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1. Zarbis N, Barth TF, Blumstein NM, Schelzig H: Pecoma of the lung: a benign tumor with extensive 18F-2-deoxy-D-glucose uptake. Interact Cardiovasc Thorac Surg; 2007 Oct;6(5):676-8
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pecoma of the lung: a benign tumor with extensive 18F-2-deoxy-D-glucose uptake.
  • A case of lung pecoma (i.e. tumors showing perivascular epithelioid cell differentiation) with extensive 18F-2-deoxy-D-glucose (FDG) uptake in PET/CT study is reported.
  • Pecomas of the lung--which include the better known clear cell 'sugar' tumor--are a subset of extremely rare lung tumors which usually react positively to both melanocytic and smooth muscle markers.
  • Although widely presumed as benign in computed tomography (CT) and positron emission tomography (PET)/CT studies they depict as malignant, thus complicating the preoperative diagnosis.

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  • (PMID = 17670735.001).
  • [ISSN] 1569-9285
  • [Journal-full-title] Interactive cardiovascular and thoracic surgery
  • [ISO-abbreviation] Interact Cardiovasc Thorac Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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2. Tsai CC, Wu WJ, Li CC, Wang CJ, Wu CH, Wu CC: Epithelioid angiomyolipoma of the kidney mimicking renal cell carcinoma: a clinicopathologic analysis of cases and literature review. Kaohsiung J Med Sci; 2009 Mar;25(3):133-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Classical renal angiomyolipoma (AML) is a common tumor that, in most cases, follows a benign course and has clearly defined radiologic and histological characteristics.
  • In all cases, renal lesions seen on computed tomography (CT) were considered as suspicious for renal cell carcinoma (RCC) without tumor extension or metastasis.
  • The final diagnosis is established by the presence of perivascular epithelioid cells and immunohistochemistry markers.

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  • (PMID = 19419918.001).
  • [ISSN] 1607-551X
  • [Journal-full-title] The Kaohsiung journal of medical sciences
  • [ISO-abbreviation] Kaohsiung J. Med. Sci.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] China (Republic : 1949- )
  • [Number-of-references] 70
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3. Adachi Y, Horie Y, Kitamura Y, Nakamura H, Taniguchi Y, Miwa K, Fujioka S, Nishimura M, Hayashi K: CD1a expression in PEComas. Pathol Int; 2008 Mar;58(3):169-73
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • According to the World Health Organization classification, neoplasms with perivascular epithelioid cell differentiation (PEComas) are mesenchymal tumors composed of histologically and immunohistochemically distinctive PEC.
  • Recently the authors reported that benign clear cell sugar tumor of the lung, one of the PEComas, expressed CD1a.
  • Nineteen PEComas were obtained, which included angiomyolipoma of the kidney or liver, lymphangiomyomatosis of the uterus or lung and clear cell sugar tumor of the lung.
  • [MeSH-major] Antigens, CD1 / metabolism. Biomarkers, Tumor / metabolism. Epithelioid Cells / metabolism. Neoplasms, Connective and Soft Tissue / metabolism

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  • (PMID = 18251780.001).
  • [ISSN] 1440-1827
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antigens, CD1; 0 / Biomarkers, Tumor; 0 / CD1a antigen
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4. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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5. Messing-Jünger AM, Riemenschneider MJ, Reifenberger G: A 21-year-old female with a third ventricular tumor. Brain Pathol; 2006 Jan;16(1):87-8, 93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A 21-year-old female with a third ventricular tumor.
  • Intraoperatively, the tumor showed a close relationship to the choroid plexus of the third ventricle.
  • Histopathology revealed a benign schwannoma of World Health Organization grade I.
  • Most of these tumors were benign schwannomas, except for 2 cases of malignant peripheral nerve sheath tumors.
  • The tumor of our patient is the first reported schwannoma of the third ventricle.
  • They may arise from autonomic perivascular nerves in the choroid plexus or from ectopic neural crest-derived cells.

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  • (PMID = 16612987.001).
  • [ISSN] 1015-6305
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Switzerland
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6. Policarpio-Nicolas ML, Covell J, Bregman S, Atkins K: Fine needle aspiration cytology of clear cell "sugar" tumor (PEComa) of the lung: report of a case. Diagn Cytopathol; 2008 Feb;36(2):89-93
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Fine needle aspiration cytology of clear cell "sugar" tumor (PEComa) of the lung: report of a case.
  • PEComa (clear cell "sugar" tumor) of the lung is a rare benign tumor of the lung probably arising from the perivascular epithelioid cells (PECs).
  • In this case report, the clinical, cytologic and immunohistochemical features clear cell "sugar" tumor of the lung are discussed and compared with the previously published literature.

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18181192.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 9005-79-2 / Glycogen
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7. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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8. Kim HS, Kim GY, Lim SJ, Ki KD, Kim HC: Giant superficial angiomyxoma of the vulva: a case report and review of the literature. J Cutan Pathol; 2010 Jun;37(6):672-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis.
  • Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma.
  • There was no perivascular accentuation of stromal cells or smooth muscle bundles.
  • The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin.

