided fine-needle biopsy and core-needle biopsy. Ann Diagn Pathol; 2007 Dec;11(6):421-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Preoperative diagnosis of clear cell "sugar" tumor of the lung by computed tomography-guided fine-needle biopsy and core-needle biopsy.
  • Clear cell "sugar" tumor (CCST) of the lung is a very rare benign neoplasm believed to arise from epithelioid cells of perivascular differentiation.
  • This tumor is typically diagnosed by thoracotomy.

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  • (PMID = 18022127.001).
  • [ISSN] 1092-9134
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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29. Nguyen TT, Gorman B, Shields D, Goodman Z: Malignant hepatic angiomyolipoma: report of a case and review of literature. Am J Surg Pathol; 2008 May;32(5):793-8
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  • Hepatic angiomyolipoma, a constituent of the group of tumors showing differentiation resembling perivascular epithelioid cells, is primarily appreciated in its benign form.
  • Here, we present a case of malignant hepatic perivascular epithelioid cells that was initially confined to the liver, treated by resection, and subsequently recurred within and metastasized beyond the liver.
  • We will discuss the features that aid in distinguishing between benign and malignant HAML, and their similarities.
  • In summary, the common features of both benign and malignant HAML include the following: the 3 basic histologic components of AML, expression of melanocytic and smooth muscle markers, invasion into adjacent normal parenchyma, and cytologic atypia.
  • [MeSH-minor] Adipocytes / chemistry. Adipocytes / pathology. Adult. Antigens, Neoplasm / analysis. Biomarkers, Tumor / analysis. Fatal Outcome. Female. Hepatectomy. Humans. Melanoma-Specific Antigens. Neoplasm Metastasis. Neoplasm Proteins / analysis. Neoplasm Recurrence, Local

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  • (PMID = 18391749.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Biomarkers, Tumor; 0 / Melanoma-Specific Antigens; 0 / Neoplasm Proteins
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