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  • (PMID = 19615026.001).
  • [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
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9. 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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10. 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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11. Gesundheit B, Parkin P, Greenberg M, Baruchel S, Senger C, Kapelushnik J, Smith C, Klement GL: The role of angiogenesis in the transformation of plexiform neurofibroma into malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. J Pediatr Hematol Oncol; 2010 Oct;32(7):548-53
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • PURPOSE: The role of angiogenesis in the transformation of peripheral neurofibroma (PNF) to malignant peripheral nerve sheath tumor (MPNST) in neurofibromatosis type 1 (NF1) remains elusive and forms the objective of this study.
  • In NF and PNF tumor cells were VEGF-, in contrast to VEGF+ tumor cells in MPNST.
  • If present, the VEGF stain was confined mainly to the perivascular spaces in PNF, unlike the mainly stromal VEGF stain in MPNST.
  • VEGF receptors also manifested a tumor stage-specific pattern.
  • It permits a pair-wise evaluation of the malignant conversion of benign PNF into its malignant counterpart, in the same patients.

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  • (PMID = 20686424.001).
  • [ISSN] 1536-3678
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; 0 / von Willebrand Factor; EC 2.7.10.1 / FLT1 protein, human; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2
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12. Wu JK, Adepoju O, De Silva D, Baribault K, Boscolo E, Bischoff J, Kitajewski J: A switch in Notch gene expression parallels stem cell to endothelial transition in infantile hemangioma. Angiogenesis; 2010 Mar;13(1):15-23
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Infantile hemangioma (IH) is the most common benign tumor of infancy, yet its pathogenesis is poorly understood.
  • Notch family members are known to play a role in vascular development during embryogenesis and postnatal tumor angiogenesis, yet the role of Notch signaling in the pathogenesis of IH has not been investigated.
  • Notch3 was localized to the interstitial cells outside of the nascent vascular channels in proliferating IH tissue sections, but became more apparent in the perivascular cells in involuting IH.

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  • (PMID = 20069356.001).
  • [ISSN] 1573-7209
  • [Journal-full-title] Angiogenesis
  • [ISO-abbreviation] Angiogenesis
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / HL096384-01; United States / NCI NIH HHS / CA / R01 CA136673; United States / NHLBI NIH HHS / HL / R01 HL096384; United States / NHLBI NIH HHS / HL / R01 HL096384-01
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD31; 0 / Calcium-Binding Proteins; 0 / Intercellular Signaling Peptides and Proteins; 0 / Membrane Proteins; 0 / RNA, Messenger; 0 / Receptors, Notch; 134324-36-0 / Serrate proteins
  • [Other-IDs] NLM/ NIHMS176679; NLM/ PMC2846229
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13. Caffo M, Caruso G, Germanò A, Galatioto S, Meli F, Tomasello F: CD68 and CR3/43 immunohistochemical expression in secretory meningiomas. Neurosurgery; 2005 Sep;57(3):551-7; discussion 551-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • OBJECTIVE: Secretory meningiomas (SMs) are unusual benign meningiomas.
  • SMs are highly vascularized lesions, with angiomatous features and a perivascular arrangement, and they are accompanied frequently by massive peritumoral edema.
  • Immunohistochemistry was used to analyze the expression of CD68 and CR3/43 in tumor specimens.
  • RESULTS: CD68-positive mononuclear cells were observed in neoplastic tissue or around pseudopsammoma bodies and in perivascular areas, with minimal expression in one patient and moderate expression in three patients.

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  • (PMID = 16145535.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, Differentiation, Myelomonocytic; 0 / CD68 antigen, human; 0 / Macrophage-1 Antigen
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14. Rongioletti F, De Lucchi S, Meyes D, Mora M, Rebora A, Zupo S, Cerruti G, Patterson JW: Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol; 2010 Jan;37(1):15-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis.
  • As for histopathological findings, large cystic spaces filled with mucin and a slight perivascular and periadnexal polyclonal infiltrate of mostly non-atypical lymphocytes without epidermotropism and with an equivalent CD4+/CD8+ cell rate were more suggestive of PFM.
  • [MeSH-minor] Adolescent. Adult. Biomarkers, Tumor / metabolism. Child. Diagnosis, Differential. Female. Gene Rearrangement. Humans. Immunohistochemistry. In Situ Hybridization, Fluorescence. Male. Middle Aged. Mucins / metabolism. Young Adult


15. Ruan J, Hyjek E, Kermani P, Christos PJ, Hooper AT, Coleman M, Hempstead B, Leonard JP, Chadburn A, Rafii S: Magnitude of stromal hemangiogenesis correlates with histologic subtype of non-Hodgkin's lymphoma. Clin Cancer Res; 2006 Oct 1;12(19):5622-31
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  • PURPOSE: Tumor stromal microenvironment promotes neoplastic growth and angiogenesis.
  • EXPERIMENTAL DESIGN: Spatial localizations of vascular and stromal cells expressing CD34, VEGFR-1, alpha-SMA, and CD68 were examined by immunohistochemistry in 42 cases of non-Hodgkin's lymphoma, including diffuse large B-cell lymphoma, Burkitt lymphoma, follicular lymphoma, and chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), and compared with benign follicular hyperplasia.
  • RESULTS: Compared with indolent lymphomas, there was a profound increase in recruitment of CD68(+) cells and VEGFR-1(+) neovessels in aggressive subtypes (including those transformed from indolent subtypes), where CD68(+) cells were localized to the perivascular region of neovessels as well as the stromal compartment.
  • The perivascular CD68(+) cells expressed VEGFR-1 and VEGF-A.
  • In contrast, there was a diffuse increase in alpha-SMA incorporation throughout the stromal compartment of indolent subtype of CLL/SLL compared with the scant perivascular pattern in aggressive subtypes.

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  • (PMID = 17020964.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Grant] United States / NHLBI NIH HHS / HL / HL 075234; United States / NHLBI NIH HHS / HL / HL 59312; United States / NHLBI NIH HHS / HL / HL 67839; United States / NCRR NIH HHS / RR / K23 RR 016814
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antigens, CD; 0 / Antigens, CD34; 0 / Antigens, Differentiation, Myelomonocytic; 0 / CD68 antigen, human; 0 / VEGFA protein, human; 0 / Vascular Endothelial Growth Factor A; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2
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16. Herawi M, Parwani AV, Edlow D, Smolev JK, Epstein JI: Glomus tumor of renal pelvis: a case report and review of the literature. Hum Pathol; 2005 Mar;36(3):299-302
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  • [Title] Glomus tumor of renal pelvis: a case report and review of the literature.
  • Glomus tumors are uncommon benign perivascular neoplasms that have rarely been described outside of their usual peripheral soft tissue sites.
  • We report a unique case of glomus tumor of the renal pelvis in a 53-year-old woman who presented with microscopic hematuria associated with obstruction of the ureteropelvic junction and marked hydronephrosis.
  • At initial gross examination, the tumor mimicked a urothelial carcinoma.
  • [MeSH-major] Glomus Tumor / diagnosis. Kidney Neoplasms / diagnosis. Kidney Pelvis

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  • (PMID = 15791575.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 13
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17. Sugita Y, Ono T, Ohshima K, Niino D, Ito M, Toda K, Baba H: Brain surface spindle cell glioma in a patient with medically intractable partial epilepsy: a variant of monomorphous angiocentric glioma? Neuropathology; 2008 Oct;28(5):516-20
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  • At surgery, a yellowish tumor was localized in the superficial cortex.
  • Histologically, the tumor was predominantly composed of elongated astrocytic cells forming rings around blood vessels.
  • Tumor cells circumferential to vessels predominanted in low cellurarity areas, whereas radial alignment with perivascular pseudorosettes was observed in more cellular regions.
  • These perivascular pseudorosettes closely resembled those of ependymoma.
  • The tumor cells showed variable cytoplasmic immunoreactivity with GFAP.
  • However, in our case the tumor had a small foci of polymorphous appearance and a comparatively high MIB-1 labeling index (8%).
  • It remains to be determined whether the behavior of monomorphous angiocentric glioma is an example of benign biological characteristics or whether it more closely resembles a low-grade malignant tumor.

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  • (PMID = 18179412.001).
  • [ISSN] 1440-1789
  • [Journal-full-title] Neuropathology : official journal of the Japanese Society of Neuropathology
  • [ISO-abbreviation] Neuropathology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
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18. Lim SD, Stallcup W, Lefkove B, Govindarajan B, Au KS, Northrup H, Lang D, Fisher DE, Patel A, Amin MB, Arbiser JL: Expression of the neural stem cell markers NG2 and L1 in human angiomyolipoma: are angiomyolipomas neoplasms of stem cells? Mol Med; 2007 Mar-Apr;13(3-4):160-5
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  • Angiomyolipomas are benign tumors of the kidney which express phenotypes of smooth muscle, fat, and melanocytes.
  • While benign, these tumors are capable of provoking life threatening hemorrhage and replacement of the kidney parenchyma, resulting in renal failure.
  • The histogenesis of these tumors is currently unclear, although currently, we believe these tumors arise from "perivascular epithelioid cells" of which no normal counterpart has been convincingly demonstrated.
  • Immunohistochemistry of human angiomyolipoma specimens revealed uniform staining of tumor cells, while renal cell carcinomas revealed positivity only of angiogenic vessels.

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  • (PMID = 17592550.001).
  • [ISSN] 1076-1551
  • [Journal-full-title] Molecular medicine (Cambridge, Mass.)
  • [ISO-abbreviation] Mol. Med.
  • [Language] ENG
  • [Grant] United States / NIAMS NIH HHS / AR / P30 AR 42687; United States / NIAMS NIH HHS / AR / R01 AR050727; United States / NIAMS NIH HHS / AR / P30 AR042687; United States / NIAMS NIH HHS / AR / R01AR 47901; United States / NIAMS NIH HHS / AR / R01 AR 050727; United States / NIAMS NIH HHS / AR / R01 AR047901
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens; 0 / Biomarkers, Tumor; 0 / Leukocyte L1 Antigen Complex; 0 / Proteoglycans; 0 / chondroitin sulfate proteoglycan 4
  • [Other-IDs] NLM/ PMC1892760
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19. Lott S, Lopez-Beltran A, Maclennan GT, Montironi R, Cheng L: Soft tissue tumors of the urinary bladder, Part I: myofibroblastic proliferations, benign neoplasms, and tumors of uncertain malignant potential. Hum Pathol; 2007 Jun;38(6):807-23
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  • [Title] Soft tissue tumors of the urinary bladder, Part I: myofibroblastic proliferations, benign neoplasms, and tumors of uncertain malignant potential.
  • These include both benign and malignant spindle cell lesions.
  • The first half of this 2-part review will describe benign myofibroblastic proliferations including inflammatory myofibroblastic tumor and postoperative spindle cell nodule; benign neoplasms including leiomyoma, hemangioma, neurofibroma, and schwannoma; and tumors of uncertain malignant potential including paraganglioma, granular cell tumor, and perivascular epithelioid cell tumor.
  • This review also describes current theories as to the pathogenesis of inflammatory myofibroblastic tumor and postoperative spindle cell nodule and details the current molecular markers identifying several of these lesions.
  • [MeSH-minor] Animals. Biomarkers, Tumor / analysis. Diagnosis, Differential. Humans. Immunohistochemistry

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  • (PMID = 17509394.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
  • [Number-of-references] 87
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20. Atassi B, Bangash AK, Bahrani A, Pizzi G, Lewandowski RJ, Ryu RK, Sato KT, Gates VL, Mulcahy MF, Kulik L, Miller F, Yaghmai V, Murthy R, Larson A, Omary RA, Salem R: Multimodality imaging following 90Y radioembolization: a comprehensive review and pictorial essay. Radiographics; 2008 Jan-Feb;28(1):81-99
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  • The classic indication of treatment response is a reduction in tumor size; however, parenchymal changes (eg, necrosis, lack of enhancement, specific findings at positron emission tomography and functional magnetic resonance imaging) and other benign findings (pleural effusions, perivascular edema, contralateral hypertrophy, ring enhancement, perihepatic fluid, fibrosis) may occur following treatment, requiring proper image interpretation.

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  • (PMID = 18203932.001).
  • [ISSN] 1527-1323
  • [Journal-full-title] Radiographics : a review publication of the Radiological Society of North America, Inc
  • [ISO-abbreviation] Radiographics
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0 / Yttrium Radioisotopes
  • [Number-of-references] 64
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21. Omori M, Toyoda H, Hirai T, Ogino T, Okada S: Angiomyofibroblastoma of the vulva: a large pedunculated mass formation. Acta Med Okayama; 2006 Aug;60(4):237-42
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  • Angiomyofibroblastoma is a rare, usually small benign mesenchymal tumor that occurs in vulvar lesions of premenopausal women.
  • The maximum dimension of the tumor measured 11 cm.
  • The resected tumor was well circumscribed with a bulging and glistening cut surface.
  • In the present case, intralesional adipose tissue was present throughout the tumor.
  • There was little stromal mucin throughout the tumor.
  • These histological findings are consistent with angiomyofibroblastoma and support the hypothesis that angiomyofibroblastoma originates from perivascular stem cells with a capacity for myofibroblastic and fatty differentiation.

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  • (PMID = 16943862.001).
  • [ISSN] 0386-300X
  • [Journal-full-title] Acta medica Okayama
  • [ISO-abbreviation] Acta Med. Okayama
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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22. 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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23. Koenig AM, Quaas A, Ries T, Yekebas EF, Gawad KA, Vashist YK, Burdelski C, Mann O, Izbicki JR, Erbersdobler A: Perivascular epitheloid cell tumour (PEComa) of the retroperitoneum - a rare tumor with uncertain malignant behaviour: a case report. J Med Case Rep; 2009;3:62
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  • [Title] Perivascular epitheloid cell tumour (PEComa) of the retroperitoneum - a rare tumor with uncertain malignant behaviour: a case report.
  • INTRODUCTION: Perivascular epitheloid cell tumours are rare mesenchymal neoplasms characterized by a proliferation of perivascular cells with an epitheloid phenotype and expression of myomelanocytic markers.
  • CASE PRESENTATION: Here we present the case of a cystic perivascular epitheloid cell tumour of the retroperitoneum associated with multifocal lung lesions.
  • The tumour displayed the typical morphological and immunohistochemical features of a perivascular epitheloid cell tumour.
  • CONCLUSION: Since lymphangioleiomyomatosis, an otherwise benign condition, belongs to the family of perivascular epitheloid cell tumours, it cannot be excluded that the lung lesions in this case in fact represent metastases from the retroperitoneal perivascular epitheloid cell tumour rather than independent neoplasms.
  • More experience with this new and unusual tumour entity is clearly needed in order to define reliable criteria for benign or malignant behaviour.

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  • (PMID = 19220895.001).
  • [ISSN] 1752-1947
  • [Journal-full-title] Journal of medical case reports
  • [ISO-abbreviation] J Med Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2649938
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24. 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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25. Lau PP, Wong OK, Lui PC, Cheung OY, Ho LC, Wong WC, To KF, Chan JK: Myopericytoma in patients with AIDS: a new class of Epstein-Barr virus-associated tumor. Am J Surg Pathol; 2009 Nov;33(11):1666-72
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  • [Title] Myopericytoma in patients with AIDS: a new class of Epstein-Barr virus-associated tumor.
  • Myopericytoma is an uncommon, benign perivascular myoid cell tumor that occurs almost exclusively in somatic soft tissues.
  • Histologically, oval to plump spindly tumor cells with uniform nuclei and scanty cytoplasm formed sheets or cuffs around gaping or narrow vascular spaces.
  • The tumor cells were immunoreactive for actin but not desmin, and showed uniform labeling for Epstein-Barr virus (EBV) encoded RNAs on in-situ hybridization.
  • This tumor type therefore also broadens the spectrum of neoplasms associated with EBV.

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  • (PMID = 19675451.001).
  • [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 / RNA, Viral; 0 / RNA-Binding Proteins; 0 / Ribosomal Proteins; 135844-68-7 / RPL22 protein, human
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26. 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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  • 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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27. Murugesan M, Courtauld E, Fisher C, Nathan S: Renal capsular PEComa--a rare cause of surgically correctable renal hypertension. Int Urol Nephrol; 2007;39(3):705-7
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  • The mass was reported as a Perivascular Epitheloid Cell (PEC) lesion (PEComa) arising from the renal capsule.
  • Perivascular Epitheloid Cell tumor (PEComa), a recently defined tumor, is extremely rare.
  • This combined immunophenotype is a characteristic feature of the Perivascular Epitheloid Cell (PEC) lesions.
  • PEComa's are usually benign, but cases have been reported in the literature which has an unfavourable outcome with metastatic dissemination.

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  • [Cites] Histopathology. 2006 Jan;48(1):75-82 [16359539.001]
  • [Cites] Pathology. 1991 Jul;23(3):185-8 [1664078.001]
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  • (PMID = 17318350.001).
  • [ISSN] 0301-1623
  • [Journal-full-title] International urology and nephrology
  • [ISO-abbreviation] Int Urol Nephrol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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28. Sen S, Senturk E, Kuman NK, Pabuscu E, Kacar F: PEComa (clear cell "sugar" tumor) of the lung: a benign tumor that presented with thrombocytosis. Ann Thorac Surg; 2009 Dec;88(6):2013-5
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  • [Title] PEComa (clear cell "sugar" tumor) of the lung: a benign tumor that presented with thrombocytosis.
  • Perivascular epithelioid cell tumors of the lung are rare, benign neoplasms, usually presenting as a solitary pulmonary nodule on chest roentgenograms.
  • [MeSH-major] Lung Neoplasms / diagnosis. Perivascular Epithelioid Cell Neoplasms / diagnosis. Thrombocytosis / complications

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  • (PMID = 19932282.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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29. Brunschweiler B, Guedj N, Lenoir T, Faillot T, Rillardon L, Guigui P: Oncogenous osteomalacia and myopericytoma of the thoracic spine: a case report. Spine (Phila Pa 1976); 2009 Nov 1;34(23):E857-60
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  • SUMMARY OF BACKGROUND DATA: Osteomalacia related to a tumor is well known.
  • The cause of the disorder is usually a highly vascularized, benign tumor of mesenchymal origin.
  • Location of the tumor in the spine is very rare.
  • Removal of the tumor is followed by resolution of osteomalacia.
  • Localization of the tumor at T5 and T6 levels was obtained by magnetic resonance imaging.
  • Surgical treatment consisted in a circumferential correction-fusion with hemivertebrectomy of T5 and T6 and tumor removal.
  • RESULTS: Tumor removal was rapidly followed by disappearance of the clinical symptoms of osteomalacia, and by correction of hypophosphatemia.
  • At 2-years follow-up, no recurrence of the tumor was detectable on imaging studies-the correction fusion remained stable.
  • Histologically, the tumor was classified as a myopericytoma.
  • However, secondary recurrence of the biologic markers due to an incomplete tumor removal was disclosed.
  • CONCLUSION: Removal of the tumor was followed by healing of the clinical features of osteomalacia, demonstrating the causal connection between the myopericytoma and the osteopathy.
  • [MeSH-major] Osteomalacia / diagnosis. Perivascular Epithelioid Cell Neoplasms / complications. Spinal Neoplasms / complications. Thoracic Vertebrae / pathology
  • [MeSH-minor] Adult. Biomarkers, Tumor. Female. Follow-Up Studies. Humans. Spine / pathology. Spine / surgery. Treatment Outcome

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  • (PMID = 19927092.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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30. Mills AM, Longacre TA: Smooth Muscle Tumors of the Female Genital Tract. Surg Pathol Clin; 2009 Dec;2(4):625-77
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  • The vast majority of these neoplasms are clinically benign and easy to diagnose.
  • (2) increased mitotic index; and (3) tumor cell necrosis.
  • Certain benign smooth muscle proliferations may show worrisome histologic features or unusual growth patterns, causing concern for leiomyosarcoma.
  • Furthermore, other tumors, including perivascular epithelioid tumors, may mimic leiomyosarcoma.

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  • [Copyright] Copyright © 2009 Elsevier Inc. All rights reserved.
  • (PMID = 26838774.001).
  • [ISSN] 1875-9181
  • [Journal-full-title] Surgical pathology clinics
  • [ISO-abbreviation] Surg Pathol Clin
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Keywords] NOTNLM ; Atypical leiomyoma / Benign metastasizing leiomyoma / Disseminated peritoneal leiomyomatosis / Female genital tract / Intravenous leiomyomatosis / Leiomyoma / Leiomyosarcoma / Lymphangioleiomyomatosis / Ovary / PEComa / STUMP / Uterus / Vagina / Vulva
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31. 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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  • 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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32. 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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33. 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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  • 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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34. 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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  • 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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35. Sousa V, Carvalho L: [Glomic tumor: presentation of an infrequent case]. Rev Port Pneumol; 2006 May-Jun;12(3):269-74
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  • [Title] [Glomic tumor: presentation of an infrequent case].
  • [Transliterated title] Tumor glómico do pulmão: Apresentação de um caso pouco frequente.
  • Glomic tumours are perivascular tumours whose cells resemble modified smooth muscular cells of the glomic body.
  • Glomic tumours are generally benign.
  • They should be classified as glomic tumor, glomangioma and glomangiomioma according to the relative abundance of glomic cells and of the vascular and muscular components.
  • [MeSH-major] Glomus Tumor / diagnosis. Lung Neoplasms / diagnosis

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  • (PMID = 16967176.001).
  • [ISSN] 0873-2159
  • [Journal-full-title] Revista portuguesa de pneumologia
  • [ISO-abbreviation] Rev Port Pneumol
  • [Language] por
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Portugal
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36. Armah HB, Parwani AV: Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. Diagn Pathol; 2007;2:45
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  • [Title] Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature.
  • BACKGROUND: Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history.
  • These reported cases of uterine PEComa-NOS have usually shown clinically benign behavior, but 13 tumors, three of them associated with tuberous sclerosis complex (TSC), exhibited local aggressive behavior and four of them showed distant metastases.
  • Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the previous uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases.
  • Immunohistochemically, the tumor cells of both phenotypes in all three lesions stained for melanocytic (HMB-45 and Melan-A/MART-1) and myoid (desmin, smooth muscle actin, and muscle-specific actin/all muscle actin/HHF-35) markers.

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  • (PMID = 18053181.001).
  • [ISSN] 1746-1596
  • [Journal-full-title] Diagnostic pathology
  • [ISO-abbreviation] Diagn Pathol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2213634
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37. 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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38. 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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  • [Cites] Am J Surg Pathol. 2005 Dec;29(12):1558-75 [16327428.001]
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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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39. Liu JL, Lin YM, Lin MC, Yeh KT, Hsu JC, Chin CJ: Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. Hematol Oncol Stem Cell Ther; 2009;2(3):426-30
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  • [Title] Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation.
  • Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs).
  • Both benign and malignant tumors have been identified, but the criteria for diagnosis of malignancy have not been fully established due to the rarity of the tumor.
  • The tumor had the characteristic histologic features of PEComa with cytologic atypia, mitotic activity of 2/10 high power field (HPF), and necrosis; it exhibited immunopositivity for HMB-45, calponin and desmin and was negative for melan-A.
  • [MeSH-major] Perivascular Epithelioid Cell Neoplasms / pathology. Uterine Neoplasms / pathology

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  • (PMID = 20139058.001).
  • [ISSN] 1658-3876
  • [Journal-full-title] Hematology/oncology and stem cell therapy
  • [ISO-abbreviation] Hematol Oncol Stem Cell Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Saudi Arabia
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40. 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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41. Chen JF, Gao HW, Wu BY, Tsai WC, Chiang CP: Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: a rare case report with molecular evidence of T-cell clonality. J Dermatol; 2010 Apr;37(4):355-9
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  • Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare, benign entity of unknown pathogenesis.
  • Pathological findings showed dermal proliferation of anomalous blood vessels lined by plump endothelium with a significant perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, scattered plasma cells and many eosinophils.
  • We present this case to expand the anatomical distribution of this rare tumor.

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  • (PMID = 20507406.001).
  • [ISSN] 1346-8138
  • [Journal-full-title] The Journal of dermatology
  • [ISO-abbreviation] J. Dermatol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antigens, CD3; 0 / Receptors, Antigen, T-Cell
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42. Dominguez-Malagon H, Valdez-Carrillo Mdel C, Cano-Valdez AM: Dermatofibroma and dermatofibrosarcoma protuberans: a comparative ultrastructural study. Ultrastruct Pathol; 2006 Jul-Aug;30(4):283-91
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  • In contrast, DF is characterized by a proliferation of multiple capillary vessels with prominent endothelium and a perivascular population of ovoid or spindled cells devoid of cell processes.
  • With the ultrastructural characteristics and the constant expression of CD34 in DFSP, a probable origin in dermal dendrocytes is postulated for this tumor.
  • The histogenesis of DF is less clear, but an origin from FXIIIa modified perivascular dermal dendrocytes is proposed.
  • [MeSH-major] Dermatofibrosarcoma / ultrastructure. Histiocytoma, Benign Fibrous / ultrastructure. Skin Neoplasms / ultrastructure


43. Navarro-Pelayo Lainez MM, Ramos-Font C, Rebollo Aguirre AC, Rodríguez-Fernández A, Llamas-Elvira JM: [Perivascular epithelioid tumors: Utility of the positron emission tomography with 18F-fluorodesoxyglucose (PET-TAC FDG) in their staging and follow-up]. Rev Esp Med Nucl; 2010 Sep-Oct;29(5):258-62
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  • [Title] [Perivascular epithelioid tumors: Utility of the positron emission tomography with 18F-fluorodesoxyglucose (PET-TAC FDG) in their staging and follow-up].
  • [Transliterated title] Tumor epitelioide perivascular uterino. Utilidad de la tomografía por emisión de positrones con (18)F-fluordesoxiglucosa en su estadificación y seguimiento.
  • Perivascular epithelioid tumors (PEComas) are a rare group of mesenchymal neoplasms with an unpredictable natural history and uncertain malignant potential.
  • The findings suggest that since this is a rare tumor, which does not always have benign behaviour, PET-CT may be a useful diagnostic imaging procedure for staging and clinical monitoring of patients who suffer this type of tumors.
  • [MeSH-major] Endometrial Neoplasms / radionuclide imaging. Estrogens. Fluorine Radioisotopes. Fluorodeoxyglucose F18. Lung Neoplasms / secondary. Neoplasms, Hormone-Dependent / radionuclide imaging. Perivascular Epithelioid Cell Neoplasms / radionuclide imaging. Positron-Emission Tomography. Radiopharmaceuticals. Tomography, X-Ray Computed

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  • [Copyright] Copyright © 2010 Elsevier España, S.L. y SEMNIM. All rights reserved.
  • (PMID = 20462672.001).
  • [ISSN] 0212-6982
  • [Journal-full-title] Revista española de medicina nuclear
  • [ISO-abbreviation] Rev Esp Med Nucl
  • [Language] spa
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Estrogens; 0 / Fluorine Radioisotopes; 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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44. Armah HB, Parwani AV: Perivascular epithelioid cell tumor. Arch Pathol Lab Med; 2009 Apr;133(4):648-54
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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.
  • Perivascular epithelioid cell tumors are mesenchymal neoplasms defined by the presence of histologically and immunohistochemically distinctive perivascular epithelioid cells.
  • The perivascular epithelioid cell has no known normal tissue counterpart and coexpresses myoid and melanocytic markers.
  • This tumor family shows marked female predominance and includes angiomyolipoma, clear cell sugar tumor, lymphangioleiomyomatosis, and a group of rare, morphologically and immunophenotypically similar tumors arising at a variety of visceral and soft tissue sites.
  • This latter subset has been collectively termed perivascular epithelioid cell tumors-not otherwise specified.
  • They are usually composed of epithelioid, but occasionally spindled, cells with clear to granular eosinophilic cytoplasm and focal perivascular accentuation.
  • Although most cases are benign, a subset behaves in a malignant fashion.
  • This review focuses on the perivascular epithelioid cell tumors-not otherwise specified subset.
  • [MeSH-major] Perivascular Epithelioid Cell Neoplasms. Soft Tissue Neoplasms

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  • (PMID = 19391667.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 30
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45. Kajo K, Zúbor P, Krivus S, Danko J: [Angiolipoleiomyoma of the uterus. Case report and literature review]. Ceska Gynekol; 2010 Feb;75(1):54-6
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  • CONCLUSION: The ALLM of the uterus is rare benign mixed mesenchyme tumor consisting of smooth muscle bundles, foci of mature fat tissue and abnormal vessels.
  • HMB-45, what distinguishes them from angiomyolipomas, which are currently categorized into so called PEComas - tumors originating from perivascular epithelial cells.

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  • (PMID = 20437838.001).
  • [ISSN] 1210-7832
  • [Journal-full-title] Ceska gynekologie
  • [ISO-abbreviation] Ceska Gynekol
  • [Language] SLO
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] Czech Republic
  • [Number-of-references] 18
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46. 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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  • [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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47. 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


48. Lopater J, Daniel L, Akiki A, Boissier R, Lechevallier E, Coulange C: [Renal epithelioid angiomyolipoma]. Prog Urol; 2009 Jul;19(7):457-61
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  • ReAML are tumors derived from perivascular epithelioid cells (PEComa).
  • There are benign PEComas, potentially aggressive PEComas and malignant PEComas.
  • Partial nephrectomy or follow-up are the benign entity treatment.
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Biomarkers, Tumor / metabolism. Doxorubicin / administration & dosage. Humans. Nephrectomy / methods. Prognosis. Protein Kinases / metabolism. Sirolimus / administration & dosage. TOR Serine-Threonine Kinases. Treatment Outcome

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  • (PMID = 19559375.001).
  • [ISSN] 1166-7087
  • [Journal-full-title] Progrès en urologie : journal de l'Association française d'urologie et de la Société française d'urologie
  • [ISO-abbreviation] Prog. Urol.
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 80168379AG / Doxorubicin; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
  • [Number-of-references] 10
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49. 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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50. Walsh SN, Sangüeza 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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51. 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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52. 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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  • [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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53. 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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  • 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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54. Rouquie D, Eggenspieler P, Algayres JP, Béchade D, Camparo P, Baranger B: [Malignant-like angiomyolipoma of the liver: report of one case and review of the literature]. Ann Chir; 2006 May;131(5):338-41
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  • Frequently found in kidney, angiomyolipoma is a rare mesenchymal tumor when diagnosed in the liver and usually benign composed of proliferative blood vessels, fatty tissue and smooth muscle.
  • We report the case of a 67-year-old woman who underwent a left hepatectomy for a 4th segment tumor unidentified after imaging and fine needle biopsy.
  • Advances in imaging and anatomopathology in particular with the concept of PEComa (Perivascular-Epithelioïd Cell) as the unifying feature should lead to the recognition of the various variant patterns and cell types.

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  • (PMID = 16386232.001).
  • [ISSN] 0003-3944
  • [Journal-full-title] Annales de chirurgie
  • [ISO-abbreviation] Ann Chir
  • [Language] fre
  • [Publication-type] Case Reports; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 16
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55. Adachi Y, Kitamura Y, Nakamura H, Taniguchi Y, Miwa K, Horie Y, Hayashi K: Benign clear (sugar) cell tumor of the lung with CD1a expression. Pathol Int; 2006 Aug;56(8):453-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Benign clear (sugar) cell tumor of the lung with CD1a expression.
  • Reported herein is a case of benign clear cell tumor of the lung in a 60-year-old man.
  • The resected tumor was 13 mm in size, well-circumscribed and was graysh-white on cut surface.
  • Histological examination showed a diffuse growth pattern of polygonal tumor cells with indented and pleomorphic nuclei, and clear abundant cytoplasm with a distinct cell border surrounded by thin-walled vascular spaces and sinusoid-type vessels.
  • In the present case there was focal immunoreactivity for S-100 protein, HMB-45, neuron-specific enolase, cathepsin B and melan A, which are consistent with reported immunohistochemical staining patterns of benign clear cell tumor.
  • Based on these findings, the tumor was diagnosed as a benign clear cell tumor of the lung.
  • Although most clear cell tumors are considered to belong to the family of neoplasms with perivascular epithelioid cell differentiation (PEComas), histogenesis of benign clear cell tumor of the lung has remained unclear.
  • This first report of CD1a expression in this tumor might provide a new insight into its histogenesis and diagnosis.
  • [MeSH-major] Adenocarcinoma, Clear Cell / metabolism. Antigens, CD1 / metabolism. Biomarkers, Tumor / metabolism. Lung Neoplasms / pathology. Solitary Pulmonary Nodule / metabolism

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  • (PMID = 16872440.001).
  • [ISSN] 1320-5463
  • [Journal-full-title] Pathology international
  • [ISO-abbreviation] Pathol. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Australia
  • [Chemical-registry-number] 0 / Antigens, CD1; 0 / Biomarkers, Tumor; 0 / CD1a antigen
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56. 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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57. 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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58. De Dominicis G, Boscaino A, Marsilia GM, D'Antonio A, Nappi O: Clear cell "sugar" tumors of urethra: a previously undescribed occurrence and review of published data. Urology; 2009 Sep;74(3):542-3
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Clear cell "sugar" tumor is a rare benign tumor considered as a member of the family of perivascular epithelioid cell tumors.
  • To the best of our knowledge, this is the first case of clear cell "sugar" tumor presented as a mass protruding from the urethra in a 15-year-old girl.
  • Pathologic examination revealed a tumor composed of epithelioid cells with "water" clear cytoplasm that stained positively for melanocytic and smooth muscle-specific markers.
  • Treatment of this tumor included a surgical excision and complete removal of the urethral mass, with examination of surgical margins.
  • [MeSH-major] Perivascular Epithelioid Cell Neoplasms. Urethral Neoplasms

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  • (PMID = 19589571.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 4
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59. Vijayabhaskar R, Mehta SS, Deodhar KK, Pramesh CS, Mistry RC: PEComa of the lung. J Cancer Res Ther; 2010 Jan-Mar;6(1):109-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Perivascular epithelioid cell tumor (PEComa), also called clear cell ''sugar'' tumor of the lung, is a rare benign tumor arising from perivascular epithelioid cells (PECs).
  • We report a case of a 15-year-old boy who presented with right lower lobe lesion which turned out to be a clear cell tumor of the lung.
  • We discuss the clinical, radiologic and immunohistochemical features of clear cell ''sugar'' tumor of lung and compare them with published literature.
  • [MeSH-major] Lung Neoplasms / pathology. Perivascular Epithelioid Cell Neoplasms / pathology

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  • (PMID = 20479562.001).
  • [ISSN] 1998-4138
  • [Journal-full-title] Journal of cancer research and therapeutics
  • [ISO-abbreviation] J Cancer Res Ther
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] India
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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60. 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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61. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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62. Chu ZG, Yu JQ, Yang ZG, Zhu ZY, Yuan HM: Myopericytoma involving the parotid gland as depicted on multidetector CT. Korean J Radiol; 2009 Jul-Aug;10(4):398-401
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • Myopericytoma is a newly proposed subgroup of perivascular tumors in the World Health Organization classification of soft tissue tumors.
  • In this study, we report a case of a benign myopericytoma with detailed multidetector CT (MDCT) findings in the parotid gland, a location that has not been described for this type of tumor previously.

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  • (PMID = 19568469.001).
  • [ISSN] 2005-8330
  • [Journal-full-title] Korean journal of radiology
  • [ISO-abbreviation] Korean J Radiol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2702050
  • [Keywords] NOTNLM ; Myopericytoma / Parotid gland / Radiograph, CT
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63. de León DC, Pérez-Montiel D, Bandera A, Villegas C, Gonzalez-Conde E, Vilchis JC: Perivascular epithelioid cell tumor of abdominal origin. Ann Diagn Pathol; 2010 Jun;14(3):173-7
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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 of abdominal origin.
  • Perivascular epithelioid tumor is not a common disease; therefore, large series are not available in the literature, and most are case reports.
  • All these neoplasms have the characteristic perivascular epithelioid cell or "PEC," but the term myomelanocyte tumor has been proposed because of the immunohistochemistry expression.
  • We report 3 cases of abdominal PEComa, 1 of them with benign clinical outcome and 2 with an aggressive behavior.
  • [MeSH-major] Epithelioid Cells / pathology. Pelvic Neoplasms / pathology. Perivascular Epithelioid Cell Neoplasms / secondary
  • [MeSH-minor] Adult. Aged. Biomarkers, Tumor / metabolism. Combined Modality Therapy. Fatal Outcome. Female. Humans. Immunohistochemistry. Tomography, X-Ray Computed

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  • (PMID = 20471562.001).
  • [ISSN] 1532-8198
  • [Journal-full-title] Annals of diagnostic pathology
  • [ISO-abbreviation] Ann Diagn Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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64. 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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